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Sample records for canine pulmonary arterial

  1. Experimental study of pulmonary thromboembolism ischemia-reperfusion injury in canine model

    International Nuclear Information System (INIS)

    Li Jianjun; Zhai Renyou; Zhang Dongpo; Huang Qiang; Yu Ping; Dai Dingke; Bao Na

    2009-01-01

    Objective: To establish a canine model of pulmonary thromboembolism ischemia- reperfusion injury (PTE IRI) that may be used for imaging study. Methods: Ten male and 10 female healthy mongrel canines with (18.6±0.8) kg/body weight, were used. A Swan-Ganz catheter was introduced into the right internal jugular vein via a preset percutaneous sheath using the Seldinger technique, and then was with further insertion the pulmonary artery. Balloon occlusion of the right inferior lobe pulmonary artery for 4 hours was followed by removing the catheter and ending with 4 hours of reperfusion. CT was performed before ischemia, 4 h after ischemia and 4 h after reperfusion. At last, dogs were killed and the bilateral inferior lung tissues were prepared for the examination by light and electronic microscopy. Results: All canine models were successfully developed pulmonary thromboembolism ischemia-reperfusion injury. The examination of CT, light and electron microscopy consistently indicated the presence of permeability pulmonary edema after reperfusion. Conclusions: A closed-chest canine model in vivo of pulmonary thromboembolism ischemia-reperfusion injury can be established with virtual pathophysiological process in human and be as well as for imaging experimental study. (authors)

  2. Novel therapeutic approach for pulmonary emphysema using gelatin microspheres releasing basic fibroblast growth factor in a canine model.

    Science.gov (United States)

    Chang, Sung Soo; Yokomise, Hiroyasu; Matsuura, Natsumi; Gotoh, Masashi; Tabata, Yasuhiko

    2014-08-01

    The prognosis of patients with emphysema is poor as there is no truly effective treatment. Our previous study showed that the alveolar space was smaller and the microvessel density was higher in a canine emphysema model after the intrapulmonary arterial administration of gelatin microspheres slowly releasing basic fibroblast growth factor (bFGF-GMS). In the present study, we evaluated the functional effect of injecting bFGF-GMS via the pulmonary artery in this canine pulmonary emphysema model. Using the porcine pancreatic elastase (PPE)-induced total emphysema model, we approximated the value of lung compliance with a Power Lab System, and performed blood gas analysis in a control group, a total emphysema group, and a bFGF group in which bFGF-GMS were injected toward the whole pulmonary artery via the femoral vein. Each group comprised five dogs. Lung compliance was higher in the total emphysema group than in the control group (p = 0.031), and the bFGF group showed no significant improvement of lung compliance vs. the total emphysema group (p = 0.112). PaO2 (partial pressure of oxygen in arterial blood) was improved by administering bFGF-GMS in the total emphysema model (p = 0.027). In the canine total emphysema model, blood gas parameters were improved by the whole pulmonary arterial administration of bFGF-GMS. This method has the potential to be an effective novel therapy for pulmonary emphysema.

  3. Dual-energy CT based vascular iodine analysis improves sensitivity for peripheral pulmonary artery thrombus detection: An experimental study in canines

    Energy Technology Data Exchange (ETDEWEB)

    Tang, Chun Xiang [Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing University, Nanjing, Jiangsu 210002 (China); Zhang, Long Jiang, E-mail: kevinzhlj@163.com [Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing University, Nanjing, Jiangsu 210002 (China); Han, Zong Hong; Zhou, Chang Sheng [Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing University, Nanjing, Jiangsu 210002 (China); Krazinski, Aleksander W.; Silverman, Justin R. [Department of Radiology and Radiological Science, Medical University of South Carolina, Charleston, SC (United States); Schoepf, U. Joseph [Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing University, Nanjing, Jiangsu 210002 (China); Department of Radiology and Radiological Science, Medical University of South Carolina, Charleston, SC (United States); Lu, Guang Ming, E-mail: cjr.luguangming@vip.163.com [Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing University, Nanjing, Jiangsu 210002 (China)

    2013-12-01

    Purpose: To evaluate the performance of dual-energy CT (DECT) based vascular iodine analysis for the detection of acute peripheral pulmonary thrombus (PE) in a canine model with histopathological findings as the reference standard. Materials and methods: The study protocol was approved by our institutional animal committee. Thrombi (n = 12) or saline (n = 4) were intravenously injected via right femoral vein in sixteen dogs, respectively. CT pulmonary angiography (CTPA) in DECT mode was performed and conventional CTPA images and DECT based vascular iodine studies using Lung Vessels application were reconstructed. Two radiologists visually evaluated the number and location of PEs using conventional CTPA and DECT series on a per-animal and a per-clot basis. Detailed histopathological examination of lung specimens and catheter angiography served as reference standard. Sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV) of DECT and CTPA were calculated on a segmental and subsegmental or more distal pulmonary artery basis. Weighted κ values were computed to evaluate inter-modality and inter-reader agreement. Results: Thirteen dogs were enrolled for final image analysis (experimental group = 9, control group = 4). Histopathological results revealed 237 emboli in 45 lung lobes in 9 experimental dogs, 11 emboli in segmental pulmonary arteries, 49 in subsegmental pulmonary arteries, 177 in fifth-order or more distal pulmonary arteries. Overall sensitivity, specificity, accuracy, PPV, and NPV for CTPA plus DECT were 93.1%, 76.9%, 87.8%, 89.4%, and 84.2% for the detection of pulmonary emboli. With CTPA versus DECT, sensitivities, specificities, accuracies, PPVs, and NPVs are all 100% for the detection of pulmonary emboli on a segmental pulmonary artery basis, 88.9%, 100%, 96.0%, 100%, and 94.1% for CTPA and 90.4%, 93.0%, 92.0%, 88.7%, and 94.1% for DECT on a subsegmental pulmonary artery basis; 23.8%, 96.4%, 50.4%, 93

  4. Pulmonary artery-to-pulmonary artery anastomoses: angiographic demonstration in patients with chronic thromboembolic pulmonary hypertension

    International Nuclear Information System (INIS)

    Hodson, J.; Graham, A.; Hughes, J.M.B.; Gibbs, J.S.R.; Jackson, J.E.

    2006-01-01

    AIM: To describe direct pulmonary artery-to-pulmonary artery anastomoses seen at pulmonary angiography in patients with chronic thromboembolic pulmonary hypertension and discuss their possible significance. MATERIALS AND METHODS: Between 1 August 2000 and 31 July 2004 43 patients (male-to-female ratio 25:18) with a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) underwent selective pulmonary angiography to assess the extent of disease and suitability for surgical pulmonary endarterectomy. The mean pulmonary artery pressure ranged from 27-84 mmHg (average of 51 mmHg). Selective bilateral digital subtraction pulmonary angiograms performed in all individuals were reviewed for the presence of intrapulmonary collaterals. RESULTS: In 15 of the 43 patients (male-to-female ratio =7:8) definite (n=12) or probable (n=3) pulmonary artery-to-pulmonary artery anastomoses were demonstrated. Of the remaining 28 patients in whom intrapulmonary collaterals were not seen it was felt that in 16 the angiograms were of insufficient diagnostic quality (grades 4-5) to exclude their presence. Twelve patients, eight of whom had angiograms of sufficient diagnostic quality (grades 1-3), demonstrated one or more areas of luxury perfusion but intrapulmonary collaterals were not seen. CONCLUSION: Direct pulmonary artery-to-pulmonary artery anastomoses were demonstrated in patients with chronic thromboembolic pulmonary hypertension, which to our knowledge have not been previously described. The importance of these collateral vessels is unclear but they may play a role in the maintenance of pulmonary parenchymal viability in patients with chronic pulmonary embolic disease. The rate of development of these collaterals and their prognostic significance in patients with chronic thromboembolic pulmonary hypertension are areas worthy of further study

  5. Pulmonary artery-to-pulmonary artery anastomoses: angiographic demonstration in patients with chronic thromboembolic pulmonary hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Hodson, J. [Department of Imaging, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Graham, A. [Department of Imaging, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Hughes, J.M.B. [Department of Respiratory Medicine, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Gibbs, J.S.R. [Department of Cardiology, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom); Jackson, J.E. [Department of Imaging, Imperial College School of Medicine, Hammersmith Hospital, Du Cane Road, London (United Kingdom)]. E-mail: jejackson@hhnt.org

    2006-03-15

    AIM: To describe direct pulmonary artery-to-pulmonary artery anastomoses seen at pulmonary angiography in patients with chronic thromboembolic pulmonary hypertension and discuss their possible significance. MATERIALS AND METHODS: Between 1 August 2000 and 31 July 2004 43 patients (male-to-female ratio 25:18) with a diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) underwent selective pulmonary angiography to assess the extent of disease and suitability for surgical pulmonary endarterectomy. The mean pulmonary artery pressure ranged from 27-84 mmHg (average of 51 mmHg). Selective bilateral digital subtraction pulmonary angiograms performed in all individuals were reviewed for the presence of intrapulmonary collaterals. RESULTS: In 15 of the 43 patients (male-to-female ratio =7:8) definite (n=12) or probable (n=3) pulmonary artery-to-pulmonary artery anastomoses were demonstrated. Of the remaining 28 patients in whom intrapulmonary collaterals were not seen it was felt that in 16 the angiograms were of insufficient diagnostic quality (grades 4-5) to exclude their presence. Twelve patients, eight of whom had angiograms of sufficient diagnostic quality (grades 1-3), demonstrated one or more areas of luxury perfusion but intrapulmonary collaterals were not seen. CONCLUSION: Direct pulmonary artery-to-pulmonary artery anastomoses were demonstrated in patients with chronic thromboembolic pulmonary hypertension, which to our knowledge have not been previously described. The importance of these collateral vessels is unclear but they may play a role in the maintenance of pulmonary parenchymal viability in patients with chronic pulmonary embolic disease. The rate of development of these collaterals and their prognostic significance in patients with chronic thromboembolic pulmonary hypertension are areas worthy of further study.

  6. Cardiovascular effects of right ventricle-pulmonary artery valved conduit implantation in experimental pulmonic stenosis

    International Nuclear Information System (INIS)

    Saida, Y.; Tanaka, R.; Fukushima, R.; Hoshi, K.; Hira, S.; Soda, A.; Iizuka, T.; Ishikawa, T.; Nishimura, T.; Yamane, Y.

    2009-01-01

    Right ventricle (RV)-pulmonary artery (PA) valved conduit (RPVC) implantation decreases RV systolic pressure in pulmonic stenosis (PS) by forming a bypass route between the RV and the PA. The present study evaluates valved conduits derived from canine aortae in a canine model of PS produced by pulmonary artery banding (PAB). Pulmonary stenosis was elicited using PAB in 10 conditioned beagles aged 8 months. Twelve weeks after PAB, the dogs were assigned to one group that did not undergo surgical intervention and another that underwent RPVC using denacol-treated canine aortic valved grafts (PAB+RPVC). Twelve weeks later, the rate of change in the RV-PA systolic pressure gradient was significantly decreased in the PAB+RPVC, compared with the PAB group (60.5+-16.7% vs. 108.9+-22.9%; p0.01). In addition, the end-diastolic RV free wall thickness (RVFWd) was significantly reduced in the PAB+RPVC, compared with the PAB group (8.2+-0.2 vs. 9.4+-0.7 mm; p0.05). Thereafter, regurgitation was not evident beyond the conduit valve and the decrease in RV pressure overload induced by RPVC was confirmed. The present results indicate that RPVC can be performed under a beating heart without cardiopulmonary bypass and adapted to dogs with various types of PS, including 'supra valvular' PS or PS accompanied by dysplasia of the pulmonary valve. Therefore, we consider that this method is useful for treating PS in small animals

  7. The Critical Role of Pulmonary Arterial Compliance in Pulmonary Hypertension

    Science.gov (United States)

    Prins, Kurt W.; Pritzker, Marc R.; Scandurra, John; Volmers, Karl; Weir, E. Kenneth

    2016-01-01

    The normal pulmonary circulation is a low-pressure, high-compliance system. Pulmonary arterial compliance decreases in the presence of pulmonary hypertension because of increased extracellular matrix/collagen deposition in the pulmonary arteries. Loss of pulmonary arterial compliance has been consistently shown to be a predictor of increased mortality in patients with pulmonary hypertension, even more so than pulmonary vascular resistance in some studies. Decreased pulmonary arterial compliance causes premature reflection of waves from the distal pulmonary vasculature, leading to increased pulsatile right ventricular afterload and eventually right ventricular failure. Evidence suggests that decreased pulmonary arterial compliance is a cause rather than a consequence of distal small vessel proliferative vasculopathy. Pulmonary arterial compliance decreases early in the disease process even when pulmonary artery pressure and pulmonary vascular resistance are normal, potentially enabling early diagnosis of pulmonary vascular disease, especially in high-risk populations. With the recognition of the prognostic importance of pulmonary arterial compliance, its impact on right ventricular function, and its contributory role in the development and progression of distal small-vessel proliferative vasculopathy, pulmonary arterial compliance is an attractive target for the treatment of pulmonary hypertension. PMID:26848601

  8. Models of experimental saccular aneurysms of carotid arteries in canine

    International Nuclear Information System (INIS)

    Zhang Haixia; Cheng Yingsheng; Li Minghua

    2006-01-01

    Objective: To study the availability by making experimental saccular aneurysm models of carotid arteries in canine similar to human intracranial aneurysms. Methods: Twenty healthy canines with experimental saccular side-wall aneurysms of carotid arteries were created successfully by surgery. Results: Forty experimental saccular side-wall aneurysms of carotid arteries were created successfully with 36 aneurysms and parent arteries maintaining patency with each other and four spontaneously occluded confirmed by angiography. Model successful rate reached 90%. Conclusions: Experimental saccular side-wall aneurysms of carotid arteries in canines were one of best models created for simulating human intracranial aneurysms. (authors)

  9. Establishing experimental model of human internal carotid artery siphon segment in canine common carotid artery

    International Nuclear Information System (INIS)

    Cui Xuee; Li Minghua; Wang Yongli; Cheng Yingsheng; Li Wenbin

    2005-01-01

    Objective: To study the feasibility of establishing experimental model of human internal carotid artery siphon segment in canine common carotid artery (CCA) by end-to-end anastomoses of one side common carotid artery segment with the other side common carotid artery. Methods: Surgical techniques were used to make siphon model in 8 canines. One side CCA was taken as the parent artery and anastomosing with the cut off contra-lateral CCA segment which has passed through within the S-shaped glass tube. Two weeks after the creation of models angiography showed the model siphons were patent. Results: Experimental models of human internal carotid artery siphon segment were successfully made in all 8 dogs. Conclusions: It is practically feasible to establish experimental canine common carotid artery models of siphon segment simulating human internal carotid artery. (authors)

  10. Pulmonary artery aneurysm

    African Journals Online (AJOL)

    Enrique

    Introduction. Pulmonary artery aneurysms are a rare finding in general radiological practice. The possible causes are myriad and diverse in pathophysiolo- gy. Patients with post-stenotic dilata- tion of the main pulmonary artery usually present fairly late with insidi- ous cardiorespiratory symptoms. Diagnosis requires ...

  11. Pulmonary arterial hypertension : an update

    NARCIS (Netherlands)

    Hoendermis, E. S.

    2011-01-01

    Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance

  12. Systemic Arterial-to-Pulmonary Artery Shunt Utilization

    African Journals Online (AJOL)

    multiruka1

    In certain circumstances, such as cyanotic neonates with tetralogy of Fallot (4) or cyanotic patients with. Tetralogy of Fallot and hypoplastic pulmonary arteries. (5), better outcomes are obtained if definitive surgery. (total correction or palliation) is preceded by creation of a systemic arterial-to-pulmonary artery shunt (SAPAS).

  13. Computerized digital image processing on radiographs of canine filariosis

    International Nuclear Information System (INIS)

    Miyatake, K.; Okamoto, Y.; Minami, S.

    1999-01-01

    For objective evaluation in the lung arterial lesions, density histogram revealed by survey thoracic radiographies of fifteen canine filariosis and five normal canine were digitally analyzed, and preparation of pulmonary artery angiogram with inflated-fixed lung, the changes in the histogram and the pulmonary arterial lesion by a soft X-ray examination were compared. In the lung areas affected by filariosis, the density histogram increased the white level and decreased the black level in each part compared to a normal lung. In comparison with the normal parameters, those of the filariosis it were significantly increased in minimum grey level values (Min), maximum grey level values (Max), and the maximum frequency grey level values (Mode) and, it was significantly decreased in maximum frequency values (MaF). The pulmonary arterial lesion of the filariosis showed obvious morphological changes such as in distinction, pruning, angiectasis, and meandering. In the grade of pulmonary arterial lesion, the parameter Min, Max, Mode and MaF were changed significantly. From these results, it was clear that the methods for the lung arterial lesions analysis of X-ray images were confirmed to be highly beneficial in the lung arterial lesions for objective diagnosis

  14. Pulmonary Artery Dissection: A Fatal Complication of Pulmonary Hypertension

    Directory of Open Access Journals (Sweden)

    Chuanchen Zhang

    2016-01-01

    Full Text Available Pulmonary artery dissection is extremely rare but it is a really life-threatening condition when it happens. Most patients die suddenly from major bleeding or tamponade caused by direct rupture into mediastinum or retrograde into the pericardial sac. What we are reporting is a rare case of a 46-year-old female patient whose pulmonary artery dissection involves both the pulmonary valve and right pulmonary artery. The patient had acute chest pain and severe dyspnea, and the diagnosis of pulmonary artery dissection was confirmed by ultrasonography and CT angiography. Moreover, its etiology, clinical manifestations, and management are also discussed in this article.

  15. Bronchial arterial infusion versus bronchial combined pulmonary arterial infusion for pulmonary metastatic tumors

    International Nuclear Information System (INIS)

    Dong Sheng; Dong Weihua; Jia Ningyang; Zhang Dianbo; Xiao Xiangsheng

    2008-01-01

    Objective: To evaluate the pulmonary metastatic tumor response to different ways of transcatheter arterial infusion. Methods: Thirty-five patients with pulmonary metastatic tumors were randomized divided into two groups: 15 patients with 49 lesions treated with bronchial arterial infusion (BAI) and 20 patients with 65 lesions treated with bronchial arterial infusion (BM)combined with pulmonary arterial infusion (PAI). The therapeutic response was assessed by the WHO evaluation criteria. Results: The total effective rate(CR + PR) of BAI was 65.3% (32/49), PAI + BAI was 61.5%(40/65) showing no statistical difference. The median survival time of BAI was 9 mo, BAI + PAI was 11.5 mo, demonstrating no statistical significance. Conclusions: BAI should be the primary treatment for pulmonary metastatic tumor. (authors)

  16. Genetics Home Reference: pulmonary arterial hypertension

    Science.gov (United States)

    ... Home Health Conditions Pulmonary arterial hypertension Pulmonary arterial hypertension Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high ...

  17. Evaluation of pulmonary artery flow in acute massive pulmonary thromboembolism with MRI

    International Nuclear Information System (INIS)

    Li Yongzhong; Li Kuncheng; Zhao Xigang; Zhao Hong

    2004-01-01

    Objective: To probe into the value of MR imaging in evaluating the pulmonary artery hemodynamics and pulmonary artery pressure in acute massive pulmonary embolism. Methods: MR studies were performed in 21 patients with acute massive pulmonary embolism (diagnosed by contrast enhanced MR pulmonary angiography) and 20 healthy volunteers. The pulmonary artery hemodynamic parameters, such as the diameters of main and right pulmonary artery, peak velocity, average velocity, flow volume, flow patterns, and ejection acceleration time in main pulmonary artery were measured. The findings in patients and volunteers were compared. The hemodynamic parameters in patients were correlated with mean pulmonary artery pressure acquired with right heart catheterization. Results: The diameters of main pulmonary artery (2.93 vs 2.52 cm) and right pulmonary artery (2.49 vs 1.92 cm) in patients and volunteers showed significant differences (t=3.55, P<0.01 and t=4.19, P<0.01, respectively); Peak velocity (85.29 vs 100.63 cm/s), average velocity (11.00 vs 17.12 cm/s), flow volume (89.15 vs 98.96 ml/s), and ejection acceleration time (105.09 vs 163.85 ms) in main pulmonary artery were significantly different between patients and volunteers (t values were 2.89, 6.37, 2.21, and 9.46, respectively; P values were 0.01, <0.01, 0.03, and <0.01, respectively). The peak velocity-time curve of main pulmonary artery acquired with velocity encoded cine of MR in patients demonstrated earlier and lower peak velocity as well as abnormal retrograde flow. In addition, linear correlations were seen between the mean pulmonary pressure and the diameter of main pulmonary artery (r=0.62, P=0.001), diameter of right pulmonary artery (r=0.63, P=0.001), and ejection acceleration time (r=-0.55, P=0.005). Conclusion: MR imaging is a promising technique not only for the detection of pulmonary thromboemboli but also for the evaluation of hemodynamic parameters in pulmonary hypertension. (author)

  18. Pulmonary Arterial Hypertension

    Science.gov (United States)

    ... heart). This type of pulmonary hypertension was called “secondary pulmonary hypertension” but is now referred to as PH, because the cause is known to be from lung disease, heart disease, or chronic thromboemboli (blood clots). Pulmonary Arterial Hypertension (PAH) used to be ...

  19. Pulmonary venous remodeling in COPD-pulmonary hypertension and idiopathic pulmonary arterial hypertension

    DEFF Research Database (Denmark)

    Andersen, Kasper Hasseriis; Andersen, Claus Bøgelund; Gustafsson, Finn

    2017-01-01

    Pulmonary vascular arterial remodeling is an integral and well-understood component of pulmonary hypertension (PH). In contrast, morphological alterations of pulmonary veins in PH are scarcely described. Explanted lungs (n = 101) from transplant recipients with advanced chronic obstructive...... pulmonary disease (COPD) and idiopathic pulmonary arterial hypertension (IPAH) were analyzed for venous vascular involvement according to a pre-specified, semi-quantitative grading scheme, which categorizes the intensity of venous remodeling in three groups of incremental severity: venous hypertensive (VH......) grade 0 = characterized by an absence of venous vascular remodeling; VH grade 1 = defined by a dominance of either arterialization or intimal fibrosis; and VH grade 2 = a substantial composite of arterialization and intimal fibrosis. Patients were grouped according to clinical and hemodynamic...

  20. Isolated Left Pulmonary Artery Agenesis: A Case Report

    Directory of Open Access Journals (Sweden)

    Tansel Ansal Balcı

    2012-08-01

    Full Text Available Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung with normal ventilation was visualized on scintigraphy. MDCT angiography of pulmonary arteries showed absent left main pulmonary artery with systemic collaterals around left hemithorax. Pulmonary artery agenesis can be asymptomatic and isolated until adulthood. Both scintigraphy and CT angiography images of pulmonary artery agenesis of a patient are rare in the literature. Pulmonary ventilation- perfusion scintigraphy can be used not only for pulmonary embolism but also pathologies involving pulmonary artery and its branches. (MIRT 2012;21:80-83

  1. Isolated Unilateral Absent Branch Pulmonary Artery with Peripheral Pulmonary Stenosis and Coronary Artery Disease

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    Sunil Abhishek B

    2017-09-01

    Full Text Available Isolated Unilateral Absent Pulmonary Artery (UAPA is a rare congenital anomaly. It is usually associated with congenital heart defects. A 45 year old male patient presented with complaints of fever with cough and expectoration for 15 days and retrosternal chest discomfort for the previous 2 days. ECG showed diffuse ST segment depression with T wave inversion in the inferior and lateral leads. Coronary Angiogram done through the right femoral approach revealed diffusely diseased Left Anterior Descending (LAD artery that was totally cut off at the mid segment. The Left Circumflex (LCx artery was providing blood supply to the right middle and lower lung areas. There was another collateral arising from the Left Subclavian Artery supplying the right middle and lower lung areas. The left pulmonary artery was normal, but branches supplying the middle and lower lobes of the right lung were absent and the upper lobe branch had pulmonary stenosis. UAPA is a rare clinical entity; collaterals from coronaries are extremely rare in this condition and till now there has not been any case report of unilateral absent branch pulmonary artery with peripheral stenosis of other branches, on the affected side and associated coronary artery disease.

  2. Aberrant origin of the upper left lobe anterior and superior lingular segmental pulmonary artery arising from the right pulmonary artery: A case report

    International Nuclear Information System (INIS)

    Cho, Yong Seok; Kang, Mi Jin; Bae, Kyung Eun; Lee, Jin Hae; Lee, Han Bee; Kim, Jae Hyung; Jeong, Myeong Ja; Kang, Tae Kyung

    2013-01-01

    Aberrant origins of the pulmonary artery are rare anomalies, but are being reported several times in the world literature. Among them, pulmonary artery sling is the most well known anomaly, which is the left pulmonary artery arising from the right pulmonary artery. In case of pulmonary artery sling, the left pulmonary artery causes compression of the trachea and esophagus while it courses in between. In this case, we describe a case on incidentally found aberrant origin of the upper left lobe anterior and superior lingular segmental pulmonary artery without any compressions of the esophagus or trachea. In the world literature, aberrant origins of the left pulmonary artery without pulmonary artery sling was reported at one time, but currently, this is the first case of aberrant origin of the upper left lobe anterior and superior lingular segmental pulmonary artery arising from the right pulmonary artery.

  3. Aberrant origin of the upper left lobe anterior and superior lingular segmental pulmonary artery arising from the right pulmonary artery: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Yong Seok; Kang, Mi Jin; Bae, Kyung Eun; Lee, Jin Hae; Lee, Han Bee; Kim, Jae Hyung; Jeong, Myeong Ja; Kang, Tae Kyung [Sanggye Paik Hospital, Inje University College of Medicine, Seoul, (Korea, Republic of)

    2013-10-15

    Aberrant origins of the pulmonary artery are rare anomalies, but are being reported several times in the world literature. Among them, pulmonary artery sling is the most well known anomaly, which is the left pulmonary artery arising from the right pulmonary artery. In case of pulmonary artery sling, the left pulmonary artery causes compression of the trachea and esophagus while it courses in between. In this case, we describe a case on incidentally found aberrant origin of the upper left lobe anterior and superior lingular segmental pulmonary artery without any compressions of the esophagus or trachea. In the world literature, aberrant origins of the left pulmonary artery without pulmonary artery sling was reported at one time, but currently, this is the first case of aberrant origin of the upper left lobe anterior and superior lingular segmental pulmonary artery arising from the right pulmonary artery.

  4. Quantitative analysis of pulmonary artery and pulmonary collaterals in preoperative patients with pulmonary artery atresia using dual-source computed tomography

    International Nuclear Information System (INIS)

    Yin Lei; Lu, Bin; Han Lei; Wu Runze; Johnson, Laura; Xu Zhongying; Jiang Shiliang; Dai Ruping

    2011-01-01

    Objective: To evaluate the value of dual-source computed tomography (DSCT) in quantitatively measuring pulmonary arteries and major aortopulmonary collateral vessels in comparison with conventional angiographic (CA) on preoperative patients with pulmonary artery atresia and ventricular septal defect (PAA-VSD). Materials and methods: Twenty PAA-VSD patients who had complete imaging data of DSCT, CA and echocardiography (ECHO) studies were retrospectively analyzed. Using final clinical diagnosis as the standard, results of DSCT, CA and ECHO on the detection of cardiac malformations, measurement of diameters of pulmonary artery and collateral vessel, as well as the values of McGoon ratio, pulmonary arterial index (PAI) and total neopulmonary arterial index (TNPAI) were derived and compared. Results: In 20 patients, 51 of 54 (94.4%) cardiac malformations were visualized by DSCT, whereas 42 (77.8%) by ECHO (p = 0.027). Fourteen cases with aortopulmonary collateral vessels were all (100%) detected by DSCT, whereas 5 cases (35.7%) by ECHO (p = 0.001), and 13 cases (92.9%) by CA (p = 0.995). Sixteen cases with confluence of native pulmonary arteries were diagnosed by DSCT, whereas 10 cases by CA (p = 0.024). Measurement of the diameters of pulmonary arteries, collateral vessels, and descending aorta at the level of diaphragm were correlated well between DSCT and CA (r = 0.95-0.99). McGoon ratio (DSCT = 1.18 ± 0.60, CA = 1.23 ± 0.64), PAI (DSCT = 130.96 ± 99.38 mm 2 /m 2 , CA = 140.91 ± 107.87 mm 2 /m 2 ) and TNPAI (DSCT = 160.31 ± 125.62 mm 2 /m 2 , CA = 169.14 ± 122.81 mm 2 /m 2 ) were calculated respectively, without significant differences between DSCT and CA by paired t-tests (all p > 0.05). Conclusion: DSCT was efficient for evaluating and measuring native pulmonary artery and aortopulmonary collateral vessels prior to surgical procedures in PAA-VSD patients. Combined with echocardiography, DSCT showed potential to replace CA for evaluating pulmonary artery

  5. Pulmonary Artery Agenesis: A Case Series

    Directory of Open Access Journals (Sweden)

    Meltem Ağca

    2015-04-01

    Full Text Available Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis.The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature.

  6. Circulating microparticles in severe pulmonary arterial hypertension increase intercellular adhesion molecule-1 expression selectively in pulmonary artery endothelium

    Directory of Open Access Journals (Sweden)

    Leslie A. Blair

    2016-10-01

    Full Text Available Abstract Background Microparticles (MPs stimulate inflammatory adhesion molecule expression in systemic vascular diseases, however it is unknown whether circulating MPs stimulate localized ICAM-1 expression in the heterogeneically distinct pulmonary endothelium during pulmonary arterial hypertension (PAH. Pulmonary vascular lesions with infiltrating inflammatory cells in PAH form in the pulmonary arteries and arterioles, but not the microcirculation. Therefore, we sought to determine whether circulating MPs from PAH stimulate pulmonary artery endothelial cell-selective ICAM-1 expression. Results Pulmonary artery endothelial cells (PAECs were exposed to MPs isolated from the circulation of a rat model of severe PAH. During late-stage (8-weeks PAH, but not early-stage (3-weeks, an increase in ICAM-1 was observed. To determine whether PAH MP-induced ICAM-1 was selective for a specific segment of the pulmonary circulation, pulmonary microvascular endothelial cells (PMVECs were exposed to late-stage PAH MPs and no increase in ICAM-1 was detected. A select population of circulating MPs, the late-stage endoglin + MPs, were used to assess their ability to stimulate ICAM-1 and it was determined that the endoglin + MPs were sufficient to promote ICAM-1 increases in the whole cell, but not surface only expression. Conclusions Late-stage, but not early-stage, MPs in a model of severe PAH selectively induce ICAM-1 in pulmonary artery endothelium, but not pulmonary microcirculation. Further, the selected endoglin + PAH MPs, but not endoglin + MPs from control, are sufficient to promote whole cell ICAM-1 in PAECs. The implications of this work are that MPs in late-stage PAH are capable of inducing ICAM-1 expression selectively in the pulmonary artery. ICAM-1 likely plays a significant role in the observed inflammatory cell recruitment, specifically to vascular lesions in the pulmonary artery and not the pulmonary microcirculation.

  7. Computed tomographic diagnosis of pulmonary artery aneurysm

    International Nuclear Information System (INIS)

    Maeno, Kouji; Kontani, Kazuhiro; Ito, Makoto; Sakurai, Noboru; Sawada, Taisei; Fukeda, Yasuhiko; Takata, Shigeo; Ikeda, Takayuki; Hattori, Nobu.

    1986-01-01

    Pulmonary artery aneurysms are rare lesions. Clagett et al reported that one aneurysm of the pulmonary artery may be found in approximately 14,000 necropsies. We have experienced a case of giant pulmonary artery aneurysm confirmed by computed tomography. A 38 year-old man with atrial septal defect admitted to Kanazawa City Hospital. He had been pointed out of a right hilar mass when he was 26 years old. His complaint was bloody sputum and cough. Pulmonary angiography was not useful for the definite diagnosis because of its mural thrombi. Enhanced computed tomography showed a giant pulmonary artery aneurysm with a mural thrombi in its cavity. This shows that enhanced computed tomography is very useful for the diagnosis of a pulmonary artery aneurysm with a mural thrombi in its cavity. (author)

  8. Computed tomographic diagnosis of pulmonary artery aneurysm

    Energy Technology Data Exchange (ETDEWEB)

    Maeno, Kouji; Kontani, Kazuhiro; Ito, Makoto; Sakurai, Noboru; Sawada, Taisei; Fukeda, Yasuhiko; Takata, Shigeo; Ikeda, Takayuki; Hattori, Nobu

    1986-05-01

    Pulmonary artery aneurysms are rare lesions. Clagett et al reported that one aneurysm of the pulmonary artery may be found in approximately 14,000 necropsies. We have experienced a case of giant pulmonary artery aneurysm confirmed by computed tomography. A 38 year-old man with atrial septal defect admitted to Kanazawa City Hospital. He had been pointed out of a right hilar mass when he was 26 years old. His complaint was bloody sputum and cough. Pulmonary angiography was not useful for the definite diagnosis because of its mural thrombi. Enhanced computed tomography showed a giant pulmonary artery aneurysm with a mural thrombi in its cavity. This shows that enhanced computed tomography is very useful for the diagnosis of a pulmonary artery aneurysm with a mural thrombi in its cavity.

  9. Beneficial Effects of Renal Denervation on Pulmonary Vascular Remodeling in Experimental Pulmonary Artery Hypertension.

    Science.gov (United States)

    Qingyan, Zhao; Xuejun, Jiang; Yanhong, Tang; Zixuan, Dai; Xiaozhan, Wang; Xule, Wang; Zongwen, Guo; Wei, Hu; Shengbo, Yu; Congxin, Huang

    2015-07-01

    Activation of both the sympathetic nervous system and the renin-angiotensin-aldosterone system is closely associated with pulmonary arterial hypertension. We hypothesized that renal denervation decreases renin-angiotensin-aldosterone activity and inhibits the progression of pulmonary arterial hypertension. Twenty-two beagles were randomized into 3 groups. The dogs' pulmonary dynamics were measured before and 8 weeks after injection of 0.1mL/kg dimethylformamide (control dogs) or 2mg/kg dehydromonocrotaline (pulmonary arterial hypertension and pulmonary arterial hypertension + renal denervation dogs). Eight weeks after injection, neurohormone levels and pulmonary tissue morphology were measured. Levels of plasma angiotensin II and endothelin-1 were significantly increased after 8 weeks in the pulmonary arterial hypertension dogs and were higher in the lung tissues of these dogs than in those of the control and renal denervation dogs (mean [standard deviation] angiotensin II: 65 [9.8] vs 38 [6.7], 46 [8.1]; endothelin-1: 96 [10.3] vs 54 [6.2], 67 [9.4]; P < .01). Dehydromonocrotaline increased the mean pulmonary arterial pressure (16 [3.4] mmHg vs 33 [7.3] mmHg; P < .01), and renal denervation prevented this increase. Pulmonary smooth muscle cell proliferation was higher in the pulmonary arterial hypertension dogs than in the control and pulmonary arterial hypertension + renal denervation dogs. Renal denervation attenuates pulmonary vascular remodeling and decreases pulmonary arterial pressure in experimental pulmonary arterial hypertension. The effect of renal denervation may contribute to decreased neurohormone levels. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  10. Rare Presentation of Left Lower Lobe Pulmonary Artery Dissection

    Directory of Open Access Journals (Sweden)

    René Hako

    2017-01-01

    Full Text Available Background. Pulmonary arterial dissection with chronic pulmonary arterial hypertension as its major cause is a very rare but life-threatening condition. In most cases the main pulmonary trunk is the affected site usually without involvement of its branches. Segmental or lobar pulmonary artery dissection is extremely rare. Case Presentation. We report a unique case of left lower lobe pulmonary artery dissection in a 70-year-old male, with confirmed chronic pulmonary hypertension. To confirm dissection MDCT pulmonary angiography was used. Multiplanar reformation (MPR images in sagittal, coronal, oblique sagittal, and curved projections were generated. This case report presents morphologic CT features of rare chronic left lobar pulmonary artery dissection associated with chronic pulmonary hypertension at a place of localised pulmonary artery calcification. CT pulmonary angiography excluded signs of thromboembolism and potential motion or flow artefacts. Conclusion. To the best of our knowledge, no case of lower lobe pulmonary artery dissection with flap calcification has been reported yet. CT imaging of the chest is a key diagnostic tool that is able to detect an intimal flap and a false lumen within the pulmonary arterial tree and is preferred in differential diagnosis of rare complications of sustained pulmonary arterial hypertension.

  11. Pulmonary arterial hypertension in children: diagnosis using ratio of main pulmonary artery to ascending aorta diameter as determined by multi-detector computed tomography.

    Science.gov (United States)

    Caro-Domínguez, Pablo; Compton, Gregory; Humpl, Tilman; Manson, David E

    2016-09-01

    The ratio of the transverse diameter of the main pulmonary artery (MPA) to ascending aorta as determined at multi-detector CT is a tool that can be used to assess the pulmonary arterial size in cases of pulmonary arterial hypertension in children. To establish a ratio of MPA to ascending aorta diameter using multi-detector CT imaging suggestive of pulmonary arterial hypertension in children. We hypothesize that a defined ratio of MPA to ascending aorta is identifiable on multi-detector CT and that higher ratios can be used to reliably diagnose the presence of pulmonary arterial hypertension in children. We calculated the multi-detector CT ratio of MPA to ascending aorta diameter in 44 children with documented pulmonary arterial hypertension by right heart catheterization and in 44 age- and gender-matched control children with no predisposing factors for pulmonary arterial hypertension. We compared this multi-detector-CT-determined ratio with the MPA pressure in the study group, as well as with the ratio of MPA to ascending aorta in the control group. A threshold ratio value was calculated to accurately identify children with pulmonary arterial hypertension. Children with documented primary pulmonary arterial hypertension have a significantly higher ratio of MPA to ascending aorta (1.46) than children without pulmonary arterial hypertension (1.11). A ratio of 1.3 carries a positive likelihood of 34 and a positive predictive value of 97% for the diagnosis of pulmonary arterial hypertension. The pulmonary arteries were larger in children with pulmonary arterial hypertension than in a control group of normal children. A CT-measured ratio of MPA to ascending aorta of 1.3 should raise the suspicion of pulmonary arterial hypertension in children.

  12. Pulmonary Arterial Hypertension and Neonatal Arterial Switch Surgery for Correction of Transposition of the Great Arteries.

    Science.gov (United States)

    Domínguez Manzano, Paula; Mendoza Soto, Alberto; Román Barba, Violeta; Moreno Galdó, Antonio; Galindo Izquierdo, Alberto

    2016-09-01

    There are few reports of the appearance of pulmonary arterial hypertension following arterial switch surgery in the neonatal period to correct transposition of the great arteries. We assessed the frequency and clinical pattern of this complication in our series of patients. Our database was reviewed to select patients with transposition of the great arteries corrected by neonatal arterial switch at our hospital and who developed pulmonary hypertension over time. We identified 2 (1.3%) patients with transposition of the great arteries successfully repaired in the first week of life who later experienced pulmonary arterial hypertension. The first patient was a 7-year-old girl diagnosed with severe pulmonary hypertension at age 8 months who did not respond to medical treatment and required lung transplantation. The anatomic pathology findings were consistent with severe pulmonary arterial hypertension. The second patient was a 24-month-old boy diagnosed with severe pulmonary hypertension at age 13 months who did not respond to medical therapy. Pulmonary hypertension is a rare but very severe complication that should be investigated in all patients with transposition of the great arteries who have undergone neonatal arterial switch, in order to start early aggressive therapy for affected patients, given the poor therapeutic response and poor prognosis involved. Copyright © 2016 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  13. Pulmonary arterial hypertension in children: diagnosis using ratio of main pulmonary artery to ascending aorta diameter as determined by multi-detector computed tomography

    International Nuclear Information System (INIS)

    Caro-Dominguez, Pablo; Manson, David E.; Compton, Gregory; Humpl, Tilman

    2016-01-01

    The ratio of the transverse diameter of the main pulmonary artery (MPA) to ascending aorta as determined at multi-detector CT is a tool that can be used to assess the pulmonary arterial size in cases of pulmonary arterial hypertension in children. To establish a ratio of MPA to ascending aorta diameter using multi-detector CT imaging suggestive of pulmonary arterial hypertension in children. We hypothesize that a defined ratio of MPA to ascending aorta is identifiable on multi-detector CT and that higher ratios can be used to reliably diagnose the presence of pulmonary arterial hypertension in children. We calculated the multi-detector CT ratio of MPA to ascending aorta diameter in 44 children with documented pulmonary arterial hypertension by right heart catheterization and in 44 age- and gender-matched control children with no predisposing factors for pulmonary arterial hypertension. We compared this multi-detector-CT-determined ratio with the MPA pressure in the study group, as well as with the ratio of MPA to ascending aorta in the control group. A threshold ratio value was calculated to accurately identify children with pulmonary arterial hypertension. Children with documented primary pulmonary arterial hypertension have a significantly higher ratio of MPA to ascending aorta (1.46) than children without pulmonary arterial hypertension (1.11). A ratio of 1.3 carries a positive likelihood of 34 and a positive predictive value of 97% for the diagnosis of pulmonary arterial hypertension. The pulmonary arteries were larger in children with pulmonary arterial hypertension than in a control group of normal children. A CT-measured ratio of MPA to ascending aorta of 1.3 should raise the suspicion of pulmonary arterial hypertension in children. (orig.)

  14. Pulmonary arterial hypertension in children: diagnosis using ratio of main pulmonary artery to ascending aorta diameter as determined by multi-detector computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Caro-Dominguez, Pablo; Manson, David E. [University of Toronto, Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada); Compton, Gregory [University of Toronto, Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada); Epworth Hospital, Epworth Medical Imaging, Richmond, VIC (Australia); Humpl, Tilman [University of Toronto, Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON (Canada)

    2016-09-15

    The ratio of the transverse diameter of the main pulmonary artery (MPA) to ascending aorta as determined at multi-detector CT is a tool that can be used to assess the pulmonary arterial size in cases of pulmonary arterial hypertension in children. To establish a ratio of MPA to ascending aorta diameter using multi-detector CT imaging suggestive of pulmonary arterial hypertension in children. We hypothesize that a defined ratio of MPA to ascending aorta is identifiable on multi-detector CT and that higher ratios can be used to reliably diagnose the presence of pulmonary arterial hypertension in children. We calculated the multi-detector CT ratio of MPA to ascending aorta diameter in 44 children with documented pulmonary arterial hypertension by right heart catheterization and in 44 age- and gender-matched control children with no predisposing factors for pulmonary arterial hypertension. We compared this multi-detector-CT-determined ratio with the MPA pressure in the study group, as well as with the ratio of MPA to ascending aorta in the control group. A threshold ratio value was calculated to accurately identify children with pulmonary arterial hypertension. Children with documented primary pulmonary arterial hypertension have a significantly higher ratio of MPA to ascending aorta (1.46) than children without pulmonary arterial hypertension (1.11). A ratio of 1.3 carries a positive likelihood of 34 and a positive predictive value of 97% for the diagnosis of pulmonary arterial hypertension. The pulmonary arteries were larger in children with pulmonary arterial hypertension than in a control group of normal children. A CT-measured ratio of MPA to ascending aorta of 1.3 should raise the suspicion of pulmonary arterial hypertension in children. (orig.)

  15. Pulmonary arterial hypertension secondary to chronic thromboemboli

    International Nuclear Information System (INIS)

    Crysikopoulos, H.; Forrest, J.V.; Olson, L.K.; Roberts, A.C.

    1989-01-01

    The authors report their experience in 150 patients with pulmonary arterial hypertension secondary to chronic pulmonary embolism (CPE). Plain film findings are variable, commonly nonspecific. Occasionally nonuniformity of pulmonary perfusion or truncation of central pulmonary arteries suggest CPE. Multiple, large, unmatched perfusion defects are the most common scintigraphic observations. CT can exclude conditions mimicking CPE, particularly fibrosing mediastinitis. Pulmonary arteriography depicts the location and distribution of emboli. CPE is becoming an increasingly important entity to recognize because of available surgical treatment. Thromboendarterectomy may improve functional status and survival. The diagnosis should be considered in any patient with pulmonary arterial hypertension

  16. Role of secretory phospholipase A(2) in rhythmic contraction of pulmonary arteries of rats with monocrotaline-induced pulmonary arterial hypertension.

    Science.gov (United States)

    Tanabe, Yoshiyuki; Saito-Tanji, Maki; Morikawa, Yuki; Kamataki, Akihisa; Sawai, Takashi; Nakayama, Koichi

    2012-01-01

    Excessive stretching of the vascular wall in accordance with pulmonary arterial hypertension (PAH) induces a variety of pathogenic cellular events in the pulmonary arteries. We previously reported that indoxam, a selective inhibitor for secretory phospholipase A(2) (sPLA(2)), blocked the stretch-induced contraction of rabbit pulmonary arteries by inhibition of untransformed prostaglandin H(2) (PGH(2)) production. The present study was undertaken to investigate involvement of sPLA(2) and untransformed PGH(2) in the enhanced contractility of pulmonary arteries of experimental PAH in rats. Among all the known isoforms of sPLA(2), sPLA(2)-X transcript was most significantly augmented in the pulmonary arteries of rats with monocrotaline-induced pulmonary hypertension (MCT-PHR). The pulmonary arteries of MCT-PHR frequently showed two types of spontaneous contraction in response to stretch; 27% showed rhythmic contraction, which was sensitive to indoxam and SC-560 (selective COX-1 inhibitor), but less sensitive to NS-398 (selective COX-2 inhibitor); and 47% showed sustained incremental tension (tonic contraction), which was insensitive to indoxam and SC-560, but sensitive to NS-398 and was attenuated to 45% of the control. Only the rhythmically contracting pulmonary arteries of MCT-PHR produced a substantial amount of untransformed PGH(2), which was abolished by indoxam. These results suggest that sPLA(2)-mediated PGH(2) synthesis plays an important role in the rhythmic contraction of pulmonary arteries of MCT-PHR.

  17. Pedunculated Pulmonary Artery Sarcoma Suggested by Transthoracic Echocardiography.

    Science.gov (United States)

    Wang, Xiaobing; Ren, Weidong; Yang, Jun

    2016-04-01

    Pulmonary artery sarcoma (PAS) is an extremely rare malignancy. It is usually found after it grows large enough to occupy almost the entire lumen of the pulmonary artery and causes serious clinical symptoms. Thus, it is usually difficult to distinguish PAS from pulmonary thromboembolism based on imaging examinations. Few case reports had shown the attachment of PAS to pulmonary artery, a key characteristic for diagnosis, and differential diagnosis of PAS. In this case, we found a PAS, which did not cause local obstruction and some tumor emboli, which obstructed the branches of the pulmonary arteries and caused pulmonary hypertension and clinical symptoms. Transthoracic echocardiography (TTE) revealed a part of the tumor attached to the intima of the main pulmonary artery with a peduncle and had obvious mobility, which was suggestive of PAS and differentiated it from the pulmonary thromboembolism. To our knowledge, this is the first case report of a pedunculated PAS suggested by TTE. Combined with pulmonary artery computed tomography angiography, the diagnosis of PAS is strongly suggested before the operation. This case indicates that TTE could reveal the attachment and mobility of PAS in the main pulmonary and may provide useful information for the diagnosis and differential diagnosis of PAS, especially a pedunculated PAS. © 2015, Wiley Periodicals, Inc.

  18. Transcatheter Treatment of “Pulmonary Artery Hypertension” due to Patent Ductus Arteriosus and Pulmonary Artery Stenosis

    OpenAIRE

    Gaio, Gianpiero; Santoro, Giuseppe; D'Alto, Michele; Palladino, Maria Teresa; Russo, Maria Giovanna; Caianiello, Giuseppe; Calabrò, Raffaele

    2006-01-01

    The association between large, left-sided patent ductus arteriosus and severe, peripheral, right pulmonary artery stenosis with no other cardiac malformation is an unreported condition that might be misdiagnosed as pulmonary hypertension due to long-standing ductal shunt. A 57-year-old man with supposed hypertensive patent ductus arteriosus underwent confirmatory cardiac catheterization. At angiography, a severe pre-hilar right pulmonary artery stenosis (peak pressure gradient, 65 mmHg) was f...

  19. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong [Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang (Korea, Republic of); Kim, Seon Jeong [Dept. of Radiology, Myongji Hospital, Goyang (Korea, Republic of)

    2017-05-15

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia.

  20. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

    International Nuclear Information System (INIS)

    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong; Kim, Seon Jeong

    2017-01-01

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia

  1. Abnormal pulmonary artery stiffness in pulmonary arterial hypertension: in vivo study with intravascular ultrasound.

    Directory of Open Access Journals (Sweden)

    Edmund M T Lau

    Full Text Available BACKGROUND: There is increasing recognition that pulmonary artery stiffness is an important determinant of right ventricular (RV afterload in pulmonary arterial hypertension (PAH. We used intravascular ultrasound (IVUS to evaluate the mechanical properties of the elastic pulmonary arteries (PA in subjects with PAH, and assessed the effects of PAH-specific therapy on indices of arterial stiffness. METHOD: Using IVUS and simultaneous right heart catheterisation, 20 pulmonary segments in 8 PAH subjects and 12 pulmonary segments in 8 controls were studied to determine their compliance, distensibility, elastic modulus and stiffness index β. PAH subjects underwent repeat IVUS examinations after 6-months of bosentan therapy. RESULTS: AT BASELINE, PAH SUBJECTS DEMONSTRATED GREATER STIFFNESS IN ALL MEASURED INDICES COMPARED TO CONTROLS: compliance (1.50±0.11×10(-2 mm(2/mmHg vs 4.49±0.43×10(-2 mm(2/mmHg, p<0.0001, distensibility (0.32±0.03%/mmHg vs 1.18±0.13%/mmHg, p<0.0001, elastic modulus (720±64 mmHg vs 198±19 mmHg, p<0.0001, and stiffness index β (15.0±1.4 vs 11.0±0.7, p = 0.046. Strong inverse exponential associations existed between mean pulmonary artery pressure and compliance (r(2 = 0.82, p<0.0001, and also between mean PAP and distensibility (r(2 = 0.79, p = 0.002. Bosentan therapy, for 6-months, was not associated with any significant changes in all indices of PA stiffness. CONCLUSION: Increased stiffness occurs in the proximal elastic PA in patients with PAH and contributes to the pathogenesis RV failure. Bosentan therapy may not be effective at improving PA stiffness.

  2. Pulmonary arterial capacitance in children with idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease: relation to pulmonary vascular resistance, exercise capacity, and survival.

    Science.gov (United States)

    Sajan, Imran; Manlhiot, Cedric; Reyes, Janette; McCrindle, Brian W; Humpl, Tilman; Friedberg, Mark K

    2011-09-01

    Pediatric pulmonary arterial hypertension (PAH), whether idiopathic PAH (iPAH) or PAH associated with congenital heart disease (aPAH), carries high morbidity and mortality. Low pulmonary arterial capacitance (PAC), defined as right ventricular stroke volume/pulmonary artery pulse pressure, is a risk factor for mortality in adults with PAH. However, the relation of PAC to pulmonary vascular resistance (PVR), exercise endurance, and survival is poorly defined in children. Catheterization and clinical data of children with PAH (mean pulmonary artery pressure >25 mm Hg) were reviewed. Children with pulmonary shunts, stents, collaterals, or pulmonary venous hypertension were excluded. Primary outcomes were 6-minute walk distance and freedom from death/lung transplant. Forty-seven patients were studied. Nineteen (43%) had iPAH, and 28 (57%) had aPAH (7.1 ± 6.2 vs 8.4 ± 5.5 years, P = .45). Patients with iPAH had higher PVR indexed for body surface area (PVRi), lower indexed PAC (PACi), lower exercise tolerance, and lower freedom from death/lung transplant than patients with aPAH. Both higher PVRi (P 1.25 mL/mm Hg per square meter and a PVRi >13 Wood units × m(2) were associated with decreased freedom from death or lung transplant. The relationships between PVRi and PACi and survival were independent of each other and not confounded by etiologic group. Low PACi and high PVRi are independently associated with low 6-minute walk distance and survival in children with PAH. Therefore, both should be assessed for better prognostication and management in this high-risk population. Copyright © 2011 Mosby, Inc. All rights reserved.

  3. Experimental research of covered stent implanted in canine hepatic artery

    International Nuclear Information System (INIS)

    Zhou Bing; Liu Linxiang; Li Minghua; Wang Yongli; Cheng Yongde

    2007-01-01

    Objective: To evaluate the feasibility of success rate of implantation, post-procedure stenosis rate, apposition ability and endothelialization level, etc. for implantation with balloon-expandable covered stent in canine hepatic artery. Methods: 8 adult canines were implanted with balloon-expendable stents covered by expandable poly Teflon ester membrane (e-PTFEM). Follow-up DSA was performed immediately, 2, 4 and 12 wk after the procedure. The canines were sacrificed for histopathologic examination and statistical analysis with correlation of implantation manenvor and angiographic manifestations. Results: 8 cases were all implanted with the covered stents in proper hepatic artery/right hepatic artery successfully; showing good apposition ability and non-opacification of the separated branches. 2 cases showed intraluminal obvious stenosis( > 50%)of the stent at 2 weeks follow-up, so did 3 cases at 12 weeks follow-up, and the total stenosis rate was 37.5% and 5 cases manifested full endothelialization (3 different locations of the sample all manifested full endothelialization), 3 cased manifested partial endothelialization (at least 1 location of the sample didn't show full endothelialization), and the two terminal parts were easier to get endothelialization than the central part. Before and after the stent implantation, hepatic function of all cases didn't demonstrate any obvious changes. Conclusions: Balloon-expandable covered stent can be implanted in canine hepatic artery. successfully, with good apposition ability, full endothelialization, and no influence on hepatic function. (authors)

  4. MRI of surgically created pulmonary artery narrowing in the dog

    Energy Technology Data Exchange (ETDEWEB)

    Hernandez, R.J.; Rocchini, A.P.; Bove, E.L.; Chenevert, T.L.; Gubin, B. (Michigan Univ., Ann Arbor (USA). Dept. of Radiology)

    1989-11-01

    Narrowing of the pulmonary arteries was created surgically in twelve dogs. In six of the dogs the narrowing was central (main pulmonary artery), and in the remaining six the narrowing was located peripherally at the hilar level of the right pulmonary artery beyond the pericardial reflection. MRI and angiography were performed in all dogs. MRI clearly delineated the site of the pulmonary band and the caliber of the pulmonary artery at the site of the band in all dogs (N=6). MRI was not able to visualize any of the stenosis of the right pulmonary arteries at the hila, beyond the pericardial reflection. In addition, optimal imaging planes to depict each segment of the central pulmonary arteries were determined. The capability to image in oblique planes is essential in evaluating the morphology of the central pulmonary arteries. (orig.).

  5. MRI of surgically created pulmonary artery narrowing in the dog

    International Nuclear Information System (INIS)

    Hernandez, R.J.; Rocchini, A.P.; Bove, E.L.; Chenevert, T.L.; Gubin, B.

    1989-01-01

    Narrowing of the pulmonary arteries was created surgically in twelve dogs. In six of the dogs the narrowing was central (main pulmonary artery), and in the remaining six the narrowing was located peripherally at the hilar level of the right pulmonary artery beyond the pericardial reflection. MRI and angiography were performed in all dogs. MRI clearly delineated the site of the pulmonary band and the caliber of the pulmonary artery at the site of the band in all dogs (N=6). MRI was not able to visualize any of the stenosis of the right pulmonary arteries at the hila, beyond the pericardial reflection. In addition, optimal imaging planes to depict each segment of the central pulmonary arteries were determined. The capability to image in oblique planes is essential in evaluating the morphology of the central pulmonary arteries. (orig.)

  6. [The effect of calcitonin gene-related peptide on collagen accumulation in pulmonary arteries of rats with hypoxic pulmonary arterial hypertension].

    Science.gov (United States)

    Li, Xian-Wei; Du, Jie; Li, Yuan-Jian

    2013-03-01

    To observe the effect of calcitonin gene-related peptide (CGRP) on pulmonary vascular collagen accumulation in hypoxia rats in order to study the effect of CGRP on hypoxic pulmonary vascular structural remodeling and its possible mechanism. Rats were acclimated for 1 week, and then were randomly divided into three groups: normoxia group, hypoxia group, and hypoxia plus capsaicin group. Pulmonary arterial hypertension was induced by hypoxia in rats. Hypoxia plus capsaicin group, rats were given capsaicin (50 mg/(kg x d), s.c) 4 days before hypoxia to deplete endogenous CGRP. Hypoxia (3% O2) stimulated proliferation of pulmonary arterial smooth muscle cells (PASMCs) and proliferation was measured by BrdU marking. The expression levels of CGRP, phosphorylated ERK1/2 (p-ERK1/ 2), collagen I and collagen III were detected by real-time PCR or Western blot. Right ventricle systolic pressure (RVSP) and mean pulmonary arterial pressure (mPAP) of pulmonary arterial hypertension (PAH) rats induced by hypoxia were higher than those of normoxia rats. By HE and Masson staining, it was demonstrated that hypoxia also significantly induced hypertrophy of pulmonary arteries and increased level of collagen accumulation. Hypoxia dramatically decreased the CGRP level and increased the expression of p-ERK1/2, collagen I, collagen III in pulmonary arteries. All these effects of hypoxia were further aggravated by pre-treatment of rats with capsaicin. CGRP concentration-dependently inhibited hypoxia-induced proliferation of PASMCs, markedly decreased the expression of p-ERK1/2, collagen I and collagen III. All these effects of CGRP were abolished in the presence of CGRP8-37. These results suggest that CGRP might inhibit hypoxia-induced PAH and pulmonary vascular remodeling, through inhibiting phosphorylation of ERK1/2 and alleviating the collagen accumulation of pulmonary arteries.

  7. Intimal sarcoma of the pulmonary artery presenting as pulmonary embolism

    OpenAIRE

    Plata, María Camila; Rey, Diana Lucía; Villaquirán, Claudio; Rosselli, Diego

    2017-01-01

    SUMMARY Pulmonary artery sarcomas are extremely rare; due to their insidious growth, diagnosis occurs late and prognosis is poor. We present the case of a 33-year-old woman with a history of dyspnea, chest pain and syncope. An obstructing mass on the right ventricle, main pulmonary artery and right branch were interpreted as a possible pulmonary embolism. RESUMEN Los sarcomas de la arteria pulmonar son extremadamente raros; debido a su crecimiento lento y silencioso, el diagnóstico suele s...

  8. Acute and chronic dissection of pulmonary artery: new challenges in pulmonary arterial hypertension?

    Science.gov (United States)

    Florczyk, Michał; Wieteska, Maria; Kurzyna, Marcin; Gościniak, Piotr; Pepke-Żaba, Joanna; Biederman, Andrzej; Torbicki, Adam

    2018-01-01

    Right ventricular failure is a leading cause of mortality in patients with pulmonary arterial hypertension (PAH). However, up to 25% of such patients die unexpectedly, without warning signs of hemodynamical decompensation. We previously documented that pulmonary artery (PA) dilatation significantly increases the risk of those deaths. Some of them may be due to dissection of PA resulting in cardiac tamponade. However, direct confirmation of this mechanism is difficult as most of such deaths occur outside hospitals. We present 4 patients with severe PAH and PA dilatation in whom PA dissection has been confirmed. Three patients had IPAH, one had PAH associated with congenital heart disease. All patients had mean pulmonary artery pressure (PAP) > 50 mmHg at diagnosis and dissection occurred late in the course of apparently well controlled disease (6 to 14 years). Several clinical elements were common to our patients - high systolic PAP, long lasting PH, progressive dilatation of PA to more than 50 mm with chest pain prior to dissection. However, clinical course followed three different patterns: sudden death due to cardiac tamponade, hemopericarditis caused by blood leaking from dissected aneurysm with imminent but not immediate cardiac tamponade, or chronic asymptomatic PA dissection. Indeed, two of our patients are alive and on lung transplantation waiting list for more than 2 years now. Further research is needed to suggest optimal management strategies for patients with stable PAH but significantly dilated proximal pulmonary arteries or confirmed PA dissection depending on the clinical presentation and expected outcome.

  9. Lung irradiation induces pulmonary vascular remodelling resembling pulmonary arterial hypertension

    NARCIS (Netherlands)

    Ghobadi, G.; Bartelds, B.; van der Veen, S. J.; Dickinson, M. G.; Brandenburg, S.; Berger, R. M. F.; Langendijk, J. A.; Coppes, R. P.; van Luijk, P.

    Background Pulmonary arterial hypertension (PAH) is a commonly fatal pulmonary vascular disease that is often diagnosed late and is characterised by a progressive rise in pulmonary vascular resistance resulting from typical vascular remodelling. Recent data suggest that vascular damage plays an

  10. Pulmonary scanning: quantitative evaluation of pulmonary arterial flow

    Energy Technology Data Exchange (ETDEWEB)

    Papaleo Netto, M; Fujioka, T [Sao Paulo Univ. (Brazil). Faculdade de Medicina; Dias Neto, A; Carvalho, N [Sao Paulo Univ. (Brazil). Centro de Medicina Nuclear

    1974-01-01

    From ten normal subjects of both sexes, the quantitative regional blood flow of the pulmonary artery was evaluated using scanning with macroaggregated radio-iodinated (/sup 131/I) albumin. It was possible to conclude that: the digital recording of data (counts/cm/sup 2/), from any particular area of interest, is the best method for this evaluation; the lung, even being a thick organ, can be well studied by quantitative scanning, since its structure doesn't hinder the passage of radiations because it is covered only by the thoracic wall; scanning can be used to evaluate regional perfusion of the pulmonary artery, based on the proportionality between density of aggregates and blood flux in the different areas; the concentration of macroaggregates on the lung's superior section never reaches more than 40% of the radioactivity of the whole lung; there is no significant difference between left and right lungs, concerning the relationship between radioactivity on the superior section and the total area and quantitative analysis of pulmonary artery flow by means of scanning is a possible, reliable, and safe technique, without distress for the patient.

  11. Pulmonary scanning: quantitative evaluation of pulmonary arterial flow

    International Nuclear Information System (INIS)

    Papaleo Netto, M.; Fujioka, T.; Dias Neto, A.; Carvalho, N.

    1974-01-01

    From ten normal subjects of both sexes, the quantitative regional blood flow of the pulmonary artery was evaluated using scanning with macroaggregated radio-iodinated ( 131 I) albumin. It was possible to conclude that: the digital recording of data (counts/cm 2 ), from any particular area of interest, is the best method for this evaluation; the lung, even being a thick organ, can be well studied by quantitative scanning, since its structure doesn't hinder the passage of radiations because it is covered only by the thoracic wall; scanning can be used to evaluate regional perfusion of the pulmonary artery, based on the proportionality between density of aggregates and blood flux in the different areas; the concentration of macroaggregates on the lung's superior section never reaches more than 40% of the radioactivity of the whole lung; there is no significant difference between left and right lungs, concerning the relationship between radioactivity on the superior section and the total area and quantitative analysis of pulmonary artery flow by means of scanning is a possible, reliable and safe technique, without distress for the patient [pt

  12. Measurement of pulmonary vascular resistance of Fontan candidates with pulmonary arterial distortion by means of pulmonary perfusion imaging

    International Nuclear Information System (INIS)

    Park, In-Sam; Mizukami, Ayumi; Tomimatsu, Hirofumi; Kondou, Chisato; Nakanishi, Toshio; Nakazawa, Makoto; Momma, Kazuo

    1998-01-01

    We measured the distribution of blood flow to the right (R) and left lung (L) by means of pulmonary perfusion imaging and calculated pulmonary vascular resistance (Rp) in 13 patients, whose right and left pulmonary artery pressures were different by 2 to 9 mmHg due to pulmonary arterial distortion (5 interruption, 8 stenosis). The right lung/left lung blood flow ratio was determined and from the ratio and the total pulmonary blood flow, which was determined using the Fick's principle, the absolute values of right and left pulmonary blood flow were calculated. Using the right and left pulmonary blood flow and the right and left pulmonary arterial pressures, right and left pulmonary vascular resistance were calculated, separately. Vascular resistance of the whole lung (Rp) was then calculated using the following equation. 1/(Rp of total lung)=1/(Rp of right lung)+1/(Rp of left lung). Rp calculated from this equation was 1.8+/-0.8 U·m 2 and all values were less than 3 U·m 2 (range 0.3-2.8). Rp estimated from the conventional method using the total pulmonary blood flow and pulmonary arterial pressures, without using the right/left blood flow ratio, ranging from 0.4 to 3.8 U·m 2 and 5 of 13 patients showed Rp>3 U·m 2 . All patients underwent Fontan operation successfully. These data indicated that this method is useful to estimate Rp and to determine the indication of Fontan operation in patients with pulmonary arterial distortions. (author)

  13. Scintigraphic study of blood perfusion of the pulmonary artery in pulmonary tuberculosis

    International Nuclear Information System (INIS)

    Certain, D.A.; Brolio, R.; Salomon, G.C.; Carvalho, N.; Barbosa, Z.L.M.

    1972-01-01

    Blood perfusion in the pulmonary artery is studied by pulmonary scintigraphy with macroaggregated albumin 131 I, in 74 cases of pulmonary tuberculosis. Results shown by scintigraphy are compared to those observed in roentgenography and also correlated with the extension and degree of the lesions, and with the presence of associated tuberculosis, considered capable of changing the scintigraphic picture. The reduction of blood flow in the pulmonary artery are observed in cases of infiltrative, fibrotic of caseous lesions, as well as in cases of minor lesions [pt

  14. Scintigraphic study of blood perfusion of the pulmonary artery in pulmonary tuberculosis

    Energy Technology Data Exchange (ETDEWEB)

    Certain, D A; Brolio, R; Salomon, G C [Sao Paulo Univ. (Brazil). Faculdade de Saude Publica; Carvalho, N [Sao Paulo Univ. (Brazil). Centro de Medicina Nuclear; Barbosa, Z L.M. [Associacao dos Sanatorios Populares de Campos do Jordao, Brazil

    1972-10-01

    Blood perfusion in the pulmonary artery is studied by pulmonary scintigraphy with macroaggregated albumin /sup 131/I, in 74 cases of pulmonary tuberculosis. Results shown by scintigraphy are compared to those observed in roentgenography and also correlated with the extension and degree of the lesions, and with the presence of associated tuberculosis, considered capable of changing the scintigraphic picture. The reduction of blood flow in the pulmonary artery are observed in cases of infiltrative, fibrotic of caseous lesions, as well as in cases of minor lesions.

  15. Initial isolated Takayasu's arteritis of the right pulmonary artery: MR appearance

    International Nuclear Information System (INIS)

    Ferretti, G.; Defaye, P.; Thony, F.; Ranchoup, Y.; Coulomb, M.

    1996-01-01

    Takayasu's arteritis involves the pulmonary artery tree in more than 50 % of the cases. Initial isolated involvement of the pulmonary artery by Takayasu's arteritis, however, is very rare. We report the case of a 34-year-old white woman who presented a clinical and radiographic pattern that mimicked an acute pulmonary embolism with pulmonary infarction. Pulmonary angiography showed stenosis lesions and occlusion of the right pulmonary artery tree. Magnetic resonance imaging demonstrated thickening of the pulmonary artery wall leading to the correct diagnosis. (orig.). With 3 figs

  16. Transcatheter Embolization of Pulmonary Artery False Aneurysm Associated with Primary Pulmonary Hypertension

    International Nuclear Information System (INIS)

    Hiraki, T.; Kanazawa, S.; Mimura, H.; Yasui, K.; Okumura, Y.; Dendo, S.; Yoshimura, K.; Takahara, M.; Hiraki, Y.

    2004-01-01

    A 29-year-old woman with primary pulmonary hypertension presented with recurrent hemoptysis. Contrast-enhanced CT of the chest demonstrated the enhanced mass surrounded by consolidation related to parenchymal hemorrhage. Pulmonary angiography suggested that the mass was a pulmonary artery false aneurysm. After a microcatheter was superselectively inserted into the parent artery of the falseaneurysm, the false aneurysm was successfully treated by transcatheterembolization with coils. Her hemoptysis has never recurred

  17. Cryoplasty for the treatment of iliac artery stenosis in canine model

    International Nuclear Information System (INIS)

    Wu Zhengzhong; Yang Weizhu; Jiang Na; Zheng Qubin; Huang Keyao; Huang Ning; Shen Quan

    2013-01-01

    Objective To evaluate the treatment effect of balloon angioplasty and cryoplasty on iliac artery stenosis in canine model. Methods: Canine models of iliac artery stenosis were established with surgical ligation and transfixion. Models were randomly divided into two groups: iliac artery stenosis treated by cryoplasty (n = 8) and by balloon angioplasty (n = 8). The degree of iliac artery stenosis of two model groups was assessed by digital subtraction angiography (DSA) immediately and two weeks after the angioplasties. Then all the dogs were executed for pathological observation of the target vessels. Results: Sixteen canine models of iliac artery stenosis were established by surgical ligation and transfixion method with 100% success rate. DSA showed there was (45 ± 12)% of residual artery stenosis in the cryoplasty group immediately after surgery, while it was (39-12)% in the balloon angioplasty group, and there was no significant difference between them (t = 3.183, P > 0.05). The artery stenosis of cryoplasty group was (48 ± 17)% after two weeks and not significantly different from that after surgery immediately (t = -1.271, P > 0.05). The artery stenosis of balloon angioplasty group was (67 ± 13)% after two weeks, and it was significantly higher compared with at after surgery immediately (t = -6.666, P < 0.01). The degree of vascular stenosis in balloon angioplasty group was severer than that in cryoplasty group two weeks after angiography (P = 0.041). The pathological examination showed artery intimal hyperplasia in cryoplasty group was milder than that in balloon angioplasty group and neointimal content of collagen in cryoplasty group was less than that in balloon angioplasty group. Conclusions: The animal models of iliac artery stenosis were established successfully with surgical ligation and transfixion by damaging the intima and media of arterial wall. Compared with balloon angioplasty, the cryoplasty was able to inhibit proliferation of intimal and reduce

  18. Mycophenolate mofetil attenuates pulmonary arterial hypertension in rats

    International Nuclear Information System (INIS)

    Suzuki, Chihiro; Takahashi, Masafumi; Morimoto, Hajime; Izawa, Atsushi; Ise, Hirohiko; Hongo, Minoru; Hoshikawa, Yasushi; Ito, Takayuki; Miyashita, Hiroshi; Kobayashi, Eiji; Shimada, Kazuyuki; Ikeda, Uichi

    2006-01-01

    Pulmonary arterial hypertension (PAH) is characterized by abnormal proliferation of smooth muscle cells (SMCs), leading to occlusion of pulmonary arterioles, right ventricular (RV) hypertrophy, and death. We investigated whether mycophenolate mofetil (MMF), a potent immunosuppresssant, prevents the development of monocrotaline (MCT)-induced PAH in rats. MMF effectively decreased RV systolic pressure and RV hypertrophy, and reduced the medial thickness of pulmonary arteries. MMF significantly inhibited the number of proliferating cell nuclear antigen (PCNA)-positive cells, infiltration of macrophages, and expression of P-selectin and interleukin-6 on the endothelium of pulmonary arteries. The infiltration of T cells and mast cells was not affected by MMF. In vitro experiments revealed that mycophenolic acid (MPA), an active metabolite of MMF, dose-dependently inhibited proliferation of human pulmonary arterial SMCs. MMF attenuated the development of PAH through its anti-inflammatory and anti-proliferative properties. These findings provide new insight into the potential role of immunosuppressants in the treatment of PAH

  19. Anomalous right coronary artery arising from the pulmonary artery and constrictive pericarditis: an unusual association

    Science.gov (United States)

    Silvestre, Odilson Marcos; Adam, Eduardo Leal; de Melo, Dirceu Thiago Pessoa; Dias, Ricardo Ribeiro; Ramires, Felix J. A.; Mady, Charles

    2013-01-01

    ABSTRACT The association of anomalous right coronary artery originating from the pulmonary artery and constrictive pericarditis has never been showed in the literature. We present the first case of this unusual association in a patient with right heart failure. After diagnosis, the patient was referred to surgery and underwent phrenic-to-phrenic pericardiectomy; graft implant of right internal thoracic artery to right coronary artery; and ligation of the anomalous origin of the right coronary artery from the pulmonary artery. Such procedures solved the potential risk of sudden death related to anomalous right coronary artery originating from the pulmonary artery and alleviated the symptoms of heart failure caused by constrictive pericarditis. PMID:24136766

  20. Dasatinib-induced pulmonary arterial hypertension - A rare late complication.

    Science.gov (United States)

    Ibrahim, Uroosa; Saqib, Amina; Dhar, Vidhya; Odaimi, Marcel

    2018-01-01

    Dasatinib is a dual Src/Abl tyrosine kinase inhibitor approved for frontline and second line treatment of chronic phase chronic myelogenous leukemia. Pulmonary arterial hypertension is defined by an increase in mean pulmonary arterial pressure >25 mmHg at rest. Dasatinib-induced pulmonary hypertension has been reported in less than 1% of patients on chronic dasatinib treatment for chronic myelogenous leukemia. The pulmonary arterial hypertension from dasatinib may be categorized as either group 1 (drug-induced) or group 5 based on various mechanisms that may be involved including the pathogenesis of the disease process of chronic myelogenous leukemia. There have been reports of dasatinib-induced pulmonary arterial hypertension being reversible. We report a case of pulmonary arterial hypertension in a 46-year-old female patient with chronic phase chronic myelogenous leukemia on dasatinib treatment for over 10 years. She had significant improvement in symptoms after discontinuation of dasatinib and initiation of vasodilators. Several clinical questions arise once patients experience significant adverse effects as discussed in our case.

  1. Anomalous Origin of Left Coronary Artery from Pulmonary Artery (ALCAPA)

    International Nuclear Information System (INIS)

    Younus, Z.; Ahmed, I.; Iftikhar, R.

    2013-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery also recognized as Bland White Garland syndrome is a very rare congenital condition. A two-months old baby boy presented with dyspnoea for two weeks and a pansystolic murmur on auscultation. The base line investigations showed cardiomegaly and bilateral basal haze on X-ray chest. ECG showed ST elevation in leads l and AVl and echocardiography showed situs solitus, levocardia, hypokinetic intraventricular septum, ejection fraction of 30%, mitral regurgitation of grade-I and an anomalous origin of the left coronary artery from pulmonary artery was diagnosed. Patient was in left heart failure. It was rectified surgically by creating a transpulmonary tunnel (Takeuchi repair). Postoperative course was uneventful and he was finally discharged in stable condition. (author)

  2. Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation.

    Science.gov (United States)

    Ebishima, Hironori; Kurosaki, Kenichi; Yoshimatsu, Jun; Shiraishi, Isao

    2017-08-01

    This study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes. The Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio - the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation. We included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, ptetralogy of Fallot.

  3. Fetal Diagnosis of Abnormal Origin of the Left Pulmonary Artery.

    Science.gov (United States)

    Tretter, Justin T; Tretter, Eric M; Rafii, Daniela Y; Anderson, Robert H; Bhatla, Puneet

    2016-08-01

    Isolated anomalies of the branch pulmonary arteries are rare, more often occurring in the setting of complex congenital heart disease. These isolated anomalies are often not identified in the prenatal period. We describe two cases of isolated anomalies of the left pulmonary artery which were identified on fetal echocardiography and confirmed postnatally, an anomalous left pulmonary artery arising from the base of the left-sided brachiocephalic artery in the setting of a right-sided aortic arch, and a left pulmonary artery sling. These two cases support our current understanding of normal and abnormal development of the extrapericardial arterial vessels and highlight the importance of meticulous attention when sweeping from the three-vessel tracheal view. © 2016, Wiley Periodicals, Inc.

  4. Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

    International Nuclear Information System (INIS)

    Sondheimer, H.M.; Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

    1982-01-01

    To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease

  5. Effect of hypothermic pulmonary artery flushing on capillary filtration coefficient.

    Science.gov (United States)

    Andrade, R S; Wangensteen, O D; Jo, J K; Tsai, M Y; Bolman, R M

    2000-07-27

    We previously demonstrated that surfactant dilution and inhibition occur immediately after pulmonary artery flushing with hypothermic modified Euro-Collins solution. Consequently, we speculated that increased capillary permeability contributed to these surfactant changes. To test this hypothesis, we evaluated the effects of hypothermic pulmonary artery flushing on the pulmonary capillary filtration coefficient (Kfc), and additionally performed a biochemical analysis of surfactant. We used a murine isolated, perfused lung model to measure the pulmonary capillary filtration coefficient and hemodynamic parameters, to determine the wet to dry weight ratio, and to evaluate surfactant by biochemical analysis of lung lavage fluid. We defined three study groups. In group I (controls), we harvested lungs without hypothermic pulmonary artery flushing, and measured Kfc immediately. In group II (in situ flush), we harvested lungs after hypothermic pulmonary artery flushing with modified Euro-Collins solution, and then measured Kfc. Experiments in groups I and II were designed to evaluate persistent changes in Kfc after pulmonary artery flushing. In group III (ex vivo flush), we flushed lungs ex vivo to evaluate transient changes in Kfc during hypothermic pulmonary artery flushing. Groups I and II did not differ significantly in capillary filtration coefficient and hemodynamics. Group II showed significant alterations on biochemical surfactant analysis and a significant increase in wet-to-dry weight ratio, when compared with group I. In group III, we observed a significant transient increase in capillary filtration coefficient during pulmonary artery flushing. Hypothermic pulmonary artery flushing transiently increases the capillary filtration coefficient, leads to an increase in the wet to dry weight ratio, and induces biochemical surfactant changes. These findings could be explained by the effects of hypothermic modified Euro-Collins solution on pulmonary capillary

  6. Spontaneously Developed Pulmonary Arterial Intramural Hematoma That Mimicked Thromboembolism

    International Nuclear Information System (INIS)

    Kang, Eun Ju; Lee, Ki Nam; Kim, In; Chane, Jong Min; Kim, Gun Jik; Yang, Dong Heon; Lee, Jong Min

    2012-01-01

    A 65-year-old woman visited our hospital with a complaint of acute onset dyspnea and radiological manifestations of pulmonary thromboembolism. The patient underwent an exploratory surgery to find a whitish-blue colored mass occupying almost the whole lumen of the main pulmonary arteries. Based on the pathological and radiological findings, the patient was diagnosed to have a pulmonary arterial intramural hematoma. Intramural hematomas are usually observed in the walls of the aorta, and we believe that an isolated intramural hematoma in the pulmonary artery has not been described previously.

  7. Denervation of pulmonary artery during mitral valve surgery in patients with high pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    А. В. Богачев-Прокофьев

    2016-01-01

    Full Text Available Objective. Pulmonary hypertension impairs the mitral valve and often leads to more severe symptoms of heart failure, low exercise tolerance and thus higher rates of morbidity and mortality. The purpose of this study is to evaluate the safety and efficacy of simultaneous radiofrequency ablation of ganglionated plexi of the pulmonary artery in patients with high pulmonary hypertension during mitral valve surgery.Methods. The inclusion criteria were based on patients’ echocardiography/right heart catheterization data. The main criteria were mean pulmonary artery pressure ≥40 mm Hg at rest and a positive reactive test with nitric oxide inhalation. From January 2014 to May 2015, 14 patients underwent radiofrequency denervation of the pulmonary artery in addition to planned mitral valve surgery. Mean patient age was 53.4±7.8 years, with 57.1% of patients being females.Results. Mean cardiopulmonary bypass time was 116±12 minutes, mean cross-clamp time was 95±13 minutes, and mean ablation time amounted to 9.5±3.1 minutes. Pulmonary artery pressure decreased significantly from a mean of 56.5±9.8 mmHg to 32.0±7.3 mmHg immediately after the operation (p<0.001, and to 28.4±5.2 mmHg and 29.7±4.4 mmHg on the first and third days at ICU respectively. Mean ICU stay was 3.1±1.2 days. There were neither early deaths nor specific complications.Conclusions. Simultaneous radiofrequency ablation of pulmonary artery ganglionated plexi when performing mitral valve surgery in patients with pulmonary hypertension is a safe and effective procedure. Further research and long-term follow-up would help to determine whether a decrease in the mean pressure of the pulmonary artery can be interpreted as a clinical advantage.

  8. Acquired pulmonary artery stenosis in four dogs.

    Science.gov (United States)

    Scansen, Brian A; Schober, Karsten E; Bonagura, John D; Smeak, Daniel D

    2008-04-15

    4 dogs with acquired pulmonary artery stenosis (PAS) were examined for various clinical signs. One was a mixed-breed dog with congenital valvular PAS that subsequently developed peripheral PAS, one was a Golden Retriever with pulmonary valve fibrosarcoma, one was a Pembroke Welsh Corgi in which the left pulmonary artery had inadvertently been ligated during surgery for correction of patent ductus arteriosus, and one was a Boston Terrier with a heart-base mass compressing the pulmonary arteries. All 4 dogs were evaluated with 2-dimensional and Doppler echocardiography to characterize the nature and severity of the stenoses; other diagnostic tests were also performed. The mixed-breed dog with valvular and peripheral PAS was euthanized, surgical resection of the pulmonic valve mass was performed in the Golden Retriever, corrective surgery was performed on the Pembroke Welsh Corgi with left pulmonary artery ligation, and the Boston Terrier with the heart-base mass was managed medically. Acquired PAS in dogs may manifest as a clinically silent heart murmur, syncope, or right-sided heart failure. The diagnosis is made on the basis of imaging findings, particularly results of 2-dimensional and Doppler echocardiography. Treatment may include surgical, interventional, or medical modalities and is targeted at resolving the inciting cause.

  9. Constructing canine carotid artery stenosis model by endovascular technique

    International Nuclear Information System (INIS)

    Cheng Guangsen; Liu Yizhi

    2005-01-01

    Objective: To establish a carotid artery stenosis model by endovascular technique suitable for neuro-interventional therapy. Methods: Twelve dogs were anesthetized, the unilateral segments of the carotid arteries' tunica media and intima were damaged by a corneous guiding wire of home made. Twenty-four carotid artery stenosis models were thus created. DSA examination was performed on postprocedural weeks 2, 4, 8, 10 to estimate the changes of those stenotic carotid arteries. Results: Twenty-four carotid artery stenosis models were successfully created in twelve dogs. Conclusions: Canine carotid artery stenosis models can be created with the endovascular method having variation of pathologic characters and hemodynamic changes similar to human being. It is useful for further research involving the new technique and new material for interventional treatment. (authors)

  10. Pulmonary arterial hypertension

    Science.gov (United States)

    2013-01-01

    Pulmonary arterial hypertension (PAH) is a chronic and progressive disease leading to right heart failure and ultimately death if untreated. The first classification of PH was proposed in 1973. In 2008, the fourth World Symposium on PH held in Dana Point (California, USA) revised previous classifications. Currently, PH is devided into five subgroups. Group 1 includes patients suffering from idiopathic or familial PAH with or without germline mutations. Patients with a diagnosis of PAH should systematically been screened regarding to underlying mutations of BMPR2 gene (bone morphogenetic protein receptor type 2) or more rarely of ACVRL1 (activine receptor-like kinase type 1), ENG (endogline) or Smad8 genes. Pulmonary veno occusive disease and pulmonary capillary hemagiomatosis are individualized and designated as clinical group 1'. Group 2 'Pulmonary hypertension due to left heart diseases' is divided into three sub-groups: systolic dysfonction, diastolic dysfonction and valvular dysfonction. Group 3 'Pulmonary hypertension due to respiratory diseases' includes a heterogenous subgroup of respiratory diseases like PH due to pulmonary fibrosis, COPD, lung emphysema or interstitial lung disease for exemple. Group 4 includes chronic thromboembolic pulmonary hypertension without any distinction of proximal or distal forms. Group 5 regroup PH patients with unclear multifactorial mechanisms. Invasive hemodynamic assessment with right heart catheterization is requested to confirm the definite diagnosis of PH showing a resting mean pulmonary artery pressure (mPAP) of ≥ 25 mmHg and a normal pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg. The assessment of PCWP may allow the distinction between pre-capillary and post-capillary PH (PCWP > 15 mmHg). Echocardiography is an important tool in the management of patients with underlying suspicion of PH. The European Society of Cardiology and the European Respiratory Society (ESC-ERS) guidelines specify its role

  11. Histopathology of the great vessels in patients with pulmonary arterial hypertension in association with congenital heart disease: large pulmonary arteries matter too.

    Science.gov (United States)

    Prapa, Matina; McCarthy, Karen P; Dimopoulos, Konstantinos; Sheppard, Mary N; Krexi, Dimitra; Swan, Lorna; Wort, S John; Gatzoulis, Michael A; Ho, Siew Yen

    2013-10-03

    Pulmonary arterial hypertension (PAH) is considered primarily a disease of the distal pulmonary arteries whereas little is known on the effect of long-standing pulmonary hypertension on the larger proximal pulmonary arteries. This study aims to investigate the structural changes in the great arteries of adults who developed PAH in association with congenital heart disease (CHD), with severe cases termed Eisenmenger syndrome. We performed macroscopic and light microscopy analyses on the great arteries of 10 formalin-fixed human hearts from patients with PAH/CHD and compared them to age-matched healthy controls. A detailed histology grading score was used to assess the severity of medial wall abnormalities. Severe atherosclerotic lesions were found macroscopically in the elastic pulmonary arteries of 4 PAH/CHD specimens and organised thrombi in 3; none were present in the controls. Significant medial wall abnormalities were present in the pulmonary trunk (PT), including fibrosis (80%), and atypical elastic pattern (80%). Cyst-like formations were present in less than one third of patients and were severe in a single case leading to wall rupture. The cumulative PT histology grading score was significantly higher in PAH/CHD cases compared to controls (parteries. These abnormalities are likely to affect haemodynamics and contribute to morbidity and mortality in this cohort. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  12. Pulmonary artery perfusion versus no pulmonary perfusion during cardiopulmonary bypass in patients with COPD

    DEFF Research Database (Denmark)

    Buggeskov, Katrine B; Sundskard, Martin M; Jonassen, Thomas

    2016-01-01

    INTRODUCTION: Absence of pulmonary perfusion during cardiopulmonary bypass (CPB) may be associated with reduced postoperative oxygenation. Effects of active pulmonary artery perfusion were explored in patients with chronic obstructive pulmonary disease (COPD) undergoing cardiac surgery. METHODS: 90...... perfusion with normothermic oxygenated blood during cardiopulmonary bypass appears to improve postoperative oxygenation in patients with COPD undergoing cardiac surgery. Pulmonary artery perfusion with hypothermic HTK solution does not seem to improve postoperative oxygenation. TRIAL REGISTRATION NUMBER...

  13. Pulmonary artery hypertension in chronic obstructive lung disease

    International Nuclear Information System (INIS)

    Dinkel, E.; Mundinger, A.; Reinbold, W.D.; Wuertemberger, G.

    1989-01-01

    Standard biplane chest X-rays were tested for the validity of morphometric criteria in the diagnosis of pulmonary artery hypertension. Twenty-seven patients suffering from chronic obstructive lung disease were examined and compared with a control group without cardiopulmonary disease. The diameter of the right and left pulmonary artery, pulmonary conus and the hilar-to-thoracic ratio were significantly increased in patients with chronic obstructive lung disease (p [de

  14. A Case of Pulmonary Hypertension Due to Fistulas Between Multiple Systemic Arteries and the Right Pulmonary Artery in an Adult Discovered for Occulted Dyspnoea.

    Science.gov (United States)

    Li, Ji-Feng; Zhai, Zhen-Guo; Kuang, Tu-Guang; Liu, Min; Ma, Zhan-Hong; Li, Yi-Dan; Yang, Yuan-Hua

    2017-08-01

    Pulmonary hypertension (PH) can be caused by a fistula between the systemic and pulmonary arteries. Here, we report a case of PH due to multiple fistulas between systemic arteries and the right pulmonary artery where the ventilation/perfusion scan showed no perfusion in the right lung. A 32-year-old male patient was hospitalised for community-acquired pneumonia. After treatment with antibiotics, the pneumonia was alleviated but dyspnoea persisted. Pulmonary hypertension was diagnosed using right heart catheterisation, which detected the mean pulmonary artery pressure as 37mmHg. The anomalies were confirmed by contrast-enhanced CT scan (CT pulmonary angiography), systemic arterial angiography and pulmonary angiography. Following embolisation of the largest fistula, the haemodynamics and oxygen dynamics did not improve, and even worsened to some extent. After supportive therapy including diuretics and oxygen, the patient's dyspnoea, WHO function class and right heart function by transthoracic echocardiography all improved during follow-up. Pulmonary hypertension can be present even when the right lung perfusion is lost. Closure of fistulas by embolisation, when those fistulas act as the proliferating vessels, may be harmful. Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  15. Congenital anomalies of the pulmonary arteries: spectrum of findings on computed tomography.

    Science.gov (United States)

    Bueno, J; Flors, L; Mejía, M

    Congenital anomalies of the pulmonary arteries are uncommon. They can occur in isolation or in association with congenital heart defects. Isolated congenital anomalies remain undiscovered until they are reported as incidental findings on imaging tests, usually not until adolescence. We review the embryological development and normal anatomy of the pulmonary arteries as well as the spectrum of computed tomography findings for various congenital anomalies: unilateral interruption of the pulmonary artery, anomalous origin of the left pulmonary artery (pulmonary artery sling), idiopathic aneurysm of the pulmonary artery, and other anomalies associated with congenital heart defects. Congenital anomalies of the pulmonary arteries represent a diagnostic challenge for clinicians and radiologists. Computed tomography is useful for their diagnosis, and general radiologists need to be familiar with their imaging appearance because they are often discovered incidentally. Copyright © 2016 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. The cancer theory of pulmonary arterial hypertension

    Science.gov (United States)

    Boucherat, Olivier; Vitry, Geraldine; Trinh, Isabelle; Paulin, Roxane; Provencher, Steeve; Bonnet, Sebastien

    2017-01-01

    Pulmonary arterial hypertension (PAH) remains a mysterious killer that, like cancer, is characterized by tremendous complexity. PAH development occurs under sustained and persistent environmental stress, such as inflammation, shear stress, pseudo-hypoxia, and more. After inducing an initial death of the endothelial cells, these environmental stresses contribute with time to the development of hyper-proliferative and apoptotic resistant clone of cells including pulmonary artery smooth muscle cells, fibroblasts, and even pulmonary artery endothelial cells allowing vascular remodeling and PAH development. Molecularly, these cells exhibit many features common to cancer cells offering the opportunity to exploit therapeutic strategies used in cancer to treat PAH. In this review, we outline the signaling pathways and mechanisms described in cancer that drive PAH cells’ survival and proliferation and discuss the therapeutic potential of antineoplastic drugs in PAH. PMID:28597757

  17. Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

    Directory of Open Access Journals (Sweden)

    Blesneac Cristina

    2016-06-01

    Full Text Available Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH, that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.

  18. 53. Bilateral ductal stenting for nonconfluent pulmonary arteries in a newborn

    Directory of Open Access Journals (Sweden)

    K. Al Dhahri

    2016-07-01

    Full Text Available Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Limited data available for bilateral PDA stenting. Bilateral PDA stenting in nonconfluent pulmonary arteries is challenging procedure but can be considered as an option in the management of complex conditions like this. 12 days old Preterm (36 weeks gestation male baby with birth weight of 2.6 kg developed respiratory distress with severe cyanosis and desaturation upto 50%. Baby was intubated and started on Prostaglandin 0.05 mic/kg/mt. His saturation improved to 80%. Echocardiogram showed complex cyanotic heart disease, Situs ambiguous, dextrocardia, complete unbalanced AV septal defect, pulmonary atresia , nonconfluent small branch pulmonary arteries supplied by the bilateral patent ductus arteriosus (PDA from right aortic arch and all four pulmonary veins form a confluence and drain into superior vena cava(SVC through vertical vein with no obstruction. Baby was taken up for PDA stenting. descending aortogram showed right aortic arch with vertical tortuous duct to right pulmonary artery (RPA and another short duct with acute angle from left subclavian artery to left pulmonary artery (LPA . Both ducti stented with coronary stents. Vertical vein angiogram showed both lungs drain to a confluence and then to SVC via ascending vertical vein with no obstruction. After stenting lung perfusion improved and the baby was stable and maintained 80% saturation on room air. Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Our case is unique with Heterotaxy, TAPVC, Dextrocardia and double ducti. Eventhough bilateral ductal stenting is technically challenging it is successful through femoral artery approach.

  19. Anomalous left the pulmonary dilemma coronary artery artery from a ...

    African Journals Online (AJOL)

    Anomalous origin ofthe left coronary artery from the pulmonary artery is an unusual congenital ... led us to review our experience of this anomaly over the past 10 years. During this .... New York: McGraw-Hill, 1978: 1345. 5. Bland EF, White PO, ...

  20. [Pregnancy in pulmonary arterial hypertension patients].

    Science.gov (United States)

    Rosengarten, Dror; Kramer, Mordechai R

    2013-09-01

    Pulmonary arterial hypertension (PAH) is a disorder defined by elevated mean pulmonary arterial pressure. PAH can be idiopathic or associated with a variety of medical conditions such as scleroderma, congenital heart disease, left heart failure, lung disease or chronic pulmonary thromboembolism. This progressive disease can cause severe right heart failure and death. Normal physiologic changes that occur during pregnancy may produce fatal consequences in PAH patients. Current guidelines recommend that pregnancy be avoided or terminated early in women with PAH. During the past decade, new advanced therapies for PAH have emerged gathering reports of successful pregnancies in patients with pulmonary hypertension. Substantial risk still exists and current recommendations have not changed. Nevertheless, in selected cases, if a patient insists on continuing the pregnancy, being fully aware of the risks involved, an intensive treatment approach should be implemented in experienced centers. This is necessary in order to control pulmonary hypertension during pregnancy and reduce the risk so as to improve outcomes. This review will focus on the pathophysiology of PAH in pregnancy and appropriate management during pregnancy, delivery and the post-partum period.

  1. Nonmassive acute pulmonary embolism: evaluation of the impact of pulmonary arterial wall distensibility on the assessment of the CT obstruction score.

    Science.gov (United States)

    Bigot, Julien; Rémy-Jardin, Martine; Duhamel, Alain; Gorgos, Andréi-Bogdan; Faivre, Jean-Baptiste; Rémy, Jacques

    2010-02-01

    To evaluate the impact of pulmonary arterial wall distensibility on the assessment of a computed tomography (CT) score in patients with nonmassive pulmonary embolism (PE) (ie, Mastora score). The arterial wall distensibility of five central pulmonary arteries (pulmonary artery trunk, right and left main pulmonary arteries, right and left interlobar pulmonary arteries) was studied on ECG-gated CT angiographic studies of the chest in 15 patients with no pulmonary arterial hypertension (group 1; mean pulmonary artery pressure: 17.2 mm Hg) and 9 patients with nonmassive PE (group 2), using 2D reconstructions at every 10% of the R-R interval. The systolic and diastolic reconstruction time windows of the examined arteries were identical in the 2 groups, obtained at 20% and 80% of the R-R interval, respectively. No statistically significant difference was observed between the mean values of the pulmonary arterial wall distensibility between the 2 groups, varying between 20.5% and 24% in group 1 and between 23.3% and 25.9% in group 2. The coefficients of variation of the average arterial surfaces were found to vary between 4.30% and 6.50% in group 1 and 4.2% and 8.4% in group 2. Except the pulmonary artery trunk in group 2, all the intraclass correlation coefficients were around 0.8 or greater than 0.8, that is the cutoff for good homogeneity of measurements. The pulmonary arterial wall systolic-diastolic distensibility does not interfere with the assessment of a CT obstruction score in the setting of nonmassive PE.

  2. Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

    International Nuclear Information System (INIS)

    Griffin, N.; Allen, D.; Wort, J.; Rubens, M.; Padley, S.

    2007-01-01

    Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity

  3. Eisenmenger syndrome and idiopathic pulmonary arterial hypertension: do parenchymal lung changes reflect aetiology?

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Allen, D. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Wort, J. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Rubens, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-06-15

    Aim: To document the pulmonary vascular changes on thin-section computed tomography (CT) in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, and to determine whether there is any correlation with pulmonary arterial pressures or the aetiology of pulmonary hypertension. Material and methods: From the National Pulmonary Hypertension Database, we identified eight patients with idiopathic pulmonary arterial hypertension and 20 patients with Eisenmenger syndrome (secondary to a ventriculoseptal defect) who had also undergone contrast-enhanced thin-section CT. CT studies were reviewed for the presence of centrilobular nodules, mosaicism, neovascularity, and bronchial artery hypertrophy. Haemodynamic data were also reviewed. Results: Centrilobular nodules, mosaicism, and neovascularity were seen in both patient groups (p > 0.05). A significantly higher number of enlarged bronchial arteries were seen in patients with Eisenmenger syndrome. There was no correlation with pulmonary arterial pressures. Conclusion: Patients with idiopathic pulmonary arterial hypertension and Eisenmenger syndrome demonstrated similar pulmonary vascular changes on CT. These changes did not predict the underlying cause of pulmonary hypertension or its severity.

  4. CT assessment of tracheobronchial anomaly in left pulmonary artery sling

    Energy Technology Data Exchange (ETDEWEB)

    Zhong, Yu-Min M.; Zhu, Ming; Sun, Ai-Min M.; Wang, Qian [Shanghai Jiao Tong University School of Medicine, Department of Radiology, Shanghai Children' s Medical Center, Shanghai (China); Jaffe, Richard B. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Gao, Wei [Shanghai Jiao Tong University School of Medicine, Department of Cardiology, Shanghai Children' s Medical Center, Shanghai (China)

    2010-11-15

    The left pulmonary artery sling (LPAS) is a rare vascular anomaly where the left pulmonary artery arises from the right pulmonary artery, passes over the right bronchus, and goes posteriorly between the trachea and esophagus. The LPAS is frequently associated with cardiac and non-cardiac defects including tracheobronchial abnormalities. To evaluate the utility of multislice CT (MSCT) and helical CT (HCT) in diagnosing and defining the tracheobronchial anomaly and anatomic relationships between the trachea and aberrant left pulmonary artery. MSCT or HCT was performed in 27 children to determine the tracheobronchial anatomy and identify tracheobronchial stenosis. Eighteen children underwent surgery. According to the Wells [6] classification of LPAS, which includes two main types and two subtypes, there were eight cases of type 1A, five cases of type 1B, six cases of type 2A and eight cases of type 2B in this group. Twenty-four of the 27 children had substantial tracheobronchial stenosis. Four died before surgery; the 18 had reanastomosis of the left pulmonary artery. Five children also had tracheoplasty; three died after surgery. CT, especially MSCT, is an ideal modality for simultaneously identifying aberrant left pulmonary artery and any associated tracheobronchial anomaly. The Wells classification is useful for operative planning. (orig.)

  5. Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children

    Science.gov (United States)

    Frank, Benjamin S.

    2018-01-01

    Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure. In recent years, a number of pulmonary arterial hypertension (PAH)-targeted therapies have become available to reduce pulmonary artery pressure and improve outcome. A growing body of evidence in both the adult and pediatric literature demonstrates enhanced quality of life, functional status, and survival among treated patients. This review provides a description of select etiologies of PH seen in pediatrics and an update on the most recent data pertaining to evaluation and management of children with PH/PAH. The available evidence for specific classes of PAH-targeted therapies in pediatrics is additionally discussed. PMID:29570688

  6. Definition, classification, and epidemiology of pulmonary arterial hypertension.

    Science.gov (United States)

    Hoeper, Marius M

    2009-08-01

    Pulmonary arterial hypertension (PAH) is a distinct subgroup of pulmonary hypertension that comprises idiopathic PAH, familial/heritable forms, and PAH associated with connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus (HIV) infection, and some other conditions. The hemodynamic definition of PAH was recently revised: PAH is now defined by a mean pulmonary artery pressure at rest > or =25 mm Hg in the presence of a pulmonary capillary wedge pressure or =30 mm Hg during exercise) that was used in the old definition of PAH has been removed because there are no robust data that would allow defining an upper limit of normal for the pulmonary pressure during exercise. The revised classification of pulmonary hypertension still consists of five major groups: (1) PAH, (2) pulmonary hypertension due to left heart disease, (3) pulmonary hypertension due to chronic lung disease and/or hypoxia, (4) chronic thromboembolic pulmonary hypertension, and (5) miscellaneous forms. Modifications have been made in some of these groups, such as the addition of schistosomiasis-related pulmonary hypertension and pulmonary hypertension in patients with chronic hemolytic anemia to group 1.

  7. Characterization of proximal pulmonary arterial cells from chronic thromboembolic pulmonary hypertension patients

    Directory of Open Access Journals (Sweden)

    Quarck Rozenn

    2012-03-01

    Full Text Available Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH is associated with proximal pulmonary artery obstruction and vascular remodeling. We hypothesized that pulmonary arterial smooth muscle (PASMC and endothelial cells (PAEC may actively contribute to remodeling of the proximal pulmonary vascular wall in CTEPH. Our present objective was to characterize PASMC and PAEC from large arteries of CTEPH patients and investigate their potential involvement in vascular remodeling. Methods Primary cultures of proximal PAEC and PASMC from patients with CTEPH, with non-thromboembolic pulmonary hypertension (PH and lung donors have been established. PAEC and PASMC have been characterized by immunofluorescence using specific markers. Expression of smooth muscle specific markers within the pulmonary vascular wall has been studied by immunofluorescence and Western blotting. Mitogenic activity and migratory capacity of PASMC and PAEC have been investigated in vitro. Results PAEC express CD31 on their surface, von Willebrand factor in Weibel-Palade bodies and take up acetylated LDL. PASMC express various differentiation markers including α-smooth muscle actin (α-SMA, desmin and smooth muscle myosin heavy chain (SMMHC. In vascular tissue from CTEPH and non-thromboembolic PH patients, expression of α-SMA and desmin is down-regulated compared to lung donors; desmin expression is also down-regulated in vascular tissue from CTEPH compared to non-thromboembolic PH patients. A low proportion of α-SMA positive cells express desmin and SMMHC in the neointima of proximal pulmonary arteries from CTEPH patients. Serum-induced mitogenic activity of PAEC and PASMC, as well as migratory capacity of PASMC, were increased in CTEPH only. Conclusions Modified proliferative and/or migratory responses of PASMC and PAEC in vitro, associated to a proliferative phenotype of PASMC suggest that PASMC and PAEC could contribute to proximal vascular remodeling in CTEPH.

  8. Bilateral multiple pulmonary artery aneurysms associated with cavitary pulmonary tuberculosis: a case report.

    Science.gov (United States)

    Pallangyo, Pedro; Lyimo, Frederick; Bhalia, Smita; Makungu, Hilda; Nyangasa, Bashir; Lwakatare, Flora; Suranyi, Pal; Janabi, Mohamed

    2017-07-19

    Pulmonary artery aneurysms constitute 50%) of cases, however, pulmonary artery aneurysm is a rare sequelae of pulmonary tuberculosis reported in about 5% of patients with chronic cavitary tuberculosis on autopsy. The natural history of this potentially fatal condition remains poorly understood and guidelines for optimal management are controversial. A 24-year-old man, a nursing student of African descent, was referred to us from an up-country regional hospital with a 4-week history of recurrent episodes of breathlessness, awareness of heartbeats and coughing blood 3 weeks after completing a 6-month course of anti-tuberculosis drugs. A physical examination revealed conjuctival and palmar pallor but there were no stigmata of connective tissue disorders, systemic vasculitides or congenital heart disease. An examination of the cardiovascular system revealed accentuated second heart sound (S 2 ) with early diastolic (grade 1/6) and holosystolic (grade 2/6) murmurs at the pulmonic and tricuspid areas respectively. Blood tests showed iron deficiency anemia, prolonged bleeding time, and mild hyponatremia. A chest radiograph revealed bilateral ovoid-shaped perihilar opacities while a computed tomography scan showed bilateral multiple pulmonary artery pseudoaneurysms with surrounding hematoma together with adjacent cystic changes, consolidations, and tree-in-bud appearance. Our patient refused to undergo surgery and died of aneurismal rupture after 9 days of hospitalization. The presence of intractable hemoptysis among patients with tuberculosis even after completion of anti-tuberculosis course should raise an index of suspicion for pulmonary artery aneurysm. Furthermore, despite of its rarity, early recognition and timely surgical intervention of pulmonary artery aneurysm is crucial to reducing morbidity and preventing the attributed mortality.

  9. Pulmonary artery aneurysm in Bechcet's disease: helical computed tomography study

    International Nuclear Information System (INIS)

    Munoz, J.; Caballero, P.; Olivera, M. J.; Cajal, M. L.; Caniego, J. L.

    2000-01-01

    Behcet's disease is a vasculitis of unknown etiology that affects arteries and veins of different sizes and can be associated with pulmonary artery aneurysms. We report the case of a patient with Behcet's disease and a pulmonary artery aneurysm who was studied by means of plain chest X ray, helical computed tomography and pulmonary arteriography. Helical computed tomography is a reliable technique for the diagnosis and follow-up of these patients. (Author) 9 refs

  10. Isolated Unilateral Pulmonary Artery Agenesis complicated by Symptomatic Aspergilloma

    LENUS (Irish Health Repository)

    Daly, A

    2017-11-01

    Isolated unilateral pulmonary artery agenesis is a rare diagnosis. Poor blood flow to the lung parenchyma renders the tissue susceptible to opportunistic infections. We present the unusual case of isolated unilateral pulmonary artery agenesis complicated by aspergilloma. Management options and considerations are discussed.

  11. Pulmonary artery pulse pressure and wave reflection in chronic pulmonary thromboembolism and primary pulmonary hypertension.

    Science.gov (United States)

    Castelain, V; Hervé, P; Lecarpentier, Y; Duroux, P; Simonneau, G; Chemla, D

    2001-03-15

    The purpose of this time-domain study was to compare pulmonary artery (PA) pulse pressure and wave reflection in chronic pulmonary thromboembolism (CPTE) and primary pulmonary hypertension (PPH). Pulmonary artery pressure waveform analysis provides a simple and accurate estimation of right ventricular afterload in the time-domain. Chronic pulmonary thromboembolism and PPH are both responsible for severe pulmonary hypertension. Chronic pulmonary thromboembolism and PPH predominantly involve proximal and distal arteries, respectively, and may lead to differences in PA pressure waveform. High-fidelity PA pressure was recorded in 14 patients (7 men/7 women, 46 +/- 14 years) with CPTE (n = 7) and PPH (n = 7). We measured thermodilution cardiac output, mean PA pressure (MPAP), PA pulse pressure (PAPP = systolic - diastolic PAP) and normalized PAPP (nPAPP = PPAP/MPAP). Wave reflection was quantified by measuring Ti, that is, the time between pressure upstroke and the systolic inflection point (Pi), deltaP, that is, the systolic PAP minus Pi difference, and the augmentation index (deltaP/PPAP). At baseline, CPTE and PPH had similar cardiac index (2.4 +/- 0.4 vs. 2.5 +/- 0.5 l/min/m2), mean PAP (59 +/- 9 vs. 59 +/- 10 mm Hg), PPAP (57 +/- 13 vs. 53 +/- 13 mm Hg) and nPPAP (0.97 +/- 0.16 vs. 0.89 +/- 0.13). Chronic pulmonary thromboembolism had shorter Ti (90 +/- 17 vs. 126 +/- 16 ms, p PPAP (0.26 +/- 0.01 vs. 0.09 +/- 0.07, p < 0.01). Our study indicated that: 1) CPTE and PPH with severe pulmonary hypertension had similar PA pulse pressure, and 2) wave reflection is elevated in both groups, and CPTE had increased and anticipated wave reflection as compared with PPH, thus suggesting differences in the pulsatile component of right ventricular afterload.

  12. Reversal of echocardiographic right-sided heart pathology in a dog with severe pulmonary hypertension: a case report

    Directory of Open Access Journals (Sweden)

    McMahon P

    2015-06-01

    Full Text Available Peggy McMahon,1 Carley Saelinger 2  1Emergency and Critical Care Department, 2Cardiology Department, Animal Specialty and Emergency Center, Los Angeles, CA, USA Abstract: Pathologic right-sided heart changes are a common echocardiographic finding in patients with pulmonary hypertension (PH. Canines with PH may have right heart pathology documented via echocardiographic color Doppler interrogation including tricuspid valve regurgitation, pulmonic valve insufficiency, elevated pulmonary arterial systolic pressure, elevated pulmonary arterial diastolic pressure, and alterations in ejection profiles. Two-dimensional echocardiographic findings may include right ventricular hypertrophy, interventricular septal flattening, paradoxical interventricular septal motion, pulmonary artery dilation, and potentially abnormal left heart dimensions. In veterinary medicine, much confidence is given to the measurement of pulmonary arterial systolic pressure estimated from tricuspid valve regurgitation to grade the severity of PH and monitor its improvement with little emphasis placed on the integration of two-dimensional echocardiographic right and left heart pathology in conjunction with Doppler findings. To the authors’ knowledge, marked improvement and/or resolution of echocardiographic-documented right heart pathology have not been previously reported in the veterinary literature. This case report documents profound echocardiographic improvement of right-sided heart disease in a dog with severe PH. Keywords: canine, pulmonary hypertension, tricuspid valve regurgitation, right heart hypertrophy, sildenafil  

  13. Prenatal diagnosis of left pulmonary artery-to-pulmonary vein fistula and its successful surgical repair in a neonate.

    Science.gov (United States)

    Ostras, Oleksii; Kurkevych, Andrii; Bohuta, Lyubomyr; Yalynska, Tetyana; Raad, Tammo; Lewin, Mark; Yemets, Illya

    2015-04-01

    Pulmonary arteriovenous fistula is a rare disease. To the best of our knowledge, prenatal diagnosis of a fistula between the left pulmonary artery and the left pulmonary vein has not been described in the medical literature. We report a case of the prenatal diagnosis of a left pulmonary artery-to-pulmonary vein fistula, followed by successful neonatal surgical repair.

  14. A case of William's syndrome associated peripheral pulmonary arterial stenosis

    International Nuclear Information System (INIS)

    Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan

    1988-01-01

    William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures

  15. Intra-arterial digital subtraction angiography of the pulmonary arteries using a flow-directed balloon catheter in the diagnosis of pulmonary embolism

    International Nuclear Information System (INIS)

    Rooij, W.J.J. van; Heeten, G.J. den

    1992-01-01

    Selective intra-arterial digital subtraction angiography (IA-DSA) of the pulmonary vessels was performed in 70 patients suspected of acute pulmonary embolism. A flow-directed Swan-Ganz pulmonary angiography catheter was used. The spatial resolution of the equipment used was 3.3 lp/mm for DSA and 6.0 lp/mm for conventional pulmonary angiography (CPA). Image quality of the angiograms was assessed by determining the highest visible branching division of the main pulmonary artery. The mean visible branching division for IA-DSA was 4.71 (range 3-7). In 10 patients where IA-DSA and CPA were performed during the same procedure there was no difference in visualization of peripheral arteries (mean 4.70 visible or for both modalities). IA-DSA makes the procedure rapid, saves on films and contrast material and allows good visualization of areas where exposure is difficult. The spatial resolution of state-of-the-art equipment permits sufficient definition of subsegmental vessels. The use of the flow-directed balloon catheter makes the examination easy to perform and minimizes the risk of catheter induced cardiac arrhythmias. (orig.) [de

  16. Pulmonary artery remodeling differs in hypoxia- and monocrotaline-induced pulmonary hypertension

    NARCIS (Netherlands)

    van Suylen, R. J.; Smits, J. F.; Daemen, M. J.

    1998-01-01

    In the present study we analyzed structural characteristics of muscular pulmonary arteries and arterioles in two classic models of pulmonary hypertension, the rat hypoxia and monocrotaline models. We hypothesized that an increase in medial cross-sectional area would result in reduction of the lumen

  17. Cardiac magnetic resonance imaging-derived pulmonary artery distensibility index correlates with pulmonary artery stiffness and predicts functional capacity in patients with pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Kang, Ki-Woon; Chang, Hyuk-Jae; Kim, Young-Jin; Choi, Byoung-Wook; Yang, Woo-In; Shim, Chi-Young; Ha, Jongwon; Chung, Namsik; Lee, Hye-Sun

    2011-01-01

    Increased stiffness of the pulmonary vascular bed is known to increase mortality in patients with pulmonary arterial hypertension (PAH); and pulmonary artery (PA) stiffness is also thought to be associated with exercise capacity. The purpose of the present study was to investigate whether cardiac magnetic resonance imaging (CMRI)-derived PA distensibility index correlates with PA stiffness estimated on right heart catheterization (RHC) and predicts functional capacity (FC) in patients with PAH. Thirty-five consecutive PAH patients (23% male, mean age, 44±13 years; 69% idiopathic) underwent CMRI, RHC, and 6-min walk test (6MWT). PA distensibility indices were derived from cross-sectional area change (%) in the transverse view, perpendicular to the axis of the main PA, on CMRI [(maximum area-minimum area)/minimum area during cardiac cycle]. Among the PA stiffness indices, pulmonary vascular resistance (PVR) and PA capacitance were calculated using hemodynamic dataset from RHC. CMRI-derived PA distensibility was inversely correlated with PVR (R 2 =0.34, P 2 =0.35, P 2 =0.61, P<0.001). Furthermore, PA distensibility <20% predicted poor FC (<400 m in 6MWT) with a sensitivity of 82% and a specificity of 94%. Non-invasive CMRI-derived PA distensibility index correlates with PA stiffness and can predict FC in patients with PAH. (author)

  18. Cardiac and pulmonary artery mensuration in feline heartworm disease

    International Nuclear Information System (INIS)

    Schafer, M.; Berry, C.R.

    1995-01-01

    A retrospective study was undertaken to quantify thoracic radiographic changes in cats with heartworm diseases, (Dirofilaria immitis). Using a blinded study format, the cardiac silhouette, thoracic cavity and pulmonary arteries were measured from thoracic radiographs of 21 cats with feline heartworm disease and 30 cats without known cardiac or pulmonary vessel pathology. Measured data were normalized to the thoracic cavity or bony structures within the radiographic field of view. The measurements were compared between the two groups of cats using an unpaired, two-tailed Student's t-test, with a p value of < 0.05 being considered significant. Cats with feline heartworm disease had enlargement of the craniocaudal aspect of the cardiac silhouette and normalized cardiac:thoracic ratio (p < 0.05) on the lateral view. Also, there was significant enlargement of the central and peripheral caudal lobar pulmonary arteries and their normalized ratios (p < 0.05) in the heartworm infected cats as visualized on the ventrodorsal projection. Tortuosity of the pulmonary arteries was seen in three of the 21 infected cats. Eleven of the 21 cats with feline heartworm disease had pulmonary parenchymal changes. Based on the present study, central and peripheral pulmonary artery enlargement as viewed on the ventrodorsal radiograph was the single best radiographic indicator of feline heartworm disease

  19. Unilateral Pulmonary Artery Agenesis with Ipsilateral Pulmonary Hypoplasia as Incidental Finding in an Asthmatic Patient

    Directory of Open Access Journals (Sweden)

    Catalina Contreras-Arias

    2014-01-01

    Full Text Available Unilateral absence of a pulmonary artery is an uncommon congenital heart disease. It can be related to respiratory symptoms such as asthma, an unsual nding in some of these patients. This paper reports the case of a 4-year-old male with recurrent respiratory infections and asthma symptoms, in who further studies found agenesia of right pulmonary artery with pulmonary hypoplasia of the same side.

  20. Anomalous origin of the left coronary artery from the pulmonary artery in children: diagnostic use of multidetector computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Shen, Quanli; Yao, Qiong; Hu, Xihong [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China)

    2016-09-15

    Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly. It is important to demonstrate the anomalous origin of the left coronary artery and its course before surgery. To explore the clinical diagnostic use of multidetector CT coronary angiography in detecting anomalous origin of the left coronary artery from the pulmonary artery in children. Nine children (2 boys, 7 girls) ages 2 months to 9 years with surgically confirmed anomalous origin of the left coronary artery from the pulmonary artery were studied. Clinical data, transthoracic echocardiography and CT coronary angiography images were retrospectively analyzed. Transthoracic echocardiography correctly diagnosed anomalous origin of the left coronary artery from the pulmonary artery in 7 of 9 patients (95% CI: 40-97%). CT coronary angiography revealed the anomalous origin of the left coronary artery in all children (95% CI: 66-100%). In a 4-year-old girl and a 9-year-old girl, CT coronary angiography showed dilation of the right coronary artery and collateral circulation between the right and the left coronary arteries. CT coronary angiography is a useful method to show the anomalous origin of the coronary artery in children with anomalous origin of the left coronary artery from the pulmonary artery, especially for patients in whom origin of the left coronary artery cannot be detected by transthoracic echocardiography. (orig.)

  1. Anomalous origin of the left coronary artery from the pulmonary artery in children: diagnostic use of multidetector computed tomography

    International Nuclear Information System (INIS)

    Shen, Quanli; Yao, Qiong; Hu, Xihong

    2016-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly. It is important to demonstrate the anomalous origin of the left coronary artery and its course before surgery. To explore the clinical diagnostic use of multidetector CT coronary angiography in detecting anomalous origin of the left coronary artery from the pulmonary artery in children. Nine children (2 boys, 7 girls) ages 2 months to 9 years with surgically confirmed anomalous origin of the left coronary artery from the pulmonary artery were studied. Clinical data, transthoracic echocardiography and CT coronary angiography images were retrospectively analyzed. Transthoracic echocardiography correctly diagnosed anomalous origin of the left coronary artery from the pulmonary artery in 7 of 9 patients (95% CI: 40-97%). CT coronary angiography revealed the anomalous origin of the left coronary artery in all children (95% CI: 66-100%). In a 4-year-old girl and a 9-year-old girl, CT coronary angiography showed dilation of the right coronary artery and collateral circulation between the right and the left coronary arteries. CT coronary angiography is a useful method to show the anomalous origin of the coronary artery in children with anomalous origin of the left coronary artery from the pulmonary artery, especially for patients in whom origin of the left coronary artery cannot be detected by transthoracic echocardiography. (orig.)

  2. Diabetes Mellitus Associates with Increased Right Ventricular Afterload and Remodeling in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Whitaker, Morgan E; Nair, Vineet; Sinari, Shripad; Dherange, Parinita A; Natarajan, Balaji; Trutter, Lindsey; Brittain, Evan L; Hemnes, Anna R; Austin, Eric D; Patel, Kumar; Black, Stephen M; Garcia, Joe G N; Yuan Md PhD, Jason X; Vanderpool, Rebecca R; Rischard, Franz; Makino, Ayako; Bedrick, Edward J; Desai, Ankit A

    2018-06-01

    Diabetes mellitus is associated with left ventricular hypertrophy and dysfunction. Parallel studies have also reported associations between diabetes mellitus and right ventricular dysfunction and reduced survival in patients with pulmonary arterial hypertension. However, the impact of diabetes mellitus on the pulmonary vasculature has not been well characterized. We hypothesized that diabetes mellitus and hyperglycemia could specifically influence right ventricular afterload and remodeling in patients with Group I pulmonary arterial hypertension, providing a link to their known susceptibility to right ventricular dysfunction. Using an adjusted model for age, sex, pulmonary vascular resistance, and medication use, associations of fasting blood glucose, glycated hemoglobin, and the presence of diabetes mellitus were evaluated with markers of disease severity in 162 patients with pulmonary arterial hypertension. A surrogate measure of increased pulmonary artery stiffness, elevated pulmonary arterial elastance (P = .012), along with reduced log(pulmonary artery capacitance) (P = .006) were significantly associated with the presence of diabetes mellitus in patients with pulmonary arterial hypertension in a fully adjusted model. Similar associations between pulmonary arterial elastance and capacitance were noted with both fasting blood glucose and glycated hemoglobin. Furthermore, right ventricular wall thickness on echocardiography was greater in pulmonary arterial hypertension patients with diabetes, supporting the link between right ventricular remodeling and diabetes. Cumulatively, these data demonstrate that an increase in right ventricular afterload, beyond pulmonary vascular resistance alone, may influence right ventricular remodeling and provide a mechanistic link between the susceptibility to right ventricular dysfunction in patients with both diabetes mellitus and pulmonary arterial hypertension. Copyright © 2018 Elsevier Inc. All rights reserved.

  3. Continuous inhaled iloprost in a neonate with d-transposition of the great arteries and severe pulmonary arterial hypertension.

    Science.gov (United States)

    Dykes, John C; Torres, Marilyn; Alexander, Plato J

    2016-03-01

    This report describes the case of a neonate with d-transposition of the great arteries and severe pulmonary arterial hypertension stabilised in the post-operative period with continuous iloprost nebulisation. To our knowledge, this is the first documented method of treating post-operative severe pulmonary arterial hypertension with continuous inhaled iloprost in a patient with complex CHD. We found this method of delivering the drug very effective in stabilising haemodynamic swings in the setting of severe pulmonary arterial hypertension.

  4. Macitentan for the treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Kholdani CA

    2014-11-01

    Full Text Available Cyrus A Kholdani,1 Wassim H Fares,2 Terence K Trow2 1Section of Pulmonary, Critical Care and Sleep Medicine, 2Yale Pulmonary Vascular Disease Program, Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA Abstract: Macitentan is the most recently approved dual endothelin-receptor antagonist (ERA for the treatment of symptomatic pulmonary arterial hypertension. Compared to other available ERAs, it demonstrates superior receptor-binding properties, with consequently improved tissue penetration, and a longer duration of action allowing for once-daily dosing. It has a favorable adverse-effect profile, with notably no demonstrable increase in the risk of hepatotoxicity or peripheral edema, but like other ERAs, it is potentially limited by significant anemia. Phase I data have demonstrated a favorable drug–drug interaction profile and no need for dose adjustment with hepatic and renal impairment. In the pivotal SERAPHIN study, treatment of symptomatic pulmonary arterial hypertension patients with macitentan led to statistically significant improvements in functional class, exercise tolerance, and hemodynamic parameters, in addition to a reduction in morbidity in an event-driven long-term trial. Keywords: endothelin, endothelin receptor antagonists, macitentan, pulmonary arterial hypertension

  5. The establishment of bilateral external iliac artery stenosis model in experimental canines and its angiographic evaluation

    International Nuclear Information System (INIS)

    Xia Yonghui; Li Weixiao; Bi Yonghua; Xu Ke

    2011-01-01

    Objective: To establish an experimental canine model of bilateral external iliac artery stenosis by surgical method with absorbable suture. Methods: Under general anesthesia bilateral external iliac arteries were partly obstructed (about 50%) with absorbable suture in ten dogs. Three months later angiography was performed to evaluate the arterial stricture degree. Results: Bilateral external iliac artery stenosis model was successfully established in eight dogs and the other two dogs died within two weeks after the procedure. Angiography performed three months after the procedure showed that the stricture degree of arterial lumen was (60.6±12.5)%. Conclusion: Satisfactory experimental canine model of bilateral external iliac artery stenosis can be established by surgical method with absorbable suture. This method can be used for reference when peripheral artery stenosis model is to be prepared in larger animals. (authors)

  6. Right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus

    International Nuclear Information System (INIS)

    Lee, Peter; McCauley, Roy; Westra, Sjirk; Baba, Timothy

    2006-01-01

    Pulmonary aplasia and bronchopulmonary foregut malformations in which a patent communication between the foregut and the pulmonary system is present are rare congenital abnormalities. Pulmonary aplasia associated with a pulmonary sling is an even rarer abnormality. We report a unique case of right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus diagnosed by multidetector helical CT. (orig.)

  7. Pulmonary arterial lesions in explanted lungs after transplantation correlate with severity of pulmonary hypertension in chronic obstructive pulmonary disease

    DEFF Research Database (Denmark)

    Carlsen, Jørn; Andersen, Kasper Hasseriis; Boesgaard, Søren

    2013-01-01

    BACKGROUND: Pulmonary vascular findings are largely unreported in end-stage chronic obstructive pulmonary disease (COPD). METHODS: Pulmonary vascular lesions in explanted lungs from 70 patients with COPD/emphysema or α-1-antitrypsin deficiency were analyzed retrospectively. Patients were stratified...... of pulmonary vascular lesions in COPD correlate with the severity of PH. Morphologic lesions similar to those characteristic of IPAH can be observed as PH in COPD progresses to levels characteristic of IPAH....... by the presence and severity of pulmonary hypertension (PH) assessed by right-heart catheterization in 3 hemodynamically distinct groups: (1) non-PH (mean pulmonary arterial pressure [mPAP]50 mm Hg; median HE Grade 4 (range 3-6), with generalized arterial dilatation and plexiform lesions. CONCLUSIONS: The extent...

  8. Rhabdomyosarcoma of the pulmonary artery

    International Nuclear Information System (INIS)

    Barth, J.; Lehmann, H.; Thermann, M.; Horny, H.P.; Stein, H.; Kiel Univ.; Kiel Univ.; Kiel Univ.

    1982-01-01

    A case of a 55-year-old man with the histological diagnosis rhabdomyosarcoma of the left pulmonary artery has been seen. Lung scanning and pulmonary arteriography are the clues for the diagnostical procedure. 55 cases from the literature are reviewed and clinical findings of the early and late stages of the diseases are discussed. Surgical treatment is the therapy of choice if ever possible; aggressive chemotherapy might be an acceptable alternative. (orig.) [de

  9. Impact of age on pulmonary artery systolic pressures at rest and with exercise

    Directory of Open Access Journals (Sweden)

    Garvan C Kane

    2016-06-01

    Full Text Available Aim: It is not well known if advancing age influences normal rest or exercise pulmonary artery pressures. The purpose of the study was to evaluate the association of increasing age with measurements of pulmonary artery systolic pressure at rest and with exercise. Subjects and methods: A total of 467 adults without cardiopulmonary disease and normal exercise capacity (age range: 18–85 years underwent symptom-limited treadmill exercise testing with Doppler measurement of rest and exercise pulmonary artery systolic pressure. Results: There was a progressive increase in rest and exercise pulmonary artery pressures with increasing age. Pulmonary artery systolic pressures at rest and with exercise were 25 ± 5 mmHg and 33 ± 9 mmHg, respectively, in those <40 years, and 30 ± 5 mmHg and 41 ± 12 mmHg, respectively, in those ≥70 years. While elevated left-sided cardiac filling pressures were excluded by protocol design, markers of arterial stiffness associated with the age-dependent effects on pulmonary pressures. Conclusion: These data demonstrate that in echocardiographically normal adults, pulmonary artery systolic pressure increases with advancing age. This increase is seen at rest and with exercise. These increases in pulmonary pressure occur in association with decreasing transpulmonary flow and increases in systemic pulse pressure, suggesting that age-associated blood vessel stiffening may contribute to these differences in pulmonary artery systolic pressure.

  10. Egg and banana sign of severe pulmonary arterial hypertension.

    Science.gov (United States)

    Veean, Satyam; Nixon, William; Keshavamurthy, Jayanth

    2018-01-01

    The egg and banana sign can be seen on chest computed tomography (CT) in patients with severe pulmonary arterial hypertension (PAH). It is identified by the presence of the pulmonary artery (PA) lateral to the aortic arch with the aortic arch being described as the banana and the PA as the egg.

  11. Pulmonary arterial hypertension in congenital heart disease: Correlation of radiologic index with hemodynamic data

    International Nuclear Information System (INIS)

    Choi, Young Hi

    1984-01-01

    It is well known that pulmonary arterial hypertension in congenital heart disease is an important prognostic factor, as is pulmonary vascular resistance. So it is tempting to get certain radiologic index that could predict the presence and the degree of pulmonary arterial hypertension. A total of 152 cases of left to right shunt with pulmonary arterial hypertension and 50 cases of left to right shunt without pulmonary arterial hypertension is presented, in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between March 1981 and February 1983. Statistical analysis of plain radiography findings with the emphasis on the correction of radiologic index with the hemodynamic data. The results are as follows: 1. The incidence of pulmonary arterial hypertension is much less in arterial septal defect than other two disease groups of left to right shunt. 2. PA/T ratio correlates well with pulmonary arterial pressure (r=0.674), especially in mild pulmonary hypertension group. No correlation in moderate pulmonary hypertension group in significant level. 3. PA/T ratio is below 38 in total cases of normal control group and in 32 cases (21.0%) among 152 cases of pulmonary arterial hypertension group. 4. The average PA/T ratio in normal pressure group of left to right shunt is 35.3, which has no significant difference from that of normal control group. 5. The average CT ratio of pulmonary arterial hypertension group is 59.0, which is larger than 49.1 of normal control group. The CT ratio shows no correlation with the pulmonary arterial pressure in statistically significant level. 6. The higher the pulmonary arterial pressure, the larger the Rp/Rs value. The Rp/Rs in atrial septal defect is 0.193 in average, the lowest value in comparison with other two disease groups.

  12. Congenital unilateral absence of the pulmonary artery in adults

    International Nuclear Information System (INIS)

    Gonzalez Garcia, Mauricio; Escalante Mora, Hector A; Lozano Castillo, Alfonso J

    2000-01-01

    Unilateral absence of a pulmonary artery is a rare anomaly. It occurs with pulmonary ipsilateral hypoplasia and it's frequently associated with other cardiovascular malformations. The majority of the cases are diagnosed in childhood. This is a case report of two adult patients of the Hospital Central de la Policia Nacional in Bogota, Colombia, with unilateral absence of the pulmonary artery one isolated and the other with patent ductus arteriosus. We describe the clinical and roentgenographic findings of this congenital anomaly

  13. Bloodless Repair of Isolated Pulmonary Artery in a Neonate.

    Science.gov (United States)

    Wang, Hanjay; Brewer, Michael P; Lai, Wyman W; Krishnamurthy, Ganga; Chai, Paul J

    2016-01-01

    Pediatric cardiac surgery, especially for small neonates, typically requires blood products to counter hemodilution during cardiopulmonary bypass. Children with congenital heart defects whose families adhere to faith-based proscriptions against blood transfusion therefore represent a challenging surgical population. Here, we report the case of a ten-day-old, 3.6-kg patient of Jehovah's Witness faith, who was diagnosed with unilateral pulmonary artery discontinuity, bilateral patent ductus arteriosus, and an otherwise structurally normal heart. Pulmonary artery reimplantation was successfully performed without giving blood products. This case adds to previous reports of successful bloodless cardiac surgery in neonates and describes the specific strategies that contributed to successful pulmonary artery reimplantation. © The Author(s) 2015.

  14. Fatal haemoptysis from the pulmonary artery as a late complication of pulmonary irradiation

    International Nuclear Information System (INIS)

    Makker, H.K.; Barnes, P.C.

    1991-01-01

    Fatal massive haemoptysis occured as a late complication of erosion of the pulmonary artery by a non-malignant ulcer of the left main bronchus. Symptoms attributed to radiation pulmonary fibrosis are uncommon. We report a case of fatal massive haemoptysis in a patient known to have postirradiation pulmonary fibrosis. (author)

  15. Pulmonary artery wave propagation and reservoir function in conscious man: impact of pulmonary vascular disease, respiration and dynamic stress tests.

    Science.gov (United States)

    Su, Junjing; Manisty, Charlotte; Simonsen, Ulf; Howard, Luke S; Parker, Kim H; Hughes, Alun D

    2017-10-15

    Wave travel plays an important role in cardiovascular physiology. However, many aspects of pulmonary arterial wave behaviour remain unclear. Wave intensity and reservoir-excess pressure analyses were applied in the pulmonary artery in subjects with and without pulmonary hypertension during spontaneous respiration and dynamic stress tests. Arterial wave energy decreased during expiration and Valsalva manoeuvre due to decreased ventricular preload. Wave energy also decreased during handgrip exercise due to increased heart rate. In pulmonary hypertension patients, the asymptotic pressure at which the microvascular flow ceases, the reservoir pressure related to arterial compliance and the excess pressure caused by waves increased. The reservoir and excess pressures decreased during Valsalva manoeuvre but remained unchanged during handgrip exercise. This study provides insights into the influence of pulmonary vascular disease, spontaneous respiration and dynamic stress tests on pulmonary artery wave propagation and reservoir function. Detailed haemodynamic analysis may provide novel insights into the pulmonary circulation. Therefore, wave intensity and reservoir-excess pressure analyses were applied in the pulmonary artery to characterize changes in wave propagation and reservoir function during spontaneous respiration and dynamic stress tests. Right heart catheterization was performed using a pressure and Doppler flow sensor tipped guidewire to obtain simultaneous pressure and flow velocity measurements in the pulmonary artery in control subjects and patients with pulmonary arterial hypertension (PAH) at rest. In controls, recordings were also obtained during Valsalva manoeuvre and handgrip exercise. The asymptotic pressure at which the flow through the microcirculation ceases, the reservoir pressure related to arterial compliance and the excess pressure caused by arterial waves increased in PAH patients compared to controls. The systolic and diastolic rate constants

  16. Massive tumor pulmonary embolism following transcatheter arterial chemoembolization of hepatocellular carcinoma

    International Nuclear Information System (INIS)

    Kim, Jeong Ho; Ko, Gi Young; Yoon, Hyun Ki; Sung, Kyu Bo

    2002-01-01

    Pulmonary embolism complicated by transcatheter arterial chemoembolization (TACE) in known to be due to the use of large amount of lipiodol as an embolic agent. To our knowledge, massive tumoral pulmonary embolism following TACE and confirmed by surgery has not been described in the literature. In this report, we detail the cas of a 49-year-old man in whom cyanosis and hypotension developed abruptly on the day of TACE.. Chest CT revealed diffuse low-attenuated lesions in both pulmonary arteries. Histopathological specimens after emergent pulmonary arterial embolectomy confirmed the presence of massive tumor emboli of hepatocellular carcinoma

  17. Massive tumor pulmonary embolism following transcatheter arterial chemoembolization of hepatocellular carcinoma

    International Nuclear Information System (INIS)

    Kim, Jeong Ho; Ko, Gi Young; Yoon, Hyun Ki; Sung, Kyu Bo

    2002-01-01

    Pulmonary embolism complicated by transcatheter arterial chemoembolization (TACE) is known to be due to the use of large amount of lipiodol as an embolic agent. To our knowledge, massive tumoral pulmonary embolism following TACE and confirmed by surgery has not been described in the literature. In this report, we detail the case of a 49-year-old man in whom cyanosis and hypotension developed abruptly on the day of TACE. Chest CT revealed diffuse low-attenuated lesions in both pulmonary arteries. Histopathological specimens after emergent pulmonary arterial embolectomy confirmed the presence of massive tumor emboli of hepatocellular carcinoma

  18. A pulmonary sequestered segment with an aberrant pulmonary arterial supply: A case of unique anomaly

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Min Chul; Woo, Jeong Joo; An, Jin Kyung; Jung, Yoon Young; Choi, Yun Sun [Dept. of Radiology, Eulji Hospital, Eulji University, Seoul (Korea, Republic of)

    2016-04-15

    We presented a rare case of a 64-year-old man with a combined anomaly of the bronchus and pulmonary artery that was detected incidentally. Computed tomography showed a hyperlucent, aerated sequestered segment of the right lower lung with an independent ectopic bronchus, which had no connection to the other airway. The affected segment was supplied by its own aberrant pulmonary artery branch from the right pulmonary trunk. This anomaly cannot be classified with any of the previously reported anomalies.

  19. Rare associations of tetralogy of Fallot with anomalous left coronary artery from pulmonary artery and totally anomalous pulmonary venous connection.

    Science.gov (United States)

    Sen, Supratim; Rao, Suresh G; Kulkarni, Snehal

    2016-06-01

    We describe the cases of two patients with tetralogy of Fallot, aged 4 years and 8 months, who were incidentally detected to have concomitant anomalous left coronary artery from pulmonary artery and total anomalous pulmonary venous connection, respectively, on preoperative imaging. They underwent surgical correction with good mid-term outcomes. In this study, we discuss the embryological basis, physiological effects, and review the literature of these two unusual associations. Awareness of these rare associations will avoid missed diagnoses and consequent surgical surprises.

  20. ROCK2 mediates the proliferation of pulmonary arterial endothelial cells induced by hypoxia in the development of pulmonary arterial hypertension

    OpenAIRE

    QIAO, FENG; ZOU, ZHITIAN; LIU, CHUNHUI; ZHU, XIAOFENG; WANG, XIAOQIANG; YANG, CHENGPENG; JIANG, TENGJIAO; CHEN, YING

    2016-01-01

    It has been reported that RhoA activation and Rho-kinase (ROCK) expression are increased in chronic hypoxic lungs, and the long-term inhibition of ROCK markedly improves the survival of patients with pulmonary arterial hypertension (PAH). However, whether Rho-kinase α (ROCK2) participates in regulation of the growth of pulmonary arterial endothelial cells (PAECs) remains unknown. The aim of the present study was to investigate the effect of hypoxia on the proliferation of PAECs and the role o...

  1. Pulmonary artery dissection in eight dogs with patent ductus arteriosus.

    Science.gov (United States)

    Scansen, Brian A; Simpson, Elaine M; López-Alvarez, Jordi; Thomas, William P; Bright, Janice M; Eason, Bryan D; Rush, John E; Dukes-McEwan, Joanna; Green, Henry W; Cunningham, Suzanne M; Visser, Lance C; Kent, Agnieszka M; Schober, Karsten E

    2015-06-01

    To describe a series of dogs with pulmonary artery dissection and patent ductus arteriosus (PDA). Eight dogs. Retrospective case series. Pulmonary artery dissection was diagnosed in 8 dogs, 3 were Weimaraners. Four dogs presented in left-sided congestive heart failure, 4 presented for murmur evaluation and without clinical signs, and 1 presented in right-sided congestive heart failure. In 7 dogs the dissection was first documented concurrent with a diagnosis of uncorrected PDA. In the other dog, with pulmonary valve stenosis and PDA, the dissection was observed on autopsy examination 17 months after balloon pulmonary valvuloplasty and ductal closure. Median age at presentation for the 7 dogs with antemortem diagnosis of pulmonary artery dissection was 3.5 years (range, 1.5-4 years). Three dogs had the PDA surgically ligated, 2 dogs did not undergo PDA closure, 1 dog failed transcatheter occlusion of the PDA with subsequent surgical ligation, 1 dog underwent successful transcatheter device occlusion of the PDA, and 1 dog had the PDA closed by transcatheter coil delivery 17 months prior to the diagnosis of pulmonary artery dissection. The 2 dogs that did not have the PDA closed died 1 and 3 years after diagnosis due to heart failure. Pulmonary artery dissection is a potential complication of PDA in dogs, the Weimaraner breed may be at increased risk, presentation is often in mature dogs, and closure of the PDA can be performed and appears to improve outcome. Copyright © 2014 Elsevier B.V. All rights reserved.

  2. Evaluation of pulmonary arterial morphology and function in cyanotic congenital heart disease by MRI and cine MRI

    International Nuclear Information System (INIS)

    Hashimoto, Ikuo; Tsubata, Shinichi; Miyazaki, Ayumi; Ichida, Fukiko; Okada, Toshio; Murakami, Arata; Futatsuya, Ryuusuke; Nakajima, Kenshuu; Nakajima, Akio

    1993-01-01

    Pulmonary arterial anatomy was evaluated by magnetic resonance imaging (MRI), angiography and two-dimensional echocardiography in 20 patients with cyanotic heart disease associated with decreased pulmonary blood flow. Excellent correlation between MRI and angiographic estimates of pulmonary artery diameter was obtained (main pulmonary artery, r=0.87; right pulmonary artery, r=0.96; left pulmonary artery, r=0.95). However, echocardiography could not describe peripheral pulmonary arteries obviously, especially left pulmonary artery. In the assessment of peripheral pulmonary stenosis or obstruction, cine MRI was superior to echocardiography. We conclude that MRI and cine MRI will play an important role in the serial evaluation of pulmonary arterial morphology and function in patients with cyanotic congenital heart disease before and after surgical repair. (author)

  3. Evaluation of pulmonary arterial morphology and function in cyanotic congenital heart disease by MRI and cine MRI

    Energy Technology Data Exchange (ETDEWEB)

    Hashimoto, Ikuo; Tsubata, Shinichi; Miyazaki, Ayumi; Ichida, Fukiko; Okada, Toshio; Murakami, Arata; Futatsuya, Ryuusuke; Nakajima, Kenshuu; Nakajima, Akio [Toyama Medical and Pharmaceutical Univ. (Japan)

    1993-01-01

    Pulmonary arterial anatomy was evaluated by magnetic resonance imaging (MRI), angiography and two-dimensional echocardiography in 20 patients with cyanotic heart disease associated with decreased pulmonary blood flow. Excellent correlation between MRI and angiographic estimates of pulmonary artery diameter was obtained (main pulmonary artery, r=0.87; right pulmonary artery, r=0.96; left pulmonary artery, r=0.95). However, echocardiography could not describe peripheral pulmonary arteries obviously, especially left pulmonary artery. In the assessment of peripheral pulmonary stenosis or obstruction, cine MRI was superior to echocardiography. We conclude that MRI and cine MRI will play an important role in the serial evaluation of pulmonary arterial morphology and function in patients with cyanotic congenital heart disease before and after surgical repair. (author).

  4. Aneurysms of proximal pulmonary arteries: CT diagnosis and preoperative assessment

    International Nuclear Information System (INIS)

    Iula, G.; Ziviello, R.; Del Vecchio, W.

    1996-01-01

    We reviewed our experience with proximal pulmonary artery aneurysm (PPAA) to determine whether accurate preoperative evaluation (crucial to differential diagnosis and surgical planning) had been obtained on the basis of CT study alone. Three patients with PPAA were studied with contrast-enhanced CT. We evaluated the size and shape of PPAAs, their proximal and distal extent, eventual presence of intraluminal thrombi, dissection, perianeurysmal fibrosis, and rupture. The results were compared with surgical findings. In two patients the aneurysm involved the pulmonary trunk and both the right and left arteries origin. In one patient the aneurysm extended from the left artery origin to the hilum of the left lung. Contrast-enhanced CT alone allows detection of aneurysm in the pulmonary trunk, in right or left pulmonary arteries with precise preoperative evaluation of the extent, size, shape, and complications. The CT imaging was unable to establish the etiologic origin and presence of small intimal tears in PPAA. (orig.)

  5. Pulmonary hemodynamics and gas exchange in off pump coronary artery bypass grafting.

    Science.gov (United States)

    Vedin, Jenny; Jensen, Ulf; Ericsson, Anders; Samuelsson, Sten; Vaage, Jarle

    2005-10-01

    To investigate the influence of cardiopulmonary bypass on pulmonary hemodynamics and gas exchange. Low risk patients admitted for elective coronary artery bypass grafting were randomized to either on (n=25) or off pump (n=25) surgery. Central hemodynamics, gas exchange, and venous admixture were studied during and up to 20 h after surgery. There was no difference in pulmonary vascular resistance index (P=0.16), right ventricular stroke work index (P>0.2), mean pulmonary artery pressure (P>0.2) or pulmonary capillary wedge pressure (P>0.2) between groups. Soon after surgery there was a tendency towards higher cardiac index (P=0.07) in the off pump group. Arterial oxygen tension (P>0.2), hematocrit (P>0.2), venous admixture (P>0.2), and arterial-venous oxygen content difference (P=0.12) did not differ between groups. This prospective, randomized study showed no difference in pulmonary hemodynamics, pulmonary gas exchange, and venous admixture, in low risk patients undergoing off pump compared to on pump coronary artery bypass surgery.

  6. Electron beam CT diagnosis of congenital unilateral absence of pulmonary artery

    International Nuclear Information System (INIS)

    Zhou Yuan; Dai Ruping; Cao Cheng; Zhang Gejun; Jing Baolian

    2003-01-01

    Objective: To evaluate the clinical value of electron beam CT (EBCT) in diagnosing congenital unilateral absence of pulmonary artery (UAPA). Methods: Patients with clinically suspected pulmonary artery disease or primary pulmonary hypertension underwent EBCT scanning. EBCT confirmed the diagnosis of UAPA in 11 patients, who were also evaluated with echocardiography and chest roentgenography. Cardioangiography and nuclear ventilation-perfusion scan were performed in some patients for a comparative study. Results: 4 female adults had UAPA with out associated congenital anomaly. 3 male children with coexisting complex congenital abnormality had unilateral absence of the left pulmonary artery and 4 patients coexisted other simple cardiovascular anomaly. EBCT scanning simultaneously displayed topographic pattern of both unilateral absence of pulmonary artery and coexisting congenital cardiovascular anomaly, as well as lung diseases. Conclusion: UAPA diagnosed in childhood usually has unilateral absence of the left pulmonary artery and associated congenital cardiovascular anomaly, while UAPA diagnosed in adult usually has UAPA on the right side without associated congenital anomaly. EBCT is one of the optimal imaging techniques in diagnosing UAPA and it greatly increases the diagnostic efficacy than echocardiography dose. Both EBCT and cardioangiography have their own advantages, however, EBCT, as a noninvasive method, should be complementary and not exclusive

  7. Study on blood supply of lung metastasis with trans-pulmonary arterial lipiodol infusion

    International Nuclear Information System (INIS)

    Zhou Jianqin; Dong Weihua; Dong Weihua; Ouyang Chang; Chang Heng; Xiao Xiangsheng

    2008-01-01

    Objective: To evaluate the blood supply of pulmonary metastases using small volume of lipiodol through pulmonary arterial infusion. Methods: 10 cases of lung metastasis were enroled including the primary tumors of liver cancer (n=5), renal carcinoma (n=3), chordoma (n=1) and malignant neurofibroma (n=1). Plain CT scan was performed to exclude calcification or ossification within metastasis and then pulmonary arterial DSA was undertaken to evaluate tumor vessels or staining. After pulmonary arteriovenous fistula or other anomalous circulation was excluded by lobar arterial DSA, small volume of lipiodol was infused under fluoroscopy (0.5-1.5 ml for each lobar artery, total volume less than 3.0 ml). CT scan was immediately performed. Blood supply of the pulmonary metastases was assessed according to the accumulation of lipiodol on CT scans. Results: No cases but one experienced cough, expectoration, suffocating or dyspnea. No complication of cerebral or visceral embolism occurred. Totally 27 nodules were studied including 6 nodules with cloudy lipiodol accumulation and 6 nodules with tiny granules of lipiodol accumulation. No enlarged tumor vessel or tumor stain was observed within all 27 nodules on pulmonary arterial DSA. Conclusions: Pulmonary artery supplys only parts of pulmonary metastases, especially those sited at the peripheral region of the lung. Infusion of small volume of lipiodol through pulmonary artery is safe, and the increased density of lung field could return normal after several days. (authors)

  8. Hypoxia-induced glucose-6-phosphate dehydrogenase overexpression and -activation in pulmonary artery smooth muscle cells: implication in pulmonary hypertension

    Science.gov (United States)

    Chettimada, Sukrutha; Gupte, Rakhee; Rawat, Dhwajbahadur; Gebb, Sarah A.; McMurtry, Ivan F.

    2014-01-01

    Severe pulmonary hypertension is a debilitating disease with an alarmingly low 5-yr life expectancy. Hypoxia, one of the causes of pulmonary hypertension, elicits constriction and remodeling of the pulmonary arteries. We now know that pulmonary arterial remodeling is a consequence of hyperplasia and hypertrophy of pulmonary artery smooth muscle (PASM), endothelial, myofibroblast, and stem cells. However, our knowledge about the mechanisms that cause these cells to proliferate and hypertrophy in response to hypoxic stimuli is still incomplete, and, hence, the treatment for severe pulmonary arterial hypertension is inadequate. Here we demonstrate that the activity and expression of glucose-6-phosphate dehydrogenase (G6PD), the rate-limiting enzyme of the pentose phosphate pathway, are increased in hypoxic PASM cells and in lungs of chronic hypoxic rats. G6PD overexpression and -activation is stimulated by H2O2. Increased G6PD activity contributes to PASM cell proliferation by increasing Sp1 and hypoxia-inducible factor 1α (HIF-1α), which directs the cells to synthesize less contractile (myocardin and SM22α) and more proliferative (cyclin A and phospho-histone H3) proteins. G6PD inhibition with dehydroepiandrosterone increased myocardin expression in remodeled pulmonary arteries of moderate and severe pulmonary hypertensive rats. These observations suggest that altered glucose metabolism and G6PD overactivation play a key role in switching the PASM cells from the contractile to synthetic phenotype by increasing Sp1 and HIF-1α, which suppresses myocardin, a key cofactor that maintains smooth muscle cell in contractile state, and increasing hypoxia-induced PASM cell growth, and hence contribute to pulmonary arterial remodeling and pathogenesis of pulmonary hypertension. PMID:25480333

  9. Selexipag in the treatment of pulmonary arterial hypertension: design, development, and therapy

    Directory of Open Access Journals (Sweden)

    Hardin EA

    2016-11-01

    Full Text Available Elizabeth Ashley Hardin,1 Kelly M Chin2 1Department of Internal Medicine, Division of Cardiology, 2Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA Abstract: Pulmonary arterial hypertension is characterized by abnormalities in the small pulmonary arteries including increased vasoconstriction, vascular remodeling, proliferation of smooth muscle cells, and in situ thrombosis. Selexipag, a novel, oral prostacyclin receptor agonist, has been shown to improve hemodynamics in a phase II clinical trial and reduce clinical worsening in a large phase III clinical trial involving patients with pulmonary arterial hypertension. In this paper, we describe the prostacyclin signaling pathway, currently available oral prostanoid medications, and the development and clinical use of selexipag. Keywords: selexipag, pulmonary arterial hypertension, prostacyclin

  10. Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review

    International Nuclear Information System (INIS)

    Cowles, Robert A.; Berdon, Walter E.

    2007-01-01

    The landmark 1933 case report from Massachusetts General Hospital by Bland, White and Garland (Am Heart J 8:787-801) described a 3-month-old child with progressive feeding problems, cardiomegaly on chest radiography, and EKG evidence of left ventricular damage. Of interest was the fact that the vigilant father of the infant was Aubrey Hampton, a radiologist and future chairman of radiology at Massachusetts General Hospital. At autopsy, the left coronary artery originated from the pulmonary artery rather than from the aorta. Effective treatment for this condition was not available until 1960 when Sabiston, Neill and Taussig showed that the blood flowed from the left coronary artery toward the pulmonary artery. The anomalous left coronary artery was ligated at its junction with the pulmonary artery and the child survived. This historical review of Bland-White-Garland syndrome, now known as anomalous left coronary artery arising from the pulmonary artery (ALCAPA), stresses the continued diagnostic significance of cardiomegaly on chest radiography and EKG changes suggesting left ventricular damage in 2- to 3-month-old infants with feeding intolerance or irritability. With a high index of suspicion, an echocardiogram can be obtained to confirm the diagnosis. Modern surgical methods involve left coronary artery translocation and afford excellent outcomes. (orig.)

  11. Mycotic pulmonary artery aneurysm as an unusual complication of thoracic actinomycosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyung-Soo; Lee, Sang-Yeub [Wonkwang University College, Iksan (Korea, Republic of); Oh, Yu-Whan; Noh, Hyung-Jun; Lee, Ki-Yeol; Kang, Eun-Young [Korea University Hospital and Korea University College, Seoul (Korea, Republic of)

    2004-03-15

    Although pulmonary artery aneurysms are a rare vascular anomaly, they are seen in a wide variety of conditions, such as congenital heart disease, infection, trauma, pulmonary hypertension, cystic medial necrosis and generalized vasculitis. To our knowledge, mycotic aneurysms caused by pulmonary actinomycosis have not been reported in the radiologic literature. Herein, a case of pulmonary actinomycosis complicated by mycotic aneurysm is presented. On CT scans, this case showed focal aneurysmal dilatation of a peripheral pulmonary artery within necrotizing pneumonia of the right lower lobe, which was successfully treated with transcatheter embolization using wire coils.

  12. Mycotic pulmonary artery aneurysm as an unusual complication of thoracic actinomycosis

    International Nuclear Information System (INIS)

    Kim, Hyung-Soo; Lee, Sang-Yeub; Oh, Yu-Whan; Noh, Hyung-Jun; Lee, Ki-Yeol; Kang, Eun-Young

    2004-01-01

    Although pulmonary artery aneurysms are a rare vascular anomaly, they are seen in a wide variety of conditions, such as congenital heart disease, infection, trauma, pulmonary hypertension, cystic medial necrosis and generalized vasculitis. To our knowledge, mycotic aneurysms caused by pulmonary actinomycosis have not been reported in the radiologic literature. Herein, a case of pulmonary actinomycosis complicated by mycotic aneurysm is presented. On CT scans, this case showed focal aneurysmal dilatation of a peripheral pulmonary artery within necrotizing pneumonia of the right lower lobe, which was successfully treated with transcatheter embolization using wire coils

  13. Detection of patent ductus arteriosus with intraoperative transesophageal echocardiography in a patient undergoing closure of coronary artery to pulmonary artery fistula.

    Science.gov (United States)

    Miyata, Yuka; Hayashi, Yukio

    2017-01-01

    Coronary artery to pulmonary artery fistula is an unusual vascular anomaly, and the shunt ratio of this fistula is usually small. We report anesthetic management of a 55-year-old female with annuloaortic ectasia, aortic valve regurgitation, and coronary artery to pulmonary artery fistula undergoing radical repair. We calculated the left-to-right shunt ratio after placement of a pulmonary artery catheter and found that the ratio was unexpectedly high. Thus, we explored the presence of another shunt by intraoperative transesophageal echocardiography and found patent ductus arteriosus undiagnosed before operation. A combination of a pulmonary artery catheter and transesophageal echocardiography is useful to explore the presence of another shunt, such as patent ductus arteriosus during anesthesia.

  14. Peripheral pulmonary arteries: identification at multi-slice spiral CT with 3D reconstruction

    International Nuclear Information System (INIS)

    Coche, Emmanuel; Pawlak, Sebastien; Dechambre, Stephane; Maldague, Baudouin

    2003-01-01

    Our objective was to analyze the peripheral pulmonary arteries using thin-collimation multi-slice spiral CT. Twenty consecutive patients underwent enhanced-spiral multi-slice CT using 1-mm collimation. Two observers analyzed the pulmonary arteries by consensus on a workstation. Each artery was identified on axial and 3D shaded-surface display reconstruction images. Each subsegmental artery was measured at a mediastinal window setting and compared with anatomical classifications. The location and branching of every subsegmental artery was recorded. The number of well-visualized sub-subsegmental arteries at a mediastinal window setting was compared with those visualized at a lung window setting. Of 800 subsegmental arteries, 769 (96%) were correctly visualized and 123 accessory subsegmental arteries were identified using the mediastinal window setting. One thousand ninety-two of 2019 sub-subsegmental arteries (54%) identified using the lung window setting were correctly visualized using the mediastinal window setting. Enhanced multi-slice spiral CT with thin collimation can be used to analyze precisely the subsegmental pulmonary arteries and may identify even more distal pulmonary arteries. (orig.)

  15. Danshensu prevents hypoxic pulmonary hypertension in rats by inhibiting the proliferation of pulmonary artery smooth muscle cells via TGF-β-smad3-associated pathway.

    Science.gov (United States)

    Zhang, Ning; Dong, Mingqing; Luo, Ying; Zhao, Feng; Li, Yongjun

    2018-02-05

    Hypoxic pulmonary hypertension is characterized by the remodeling of pulmonary artery. Previously we showed that tanshinone IIA, one lipid-soluble component from the Chinese herb Danshen, ameliorated hypoxic pulmonary hypertension by inhibiting pulmonary artery remodeling. Here we explored the effects of danshensu, one water-soluble component of Danshen, on hypoxic pulmonary hypertension and its mechanism. Rats were exposed to hypobaric hypoxia for 4 weeks to develop hypoxic pulmonary hypertension along with administration of danshensu. Hemodynamics and pulmonary arterial remodeling index were measured. The effects of danshensu on the proliferation of primary pulmonary artery smooth muscle cells and transforming growth factor-β-smad3 pathway were assessed in vitro. Danshensu significantly decreased the right ventricle systolic pressure, the right ventricle hypertrophy and pulmonary vascular remodeling index in hypoxic pulmonary hypertension rats. Danshensu also reduced the increased expression of transforming growth factor-β and phosphorylation of smad3 in pulmonary arteries in hypoxic pulmonary hypertension rats. In vitro, danshensu inhibited the hypoxia- or transforming growth factor-β-induced proliferation of primary pulmonary artery smooth muscle cells. Moreover, danshensu decreased the hypoxia-induced expression and secretion of transforming growth factor in primary pulmonary adventitial fibroblasts and NR8383 cell line, inhibited the hypoxia or transforming growth factor-β-induced phosphorylation of smad3 in rat primary pulmonary artery smooth muscle cells. These results demonstrate that danshensu ameliorates hypoxic pulmonary hypertension in rats by inhibiting the hypoxia-induced proliferation of pulmonary artery smooth muscle cells, and the inhibition effects is associated with transforming growth factor-β-smad3 pathway. Therefore danshensu may be a potential treatment for hypoxic pulmonary hypertension. Copyright © 2017 Elsevier B.V. All rights

  16. Computerized axial tomography of the chest for visualization of ''absent'' pulmonary arteries

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    Sondheimer, H.M. (Upstate Medical Center, Syracuse, NY); Oliphant, M.; Schneider, B.; Kavey, R.E.W.; Blackman, M.S.; Parker, F.B. Jr.

    1982-05-01

    To expand the search for central pulmonary arteries in six patients with absence of cardiac-pulmonary continuity, computerized axial tomography (CAT) of the chest was performed. The CAT scans were compared with previous arteriograms and pulmonary vein wedge angiograms. Three patients with type IV truncus arteriosus were studied, and none had a central, right or left pulmonary artery on CAT scan. However, two patients with tetralogy of Fallot with pulmonary atresia and a patent ductus arteriosus to the right lung demonstrated the presence of a left pulmonary artery. In addition, one child with truncus arteriosus with ''absent'' left pulmonary artery demonstrated a left pulmonary artery on the CAT scan. The CAT scan may therefore enhance our ability to search for disconnected pulmonary arteries in children with complex cyanotic congenital heart disease.

  17. Primary extraskeletal myxoid chondrosarcoma of pulmonary arteries: a rare mimic of acute pulmonary thromboembolism.

    Science.gov (United States)

    Gadabanahalli, Karthik; Belaval, Vinay V; Bhat, Venkatraman; Gorur, Imran M

    2015-04-01

    Primary extraskeletal myxoid chondrosarcoma of the pulmonary arteries is a very rare entity. Multimodality imaging reports on this entity are few. Myxoid chondrosarcoma is characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas, and are most commonly found in the lower extremities, limb girdles, distal extremities and trunk. We report an unusual case of a 31-year old man with histopathologically proven extraskeletal myxoid chondrosarcoma of the pulmonary arteries mimicking acute pulmonary thromboembolism. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  18. Bovine aortic arch and idiopathic pulmonary artery aneurysm associated with bronchial compression

    Directory of Open Access Journals (Sweden)

    Süleyman Sezai Yıldız

    2015-09-01

    Full Text Available The left common carotid artery originating from the brachiocephalic trunk is termed the bovine aortic arch. Although it is the third most-common normal variant found in 9% humans, the origin of this term remains unclear. Until now, It has not been reported in the literature bovine aortic arch togetherness with pulmonary aneurysm and bronchial compression. Herein, we present a case with bovine aorta arch and pulmonary artery aneurysm associated with bronchial compression, which is incidentally detected by X-ray film. A 56-year-old Caucasian female admitted to the cardiology clinic with complaint of chest pain. Physical examination was unremarkable. Blood biochemistry values and cardiac markers were in normal range. Chest radiography revealed a widened mediastinum and prominent pulmonary conus with no active pulmonary disease. A subsequent transthoracic echocardiography revealed left ventricular hypertrophy, left atrial enlargement (diameter: 41 mm, mild mitral and tricuspid valve insufficiency, dilatation of main pulmonary artery (parasternal short-axis view diameter: 33 mm, normal pulmonary artery pressure and normal left ventricular systolic function. Computed tomography revealed bovine aortic arch associated with pulmonary artery aneurysm (diameter: 53 mm. And left main bronch of trachea was critically squeezed by aortic arch. Aortic and pulmonary vascular anomalies should be considered in patients with chest pain. And, identification with imaging modalities is important for prevention of chronic and irreversible complications.

  19. Fibrosing Mediastinitis: Successful Stenting of the Pulmonary Artery

    Directory of Open Access Journals (Sweden)

    Rennae Thiessen

    2008-01-01

    Full Text Available Fibrosing mediastinitis is a rare benign condition, which can cause compression of the pulmonary or systemic vessels, tracheobronchial tree, coronary arteries or esophagus, leading to disabling clinical symptoms and even death. The case of a 26-year-old woman who presented with dyspnea is described. She was found to have 80% stenosis of the right pulmonary artery secondary to fibrosing mediastinitis. The stenosis was managed successfully with an endovascular Palmaz-Schatz stent, and the patient remains symptom-free 10 years later.

  20. Aortic dissection presenting with secondary pulmonary hypertension caused by compression of the pulmonary artery by dissecting hematoma: a case report

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Ryu, Sang Wan; Choi, Yong Sun; Ahn, Byoung Hee

    2004-01-01

    The rupture of an acute dissection of the ascending aorta into the space surrounding the pulmonary artery is an uncommon occurrence. No previous cases of transient pulmonary hypertension caused by a hematoma surrounding the pulmonary artery have been documented in the literature. Herein, we report a case of acute aortic dissection presenting as secondary pulmonary hypertension

  1. Contribution of reactive oxygen species to the pathogenesis of pulmonary arterial hypertension

    Science.gov (United States)

    Naik, Jay S.; Weise-Cross, Laura; Detweiler, Neil D.; Herbert, Lindsay M.; Yellowhair, Tracylyn R.; Resta, Thomas C.

    2017-01-01

    Pulmonary arterial hypertension is associated with a decreased antioxidant capacity. However, neither the contribution of reactive oxygen species to pulmonary vasoconstrictor sensitivity, nor the therapeutic efficacy of antioxidant strategies in this setting are known. We hypothesized that reactive oxygen species play a central role in mediating both vasoconstrictor and arterial remodeling components of severe pulmonary arterial hypertension. We examined the effect of the chemical antioxidant, TEMPOL, on right ventricular systolic pressure, vascular remodeling, and enhanced vasoconstrictor reactivity in both chronic hypoxia and hypoxia/SU5416 rat models of pulmonary hypertension. SU5416 is a vascular endothelial growth factor receptor antagonist and the combination of chronic hypoxia/SU5416 produces a model of severe pulmonary arterial hypertension with vascular plexiform lesions/fibrosis that is not present with chronic hypoxia alone. The major findings from this study are: 1) compared to hypoxia alone, hypoxia/SU5416 exposure caused more severe pulmonary hypertension, right ventricular hypertrophy, adventitial lesion formation, and greater vasoconstrictor sensitivity through a superoxide and Rho kinase-dependent Ca2+ sensitization mechanism. 2) Chronic hypoxia increased medial muscularization and superoxide levels, however there was no effect of SU5416 to augment these responses. 3) Treatment with TEMPOL decreased right ventricular systolic pressure in both hypoxia and hypoxia/SU5416 groups. 4) This effect of TEMPOL was associated with normalization of vasoconstrictor responses, but not arterial remodeling. Rather, medial hypertrophy and adventitial fibrotic lesion formation were more pronounced following chronic TEMPOL treatment in hypoxia/SU5416 rats. Our findings support a major role for reactive oxygen species in mediating enhanced vasoconstrictor reactivity and pulmonary hypertension in both chronic hypoxia and hypoxia/SU5416 rat models, despite a

  2. Preceding bronchial cutting for exposure of the pulmonary artery buried in scar tissue after chemoradiotherapy.

    Science.gov (United States)

    Nomori, Hiroaki; Cong, Yue; Sugimura, Hiroshi

    2017-01-01

    It is often difficult to expose the pulmonary artery buried in a scar tissue, especially in lung cancer patients that responded well to neoadjuvant chemoradiotherapy. Difficulty to access pulmonary artery branches may lead to potentially unnecessary pneumonectomy. To complete lobectomy in such cases, a technique with preceding bronchial cutting for exposure of the pulmonary artery is presented. After dissecting the pulmonary vein, the lobar bronchus is cut from the opposite side of the pulmonary artery with scissors. The back wall of the lobar bronchus is cut using a surgical knife from the luminal face, which can expose the pulmonary artery behind the bronchial stump and then complete lobectomy. Fourteen patients have been treated using the present technique, enabling complete resection by lobectomy (including sleeve lobectomy in 3 patients) without major bleeding. The present procedure can expose pulmonary artery buried in scar tissue, resulting in making the lobectomy safer.

  3. Method for imaging pulmonary arterial hypoplasia

    International Nuclear Information System (INIS)

    Triantafillou, M.

    2000-01-01

    Full text: Pulmonary hypoplasia represents an incomplete development of the lung, resulting in the reduction of distended lung volume. This is associated with small or absent number of airway divisions, alveoli, arteries and veins. Unilateral pulmonary Hypoplasia is often asymptomatic and may be demonstrated as a hypodense lung on a chest X-ray. Computer Tomography (CT) scanning would show anatomical detail and proximal vessels. Magnetic Resonance Imaging (MRI) will show no more detail than which the CT scan has already demonstrated. It is, also, difficult to visualise collateral vessels from systemic and/or bronchial vessels on both these modalities. Pulmonary Angiography would give the definitive answer, but it is time consuming and has significant risks associated with the procedure. There are high costs associated with these modalities. Nuclear Medicine Ventilation/Perfusion (V/Q) scan performed on these patients would demonstrate diminished ventilation due to reduced lung volume and absence of perfusion to the hypoplastic lung. To date, we have performed V/Q lung scan on two children in our department. Both cases demonstrate diminished ventilation with no perfusion to the hypoplastic lung. Though the gold standard is Pulmonary Angiography, V/Q scanning is cost effective, less time consuming and a non invasive procedure that can be performed as an outpatient. It is accurate as it demonstrates absent lung perfusion, confirming the patient has pulmonary arterial hypoplasia. Copyright (2000) The Australian and New Zealand Society of Nuclear Medicine Inc

  4. Right ventricle performances with echocardiography and 99mTc myocardial perfusion imaging in pulmonary arterial hypertension patients.

    Science.gov (United States)

    Liu, Jie; Fei, Lei; Huang, Guang-Qing; Shang, Xiao-Ke; Liu, Mei; Pei, Zhi-Jun; Zhang, Yong-Xue

    2018-05-01

    Right heart catheterization is commonly used to measure right ventricle hemodynamic parameters and is the gold standard for pulmonary arterial hypertension diagnosis; however, it is not suitable for patients' long-term follow-up. Non-invasive echocardiography and nuclear medicine have been applied to measure right ventricle anatomy and function, but the guidelines for the usefulness of clinical parameters remain to be established. The goal of this study is to identify reliable clinical parameters of right ventricle function in pulmonary arterial hypertension patients and analyze the relationship of these clinical parameters with the disease severity of pulmonary arterial hypertension. In this study, 23 normal subjects and 23 pulmonary arterial hypertension patients were recruited from January 2015 to March 2016. Pulmonary arterial hypertension patients were classified into moderate and severe pulmonary arterial hypertension groups according to their mean pulmonary arterial pressure levels. All the subjects were subjected to physical examination, chest X-ray, 12-lead electrocardiogram, right heart catheterization, two-dimensional echocardiography, and technetium 99m ( 99m Tc) myocardial perfusion imaging. Compared to normal subjects, the right heart catheterization indexes including right ventricle systolic pressure, right ventricle end diastolic pressure, pulmonary artery systolic pressure, pulmonary artery diastolic pressure, pulmonary vascular resistance, and right ventricle end systolic pressure increased in pulmonary arterial hypertension patients and were correlated with mean pulmonary arterial pressure levels. Echocardiography parameters, including tricuspid regurgitation peak velocity, tricuspid regurgitation pressure gradient, tricuspid annular plane systolic excursion and fractional area, right ventricle-myocardial performance index, were significantly associated with the mean pulmonary arterial pressure levels in pulmonary arterial hypertension patients

  5. Autologous Transfusion of Stored Red Blood Cells Increases Pulmonary Artery Pressure

    Science.gov (United States)

    Pinciroli, Riccardo; Stowell, Christopher P.; Wang, Lin; Yu, Binglan; Fernandez, Bernadette O.; Feelisch, Martin; Mietto, Cristina; Hod, Eldad A.; Chipman, Daniel; Scherrer-Crosbie, Marielle; Bloch, Kenneth D.; Zapol, Warren M.

    2014-01-01

    Rationale: Transfusion of erythrocytes stored for prolonged periods is associated with increased mortality. Erythrocytes undergo hemolysis during storage and after transfusion. Plasma hemoglobin scavenges endogenous nitric oxide leading to systemic and pulmonary vasoconstriction. Objectives: We hypothesized that transfusion of autologous blood stored for 40 days would increase the pulmonary artery pressure in volunteers with endothelial dysfunction (impaired endothelial production of nitric oxide). We also tested whether breathing nitric oxide before and during transfusion could prevent the increase of pulmonary artery pressure. Methods: Fourteen obese adults with endothelial dysfunction were enrolled in a randomized crossover study of transfusing autologous, leukoreduced blood stored for either 3 or 40 days. Volunteers were transfused with 3-day blood, 40-day blood, and 40-day blood while breathing 80 ppm nitric oxide. Measurements and Main Results: The age of volunteers was 41 ± 4 years (mean ± SEM), and their body mass index was 33.4 ± 1.3 kg/m2. Plasma hemoglobin concentrations increased after transfusion with 40-day and 40-day plus nitric oxide blood but not after transfusing 3-day blood. Mean pulmonary artery pressure, estimated by transthoracic echocardiography, increased after transfusing 40-day blood (18 ± 2 to 23 ± 2 mm Hg; P transfusing 3-day blood (17 ± 2 to 18 ± 2 mm Hg; P = 0.5). Breathing nitric oxide decreased pulmonary artery pressure in volunteers transfused with 40-day blood (17 ± 2 to 12 ± 1 mm Hg; P Transfusion of autologous leukoreduced blood stored for 40 days was associated with increased plasma hemoglobin levels and increased pulmonary artery pressure. Breathing nitric oxide prevents the increase of pulmonary artery pressure produced by transfusing stored blood. Clinical trial registered with www.clinicaltrials.gov (NCT 01529502). PMID:25162920

  6. Pediatric pulmonary arterial hypertension : Towards optimal classification, treatment strategies and outcome

    NARCIS (Netherlands)

    Zijlstra, Willemijn

    2017-01-01

    Pulmonary arterial hypertension (PAH) is a rare, progressive disease of the small pulmonary arteries and has a poor prognosis. Median survival of children with PAH is <3 years if untreated. The development of PAH-targeted drugs and the introduction of evidence-based treatment guidelines have greatly

  7. Remodeling of the pulmonary artery induced by metastatic gastric carcinoma: a histopathological analysis of 51 autopsy cases

    International Nuclear Information System (INIS)

    Ishiwatari, Takao; Yamamoto, Yoshiro; Nakayama, Haruo; Shibuya, Kazutoshi; Okubo, Yoichiro; Tochigi, Naobumi; Wakayama, Megumi; Nemoto, Tetsuo; Kobayashi, Junko; Shinozaki, Minoru; Aki, Kyoko; Sasai, Daisuke

    2014-01-01

    Gastric carcinoma remains the second commonest cause of cancer deaths worldwide. Presence of the carcinoma cell in the pulmonary artery is serious condition that might cause remodeling of the pulmonary artery. The present study conducted detailed histopathological analyses to elucidate how gastric carcinoma cells may affect the structure and hemodynamics of pulmonary arteries. Remodeling of the pulmonary artery was assessed based on measurements of arterial diameters and stenosis rates from the autopsies, and their correlation were also validated. We additionally calculated 95 percent confidential intervals (CIs) for the rate of stenosis in groups of pulmonary arteries of different caliber zones (under 100, 100 to 300, and over 300 micrometer). The right ventricular thickness was measured and examined whether it correlated with the rate of pulmonary arterial stenosis. A total of 4612 autopsy cases were recorded at our institute, among which 168 had gastric carcinoma. Finally, 51 cases of the gastric carcinoma were employed for the study which had carcinoma cells in the lumen of the pulmonary artery. The mean right ventricular wall thickness of these cases was 3.14 mm. There were significant positive associations between the rates of pulmonary arterial stenosis and right ventricular thickness from pulmonary arteries of diameter under 100, 100 to 300, and over 300 micrometer. In these zones, 31, 31, and 33 cases had rates of pulmonary arterial stenosis that were below the lower limit of the 95 percent CI values, respectively. On the other hand, among cases with significant pulmonary stenosis, 17 of 18 cases with stenosis in the over 300 micrometer zone involved pulmonary arteries of both in the under 100 and 100 to 300 micrometer zones. One-third of autopsy with advanced gastric carcinoma had carcinoma cells in lumen of pulmonary artery, but implantation and proliferation may be essential to induce intimal thickening that causes an increasing of pulmonary arterial

  8. Coil embolization of mycotic pulmonary artery aneurysm: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jeen Woo; Lee, Jae Kyo; Hwang, Mi Soo; Cho, Kil Ho [College of Medicine, Yeungnam University, Taegu (Korea, Republic of)

    2000-02-01

    Aneurysms of the pulmonary artery are rare, with mycotic aneurysms occurring most frequently. This latter type may also occur in association with a lung abscess or septicemia, particularly in drug addicts. As far as we are aware, the radiologic findings of mycotic aneurysm of the pulmanary artery have not been reported in Korea. We present the simple chest radiographs, as well as the CT and angiographic findings, of a case of aneurysm of the pulmonary artery which was successfully embolized using a coil. (author)

  9. Coil embolization of mycotic pulmonary artery aneurysm: a case report

    International Nuclear Information System (INIS)

    Kim, Jeen Woo; Lee, Jae Kyo; Hwang, Mi Soo; Cho, Kil Ho

    2000-01-01

    Aneurysms of the pulmonary artery are rare, with mycotic aneurysms occurring most frequently. This latter type may also occur in association with a lung abscess or septicemia, particularly in drug addicts. As far as we are aware, the radiologic findings of mycotic aneurysm of the pulmanary artery have not been reported in Korea. We present the simple chest radiographs, as well as the CT and angiographic findings, of a case of aneurysm of the pulmonary artery which was successfully embolized using a coil. (author)

  10. An unusual neonatal presentation of anomalous origin of the left coronary artery arising from the pulmonary artery.

    Science.gov (United States)

    Garty, Y; Guri, A; Shinwell, E S; Matitiau, A

    2008-01-01

    We describe a previously unreported neonatal presentation of an anomalous origin of the left coronary artery arising from the pulmonary artery. This is a full-term female infant after normal pregnancy and delivery. The baby was diagnosed at 2 days of age due to weak femoral pulses noted on the routine nursery discharge examination. The cardiac examination revealed weak pulses everywhere and mild tachypnea and tachycardia. An electrocardiogram showed clear signs of ischemia. Echocardiography demonstrated an anomalous origin of the left coronary artery arising from the pulmonary artery with bidirectional blood flow. There was a severely depressed left ventricular function and mild mitral valve regurgitation. At 4 days of age, the infant underwent complete successful surgical repair with reimplantation of the anomalous coronary artery to the aorta. She recovered slowly but well. Fifteen months later she is doing well with no cardiac residua. A neonatal presentation is very unusual due to protective high pulmonary resistance after birth, with gradual decline in pressure and gradual onset of heart failure. This case may be related to an unusually rapid drop in pulmonary vascular resistance causing very early cardiac ischemia. (c) 2007 S. Karger AG, Basel.

  11. Total Pulmonary Artery Atresia Associated with Abnormal Pulmonary Venous Drainage as a Rare Presentation of Scimitar Syndrome

    Directory of Open Access Journals (Sweden)

    Reza Javadrashid

    2013-09-01

    Full Text Available Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex, and variable malformation of the right lung characterized by an abnormal right sided pulmonary drainage into the inferior vena cava, malformation of the right lung, abnormal arterial supply, and sometimes cardiac malformation. Despite the varying degrees of pulmonary hypoplasia and pulmonary artery hypertension, about half of the patients with scimitar syndrome are asymptomatic or mildly symptomatic when the diagnosis is made. Neonates have severe symptoms and worse prognosis while older children come to light because of recurrent respiratory infections, heart murmur, or an abnormal chest radiograph.

  12. A case of William's syndrome associated peripheral pulmonary arterial stenosis

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    Jung, Kyung Hwa; Hwang, Mi Soo; Kim, Sun Yong; Chang, Jae Chun; Park, Bok Hwan [College of Medicine, Yeungam University, Daegu (Korea, Republic of)

    1988-06-15

    William's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that 'infantile hypercalcemia', 'peculiar facies' and 'supravalvular aortic stenosis.' In has other many vascular anomalies, such as peripheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis, and renal aterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it is very rare disease entity that has been reported rarely in Korea. Recently authors experienced a case that was questioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminary radiologically and this case was confirmed by operation. Here we report a case of William's syndrome with peripheral pulmonary arterial stenosis and reviewed literatures.

  13. Pulmonary arterial stent implantation in an adult with Williams syndrome

    NARCIS (Netherlands)

    Reesink, Herre J.; Henneman, Onno D. F.; van Delden, Otto M.; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

    2007-01-01

    We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent

  14. Percutaneous Stent-Graft Repair of a Mycotic Pulmonary Artery Pseudoaneurysm

    International Nuclear Information System (INIS)

    Chou Meichun; Liang Hueilung; Pan Huayban; Yang Chienfang

    2006-01-01

    Ruptured mycotic pulmonary pseudoaneurysm is a lethal complication. Emergent surgical repair is usually recommended, but still associated with a high mortality rate. We present a patient in whom mycotic pulmonary pseudoaneurysm was a complication after surgical lobectomy 2 weeks earlier. This patient had suffered from repeated massive hemoptysis. After emergent surgical repair of the ruptured pulmonary artery stump, another episode of massive hemorrhage occurred. The pulmonary arteriogram revealed a segmental stenosis and a large, wide-necked, lobulated pseudoaneurysm at the left proximal pulmonary artery. We deployed a balloon-expandable stent-graft (48 mm in length mounted on a 12 mm x 40 mm angioplasty balloon) across the stenotic segment and the neck of the pulmonary pseudoaneurysm. Hemostasis was achieved immediately and, under a 4-week antibiotic treatment, patient was transferred to a local hospital for medical care. This case report demonstrates the benefit of minimally invasive endovascular therapy in a critically ill patient. A literature review of the etiology and management of mycotic pulmonary pseudoaneurysm is included

  15. A CASE OF IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION IN MALE

    OpenAIRE

    Poongavanam Paranthaman; Ramani Bala Subra Manian; Thenrajan Balaji; Jayakrishnan Jayakumar; Govindaraj Ranjani

    2016-01-01

    Primary Pulmonary Hypertension is a rare disease occurring in 1-2 per million population. It is 2-4 times more common in female. Idiopathic or primary pulmonary hypertension is defined as a disorder with no identifiable cause in which resting mean pulmonary artery pressure in adults is above 25 mmHg and 30 mmHg with exercise. Idiopathic or primary pulmonary hypertension is diagnosed after ruling out all the possible secondary causes of pulmonary hypertension. We are presenting a ...

  16. Response of pulmonary artery intimal sarcoma to surgery, radiotherapy and chemotherapy: a case report

    Directory of Open Access Journals (Sweden)

    Long Hong-qing

    2008-06-01

    Full Text Available Abstract Introduction Pulmonary artery intimal sarcoma is a rare disease with no characteristic symptoms. It is difficult to diagnose early and is frequently misdiagnosed as a pulmonary embolism. Case presentation Here we report a case of pulmonary artery intimal sarcoma in a 54-year-old woman presenting with complaints of shortness of breath on exertion. Echocardiography and a computed tomography scan showed that the right pulmonary artery trunk was blocked by a low-density mass. The patient was diagnosed with pulmonary artery intimal sarcoma by pathology and a complete mass resection was performed. After experiencing 10 months of disease-free survival, she was re-admitted because of the recurrence and metastasis of the tumor. Radiotherapy and chemotherapy were performed; however, only limited success was achieved. The patient died 15 months after the initial onset of symptoms. Conclusion Some patients with intimal sarcoma of the pulmonary artery can benefit from radiotherapy and chemotherapy as well as surgery.

  17. The pulmonary artery does not participate in the blood supply of lung cancer: experimental and DSA study

    International Nuclear Information System (INIS)

    Han Mingjun; Feng Gansheng; Yang Jianyong; Su Hongying; Zhao Zhongchun

    2000-01-01

    Objective: To investigate whether or not the pulmonary artery participates in the blood supply of lung cancer and its change of morphology and blood flow in lung cancer. Methods: Two different colors of silicone were injected separately into the bronchial and pulmonary arteries of 33 rat models with squamous cell carcinoma of lung. The origin of blood supply of lung cancer and the morphologic change of pulmonary artery were observed under a stereo-microscope. The DSA of bronchial and pulmonary artery were performed simultaneously in 28 patients with lung cancer. Results: The pulmonary branch of rat and patients were reduced,thinned and occluded in the affected lung. The pulmonary artery did not form tumor vessel,and pulmonary blood flow and perfusion were reduced or absent in the affected area. Conclusion: The pulmonary artery did not participate in the blood supply of lung cancer. It is unreasonable to perform transcatheter chemo-embolization for lung cancer via pulmonary artery

  18. DSA - a helpful tool in diagnosis of aberrant left pulmonary artery (vascular sling) in adults

    International Nuclear Information System (INIS)

    Mooyaart, E.L.; Boomsma, J.H.B.; Postmus, P.E.; Formanek, G.A.

    1985-01-01

    Two new adult patients with aberrant origin of the left pulmonary artery from the right pulmonary artery - pulmonary artery sling - are described, totalling the published adult cases to eight. Differentiation from a mediastinal mass closely mimicking this vascular anomaly is discussed. For the definitive diagnosis, digital subtraction angiography was applied for the first time. The clearest demonstration of the anatomy is in 20-25 0 RPO and 20-25 0 sitting position. The aberrant left pulmonary artery in adults is asymptomatic.

  19. Revisiting the Role of TRP, Orai, and ASIC Channels in the Pulmonary Arterial Response to Hypoxia

    Directory of Open Access Journals (Sweden)

    Roberto V. Reyes

    2018-05-01

    Full Text Available The pulmonary arteries are exquisitely responsive to oxygen changes. They rapidly and proportionally contract as arterial PO2 decrease, and they relax as arterial PO2 is re-established. The hypoxic pulmonary vasoconstriction (HPV is intrinsic since it does not require neural or endocrine factors, as evidenced in isolated vessels. On the other hand, pulmonary arteries also respond to sustained hypoxia with structural and functional remodeling, involving growth of smooth muscle medial layer and later recruitment of adventitial fibroblasts, secreted mitogens from endothelium and changes in the response to vasoconstrictor and vasodilator stimuli. Hypoxic pulmonary arterial vasoconstriction and remodeling are relevant biological responses both under physiological and pathological conditions, to explain matching between ventilation and perfusion, fetal to neonatal transition of pulmonary circulation and pulmonary artery over-constriction and thickening in pulmonary hypertension. Store operated channels (SOC and receptor operated channels (ROC are plasma membrane cationic channels that mediate calcium influx in response to depletion of internal calcium stores or receptor activation, respectively. They are involved in both HPV and pathological remodeling since their pharmacological blockade or genetic suppression of several of the Stim, Orai, TRP, or ASIC proteins in SOC or ROC complexes attenuate the calcium increase, the tension development, the pulmonary artery smooth muscle proliferation, and pulmonary arterial hypertension. In this Mini Review, we discussed the evidence obtained in in vivo animal models, at the level of isolated organ or cells of pulmonary arteries, and we identified and discussed the questions for future research needed to validate these signaling complexes as targets against pulmonary hypertension.

  20. A mycotic pulmonary artery aneurysm associated with candida endocarditis: Case report

    International Nuclear Information System (INIS)

    Moon, Jin Il; Lee, Ji Won; Jeong, Yeon Joo; Song, Seung Hwan

    2014-01-01

    We report a case of a mycotic pulmonary aneurysm associated with Candida endocarditis in a 53-year-old male with lymphoma. The initial diagnosis was a pulmonary artery aneurysm attributable to vasculitis, such as that associated with Behcet's disease, but a mycotic pulmonary artery aneurysm was later considered as a differential diagnosis. Identification of valve vegetation on the chest CT was helpful in this regard. We review the literature on the disease etiology, radiological findings, and management options.

  1. Late Posthemorrhagic Structural and Functional Changes in Pulmonary Circulation Arteries

    Directory of Open Access Journals (Sweden)

    S. A. Andreyeva

    2008-01-01

    Full Text Available Objective: to reveal the major regularities and mechanisms of morphological changes in the rat pulmonary circulation arteries in the late posthemorrhagic period and to compare them with age-related features of the vessels. Materials and methods: experiments to generate graduated hemorrhagic hypotension with the blood pressure being maintained at 40 mm Hg were carried out on young (5—6-month albino male Wistar rats. Throughout hypotension and 60 days after blood loss, the blood was tested to determine low and average molecular-weight substances by spectrophotometry and the pro- and antioxidative systems by chemiluminescence. Pulmonary circulation arteries were morphologically studied in young animals, rats in the late posthemorrhagic period and old (24—25-month rats. Results. Sixty-minute hemorrhagic hypotension leads to the development of endotoxemia and imbalance of the pro- and antioxidative systems, the signs of which are observed in the late periods (2 months after hypotension. At the same time, the posthemorrhagic period is marked by the significant pulmonary circulation arterial morphological changes comparable with their age-related alterations in old rat. This shows up mainly in the reorganization of a connective tissue component in the vascular wall: the elevated levels of individual collagen fibers, their structural changes, elastic medial membrane destruction and deformity. At the same time, there is a change in the morphometric parameters of vessels at all study stages while their lowered flow capacity is only characteristic for intraorgan arteries. Conclusion: The increased activity of free radical oxidation and endotoxemia may be believed to be one of the causes of morphological changes in pulmonary circulation arteries in the late posthemorrhagic period, which is similar to age-related vascular alterations. Key words: hemorrhagic hypotension, pulmonary circulation arteries, free radical oxidation, endotoxemia, remodeling, late

  2. Apical Hypertrophic Cardiomyopathy in Association with PulmonaryArtery Hypertension

    Directory of Open Access Journals (Sweden)

    Mehdi Peighambari

    2012-09-01

    Full Text Available Apical Hypertrophic Cardiomyopathy is an uncommon condition constituting 1% -2% of the cases with Hypertrophic Cardiomyopathy (HCM diagnosis. We interestingly report two patients with apical hypertrophic cardiomyopathy in association with significant pulmonary artery hypertension without any other underlying reason for pulmonary hypertension. The patients were assessed by echocardiography, cardiac catheterization and pulmonary function parameters study.

  3. Peroxynitrite-induced relaxation in isolated canine cerebral arteries and mechanisms of action

    International Nuclear Information System (INIS)

    Li Jianfeng; Li Wenyan; Altura, Bella T.; Altura, Burton M.

    2004-01-01

    The present study was undertaken to determine the vascular actions of peroxynitrite (ONOO - ), the product of superoxide and nitric oxide (NO), in isolated canine cerebral arteries and to gain insight into its potential mechanisms of action. In the absence of any vasoactive agent, ONOO - (from 10 -7 to 10 -6 M) was able to reduce the basal tension. In prostaglandin F2α-precontracted canine basilar arterial rings, ONOO - elicited concentration-dependent relaxation at concentrations from 10 -8 to 10 -5 M. The effective concentrations producing approximately 50% maximal relaxation (EC 50 ) to ONOO - were 4.06 x 10 -6 and 4.12 x 10 -6 M in intact and denuded rings, respectively (P > 0.05). No significant differences in relaxation responses were found in ring preparations with or without endothelium (P > 0.05). The presence of either 5 μM methylene blue (MB) or 5 μM 1H-[1,2,4]oxadiazolo-[4,3-α]quinoxalin-1-one (ODQ) significantly inhibited the relaxations induced by ONOO - . Tetraethylammonium chloride (T-2265) significantly decreased the ONOO - -induced relaxations in a concentration-dependent manner. However, ONOO - had no effect on rings precontracted by high KCL (P > 0.05). Addition of low concentrations of calyculin A (50 nM) was able to abolish the ONOO - -induced relaxation. Furthermore, ONOO - significantly inhibited calcium-induced contractions of K + -depolarized canine cerebral rings in a concentration-related manner. Lastly, a variety of pharmacological agents and antagonists including L-NMMA, L-arginine, indomethacin, atropine, naloxone, diphenhydramine, cimetine, glibenclamide, haloperidol, etc., did not influence the relaxant effects of ONOO - on the rings. Our new results suggest that ONOO - -triggered relaxation, on canine cerebral arteries, is mediated by elevation of cyclic guanosine monophosphate (cGMP) levels, membrane hyperpolarization via K+ channel activation, activation of myosin light chain phosphatase activity, and interference with

  4. Antioxidant mechanism of Rutin on hypoxia-induced pulmonary arterial cell proliferation.

    Science.gov (United States)

    Li, Qian; Qiu, Yanli; Mao, Min; Lv, Jinying; Zhang, Lixin; Li, Shuzhen; Li, Xia; Zheng, Xiaodong

    2014-11-18

    Reactive oxygen species (ROS) are involved in the pathologic process of pulmonary arterial hypertension as either mediators or inducers. Rutin is a type of flavonoid which exhibits significant scavenging properties on oxygen radicals both in vitro and in vivo. In this study, we proposed that rutin attenuated hypoxia-induced pulmonary artery smooth muscle cell (PASMC) proliferation by scavenging ROS. Immunofluorescence data showed that rutin decreased the production of ROS, which was mainly generated through mitochondria and NADPH oxidase 4 (Nox4) in pulmonary artery endothelial cells (PAECs). Western blot results provided further evidence on rutin increasing expression of Nox4 and hypoxia-inducible factor-1α (HIF-1α). Moreover, cell cycle analysis by flow cytometry indicated that proliferation of PASMCs triggered by hypoxia was also repressed by rutin. However, N-acetyl-L-cysteine (NAC), a scavenger of ROS, abolished or diminished the capability of rutin in repressing hypoxia-induced cell proliferation. These data suggest that rutin shows a potential benefit against the development of hypoxic pulmonary arterial hypertension by inhibiting ROS, subsequently preventing hypoxia-induced PASMC proliferation.

  5. Antioxidant Mechanism of Rutin on Hypoxia-Induced Pulmonary Arterial Cell Proliferation

    Directory of Open Access Journals (Sweden)

    Qian Li

    2014-11-01

    Full Text Available Reactive oxygen species (ROS are involved in the pathologic process of pulmonary arterial hypertension as either mediators or inducers. Rutin is a type of flavonoid which exhibits significant scavenging properties on oxygen radicals both in vitro and in vivo. In this study, we proposed that rutin attenuated hypoxia-induced pulmonary artery smooth muscle cell (PASMC proliferation by scavenging ROS. Immunofluorescence data showed that rutin decreased the production of ROS, which was mainly generated through mitochondria and NADPH oxidase 4 (Nox4 in pulmonary artery endothelial cells (PAECs. Western blot results provided further evidence on rutin increasing expression of Nox4 and hypoxia-inducible factor-1α (HIF-1α. Moreover, cell cycle analysis by flow cytometry indicated that proliferation of PASMCs triggered by hypoxia was also repressed by rutin. However, N-acetyl-L-cysteine (NAC, a scavenger of ROS, abolished or diminished the capability of rutin in repressing hypoxia-induced cell proliferation. These data suggest that rutin shows a potential benefit against the development of hypoxic pulmonary arterial hypertension by inhibiting ROS, subsequently preventing hypoxia-induced PASMC proliferation.

  6. A mycotic pulmonary artery aneurysm associated with candida endocarditis: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Jin Il; Lee, Ji Won; Jeong, Yeon Joo; Song, Seung Hwan [Pusan National University School of Medicine, Medical Research Institute, Pusan National University Hospital, Busan (Korea, Republic of)

    2014-03-15

    We report a case of a mycotic pulmonary aneurysm associated with Candida endocarditis in a 53-year-old male with lymphoma. The initial diagnosis was a pulmonary artery aneurysm attributable to vasculitis, such as that associated with Behcet's disease, but a mycotic pulmonary artery aneurysm was later considered as a differential diagnosis. Identification of valve vegetation on the chest CT was helpful in this regard. We review the literature on the disease etiology, radiological findings, and management options.

  7. Development of occlusive neointimal lesions in distal pulmonary arteries of endothelin B receptor-deficient rats: a new model of severe pulmonary arterial hypertension.

    Science.gov (United States)

    Ivy, D Dunbar; McMurtry, Ivan F; Colvin, Kelley; Imamura, Masatoshi; Oka, Masahiko; Lee, Dong-Seok; Gebb, Sarah; Jones, Peter Lloyd

    2005-06-07

    Human pulmonary arterial hypertension (PAH) is characterized by proliferation of vascular smooth muscle and, in its more severe form, by the development of occlusive neointimal lesions. However, few animal models of pulmonary neointimal proliferation exist, thereby limiting a complete understanding of the pathobiology of PAH. Recent studies of the endothelin (ET) system demonstrate that deficiency of the ET(B) receptor predisposes adult rats to acute and chronic hypoxic PAH, yet these animals fail to develop neointimal lesions. Herein, we determined and thereafter showed that exposure of ET(B) receptor-deficient rats to the endothelial toxin monocrotaline (MCT) leads to the development of neointimal lesions that share hallmarks of human PAH. The pulmonary hemodynamic and morphometric effects of 60 mg/kg MCT in control (MCT(+/+)) and ET(B) receptor-deficient (MCT(sl/sl)) rats at 6 weeks of age were assessed. MCT(sl/sl) rats developed more severe PAH, characterized by elevated pulmonary artery pressure, diminished cardiac output, and right ventricular hypertrophy. In MCT(sl/sl) rats, morphometric evaluation revealed the presence of neointimal lesions within small distal pulmonary arteries, increased medial wall thickness, and decreased arterial-to-alveolar ratio. In keeping with this, barium angiography revealed diminished distal pulmonary vasculature of MCT(sl/sl) rat lungs. Cells within neointimal lesions expressed smooth muscle and endothelial cell markers. Moreover, cells within neointimal lesions exhibited increased levels of proliferation and were located in a tissue microenvironment enriched with vascular endothelial growth factor, tenascin-C, and activated matrix metalloproteinase-9, factors already implicated in human PAH. Finally, assessment of steady state mRNA showed that whereas expression of ET(B) receptors was decreased in MCT(sl/sl) rat lungs, ET(A) receptor expression increased. Deficiency of the ET(B) receptor markedly accelerates the progression of

  8. Development of Occlusive Neointimal Lesions in Distal Pulmonary Arteries of Endothelin B Receptor–Deficient Rats: A New Model of Severe Pulmonary Arterial Hypertension

    Science.gov (United States)

    Ivy, D. Dunbar; McMurtry, Ivan F.; Colvin, Kelley; Imamura, Masatoshi; Oka, Masahiko; Lee, Dong-Seok; Gebb, Sarah; Jones, Peter Lloyd

    2007-01-01

    Background Human pulmonary arterial hypertension (PAH) is characterized by proliferation of vascular smooth muscle and, in its more severe form, by the development of occlusive neointimal lesions. However, few animal models of pulmonary neointimal proliferation exist, thereby limiting a complete understanding of the pathobiology of PAH. Recent studies of the endothelin (ET) system demonstrate that deficiency of the ETB receptor predisposes adult rats to acute and chronic hypoxic PAH, yet these animals fail to develop neointimal lesions. Herein, we determined and thereafter showed that exposure of ETB receptor–deficient rats to the endothelial toxin monocrotaline (MCT) leads to the development of neointimal lesions that share hallmarks of human PAH. Methods and Results The pulmonary hemodynamic and morphometric effects of 60 mg/kg MCT in control (MCT+/+) and ETB receptor–deficient (MCTsl/sl) rats at 6 weeks of age were assessed. MCTsl/sl rats developed more severe PAH, characterized by elevated pulmonary artery pressure, diminished cardiac output, and right ventricular hypertrophy. In MCTsl/sl rats, morphometric evaluation revealed the presence of neointimal lesions within small distal pulmonary arteries, increased medial wall thickness, and decreased arterial-to-alveolar ratio. In keeping with this, barium angiography revealed diminished distal pulmonary vasculature of MCTsl/sl rat lungs. Cells within neointimal lesions expressed smooth muscle and endothelial cell markers. Moreover, cells within neointimal lesions exhibited increased levels of proliferation and were located in a tissue microenvironment enriched with vascular endothelial growth factor, tenascin-C, and activated matrix metalloproteinase-9, factors already implicated in human PAH. Finally, assessment of steady state mRNA showed that whereas expression of ETB receptors was decreased in MCTsl/sl rat lungs, ETA receptor expression increased. Conclusions Deficiency of the ETB receptor markedly

  9. Pulmonary artery pressure increases during commercial air travel in healthy passengers.

    Science.gov (United States)

    Smith, Thomas G; Talbot, Nick P; Chang, Rae W; Wilkinson, Elizabeth; Nickol, Annabel H; Newman, David G; Robbins, Peter A; Dorrington, Keith L

    2012-07-01

    It is not known whether the mild hypoxia experienced by passengers during commercial air travel triggers hypoxic pulmonary vasoconstriction and increases pulmonary artery pressure in flight. Insidious pulmonary hypertensive responses could endanger susceptible passengers who have cardiopulmonary disease or increased hypoxic pulmonary vascular sensitivity. Understanding these effects may improve pre-flight assessment of fitness-to-fly and reduce in-flight morbidity and mortality. Eight healthy volunteers were studied during a scheduled commercial airline flight from London, UK, to Denver, CO. The aircraft was a Boeing 777 and the duration of the flight was 9 h. Systolic pulmonary artery pressure (sPAP) was assessed by portable Doppler echocardiography during the flight and over the following week in Denver, where the altitude (5280 ft/1610 m) simulates a commercial airliner environment. Cruising cabin altitude ranged between 5840 and 7170 ft (1780 to 2185 m), and mean arterial oxygen saturation was 95 +/- 0.6% during the flight. Mean sPAP increased significantly in flight by 6 +/- 1 mmHg to 33 +/- 1 mmHg, an increase of approximately 20%. After landing in Denver, sPAP was still 3 +/- 1 mmHg higher than baseline and remained elevated at 30 +/- 1 mmHg for a further 12 h. Pulmonary artery pressure increases during commercial air travel in healthy passengers, raising the possibility that hypoxic pulmonary hypertension could develop in susceptible individuals. A hypoxia altitude simulation test with simultaneous echocardiography ('HAST-echo') may be beneficial in assessing fitness to fly in vulnerable patients.

  10. Reversal of reflex pulmonary vasoconstriction induced by main pulmonary arterial distension.

    Science.gov (United States)

    Juratsch, C E; Grover, R F; Rose, C E; Reeves, J T; Walby, W F; Laks, M M

    1985-04-01

    Distension of the main pulmonary artery (MPA) induces pulmonary hypertension, most probably by neurogenic reflex pulmonary vasoconstriction, although constriction of the pulmonary vessels has not actually been demonstrated. In previous studies in dogs with increased pulmonary vascular resistance produced by airway hypoxia, exogenous arachidonic acid has led to the production of pulmonary vasodilator prostaglandins. Hence, in the present study, we investigated the effect of arachidonic acid in seven intact anesthetized dogs after pulmonary vascular resistance was increased by MPA distention. After steady-state pulmonary hypertension was established, arachidonic acid (1.0 mg/min) was infused into the right ventricle for 16 min; 15-20 min later a 16-mg bolus of arachidonic acid was injected. MPA distension was maintained throughout the study. Although the infusion of arachidonic acid significantly lowered the elevated pulmonary vascular resistance induced by MPA distension, the pulmonary vascular resistance returned to control levels only after the bolus injection of arachidonic acid. Notably, the bolus injection caused a biphasic response which first increased the pulmonary vascular resistance transiently before lowering it to control levels. In dogs with resting levels of pulmonary vascular resistance, administration of arachidonic acid in the same manner did not alter the pulmonary vascular resistance. It is concluded that MPA distension does indeed cause reflex pulmonary vasoconstriction which can be reversed by vasodilator metabolites of arachidonic acid. Even though this reflex may help maintain high pulmonary vascular resistance in the fetus, its function in the adult is obscure.

  11. Arterial tortuosity syndrome: An extremely rare disease presenting as a mimic of pulmonary sling

    Directory of Open Access Journals (Sweden)

    Amy Farkas, MD

    2018-02-01

    Full Text Available Pulmonary sling is the anatomic variant defined by the aberrant origin of the left pulmonary artery from the right pulmonary artery. This patient presented with a mimic of pulmonary sling as a result of an extremely rare condition, arterial tortuosity syndrome (ATS. The patient was first diagnosed with pulmonary sling on prenatal echocardiogram performed by cardiology. Computed tomography angiography of the chest obtained at birth to evaluate respiratory depression demonstrated ATS. The early detection of ATS has been demonstrated to improve patient outcome. This case provides an overview of the typical imaging features of ATS to aid radiologists in making this uncommon diagnosis.

  12. Pulmonary Arterial Capacitance Predicts Cardiac Events in Pulmonary Hypertension Due to Left Heart Disease.

    Directory of Open Access Journals (Sweden)

    Koichi Sugimoto

    Full Text Available Although pulmonary hypertension due to left heart disease (LHD-PH accounts for the largest proportion of pulmonary hypertension, few reports on the epidemiological analysis of LHD-PH exist. Recently, pulmonary arterial capacitance (PAC has attracted attention as a possible factor of right ventricular afterload along with pulmonary vascular resistance. We therefore investigated the clinical significance of PAC in LHD-PH.The subject consisted of 252 LHD-PH patients (145 men, mean age 63.4 ± 14.7 years diagnosed by right heart catheterization. PAC was estimated by the ratio between stroke volume and pulmonary arterial pulse pressure. Patients were classified into four groups according to the PAC (1st quartile was 0.74 to 1.76 ml/mmHg, the 2nd quartile 1.77 to 2.53 ml/mmHg, the 3rd quartile 2.54 to 3.59 ml/mmHg, and the 4th quartile 3.61 to 12.14 ml/mmHg. The end-points were defined as rehospitalization due to worsening heart failure and/or cardiac death. The Cox proportional hazard regression model was used to determine what variables were associated with cardiac events.The patients in the 1st quartile had the lowest cardiac index and stroke volume index, and the highest mean pulmonary arterial pressure, mean pulmonary capillary wedge pressure, and pulmonary vascular resistance compared with the 2nd, 3rd, and 4th quartiles. Fifty-four patients experienced cardiac events during the follow-up period (median 943 days. The event-free rate of the 1st quartile was significantly lower than that of the 3rd and 4th quartiles (66.7% vs 82.5% [3rd quartile], P = 0.008; and 92.1% [4th quartile], P < 0.001. The Cox hazard analysis revealed that PAC was significantly associated with cardiac events (HR 0.556, 95% CI 0.424-0.730, P < 0.001.PAC is useful in the prediction of cardiac event risk in LHD-PH patients.

  13. Pulmonary Arterial Capacitance Predicts Cardiac Events in Pulmonary Hypertension Due to Left Heart Disease

    Science.gov (United States)

    Sugimoto, Koichi; Yoshihisa, Akiomi; Nakazato, Kazuhiko; Jin, Yuichiro; Suzuki, Satoshi; Yokokawa, Tetsuro; Misaka, Tomofumi; Yamaki, Takayoshi; Kunii, Hiroyuki; Suzuki, Hitoshi; Saitoh, Shu-ichi; Takeishi, Yasuchika

    2016-01-01

    Background Although pulmonary hypertension due to left heart disease (LHD-PH) accounts for the largest proportion of pulmonary hypertension, few reports on the epidemiological analysis of LHD-PH exist. Recently, pulmonary arterial capacitance (PAC) has attracted attention as a possible factor of right ventricular afterload along with pulmonary vascular resistance. We therefore investigated the clinical significance of PAC in LHD-PH. Methods The subject consisted of 252 LHD-PH patients (145 men, mean age 63.4 ± 14.7 years) diagnosed by right heart catheterization. PAC was estimated by the ratio between stroke volume and pulmonary arterial pulse pressure. Patients were classified into four groups according to the PAC (1st quartile was 0.74 to 1.76 ml/mmHg, the 2nd quartile 1.77 to 2.53 ml/mmHg, the 3rd quartile 2.54 to 3.59 ml/mmHg, and the 4th quartile 3.61 to 12.14 ml/mmHg). The end-points were defined as rehospitalization due to worsening heart failure and/or cardiac death. The Cox proportional hazard regression model was used to determine what variables were associated with cardiac events. Results The patients in the 1st quartile had the lowest cardiac index and stroke volume index, and the highest mean pulmonary arterial pressure, mean pulmonary capillary wedge pressure, and pulmonary vascular resistance compared with the 2nd, 3rd, and 4th quartiles. Fifty-four patients experienced cardiac events during the follow-up period (median 943 days). The event-free rate of the 1st quartile was significantly lower than that of the 3rd and 4th quartiles (66.7% vs 82.5% [3rd quartile], P = 0.008; and 92.1% [4th quartile], P < 0.001). The Cox hazard analysis revealed that PAC was significantly associated with cardiac events (HR 0.556, 95% CI 0.424–0.730, P < 0.001). Conclusion PAC is useful in the prediction of cardiac event risk in LHD-PH patients. PMID:27875533

  14. Cryoplasty for Canine Iliac Artery Stenosis and its Impact on Expression of TIMP-2 and MMP-2.

    Science.gov (United States)

    Wu, Zhengzhong; Zang, Shengbing; Liu, Wenwen; Jiang, Na; Yang, Weizhu

    2015-01-01

    This study was performed to observe the effects of cryoplasty on canine iliac artery stenosis and the expression of tissue inhibition of matrix metalloproteinase 2 (TIMP-2) and matrix metalloproteinase 2 (MMP-2). We produced a reliable canine model to mimic the atherosclerotic stenosis in the iliac artery by suturing the artery followed by vessel ligation to create an injury to intimal and medial walls. Sixteen mongrel dogs with iliac artery stenosis were randomized to conventional balloon angioplasty (n = 8) or cryoplasty (n = 8). Four weeks posttreatment, the cryoplasty group with less collagen fibers and smooth muscle demonstrated significantly larger luminal diameter of iliac artery compared to the balloon angioplasty group (P < .001). Expression of TIMP-2 significantly increased and expression of MMP-2 significantly reduced in iliac artery of the cryoplasty group compared to conventional balloon angioplasty. Our study suggests cryoplasty might increase the expression of TIMP-2 and decrease the expression of MMP-2, thereby inhibiting vascular hyperplasia and collagen fibers synthesis of the stenotic vessels. © The Author(s) 2015.

  15. Management of pulmonary arterial hypertension.

    LENUS (Irish Health Repository)

    Judge, Eoin P

    2013-02-01

    Pulmonary arterial hypertension (PAH) is a complex disease with a high mortality. Management of this disease is underpinned by supportive and general therapies delivered by multidisciplinary teams in specialist centres. In recent years, a number of PAH-specific therapies have improved patient outcomes. This article will discuss the management of PAH in the context of relevant recently published studies in this area.

  16. THE RESTRICTED SURGICAL RELEVANCE OF MORPHOLOGIC CRITERIA TO CLASSIFY SYSTEMIC-PULMONARY COLLATERAL ARTERIES IN PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT

    NARCIS (Netherlands)

    DERUITER, MC; GITTENBERGERDEGROOT, AC; BOGERS, AJJC; ELZENGA, NJ

    1994-01-01

    Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic

  17. The dilatation of main pulmonary artery and right ventricle observed by enhanced chest computed tomography predict poor outcome in inoperable chronic thromboembolic pulmonary hypertension.

    Science.gov (United States)

    Ema, Ryogo; Sugiura, Toshihiko; Kawata, Naoko; Tanabe, Nobuhiro; Kasai, Hajime; Nishimura, Rintaro; Jujo, Takayuki; Shigeta, Ayako; Sakao, Seiichiro; Tatsumi, Koichiro

    2017-09-01

    Dilatation of the pulmonary artery and right ventricle on chest computed tomography images is often observed in patients with pulmonary hypertension. The clinical significance of these image findings has not been defined in chronic thromboembolic pulmonary hypertension. We investigated whether the pulmonary arterial and right ventricle dilatation was associated with poor outcome in chronic thromboembolic pulmonary hypertension. This was a retrospective cohort investigation in 60 subjects with inoperable chronic thromboembolic pulmonary hypertension diagnosed consecutively between 1997 and 2010 at Chiba University Hospital. Digital scout multi-detector chest computed tomography images were obtained. The main pulmonary arterial to ascending aortic diameter ratio and the right ventricular to left ventricular diameter ratio were calculated. Main pulmonary arterial to ascending aortic diameter ratio ranged from 0.85 to 1.84, and right ventricular to left ventricular diameter ratio ranged from 0.71 to 2.88. During the observation period of 1284.5days (range, 21-4550days), 13 patients required hospitalization due to worsening; 6 of them died. Kaplan-Meier analysis showed significant differences in hospitalization between the patients with main pulmonary arterial to ascending aortic diameter ratio of ≥1.1 and pulmonary hypertension. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  18. Intravenous clonidine administration and its ability to reduce pulmonary arterial pressure in patients undergoing heart surgery

    Directory of Open Access Journals (Sweden)

    Benedito Barbosa João

    2014-01-01

    Full Text Available Objective: Evaluate the ability of clonidine to reduce pulmonary arterial pressure in patients with pulmonary hypertension undergoing heart surgery, either by reducing the pressure values from the direct measurement of pulmonary arterial pressure or by reducing or eliminating the need for intraoperative dobutamine and nitroprusside. Method: Randomized, double-blind, placebo-controlled, comparative study conducted in 30 patients with pulmonary arterial hypertension type 2 undergoing cardiac surgery. Mean pulmonary arterial pressure and dosage of dobutamine and sodium nitroprusside were assessed four times: before intravenous administration of clonidine (2 μg/kg or placebo (T0, 30 min after tested treatment and before cardiopulmonary bypass (T1, immediately after CPB (T2, 10 min after protamine injection (T3. Results: There were no significant differences regarding mean pulmonary arterial pressure at any time of evaluation. There was no significant difference between groups regarding other variables, such as mean systemic arterial pressure, heart rate, total dose of dobutamine, total dose of sodium nitroprusside, and need for fentanyl. Conclusion: Data analysis from patients included in this study allows us to conclude that intravenous clonidine (2 μg/kg was not able to reduce the mean pulmonary arterial pressure in patients with pulmonary hypertension in group 2 (pulmonary venous hypertension, undergoing heart surgery, or reduce or eliminate the need for intraoperative administration of dobutamine and sodium nitroprusside. Keywords: Clonidine, Pulmonary hypertension, Heart surgery

  19. Pulmonary arterial hypertension in congenital cardiac disease - the need for refinement of the Evian-Venice classification

    NARCIS (Netherlands)

    van Albada, Mirjam E.; Berger, Rolf M. F.

    Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified, in the Evian-Venice classification, as Pulmonary Arterial Hypertension, which includes a heterogeneous group of conditions. Emerging options for treatment of patients with pulmonary arterial

  20. Pulmonary Artery Size in Interstitial Lung Disease and Pulmonary Hypertension: Association with Interstitial Lung Disease Severity and Diagnostic Utility

    Directory of Open Access Journals (Sweden)

    Matthew Chin

    2018-06-01

    Full Text Available PurposeIt is postulated that ILD causes PA dilatation independent of the presence of pulmonary hypertension (PH, so the use of PA size to screen for PH is not recommended. The aims of this study were to investigate the association of PA size with the presence and severity of ILD and to assess the diagnostic accuracy of PA size for detecting PH.MethodsIncident patients referred to a tertiary PH centre underwent baseline thoracic CT, MRI and right heart catheterisation (RHC. Pulmonary artery diameter was measured on CT pulmonary angiography and pulmonary arterial areas on MRI. A thoracic radiologist scored the severity of ILD on CT from 0 to 4, 0 = absent, 1 = 1–25%, 2 = 26–50%, 3 = 51–75%, and 4 = 76–100% extent of involvement. Receiver operating characteristic analysis and linear regression were employed to assess diagnostic accuracy and independent associations of PA size.Results110 had suspected PH due to ILD (age 65 years (SD 13, M:F 37:73 and 379 had suspected PH without ILD (age 64 years (SD 13, M:F 161:218. CT derived main PA diameter was accurate for detection of PH in patients both with and without ILD - AUC 0.873, p =< 0.001, and AUC 0.835, p =< 0.001, respectively, as was MRI diastolic PA area, AUC 0.897, p =< 0.001, and AUC 0.857, p =< 0.001, respectively Significant correlations were identified between mean pulmonary arterial pressure (mPAP and PA diameter in ILD (r = 0.608, p < 0.001, and non-ILD cohort (r = 0.426, p < 0.001. PA size was independently associated with mPAP (p < 0.001 and BSA (p = 0.001, but not with forced vital capacity % predicted (p = 0.597, Transfer factor of the lungs for carbon monoxide (TLCO % predicted (p = 0.321 or the presence of ILD on CT (p = 0.905. The severity of ILD was not associated with pulmonary artery dilatation (r = 0.071, p = 0.459.ConclusionsPulmonary arterial pressure elevation leads to pulmonary arterial dilation, which is not independently influenced by the presence or severity of

  1. Coil Embolization Treatment in Pulmonary Artery Branch Rupture During Swan-Ganz Catheterization

    International Nuclear Information System (INIS)

    Gottwalles, Yannick; Wunschel-Joseph, Marie-Eve; Hanssen, Michel

    2000-01-01

    Rupture of the pulmonary artery or one of its branches during Swan-Ganz catheterization is a complication that is rare but remains fatal in almost 50% of cases. The risk factors and mechanisms involved in the pathogenesis of this accident have been widely reported. Management is twofold: resuscitation procedures and specific medical or even surgical treatment. We report a case of pulmonary artery rupture occurring during Swan-Ganz catheterization that was treated by coil embolization. This technique, which is quick and simple to use, would appear to be very promising. This is the first case of successful emergency treatment of pulmonary artery rupture using an endovascular technique

  2. Noninvasive estimation of pulmonary arterial pressure by analysis of pulmonary blood-flow distribution

    International Nuclear Information System (INIS)

    Konstam, M.A.; Strauss, H.W.; Alpert, N.M.; Miller, S.W.; Murphy, R.X.; Greene, R.E.; McKusick, K.A.

    1979-01-01

    To determine whether a correlation exists between pulmonary arterial (PA) pressure (P/sub a/) and the distribution of pulmonary blood flow, this distribution was measured in four upright dogs in the control state and during intravenous infusions of epinephrine or prostaglandin F/sub 2α/. During suspension of respiration, 15 mCi of Xe-133 were injected intravenously, and perfusion and equilibration lung images were recorded with a scintillation camera. The procedure was performed several times on each dog, with and without pharmacological elevation of PA pressure by 5 to 50 cm H 2 O. For each scintigram, the relative blood flow per unit ventilated lung volume (F) was plotted against centimeters above the hilum (h). Pulmonary arterial pressure was derived from each curve, assuming the relation F = B(P/sub a/ - hD) 2 , where B = constant and D = specific gravity of blood. Calculated PA pressure correlated strongly (r = 0.83) with measured PA pressure, suggesting a possible means of noninvasive estimation of PA pressure

  3. Pulmonary arterial dissection in a post-partum patient with patent ductus arteriosus: Case report and review of the literature.

    Science.gov (United States)

    Yaman, Mehmet; Arslan, Uğur; Ateş, Ahmet Hakan; Aksakal, Aytekin

    2015-02-26

    Pulmonary arterial dissection is an uncommon but usually a deadly complication of chronic pulmonary hypertension. A 26-year-old female patient was admitted to our clinics with sudden dyspnea and chest discomfort one hour after giving birth to twins by vaginal delivery. An echocardiography was performed with a pre-diagnosis of pulmonary embolism. However, echocardiographic examination revealed a dilated main pulmonary artery and a dissection flap extending from main pulmonary artery to left pulmonary artery. In summary, in this report, we described a very rare case of pulmonary artery dissection in a pregnant patient with a previously un-diagnosed patent ductus arteriosus without an obvious rise in pulmonary artery pressure and reviewed the relevant literature.

  4. [Pulmonary arterial hypertension in women].

    Science.gov (United States)

    Sanchez, O; Marié, E; Lerolle, U; Wermert, D; Israël-Biet, D; Meyer, G

    2008-04-01

    Pulmonary arterial hypertension (PAH) is a rare condition characterized by sustained elevation in pulmonary arterial resistance leading to right heart failure. PAH afflicts predominantly women. Echocardiography is the initial investigation of choice for non-invasive detection of PAH but right-heart catheterization is necessary to confirm the diagnosis. Conventional treatment includes non-specific drugs (warfarin, diuretics, oxygen). The endothelin-1 receptor antagonist bosentan, the phosphodiesterase-5 inhibitor sildenafil, and prostanoids have been shown to improve symptoms, exercise capacity and haemodynamics. Intravenous prostacyclin is the first-line treatment for the most severely affected patients. Despite the most modern treatment the overall mortality rate of pregnant women with severe PAH remains high. Therefore, pregnancy is contraindicated in women with PAH and an effective method of contraception is recommended in women of childbearing age. Therapeutic abortion should be offered, particularly when early deterioration occurs. If this option is not accepted, intravenous prostacyclin should be considered promptly. Recent advances in the management of PAH have markedly improved prognosis and have resulted in more women of childbearing age considering pregnancy. A multidisciplinary approach should give new insights into cardiopulmonary, obstetric and anaesthetic management during pregnancy, delivery and the post-partum period.

  5. Associations between thoracic radiographic changes and severity of pulmonary arterial hypertension diagnosed in 60 dogs via Doppler echocardiography: A retrospective study.

    Science.gov (United States)

    Adams, Dustin S; Marolf, Angela J; Valdés-Martínez, Alejandro; Randall, Elissa K; Bachand, Annette M

    2017-07-01

    Doppler echocardiography is a noninvasive method for estimating and grading pulmonary arterial hypertension. No current literature associates significance of radiographic findings with severity of pulmonary arterial hypertension. We hypothesized that the number and conspicuity of radiographic findings suggestive of pulmonary arterial hypertension would be greater based on the severity of pulmonary arterial hypertension. Dogs with pulmonary arterial hypertension and normal control dogs were included in this retrospective, case control study. Three radiologists blinded to echocardiographic results scored thoracic radiographs for right ventricular and main pulmonary artery enlargement and pulmonary lobar artery enlargement, tortuosity, and blunting by multiple methods. Presence or absence of each finding was scored in an additive fashion and averaged for each grade of pulmonary arterial hypertension severity. Seventy-one dogs (60 dogs with pulmonary arterial hypertension and 11 control dogs) of which some had multiple studies were included: 20 mild, 21 moderate, 25 severe, and 11 absent pulmonary arterial hypertension. The following radiographic findings were significantly associated with increasing pulmonary arterial hypertension severity: right ventricular enlargement by "reverse D" and "3/5-2/5 cardiac ratio" methods, main pulmonary artery enlargement, and caudal lobar artery enlargement by the "3rd rib" method. Mean scores for severe pulmonary arterial hypertension and normal dogs were significantly different (P-value < 0.0001). Mean scores between different pulmonary arterial hypertension grades increased with severity but were not statistically significant. Individually and in combination, radiographic findings performed poorly in differentiating severity of pulmonary arterial hypertension. Findings indicated that thoracic radiographs should be utilized in conjunction with Doppler echocardiography in a complete diagnostic work-up for dogs with suspected

  6. Trial occlusion to assess the risk of persistent pulmonary arterial hypertension after closure of a large patent ductus arteriosus in adolescents and adults with elevated pulmonary artery pressure.

    Science.gov (United States)

    Zhang, Duan-Zhen; Zhu, Xian-Yang; Lv, Bei; Cui, Chun-Sheng; Han, Xiu-Min; Sheng, Xiao-Tang; Wang, Qi-Guang; Zhang, Po

    2014-08-01

    No method is available to predict whether patients with patent ductus arteriosus (PDA) and severe pulmonary arterial hypertension (PAH) will show persistent postprocedural PAH (PP-PAH) after PDA closure. This study evaluated the usefulness of trial occlusion for predicting PP-PAH after transcatheter PDA closure in patients with severe PAH. Trial occlusion was performed in 137 patients (age ≥12 years) with PDA and severe PAH. All patients undergoing trial occlusion had a mean pulmonary artery pressure ≥45 mm Hg, pulmonary:systemic flow (Qp/Qs) ratio >1.5, and pulmonary:systemic resistance (Rp/Rs) ratio closure. Linear correlation analysis revealed weak or moderate relationships between the baseline and post-trial pulmonary artery pressures and pulmonary:systemic pressure (Pp/Ps) ratios. Patients were followed up for 1 to 10 years (median: 5 years). PP-PAH (systolic pulmonary artery pressure >50 mm Hg by Doppler echocardiography) was detected in 17 patients (13%), who displayed no significant differences in sex and age compared with patients without PP-PAH. According to discriminant analysis, the strongest discriminators between patients with and without PP-PAH were the baseline left ventricular end-diastolic volume and the baseline and post-trial systolic Pp/Ps ratios. In particular, a post-trial systolic Pp/Ps ratio >0.5 correctly classified 100% of the PP-PAH and non-PAH patients. Trial occlusion is a feasible method to predict PP-PAH in patients with PDA and severe PAH. A post-trial systolic Pp/Ps ratio >0.5 indicates a high risk of PP-PAH occurrence after device closure. © 2014 American Heart Association, Inc.

  7. Drug-induced pulmonary arterial hypertension: a recent outbreak

    Directory of Open Access Journals (Sweden)

    Gérald Simonneau

    2013-09-01

    Full Text Available Pulmonary arterial hypertension (PAH is a rare disorder characterised by progressive obliteration of the pulmonary microvasculature resulting in elevated pulmonary vascular resistance and premature death. According to the current classification PAH can be associated with exposure to certain drugs or toxins, particularly to appetite suppressant intake drugs, such as aminorex, fenfluramine derivatives and benfluorex. These drugs have been confirmed to be risk factors for PAH and were withdrawn from the market. The supposed mechanism is an increase in serotonin levels, which was demonstrated to act as a growth factor for the pulmonary artery smooth muscle cells. Amphetamines, phentermine and mazindol were less frequently used, but are considered possible risk factors, for PAH. Dasatinib, dual Src/Abl kinase inhibitor, used in the treatment of chronic myelogenous leukaemia was associated with cases of severe PAH, potentially in part reversible after dasatinib withdrawal. Recently, several studies have raised the issue of potential endothelial dysfunction that could be induced by interferon, and a few cases of PAH have been reported with interferon therapy. PAH remains a rare complication of these drugs, suggesting possible individual susceptibility, and further studies are needed to identify patients at risk of drug-induced PAH.

  8. Pulmonary Artery Aneurysm/Pseudoaneurysm, a Delayed Complication of Lung Abscess: A Case Report.

    Science.gov (United States)

    Oguma, Tsuyoshi; Morise, Masahiro; Harada, Kazuki; Tanaka, Jun; Sato, Masako; Horio, Yukihiro; Takiguchi, Hiroto; Tomomatsu, Hiromi; Tomomatsu, Katsuyoshi; Takihara, Takahisa; Niimi, Kyoko; Hayama, Naoki; Aoki, Takuya; Urano, Tetsuya; Ito, Chihiro; Koizumi, Jun; Asano, Koichiro

    2015-09-20

    Massive hemoptysis mostly arises from the bronchial arteries; however, bleeding can also occur from a lesion in injured pulmonary arteries, such as pulmonary artery aneurysm/pseudoaneurysm (PAA/PAP), during pulmonary infection. A 66-year-old man was admitted with a diagnosis of lung abscess in the right lower lobe that was complicated with pyothorax. Intravenous administration of antibiotics and thoracic drainage successfully controlled the infection and inflammation until day 16, when the patient began to exhibit hemoptysis and bloody pleural effusion. Enhanced computed tomography (CT) with multi-planer reconstruction (MPR) images showed a highly enhanced mass inside the abscess fed by the pulmonary artery, suggesting PAA/PAP. Pulmonary angiography confirmed PAA/PAP, and embolization with coils successfully stopped both the bleeding into the sputum and pleural effusion, with a collapsed aneurysm visible on chest CT scan. Clinicians should consider the possibility of PAA/PAP in the differential diagnosis of hemoptysis during the treatment of patients with lung abscess. MPR CT is helpful for the diagnosis of PAA/PAP and its feeding vessels.

  9. Right Coronary Artery Originated from the Left Anterior Descending Artery in a Patient with Congenital Pulmonary Valvular Stenosis

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    Yusuf Hoşoğlu

    2013-01-01

    Full Text Available The single coronary artery, anomalous origin of the right coronary artery from the left anterior descending artery, is a benign and very rare coronary artery anomaly. We firstly present a case with this type of single coronary artery and congenital pulmonary valvular stenosis with large poststenotic dilatation.

  10. Role of Lipoxygenase Metabolites of Arachidonic Acid in Enhanced Pulmonary Artery Contractions of Female Rabbits

    OpenAIRE

    Pfister, Sandra L.

    2011-01-01

    Pulmonary arterial hypertension is characterized by elevated pulmonary artery pressure and vascular resistance. In women the incidence is 4 fold greater than that in men. Studies suggest sustained vasoconstriction is a factor in increased vascular resistance. Possible vasoconstrictor mediators include arachidonic acid-derived lipoxygenase metabolites. Our studies in rabbits showed enhanced endothelium-dependent contractions to arachidonic acid in pulmonary arteries from females compared to ma...

  11. Pediatric Perioperative Pulmonary Arterial Hypertension: A Case-Based Primer

    Directory of Open Access Journals (Sweden)

    Shilpa Shah

    2017-10-01

    Full Text Available The perioperative period is an extremely tenuous time for the pediatric patient with pulmonary arterial hypertension. This article will discuss a multidisciplinary approach to preoperative planning, the importance of early identification of pulmonary hypertensive crises, and practical strategies for postoperative management for this unique group of children.

  12. Pulmonary Effective Arterial Elastance as a Measure of Right Ventricular Afterload and Its Prognostic Value in Pulmonary Hypertension Due to Left Heart Disease.

    Science.gov (United States)

    Tampakakis, Emmanouil; Shah, Sanjiv J; Borlaug, Barry A; Leary, Peter J; Patel, Harnish H; Miller, Wayne L; Kelemen, Benjamin W; Houston, Brian A; Kolb, Todd M; Damico, Rachel; Mathai, Stephen C; Kasper, Edward K; Hassoun, Paul M; Kass, David A; Tedford, Ryan J

    2018-04-01

    Patients with combined post- and precapillary pulmonary hypertension due to left heart disease have a worse prognosis compared with isolated postcapillary. However, it remains unclear whether increased mortality in combined post- and precapillary pulmonary hypertension is simply a result of higher total right ventricular load. Pulmonary effective arterial elastance (Ea) is a measure of total right ventricular afterload, reflecting both resistive and pulsatile components. We aimed to test whether pulmonary Ea discriminates survivors from nonsurvivors in patients with pulmonary hypertension due to left heart disease and if it does so better than other hemodynamic parameters associated with combined post- and precapillary pulmonary hypertension. We combined 3 large heart failure patient cohorts (n=1036) from academic hospitals, including patients with pulmonary hypertension due to heart failure with preserved ejection fraction (n=232), reduced ejection fraction (n=335), and a mixed population (n=469). In unadjusted and 2 adjusted models, pulmonary Ea more robustly predicted mortality than pulmonary vascular resistance and the transpulmonary gradient. Along with pulmonary arterial compliance, pulmonary Ea remained predictive of survival in patients with normal pulmonary vascular resistance. The diastolic pulmonary gradient did not predict mortality. In addition, in a subset of patients with echocardiographic data, Ea and pulmonary arterial compliance were better discriminators of right ventricular dysfunction than the other parameters. Pulmonary Ea and pulmonary arterial compliance more consistently predicted mortality than pulmonary vascular resistance or transpulmonary gradient across a spectrum of left heart disease with pulmonary hypertension, including patients with heart failure with preserved ejection fraction, heart failure with reduced ejection fraction, and pulmonary hypertension with a normal pulmonary vascular resistance. © 2018 American Heart Association

  13. Analysis of the degree of pulmonary thallium washout after exercise in patients with coronary artery disease

    International Nuclear Information System (INIS)

    Levy, R.; Rozanski, A.; Berman, D.S.; Garcia, E.; Van Train, K.; Maddahi, J.; Swan, H.J.

    1983-01-01

    An abnormal increase in pulmonary thallium activity may be visualized on post-stress thallium images in patients with coronary artery disease. Because this increased pulmonary thallium activity usually disappears by the time of redistribution imaging, this study was designed to assess whether measurement of the degree of pulmonary thallium washout between stress and redistribution might improve the detection of increased pulmonary thallium activity in patients with coronary artery disease. Quantitative analysis revealed abnormal (that is, greater than 2 standard deviations of normal values) pulmonary thallium washouts in 59 (64%) of 92 patients with coronary artery disease, but in only 2 (25%) of 8 subjects with angiographically normal arteries (p less than 0.06). By comparison, the visual analysis of pulmonary thallium washout and use of initial pulmonary to myocardial thallium ratio were significantly (p less than 0.05) less sensitive in detecting abnormality in patients with coronary artery disease. Abnormal pulmonary thallium washout was related to both the anatomic extent and functional severity of disease: it occurred with greatest frequency in patients with multivessel disease and in those with exercise-induced left ventricular dysfunction (p less than 0.005). When added to the quantitative analysis of myocardial scintigraphy, the analysis of pulmonary thallium washout increased the detection of coronary artery disease from 84 to 93% (p less than 0.05), but the sample size was too small to assess specificity

  14. Fractal Dimension Analysis of MDCT Images for Quantifying the Morphological Changes of the Pulmonary Artery Tree in Patients with Pulmonary Hypertension

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    Sun, Haitao; Li, Ning; Guo, Lijun; Gao, Fei; Liu, Cheng [Shandong University, Shandong Medical Imaging Research Institute, Shandong (Korea, Republic of)

    2011-06-15

    The aim of this study was to use fractal dimension (FD) analysis on multidetector CT (MDCT) images for quantifying the morphological changes of the pulmonary artery tree in patients with pulmonary hypertension (PH). Fourteen patients with PH and 17 patients without PH as controls were studied. All of the patients underwent contrast-enhanced helical CT and transthoracic echocardiography. The pulmonary artery trees were generated using post-processing software, and the FD and projected image area of the pulmonary artery trees were determined with Image J software in a personal computer. The FD, the projected image area and the pulmonary artery pressure (PAP) were statistically evaluated in the two groups. The FD, the projected image area and the PAP of the patients with PH were higher than those values of the patients without PH (p < 0.05, t-test). There was a high correlation of FD with the PAP (r = 0.82, p < 0.05, partial correlation analysis). There was a moderate correlation of FD with the projected image area (r = 0.49, p < 0.05, partial correlation analysis). There was a correlation of the PAP with the projected image area (r = 0.65, p < 0.05, Pearson correlation analysis). The FD of the pulmonary arteries in the PH patients was significantly higher than that of the controls. There is a high correlation of FD with the PAP.

  15. Fractal Dimension Analysis of MDCT Images for Quantifying the Morphological Changes of the Pulmonary Artery Tree in Patients with Pulmonary Hypertension

    International Nuclear Information System (INIS)

    Sun, Haitao; Li, Ning; Guo, Lijun; Gao, Fei; Liu, Cheng

    2011-01-01

    The aim of this study was to use fractal dimension (FD) analysis on multidetector CT (MDCT) images for quantifying the morphological changes of the pulmonary artery tree in patients with pulmonary hypertension (PH). Fourteen patients with PH and 17 patients without PH as controls were studied. All of the patients underwent contrast-enhanced helical CT and transthoracic echocardiography. The pulmonary artery trees were generated using post-processing software, and the FD and projected image area of the pulmonary artery trees were determined with Image J software in a personal computer. The FD, the projected image area and the pulmonary artery pressure (PAP) were statistically evaluated in the two groups. The FD, the projected image area and the PAP of the patients with PH were higher than those values of the patients without PH (p < 0.05, t-test). There was a high correlation of FD with the PAP (r = 0.82, p < 0.05, partial correlation analysis). There was a moderate correlation of FD with the projected image area (r = 0.49, p < 0.05, partial correlation analysis). There was a correlation of the PAP with the projected image area (r = 0.65, p < 0.05, Pearson correlation analysis). The FD of the pulmonary arteries in the PH patients was significantly higher than that of the controls. There is a high correlation of FD with the PAP.

  16. Experimental study of a closed-chest pulmonary embolism-reperfusion injury canine model by means of Swan-Ganz catheter

    International Nuclear Information System (INIS)

    Bao Na; Zhai Renyou Jiang Tao; Wang Yajie; Zheng Juan; Wang Chen

    2007-01-01

    Objective: To establish a closed-chest pulmonary embolism-reperfusion animal model by Swan-Ganz catheter and to explore the mechanisms of pulmonary embolism (PE)-reperfusion injury (RI). Methods: Experiments were made on 14 mongrel dogs, ranging in weight from 15 to 18 kg, anesthetized with 3% pentobarbital sodium. The dogs were intubated with I. D. 7 endotracheal tubes. Under sterile conditions, a 7 F Swan-Ganz catheter via the external jugular vein was positioned in the unilateral pulmonary diaphragmatic lobe (DL) artery. Occlusion/reperfusion of the DL artery was controlled with 1.2 ml diluted contrast agent filled into/drawn from the balloon. After the 24 h PE, the balloon was deflated to result in 4 h reperfusion of the DL. Measurements of blood gases and tumor necrosis factor-α (TNF-α)were made at normal condition, at 24 h PE and at 4 h reperfusion. Thin-section CT scans were performed at normal condition, 24 h PE, 30 rain, 1, 2, 3 and 4 h reperfusion, respectively. At the end of each experiment, tissue specimens of bilateral diaphragmatic lobes were obtained for both wet/dry (W/D) weight ratio and for pathological study. Results: Reperfusion pulmonary edema (RPE) was an acute, mixed, noncardiogenic edema that was observed in all 14 dogs who had been successfully established as PE/RI animal models. RPE demonstrated heterogeneous ground-glass opacifications that predominated in the areas distal to the recanalized vessels. It manifested pathologically as an edematous lung infihrated by inflammatory cells. The mean of PaO 2 and TNF-α of 4 h reperfusion was (81 ± 4) mm Hg( 1 mm Hg =0.133 kPa) and (16.0 ± 2.5)pg/ml, which were significantly different (P<0.05) from normal value [(96 ± 6)mm Hg and (13.9 ± 2.0) pg/ml]. The W/D of the injured lung (6.3 ± 1.2) was significantly greater (P<0.01) than that of the contralateral lung (4.5 ± 1.2), suggesting that the increase in the lung water was due to reperfusion injury. Conclusion: The closed-chest canine model

  17. [Cardiac catheterization and pulmonary vasoreactivity testing in children with idiopathic pulmonary arterial hypertension].

    Science.gov (United States)

    Zhang, Chen; Li, Qiangqiang; Liu, Tianyang; Gu, Hong

    2014-06-01

    As an important method of hemodynamic assessment in idiopathic pulmonary arterial hypertension (IPAH), cardiac catheterization combined with pulmonary vasoreactivity testing remains with limited experience in children, and the acute pulmonary vasodilator agents as well as response criteria for vasoreactivity testing remain controversial. The aim of this study was to investigate the clinical importance, agent selection, and responder definition of cardiac catheterization combined with pulmonary vasoreactivity testing in pediatric IPAH. The patients admitted to Department of Pediatric Cardiology of Beijing Anzhen Hospital between April 2009 and September 2013 with suspected IPAH, under 18 years of age, with WHO functional class II or III, were enrolled. All the patients were arranged to receive left and right heart catheterization and pulmonary vasoreactivity testing with inhalation of pure oxygen and iloprost (PGI2) respectively. Hemodynamic changes were analyzed, and two criteria, the European Society of Cardiology recommendation criteria (Sitbon criteria) and traditional application criteria (Barst criteria), were used to evaluate the test results. Thirty-nine cases of children with suspected IPAH underwent cardiac catheterization. In 4 patients IPAH was excluded; 4 patients developed pulmonary hypertension crisis. The other 31 patients received standard cardiac catheterization and pulmonary vasoreactivity testing. Baseline mean pulmonary artery pressure (mPAP) was (66 ± 16) mmHg (1 mmHg = 0.133 kPa), and pulmonary vascular resistance index (PVRI) (17 ± 8) Wood U · m². After inhalation of pure oxygen, mPAP fell to (59 ± 16) mmHg, and PVRI to (14 ± 8) Wood U · m² (t = 4.88 and 4.56, both P hypertension crisis is an important complication of cardiac catheterization in pediatric IPAH. Younger age, general anesthesia, crisis history, and poor heart function are important risk factors for pulmonary hypertension crisis. PGI2 is a relatively ideal agent for

  18. Pregnancy in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Karen M. Olsson

    2016-12-01

    Full Text Available Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who become pregnant. Early termination should be discussed. Women who choose to continue with their pregnancy should be treated at specialised pulmonary hypertension centres with experience in managing PAH during and after pregnancy.

  19. Pregnancy in pulmonary arterial hypertension.

    Science.gov (United States)

    Olsson, Karen M; Channick, Richard

    2016-12-01

    Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who become pregnant. Early termination should be discussed. Women who choose to continue with their pregnancy should be treated at specialised pulmonary hypertension centres with experience in managing PAH during and after pregnancy. Copyright ©ERS 2016.

  20. Three-dimensional segmentation of pulmonary artery volume from thoracic computed tomography imaging

    Science.gov (United States)

    Lindenmaier, Tamas J.; Sheikh, Khadija; Bluemke, Emma; Gyacskov, Igor; Mura, Marco; Licskai, Christopher; Mielniczuk, Lisa; Fenster, Aaron; Cunningham, Ian A.; Parraga, Grace

    2015-03-01

    Chronic obstructive pulmonary disease (COPD), is a major contributor to hospitalization and healthcare costs in North America. While the hallmark of COPD is airflow limitation, it is also associated with abnormalities of the cardiovascular system. Enlargement of the pulmonary artery (PA) is a morphological marker of pulmonary hypertension, and was previously shown to predict acute exacerbations using a one-dimensional diameter measurement of the main PA. We hypothesized that a three-dimensional (3D) quantification of PA size would be more sensitive than 1D methods and encompass morphological changes along the entire central pulmonary artery. Hence, we developed a 3D measurement of the main (MPA), left (LPA) and right (RPA) pulmonary arteries as well as total PA volume (TPAV) from thoracic CT images. This approach incorporates segmentation of pulmonary vessels in cross-section for the MPA, LPA and RPA to provide an estimate of their volumes. Three observers performed five repeated measurements for 15 ex-smokers with ≥10 pack-years, and randomly identified from a larger dataset of 199 patients. There was a strong agreement (r2=0.76) for PA volume and PA diameter measurements, which was used as a gold standard. Observer measurements were strongly correlated and coefficients of variation for observer 1 (MPA:2%, LPA:3%, RPA:2%, TPA:2%) were not significantly different from observer 2 and 3 results. In conclusion, we generated manual 3D pulmonary artery volume measurements from thoracic CT images that can be performed with high reproducibility. Future work will involve automation for implementation in clinical workflows.

  1. Isolated unilateral absence of the right pulmonary artery in two cats visualized by computed tomography angiography

    Directory of Open Access Journals (Sweden)

    Tyler JM Jordan

    2016-10-01

    Full Text Available Case series summary Two cats were evaluated for progressive exercise intolerance, dyspnea and unilateral infiltrate of the left lung. Computed tomography angiography (CTA revealed absence of the right pulmonary artery in both cats with systemic arterial collateral vessels perfusing the right segmental pulmonary arteries. In one case, the collateral vessels arose from the esophageal artery, while in the other case they derived off the right costocervical trunk. One cat was diagnosed with pulmonary hypertension and was euthanized owing to progressive respiratory distress despite medical management with sildenafil, pimobendan, clopidogrel and furosemide. The other cat, without echocardiographic evidence of pulmonary hypertension, was successfully managed with furosemide and enalapril for more than 4 years. Relevance and novel information CTA allowed visualization of a rare congenital heart malformation, unilateral absence of the right pulmonary artery, in two cats and accurately characterized the source of collateral blood supply to the affected lung. Severe pulmonary hypertension may be a negative prognostic factor in cats with this condition as medical therapy in the cat without evidence of pulmonary hypertension resolved clinical signs, while the cat with severe pulmonary hypertension died from the disease.

  2. Quantitative determination of pulmonary artery flow by scintiscanning in patients with mitral stenosis

    International Nuclear Information System (INIS)

    Carvalho, N.; Fujioka, T.; Dias Neto, A.; Papaleo Netto, M.

    1974-01-01

    In twenty-two patients with pure or predominant mitral stenosis, with pulmonary hypertension, the quantitative blood flow of the pulmonary artery systems through the distribution of macroaggregated radio-iodinated ( 131 I) albumin is studied, by pulmonary digital scanning. Through the relations ship between the radioactive concentration in each organ, it is possible to classify three types of images: normal, balanced and inverted. This technique is useful for quantitative analysis of pulmonary artery flow, as a test that should preced cardiac catheterization and in the follow-up of the surgical results [pt

  3. Axial flow velocity patterns in a normal human pulmonary artery model: pulsatile in vitro studies.

    Science.gov (United States)

    Sung, H W; Yoganathan, A P

    1990-01-01

    It has been clinically observed that the flow velocity patterns in the pulmonary artery are directly modified by disease. The present study addresses the hypothesis that altered velocity patterns relate to the severity of various diseases in the pulmonary artery. This paper lays a foundation for that analysis by providing a detailed description of flow velocity patterns in the normal pulmonary artery, using flow visualization and laser Doppler anemometry techniques. The studies were conducted in an in vitro rigid model in a right heart pulse duplicator system. In the main pulmonary artery, a broad central flow field was observed throughout systole. The maximum axial velocity (150 cm s-1) was measured at peak systole. In the left pulmonary artery, the axial velocities were approximately evenly distributed in the perpendicular plane. However, in the bifurcation plane, they were slightly skewed toward the inner wall at peak systole and during the deceleration phase. In the right pulmonary artery, the axial velocity in the perpendicular plane had a very marked M-shaped profile at peak systole and during the deceleration phase, due to a pair of strong secondary flows. In the bifurcation plane, higher axial velocities were observed along the inner wall, while lower axial velocities were observed along the outer wall and in the center. Overall, relatively low levels of turbulence were observed in all the branches during systole. The maximum turbulence intensity measured was at the boundary of the broad central flow field in the main pulmonary artery at peak systole.

  4. Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions

    NARCIS (Netherlands)

    Blok, Ilja M.; van Riel, Annelieke C. M. J.; Mulder, Barbara J. M.; Bouma, Berto J.

    2015-01-01

    Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary

  5. Isolated pulmonary veno-occlusive disease and pulmonary arterial thrombosis in systemic sclerosis – a lethal combination

    Directory of Open Access Journals (Sweden)

    Arun Jeevagan

    2010-05-01

    Full Text Available Arun JeevaganGeneral Medicine, Ipswich NHS Hospital, UKBackground: Isolated pulmonary hypertension secondary to systemic sclerosis is not uncommon. Our patient with systemic sclerosis presented with a very aggressive form of pulmonary hypertension due to a lethal combination of pulmonary veno-occlusive disease (PVOD and pulmonary arterial thrombosis. This combined presentation has never before been reported in medical literature.Case report: A 75-year-old woman with a 4-month history of atypical chest pains was admitted with a 3-week history of worsening symptoms of shortness of breath, reduced exercise tolerance, and bilateral pitting edema. On examination she had thickened skin in her hands, telangiectasia on her face, maculopapular rash in her legs, raised jugular venous pressure, and bilateral pitting edema. Her autoimmune profile revealed positive anticentromere antibodies, and her echocardiogram showed right ventricular systolic pressure of 91 mmHg. She also had renal impairment secondary to hypoperfusion. A diagnosis of isolated pulmonary hypertension secondary to limited systemic sclerosis was made. As she was clinically improving on slow diuretic infusion and awaiting transfer to a specialist center for management of pulmonary hypertension, our patient died due to cardiopulmonary arrest. Her postmortem revealed that she died of a combination of PVOD and pulmonary arteriopathy due to thrombosis.Conclusion: This is clearly a unique case both in presentation and difficulty of management. Pulmonary vasodilators used in therapy of pulmonary arteriopathy can be detrimental in patients with PVOD. There is no definitive investigation, curative treatment, or management, that exists for a combination of PVOD and pulmonary arteriopathy due to thrombosis secondary to systemic sclerosis.Keywords: pulmonary veno-occlusive disease, pulmonary arterial hypertension, systemic sclerosis, pulmonary arteriopathy with thrombosis

  6. Pulmonary artery hypertension in chronic obstructive lung disease. The validity of morphometric tests in radiodiagnosis of the thorax

    Energy Technology Data Exchange (ETDEWEB)

    Dinkel, E; Mundinger, A; Reinbold, W D; Wuertemberger, G

    1989-06-01

    Standard biplane chest X-rays were tested for the validity of morphometric criteria in the diagnosis of pulmonary artery hypertension. Twenty-seven patients suffering from chronic obstructive lung disease were examined and compared with a control group without cardiopulmonary disease. The diameter of the right and left pulmonary artery, pulmonary conus and the hilar-to-thoracic ratio were significantly increased in patients with chronic obstructive lung disease (p<0.0001). Measurement of the right pulmonary artery was 19.7+-3.9 mm compared to 13.6+-1.2 mm of the control group; mean hilar thoracic index was 0.35 compared to 0.31. Thus if the width of the descending branch of the right pulmonary artery was above 16 mm, pulmonary arterial hypertension was suggested, with a specificity of almost 100%, although the sensitivity of the diagnosis was only 59%. The mean pulmonary arterial pressure obtained by right heart catheterization correlated poorly with the morphometric criteria obtained. (orig.).

  7. Pulmonary artery occlusion pressure estimation by transesophageal echocardiography: is simpler better?

    Science.gov (United States)

    Voga, Gorazd

    2008-01-01

    The measurement of pulmonary artery occlusion pressure (PAOP) is important for estimation of left ventricular filling pressure and for distinction between cardiac and non-cardiac etiology of pulmonary edema. Clinical assessment of PAOP, which relies on physical signs of pulmonary congestion, is uncertain. Reliable PAOP measurement can be performed by pulmonary artery catheter, but it is possible also by the use of echocardiography. Several Doppler variables show acceptable correlation with PAOP and can be used for its estimation in cardiac and critically ill patients. Noninvasive PAOP estimation should probably become an integral part of transthoracic and transesophageal echocardiographic evaluation in critically ill patients. However, the limitations of both methods should be taken into consideration, and in specific patients invasive PAOP measurement is still unavoidable, if the exact value of PAOP is needed.

  8. Anomalous Origin of One Pulmonary Artery From the Ascending Aorta: From Diagnosis to Treatment in Angola.

    Science.gov (United States)

    Manuel, Valdano; Sousa-Uva, Miguel; Morais, Humberto; Magalhães, Manuel P; Pedro, Albino; Miguel, Gade; Nunes, Maria A S; Gamboa, Sebastiana; Júnior, António P F

    2015-10-01

    Anomalous origin of one pulmonary artery is a rare congenital heart disease in which one pulmonary artery branch originates from the ascending aorta. To describe the experience of a cardiothoracic center in an African country to repair anomalous origin of one pulmonary artery in the context of Portugal-Angola collaboration. Between March 2011 and March 2015, four consecutive patients with anomalous origin of pulmonary artery branch underwent surgical correction. The mean age was 1.6 months. The mean weight was 4 kg. All had right pulmonary artery branch originating from the ascending aorta. All patients underwent direct implantation of right pulmonary branch to main pulmonary artery. Two patents had patent ductus arteriosus and one had atrial septal defect. Two patients had pulmonary hypertension. There was no registration of death. The mean cardiopulmonary bypass time was 75.5 ± 4.5 minutes, mean aortic cross-clamping time was 40 ± 5.6 minutes, and mean duration of the postoperative intensive care unit stay was 6.8 ± 5.7 days. At discharge, one patient had residual gradient of 25 mm Hg, the remainder had no significant gradient. The mean follow-up time was 11 months (5-28 months). One week after discharge, one patient presented operative wound dehiscence. At the last follow-up, all patients were alive, and no significant residual gradient or stenosis at site of anastomosis was observed. No reintervention was required. Anomalous origin of one pulmonary artery is a rare but potentially treatable lesion if operated early in life. Direct implantation was a good technique with good short-term results. © The Author(s) 2015.

  9. Effect of implanted radioactive 125I seeds on normal tissue structures of bronchus, esophagus, pulmonary artery, pulmonary vein and alveolus in dogs

    International Nuclear Information System (INIS)

    Qi Liangchen; Han Zhenguo; Yang Bin; Heersitai

    2008-01-01

    Objective: To investigate the effect of implanted radioactive 125 I seeds on normal tissue structures of bronchus, esophagus, pulmonary artery, pulmonary vein and alveolus in dogs. Methods: Nine healthy male dogs weighing 17-21 kg were randomly divided into three groups: 30 d, 60 d experimental groups and control group. Radioactive 125 I seeds (3.7 x 10 7 Bg, 1.0 mCi) were implanted into the sides of bronchus, esophagus, pulmonary artery, pulmonary vein respectively, the samples of bronchus, esophagus, pulmonary artery, pulmonary vein were taken 30 and 60 d after transplantation, HE staining was used to observe the pathologic changes of the tissues under light microscope. Results: The damages of normal bronchus, esophagus, pulmonary artery, pulmonary vein and alveolus after radioactive 125 I seeds implantation in 30 d group were weaker than those in control group and 60 d group, there were no complications such as perforation, hemorrhage, necrosis, etc. Histopathological score indicated that the scores of bronchus, esophagus and alveolar in 30 d group and 60 d group were higher than those in control group (P 0.05); there was no significant difference in histopathological score of pulmonary vein among all groups (P>0.05). Conclusion: The implanted radioactive 125 I seeds can damage all kinds of tissues at different degrees, but this kind of damage is reversible, the dog may repair the damage through its own repair ability, its clinical application is safe. (authors)

  10. Biaxial Properties of the Left and Right Pulmonary Arteries in a Monocrotaline Rat Animal Model of Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Pursell, Erica R; Vélez-Rendón, Daniela; Valdez-Jasso, Daniela

    2016-11-01

    In a monocrotaline (MCT) induced-pulmonary arterial hypertension (PAH) rat animal model, the dynamic stress-strain relation was investigated in the circumferential and axial directions using a linear elastic response model within the quasi-linear viscoelasticity theory framework. Right and left pulmonary arterial segments (RPA and LPA) were mechanically tested in a tubular biaxial device at the early stage (1 week post-MCT treatment) and at the advanced stage of the disease (4 weeks post-MCT treatment). The vessels were tested circumferentially at the in vivo axial length with matching in vivo measured pressure ranges. Subsequently, the vessels were tested axially at the mean pulmonary arterial pressure by stretching them from in vivo plus 5% of their length. Parameter estimation showed that the LPA and RPA remodel at different rates: axially, both vessels decreased in Young's modulus at the early stage of the disease, and increased at the advanced disease stage. Circumferentially, the Young's modulus increased in advanced PAH, but it was only significant in the RPA. The damping properties also changed in PAH; in the LPA relaxation times decreased continuously as the disease progressed, while in the RPA they initially increased and then decreased. Our modeling efforts were corroborated by the restructuring organization of the fibers imaged under multiphoton microscopy, where the collagen fibers become strongly aligned to the 45 deg angle in the RPA from an uncrimped and randomly organized state. Additionally, collagen content increased almost 10% in the RPA from the placebo to advanced PAH.

  11. Relationship between pulmonary artery volumes at computed tomography and pulmonary artery pressures in patients with- and without pulmonary hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Froelich, Jens J. [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: jens.froelich@klinikum-hef.de; Koenig, Helmut [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: helmut.koenig@siemens.com; Knaak, Lennard [Department of Medicine, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: froehlic@staff.uni-marburg.de; Krass, Stefan [MeVis Research, Universitaetsallee 29, 28359 Bremen (Germany)], E-mail: krass@mevis.de; Klose, Klaus J. [Department of Radiology, Philipps-University Hospital, Baldingerstrasse, 35043 Marburg (Germany)], E-mail: klose@med.uni-marburg.de

    2008-09-15

    Objectives: This study was designed to determine the relationship between pulmonary artery (PA) volumes at computed tomography (CT) and PA pressures at right-sided heart catheterization in patients with and without pulmonary hypertension (PAH) to develop a noninvasive CT method of PA pressure quantification. Materials and methods: Sixteen patients with chronic sleep apnea syndrome underwent contrast enhanced helical CT (slice thickness 3 mm; pitch 2; increment 2 mm) at inspiration. Eight patients had PAH while cardiopulmonary disease has been excluded in eight other patients. Vascular volumes were determined using a 3D technique (threshold seeded vascular tracing algorithm; thresholds -600 H [lower] and 3000 H [upper]). Right-sided heart catheterization measurements were available for linear regression analysis of PA volumes and pressures. Results: Correlation between PA pressures and volumes (normalized for BMI), was high in both groups (without PAH: r = .85; with PAH .90, Pearson). Compared to elevated PA pressures in patients with pulmonary hypertension (p < .005), PA volumes also were significantly increased (p < .05) among the groups. Conclusions: High correlation was found between PA volumes and mean PA pressures in patients with- and without PAH. Significant differences in PA volumes at CT-volumetry may admit non-invasive determination of pulmonary hypertension.

  12. Relationship between pulmonary artery volumes at computed tomography and pulmonary artery pressures in patients with- and without pulmonary hypertension

    International Nuclear Information System (INIS)

    Froelich, Jens J.; Koenig, Helmut; Knaak, Lennard; Krass, Stefan; Klose, Klaus J.

    2008-01-01

    Objectives: This study was designed to determine the relationship between pulmonary artery (PA) volumes at computed tomography (CT) and PA pressures at right-sided heart catheterization in patients with and without pulmonary hypertension (PAH) to develop a noninvasive CT method of PA pressure quantification. Materials and methods: Sixteen patients with chronic sleep apnea syndrome underwent contrast enhanced helical CT (slice thickness 3 mm; pitch 2; increment 2 mm) at inspiration. Eight patients had PAH while cardiopulmonary disease has been excluded in eight other patients. Vascular volumes were determined using a 3D technique (threshold seeded vascular tracing algorithm; thresholds -600 H [lower] and 3000 H [upper]). Right-sided heart catheterization measurements were available for linear regression analysis of PA volumes and pressures. Results: Correlation between PA pressures and volumes (normalized for BMI), was high in both groups (without PAH: r = .85; with PAH .90, Pearson). Compared to elevated PA pressures in patients with pulmonary hypertension (p < .005), PA volumes also were significantly increased (p < .05) among the groups. Conclusions: High correlation was found between PA volumes and mean PA pressures in patients with- and without PAH. Significant differences in PA volumes at CT-volumetry may admit non-invasive determination of pulmonary hypertension

  13. MAO-A promoter polymorphism and idiopathic pulmonary arterial ...

    Indian Academy of Sciences (India)

    Introduction. Monoamine oxidases (MAO) are mitochondrial enzymes ... liver and later in lungs by the enzyme MAO-A, via ox- ... Diagnosis was based on WHO criteria .... treatment of pulmonary arterial hypertension of the European so-.

  14. Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases

    Directory of Open Access Journals (Sweden)

    V Govindaraj

    2017-01-01

    Full Text Available Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

  15. Coronary Artery Anomalies in Animals

    Directory of Open Access Journals (Sweden)

    Brian A. Scansen

    2017-04-01

    Full Text Available Coronary artery anomalies represent a disease spectrum from incidental to life-threatening. Anomalies of coronary artery origin and course are well-recognized in human medicine, but have received limited attention in veterinary medicine. Coronary artery anomalies are best described in the dog, hamster, and cow though reports also exist in the horse and pig. The most well-known anomaly in veterinary medicine is anomalous coronary artery origin with a prepulmonary course in dogs, which limits treatment of pulmonary valve stenosis. A categorization scheme for coronary artery anomalies in animals is suggested, dividing these anomalies into those of major or minor clinical significance. A review of coronary artery development, anatomy, and reported anomalies in domesticated species is provided and four novel canine examples of anomalous coronary artery origin are described: an English bulldog with single left coronary ostium and a retroaortic right coronary artery; an English bulldog with single right coronary ostium and transseptal left coronary artery; an English bulldog with single right coronary ostium and absent left coronary artery with a prepulmonary paraconal interventricular branch and an interarterial circumflex branch; and a mixed-breed dog with tetralogy of Fallot and anomalous origin of all coronary branches from the brachiocephalic trunk. Coronary arterial fistulae are also described including a coronary cameral fistula in a llama cria and an English bulldog with coronary artery aneurysm and anomalous shunting vessels from the right coronary artery to the pulmonary trunk. These examples are provided with the intent to raise awareness and improve understanding of such defects.

  16. Transcatheter closure of patent ductus arteriosus with severe pulmonary arterial hypertension in adults

    International Nuclear Information System (INIS)

    Zhao Shihua; Yan Chaowu; Jiang Shiliang; Xu Zhongying; Huang Lianjun; Ling Jian; Zheng Hong; Wang Cheng; Hu Haibo; Wu Wenhui; Li Shiguo; Dai Ruping

    2006-01-01

    Objective: To evaluate the effect of self-expandable occluder on closure of patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PH) in adults. Methods: Twenty-eight adult patients underwent transcatheter closure of PDA at a mean age of (31.3±11.6) years [(18-58) years]. Either Amplatzer duct occluder or domestic device was used in the present study. X-ray, EKG and UCG were repeated in one day, one month, three months, and six months. Results: Twenty of the 28 patients had successful occlusion, and the other 8 patients were given up. In the successful group, the narrowest diameter of PDA was (10.4±2.7) mm [(6-16) mm], the diameter of selected occluder was (15.6±3.2)mm [(10-20) mm] at the end of pulmonary artery. Systemic artery oxygen saturation (SAsat) before and after oxygen inhalation was (93.5±1.8)%, (98.2±1.8)%, respectively (P<0.01). Systolic pulmonary arterial pressure decreased significantly after trial occlusion from (95.5± 24.l) mm Hg to (56.3±18.3) mm Hg (P<0.01); Mean pulmonary arterial pressures decreased significantly from (70.8±18.2) mm Hg to (41.0±13.8 )mm Hg (P<0. 01), too. Six months later UCG showed that the dimensions of left atrium, left ventricle and pulmonary artery attenuated significantly with one exception; X rays showed decreased pulmonary vascularity or cardiac size as well [cardio-thoracic ratio: (59.2±6.6)% vs (54.2±3.3)%, P<0.01]. In given up group, four patients were defined as Eisenmenger syndromes with differential cyanosis. They presented with elevated systolic pulmonary arterial pressure [(110.3±13.9) mm Hg vs (139.5±20.0)mm Hg, P<0.01], or decreased systolic aortic pressure[(116.0±20.2)mm Hg vs (106.3±16.9) mm Hg, P<0.05]after occlusion. The others included two cases with large residual shunt, and two cases with worsening of symptoms. Conclusions: Transcatheter closure of PDA with reversible severe PH is feasible, effective and safe in adults. (authors)

  17. Transcatheter closure of patent ductus arteriosus with severe pulmonary arterial hypertension in adults

    Energy Technology Data Exchange (ETDEWEB)

    Shihua, Zhao; Chaowu, Yan; Shiliang, Jiang; Zhongying, Xu; Lianjun, Huang; Jian, Ling; Hong, Zheng; Cheng, Wang; Haibo, Hu; Wenhui, Wu; Shiguo, Li; Ruping, Dai [Department of Radiology, Cardiovascular Inst. and Fuwai Hospital, Chinese Academy of Medical Sciences, Beijing Union Medical College, Beijing (China)

    2006-11-15

    Objective: To evaluate the effect of self-expandable occluder on closure of patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PH) in adults. Methods: Twenty-eight adult patients underwent transcatheter closure of PDA at a mean age of (31.3{+-}11.6) years [(18-58) years]. Either Amplatzer duct occluder or domestic device was used in the present study. X-ray, EKG and UCG were repeated in one day, one month, three months, and six months. Results: Twenty of the 28 patients had successful occlusion, and the other 8 patients were given up. In the successful group, the narrowest diameter of PDA was (10.4{+-}2.7) mm [(6-16) mm], the diameter of selected occluder was (15.6{+-}3.2)mm [(10-20) mm] at the end of pulmonary artery. Systemic artery oxygen saturation (SAsat) before and after oxygen inhalation was (93.5{+-}1.8)%, (98.2{+-}1.8)%, respectively (P<0.01). Systolic pulmonary arterial pressure decreased significantly after trial occlusion from (95.5{+-} 24.l) mm Hg to (56.3{+-}18.3) mm Hg (P<0.01); Mean pulmonary arterial pressures decreased significantly from (70.8{+-}18.2) mm Hg to (41.0{+-}13.8 )mm Hg (P<0. 01), too. Six months later UCG showed that the dimensions of left atrium, left ventricle and pulmonary artery attenuated significantly with one exception; X rays showed decreased pulmonary vascularity or cardiac size as well [cardio-thoracic ratio: (59.2{+-}6.6)% vs (54.2{+-}3.3)%, P<0.01]. In given up group, four patients were defined as Eisenmenger syndromes with differential cyanosis. They presented with elevated systolic pulmonary arterial pressure [(110.3{+-}13.9) mm Hg vs (139.5{+-}20.0)mm Hg, P<0.01], or decreased systolic aortic pressure[(116.0{+-}20.2)mm Hg vs (106.3{+-}16.9) mm Hg, P<0.05]after occlusion. The others included two cases with large residual shunt, and two cases with worsening of symptoms. Conclusions: Transcatheter closure of PDA with reversible severe PH is feasible, effective and safe in adults. (authors)

  18. Peptide-micelle hybrids containing fasudil for targeted delivery to the pulmonary arteries and arterioles to treat pulmonary arterial hypertension.

    Science.gov (United States)

    Gupta, Nilesh; Ibrahim, Hany M; Ahsan, Fakhrul

    2014-11-01

    This study investigates the respirability and efficacy of peptide-micelle hybrid nanoparticles as carriers for inhalational therapy of pulmonary arterial hypertension (PAH). CARSKNKDC (CAR), a cell-penetrating and lung-homing peptide, conjugated polyethylene glycol-distearoyl-phosphoethanolamine micelles containing fasudil, an investigational anti-PAH drug, were prepared by solvent evaporation method and characterized for various physicochemical properties. The pharmacokinetics and pharmacological efficacy of hybrid particles containing fasudil were evaluated in healthy rats and monocrotaline-induced PAH rats. CAR micelles containing fasudil had an entrapment efficiency of approximately 58%, showed controlled release of the drug, and were monodispersed with an average size of approximately 14 nm. Nuclear magnetic resonance scan confirmed the drug's presence in the core of peptide-micelle hybrid particles. Compared with plain micelles, CAR peptide increased the cellular uptake by approximately 1.7-fold and extended the drug half-life by approximately fivefold. The formulations were more prone to accumulate in the pulmonary vasculature than in the peripheral blood, which is evident from the ratio of the extent of reduction of pulmonary and systemic arterial pressures. On the whole, this study demonstrates that peptide-polymer hybrid micelles can serve as inhalational carriers for PAH therapy. © 2014 Wiley Periodicals, Inc. and the American Pharmacists Association.

  19. Management of Pulmonary Arterial Hypertension in Children

    NARCIS (Netherlands)

    Roofthooft, M. T. R.; van Loon, R. L. E.; Berger, R. M. F.

    2010-01-01

    In this review we discuss the new anti- Pulmonary Arterial Hypertension [PAH] drugs and the available data on their use in paediatric PAH. Treatment of patients with PAH, children and adults, is aimed at a reduction of symptoms, survival and improvement of haemodynamics as well as exercise capacity.

  20. Measurement of regional pulmonary blood volume in patients with increased pulmonary blood flow or pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Wollmer, P.; Rozcovek, A.; Rhodes, C.G.; Allan, R.M.; Maseri, A.

    1984-01-01

    The effects of chronic increase in pulmonary blood flow and chronic pulmonary hypertension on regional pulmonary blood volume was measured in two groups of patients. One group of patients had intracardiac, left-to-right shunts without appreciable pulmonary hypertension, and the other consisted of patients with Eisenmenger's syndrome or primary pulmonary hypertension, i.e. patients with normal or reduced blood flow and severe pulmonary hypertension. A technique based on positron tomography was used to measure lung density (by transmission scanning) and regional pulmonary blood volume (after inhalation of /sup 11/CO). The distribution of pulmonary blood volume was more uniform in patients with chronic increase in pulmonary blood flow than in normal subjects. There were also indications of an absolute increase in intrapulmonary blood volume by about 15%. In patients with chronic pulmonary arterial hypertension, the distribution of pulmonary blood volume was also abnormally uniform. There was, however, no indication that overall intrapulmonary blood volume was substantially different from normal subjects. The abnormally uniform distribution of pulmonary blood volume can be explained by recruitment and/or dilatation of vascular beds. Intrapulmonary blood volume appears to be increased in patients with intracardiac, left-to-right shunts. With the development of pulmonary hypertension, intrapulmonary blood volume falls, which may be explained by reactive changes in the vasculature and/or obliteration of capillaries

  1. [Pulmonary hypertensive crisis in children with idiopathic pulmonary arterial hypertension undergoing cardiac catheterization: the risk factors and clinical aspects].

    Science.gov (United States)

    Zhang, C; Zhu, Y; Li, Q Q; Gu, H

    2018-06-02

    Objective: To investigate the risk factors, clinical features, treatments, and prevention of pulmonary hypertensive crisis (PHC) in children with idiopathic pulmonary arterial hypertension (IPAH) undergoing cardiac catheterization. Methods: This retrospective study included 67 children who were diagnosed with IPAH and underwent cardiac catheterization between April 2009 and June 2017 in Beijing Anzhen Hospital. The medical histories, clinical manifestations, treatments, and outcomes were characterized. Statistical analyses were performed using t test, χ(2) test and a multiple Logistic regression analysis. Results: During cardiac catheterization, five children developed PHC who presented with markedly elevated pulmonary artery pressure and central venous pressure, decline in systemic arterial pressure and oxygen saturation. Heart rate decreased in 4 cases and increased in the remaining one. After the treatments including cardiopulmonary resuscitation, pulmonary vasodilator therapy, improving cardiac output and blood pressure, and correction of acidosis, 4 of the 5 cases recovered, while 1 died of severe right heart failure with irreversible PHC 3 days after operation. Potential PHC was considered in 7 other patients, whose pulmonary artery pressure increased and exceeded systemic arterial pressure, oxygen saturation decreased, and central venous pressure and vital signs were relatively stable. Univariate analysis showed that the risk factors of PHC in children with IPAH undergoing cardiac catheterization were younger age ( t= 3.160, P= 0.004), low weight ( t= 4.004, Phistory of syncope (χ(2)=4.948, P= 0.026), and WHO cardiac functional class Ⅲ or Ⅳ (χ(2)=19.013, Pcatheterization. WHO cardiac functional class may be associated with PHC. Integrated treatment is required for these patients. Reducing risk factors, early identification, and active treatment may help to prevent the occurrence and progression of PHC.

  2. Iron Deficiency in COPD Associates with Increased Pulmonary Artery Pressure Estimated by Echocardiography

    DEFF Research Database (Denmark)

    Plesner, Louis L; Schoos, Mikkel M; Dalsgaard, Morten

    2017-01-01

    OBJECTIVES: Iron deficiency (ID) might augment chronic pulmonary hypertension in chronic obstructive pulmonary disease (COPD). This observational study investigates the association between ID and systolic pulmonary artery pressure estimated by echocardiography in non-anaemic COPD outpatients...

  3. A comparative study of pulmonary hypoperfusion secondary to cancerous invasion of central pulmonary artery in lung cancer with CT and DSA

    International Nuclear Information System (INIS)

    Guo Dajing; Miao Jingtao; Lu Wusheng; Bai Hongli

    2003-01-01

    Objective: To study CT features of pulmonary hypoperfusion in lung cancer with cancerous invasion of central pulmonary artery (CICPA) and the diagnostic value of CT. Methods: The pre-operative pulmonary DSA and contrast-enhanced spiral CT (including HRCT) findings in 20 cases of lung cancer with CICPA were carried out prospectively and comparatively. On DSA and CT, the distribution and quantitative variation of subpleural terminal pulmonary arterioles (pulmonary vessel signs), the filling degree of lung capillary, the post-contrast density changes of lung parenchyma, and the fluctuations of the time-density curve (pulmonary parenchymal signs) were blindly observed. The CT and DSA findings and their consistence were analyzed statistically. The diagnostic values of CT in secondary pulmonary hypoperfusion in lung cancer with CICPA were evaluated. Results: There were 30 lobar arteries with cancerous invasion among the 20 cases and 90% (27/30) of them presented pulmonary hypoperfusion on DSA. For diagnosing pulmonary hypoperfusion, CT had a sensitivity of 78%(21/27), a specificity of 100% (3/3), and an accuracy of 80%(24/30). There were no statistical correlation between pulmonary hypoperfusion and the degree of central pulmonary artery (CPA) stenosis (r s =0.40, P=0.073). Pulmonary vessel signs on CT had a good consistence with that on DSA (Kappa=0.69, P<0.001), while that of pulmonary parenchymal signs was lower (Kappa=0.37, P=0.011). Conclusion: Pulmonary hypoperfusion is one of the common findings in lung cancer with CICPA. CT is of great value in qualitative diagnosis on it, while for quantitative diagnosis, its value is limited to a certain degree

  4. Primary intimal sarcoma of the pulmonary artery 1 case report and retrospective analysis of 20 cases published in China

    International Nuclear Information System (INIS)

    Guo Lingchuan; Chen Yanbin

    2012-01-01

    Objective: To explore the clinical and histopathological features of primary, intimal sarcoma of the pulmonary artery. Methods: One case of primary intimal sarcoma of the pulmonary artery diagnosed in our hospital and 20 cases published openly in China were analysed retrospectively. Results: The clinical presentation of primary intimal sarcoma of the pulmonary artery was progressive dyspnea, cough, chest pain, syncope, bloody phlegm, fever and fatigue. From the radiological finding, its signs were of pulmonary, artery dilation, reduced pulmonary vasculature and cardiomegaly, soft tissue mass near the right ventricle outflow tract. Histological examination of intimal sarcoma of the pulmonary artery revealed a poorly differentiated mesenchymal tumor of fibroblastic or myofibroblastic differentiation, consisting of mildly atypical spindle cells with atypia, mitosis, and nuclear polymorphism. Immunohistochemical analysis showed positive staining with antibodies against vimentin, alpha-smooth muscle actin, while negative for desmin. Conclusion: Primary, intimal sarcoma of the pulmonary artery has atypical clinical and radiological manifestation with poor prognosis, its definite diagnosis depends on histopathology. (authors)

  5. Percutaneous removal of pulmonary artery emboli with hydrolyser catheter in pigs

    International Nuclear Information System (INIS)

    Lacoursiere, L.; Millward, S.; Veinot, J.P.; Labinaz, M.

    2001-01-01

    To evaluate the efficacy and safety of the Hydrolyser catheter for per,cutaneous treatment of massive pulmonary embolism in pigs. Twelve pigs, each weighing between 55 kg and 89 kg, were used. Radio-opaque 9 cm x 0.8 cm and 4.5 cm x 0.8 cm clots, produced by mixing pig blood with iodinated contrast agent in vacutainers, were injected via the jugular vein until central pulmonary embolism (main and proximal lobar arteries) was obtained with significant systemic and pulmonary hemodynamic modifications. From a femoral approach, the 7-French Hydrolyser thrombectomy catheter was run over a 0.025-inch (0.64-mm) guide wire to remove the pulmonary emboli. Hemodynamic, gasometric and angiographic monitoring was performed before and after treatment. The procedure's safety and completeness of emboli removal was assessed by cardiopulmonary autopsy. Three of the 12 pigs died during embolization. Thrombectomy was therefore performed in 9, and central emboli could be obtained in 7 of the 9. The Hydrolyser could be manipulated only in central pulmonary arteries and could aspirate only central emboli in 5 of the 7 pigs that had them. Despite minimal angiographic improvement seen in these 5, there was no significant hemodynamic and gasometric improvement after treatment. The procedure induced an increase in free hemoglobin blood levels. Autopsies revealed an average of 2 endothelial injuries per pig (mainly adherent endocardial thrombi) in both nontreated (n = 3) and Hydrolyser-treated (n = 9) groups. The Hydrolyser thrombectomy catheter can be promptly positioned and easily steered in central pulmonary arteries. It can be used to partially remove central emboli, but not peripheral pulmonary emboli. Most of the injuries observed may not have been strictly related to Hydrolyser use. The pig might not be a suitable animal model for treatment of massive pulmonary embolism. (author)

  6. Echocardiographic assessment of systolic pulmonary arterial pressure in HIV-positive patients.

    Directory of Open Access Journals (Sweden)

    Mehrnaz Rasoulinejad

    2014-11-01

    Full Text Available Pulmonary hypertension is rare but is one of the complications that occur due to HIV infection. Symptoms of HIV-associated pulmonary arterial hypertension are often non-specific but the main symptom of the disease is dyspnea. In this cross-sectional study, we measured systolic pulmonary arterial pressure (SPAP by echocardiographic methods among HIV-positive patients who received ART. This research is a descriptive, cross-sectional study of 170 HIV-positive patients that was conducted in Imam-Khomeini hospital, Tehran, Iran during 2011-2013. All patients regularly received antiretroviral therapy at least for recent 2 years. There were not any cardiopulmonary symptoms (cough, dyspnea, exertional fatigue and chest discomfort in these patients. All participants underwent echocardiography to estimate SPAP. The participants comprised 108 males (63.5% and 62 females (46.5%. The mean age of patients was 41 years old, and the mean duration of HIV infection was 5.5 years. The mean CD4 cell count was 401 cell/µl. The principal regimen of antiretroviral therapy included two nucleoside reverse transcriptase inhibitor (NRTI and one non-nucleoside reverse transcriptase inhibitor (NNRTI in the hospital. The mean of systolic pulmonary arterial pressure was 25 mmHg in the participants; 156 (93.4% of them had SPAP ≤ 30 mmHg (normal, six (3.6% had SPAP: 31-35 mmHg (borderline and five (3% had SPAP > 35 mmHg (pulmonary hypertension. Our results indicated a significant increase of pulmonary hypertension in asymptomatic HIV-positive patients that had no association with any other risk factor. Also, antiretroviral therapy was not a risk factor for pulmonary hypertension in this study.

  7. Evaluation of the grading and disorder assessment of congenital heart disease with pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Ding Zhongru; Qin Yongwen

    2008-01-01

    Pulmonary arterial hypertension is one of the most common and serious complications in congenital heart disease. Identification of whether the pulmonary, arterial hypertension is dynamic or resistance remains as the great importance for deciding to transfer for surgery, intervention or conservative therapy and directly concerning with the prognosis and choice of treatment. This review mainly deals with the problems such as grading, staging, pathophysiology and the correlative mechanism with clinical assessment of pulmonary. arterial hypertension in congenital heart disease and furthermore providing comprehensive informations for clinical diagnosis and treatment. (authors)

  8. Noninvasive Doppler tissue measurement of pulmonary artery compliance in children with pulmonary hypertension.

    Science.gov (United States)

    Dyer, Karrie; Lanning, Craig; Das, Bibhuti; Lee, Po-Feng; Ivy, D Dunbar; Valdes-Cruz, Lilliam; Shandas, Robin

    2006-04-01

    We have shown previously that input impedance of the pulmonary vasculature provides a comprehensive characterization of right ventricular afterload by including compliance. However, impedance-based compliance assessment requires invasive measurements. Here, we develop and validate a noninvasive method to measure pulmonary artery (PA) compliance using ultrasound color M-mode (CMM) Doppler tissue imaging (DTI). Dynamic compliance (C(dyn)) of the PA was obtained from CMM DTI and continuous wave Doppler measurement of the tricuspid regurgitant velocity. C(dyn) was calculated as: [(D(s) - D(d))/(D(d) x P(s))] x 10(4); where D(s) = systolic diameter, D(d) = diastolic diameter, and P(s) = systolic pressure. The method was validated both in vitro and in 13 patients in the catheterization laboratory, and then tested on 27 pediatric patients with pulmonary hypertension, with comparison with 10 age-matched control subjects. C(dyn) was also measured in an additional 13 patients undergoing reactivity studies. Instantaneous diameter measured using CMM DTI agreed well with intravascular ultrasound measurements in the in vitro models. Clinically, C(dyn) calculated by CMM DTI agreed with C(dyn) calculated using invasive techniques (23.4 +/- 16.8 vs 29.1 +/- 20.6%/100 mm Hg; P = not significant). Patients with pulmonary hypertension had significantly lower peak wall velocity values and lower C(dyn) values than control subjects (P < .01). C(dyn) values followed an exponentially decaying relationship with PA pressure, indicating the nonlinear stress-strain behavior of these arteries. Reactivity in C(dyn) agreed with reactivity measured using impedance techniques. The C(dyn) method provides a noninvasive means of assessing PA compliance and should be useful as an additional measure of vascular reactivity subsequent to pulmonary vascular resistance in patients with pulmonary hypertension.

  9. Contrast Media Delivery in the Assessment of Anomalous Left Coronary Artery From the Pulmonary Artery.

    Science.gov (United States)

    Saade, Charbel; Al-Hamra, Salam; Al-Mohiy, Hussain; El-Merhi, Fadi

    2016-05-01

    A patient with a history of mitral valve prolapse and regurgitation that was corrected with a mitral ring repair 15 years earlier received a diagnosis of anomalous left coronary artery arising from the pulmonary artery and underwent repair. Coronary computed tomography angiography (CTA) was employed to image the patient before surgical intervention. Synchronizing contrast media administration to opacify the right coronary artery in the arterial phase and the left coronary artery in the venous phase required a test-bolus approach. Matching compromised cardiovascular dynamics with patient-specific contrast media administration protocols was improved considerably with the use of a test-bolus technique during electrocardiography-gated coronary CTA.

  10. Choice of marker for assessment of RV dysfunction in acute pulmonary embolism : NT-proBNP, pulmonary artery systolic pressure, mean arterial pressure, or blood pressure index.

    Science.gov (United States)

    Ates, H; Ates, I; Kundi, H; Yilmaz, F M

    2017-12-01

    We aimed to examine the value of NT-proBNP, pulmonary artery systolic pressure (PASP), blood pressure index (BPI), and mean arterial pressure (MAP) in the determination of right ventricular dysfunction (RVD) in patients with acute pulmonary embolism (APE). A total of 547 patients diagnosed with APE were included in the study. Demographic characteristics and comorbid conditions of patients were recorded in patient files. For blood pressure measurement, a calibrated digital blood pressure monitor was used at regular intervals. Blood samples were taken from patients at the time of admission for hemogram, biochemical, and hemostasis blood tests. Echocardiography was performed on all patients to detect RVD and evaluate pulmonary artery pressure. PASP (p blood pressure (p blood cell (p AUC ± SE = 0.975 ± 0.006; p < 0.001) was found to be the best predictor of RVD with a higher sensitivity (92.8%) and specificity (100%). We found that BPI had a better diagnostic discrimination for RVD compared with PASP and NT-proBNP.

  11. Compression of the Right Pulmonary Artery by a Massive Defects on Pulmonary Scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Makis, William [Brandon Regional Health Centre, Brandon (Canada); Derbekyan, Vilma [McGill Univ. Health Centre, Montreal (Canada)

    2012-03-15

    A 67 year old woman, who presented with a 2 month history of dyspnea, had a vectilation and perfusion lung scan that showed absent perfusion of the entire right lung scan that showed absent perfusion of the entire right lung with normal ventilation, as well as a rounded matched defect in the left lower lung adjacent to mialine, suspicious for an aortic aneurysm or dissection. CT pulmonary angiography revealed a massive descending aortic aneurysm compressing the right pulmonary artery as well as the left lung parenchyma, accounting for the bilateral perfusion scan defects. We present the Xe 133 ventilation, Tc 99m MAA perfusion and CT pulmonary angiography imaging findings of this rare case.

  12. Relationship of daily arterial blood pressure monitoring readings and arterial stiffness profile in male patients with chronic obstructive pulmonary disease combined with arterial hypertension

    Directory of Open Access Journals (Sweden)

    Karoli N.A.

    2013-06-01

    Full Text Available The aim of the study was to determine correlation between arterial blood pressure daily rhythm and daily profile of arterial stiffness in male patients with chronic obstructive pulmonary disease (COPD and arterial hypertension. Materials et methods: Prospective investigation comprised 45 male patients with COPD and arterial hypertension. Individuals of 40 years younger and 80 years elder, patients with diabetes, stroke, angina pectoris, or heart infarction, vascular diseases, and exacerbation of chronic disease, bronchial and pulmonary diseases of other etiology were excluded from the analyses. Comparison group included 47 patients with essential arterial hypertension and without chronic respiratory diseases closely similar on general parameters with patients from main clinical series. Twenty-four-hour arterial blood pressure monitoring (ABPM and daily arterial stiffness monitoring were performed using BPLab® MnSDP-2 apparatus (Petr Telegin, Russian Federation. Results: Patients with COPD combined with arterial hypertension with raised arterial stiffness measures prevail over individuals in essential hypertension group. There is pathological alteration of the ABPM circadian rhythm and raised «Pressure load» values in raised arterial stiffness group. Conclusion: We found ABPM raised parameters in patients with COPD and arterial hypertension. It confirms necessity of ABPM in daily arterial stiffness assessment in patients with COPD.

  13. Aortic homograft for pulmonary artery augmentation in single lung transplantation.

    Science.gov (United States)

    Rueda, Pablo; Morales, Jose; Guzman, Enrique; Tellez, Jose L; Niebla, Benito A; Avalos, Alejandro; Patiño, Hilda

    2005-06-01

    We present a case of unilateral lung transplantation in which a segment of the donor's descending aorta was used as a homograft for pulmonary artery augmentation in the donor lung. This technique can be used when the donor's lung artery has been cut at the base of the hilum during the harvesting procedure.

  14. Congenital coronary-pulmonary artery fistula originating from right and left coronary artery

    Directory of Open Access Journals (Sweden)

    Ali Kemal Gür

    2013-12-01

    Full Text Available Coronary artery fistula (CAF is a rare congenital anomalywith an incidence of 1 in 50 000 live births. The fistula wasobserved at the right coronary artery in 53%, the left coronaryartery in 42% and both coronary artery in 5% of thecases. Echocardiography examination in a 46 year-oldwoman with the symptoms of chest pain, palpitation anddyspnea revealed a severe mitral valve insufficiency anda moderate to severe tricuspid valve insufficiency. A CAForiginating from the proximal part of the left anterior descendingartery (LAD and another fistula originating fromosteal part of the right coronary artery (RCA were detectedby coronary angiography. Both fistulas were draininginto the main pulmonary artery. The coronary artery fistulaclosed under cardiopulmonary by-pass. Mitral insufficiencyoriginated from the posterior leaflet was diagnosedintra-operative exploration, and thereafter it was repairedwith mitral annuloplasty including a quadrangular resectionand use of a 32 No St Jude mitral ring. Tricuspid valvewas repaired with Calangos Ring annuloplasty. Followingsix day hospital stay, the woman was discharged free ofany symptom.Key words: Dyspnea, double arteriovenous fistula, mitral and tricuspid insufficiency

  15. Roles of preoperative arterial blood gas tests in the surgical treatment of scoliosis with moderate or severe pulmonary dysfunction.

    Science.gov (United States)

    Liu, Jia-Ming; Shen, Jian-Xiong; Zhang, Jian-Guo; Zhao, Hong; Li, Shu-Gang; Zhao, Yu; Qiu, Giu-Xing

    2012-01-01

    It has been stated that preoperative pulmonary function tests are essential to assess the surgical risk in patients with scoliosis. Arterial blood gas tests have also been used to evaluate pulmonary function before scoliotic surgery. However, few studies have been reported. The aim of this study was to investigate the roles of preoperative arterial blood gas tests in the surgical treatment of scoliosis with moderate or severe pulmonary dysfunction. This study involved scoliotic patients with moderate or severe pulmonary dysfunction (forced vital capacity treatment between January 2002 and April 2010. A total of 73 scoliotic patients (23 males and 50 females) with moderate or severe pulmonary dysfunction were included. The average age of the patients was 16.53 years (ranged 10 - 44). The demographic distribution, medical records, and radiographs of all patients were collected. All patients received arterial blood gas tests and pulmonary function tests before surgery. The arterial blood gas tests included five parameters: partial pressure of arterial oxygen, partial pressure of arterial carbon dioxide, alveolar-arterial oxygen tension gradient, pH, and standard bases excess. The pulmonary function tests included three parameters: forced expiratory volume in 1 second ratio, forced vital capacity ratio, and peak expiratory flow ratio. All five parameters of the arterial blood gas tests were compared between the two groups with or without postoperative pulmonary complications by variance analysis. Similarly, all three parameters of the pulmonary function tests were compared. The average coronal Cobb angle before surgery was 97.42° (range, 50° - 180°). A total of 15 (20.5%) patients had postoperative pulmonary complications, including hypoxemia in 5 cases (33.3%), increased requirement for postoperative ventilatory support in 4 (26.7%), pneumonia in 2 (13.3%), atelectasis in 2 (13.3%), pneumothorax in 1 (6.7%), and hydrothorax in 1 (6.7%). No significant differences

  16. Pulmonary Arterial Stiffness: Toward a New Paradigm in Pulmonary Arterial Hypertension Pathophysiology and Assessment.

    Science.gov (United States)

    Schäfer, Michal; Myers, Cynthia; Brown, R Dale; Frid, Maria G; Tan, Wei; Hunter, Kendall; Stenmark, Kurt R

    2016-01-01

    Stiffening of the pulmonary arterial bed with the subsequent increased load on the right ventricle is a paramount feature of pulmonary hypertension (PH). The pathophysiology of vascular stiffening is a complex and self-reinforcing function of extracellular matrix remodeling, driven by recruitment of circulating inflammatory cells and their interactions with resident vascular cells, and mechanotransduction of altered hemodynamic forces throughout the ventricular-vascular axis. New approaches to understanding the cell and molecular determinants of the pathophysiology combine novel biopolymer substrates, controlled flow conditions, and defined cell types to recapitulate the biomechanical environment in vitro. Simultaneously, advances are occurring to assess novel parameters of stiffness in vivo. In this comprehensive state-of-art review, we describe clinical hemodynamic markers, together with the newest translational echocardiographic and cardiac magnetic resonance imaging methods, to assess vascular stiffness and ventricular-vascular coupling. Finally, fluid-tissue interactions appear to offer a novel route of investigating the mechanotransduction processes and disease progression.

  17. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with congenital heart disease

    Directory of Open Access Journals (Sweden)

    Antonio Lopes

    2014-01-01

    Full Text Available Congenital heart disease (CHD with intracardiac/extracardiac shunts is an important etiology of pulmonary arterial hypertension (PAH. The majority of children with congenital cardiac shunts do not develop advanced pulmonary vasculopathy, as surgical repair of the anomalies is now performed early in life. However, if not repaired early, some defects will inevitably lead to pulmonary vascular disease (truncus arteriosus, transposition of the great arteries associated with a ventricular septal defect (VSD, atrioventricular septal defects remarkably in Down syndrome, large, nonrestrictive VSDs, patent ductus arteriosus and related anomalies. The majority of patients are now assigned to surgery based on noninvasive evaluation only. PAH becomes a concern (requiring advanced diagnostic procedures in about 2-10% of them. In adults with CHD, the prevalence of advanced pulmonary vasculopathy (Eisenmenger syndrome is around 4-12%. [1] This article will discuss the diagnostic and management approach for PAH associated with CHD (PAH-CHD.

  18. [Right branch pulmonary artery stenosis with supravalvar aortic stenosis as a complication of Lecompte maneuver for tetralogy of Fallot associated with absent pulmonary valve].

    Science.gov (United States)

    Honda, Yoshihiro; Suzuki, Shoji; Kaga, Shigeaki; Yoshida, Yukiyo; Kimura, Mitsuhiro; Kamiya, Kentaro; Sakakibara, Kenji; Katsu, Masatake

    2015-05-01

    The patient was diagnosed with tetralogy of Fallot associated with absent pulmonary valve syndrome and a low birth weight of 1,912 g. He suffered from respiratory distress on day 14 and received non-invasive positive pressure ventilation. At 5 months of age and 4.1 kg, he underwent intracardiac repair including right ventricular outflow repair with a monocusp patch, patch closure of the ventricular septum defect and right pulmonary transposition to the anterior of the ascending aorta following the Lecompte maneuver for airway decompression. He was subsequently discharged to home and exhibited an uneventful clinical course with non-invasive positive pressure ventilation for 5 months postoperatively. However, right pulmonary artery and supra-aortic stenosis was noted 2 years after the operation. Computed tomography (CT) and angiography showed ascending aorta strangulation by the right pulmonary artery with right ventricular outflow regurgitation. Right pulmonary artery reconstruction using polytetrafluoroethylene graft interposition and repeat right ventricular outflow repair with bicuspid hand-sewn valves was therefore performed;the postoperative course was uneventful. Pre- and post-operative management using non-invasive positive pressure ventilation and airway decompression with pulmonary artery translocation is a useful strategy in patients exhibiting symptomatic tetralogy of Fallot associated with absent pulmonary valve syndrome in the neonatal period.

  19. Mean pressure of pulmonary arteries in non-callous silicosis at states 1 to 2 as determined by radiology in comparison to parameters of pulmonary function

    Energy Technology Data Exchange (ETDEWEB)

    Jakob, M; Konrad, A; Adam, P; Scharf, R

    1984-11-19

    Fifty coal miners suffering from reticulonodular silicosis underwent catheterization of the right heart and showed latent or manifest pulmonary hypertension. It has been found that the statistical relationship between pulmonary arterial pressure and pulmonary function was only a loose one. Thirty percent of the studied subjects presented no alterations of the lung function but an increased pulmonary artery mean pressure at rest and under exercise, indicating that the Euler-Liljestrand-mechanism could hardly be responsible for these pressure changes. Subjects with silicosis who presented impaired lung function showed statistically significant higher pulmonary artery pressures than those without alteration of the lung function. It is likely that an additional pressure increase occurs secondary to Euler-Liljestrand-mechanism, when restrictive or obstructive impairments of ventilation emerge from the pneumoconiosis.

  20. Multifocal central serous chorioretinopathy with photoreceptor-retinal pigment epithelium diastasis in heritable pulmonary arterial hypertension

    DEFF Research Database (Denmark)

    Li, Xiao Qiang; Pryds, Anders; Carlsen, Jørn

    2015-01-01

    PURPOSE: To report atypical central serous chorioretinopathy and choroidal thickening in a patient with heritable pulmonary arterial hypertension. METHODS: A 40-year-old man with heritable pulmonary arterial hypertension presented with blurred vision in his left eye and was followed up for 1 year...

  1. Liposomal Fasudil, a Rho-Kinase Inhibitor, for Prolonged Pulmonary Preferential Vasodilation in Pulmonary Arterial Hypertension

    Science.gov (United States)

    Gupta, Vivek; Gupta, Nilesh; Shaik, Imam H.; Mehvar, Reza; McMurtry, Ivan F.; Oka, Masahiko; Nozik-Grayck, Eva; Komatsu, Masanobu; Ahsan, Fakhrul

    2013-01-01

    Current pharmacological interventions for pulmonary arterial hypertension (PAH) require continuous infusions, multiple inhalations, or oral administration of drugs that act on various pathways involved in the pathogenesis of PAH. However, invasive methods of administration, short duration of action, and lack of pulmonary selectivity result in noncompliance and poor patient outcomes. In this study, we tested the hypothesis that encapsulation of an investigational anti-PAH molecule fasudil (HA-1077), a Rho-kinase inhibitor, into liposomal vesicles results in prolonged vasodilation in distal pulmonary arterioles. Liposomes were prepared by hydration and extrusion method and fasudil was loaded by ammonium sulfate-induced transmembrane electrochemical gradient. Liposomes were then characterized for various physicochemical properties. Optimized formulations were tested for pulmonary absorption and their pharmacological efficacy in a monocrotaline (MCT) induced rat model of PAH. The entrapment efficiency of optimized liposomal fasudil formulations was between 68.1±0.8% and 73.6±2.3%, and the cumulative release at 37°C was 98–99% over a period of 5 days. Compared to intravenous (IV) fasudil, a ~10 fold increase in the terminal plasma half-life was observed when liposomal fasudil was administered as aerosols. The t1/2 of IV fasudil was 0.39±0.12 h. and when given as liposomes via pulmonary route, the t1/2 extended to 4.71±0.72 h. One h after intratracheal instillation of liposomal fasudil, mean pulmonary arterial pressure (MPAP) was reduced by 37.6±5.7% and continued to decrease for about 3 h, suggesting that liposomal formulations produced pulmonary preferential vasodilation in MCT induced PAH rats. Overall, this study established the proof-of-principle that aerosolized liposomal fasudil is a feasible option for a non-invasive, controlled release and pulmonary preferential treatment of PAH. PMID:23353807

  2. Plasma proteome analysis in patients with pulmonary arterial hypertension: an observational cohort study.

    Science.gov (United States)

    Rhodes, Christopher J; Wharton, John; Ghataorhe, Pavandeep; Watson, Geoffrey; Girerd, Barbara; Howard, Luke S; Gibbs, J Simon R; Condliffe, Robin; Elliot, Charles A; Kiely, David G; Simonneau, Gerald; Montani, David; Sitbon, Olivier; Gall, Henning; Schermuly, Ralph T; Ghofrani, H Ardeschir; Lawrie, Allan; Humbert, Marc; Wilkins, Martin R

    2017-09-01

    Idiopathic and heritable pulmonary arterial hypertension form a rare but molecularly heterogeneous disease group. We aimed to measure and validate differences in plasma concentrations of proteins that are associated with survival in patients with idiopathic or heritable pulmonary arterial hypertension to improve risk stratification. In this observational cohort study, we enrolled patients with idiopathic or heritable pulmonary arterial hypertension from London (UK; cohorts 1 and 2), Giessen (Germany; cohort 3), and Paris (France; cohort 4). Blood samples were collected at routine clinical appointment visits, clinical data were collected within 30 days of blood sampling, and biochemical data were collected within 7 days of blood sampling. We used an aptamer-based assay of 1129 plasma proteins, and patient clinical details were concealed to the technicians. We identified a panel of prognostic proteins, confirmed with alternative targeted assays, which we evaluated against the established prognostic risk equation for pulmonary arterial hypertension derived from the REVEAL registry. All-cause mortality was the primary endpoint. 20 proteins differentiated survivors and non-survivors in 143 consecutive patients with idiopathic or heritable pulmonary arterial hypertension with 2 years' follow-up (cohort 1) and in a further 75 patients with 2·5 years' follow-up (cohort 2). Nine proteins were both prognostic independent of plasma NT-proBNP concentrations and confirmed by targeted assays. The functions of these proteins relate to myocardial stress, inflammation, pulmonary vascular cellular dysfunction and structural dysregulation, iron status, and coagulation. A cutoff-based score using the panel of nine proteins provided prognostic information independent of the REVEAL equation, improving the C statistic from area under the curve 0·83 (for REVEAL risk score, 95% CI 0·77-0·89; parterial hypertension in cohort 3 (p=0·0133). The protein panel was validated in 93 patients

  3. Management of massive hemoptysis in pulmonary tuberculosis and bronchiectasis by bronchial arterial embolization

    International Nuclear Information System (INIS)

    Fan Yong; Yin Baoquan; Han Bingsen; He Nengshu

    2005-01-01

    Objective: To probe into the angiographic signs and the variations of bronchial arteries for pulmonary tuberculosis or bronchiectasis with massive hemoptysis. Methods: 25 patients with pulmonary tuberculosis and 15 patients suffered from bronchiectasis accompanied by massive hemoptysis were undertaken bronchial arterial embolization (BAE). All patients were embolized with gelfoam including 32 with spring coils in addition. Results: 63 arteries demonstrated angiographic signs of hemoptysis in 40 patients. The immediate stanching rate was 92.5%(37/40). The bronchopulmonary shunt formation sign shown by angiograph was the major feature of tuberculosis (P=0.0528) and the enlarged tortuous arteries in bronchiectasis were more to be demonstrated than in tuberculosis (P<0.05). Conclusions: The BAE for patients with tuberculosis ought to be performed in the smaller arteries. BAE for patients with bronchiectasis should to be taken in the trunk of arteries. (authors)

  4. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    International Nuclear Information System (INIS)

    Griffin, N.; Mansfield, L.; Redmond, K.C.; Dusmet, M.; Goldstraw, P.; Mittal, T.K.; Padley, S.

    2007-01-01

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution

  5. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Mansfield, L. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Redmond, K.C. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Dusmet, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Goldstraw, P. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Mittal, T.K. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-03-15

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

  6. Pleiotropic effects of statins in distal human pulmonary artery smooth muscle cells

    Directory of Open Access Journals (Sweden)

    Butrous Ghazwan S

    2011-10-01

    Full Text Available Abstract Background Recent clinical data suggest statins have transient but significant effects in patients with pulmonary arterial hypertension. In this study we explored the molecular effects of statins on distal human pulmonary artery smooth muscle cells (PASMCs and their relevance to proliferation and apoptosis in pulmonary arterial hypertension. Methods Primary distal human PASMCs from patients and controls were treated with lipophilic (simvastatin, atorvastatin, mevastatin and fluvastatin, lipophobic (pravastatin and nitric-oxide releasing statins and studied in terms of their DNA synthesis, proliferation, apoptosis, matrix metalloproteinase-9 and endothelin-1 release. Results Treatment of human PASMCs with selected statins inhibited DNA synthesis, proliferation and matrix metalloproteinase-9 production in a concentration-dependent manner. Statins differed in their effectiveness, the rank order of anti-mitogenic potency being simvastatin > atorvastatin > > pravastatin. Nevertheless, a novel nitric oxide-releasing derivative of pravastatin (NCX 6550 was effective. Lipophilic statins, such as simvastatin, also enhanced the anti-proliferative effects of iloprost and sildenafil, promoted apoptosis and inhibited the release of the mitogen and survival factor endothelin-1. These effects were reversed by mevalonate and the isoprenoid intermediate geranylgeranylpyrophosphate and were mimicked by inhibitors of the Rho and Rho-kinase. Conclusions Lipophilic statins exert direct effects on distal human PASMCs and are likely to involve inhibition of Rho GTPase signalling. These findings compliment some of the recently documented effects in patients with pulmonary arterial hypertension.

  7. Computational Fluid Dynamics Modeling of the Human Pulmonary Arteries with Experimental Validation.

    Science.gov (United States)

    Bordones, Alifer D; Leroux, Matthew; Kheyfets, Vitaly O; Wu, Yu-An; Chen, Chia-Yuan; Finol, Ender A

    2018-05-21

    Pulmonary hypertension (PH) is a chronic progressive disease characterized by elevated pulmonary arterial pressure, caused by an increase in pulmonary arterial impedance. Computational fluid dynamics (CFD) can be used to identify metrics representative of the stage of PH disease. However, experimental validation of CFD models is often not pursued due to the geometric complexity of the model or uncertainties in the reproduction of the required flow conditions. The goal of this work is to validate experimentally a CFD model of a pulmonary artery phantom using a particle image velocimetry (PIV) technique. Rapid prototyping was used for the construction of the patient-specific pulmonary geometry, derived from chest computed tomography angiography images. CFD simulations were performed with the pulmonary model with a Reynolds number matching those of the experiments. Flow rates, the velocity field, and shear stress distributions obtained with the CFD simulations were compared to their counterparts from the PIV flow visualization experiments. Computationally predicted flow rates were within 1% of the experimental measurements for three of the four branches of the CFD model. The mean velocities in four transversal planes of study were within 5.9 to 13.1% of the experimental mean velocities. Shear stresses were qualitatively similar between the two methods with some discrepancies in the regions of high velocity gradients. The fluid flow differences between the CFD model and the PIV phantom are attributed to experimental inaccuracies and the relative compliance of the phantom. This comparative analysis yielded valuable information on the accuracy of CFD predicted hemodynamics in pulmonary circulation models.

  8. ITE inhibits growth of human pulmonary artery endothelial cells.

    Science.gov (United States)

    Pang, Ling-Pin; Li, Yan; Zou, Qing-Yun; Zhou, Chi; Lei, Wei; Zheng, Jing; Huang, Shi-An

    2017-10-01

    Pulmonary arterial hypertension (PAH), a deadly disorder is associated with excessive growth of human pulmonary artery endothelial (HPAECs) and smooth muscle (HPASMCs) cells. Current therapies primarily aim at promoting vasodilation, which only ameliorates clinical symptoms without a cure. 2-(1'H-indole-3'-carbonyl)-thiazole-4-carboxylic acid methyl ester (ITE) is an endogenous aryl hydrocarbon receptor (AhR) ligand, and mediates many cellular function including cell growth. However, the roles of ITE in human lung endothelial cells remain elusive. Herein, we tested a hypothesis that ITE inhibits growth of human pulmonary artery endothelial cells via AhR. Immunohistochemistry was performed to localize AhR expression in human lung tissues. The crystal violet method and MTT assay were used to determine ITE's effects on growth of HPAECs. The AhR activation in HPAECs was confirmed using Western blotting and RT-qPCR. The role of AhR in ITE-affected proliferation of HPAECs was assessed using siRNA knockdown method followed by the crystal violet method. Immunohistochemistry revealed that AhR was present in human lung tissues, primarily in endothelial and smooth muscle cells of pulmonary veins and arteries, as well as in bronchial and alveolar sac epithelia. We also found that ITE dose- and time-dependently inhibited proliferation of HPAECs with a maximum inhibition of 83% at 20 µM after 6 days of treatment. ITE rapidly decreased AhR protein levels, while it increased mRNA levels of cytochrome P450 (CYP), family 1, member A1 (CYP1A1) and B1 (CYP1B1), indicating activation of the AhR/CYP1A1 and AhR/CYP1B1 pathways in HPAECs. The AhR siRNA significantly suppressed AhR protein expression, whereas it did not significantly alter ITE-inhibited growth of HPAECs. ITE suppresses growth of HPAECs independent of AhR, suggesting that ITE may play an important role in preventing excessive growth of lung endothelial cells.

  9. Selexipag for the Treatment of Pulmonary Arterial Hypertension

    DEFF Research Database (Denmark)

    Sitbon, Olivier; Channick, Richard; Chin, Kelly M

    2015-01-01

    BACKGROUND: In a phase 2 trial, selexipag, an oral selective IP prostacyclin-receptor agonist, was shown to be beneficial in the treatment of pulmonary arterial hypertension. METHODS: In this event-driven, phase 3, randomized, double-blind, placebo-controlled trial, we randomly assigned 1156 pati...

  10. Flash pulmonary edema in patients with renal artery stenosis--the Pickering Syndrome

    DEFF Research Database (Denmark)

    Pelta, Anna; Andersen, Ulrik B; Just, Sven

    2010-01-01

    We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases.......We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases....

  11. A unique case of pulmonary artery catheter bleeding from the oximetry connection port

    Directory of Open Access Journals (Sweden)

    Suman Rajagopalan

    2014-12-01

    Full Text Available Pulmonary artery catheter is an invasive monitor usually placed in high-risk cardiac surgical patients to optimize the cardiac functions. We present this case of blood oozing from the oximetry connection port of the pulmonary artery catheter that resulted in the inability to monitor continuous cardiac output requiring replacement of the catheter. The cause of this abnormal bleeding was later confirmed to be due to a manufacturing defect.

  12. Liposomal nanoparticles encapsulating iloprost exhibit enhanced vasodilation in pulmonary arteries

    Directory of Open Access Journals (Sweden)

    Jain PP

    2014-07-01

    Full Text Available Pritesh P Jain,1 Regina Leber,1,2 Chandran Nagaraj,1 Gerd Leitinger,3 Bernhard Lehofer,4 Horst Olschewski,1,5 Andrea Olschewski,1,6 Ruth Prassl,1,4 Leigh M Marsh11Ludwig Boltzmann Institute for Lung Vascular Research, 2Biophysics Division, Institute of Molecular Biosciences, University of Graz, 3Research Unit Electron Microscopic Techniques, Institute of Cell Biology, Histology, and Embryology, 4Institute of Biophysics, 5Division of Pulmonology, Department of Internal Medicine, 6Department of Anesthesiology and Intensive Care Medicine, Medical University of Graz, Graz, AustriaAbstract: Prostacyclin analogues are standard therapeutic options for vasoconstrictive diseases, including pulmonary hypertension and Raynaud’s phenomenon. Although effective, these treatment strategies are expensive and have several side effects. To improve drug efficiency, we tested liposomal nanoparticles as carrier systems. In this study, we synthesized liposomal nanoparticles tailored for the prostacyclin analogue iloprost and evaluated their pharmacologic efficacy on mouse intrapulmonary arteries, using a wire myograph. The use of cationic lipids, stearylamine, or 1,2-di-(9Z-octadecenoyl-3-trimethylammonium-propane (DOTAP in liposomes promoted iloprost encapsulation to at least 50%. The addition of cholesterol modestly reduced iloprost encapsulation. The liposomal nanoparticle formulations were tested for toxicity and pharmacologic efficacy in vivo and ex vivo, respectively. The liposomes did not affect the viability of human pulmonary artery smooth muscle cells. Compared with an equivalent concentration of free iloprost, four out of the six polymer-coated liposomal formulations exhibited significantly enhanced vasodilation of mouse pulmonary arteries. Iloprost that was encapsulated in liposomes containing the polymer polyethylene glycol exhibited concentration-dependent relaxation of arteries. Strikingly, half the concentration of iloprost in liposomes elicited

  13. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

    International Nuclear Information System (INIS)

    Greenberg, S.B.; Crisci, K.L.; Koenig, P.; Robinson, B.; Anisman, P.; Russo, P.

    1997-01-01

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.)

  14. Decline in arterial partial pressure of oxygen after exercise: a surrogate marker of pulmonary vascular obstructive disease in patients with atrial septal defect and severe pulmonary hypertension.

    Science.gov (United States)

    Laksmivenkateshiah, Srinivas; Singhi, Anil K; Vaidyanathan, Balu; Francis, Edwin; Karimassery, Sundaram R; Kumar, Raman K

    2011-06-01

    To examine the utility of decline in arterial partial pressure of oxygen after exercise as a marker of pulmonary vascular obstructive disease in patients with atrial septal defect and pulmonary hypertension. Treadmill exercise was performed in 18 patients with atrial septal defect and pulmonary hypertension. Arterial blood gas samples were obtained before and after peak exercise. A decline in the arterial pressure of oxygen of more than 10 millimetres of mercury after exercise was considered significant based on preliminary tests conducted on the controls. Cardiac catheterisation was performed in all patients and haemodynamic data sets were obtained on room air, oxygen, and a mixture of oxygen and nitric oxide (30-40 parts per million). There were 10 patients who had more than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise and who had a basal pulmonary vascular resistance index of more than 7 Wood units per square metre. Out of eight patients who had less than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise, seven had a basal pulmonary vascular resistance index of less than 7 Wood units per square metre, p equals 0.0001. A decline in arterial partial pressure of oxygen of more than 10 millimetres of mercury predicted a basal pulmonary vascular resistance index of more than 7 Wood units per square metre with a specificity of 100% and a sensitivity of 90%. A decline in arterial partial pressure of oxygen following exercise appears to predict a high pulmonary vascular resistance index in patients with atrial septal defect and pulmonary hypertension. This test is a useful non-invasive marker of pulmonary vascular obstructive disease in this subset.

  15. In vitro and in vivo studies of pulmonary artery flow

    International Nuclear Information System (INIS)

    Sahn, D.J.; Yoganathan, A.P.

    1986-01-01

    A variety of interesting intracardiac flow patterns have been recorded by pulsed and continuous wave Doppler technologies in humans with heart disease. Some of these patterns have, in fact, been difficult to explain and are now more easily understood using color Doppler flow mapping systems which show the spatial location of flow. The authors performed a number of studies in patients, as well as studies in in vitro systems to model some of the phenomenon that the authors observed in the pulmonary artery. Their studies with Doppler flow mapping in the clinical situation, in the in vitro model, and in the animal models of congenital heart disorders lend insights into the complex hydrodynamics present in the pulmonary artery

  16. Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report

    Science.gov (United States)

    2012-01-01

    Abstract Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall or by histologic type. Angiosarcomas are malignant neoplasms that arise from the endothelial lining of the blood vessels; those arising in the intimal compartment of pulmonary artery are rare. We report a case of pulmonary artery angiosarcoma in a 36-year old female with pulmonary masses. The patient had no other primary malignant neoplasm, thus excluding a metastatic lesion. Gross examination revealed a thickened right pulmonary artery and a necrotic and hemorrhagic tumor, filling and occluding the vascular lumen. The mass extended distally, within the pulmonary vasculature of the right lung. Microscopically, an intravascular undifferentiated tumor was identified. The tumor cells showed expression for vascular markers VEGFR, VEGFR3, PDGFRa, FGF, Ulex europaeus, FVIII, FLI-1, CD31 and CD34; p53 was overexpressed and Ki67 proliferative rate was increased. Intravascular angiosarcomas are aggressive neoplasms, often associated with poor outcome. Virtual slide The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2315906377648045. PMID:23134683

  17. Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?

    Science.gov (United States)

    Sinha, Santosh Kumar; Khanra, Dibbendhu; Jha, Mukesh Jitendra; Singh, Karandeep; Razi, Mahamdulla; Goel, Amit; Mishra, Vikas; Asif, Mohammad; Sachan, Mohit; Afdaali, Nasar; Kumar, Ashutosh; Thakur, Ramesh; Krishna, Vinay; Pandey, Umeshwar; Varma, Chandra Mohan

    2016-10-01

    ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery) is a rare disease but lethal with clinical expression from myocardial infarction, congestive heart failure to death during early infancy and unusual survival to adulthood. We report a 73-year-old woman with ALCAPA who presented with exertional dyspnea (NYHA functional class II) over past 2 years. Physical examination revealed soft S, long mid diastolic rumbling murmur and apical pan-systolic murmur. Electrocardiography displayed biatrial enlargement and poor R progression and normal sinus rhythm. Echocardiography established calcified severe mitral stenosis (MS), presence of continuous flow entering the pulmonary trunk, turbulent continuous flow in inter-ventricular septum with left to right shunt in contrast echocardiography and normal systolic function. Coronary angiogram showed absence of left coronary artery (LCA) originating from aorta, dilated and tortuous right coronary artery (RCA) and abundant Rentrop grade 3 intercoronary collateral communicating with LCA originating from pulmonary trunk which was also confirmed on coronary CT angiogram thus establishing diagnosis of ALCAPA. It is exceedingly rare to be associated with severe MS. However, such a long survival in our patient can be explained by the severe pulmonary arterial hypertension which may be contributing to lesser coronary steal.

  18. A Rare Case of Pulmonary Artery Sling with the VACTERL Association in a 20-Month-Old Infant.

    Science.gov (United States)

    Ghandi, Yazdan; Shafiee, Akbar; Sharifi, Mehrazad; Bolandnazar, Najmeh Sadat

    2017-07-01

    The VACTERL association, co-occurrence of vertebral, anorectal, cardiac, tracheoesophageal, genitourinary, and limb malformations, is a rare congenital anomaly. Several cardiac anomalies have been reported as a part of the VACTERL association, particularly ventricular and atrial septal defects. Pulmonary artery sling is a rare congenital abnormality in which the left pulmonary artery arises from the right pulmonary artery. This anomaly is not frequently observed in the VACTERL association and has been rarely reported. A 20-month-old girl was admitted to our hospital due to pneumonia in the right lung, which had pulmonary artery sling as a part of the VACTERL association. Barium meal X-ray showed pressure effects on the esophagus, and computed tomography angiography revealed pulmonary artery sling. Pneumonia management was done. However, the parents of our patient refused to give consent for the surgical correction of this vascular anomaly. Three months after discharge from the hospital, the patient was visited, at which time the parents again refused surgery and treatment for their daughter despite our recommendations.

  19. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

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    Greenberg, S.B.; Crisci, K.L.; Koenig, P.; Robinson, B.; Anisman, P.; Russo, P. [St. Christopher`s Hospital for Children, Front Street at Erie Avenue, Philadelphia, PA 19134 (United States)

    1997-12-01

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.) With 2 figs., 3 tabs., 11 refs.

  20. Role of lipoxygenase metabolites of arachidonic acid in enhanced pulmonary artery contractions of female rabbits.

    Science.gov (United States)

    Pfister, Sandra L

    2011-04-01

    Pulmonary arterial hypertension is characterized by elevated pulmonary artery pressure and vascular resistance. In women the incidence is 4-fold greater than that in men. Studies suggest that sustained vasoconstriction is a factor in increased vascular resistance. Possible vasoconstrictor mediators include arachidonic acid-derived lipoxygenase (LO) metabolites. Our studies in rabbits showed enhanced endothelium-dependent contractions to arachidonic acid in pulmonary arteries from females compared with males. Because treatment with a nonspecific LO inhibitor reduced contractions in females but not males, the present study identified which LO isoform contributes to sex-specific pulmonary artery vasoconstriction. The 15- and 5- but not 12-LO protein expressions were greater in females. Basal and A23187-stimulated release of 15-, 5-, and 12-hydroxyeicosatetraenoic acids (HETEs) from females and males were measured by liquid chromatography/mass spectrometry. Only 15-HETE synthesis was greater in females compared with males under both basal and stimulated conditions. Vascular contractions to 15-HETE were enhanced in females compared with males (maximal contraction: 44±6%versus 25±3%). The specific 15-LO inhibitor PD146176 (12 μmol/L) decreased arachidonic acid-induced contractions in females (maximal contraction: 93±4% versus 57±10%). If male pulmonary arteries were incubated with estrogen (1 μmol/L, 18 hours), protein expression of 15-LO and 15-HETE production increased. Mechanisms to explain the increased incidence of pulmonary hypertension in women are not known. Results suggest that the 15-LO pathway is different between females and males and is regulated by estrogen. Understanding this novel sex-specific mechanism may provide insight into the increased incidence of pulmonary hypertension in females.

  1. Labeling the pulmonary arterial tree in CT images for automatic quantification of pulmonary embolism

    NARCIS (Netherlands)

    Peters, R.J.M.; Marquering, H.A.; Dogan, H.; Hendriks, E.A.; De Roos, A.; Reiber, J.H.C.; Stoel, B.C.

    2007-01-01

    Contrast-enhanced CT Angiography has become an accepted diagnostic tool for detecting Pulmonary Embolism (PE). The CT obstruction index proposed by Qanadli, which is based on the number of obstructed arterial segments, enables the quantification of PE severity. Because the required manual

  2. Experimental evaluation of a new retrievable inferior vena cava filter for protection from acute pulmonary embolism in canine

    International Nuclear Information System (INIS)

    Zhou Chungao; Shi Haibing; Liu Sheng; Wang Chenghu; Liu Hairi; Li Linsun

    2007-01-01

    Objective: To evaluate the validity, safety and feasibility of a new retrieval inferior vena cava filter for the prevention of pulmonary embolism in an animal model. Methods: The model of deep iliofemoral venous thrombosis was established in 12 experimental dogs. In control group(6 experimental dogs), the deep venous thrombosis was made to fall off directly. In experimental group (6 experimental dogs), the deep venous thrombosis was made to fall off with an implanted filter in inferior vena cava. The filter's thrombus-trapping efficacy was evaluated by angiography of pulmonary artery, measurement of the mean pressure of pulmonary artery and arterial oxygen saturation before and after the deep venous thrombus falling off. Results: All filters implanted in the experimental dogs could successfully capture clot coming from deep venous thrombosis. There was no case of pulmonary embolism in experimental groups. On the other hand, pulmonary embolism occurred following the fall of deep venous thrombus in all dogs of control group. Conclusion: The retrievable inferior vena cava filter can effectively prevent from the pulmonary embolism due to falling off of the emboli from deep venous thrombosis. The process of implantation and retrieval is relatively simple and easy. (authors)

  3. Disconnection of pulmonary and systemic arterial stiffness in COPD

    Directory of Open Access Journals (Sweden)

    Weir-McCall JR

    2018-05-01

    Full Text Available Jonathan R Weir-McCall,1 Patrick SK Liu-Shiu-Cheong,2 Allan D Struthers,1 Brian J Lipworth,2 J Graeme Houston1 1Division of Molecular and Clinical Medicine, Medical Research Institute, University of Dundee, Dundee, UK; 2Scottish Centre for Respiratory Research, Medical Research Institute, University of Dundee, Dundee, UK Background: Both pulmonary arterial stiffening and systemic arterial stiffening have been described in COPD. The aim of the current study was to assess pulse wave velocity (PWV within these two arterial beds to determine whether they are separate or linked processes. Materials and methods: In total, 58 participants with COPD and 21 healthy volunteers (HVs underwent cardiac magnetic resonance imaging (MRI and were tested with a panel of relevant biomarkers. Cardiac MRI was used to quantify ventricular mass, volumes, and pulmonary (pulse wave velocity [pPWV] and systemic pulse wave velocity [sPWV]. Results: Those with COPD had higher pPWV (COPD: 2.62 vs HV: 1.78 ms-1, p=0.006, higher right ventricular mass/volume ratio (RVMVR; COPD: 0.29 vs HV: 0.25 g/mL, p=0.012, higher left ventricular mass/volume ratio (LVMVR; COPD: 0.78 vs HV: 0.70 g/mL, p=0.009, and a trend toward a higher sPWV (COPD: 8.7 vs HV: 7.4 ms-1, p=0.06. Multiple biomarkers were elevated: interleukin-6 (COPD: 1.38 vs HV: 0.58 pg/mL, p=0.02, high-sensitivity C-reactive protein (COPD: 6.42 vs HV: 2.49 mg/L, p=0.002, surfactant protein D (COPD: 16.9 vs HV: 9.13 ng/mL, p=0.001, N-terminal pro-brain natriuretic peptide (COPD: 603 vs HV: 198 pg/mL, p=0.001, and high-sensitivity troponin I (COPD: 2.27 vs HV: 0.92 pg/mL, p<0.001. There was a significant relationship between sPWV and LVMVR (p=0.01 but not pPWV (p=0.97 nor between pPWV and RVMVR (p=0.27. Conclusion: Pulmonary arterial stiffening and systemic arterial stiffening appear to be disconnected and should therefore be considered independent processes in COPD. Further work is warranted to determine whether both these

  4. Pulmonary Arterial Stent Implantation in an Adult with Williams Syndrome

    International Nuclear Information System (INIS)

    Reesink, Herre J.; Henneman, Onno D. F.; Delden, Otto M. van; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

    2007-01-01

    We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent implantation. Short-term follow-up showed a good clinical result with excellent patency of the stents but early restenosis of the segments in which only balloon angioplasty was performed. These stenoses were subsequently also treated successfully by stent implantation. Stent patency was observed 3 years after the first procedure

  5. Anomalous origin of the left coronary artery from the pulmonary artery with patent ductus arteriosus: a must to recognize entity.

    Science.gov (United States)

    Awasthy, Neeraj; Marwah, Ashutosh; Sharma, Rajesh; Dalvi, Bharat

    2010-09-01

    Anomalous left coronary artery from the pulmonary trunk (ALCAPA) presents in early infancy with a clinical picture of congestive heart failure with left ventricular (LV) dysfunction and mitral insufficiency. These manifestations of myocardial ischaemia may be masked in the presence of an associated patent ductus arteriosus (PDA) or ventricular septal defect (VSD) which prevents the fall of pulmonary artery pressures and allows perfusion of the anomalous coronary artery. We present a case of a patient with large PDA-associated ALCAPA and preserved LV function. The importance of such a finding lies in the fact that VSD closure or PDA ligation in such cases would unmask the ALCAPA.

  6. Isolated unilateral absence of the pulmonary artery. Review of the world literature and guidelines for surgical repair.

    Science.gov (United States)

    Shakibi, J G; Rastan, H; Nazarian, I; Paydar, M; Aryanpour, I; Siassi, B

    1978-05-01

    A 10-month-old boy is presented who had isolated unilateral absence of the right pulmonary artery. He suffered from hemoptysis and severe congestive heart failure. The patient underwent prosthetic anastomosis of the right to the main pulmonary artery. Although the hemodynamic response was favorable, his oxygenation did not improve due to diffuse pulmonary arteriovenous fistulae of the affected lung. The patient succumbed 3 months after operation due to massive uncontrollable hemoptysis from the right lung. Isolated unilateral absence of the pulmonary artery is a rare lesion. In our review of the world literature as of November 1976, 47 cases (including this report) of the unilateral absence of the pulmonary artery have been reported. Of these 25.5% had pulmonary hypertension and only 4 cases underwent successful repair of the lesion. Though repair of this defect can be carried out, the result may not be always gratifying. Our experience with this case has led us to consider a lung biopsy before proceeding to the surgical repair of the lesion. If the affected lung shows arteriovenous abnormalities the operation should not be recommended.

  7. Flow-related Right Ventricular - Pulmonary Arterial Pressure Gradients during Exercise.

    Science.gov (United States)

    Wright, Stephen P; Opotowsky, Alexander R; Buchan, Tayler A; Esfandiari, Sam; Granton, John T; Goodman, Jack M; Mak, Susanna

    2018-06-06

    The assumption of equivalence between right ventricular and pulmonary arterial systolic pressure is fundamental to several assessments of right ventricular or pulmonary vascular hemodynamic function. Our aims were to 1) determine whether systolic pressure gradients develop across the right ventricular outflow tract in healthy adults during exercise, 2) examine the potential correlates of such gradients, and 3) consider the effect of such gradients on calculated indices of right ventricular function. Healthy untrained and endurance-trained adult volunteers were studied using right-heart catheterization at rest and during submaximal cycle ergometry. Right ventricular and pulmonary artery pressures were simultaneously transduced, and cardiac output was determined by thermodilution. Systolic pressures, peak and mean gradients, and indices of chamber, vascular, and valve function were analyzed offline. Summary data are reported as mean ± standard deviation or median [interquartile range]. No significant right ventricular outflow tract gradients were observed at rest (mean gradient = 4 [3-5] mmHg), and calculated effective orifice area was 3.6±1.0 cm2. Right ventricular systolic pressure increases during exercise were greater than that of pulmonary artery systolic pressure. Accordingly, mean gradients developed during light exercise (8 [7-9] mmHg) and increased during moderate exercise (12 [9-14] mmHg, p < 0.001). The magnitude of the mean gradient was linearly related to cardiac output (r2 = 0.70, p < 0.001). In healthy adults without pulmonic stenosis, systolic pressure gradients develop during exercise, and the magnitude is related to blood flow rate.

  8. Implantation port-catheter permanent indwelling of pulmonary artery in treating lung metastasis from HCC

    International Nuclear Information System (INIS)

    Cheng Jiemin; Wang Jianhua; Yan Zhiping; Wang Xiaolin; Gong Gaoquan; Liu Qingxin

    2000-01-01

    Objective: To observe the efficacy of a percutaneous implantation port-catheter permanent indwelling pulmonary artery for regional chemotherapy of the metastatic lung cancer from HCC. Methods: Between 1995 and 1999, 62 patients (42 males, 20 females; mean age 46 years) suffering from the metastatic lung cancer from HCC underwent percutaneous implantation of port-catheter permanent indwelling pulmonary artery using the right subclavian vein. In 19 patients with metastatic tumor located on one side of the lung, an indwelling catheter was placed into the ipsilateral side pulmonary artery. With metastasis of both sides, the catheter was inserted into the main trunk of pulmonary artery. The regimens of the chemotherapy were 5-FU + CDDP + MMC(FDM) or 5-FU + CDDP + MMC(FDA). Results: The interventional procedure was successfully completed in all 62 cases (100%). The complications occurred in 8% cases, including infections (3.2%), unhealed wound (1.6%) and pneumothorax (3.2%). The treatment effects of 3-months after the procedure were as follows: the obvious decrease of lung tumor size was 35.5%; stable disease (SD) 32.3% and progressive disease (PD) 32.3%. 6 months follow-up: 12 patients were dead (12/62) and the others are still doing well. The response rates were 22.6%, partial response (PR) 32.3%; stable disease (SD) 25.8% and progressive disease (PD) 32.3%. Conclusions: The percutaneous implantation techniques of pulmonary arterial port-catheter could be a good method in the treatment of metastatic lung cancer from HCC because of it is simple, with few complications and positive effect

  9. Elevated plasma endothelin-1 and pulmonary arterial pressure in children exposed to air pollution.

    Science.gov (United States)

    Calderón-Garcidueñas, Lilian; Vincent, Renaud; Mora-Tiscareño, Antonieta; Franco-Lira, Maricela; Henríquez-Roldán, Carlos; Barragán-Mejía, Gerardo; Garrido-García, Luis; Camacho-Reyes, Laura; Valencia-Salazar, Gildardo; Paredes, Rogelio; Romero, Lina; Osnaya, Hector; Villarreal-Calderón, Rafael; Torres-Jardón, Ricardo; Hazucha, Milan J; Reed, William

    2007-08-01

    Controlled exposures of animals and humans to particulate matter (PM) or ozone air pollution cause an increase in plasma levels of endothelin-1, a potent vasoconstrictor that regulates pulmonary arterial pressure. The primary objective of this field study was to determine whether Mexico City children, who are chronically exposed to levels of PM and O(3) that exceed the United States air quality standards, have elevated plasma endothelin-1 levels and pulmonary arterial pressures. We conducted a study of 81 children, 7.9 +/- 1.3 years of age, lifelong residents of either northeast (n = 19) or southwest (n = 40) Mexico City or Polotitlán (n = 22), a control city with PM and O(3) levels below the U.S. air quality standards. Clinical histories, physical examinations, and complete blood counts were done. Plasma endothelin-1 concentrations were determined by immunoassay, and pulmonary arterial pressures were measured by Doppler echocardiography. Mexico City children had higher plasma endothelin-1 concentrations compared with controls (p < 0.001). Mean pulmonary arterial pressure was elevated in children from both northeast (p < 0.001) and southwest (p < 0.05) Mexico City compared with controls. Endothelin-1 levels in Mexico City children were positively correlated with daily outdoor hours (p = 0.012), and 7-day cumulative levels of PM air pollution < 2.5 mum in aerodynamic diameter (PM(2.5)) before endothelin-1 measurement (p = 0.03). Chronic exposure of children to PM(2.5) is associated with increased levels of circulating endothelin-1 and elevated mean pulmonary arterial pressure.

  10. Elevated Plasma Endothelin-1 and Pulmonary Arterial Pressure in Children Exposed to Air Pollution

    Science.gov (United States)

    Calderón-Garcidueñas, Lilian; Vincent, Renaud; Mora-Tiscareño, Antonieta; Franco-Lira, Maricela; Henríquez-Roldán, Carlos; Barragán-Mejía, Gerardo; Garrido-García, Luis; Camacho-Reyes, Laura; Valencia-Salazar, Gildardo; Paredes, Rogelio; Romero, Lina; Osnaya, Hector; Villarreal-Calderón, Rafael; Torres-Jardón, Ricardo; Hazucha, Milan J.; Reed, William

    2007-01-01

    Background Controlled exposures of animals and humans to particulate matter (PM) or ozone air pollution cause an increase in plasma levels of endothelin-1, a potent vasoconstrictor that regulates pulmonary arterial pressure. Objectives The primary objective of this field study was to determine whether Mexico City children, who are chronically exposed to levels of PM and O3 that exceed the United States air quality standards, have elevated plasma endothelin-1 levels and pulmonary arterial pressures. Methods We conducted a study of 81 children, 7.9 ± 1.3 years of age, lifelong residents of either northeast (n = 19) or southwest (n = 40) Mexico City or Polotitlán (n = 22), a control city with PM and O3 levels below the U.S. air quality standards. Clinical histories, physical examinations, and complete blood counts were done. Plasma endothelin-1 concentrations were determined by immunoassay, and pulmonary arterial pressures were measured by Doppler echocardiography. Results Mexico City children had higher plasma endothelin-1 concentrations compared with controls (p < 0.001). Mean pulmonary arterial pressure was elevated in children from both northeast (p < 0.001) and southwest (p < 0.05) Mexico City compared with controls. Endothelin-1 levels in Mexico City children were positively correlated with daily outdoor hours (p = 0.012), and 7-day cumulative levels of PM air pollution < 2.5 μm in aerodynamic diameter (PM2.5) before endothelin-1 measurement (p = 0.03). Conclusions Chronic exposure of children to PM2.5 is associated with increased levels of circulating endothelin-1 and elevated mean pulmonary arterial pressure. PMID:17687455

  11. Outcomes of Percutaneous Closure of Patent Ductus Arteriosus Accompanied With Unilateral Absence of Pulmonary Artery.

    Science.gov (United States)

    Yang, Yankun; Zheng, Hong; Xu, Zhongying; Zhang, Gejun; Jin, Jinglin; Hu, Haibo; Tian, Tao; Zhou, Xianliang

    2017-04-01

    Limited data have reported the outcomes of percutaneous closure of patent ductus arteriosus (PDA) in patients with unilateral absence of pulmonary artery (UAPA). This study aimed to evaluate the symptomatology, diagnosis and therapy, especially the transcatheter closure of PDA in patients with PDA associated with UAPA. Patients diagnosed with PDA and UAPA were retrospectively enrolled from August 2010 through January 2016. Clinical data, treatment and follow-up information were evaluated. Thirteen patients (6 males and 7 females) were diagnosed with PDA associated with UAPA. Percutaneous closure was successfully conducted in 6 patients successfully. The median age was 7 years (7 months to 37 years). The mean diameter of the PDA and occluders were 4.7 ± 1.8mm (2-7mm) and 11.3 ± 3.9mm (6-14mm), respectively. The mean pulmonary artery pressure was 41.5 ± 13.5mmHg (25-62mmHg). The diameter of PDA has no relationship with the degree of pulmonary artery pressure (r = 0.239, P = 0.648). In 4 patients, systolic pulmonary arterial pressure decreased significantly after closure with 69.0 ± 10.7 versus 48.0 ± 11.3mmHg (P = 0.146), and also the mean pulmonary arterial pressure was 54.5 ± 5.7 mm Hg versus 30.5 ± 3.9mmHg (P = 0.04). In all, 1 patient had a trace residual shunt, which disappeared within 24 hours. In appropriate patients with PDA associated with UAPA, transcatheter closure of PDA has the potential to improve the pulmonary artery hypertension. Further follow-up is required to monitor the long-term outcomes. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  12. Signal transduction in the development of pulmonary arterial hypertension

    Science.gov (United States)

    Malenfant, Simon; Neyron, Anne-Sophie; Paulin, Roxane; Potus, François; Meloche, Jolyane; Provencher, Steeve; Bonnet, Sébastien

    2013-01-01

    Pulmonary arterial hypertension (PAH) is a unique disease. Properly speaking, it is not a disease of the lung. It can be seen more as a microvascular disease occurring mainly in the lungs and affecting the heart. At the cellular level, the PAH paradigm is characterized by inflammation, vascular tone imbalance, pulmonary arterial smooth muscle cell proliferation and resistance to apoptosis and the presence of in situ thrombosis. At a clinical level, the aforementioned abnormal vascular properties alter physically the pulmonary circulation and ventilation, which greatly influence the right ventricle function as it highly correlates with disease severity. Consequently, right heart failure remains the principal cause of death within this cohort of patients. While current treatment modestly improve patients’ conditions, none of them are curative and, as of today, new therapies are lacking. However, the future holds potential new therapies that might have positive influence on the quality of life of the patient. This article will first review the clinical presentation of the disease and the different molecular pathways implicated in the pathobiology of PAH. The second part will review tomorrow's future putative therapies for PAH. PMID:24015329

  13. Efficacy of pulmonary artery banding in patients with uneventricular defects

    Directory of Open Access Journals (Sweden)

    Ю. С. Синельников

    2015-10-01

    Full Text Available Long-term results where evaluated for different methods of pulmonary artery banding (PAB in 25 patients with univentricular congenital heart defects with used three methods. Hospital mortality was 8%. Second stage palliation was performed in 61% of patients. More tight PA banding in patients with univentriclar gave opportunity to perform 2nd stage of operation in 100% of patients, decrease complications and mortality rate, modify pulmonary circulation effectively.

  14. The Voice of the Heart: Vowel-Like Sound in Pulmonary Artery Hypertension

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    Mohamed Elgendi

    2018-04-01

    Full Text Available Increased blood pressure in the pulmonary artery is referred to as pulmonary hypertension and often is linked to loud pulmonic valve closures. For the purpose of this paper, it was hypothesized that pulmonary circulation vibrations will create sounds similar to sounds created by vocal cords during speech and that subjects with pulmonary artery hypertension (PAH could have unique sound signatures across four auscultatory sites. Using a digital stethoscope, heart sounds were recorded at the cardiac apex, 2nd left intercostal space (2LICS, 2nd right intercostal space (2RICS, and 4th left intercostal space (4LICS undergoing simultaneous cardiac catheterization. From the collected heart sounds, relative power of the frequency band, energy of the sinusoid formants, and entropy were extracted. PAH subjects were differentiated by applying the linear discriminant analysis with leave-one-out cross-validation. The entropy of the first sinusoid formant decreased significantly in subjects with a mean pulmonary artery pressure (mPAp ≥ 25 mmHg versus subjects with a mPAp < 25 mmHg with a sensitivity of 84% and specificity of 88.57%, within a 10-s optimized window length for heart sounds recorded at the 2LICS. First sinusoid formant entropy reduction of heart sounds in PAH subjects suggests the existence of a vowel-like pattern. Pattern analysis revealed a unique sound signature, which could be used in non-invasive screening tools.

  15. Cerivastatin Nano-Liposome as a Potential Disease Modifying Approach for the Treatment of Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Lee, Young; Pai, S Balakrishna; Bellamkonda, Ravi V; Thompson, David H; Singh, Jaipal

    2018-04-25

    In this study, we have investigated nano-liposome as an approach to tailor the pharmacology of cerivastatin as a disease modifying drug for pulmonary arterial hypertension (PAH). Cerivastatin encapsulated liposomes with an average diameter of 98±27 nm were generated by thin film and freeze-thaw process. The nano-liposomes demonstrated sustained drug release kinetics in vitro and inhibited proliferation of pulmonary artery smooth muscle cells with significantly less cellular cytotoxicity as compared to free cerivastatin. When delivered by inhalation to a rat model of monocrotalin induced PAH, cerivastatin significantly reduced pulmonary artery pressure from 55.13±9.82 mmHg to 35.56±6.59 mmHg (P < 0.001) and diminished pulmonary artery wall thickening. Echocardiography showed that cerivastatin significantly reduced right ventricle thickening (0.34±0.02 cm monocrotalin vs. 0.26±0.02 cm cerivastatin; P < 0.001) and increased pulmonary artery acceleration time (13.98±1.14 ms monocrotalin vs. 21.07±2.80 ms cerivastatin; P < 0.001). Nano-liposomal cerivastatin was equally effective or slightly better than cerivastatin in reducing pulmonary artery pressure (67.06±13.64 mmHg monocrotalin; 46.31±7.64 mmHg cerivastatin vs. 37.32±9.50 mmHg liposomal cerivastatin) and improving parameters of right ventricular function as measured by increasing pulmonary artery acceleration time (24.68±3.92 ms monocrotalin; 32.59±6.10 ms cerivastatin vs. 34.96±7.51 ms liposomal cerivastatin). More importantly, the rate and magnitude of toxic cerivastatin metabolite lactone generation from the intratracheally administered nano-liposomes was significantly lower as compared to intravenously administered free cerivastatin. These studies show that nano-liposome encapsulation improved in vitro and in vivo pharmacological and safety profile of cerivastatin and may represent a safer approach as a disease modifying therapy for PAH. The American Society for Pharmacology and Experimental

  16. Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report

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    Bohn Olga L

    2012-11-01

    Full Text Available Abstract Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall or by histologic type. Angiosarcomas are malignant neoplasms that arise from the endothelial lining of the blood vessels; those arising in the intimal compartment of pulmonary artery are rare. We report a case of pulmonary artery angiosarcoma in a 36-year old female with pulmonary masses. The patient had no other primary malignant neoplasm, thus excluding a metastatic lesion. Gross examination revealed a thickened right pulmonary artery and a necrotic and hemorrhagic tumor, filling and occluding the vascular lumen. The mass extended distally, within the pulmonary vasculature of the right lung. Microscopically, an intravascular undifferentiated tumor was identified. The tumor cells showed expression for vascular markers VEGFR, VEGFR3, PDGFRa, FGF, Ulex europaeus, FVIII, FLI-1, CD31 and CD34; p53 was overexpressed and Ki67 proliferative rate was increased. Intravascular angiosarcomas are aggressive neoplasms, often associated with poor outcome. Virtual slide The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2315906377648045.

  17. Erythropoietin Attenuates Pulmonary Vascular Remodeling in Experimental Pulmonary Arterial Hypertension through Interplay between Endothelial Progenitor Cells and Heme Oxygenase

    OpenAIRE

    van Loon, Rosa Laura E; Bartelds, Beatrijs; Wagener, Frank A D T G; Affara, Nada; Mohaupt, Saffloer; Wijnberg, Hans; Pennings, Sebastiaan W C; Takens, Janny; Berger, Rolf M F

    2015-01-01

    BACKGROUND: Pulmonary arterial hypertension (PAH) is a pulmonary vascular disease with a high mortality, characterized by typical angio-proliferative lesions. Erythropoietin (EPO) attenuates pulmonary vascular remodeling in PAH. We postulated that EPO acts through mobilization of endothelial progenitor cells (EPCs) and activation of the cytoprotective enzyme heme oxygenase-1 (HO-1). METHODS: Rats with flow-associated PAH, resembling pediatric PAH, were treated with HO-1 inducer EPO in the pre...

  18. Erythropoietin Attenuates Pulmonary Vascular Remodeling in Experimental Pulmonary Arterial Hypertension through Interplay between Endothelial Progenitor Cells and Heme Oxygenase

    OpenAIRE

    van Loon, Rosa Laura E.; Bartelds, Beatrijs; Wagener, Frank A. D. T. G.; Affara, Nada; Mohaupt, Saffloer; Wijnberg, Hans; Pennings, Sebastiaan W. C.; Takens, Janny; Berger, Rolf M. F.

    2015-01-01

    Background Pulmonary arterial hypertension (PAH) is a pulmonary vascular disease with a high mortality, characterized by typical angio-proliferative lesions. Erythropoietin (EPO) attenuates pulmonary vascular remodeling in PAH. We postulated that EPO acts through mobilization of endothelial progenitor cells (EPCs) and activation of the cytoprotective enzyme heme oxygenase-1 (HO-1). Methods Rats with flow-associated PAH, resembling pediatric PAH, were treated with HO-1 inducer EPO i...

  19. When a pulmonary embolism is not a pulmonary embolism: a rare case of primary pulmonary leiomyosarcoma

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    Nargiz Muganlinskaya

    2015-12-01

    Full Text Available Arterial leiomyosarcomas account for up to 21% of vascular leiomyosarcomas, with 56% of arterial leiomyosarcomas occurring in the pulmonary artery. While isolated cases of primary pulmonary artery leiomyosarcoma document survival up to 36 months after treatment, these uncommon, aggressive tumors are highly lethal, with 1-year survival estimated at 20% from the onset of symptoms. We discuss a rare case of a pulmonary artery leiomyosarcoma that was originally diagnosed as a pulmonary embolism (PE. A 72-year-old Caucasian female was initially diagnosed with ‘saddle pulmonary embolism’ based on computerized tomographic angiography of the chest 2 months prior to admission and placed on anticoagulation. Dyspnea escalated, and serial computed tomography scans showed cardiomegaly with pulmonary emboli involving the right and left main pulmonary arteries with extension into the right and left upper and lower lobe branches. An echocardiogram on admission showed severe pulmonary hypertension with a pulmonary artery pressure of 82.9 mm Hg, and a severely enlarged right ventricle. Respiratory distress and multiorgan failure developed and, unfortunately, the patient expired. Autopsy showed a lobulated, yellow mass throughout the main pulmonary arteries measuring 13 cm in diameter. The mass extended into the parenchyma of the right upper lobe. On microscopy, the mass was consistent with a high-grade primary pulmonary artery leiomyosarcoma. Median survival of patients with primary pulmonary artery leiomyosarcoma without surgery is one and a half months, and mortality is usually due to right-sided heart failure. Pulmonary artery leiomyosarcoma is a rare but highly lethal disease commonly mistaken for PE. Thus, we recommend clinicians to suspect this malignancy when anticoagulation fails to relieve initial symptoms. In conclusion, early detection and suspicion of pulmonary artery leiomyosarcoma should be considered in patients refractory to anticoagulation

  20. Pulmonary artery dissection following balloon valvuloplasty in a dog with pulmonic stenosis.

    Science.gov (United States)

    Grint, K A; Kellihan, H B

    2017-04-01

    A 3-month-old, 9.9 kg, male pit bull cross was referred for evaluation of collapse. A left basilar systolic heart murmur graded V/VI and a grade IV/VI right basilar systolic heart murmur were ausculted. Echocardiography showed severe pulmonic stenosis characterized by annular hypoplasia, leaflet thickening, and leaflet fusion. After 1 month of atenolol therapy, a pulmonic valve balloon valvuloplasty procedure was performed, and the intra-operative right ventricular pressure was reduced by 43%. Echocardiography, performed the following day, showed apparent rupture of a pulmonary valve leaflet and a membranous structure within the pulmonary artery consistent with a dissecting membrane. Short-term follow-up has shown no apparent progression of the pulmonary artery dissection and the patient remains free of clinical signs. Copyright © 2016 Elsevier B.V. All rights reserved.

  1. Radionuclide scanning in a study of the pulmonary blood flow with the open arterial canal

    International Nuclear Information System (INIS)

    Rizaev, M.N.; Gulyamov, D.S.; Khodzhibekov, M.Kh.; Anvarov, M.A.

    1980-01-01

    Characteristic features of the distribution of the arterial pulmonary blood flow have been studied in 32 patients with the open arterial flow by lung scanning with albumin macroaggregate and 131 I. Research was conducted using the gamma-Ochamber ''Fo Gamma LFV'' and the scanner ''Magnaskaner 500I''. Disordered distribution of the pulmonary blood flow expressed in its unilateral decrease was detected in 17 patients (52.9%). A higher frequency of pulmonary hypertension was noted in these patients versus those with a relatively normal distribution of the pulmonary blood flow. A severe course of the disease was observed in high pulmonary hypertension combined with sharp suppression of the blood flow in one of the lungs or with signs of the shift from the right to the left side

  2. Aborted sudden cardiac death in a young male with anomalous left coronary artery arising from the pulmonary artery

    Directory of Open Access Journals (Sweden)

    Chih-Han Huang

    2017-01-01

    Full Text Available Anomalous left coronary artery arising from the pulmonary artery (ALCAPA is a rare type of congenital coronary abnormality that may be associated with early infant mortality and sudden adult cardiac death. We report a case regarding a 23-year-old male who collapsed during a marathon race and was resuscitated with cardiopulmonary resuscitation. Subsequent workups verified the diagnosis of ALCAPA. The patient underwent surgical intervention with obliteration of the ALCAPA orifice and coronary artery bypass grafting with left internal mammary artery to left anterior descending coronary artery. The procedure was done smoothly, and he was discharged uneventfully.

  3. A Case of Pulmonary Artery Aneurysm Associated with Patent Ductus Arteriosus : Detection by Radionuclide Cardiac Angiography

    International Nuclear Information System (INIS)

    Sohn, I.; Lee, M. C.; Cho, B. Y.; Koh, C. S.; Yoon, Y. S.; Hong, C. Y.; Rho, J. R.; Youn, K. M.; Han, M. C.

    1981-01-01

    A Case of main pulmonary artery aneurysm in a 9-year-old boy with patent ductus arteriosus is presented. In this case presented with a huge mass density on the chest X-ray, radionuclide cardiac angiography showed a vascular lesion, which was confirmed as an aneurysm of the main pulmonary artery at roentgenologic angiogram. The aneurysm appeared following an episode of bacterial endocarditis and pulmonary hypertension. A successful aneurysmectomy with multiple ligation of ductus arteriosus was performed.

  4. A Case of Pulmonary Artery Aneurysm Associated with Patent Ductus Arteriosus : Detection by Radionuclide Cardiac Angiography

    Energy Technology Data Exchange (ETDEWEB)

    Sohn, I; Lee, M C; Cho, B Y; Koh, C S; Yoon, Y S; Hong, C Y; Rho, J R; Youn, K M; Han, M C [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1981-09-15

    A Case of main pulmonary artery aneurysm in a 9-year-old boy with patent ductus arteriosus is presented. In this case presented with a huge mass density on the chest X-ray, radionuclide cardiac angiography showed a vascular lesion, which was confirmed as an aneurysm of the main pulmonary artery at roentgenologic angiogram. The aneurysm appeared following an episode of bacterial endocarditis and pulmonary hypertension. A successful aneurysmectomy with multiple ligation of ductus arteriosus was performed.

  5. Pulmonary artery wave propagation and reservoir function in conscious man: impact of pulmonary vascular disease, respiration and dynamic stress tests

    DEFF Research Database (Denmark)

    Su, Junjing; Manisty, Charlotte; Simonsen, Ulf

    2017-01-01

    Detailed haemodynamic analysis may provide novel insights into the pulmonary circulation. Therefore, wave intensity and reservoir-excess pressure analyses were applied in the pulmonary artery to characterize changes in wave propagation and reservoir function during spontaneous respiration......, recordings were also obtained during Valsalva manoeuvre and handgrip exercise. The asymptotic pressure at which the flow through the microcirculation ceases, the reservoir pressure related to arterial compliance and the excess pressure caused by arterial waves increased in PAH patients compared to controls....... The systolic and diastolic rate constants also increased, while the diastolic time constant decreased. The forward compression wave energy decreased by ∼8% in controls and ∼6% in PAH patients during expiration compared to inspiration, while the wave speed remained unchanged throughout the respiratory cycle...

  6. Diagnostic Value of Transthoracic Echocardiography in Patients With Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery

    Science.gov (United States)

    Li, Rong-Juan; Sun, Zhonghua; Yang, Jiao; Yang, Ya; Li, Yi-Jia; Leng, Zhao-Ting; Liu, Guo-Wen; Pu, Li-Hong

    2016-01-01

    Abstract Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality associated with early infant mortality and sudden death in adults. Transthoracic echocardiography (TTE) plays an important role in early detection and diagnosis of ALCAPA as a noninvasive modality. However, its diagnostic value is not well studied. The purpose of this study is to determine the performance of TTE in the diagnostic assessment of ALCAPA as compared with coronary CT and invasive coronary angiography. A total of 22 patients (13 women and 9 men, mean age, 12.9 ± 19.5 years) with ALCAPA who underwent echocardiographic examination for clinical diagnosis were retrospectively reviewed and analyzed. Transthoracic echocardiographic features of ALCAPA were analyzed and its diagnostic value was compared with invasive coronary angiography and coronary CT angiography (CTA) with surgical findings serving as the gold standard. Surgery was performed in all of the patients to establish the dual coronary artery system. Five underwent the Takeuchi procedure and 17 had re-implantation of the anomalous left coronary artery. Of 20 patients, echocardiographic diagnoses were in good agreement with findings at surgery, resulting in the diagnostic accuracy of 90.9%. Two cases were misdiagnosed—one as the right coronary artery to pulmonary artery fistula and the other as rheumatic heart disease. The echocardiographic features of these patients with ALCAPA included: abnormal left coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow in 20 patients; enlargement of the right coronary artery in 17 patients; abundant intercoronary septal collaterals in 17 patients; and moderate and significant mitral regurgitation in 14 patients. The diagnostic accuracy of invasive coronary angiography (in 17 patients) and coronary CTA (in 9 patients) was 100%. This study shows that TTE is an accurate, noninvasive imaging modality

  7. [The heart catheter table is not the operating table : Intraindividual comparison of pulmonary artery pressures].

    Science.gov (United States)

    Ziegler, M U; Reinelt, H

    2018-05-01

    Patients undergoing cardiac surgery need extensive and invasive monitoring, which needs to be individually adapted for each patient and requires a diligent risk-benefit analysis. The use of a pulmonary artery catheter (PAC) seems to be justifiable in certain cases; therefore, the preoperative diagnosis of pulmonary hypertension represents an indication for perioperative monitoring with PAC in the S3 guidelines of the German Society for Anesthesiology and Intensive Care Medicine (DGAI). In many cases, however, this preoperative diagnosis cannot be confirmed intraoperatively. We wanted to find out whether this is just an impression or whether there actually are significant differences between preoperative, intraoperative and postoperative pulmonary artery pressures. After obtaining ethical approval, we retrospectively compared the pulmonary pressures of cardiac surgery patients with an elevated pulmonary pressure during preoperative right heart catheterization with those obtained intraoperatively and postoperatively by means of a PAC. All patients with a preoperatively documented pulmonary artery pressure of 40 mmHg or above and an intraoperative use of a PAC during a 4-year period were included. Exclusion criteria were intracardiac shunts, cardiogenic shock, emergency procedures, pulmonary hypertension of non-cardiac origin and a time span of more than 1 year between right heart catheterization and surgery. We included 90 patients. In the whole group and in the subgroups (according to diagnosis, time elapsed between heart catheterization and operation and pulmonary pressure), there were significant differences between preoperative and intraoperative pulmonary and systemic pressures. Systemic and pulmonary artery pressures were significantly higher during preoperative catheterization than intraoperatively. The systemic systolic pressure/systolic pulmonary pressure ratio, however, remained constant. The intraoperative and postoperative systemic and pulmonary

  8. Gerbode defect following endocarditis and misinterpreted as severe pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Allajbeu Iris

    2010-09-01

    Full Text Available Abstract A Gerbode -type defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. It is usually congenital, but rarely is acquired, as a complication of endocarditis. This can be anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve. However, identification of an actual communication is often extremely difficult, so a careful and meticulous echocardiogram should be done in order to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension. The large systolic pressure gradient between the left ventricle and the right atrium would expectedly result in a high velocity systolic Doppler flow signal in right atrium and it can be sometimes mistakably diagnosed as tricuspid regurgitant jet simulating pulmonary arterial hypertension. We present a rare case of young woman, with endocarditis who presented with severe pulmonary arterial hypertension. The preoperative diagnosis of left ventricle to right atrial communication (acquired Gerbode defect was suspected initially by echocardiogram and confirmed at the time of the surgery. A point of interest, apart from the diagnostic problem, was the explanation for its mechanism and presentation. The probability of a bacterial etiology of the defect is high in this case.

  9. Bronchial Artery Embolization in the Management of Pulmonary Parenchymal Endometriosis with Hemoptysis

    International Nuclear Information System (INIS)

    Kervancioglu, Selim; Andic, Cagatay; Bayram, Nazan; Telli, Cumali; Sarica, Akif; Sirikci, Akif

    2008-01-01

    Pulmonary parenchymal endometriosis is extremely rare and usually manifests itself with a recurrent hemoptysis associated with the menstrual cycle. The therapies proposed for women with endometriosis consist of medical treatments and surgery. Bronchial artery embolization has become a well-established and minimally invasive treatment modality for hemoptysis, and to the best of our knowledge, it has not been reported in pulmonary endometriosis. We report a case of pulmonary parenchymal endometriosis treated with embolotheraphy for hemoptysis.

  10. Perinatal/Neonatal case presentation: pulmonary artery sling associated with respiratory distress.

    Science.gov (United States)

    Healey, David; Ron, Nitin; Hromada, Andrew; Chhabra, Manoj

    2016-01-01

    Pulmonary artery sling is a very rare cause of pediatric respiratory distress. The estimated prevalence of the disease was first determined by Yu et al. in 2008 as 59 per million school-aged children. Associated symptoms are cough, wheezing, and feeding difficulty, all of which are common in routine outpatient pediatric clinical encounters. We report a case of a premature male neonate twin who was admitted to the neonatal intensive care unit with respiratory distress and pneumothorax. His presentation, as well as the etiology of his pulmonary disease, was felt to be consistent with those of numerous other premature infants. Akin to this was his delayed discharge on account of his slow progress with oral feeding. Parents gave a history of tachypnea and feeding difficulty to his doctors. He presented twice to the emergency room in respiratory distress. At 4 months of age, while in hospital for a pulmonary infection, he had an echocardiogram that revealed a pulmonary artery sling. We review the literature on this vascular anomaly, discuss its diagnosis and management, and critique the clinical thinking that determined this child's course from the perspective of availability heuristics.

  11. Patient engagement and self-management in pulmonary arterial hypertension

    DEFF Research Database (Denmark)

    Graarup, Jytte; Ferrari, Pisana; Howard, Luke S

    2016-01-01

    of the patient may improve their ability to cope with pulmonary arterial hypertension, as well as help them to become effective in the self-management of their disease. Successful patient engagement can be achieved through effective education and the delivery and communication of timely, high-quality information...

  12. Bilateral pulmonary arterial banding results in an increased need for subsequent pulmonary artery interventions.

    Science.gov (United States)

    Davies, Ryan R; Radtke, Wolfgang A; Klenk, Dore; Pizarro, Christian

    2014-02-01

    Despite increasing use of bilateral branch pulmonary artery banding (bPAB), both as a temporary stabilizing treatment and as part of comprehensive hybrid management of hypoplastic left heart syndrome, little is known about the long-term outcomes of the pulmonary arteries (PAs) in banded patients. We conducted a retrospective review of all patients with ductal-dependent systemic circulation (2001-2013) undergoing bPAB placement at a single institution (bPAB, n = 50); patients who underwent a stage I Norwood procedure (Norwood, n = 53) were used for comparison. The need for PA interventions (surgical arterioplasty, balloon angioplasty, and stent implantation) and PA growth were assessed. Bands were in place for a median of 76 days. PA growth and size were similar between groups, but bPAB patients required more interventions (1.4 ± 2.9 vs 0.5 ± 1.2, P = .01). In competing risks analysis, only 20% of bPAB patients were alive and free from intervention at 5 years after bPAB removal. Multivariable Cox proportional hazards regression of operative interventions within the bPAB group demonstrated the following risk factors: subsequent 2-ventricle repairs (hazard ratio [HR], 2.2; 95% confidence interval [CI], 0.7-6.7), smallest band diameter (HR per additional millimeter, 0.059; 95% CI, 0.004-0.849), and duration of band placement more than 90 days (HR, 3.5; 95% CI, 1.0-12.6). Hemodynamics and Fontan candidacy did not differ between groups. Patients with bPAB require additional interventions at earlier time points than Norwood patients. Patients with smaller bands and longer duration of banding are at high risk. Despite stenoses requiring additional interventions, Fontan candidacy is maintained. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  13. The importance of echocardiography in diagnostics of idiopathic pulmonary arterial hypertension: A case report

    Directory of Open Access Journals (Sweden)

    Stojković Gabrijela

    2011-01-01

    Full Text Available Introduction. Idiopathic pulmonary arterial hypertension (IPAH is rare and difficult progressive disease with prevalence of approximately 15 cases per million residents, with predominant female cases. Case Outline. A 47-year-old female presented with symptoms and signs of the right heart chambers failure. Over prior seven years the patient had the feeling of suffocation and fatigue when walking, and received treatment for bronchial asthma. Physical examination revealed a marked loud second heart sound over the pulmonary artery. Electrocardiogram: right ventricular hypertrophy. Spirometric (pulmonary capacity test, cardiac perfusion scan and spiral CT scanning excluded secondary pulmonary arterial hypertension. Blood testing for connective tissue diseases and HIV were within normal reference limits. Transthoracic colour Doppler echocardiography demonstrated a mild tricuspid regurgitation with high values of estimated maximal and middle systolic pressure of the right ventricle (135/110 mm Hg, and excluded previous heart disease. Cardiac catheterization confirmed IPAH diagnosis, with systolic right ventricular pressure of 101/47/66 mm Hg and pulmonary capillary pressure of 30/13/10 mm Hg. Basic therapy with sildenafil, nevertheless, considerable limitations of strain tolerance was still present. Conclusion. IPAH is a severe heart disease with non-specific signs and symptoms. Screening for IPAH is transthoracic colour Doppler echocardiography shows high correlation with cardiac catheterization.

  14. Update on the clinical utility of sildenafil in the treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Gautam V Ramani

    2010-05-01

    Full Text Available Gautam V Ramani, Myung H ParkUniversity of Maryland, Baltimore, MD, USAAbstract: Sildenafil is an orally administered phosphodiesterase type 5 inhibitor that is approved for the treatment of pulmonary arterial hypertension (PAH. The hemodynamic effects of sildenafil are mitigated primarily via potentiating the effects of endogenous nitric oxide, leading to smooth muscle cell relaxation and reductions in pulmonary arterial pressures and pulmonary vascular resistance. When added to standard background therapy in patients with idiopathic or associated PAH from congenital heart disease, anorexigen use, or connective tissue disease, sildenafil treatment results in improved exercise capacity as measured by 6 minute walk distance, improved hemodynamics, and favorable changes in quality of life. Sildenafil use is contraindicated with concomitant nitrate administration, and caution should be exercised when used in combination with antihypertensive agents due to risks of precipitating hypotension. Side effects are generally mild, and include flushing, headaches, and epistaxis. The combination of sildenafil with intravenous epoprostenol is safe and well tolerated, and further improves exercise capacity. Sildenafil is approved only for treatment of PAH, and although emerging data suggest a potential role in treating other types of pulmonary hypertension, larger trials are required to confirm these findings. Keywords: sildenafil, pulmonary arterial hypertension, phosphodiesterase type 5 inhibitor

  15. Automated assessment of aortic and main pulmonary arterial diameters using model-based blood vessel segmentation for predicting chronic thromboembolic pulmonary hypertension in low-dose CT lung screening

    Science.gov (United States)

    Suzuki, Hidenobu; Kawata, Yoshiki; Niki, Noboru; Sugiura, Toshihiko; Tanabe, Nobuhiro; Kusumoto, Masahiko; Eguchi, Kenji; Kaneko, Masahiro

    2018-02-01

    Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by obstruction of the pulmonary vasculature by residual organized thrombi. A morphological abnormality inside mediastinum of CTEPH patient is enlargement of pulmonary artery. This paper presents an automated assessment of aortic and main pulmonary arterial diameters for predicting CTEPH in low-dose CT lung screening. The distinctive feature of our method is to segment aorta and main pulmonary artery using both of prior probability and vascular direction which were estimated from mediastinal vascular region using principal curvatures of four-dimensional hyper surface. The method was applied to two datasets, 64 lowdose CT scans of lung cancer screening and 19 normal-dose CT scans of CTEPH patients through the training phase with 121 low-dose CT scans. This paper demonstrates effectiveness of our method for predicting CTEPH in low-dose CT screening.

  16. Echocardiographic Assessment of Pulmonary Arterial Hypertension for Pediatricians and Neonatologists

    Directory of Open Access Journals (Sweden)

    Gregory James Skinner

    2017-09-01

    Full Text Available There is a growing awareness of the role that increased pulmonary vascular resistance (PVR plays in many pathologies; therefore, assessment of pulmonary artery pressure (PAP is an increasingly requested investigation in the critical care environment. This article will go through the basic concepts regarding PAP and PVR, then will go on to outline the various echocardiographic parameters which are used to assess them. Finally, an outline of how to undertake this assessment will be presented.

  17. Single left coronary ostium and an anomalous prepulmonic right coronary artery in 2 dogs with congenital pulmonary valve stenosis.

    Science.gov (United States)

    Visser, Lance C; Scansen, Brian A; Schober, Karsten E

    2013-06-01

    A coronary artery anomaly characterized by the presence of a single left coronary ostium with absence of the right coronary ostium and an anomalous prepulmonic right coronary artery course was observed in two dogs with concurrent congenital pulmonary valve stenosis. This unique coronary artery anatomy is similar to the previously described single right coronary ostium with anomalous prepulmonic left coronary artery, the so-called type R2A anomaly, in that an anomalous coronary artery encircles the pulmonary valve annulus. Both dogs of this report, a boxer and an English bulldog, were of breeds known to be at risk for the type R2A anomaly. As such, veterinarians should be aware that the echocardiographic presence of a left coronary ostium in a dog with pulmonary valve stenosis does not exclude the possibility of a prepulmonic coronary artery anomaly that may enhance the risk of complications during balloon pulmonary valvuloplasty. A descriptive naming convention for coronary artery anomalies in dogs is also presented, which may be preferable to the older coding classification scheme. Copyright © 2013 Elsevier B.V. All rights reserved.

  18. The Right Ventricle Explains Sex Differences in Survival in Idiopathic Pulmonary Arterial Hypertension

    NARCIS (Netherlands)

    Jacobs, W.; van de Veerdonk, M.C.; Trip, P.; de Man, F.S.; Heymans, M.W.; Marcus, J.T.; Kawut, S.M.; Bogaard, H.J.; Boonstra, A.; Vonk-Noordegraaf, A.

    2014-01-01

    Background: Male sex is an independent predictor of worse survival in pulmonary arterial hypertension (PAH). This finding might be explained by more severe pulmonary vascular disease, worse right ventricular (RV) function, or different response to therapy. The aim of this study was to investigate

  19. Congenital anomalous/aberrant systemic artery to pulmonary venous fistula: Closure with vascular plugs & coil embolization

    Directory of Open Access Journals (Sweden)

    Pankaj Jariwala

    2014-01-01

    Full Text Available A 7-month-old girl with failure to thrive, who, on clinical and diagnostic evaluation [echocardiography & CT angiography] to rule out congenital heart disease, revealed a rare vascular anomaly called systemic artery to pulmonary venous fistula. In our case, there was dual abnormal supply to the entire left lung as1 anomalous supply by normal systemic artery [internal mammary artery]2 and an aberrant feeder vessel from the abdominal aorta. Left Lung had normal bronchial connections and normal pulmonary vasculature. The fistula drained through the pulmonary veins to the left atrium leading to ‘left–left shunt’. Percutaneous intervention in two stages was performed using Amplatzer vascular plugs and coil embolization to close them successfully. The patient gained significant weight in follow up with other normal developmental and mental milestones.

  20. Mid-term outcomes of patients undergoing adjustable pulmonary artery banding

    Directory of Open Access Journals (Sweden)

    Sachin Talwar

    2016-01-01

    Conclusions: Patients undergoing APAB fulfilled the desired objectives of the pulmonary artery banding (PAB with minimum PA complications in the mid-term. This added to the early postoperative benefits, makes the APAB an attractive alternative to the CPAB.

  1. Congenital pulmonary steal associated with Tetralogy of Fallot, right aortic arch and an isolated left carotid artery

    Energy Technology Data Exchange (ETDEWEB)

    Tozzi, R.; Kiley, R.; Doyle, E.; Hernanz-Schulman, M.; Genieser, N.; Ambrosino, M.; Pinto, R.

    1989-07-01

    In patients with Tetralogy of Fallot, collateral supply to the pulmonary artery from systemic arterial sources is frequently encountered. However, collateral blood flow arising from the cerebral circulation has, to our knowledge, not been reported in this condition. We describe a patient with Tetralogy of Fallot in whom the left pulmonary artery filled directly via retrograde flow from the left carotid artery. Review of the literature in search of a similar case was unrevealing. A theoretical embryologic basis for this anomaly is discussed. (orig.).

  2. Congenital pulmonary steal associated with Tetralogy of Fallot, right aortic arch and an isolated left carotid artery

    International Nuclear Information System (INIS)

    Tozzi, R.; Kiley, R.; Doyle, E.; Hernanz-Schulman, M.; Genieser, N.; Ambrosino, M.; Pinto, R.

    1989-01-01

    In patients with Tetralogy of Fallot, collateral supply to the pulmonary artery from systemic arterial sources is frequently encountered. However, collateral blood flow arising from the cerebral circulation has, to our knowledge, not been reported in this condition. We describe a patient with Tetralogy of Fallot in whom the left pulmonary artery filled directly via retrograde flow from the left carotid artery. Review of the literature in search of a similar case was unrevealing. A theoretical embryologic basis for this anomaly is discussed. (orig.)

  3. [Part II: basic hemodynamic monitoring and the use of pulmonary artery catheter].

    Science.gov (United States)

    Dias, Fernando Suparregui; Rezende, Ederlon; Mendes, Ciro Leite; Réa-Neto, Alvaro; David, Cid Marcos; Schettino, Guilherme; Lobo, Suzana Margareth Ajeje; Barros, Alberto; Silva, Eliézer; Friedman, Gilberto; Amaral, José Luiz Gomes do; Park, Marcelo; Monachini, Maristela; Oliveira, Mirella Cristine de; Assunção, Murillo Santucci César; Akamine, Nelson; Mello, Patrícia Veiga C; Pereira, Renata Andréa Pietro; Costa Filho, Rubens; Araújo, Sebastião; Félix Pinto, Sérgio; Ferreira, Sérgio; Mitushima, Simone Mattoso; Agareno, Sydney; Brilhante, Yuzeth Nóbrega de Assis

    2006-03-01

    Monitoring of vital functions is one of the most important tools in the management of critically ill patients. Nowadays is possible to detect and analyze a great deal of physiologic data using a lot of invasive and non-invasive methods. The intensivist must be able to select and carry out the most appropriate monitoring technique according to the patient requirements and taking into account the benefit/risk ratio. Despite the fast development of non invasive monitoring techniques, invasive hemodynamic monitoring using Pulmonary Artery Catheter still is one of the basic procedures in Critical Care. The aim was to define recommendations about clinical utility of basic hemodynamic monitoring methods and the Use of Pulmonary Artery Catheter. Modified Delphi methodology was used to create and quantify the consensus between the participants. AMIB indicated a coordinator who invited more six experts in the area of monitoring and hemodynamic support to constitute the Consensus Advisory Board. Twenty-five physicians and nurses selected from different regions of the country completed the expert panel, which reviewed the pertinent bibliography listed at the MEDLINE in the period from 1996 to 2004. Recommendations were made based on 55 questions about the use of central venous pressure, invasive arterial pressure, pulmonary artery catheter and its indications in different settings. Evaluation of central venous pressure and invasive arterial pressure, besides variables obtained by the PAC allow the understanding of cardiovascular physiology that is of great value to the care of critically ill patients. However, the correct use of these tools is fundamental to achieve the benefits due to its use.

  4. Role of iloprost and bosentan in pulmonary arterial hypertension ...

    African Journals Online (AJOL)

    Results: Several studies were able to outline the haemodynamic advantages of iloprost and bosentan in pulmonary arterial hypertension, as evident by improvement in six-minute walk test of patients treated with these agents. Conclusion: This review was able to outline the pharmacotherapeutic benefits and role played by ...

  5. Preventive Role of Hilar Parasympathetic Ganglia on Pulmonary Artery Vasospasm in Subarachnoid Hemorrhage: An Experimental Study.

    Science.gov (United States)

    Araz, Omer; Aydin, Mehmet Dumlu; Gundogdu, Betul; Altas, Ender; Cakir, Murteza; Calikoglu, Cagatay; Atalay, Canan; Gundogdu, Cemal

    2015-01-01

    Pulmonary arteries are mainly innervated by sympathetic vasoconstrictor and parasympathetic vasodilatory fibers. We examined whether there is a relationship between the neuron densities of hilar parasympathetic ganglia and pulmonary vasospasm in subarachnoid hemorrhage (SAH). Twenty-four rabbits were divided into two groups: control (n=8) and SAH (n=16). The animals were observed for 20 days following experimental SAH. The number of hilar parasympathetic ganglia and their neuron densities were determined. Proportion of pulmonary artery ring surface to lumen surface values was accepted as vasospasm index (VSI). Neuron densities of the hilar ganglia and VSI values were compared statistically. Animals in the SAH group experienced either mild (n=6) or severe (n=10) pulmonary artery vasospasm. In the control group, the mean VSI of pulmonary arteries was 0.777±0.048 and the hilar ganglion neuron density was estimated as 12.100±2.010/mm 3 . In SAH animals with mild vasospasm, VSI=1.148±0.090 and neuron density was estimated as 10.110±1.430/mm 3 ; in animals with severe vasospasm, VSI=1.500±0.120 and neuron density was estimated as 7.340±990/mm 3 . There was an inverse correlation between quantity and neuron density of hilar ganglia and vasospasm index value. The low numbers and low density of hilar parasympathetic ganglia may be responsible for the more severe artery vasospasm in SAH.

  6. Bullet embolism of pulmonary artery: a case report

    Directory of Open Access Journals (Sweden)

    Mauricio Gustavo Ieiri Yamanari

    2014-04-01

    Full Text Available The authors report the case of a patient victim of gunshots, with a very rare complication: venous bullet embolism from the left external iliac vein to the lingular segment of the left pulmonary artery. Diagnosis is made with whole-body radiography or computed tomography. Digital angiography is reserved for supplementary diagnosis or to be used as a therapeutic procedure.

  7. Racial and ethnic differences in pulmonary arterial hypertension

    OpenAIRE

    Al-Naamani, Nadine; Paulus, Jessica K.; Roberts, Kari E.; Pauciulo, Michael W.; Lutz, Katie; Nichols, William C.; Kawut, Steven M.

    2017-01-01

    This study explores the racial and ethnic differences in presentation, severity, and treatment of patients with pulmonary arterial hypertension (PAH) in a large multicenter registry. African American and Hispanic patients are more likely to present with associated PAH compared to non-Hispanic whites. Hispanic patients with PAH were less likely to be treated with PAH-specific medications compared to non-Hispanic whites.

  8. Bullet embolism of pulmonary artery: a case report

    International Nuclear Information System (INIS)

    Yamanari, Mauricio Gustavo Ieiri; Mansur, Maria Clara Dias; Kay, Fernando Uliana; Silverio, Paulo Rogerio Barboza; Jayanthi, Shri Krishna; Funari, Marcelo Buarque de Gusmao

    2014-01-01

    The authors report the case of a patient victim of gunshots, with a very rare complication: venous bullet embolism from the left external iliac vein to the lingular segment of the left pulmonary artery. Diagnosis is made with whole-body radiography or computed tomography. Digital angiography is reserved for supplementary diagnosis or to be used as a therapeutic procedure. (author)

  9. Arterial morphology responds differently to Captopril then N-acetylcysteine in a monocrotaline rat model of pulmonary hypertension

    Science.gov (United States)

    Molthen, Robert; Wu, Qingping; Baumgardt, Shelley; Kohlhepp, Laura; Shingrani, Rahul; Krenz, Gary

    2010-03-01

    Pulmonary hypertension (PH) is an incurable condition inevitably resulting in death because of increased right heart workload and eventual failure. PH causes pulmonary vascular remodeling, including muscularization of the arteries, and a reduction in the typically large vascular compliance of the pulmonary circulation. We used a rat model of monocrotaline (MCT) induced PH to evaluated and compared Captopril (an angiotensin converting enzyme inhibitor with antioxidant capacity) and N-acetylcysteine (NAC, a mucolytic with a large antioxidant capacity) as possible treatments. Twenty-eight days after MCT injection, the rats were sacrificed and heart, blood, and lungs were studied to measure indices such as right ventricular hypertrophy (RVH), hematocrit, pulmonary vascular resistance (PVR), vessel morphology and biomechanics. We implemented microfocal X-ray computed tomography to image the pulmonary arterial tree at intravascular pressures of 30, 21, 12, and 6 mmHg and then used automated vessel detection and measurement algorithms to perform morphological analysis and estimate the distensibility of the arterial tree. The vessel detection and measurement algorithms quickly and effectively mapped and measured the vascular trees at each intravascular pressure. Monocrotaline treatment, and the ensuing PH, resulted in a significantly decreased arterial distensibility, increased PVR, and tended to decrease the length of the main pulmonary trunk. In rats with PH induced by monocrotaline, Captopril treatment significantly increased arterial distensibility and decrease PVR. NAC treatment did not result in an improvement, it did not significantly increase distensibility and resulted in further increase in PVR. Interestingly, NAC tended to increase peripheral vascular density. The results suggest that arterial distensibility may be more important than distal collateral pathways in maintaining PVR at normally low values.

  10. Echobiometrics kidney and renal artery triplex doppler of canine fetuses

    Directory of Open Access Journals (Sweden)

    M.A.R. Feliciano

    2014-04-01

    Full Text Available The aim of this study was to assess the sogographic parameters and biometry of canine fetal kidneys using the B mode, and to determinate the vascular index of the fetal renal arteries using the Doppler Triplex. Twenty four Shi-tzu and Pug, weighting between 4 and 10kg, aging between 4 and 6 years old were evaluated. The B mode, the fetal renal echobiometry and regularity of the renal surface, echotexture and cortex:medular ratio were evaluated during the 5th, 6th, 7th and 8th weeks of pregnancy. At the same time point of the B mode evaluation, the Doppler Triplex was carried out to assess the sistolic peak velocity (SPV, end diastolic velocity (EDV, vascular resistive (RI and pulsatility index (PI. B mode revealed no fetal renal abnormalities and echobiometry showed important measurements during fetal development (P0.05. B mode and Doppler Triplex were important tools for the assessment of fetal renal development, using echobiometry and renal arterial index in canie fetuses.

  11. Endothelin receptor antagonist and airway dysfunction in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Borst Mathias M

    2009-12-01

    Full Text Available Abstract Background In idiopathic pulmonary arterial hypertension (IPAH, peripheral airway obstruction is frequent. This is partially attributed to the mediator dysbalance, particularly an excess of endothelin-1 (ET-1, to increased pulmonary vascular and airway tonus and to local inflammation. Bosentan (ET-1 receptor antagonist improves pulmonary hemodynamics, exercise limitation, and disease severity in IPAH. We hypothesized that bosentan might affect airway obstruction. Methods In 32 IPAH-patients (19 female, WHO functional class II (n = 10, III (n = 22; (data presented as mean ± standard deviation pulmonary vascular resistance (11 ± 5 Wood units, lung function, 6 minute walk test (6-MWT; 364 ± 363.7 (range 179.0-627.0 m, systolic pulmonary artery pressure, sPAP, 79 ± 19 mmHg, and NT-proBNP serum levels (1427 ± 2162.7 (range 59.3-10342.0 ng/L were measured at baseline, after 3 and 12 months of oral bosentan (125 mg twice per day. Results and Discussion At baseline, maximal expiratory flow at 50 and 25% vital capacity were reduced to 65 ± 25 and 45 ± 24% predicted. Total lung capacity was 95.6 ± 12.5% predicted and residual volume was 109 ± 21.4% predicted. During 3 and 12 months of treatment, 6-MWT increased by 32 ± 19 and 53 ± 69 m, respectively; p Conclusion This study gives first evidence in IPAH, that during long-term bosentan, improvement of hemodynamics, functional parameters or serum biomarker occur independently from persisting peripheral airway obstruction.

  12. A 50-year-old woman with haemoptysis, cough and tachypnea: cholesterol pneumonia accompanying with pulmonary artery hypertension.

    Science.gov (United States)

    Li, Mengxi; Zhang, Nuofu; Zhou, Ying; Li, Jinhui; Gu, Yingying; Wang, Jian; Liu, Chunli

    2017-03-01

    Lipoid pneumonia is an uncommon disease caused by the presence of lipid in the alveoli. Here we described a case of a 50-year-old woman with haemoptysis, cough and tachypnea, who was diagnosed with cholesterol pneumonia accompanying with pulmonary artery hypertension. The extremely high pulmonary artery pressure achieved, in this case, is alarming and should alert the physicians that the cholesterol pneumonia may be one of the underlying causes of pulmonary artery hypertension. After a treatment of methylprednisolone, her clinical symptoms were significantly improved, which suggested that steroid might be a promising therapeutic for patients with cholesterol pneumonia. © 2015 John Wiley & Sons Ltd.

  13. Cardiac causes of pulmonary arterial hypertension: assessment with multidetector CT

    Energy Technology Data Exchange (ETDEWEB)

    Hoey, Edward T.D.; Gopalan, Deepa; Agrawal, S.K.B. [Papworth Hospital, Cambridge (United Kingdom); Screaton, Nicholas J. [Papworth Hospital, Cambridge (United Kingdom); Papworth Hospital NHS Trust, Diagnostic Centre, Department of Radiology, Papworth Everard, Cambridgeshire (United Kingdom)

    2009-11-15

    The causes of pulmonary arterial hypertension (PAH) are diverse and include multiple congenital and acquired cardiac diseases as well as diseases primarily affecting the pulmonary vasculature, lung, pleura and chest wall. The traditional role of CT in evaluating PAH includes assessment of pulmonary vasculature and lung parenchyma with limited assessment of the heart. Advances in multidetector CT technology with improved spatial and temporal resolution now permit accurate delineation of cardiac morphology. CT pulmonary angiography (CTPA) is widely utilised in the workup of patients with suspected pulmonary vascular disease and can identify both pulmonary and cardiac causes. As the initial presentation for CTPA is often precipitated by nonspecific, unexplained symptoms and therefore undertaken by a general radiologist, it is important that a systematic approach to the interpretation of these studies, including cardiac evaluation, is routinely adopted. This paper reviews the CT evaluation in pulmonary hypertension with a particular focus on the cardiac causes, their subclassification into congenital systemic to pulmonary shunts and secondary to left heart disease, and their imaging features. It emphasises the use of a systematic approach to interpretation of CTPA examinations both in patients with known PAH and those with previously unsuspected disease. (orig.)

  14. Cardiac causes of pulmonary arterial hypertension: assessment with multidetector CT

    International Nuclear Information System (INIS)

    Hoey, Edward T.D.; Gopalan, Deepa; Agrawal, S.K.B.; Screaton, Nicholas J.

    2009-01-01

    The causes of pulmonary arterial hypertension (PAH) are diverse and include multiple congenital and acquired cardiac diseases as well as diseases primarily affecting the pulmonary vasculature, lung, pleura and chest wall. The traditional role of CT in evaluating PAH includes assessment of pulmonary vasculature and lung parenchyma with limited assessment of the heart. Advances in multidetector CT technology with improved spatial and temporal resolution now permit accurate delineation of cardiac morphology. CT pulmonary angiography (CTPA) is widely utilised in the workup of patients with suspected pulmonary vascular disease and can identify both pulmonary and cardiac causes. As the initial presentation for CTPA is often precipitated by nonspecific, unexplained symptoms and therefore undertaken by a general radiologist, it is important that a systematic approach to the interpretation of these studies, including cardiac evaluation, is routinely adopted. This paper reviews the CT evaluation in pulmonary hypertension with a particular focus on the cardiac causes, their subclassification into congenital systemic to pulmonary shunts and secondary to left heart disease, and their imaging features. It emphasises the use of a systematic approach to interpretation of CTPA examinations both in patients with known PAH and those with previously unsuspected disease. (orig.)

  15. Pulmonary vasculature in dogs assessed by three-dimensional fractal analysis and chemometrics

    DEFF Research Database (Denmark)

    Müller, Anna V; Marschner, Clara B; Kristensen, Annemarie T

    2017-01-01

    Fractal analysis of canine pulmonary vessels could allow quantification of their space-filling properties. Aims of this prospective, analytical, cross-sectional study were to describe methods for reconstructing three dimensional pulmonary arterial vascular trees from computed tomographic pulmonary...... angiogram, applying fractal analyses of these vascular trees in dogs with and without diseases that are known to predispose to thromboembolism, and testing the hypothesis that diseased dogs would have a different fractal dimension than healthy dogs. A total of 34 dogs were sampled. Based on computed...... for each dog using a semiautomated segmentation technique. Vascular three-dimensional reconstructions were then evaluated using fractal analysis. Fractal dimensions were analyzed, by group, using analysis of variance and principal component analysis. Fractal dimensions were significantly different among...

  16. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    Energy Technology Data Exchange (ETDEWEB)

    Robida, A.; Fettich, D.

    1985-09-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease.

  17. A region-specific quantitative profile of autonomic innervation of the canine left atrium and pulmonary veins.

    Science.gov (United States)

    Gao, Chong-han; Wang, Fei; Jiang, Rong; Zhang, Jin; Mou, Huamin; Yin, Yue-hui

    2011-07-05

    The aim of the present study was to determine and quantify the cardiac autonomic innervation of the canine atria and pulmonary vein. Tissue specimens were taken from the canine pulmonary veins (PVs), posterior left atrium (PLA), left atrial roof (LAR), anterior left atrium (ALA), interatrial septum (IAS), and left atrial appendage (LAA) respectively for immunohistochemical analysis and nerve density determination. Both sympathetic and parasympathetic nerve densities decreased in the order: PLA>PV>IAS>LAR>ALA>LAA. For sympathetic nerve, multiple comparisons between any two regions showed a significant difference (PIAS vs. LAR, and LAR vs. ALA; for parasympathetic nerve, all the differences between any pair of regions were statistically significant (PIAS vs. LAR, LAR vs. ALA, and ALA vs. LAA. For both nerve types, there was a decreasing gradient of nerve densities from the external to internal layer (P<0.001, for each comparisons). Nerve density at the ostia for either nerve type was significantly higher than at the distal segments of PVs (P<0.001). In summary, the LA and PVs are innervated by sympathetic and parasympathetic nerves in a regionally heterogeneous way, which may be important for the pathophysiological investigation and ablation therapy of atrial fibrillation (AF). Copyright © 2011 Elsevier B.V. All rights reserved.

  18. A review of wave mechanics in the pulmonary artery with an emphasis on wave intensity analysis

    DEFF Research Database (Denmark)

    Su, Junjing; Hilberg, Ole; Howard, Luke

    2016-01-01

    Mean pulmonary arterial pressure and pulmonary vascular resistance (PVR) remain the most common haemodynamic measures to evaluate the severity and prognosis of pulmonary hypertension. However, PVR only captures the non-oscillatory component of the right ventricular hydraulic load and neglects...

  19. Patent ductus arteriosus with persistent pulmonary artery hypertension after transcatheter closure.

    Science.gov (United States)

    Feng, Jianqi; Kong, Xiangqing; Sheng, Yanhui; Yang, Rong

    2016-01-01

    To observe the change in pulmonary artery systolic pressure (PASP) of patients with persistent pulmonary arterial hypertension (PAH) after patent ductus arteriosus (PDA) occlusion. After occlusion of PDA in patients with PAH, some patients still tend to suffer from persistent PAH. A chest X-ray, an electrocardiogram, and an echocardiogram were performed on nine patients at 24 hours, 1 and 6 months, and 1 year serially. There was a significant fall ( P 0.05). During the follow up, there was a further fall in the PASP in five patients (No 1, 5, 6, 7, and 8). Four patients (No 2, 3, 4, and 8) showed the evidence of worsening PAH and were treated with sildenafil. Patient 2 died from acute right heart failure after a period of 11 months from the time of transcatheter closure, triggered by pulmonary infection. Some patients with borderline hemodynamic data with PDA and PAH can deteriorate or keep sustained PAH after PDA closure. The treatment of permanent closure to these patients must be cautious.

  20. Anomalous left coronary artery from the pulmonary artery with a large patent ductus arteriosus: aversion of a catastrophe.

    Science.gov (United States)

    Aggarwal, Sanjeev; Delius, Ralph E; Pettersen, Michael D

    2013-01-01

    We present an infant who had an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) and a large patent ductus arteriosus (PDA), who was diagnosed before a potentially catastrophic closure of PDA. In the presence of normal left ventricular function and the absence of coronary artery collaterals, it is difficult to diagnose ALCAPA. A disproportionate degree of left ventricular dilation and severity of mitral valve regurgitation relative to the degree of PDA shunt, and echogenic papillary muscles on an echocardiogram should raise a suspicion of coronary artery anomalies. The infant underwent surgical ligation of PDA with translocation of coronary arteries and had an uneventful recovery. © 2012 Wiley Periodicals, Inc.

  1. Hyperthyroidism enhances 5-HT-induced contraction of the rat pulmonary artery: role of calcium-activated chloride channel activation.

    Science.gov (United States)

    Oriowo, Mabayoje A; Oommen, Elsie; Khan, Islam

    2011-11-01

    Experimentally-induced hyperthyroidism in rodents is associated with signs and symptoms of pulmonary hypertension. The main objective of the present study was to investigate the effect of thyroxine-induced pulmonary hypertension on the contractile response of the pulmonary artery to 5-HT and the possible underlying signaling pathway. 5-HT concentration-dependently contracted artery segments from control and thyroxine-treated rats with pD(2) values of 5.04 ± 0.19 and 5.34 ± 0.14, respectively. The maximum response was significantly greater in artery segments from thyroxine-treated rats. Neither BW 723C86 (5-HT(2B)-receptor agonist) nor CP 93129 (5-HT(1B)-receptor agonist) contracted ring segments of the pulmonary artery from control and thyroxine-treated rats at concentrations up to 10(-4)M. There was no significant difference in the level of expression of 5-HT(2A)-receptor protein between the two groups. Ketanserin (3 × 10(-8)M) produced a rightward shift of the concentration-response curve to 5-HT in both groups with equal potency (-logK(B) values were 8.1 ± 0.2 and 7.9 ± 0.1 in control and thyroxine-treated rats, respectively). Nifedipine (10(-6)M) inhibited 5-HT-induced contractions in artery segments from control and thyroxine-treated rats and was more effective against 5-HT-induced contraction in artery segments for thyroxine-treated rats. The calcium-activated chloride channel blocker, niflumic acid (10(-4)M) also inhibited 5-HT-induced contractions in artery segments from control and thyroxine-treated rats and was more effective against 5-HT-induced contraction in artery segments for thyroxine-treated rats. It was concluded that hyperthyroidism enhanced 5-HT-induced contractions of the rat pulmonary artery by a mechanism involving increased activity of calcium-activated chloride channels. Copyright © 2011 Elsevier B.V. All rights reserved.

  2. Where does the pulmonary artery catheter float: Transesophageal echocardiography evaluation

    Directory of Open Access Journals (Sweden)

    Deepak K Tempe

    2015-01-01

    Full Text Available Background: Pulmonary artery (PA catheter provides a variety of cardiac and hemodynamic parameters. In majority of the patients, the catheter tends to float in the right pulmonary artery (RPA than the left pulmonary artery (LPA. We evaluated the location of PA catheter with the help of transesophageal echocardiography (TEE to know the incidence of its localization. Three views were utilized for this purpose; midesophageal ascending aorta (AA short-axis view, modified mid esophageal aortic valve long-axis view, and modified bicaval view. Methods: We enrolled 135 patients undergoing elective cardiac surgery where both the PA catheter and TEE were to be used; for this prospective observational study. PA catheter was visualized by TEE in the above mentioned views and the degree of clarity of visualization by three views was also noted. Position of the PA catheter was further confirmed by a postoperative chest radiograph. Results: One patient was excluded from the data analysis. PA catheter was visualized in RPA in 129 patients (96% and in LPA in 4 patients (3%. In 1 patient, the catheter was visualized in main PA in the chest radiograph. The midesophageal AA short-axis, modified aortic valve long-axis, and modified bicaval view provided good visualization in 51.45%, 57.4%, and 62.3% patients respectively. Taken together, PA catheter visualization was good in 128 (95.5% patients. Conclusion: We conclude that the PA catheter has a high probability of entering the RPA as compared to LPA (96% vs. 3% and TEE provides good visualization of the catheter in RPA.

  3. Localized-low attenuation of the lung on thin-section CT in experimentally induced pulmonary arterial occlusion with balloon catheter in pigs

    International Nuclear Information System (INIS)

    Lee, Hyun Ju; Goo, Jin Mo; Im, Jung Gi; Kim, Ji Hye

    2008-01-01

    To determine whether a localized low-attenuation (LLA) is induced on a thin-section CT (TSCT) during an acute pulmonary arterial occlusion in pigs. In eight pigs, 14 sites of the descending pulmonary artery were obstructed using balloon catheters. The lung TSCTs were obtained immediately after pulmonary artery obstruction (n=13), 10 min (n=10), 30 min (n=14) and 60 min (n=14) after pulmonary artery obstruction at the end of expiration. The TSCTs were also obtained after balloon-deflation at the end of expiration (n=11) and with the balloon-reinflation at inspiration (n=6). Of the 14 sites of pulmonary artery obstruction, 11 (79%) showed LLA. However, LLA progressively became fainter or disappeared on a follow-up CT in seven sites. When the balloon was deflated, 10 of the 11 sites measured showed no change in lung attenuation. After full inspiration, LLA disappeared in three of the six sites. The corresponding areas of LLA on the CT showed a statistically significant increase compared to the baseline CT immediately after inflation (ρ =0.021) and 30 minutes after inflation (ρ = 0.041), and after balloon deflation (ρ = 0.036). LLA was induced by acute pulmonary artery obstruction. However, LLA, gradually faded over the 60 minutes following obstruction. LLAs were maintained despite the restoration of pulmonary arterial flow, but disappeared as a result of a full inspiration. Thus, LLA might be caused by air trapping

  4. Endovascular Treatment of an Aneurysmal Aberrant Systemic Artery Supplying a Pulmonary Sequestrum

    DEFF Research Database (Denmark)

    Kristensen, Katrine Lawaetz; Duus, Louise Aarup; Elle, Bo

    2015-01-01

    An aberrant systemic artery originating from the abdominal aorta supplying a pulmonary sequestration is a rare congenital malformation. This causes a left-to-left shunt. Symptoms include recurrent pneumonias, hemoptysis, and, in the long term, heart failure. Aneurysm of the aberrant vessel...... is rarely seen. Traditionally, treatment of pulmonary sequestrations includes ligation of the feeding vessel and lobectomy. A new promising treatment is an endovascular approach. Only a few cases describe endovascular treatment of pulmonary sequestration. This is the first published case of a giant...

  5. Pleural subxyphoid drain confers better pulmonary function and clinical outcomes in chronic obstructive pulmonary disease after off-pump coronary artery bypass grafting: a randomized controlled trial.

    Science.gov (United States)

    Guizilini, Solange; Viceconte, Marcela; Esperança, Gabriel Tavares da M; Bolzan, Douglas W; Vidotto, Milena; Moreira, Rita Simone L; Câncio, Andréia Azevedo; Gomes, Walter J

    2014-01-01

    To evaluate the lung function and clinical outcome in severe chronic obstructive pulmonary disease in patients undergoing off-pump coronary artery bypass grafting with left internal thoracic artery graft, comparing the pleural drain insertion in the intercostal versus subxyphoid region. A randomized controlled trial. Chronic obstructive pulmonary disease patients were randomized into two groups according pleural drain site: II group (n=27) - pleural drain in intercostal space; SI group (n=29) - pleural drain in the subxyphoid region. Spirometry values (Forced Vital Capacity - and Forced expiratory volume in 1 second) were obtained on preoperative and 1, 3 and 5 postoperative days. Chest x-ray from preoperative until postoperative day 5 (POD5) was performed for monitoring respiratory events, such as atelectasis and pleural effusion. Pulmonary shunt fraction and pain score was evaluate preoperatively and on postoperative day 1. In both groups there was a significant decrease of the spirometry values (Forced Vital Capacity and Forced expiratory volume in 1 second) until POD5 (Ppleural drainage in severe Chronic obstructive pulmonary disease patients determined better preservation and recovery of pulmonary capacity and volumes with lower pulmonary shunt fraction and better clinical outcomes on early postoperative off-pump coronary artery bypass grafting.

  6. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    International Nuclear Information System (INIS)

    Robida, A.; Fettich, D.

    1985-01-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease. (orig.)

  7. Acute pulmonary edema following inflation of arterial tourniquet.

    Science.gov (United States)

    Santhosh, M C B; Pai, R B; Rao, R P

    2014-10-01

    Arterial tourniquets are used as one of the methods for reducing blood loss and for allowing blood free surgical field. A 20-year-old, 45 kg healthy female with a sphere shaped pendunculated hemangioma in the popliteal fossa of her left lower limb was applied with arterial tourniquet after exsanguination. The procedure was performed under general anesthesia. Soon after exsanguination and tourniquet inflation, the patient developed pulmonary edema which subsided after deflating the tourniquet. The clinical evolution, treatment and pathophysiology of this complication are described. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

  8. Single-energy computed tomography-based pulmonary perfusion imaging: Proof-of-principle in a canine model

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, Tokihiro, E-mail: toyamamoto@ucdavis.edu [Department of Radiation Oncology, University of California Davis School of Medicine, Sacramento, California 95817 (United States); Kent, Michael S.; Wisner, Erik R. [Department of Surgical and Radiological Sciences, University of California Davis School of Veterinary Medicine, Davis, California 95616 (United States); Johnson, Lynelle R.; Stern, Joshua A. [Department of Medicine and Epidemiology, University of California Davis School of Veterinary Medicine, Davis, California 95616 (United States); Qi, Lihong [Department of Public Health Sciences, University of California Davis, Davis, California 95616 (United States); Fujita, Yukio [Department of Radiation Oncology, Tokai University, Isehara, Kanagawa 259-1193 (Japan); Boone, John M. [Department of Radiology, University of California Davis School of Medicine, Sacramento, California 95817 (United States)

    2016-07-15

    Purpose: Radiotherapy (RT) that selectively avoids irradiating highly functional lung regions may reduce pulmonary toxicity, which is substantial in lung cancer RT. Single-energy computed tomography (CT) pulmonary perfusion imaging has several advantages (e.g., higher resolution) over other modalities and has great potential for widespread clinical implementation, particularly in RT. The purpose of this study was to establish proof-of-principle for single-energy CT perfusion imaging. Methods: Single-energy CT perfusion imaging is based on the following: (1) acquisition of end-inspiratory breath-hold CT scans before and after intravenous injection of iodinated contrast agents, (2) deformable image registration (DIR) for spatial mapping of those two CT image data sets, and (3) subtraction of the precontrast image data set from the postcontrast image data set, yielding a map of regional Hounsfield unit (HU) enhancement, a surrogate for regional perfusion. In a protocol approved by the institutional animal care and use committee, the authors acquired CT scans in the prone position for a total of 14 anesthetized canines (seven canines with normal lungs and seven canines with diseased lungs). The elastix algorithm was used for DIR. The accuracy of DIR was evaluated based on the target registration error (TRE) of 50 anatomic pulmonary landmarks per subject for 10 randomly selected subjects as well as on singularities (i.e., regions where the displacement vector field is not bijective). Prior to perfusion computation, HUs of the precontrast end-inspiratory image were corrected for variation in the lung inflation level between the precontrast and postcontrast end-inspiratory CT scans, using a model built from two additional precontrast CT scans at end-expiration and midinspiration. The authors also assessed spatial heterogeneity and gravitationally directed gradients of regional perfusion for normal lung subjects and diseased lung subjects using a two-sample two-tailed t

  9. Single-energy computed tomography-based pulmonary perfusion imaging: Proof-of-principle in a canine model.

    Science.gov (United States)

    Yamamoto, Tokihiro; Kent, Michael S; Wisner, Erik R; Johnson, Lynelle R; Stern, Joshua A; Qi, Lihong; Fujita, Yukio; Boone, John M

    2016-07-01

    Radiotherapy (RT) that selectively avoids irradiating highly functional lung regions may reduce pulmonary toxicity, which is substantial in lung cancer RT. Single-energy computed tomography (CT) pulmonary perfusion imaging has several advantages (e.g., higher resolution) over other modalities and has great potential for widespread clinical implementation, particularly in RT. The purpose of this study was to establish proof-of-principle for single-energy CT perfusion imaging. Single-energy CT perfusion imaging is based on the following: (1) acquisition of end-inspiratory breath-hold CT scans before and after intravenous injection of iodinated contrast agents, (2) deformable image registration (DIR) for spatial mapping of those two CT image data sets, and (3) subtraction of the precontrast image data set from the postcontrast image data set, yielding a map of regional Hounsfield unit (HU) enhancement, a surrogate for regional perfusion. In a protocol approved by the institutional animal care and use committee, the authors acquired CT scans in the prone position for a total of 14 anesthetized canines (seven canines with normal lungs and seven canines with diseased lungs). The elastix algorithm was used for DIR. The accuracy of DIR was evaluated based on the target registration error (TRE) of 50 anatomic pulmonary landmarks per subject for 10 randomly selected subjects as well as on singularities (i.e., regions where the displacement vector field is not bijective). Prior to perfusion computation, HUs of the precontrast end-inspiratory image were corrected for variation in the lung inflation level between the precontrast and postcontrast end-inspiratory CT scans, using a model built from two additional precontrast CT scans at end-expiration and midinspiration. The authors also assessed spatial heterogeneity and gravitationally directed gradients of regional perfusion for normal lung subjects and diseased lung subjects using a two-sample two-tailed t-test. The mean TRE

  10. Single-energy computed tomography-based pulmonary perfusion imaging: Proof-of-principle in a canine model

    International Nuclear Information System (INIS)

    Yamamoto, Tokihiro; Kent, Michael S.; Wisner, Erik R.; Johnson, Lynelle R.; Stern, Joshua A.; Qi, Lihong; Fujita, Yukio; Boone, John M.

    2016-01-01

    Purpose: Radiotherapy (RT) that selectively avoids irradiating highly functional lung regions may reduce pulmonary toxicity, which is substantial in lung cancer RT. Single-energy computed tomography (CT) pulmonary perfusion imaging has several advantages (e.g., higher resolution) over other modalities and has great potential for widespread clinical implementation, particularly in RT. The purpose of this study was to establish proof-of-principle for single-energy CT perfusion imaging. Methods: Single-energy CT perfusion imaging is based on the following: (1) acquisition of end-inspiratory breath-hold CT scans before and after intravenous injection of iodinated contrast agents, (2) deformable image registration (DIR) for spatial mapping of those two CT image data sets, and (3) subtraction of the precontrast image data set from the postcontrast image data set, yielding a map of regional Hounsfield unit (HU) enhancement, a surrogate for regional perfusion. In a protocol approved by the institutional animal care and use committee, the authors acquired CT scans in the prone position for a total of 14 anesthetized canines (seven canines with normal lungs and seven canines with diseased lungs). The elastix algorithm was used for DIR. The accuracy of DIR was evaluated based on the target registration error (TRE) of 50 anatomic pulmonary landmarks per subject for 10 randomly selected subjects as well as on singularities (i.e., regions where the displacement vector field is not bijective). Prior to perfusion computation, HUs of the precontrast end-inspiratory image were corrected for variation in the lung inflation level between the precontrast and postcontrast end-inspiratory CT scans, using a model built from two additional precontrast CT scans at end-expiration and midinspiration. The authors also assessed spatial heterogeneity and gravitationally directed gradients of regional perfusion for normal lung subjects and diseased lung subjects using a two-sample two-tailed t

  11. The mechanical properties of the systemic and pulmonary arteries of Python regius correlate with blood pressures.

    Science.gov (United States)

    van Soldt, Benjamin J; Danielsen, Carl Christian; Wang, Tobias

    2015-12-01

    Pythons are unique amongst snakes in having different pressures in the aortas and pulmonary arteries because of intraventricular pressure separation. In this study, we investigate whether this correlates with different blood vessel strength in the ball python Python regius. We excised segments from the left, right, and dorsal aortas, and from the two pulmonary arteries. These were subjected to tensile testing. We show that the aortic vessel wall is significantly stronger than the pulmonary artery wall in P. regius. Gross morphological characteristics (vessel wall thickness and correlated absolute amount of collagen content) are likely the most influential factors. Collagen fiber thickness and orientation are likely to have an effect, though the effect of collagen fiber type and cross-links between fibers will need further study. © 2015 Wiley Periodicals, Inc.

  12. Paracrine effects of bone marrow-derived endothelial progenitor cells: cyclooxygenase-2/prostacyclin pathway in pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Dong-Mei Jiang

    Full Text Available BACKGROUND: Endothelial dysfunction is the pathophysiological characteristic of pulmonary arterial hypertension (PAH. Some paracrine factors secreted by bone marrow-derived endothelial progenitor cells (BMEPCs have the potential to strengthen endothelial integrity and function. This study investigated whether BMEPCs have the therapeutic potential to improve monocrotaline (MCT-induced PAH via producing vasoprotective substances in a paracrine fashion. METHODS AND RESULTS: Bone marrow-derived mononuclear cells were cultured for 7 days to yield BMEPCs. 24 hours or 3 weeks after exposure to BMEPCs in vitro or in vivo, the vascular reactivity, cyclooxygenase-2 (COX-2 expression, prostacyclin (PGI2 and cAMP release in isolated pulmonary arteries were examined respectively. Treatment with BMEPCs could improve the relaxation of pulmonary arteries in MCT-induced PAH and BMEPCs were grafted into the pulmonary bed. The COX-2/prostacyclin synthase (PGIS and its progenies PGI2/cAMP were found to be significantly increased in BMEPCs treated pulmonary arteries, and this action was reversed by a selective COX-2 inhibitor, NS398. Moreover, the same effect was also observed in conditioned medium obtained from BMEPCs culture. CONCLUSIONS: Implantation of BMEPCs effectively ameliorates MCT-induced PAH. Factors secreted in a paracrine fashion from BMEPCs promote vasoprotection by increasing the release of PGI2 and level of cAMP.

  13. In Pulmonary Arterial Hypertension, Reduced BMPR2 Promotes Endothelial-to-Mesenchymal Transition via HMGA1 and Its Target Slug

    NARCIS (Netherlands)

    Hopper, Rachel K.; Moonen, Jan-Renier A. J.; Diebold, Isabel; Cao, Aiqin; Rhodes, Christopher J.; Tojais, Nancy F.; Hennigs, Jan K.; Gu, Mingxia; Wang, Lingli; Rabinovitch, Marlene

    2016-01-01

    Background-We previously reported high-throughput RNA sequencing analyses that identified heightened expression of the chromatin architectural factor High Mobility Group AT-hook 1 (HMGA1) in pulmonary arterial endothelial cells (PAECs) from patients who had idiopathic pulmonary arterial hypertension

  14. Pericardial effusion in pulmonary arterial hypertension

    Science.gov (United States)

    2013-01-01

    Abstract Pulmonary arterial hypertension (PAH) is a serious condition that can lead to right heart failure and death. Pericardial effusion in PAH is associated with significant morbidity and mortality, and its pathogenesis is complex and poorly understood. There are few data on the prevalence of pericardial effusion in PAH, and more importantly, the management of pericardial effusion is controversial. Current literature abounds with case reports, case series, and retrospective studies that have limited value for assessing this association. Hence, we summarize the available evidence on this ominous association and identify areas for future research. PMID:24618534

  15. A clinical evaluation of the intraluminal bronchial artery infusion with reptilase or plus additional target artery embolization in treatment of severe hemoptysis in patients with pulmonary tuberculosis

    International Nuclear Information System (INIS)

    Lu Puxuan; Cai Xiongmao; Cao Mairui; Yu Weiye; Yang Gendong; Chen Xinchun; Sun Yanzhi; Liu Jinqing; Gong Xiaolong; Fu Xiangdong; Deng Yongcong; Liu Zhi; Zheng Guangping

    2003-01-01

    Objective: To investigate the effect of bronchial artery infusion with Reptilase or plus additional target artery embolization in treatment of massive hemoptysis in patients with pulmonary tuberculosis. Methods: Among 34 patients with massive hemoptysis (≥600 ml/24 hours) resultant from pulmonary tuberculosis, 21 cases (group 1) received by bronchial artery infusion with Reptilase (1-3KU) plus target artery embolization with gelfoam sponge. In the rest 13 cases (group 2) a simple bronchial artery infusion with Reptilase (1-3KU) was performed because the bleeding target arteries were inaccessible. Results: In total 32 cases out of 34 (94.1%) the hemoptysis ceased after the intervention, while 1 case in each group respectively needed further surgery. Among the cured 34 cases, single treatment was done in 32 cases, twice interventions were performed in 1 case and triple intraluminal therapies were done in 1 case. There is no significant difference between the efficiency of the treatment applied in group 1 and group 2 (P>0.05). Conclusion: Bronchial artery infusion with Reptilase or plus additional target artery embolization is effective and safe method in the management of massive hemoptysis in patients with pulmonary tuberculosis. When the target the bleed artery is inaccessible, local intraluminal infusion with Reptilase (1-3KU) is an acceptable option

  16. β2-Adrenergic receptor-dependent attenuation of hypoxic pulmonary vasoconstriction prevents progression of pulmonary arterial hypertension in intermittent hypoxic rats.

    Directory of Open Access Journals (Sweden)

    Hisashi Nagai

    Full Text Available In sleep apnea syndrome (SAS, intermittent hypoxia (IH induces repeated episodes of hypoxic pulmonary vasoconstriction (HPV during sleep, which presumably contribute to pulmonary arterial hypertension (PAH. However, the prevalence of PAH was low and severity is mostly mild in SAS patients, and mild or no right ventricular hypertrophy (RVH was reported in IH-exposed animals. The question then arises as to why PAH is not a universal finding in SAS if repeated hypoxia of sufficient duration causes cycling HPV. In the present study, rats underwent IH at a rate of 3 min cycles of 4-21% O2 for 8 h/d for 6 w. Assessment of diameter changes in small pulmonary arteries in response to acute hypoxia and drugs were performed using synchrotron radiation microangiography on anesthetized rats. In IH-rats, neither PAH nor RVH was observed and HPV was strongly reversed. Nadolol (a hydrophilic β(1, 2-blocker augmented the attenuated HPV to almost the same level as that in N-rats, but atenolol (a hydrophilic β1-blocker had no effect on the HPV in IH. These β-blockers had almost no effect on the HPV in N-rats. Chronic administration of nadolol during 6 weeks of IH exposure induced PAH and RVH in IH-rats, but did not in N-rats. Meanwhile, atenolol had no effect on morphometric and hemodynamic changes in N and IH-rats. Protein expression of the β1-adrenergic receptor (AR was down-regulated while that of β2AR was preserved in pulmonary arteries of IH-rats. Phosphorylation of p85 (chief component of phosphoinositide 3-kinase (PI3K, protein kinase B (Akt, and endothelial nitric oxide synthase (eNOS were abrogated by chronic administration of nadolol in the lung tissue of IH-rats. We conclude that IH-derived activation of β2AR in the pulmonary arteries attenuates the HPV, thereby preventing progression of IH-induced PAH. This protective effect may depend on the β2AR-Gi mediated PI3K/Akt/eNOS signaling pathway.

  17. Inferior phrenic arteries supply to the pulmonary hemorrhagic lesions: angiographic identification and interventional management

    International Nuclear Information System (INIS)

    Wang Maoqiang; Liu Fengyong; Duan Feng; Song Peng; Wang Zhijun; Wang Zhongpu

    2007-01-01

    Objective: To describe the manifestations of the inferior phrenic arteries (IPA) supply to the pulmonary hemorrhagic lesions and to evaluate the safety and efficacy of transcatheter arterial embolization (TAE) of the IPA. Methods: The clinical data and imaging findings of eighteen patients with the additional blood supply to the pulmonary hemorrhagic lesions from the IPA were evaluated retrospectively. The causes of the bleeding were lung malignancies in 9, bronchiectasis in 7, and chronic inflammation in 2 patients. TAE supplementally was performed in patients with IPA supply to the pulmonary lesions, using polyvinyl alcohol particles, gelatin sponge particles, and microcoils. Results: Selective arteriogram demonstrates an enlarged IPA, with numerous branches and hypervascularity in all 18 cases, with tumor staining in 9, the contrast material extravasation in 6, and non-specific staining in 2 cases. In addition, IPA-to-pulmonary shunting was found in 9 cases. All the lesions supplying by IPA were adjacent to the pleurae, including adjacent to the diaphragmatic pleura in 11, the mediastinal pleura in 5, and the lateral pleura of the lower lobe in 2 cases. Technical success of IPA embolization was achieved in the 18 cases. Embolization of other nonbronchial systemic arteries (the internal thoracic artery in 7 and intercostal artery in 3) was performed at the same session. All bleeding ceased immediately after' supplemental IPA embolization. Follow-up time ranged from 8 months to 4 years. Mild recurrent hemoptysis occurred in 3 patients at 1,2, 6 months respectively, after the embolization. These patients were responsive to conservative management. Recurrent bleeding did not occur in 15 patients during the follow-up. Conclusion: The pulmonary hemorrhagic lesions, especially adjacent to the diaphragmatic and mediastinal pleurae, can be supplied by IPA, and may result in clinical failure following BAE. Supplemental TAE of IPA is a safe and effective adjunct to BAE in

  18. Lung Abscess as Delayed Manifestation of Pulmonary Arterial Narrowing After Sleeve Resection.

    Science.gov (United States)

    Frenzen, Frederik S; Lesser, Thomas; Platzek, Ivan; Riede, Frank-Thomas; Kolditz, Martin

    2017-08-01

    A patient who had undergone right upper bilobectomy because of a carcinoid experienced lung abscesses 17 months after operation. After recurrences, despite different antibiotic agents, dual-energy computed tomography showed subtotal stenosis of the right lower lobe pulmonary artery with marked pulmonary perfusion-reduction. Rare causes of lung-abscesses should be considered. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  19. Clopidogrel in infants with systemic-to-pulmonary-artery shunts

    DEFF Research Database (Denmark)

    Wessel, David L; Berger, Felix; Li, Jennifer S

    2013-01-01

    BACKGROUND: Infants with cyanotic congenital heart disease palliated with placement of a systemic-to-pulmonary-artery shunt are at risk for shunt thrombosis and death. We investigated whether the addition of clopidogrel to conventional therapy reduces mortality from any cause and morbidity relate......-related morbidity. (Funded by Sanofi-Aventis and Bristol-Myers Squibb; ClinicalTrials.gov number, NCT00396877.)....

  20. Pulmonary arterial hypertension as a manifestation of lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Stark, P; Sargent, E N; Boylen, T; Jaramillo, D

    1987-08-01

    We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor.

  1. Electrocautery device does not provide adequate pulmonary vessel sealing in transumbilical anatomic pulmonary lobectomy.

    Science.gov (United States)

    Liu, Hung-Ping; Chu, Yen; Wu, Yi-Cheng; Hsieh, Ming-Ju; Liu, Chieng-Ying; Chen, Tzu-Ping; Chao, Yin-Kai; Wu, Ching-Yang; Yeh, Chi-Ju; Ko, Po-Jen; Liu, Yun-Hen

    2016-05-01

    Safe pulmonary vessel sealing device plays a crucial role in anatomic lung resection. In 2014, we reported high rates of massive bleeding complications during transumbilical lobectomy in a canine model due to difficulty in managing the pulmonary vessel with an endostapler. In this animal survival series, we aimed to evaluate the outcome of pulmonary vessel sealing with an electrocautery device to simplify the transumbilical thoracic surgery. Under general anesthesia, a 3-cm longitudinal incision was made over the umbilicus. Under video guidance, a bronchoscope was inserted through the incision for exploration. The diaphragmatic wound was created with an electrocautery knife and used as the entrance into the thoracic cavity. Using the transumbilical technique, anatomic lobectomy was performed with electrosurgical devices and endoscopic vascular staplers in 15 canines. Transumbilical endoscopic anatomic lobectomy was successfully completed in 12 of the 15 animals. Intraoperative bleeding developed in three animals during pulmonary hilum dissection, where one animal was killed due to hemodynamic instability and the other two animals required thoracotomy to complete the operation. There were five delayed bleeding and surgical mortality cases caused by inadequate vessel sealing by electrosurgical devices. Postmortem examination confirmed correct transumbilical lobectomy in the twelve animals that survived the operations. Transumbilical anatomic lobectomy is technically feasible in a canine model; however, the electrosurgical devices were not effective in sealing the pulmonary vessel in the current canine model.

  2. Decreased creatine kinase is linked to diastolic dysfunction in rats with right heart failure induced by pulmonary artery hypertension

    NARCIS (Netherlands)

    Fowler, Ewan D.; Benoist, David; Drinkhill, Mark J.; Stones, Rachel; Helmes, Michiel; Wüst, Rob C. I.; Stienen, Ger J. M.; Steele, Derek S.; White, Ed

    2015-01-01

    Our objective was to investigate the role of creatine kinase in the contractile dysfunction of right ventricular failure caused by pulmonary artery hypertension. Pulmonary artery hypertension and right ventricular failure were induced in rats by monocrotaline and compared to saline-injected control

  3. A Behcet’s Disease Patient with Right Ventricular Thrombus, Pulmonary Artery Aneurysms, and Deep Vein Thrombosis Complicating Recurrent Pulmonary Thromboembolism

    Directory of Open Access Journals (Sweden)

    Selvi Aşker

    2013-01-01

    Full Text Available Intracardiac thrombus, pulmonary artery aneurysms, deep vein thrombosis, and pulmonary thromboembolism are rarely seen symptoms of Behcet’s disease. A 20-year-old female patient was admitted for complaints of cough, fever, palpitations, and chest pain. On the dynamic thorax computed tomograms (CT obtained because of significantly enlarged hilar structures seen on chest radiograms, aneurysmal dilatation of the pulmonary artery segments bilaterally, chronic thrombus with collapse, and consolidation substances compatible with pulmonary embolism involving both lower lobes have been observed. It is learned that, four years ago, the patient had been diagnosed with Behcet’s disease and received colchicine treatment but not regularly. The patient was hospitalized. On the transthoracic echocardiogram, a thrombosis with a dimension of 4.2 × 1.6 cm was recognized in the right ventricle. On abdomen CT, aneurysmal iliac veins and deep vein thrombus on Doppler ultrasonograms were diagnosed. At the controls after three months of immunosuppressive and anticoagulant therapies, some clinical and radiological improvements were recognized. The patient suspended the treatment for a month and the thrombus recurred. We present our case in order to show the effectiveness of immunosuppressive and anticoagulant therapies and rarely seen pulmonary thromboembolism in recurrent Behcet’s disease.

  4. Treatment of a Left Internal Mammary Artery to Pulmonary Artery Fistula with Polytetrafluoroethylene Covered Stents: A Case Report and Review of the Literature

    International Nuclear Information System (INIS)

    Abbott, J. Dawn; Brennan, Joseph J.; Remetz, Michael S.

    2004-01-01

    Internal mammary artery (IMA) to pulmonary artery (PA) fistula is a rare complication of coronary artery bypass grafting (CABG) that may present as myocardial ischemia. We describe a case of left IMA-to-PA fistula treated with balloon expandable coronary polytetrafluoroethylene (PTFE) graft stents and review previously reported cases of this entity

  5. Cellular, pharmacological, and biophysical evaluation of explanted lungs from a patient with sickle cell disease and severe pulmonary arterial hypertension.

    Science.gov (United States)

    Rogers, Natasha M; Yao, Mingyi; Sembrat, John; George, M Patricia; Knupp, Heather; Ross, Mark; Sharifi-Sanjani, Maryam; Milosevic, Jadranka; St Croix, Claudette; Rajkumar, Revathi; Frid, Maria G; Hunter, Kendall S; Mazzaro, Luciano; Novelli, Enrico M; Stenmark, Kurt R; Gladwin, Mark T; Ahmad, Ferhaan; Champion, Hunter C; Isenberg, Jeffrey S

    2013-12-01

    Pulmonary hypertension is recognized as a leading cause of morbidity and mortality in patients with sickle cell disease (SCD). We now report benchtop phenotyping from the explanted lungs of the first successful lung transplant in SCD. Pulmonary artery smooth muscle cells (PASMCs) cultured from the explanted lungs were analyzed for proliferate capacity, superoxide (O2 (•-)) production, and changes in key pulmonary arterial hypertension (PAH)-associated molecules and compared with non-PAH PASMCs. Upregulation of several pathologic processes persisted in culture in SCD lung PASMCs in spite of cell passage. SCD lung PASMCs showed growth factor- and serum-independent proliferation, upregulation of matrix genes, and increased O2 (•-) production compared with control cells. Histologic analysis of SCD-associated PAH arteries demonstrated increased and ectopically located extracellular matrix deposition and degradation of elastin fibers. Biomechanical analysis of these vessels confirmed increased arterial stiffening and loss of elasticity. Functional analysis of distal fifth-order pulmonary arteries from these lungs demonstrated increased vasoconstriction to an α1-adrenergic receptor agonist and concurrent loss of both endothelial-dependent and endothelial-independent vasodilation compared with normal pulmonary arteries. This is the first study to evaluate the molecular, cellular, functional, and mechanical changes in end-stage SCD-associated PAH.

  6. Pulmonary Artery Pseudoaneurysm: A Rare Cause of Fatal Massive Hemoptysis

    Directory of Open Access Journals (Sweden)

    Himaja Koneru

    2018-01-01

    Full Text Available Pulmonary artery pseudoaneurysm (PAPA, an uncommon complication of pyogenic bacterial and fungal infections and related septic emboli, is associated with high mortality. The pulmonary artery (PA lacks an adventitial wall; therefore, repeated endovascular seeding of the PA with septic emboli creates saccular dilations that are more likely to rupture than systemic arterial aneurysms. The most common clinical presentation of PAPA is massive hemoptysis and resultant worsening hypoxemia. Computed tomography angiography is the preferred diagnostic modality for PAPA; typical imaging patterns include focal outpouchings of contrast adjacent to a branch of the PA following the same contrast density as the PA in all phases of the study. In mycotic PAPAs, multiple synchronous lesions are often seen in segmental and subsegmental PAs due to ongoing embolic phenomena. The recommended approach for a mycotic PAPA is prolonged antimicrobial therapy; for massive hemoptysis, endovascular treatment (e.g., coil embolization, stenting, or embolization of the feeding vessel is preferred. PAPA resection and lobectomy are a last resort, generally reserved for patients with uncontrolled hemoptysis or pleural hemorrhage. We present a case of a 28-year-old woman with necrotizing pneumonia from intravenous drug use who ultimately died from massive hemoptysis and shock after a ruptured PAPA.

  7. Pulmonary Arterial Hypertension in Adults: Novel Drugs and Catheter Ablation Techniques Show Promise? Systematic Review on Pharmacotherapy and Interventional Strategies

    Directory of Open Access Journals (Sweden)

    Salvatore Rosanio

    2014-01-01

    Full Text Available This systematic review aims to provide an update on pharmacological and interventional strategies for the treatment of pulmonary arterial hypertension in adults. Currently US Food and Drug Administration approved drugs including prostanoids, endothelin-receptor antagonists, phosphodiesterase type-5 inhibitors, and soluble guanylate-cyclase stimulators. These agents have transformed the prognosis for pulmonary arterial hypertension patients from symptomatic improvements in exercise tolerance ten years ago to delayed disease progression today. On the other hand, percutaneous balloon atrioseptostomy by using radiofrequency perforation, cutting balloon dilatation, or insertion of butterfly stents and pulmonary artery catheter-based denervation, both associated with very low rate of major complications and death, should be considered in combination with specific drugs at an earlier stage rather than late in the progression of pulmonary arterial hypertension and before the occurrence of overt right-sided heart failure.

  8. Pulmonary arterial hypertension as a manifestation of lupus erythematosus

    International Nuclear Information System (INIS)

    Stark, P.; Jaramillo, D.

    1987-01-01

    We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor. (orig.) [de

  9. Pulmonary artery enlargement and cystic fibrosis pulmonary exacerbations: a cohort study

    Science.gov (United States)

    Wells, J. Michael; Farris, Roopan F.; Gosdin, Taylor A.; Dransfield, Mark T.; Wood, Michelle E.; Bell, Scott C.; Rowe, Steven M.

    2017-01-01

    Background Acute pulmonary exacerbations are associated with progressive lung function decline and increased mortality in cystic fibrosis (CF). The role of pulmonary vascular disease in pulmonary exacerbations is unknown. We investigated the association between pulmonary artery enlargement (PA:A>1), a marker of pulmonary vascular disease, and exacerbations. Methods We analyzed clinical, computed tomography (CT), and prospective exacerbation data in a derivation cohort of 74 adult CF patients, measuring the PA:A at the level of the PA bifurcation. We then replicated our findings in a validation cohort of 190 adult CF patients. Patients were separated into groups based on the presence or absence of a PA:A>1 and were followed for 1-year in the derivation cohort and 2-years in the validation cohort. The primary endpoint was developing ≥1 acute pulmonary exacerbation during follow-up. Linear and logistic regression models were used to determine associations between clinical factors, the PA:A ratio, and pulmonary exacerbations. We used Cox regression to determine time to first exacerbation in the validation cohort. Findings We found that PA:A>1 was present in n=37/74 (50%) of the derivation and n=89/190 (47%) of the validation cohort. In the derivation cohort, n=50/74 (68%) had ≥1 exacerbation at 1 year and n=133/190 (70%) in the validation cohort had ≥1 exacerbation after 2 years. PA:A>1 was associated with younger age in both cohorts and with elevated sweat chloride (100.5±10.9 versus 90.4±19.9mmol/L, difference between groups 10.1mmol/L [95%CI 2.5–17.7], P=0.017) in the derivation group. PA:A>1 was associated with exacerbations in the derivation (OR 3.49, 95%CI 1.18–10.3, P=0.023) and validation (OR 2.41, 95%CI 1.06–5.52, P=0.037) cohorts when adjusted for confounders. Time to first exacerbation was shorter in PA:A>1 versus PA:Apulmonary exacerbation risk in two well-characterized cohorts. PA:A may be a predictive marker in CF. PMID:27298019

  10. Paradoxical emboli: demonstration using helical computed tomography of the pulmonary artery associated with abdominal computed tomography

    International Nuclear Information System (INIS)

    Delalu, P.; Ferretti, G.R.; Bricault, I.; Ayanian, D.; Coulomb, M.

    2000-01-01

    We report the case of a 60-year-old woman with a recent history of a cerebrovascular accident. Because of clinical suspicion of pulmonary embolism and negative Doppler ultrasound findings of the lower limbs, spiral computed tomography of the pulmonary artery was performed and demonstrated pulmonary emboli. We emphasize the role of computed tomography of the abdomen, performed 3 min after the thoracic acquisition, which showed an unsuspected thrombus within the abdominal aorta and the left renal artery with infarction of the left kidney. Paradoxical embolism was highly suspected on computed tomography data and confirmed by echocardiography which demonstrated a patent foramen ovale. (orig.)

  11. Effects of exercise training in patients with idiopathic pulmonary arterial hypertension

    NARCIS (Netherlands)

    de Man, F.S.; Handoko, M.L.; Groepenhoff, H.; van 't Hul, A.J.; Abbink, J.; Koppers, R.J.H.; Grotjohan, H.P.; Twisk, J.W.R.; Bogaard, H.J.; Boonstra, A.; Postmus, P.E.; Westerhof, N.; van der Laarse, W.J.; Vonk Noordegraaf, A.

    2009-01-01

    We determined the physiological effects of exercise training on exercise capacity and quadriceps muscle function in patients with idiopathic pulmonary arterial hypertension (iPAH). In total, 19 clinically stable iPAH patients (New York Heart Association II-III) underwent a supervised exercise

  12. Pulmonary artery perfusion versus no perfusion during cardiopulmonary bypass for open heart surgery in adults

    DEFF Research Database (Denmark)

    Buggeskov, Katrine B; Grønlykke, Lars; Risom, Emilie C

    2018-01-01

    BACKGROUND: Available evidence has been inconclusive on whether pulmonary artery perfusion during cardiopulmonary bypass (CPB) is associated with decreased or increased mortality, pulmonary events, and serious adverse events (SAEs) after open heart surgery. To our knowledge, no previous systematic...... handsearched retrieved study reports and scanned citations of included studies and relevant reviews to ensure that no relevant trials were missed. We searched for ongoing trials and unpublished trials in the World Health Organization International Clinical Trials Registry Platform (ICTRP) and at clinicaltrials......). We used GRADE principles to assess the quality of evidence. MAIN RESULTS: We included in this review four RCTs (210 participants) reporting relevant outcomes. Investigators randomly assigned participants to pulmonary artery perfusion with blood versus no perfusion during CPB. Only one trial included...

  13. Pulmonary Artery Perforation as a Complication of Pleural Drainage - Case Report.

    Science.gov (United States)

    Gołota, Janusz Jacek; Kiliańczyk, Michał; Szmyt, Piotr

    2016-10-01

    The case presented is a drainage of empyema of the left pleural cavity complicated with the penetration of the lung parenchyma and perforation of the left pulmonary artery with drain displacement to the right pulmonary artery. The cause of complications has been shown along with the possible ways of conduct. Drainage of the pleural cavity is a primary surgery in the chest. The presence of pleural air or fluid is an indication for its implementation. This procedure usually has no complications. Time pressure, anatomical anomalies, changes of the topography in the course of co-morbidities are the factors allowing for the creation of many complications. The aim of the work is to remind about the technical performance of this simple surgical procedure and to note that its complications may directly threaten the life of the patient. This is the first case reported in Polish literature.

  14. Transformation of a Ruptured Giant Pulmonary Artery Aneurysm into an Air Cavity After Transcatheter Embolization in a Behcet's Patient

    International Nuclear Information System (INIS)

    Cil, Barbaros E.; Turkbey, Baris; Canyigit, Murat; Kumbasar, Ozlem O.; Celik, Gokhan; Demirkazik, Figen B.

    2006-01-01

    Pulmonary artery aneurysms due to Behcet's disease are mainly seen in young males and very rarely in females. To our knowledge there are only 10 cases reported in the related literature. Emergent transcatheter embolization was performed in a female patient with a known history of Behcet's disease in whom massive hemoptysis developed because of rupture of a giant pulmonary artery aneurysm. At 6-month follow-up, transformation of the aneurysm sac into an air cavity was detected. To our knowledge, such a transformation has never been reported in the literature before. Embolization of the pulmonary artery aneurysm and the mechanism of cavity transformation are reviewed and discussed

  15. SECONDARY PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC DISEASES OF CONNECTIVE TISSUE

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2016-01-01

    Full Text Available Modern definition of pulmonary arterial hypertension (PAH as well as data on prevalence and incidence of secondary PAH in systemic disease of connective tissue is presented,  including data of USA, France and Scotland registers. The main chains of pathogenesis, classification approaches, clinical features and diagnostics are described. 

  16. SECONDARY PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC DISEASES OF CONNECTIVE TISSUE

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2009-01-01

    Full Text Available Modern definition of pulmonary arterial hypertension (PAH as well as data on prevalence and incidence of secondary PAH in systemic disease of connective tissue is presented,  including data of USA, France and Scotland registers. The main chains of pathogenesis, classification approaches, clinical features and diagnostics are described. 

  17. Relationship between signal intensity of blood flow in the pulmonary artery obtained by magnetic resonance imaging and results of right cardiac catheterization in patients with pulmonary disease

    Energy Technology Data Exchange (ETDEWEB)

    Yuguchi, Yasutoshi; Nagao, Keiichi; Kouno, Norihiro; Tanabe, Nobuhiro; Okita, Shinya; Tojima, Hirokazu; Okada, Osamu; Kuriyama, Takayuki [Chiba Univ. (Japan). School of Medicine; Yamaguchi, Tetsuo

    1992-08-01

    Electrocardiogram-gated spin-echo magnetic resonance (MR) images of the chest were obtained in five normal controls and 35 patients with pulmonary disease (11 chronic obstructive pulmonary disease, 6 pulmonary thromboembolism, 5 primary pulmonary hypertension, 4 interstitial pulmonary disease, 4 pulmonary hypertension with disturbance of portal circulation, and 5 other diseases) who underwent right cardiac catheterization. In transverse images at the level of the right main pulmonary artery (rPA) and sagittal images at the level through the midsternal line and the spinal cord, the signal intensity of blood flow in the rPA was quantitatively evaluated, and the correlations with the MR signal intensity of intravascular flow and the parameters of hemodynamics were studied. In diastole MR images of both normal controls and patients mostly showed a significant signal and visible flow images. In systolic MR images, the mean values of hemodynamic parameters (mean pulmonary arterial pressure (mPAP), pulmonary arteriolar resistance (PAR), and cardiac index (CI)) were abnormal in patients with significant signal intensity of flow compared with those in patients without sufficient MR signal. The signal intensity was not correlated with mPAP; however, it significantly increased as PAR increased, and it increased as CI decreased both in diastole and in systole. Especially in systole, there was good correlation between the signal intensity in transverse MR images and CI and between signal intensity in sagittal MR images and PAR . These results suggest that the signal intensity of blood flow in the rPA on MR images can be used as an index of the severity of right heart failure associated with pulmonary disease. MR imaging is a useful modality to evaluate pulmonary circulation disturbance because of its ability to assess blood flow in the pulmonary artery noninvasively without interference from other structures such as bone and normal lung. (J.P.N.).

  18. Two-years therapy with bosentan of pulmonary arterial hypertension related to connective tissue diseases

    Directory of Open Access Journals (Sweden)

    M. Rizzo

    2011-09-01

    Full Text Available Objective: Pulmonary arterial hypertension (PAH is a rare but severe complication of connective tissue diseases (CTD, with a negative impact on patients survival. Bosentan, a receptor antagonist of endothelin, has been proved effective for the treatment of PAH. The aim of this study was to evaluate the effects and the safety of bosentan administered for 2 years in a group of patients with PAH related to CTD. Methods: Twelve patients with PAH related to systemic sclerosis (8 cases, SLE (2 cases, mixed connective tissue disease (1 case and polymyositis (1 case attending the Rheumatology Unit of Padova University were treated with bosentan for two years. Distance walked in 6 minutes, right ventricular systolic pressure and mean pulmonary artery pressure estimated by doppler echocardiography were evaluated at baseline and after 6, 12, 18 and 24 months of treatment. Safety was assessed by laboratory tests performed every two months. Results: During bosentan treatment, a significant decrease of right ventricular systolic pressure was observed after 6, 12, 18 and 24 months in comparison to baseline, whereas pulmonary artery mean pressure remained unchanged. Distance walked in 6 minutes slightly increased after 6 and 12 months, but significantly decreased after 18 and 24 months, mostly because complications of CTD which compromised the ability to walk arose in 4 patients. Adverse events related to bosentan were observed in 2 cases. Conclusions: Bosentan has been demonstrated effective in reducing pulmonary arterial pressure in a two-year period of treatment. Exercise capacity improved only in the first year of therapy and worsened thereafter, suggesting the opportunity of a combination therapy for a long-term treatment of PAH related to CTD.

  19. Effects of calcitonin gene-related peptide on canine cerebral artery strips and the in-vivo vertebral blood flow in dogs.

    Science.gov (United States)

    Ikegaki, I; Suzuki, Y; Satoh, S; Asano, T; Shibuya, M; Sugita, K

    1989-10-01

    The effects of calcitonin gene-related peptide (CGRP) on canine cerebral arteries and on vertebral blood flow were investigated in-vivo and in-vitro and the findings compared with the effects of vasoactive intestinal peptide (VIP) and substance P. Administration of CGRP into the vertebral artery caused a dose-dependent and long-lasting increase in blood flow. The in-vivo vasodilatory effects of substance P and VIP were short-lasting. CGRP (0.1 to 100 nmol/l) elicited a concentration-dependent relaxation of the isolated middle cerebral and basilar arteries when the tissues were precontracted by exposure to prostaglandin F2 alpha (PGF2 alpha). This effect was not antagonized by propranolol, atropine, tetrodotoxin, (N-Ac-Tyr1, D-Phe2)-growth hormone-releasing factor(1-29)-NH2 or (D-Pro2, D-Trp7,9) substance P. CGRP also reduced concentration-dependently the contraction of cerebral arteries induced by KCl or 9,11-epithio-11,12-metano-thromboxane A2 (STXA2). Mechanical removal of the endothelium did not abolish the vasodilatory response to CGRP. In PGF2 alpha-contracted canine cerebral arteries, VIP (0.1 to 100 nmol/l) was less potent a vasodilator than CGRP. At low concentrations (0.01 to 1 nmol/l) substance P elicited a rapid and short-lasting relaxation, and in the absence of endothelium this relaxation disappeared. These findings are clear evidence that CGRP modulates vascular tone.

  20. Canine histiocytic sarcoma presenting as a target lesion on brain magnetic resonance imaging and as a solitary pulmonary mass.

    Science.gov (United States)

    Hicks, Jill; Barber, Renee; Childs, Bronwen; Kirejczyk, Shannon Gm; Uhl, Elizabeth W

    2017-04-17

    A 6-year-old spayed female miniature schnauzer presented with generalized seizures and progressive multifocal intracranial neurologic disease. Thoracic radiographs and computed tomography (CT) revealed a large solitary pulmonary mass within the right cranial lung lobe. On brain magnetic resonance imaging (MRI), a solitary intraparenchymal mass within the left piriform lobe had a "target" appearance on both pre- and postcontrast sequences. Cerebrospinal fluid was unremarkable and histopathology indicated both masses represented histiocytic sarcoma. This case represents an uncommonly reported MRI appearance of histiocytic sarcoma in the canine brain and a large, solitary-appearing pulmonary histiocytic sarcoma in the same dog. © 2017 American College of Veterinary Radiology.

  1. Pan-nitinol occluder and special delivery device for closure of patent ductus arteriosus: a canine-model feasibility study.

    Science.gov (United States)

    Jiang, Hai-bin; Bai, Yuan; Zong, Gang-jun; Han, Lin; Li, Wei-ping; Lu, Yang; Qin, Yong-wen; Zhao, Xian-xian

    2013-01-01

    The aim of this study was to evaluate a new type of occluder for patent ductus arteriosus. Patent ductus arteriosus was established in a canine model by anastomosing a length of autologous jugular vein to the descending aorta and the left pulmonary artery in an end-to-side fashion. Transcatheter closure of each patent ductus arteriosus was performed on 10 dogs, which were then monitored for as long as 6 months with aortography, echocardiography, and histologic evaluation. Transcatheter closure with use of the novel pan-nitinol device was successful in all canine models. Postoperative echocardiography showed that the location and shape of the occluders were normal, without any residual shunting. Further histologic evaluation confirmed that the occluder surface was completely endothelialized 3 months after implantation. Transcatheter patent ductus arteriosus closure with the pan-nitinol occluder can be performed safely and successfully in a canine model and shows good biological compatibility and low mortality rates.

  2. CT pulmonary angiography of adult pulmonary vascular diseases: Technical considerations and interpretive pitfalls

    International Nuclear Information System (INIS)

    Taslakian, Bedros; Latson, Larry A.; Truong, Mylene T.; Aaltonen, Eric; Shiau, Maria C.; Girvin, Francis; Alpert, Jeffrey B.; Wickstrom, Maj; Ko, Jane P.

    2016-01-01

    Highlights: • CTPA plays a key role in the evaluation of pulmonary vascular diseases. • Improvements in CT technology have improved visualization of pulmonary arteries. • Knowledge of the technical pitfalls is essential for accurate diagnosis. • Dual energy CT imaging enables parenchymal iodine evaluation. • An awareness of the entities affecting the pulmonary arteries is important. - Abstract: Computed tomography pulmonary angiography (CTPA) has become the primary imaging modality for evaluating the pulmonary arteries. Although pulmonary embolism is the primary indication for CTPA, various pulmonary vascular abnormalities can be detected in adults. Knowledge of these disease entities and understanding technical pitfalls that can occur when performing CTPA are essential to enable accurate diagnosis and allow timely management. This review will cover a spectrum of acquired abnormalities including pulmonary embolism due to thrombus and foreign bodies, primary and metastatic tumor involving the pulmonary arteries, pulmonary hypertension, as well as pulmonary artery aneurysms and stenoses. Additionally, methods to overcome technical pitfalls and interventional treatment options will be addressed.

  3. CT pulmonary angiography of adult pulmonary vascular diseases: Technical considerations and interpretive pitfalls

    Energy Technology Data Exchange (ETDEWEB)

    Taslakian, Bedros, E-mail: bedros.taslakian@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States); Latson, Larry A., E-mail: larry.latson@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States); Truong, Mylene T., E-mail: mtruong@mdanderson.org [Department of Radiology, University of Texas, MD Anderson Cancer Center, TX (United States); Aaltonen, Eric, E-mail: Eric.Aaltonen@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States); Shiau, Maria C., E-mail: Maria.Shiau@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States); Girvin, Francis, E-mail: Francis.Girvin@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States); Alpert, Jeffrey B., E-mail: Jeffrey.Alpert@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States); Wickstrom, Maj, E-mail: Maj.Wickstrom@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States); Ko, Jane P., E-mail: Jane.Ko@nyumc.org [Department of Radiology, NYU Langone Medical Center, NY (United States)

    2016-11-15

    Highlights: • CTPA plays a key role in the evaluation of pulmonary vascular diseases. • Improvements in CT technology have improved visualization of pulmonary arteries. • Knowledge of the technical pitfalls is essential for accurate diagnosis. • Dual energy CT imaging enables parenchymal iodine evaluation. • An awareness of the entities affecting the pulmonary arteries is important. - Abstract: Computed tomography pulmonary angiography (CTPA) has become the primary imaging modality for evaluating the pulmonary arteries. Although pulmonary embolism is the primary indication for CTPA, various pulmonary vascular abnormalities can be detected in adults. Knowledge of these disease entities and understanding technical pitfalls that can occur when performing CTPA are essential to enable accurate diagnosis and allow timely management. This review will cover a spectrum of acquired abnormalities including pulmonary embolism due to thrombus and foreign bodies, primary and metastatic tumor involving the pulmonary arteries, pulmonary hypertension, as well as pulmonary artery aneurysms and stenoses. Additionally, methods to overcome technical pitfalls and interventional treatment options will be addressed.

  4. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt : Does the beneficial effect persist?

    NARCIS (Netherlands)

    van Loon, Rosa Laura E.; Hoendermis, Elke S.; Duffels, Marielle G. J.; Vonk-Noordegraaf, Anton; Mulder, Barbara J. M.; Hillege, Hans L.; Berger, Rolf M. F.

    2007-01-01

    BACKGROUND: Data on long-term response to bosentan in adults and especially children with pulmonary arterial hypertension (PAH) associated with systemic-to-pulmonary shunt are scarce. METHODS: We studied bosentan efficacy in 30 patients (20 adults, 10 children) with the disease at short- (4 months),

  5. Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: Does the beneficial effect persist?

    NARCIS (Netherlands)

    van Loon, Rosa Laura E.; Hoendermis, Elke S.; Duffels, Marielle G. J.; Vonk-Noordegraaf, Anton; Mulder, Barbara J. M.; Hillege, Hans L.; Berger, Rolf M. F.

    2007-01-01

    Background Data on long-term response to bosentan in adults and especially children with pulmonary arterial hypertension (PAH) associated with systemic-to-pulmonary shunt are scarce. Methods We studied bosentan efficacy in 30 patients (20 adults, 10 children) with the disease at short- (4 months),

  6. Migration to the pulmonary artery of nine metallic coils placed in the internal iliac vein for treatment of giant rectal varices

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    Wataru Yamasaki

    2012-07-01

    Full Text Available Transcatheter venous embolization with metallic coils is a safe and reliable method for the treatment of pelvic congestion syndrome and pelvic varicocele. While rare, coil migration to the pulmonary arteries is potentially fatal. We report the migration to the pulmonary artery of a cluster of nine metallic microcoils placed in the internal iliac vein to obliterate giant rectal varices. Our patient suffered no severe sequelae. To avoid coil migration to the pulmonary arteries, the coils chosen for placement must take into consideration the characteristics of the target vessels, particularly of larger veins.

  7. A different kind of Christmas tree: anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA).

    Science.gov (United States)

    Afolabi-Brown, Olayinka; Witzke, Christian; Moldovan, Raul; Pressman, Gregg

    2014-02-01

    Anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital coronary anomaly that has an incidence of 0.002%. We report a case of a previously healthy female who presented to our hospital with pneumonia and was incidentally discovered to have ARCAPA. This was initially diagnosed on echocardiography by the unusual echocardiographic finding of multiple color flow Doppler signals around the right ventricular free wall and apex which were subsequently confirmed by angiography to be due to extensive collateral circulation between the left and right coronary arteries. This represents an unusual echocardiographic manifestation of this very rare condition. © 2013, Wiley Periodicals, Inc.

  8. Angiojet thrombectomy for Blalock-Taussig shunt and pulmonary artery thrombus in an infant with tetralogy of fallot

    Directory of Open Access Journals (Sweden)

    Brody Wehman

    2014-01-01

    Full Text Available We describe a new technique for treatment of shunt thrombosis in infants with complex anatomical defects. A 2-month-old girl with Tetralogy of Fallot underwent placement of a modified Blalock-Taussig shunt (MBTS at day of life (DOL 6 with revision at DOL 20. Following this surgery, the patient became hypotensive and hypoxic with new evidence of lack of flow through the MBTS on echocardiography. Angiography showed an occluded MBTS and right pulmonary artery with patent distal branches with normal pulmonary venous return. Balloon angioplasty was attempted but failed to fully recanalize the right pulmonary artery (RPA and MBTS. An AngioJet catheter was then passed through the shunt and RPA to perform rheolyticthrombectomy. Subsequent angiogram showed a widely patent RPA and MBTS. An echocardiogram at 1-month post-thrombectomy showed a widely patent MBTS with continuous flow seen entering both branch pulmonary arteries. The AngioJet system for thrombectomy provides a viable option for complex thrombus removal in patients refractory to other methods.

  9. Mediastinal lymphadenopathy and pulmonary arterial hypertension in mixed connective tissue disease

    International Nuclear Information System (INIS)

    Guit, G.L.; Shaw, P.C.; Ehrlich, J.; Kroon, H.M.; Oudkerk, M.

    1985-01-01

    A case of mixed connective tissue disease (MCTD) is presented in which mediastinal lymphadenopathy was the most prominent radiological finding detected by plain chest radiographs and computed tomography. Pulmonary arterial hypertension, which is a rare and often fatal complication of MCTD, also developed in this patient

  10. Energy efficiency and pulmonary artery flow after balloon pulmonary angioplasty for inoperable, chronic thromboembolic pulmonary hypertension: Analysis by phase-contrast MRI

    Energy Technology Data Exchange (ETDEWEB)

    Nagao, Michinobu, E-mail: nagao.michinobu@twmu.ac.jp [Department of Diagnostic Imaging & Nuclear Medicine, Tokyo Women’s Medical University, Tokyo (Japan); Yamasaki, Yuzo [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Fukuoka (Japan); Abe, Kohtaro; Hosokawa, Kazuya [Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka (Japan); Kawanami, Satoshi [Department of Molecular Imaging & Diagnosis, Graduate School of Medical Sciences, Kyushu University, Fukuoka (Japan); Kamitani, Takeshi; Yamanouchi, Torahiko [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Fukuoka (Japan); Yabuuchi, Hidetake [Department of Medical Sciences, Graduate School of Medical Sciences, Kyushu University, Fukuoka (Japan); Fukushima, Kenji [Department of Diagnostic Imaging & Nuclear Medicine, Tokyo Women’s Medical University, Tokyo (Japan); Honda, Hiroshi [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Fukuoka (Japan)

    2017-02-15

    Purpose: The aims of this study were to propose a new quantitative method for pulmonary artery (PA) flow energetics using phase-contrast magnetic resonance imaging (PC-MRI), and to investigate how balloon pulmonary angioplasty (BPA) impacts energetics in chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods: PC-MRI at 3-Teslar and with a flow sensitive gradient echo was used to examine energetics prior to and following BPA for 24 CTEPH patients. Stroke volume (m; ml) and mean velocity (V; mm/s) for the main pulmonary artery (PA), right PA, and left PA were calculated from a time-flow curve derived from PC-MRI. Based on the Bernoulli principle, PA energy was identified as 1/2 mV{sup 2} (μj/kg), and energy loss was defined as the following equation “energy loss = main PA energy − (rt. PA energy + lt. PA energy)”. Results: Right PA energy was significantly greater post-BPA than pre-BPA (61 ± 55 vs. 32 ± 40 μj/kg). There was no difference in main PA and left PA energies. Energy loss was significantly decreased post-BPA (18 ± 97 μj/kg) than pre-BPA (79 ± 125 μj/kg). An optimal cutoff of left PA energy of 45 μj/kg pre-BPA can be used to predict patients with mPAP ≥ 30 mmHg after BPA, with an area under the curve of 0.91, 78% sensitivity, and 92% specificity. Conclusion: Analysis of PA energetics using phase-contrast MRI demonstrates that BPA improves energy loss in CTEPH. In addition, BPA responses can be predicted by PA energy status pre-treatment.

  11. Right Heart End-Systolic Remodeling Index Strongly Predicts Outcomes in Pulmonary Arterial Hypertension: Comparison With Validated Models.

    Science.gov (United States)

    Amsallem, Myriam; Sweatt, Andrew J; Aymami, Marie C; Kuznetsova, Tatiana; Selej, Mona; Lu, HongQuan; Mercier, Olaf; Fadel, Elie; Schnittger, Ingela; McConnell, Michael V; Rabinovitch, Marlene; Zamanian, Roham T; Haddad, Francois

    2017-06-01

    Right ventricular (RV) end-systolic dimensions provide information on both size and function. We investigated whether an internally scaled index of end-systolic dimension is incremental to well-validated prognostic scores in pulmonary arterial hypertension. From 2005 to 2014, 228 patients with pulmonary arterial hypertension were prospectively enrolled. RV end-systolic remodeling index (RVESRI) was defined by lateral length divided by septal height. The incremental values of RV free wall longitudinal strain and RVESRI to risk scores were determined. Mean age was 49±14 years, 78% were female, 33% had connective tissue disease, 52% were in New York Heart Association class ≥III, and mean pulmonary vascular resistance was 11.2±6.4 WU. RVESRI and right atrial area were strongly connected to the other right heart metrics. Three zones of adaptation (adapted, maladapted, and severely maladapted) were identified based on the RVESRI to RV systolic pressure relationship. During a mean follow-up of 3.9±2.4 years, the primary end point of death, transplant, or admission for heart failure was reached in 88 patients. RVESRI was incremental to risk prediction scores in pulmonary arterial hypertension, including the Registry to Evaluate Early and Long-Term PAH Disease Management score, the Pulmonary Hypertension Connection equation, and the Mayo Clinic model. Using multivariable analysis, New York Heart Association class III/IV, RVESRI, and log NT-proBNP (N-Terminal Pro-B-Type Natriuretic Peptide) were retained (χ 2 , 62.2; P right heart metrics, RVESRI demonstrated the best test-retest characteristics. RVESRI is a simple reproducible prognostic marker in patients with pulmonary arterial hypertension. © 2017 American Heart Association, Inc.

  12. Advanced pulmonary arterial hypertension: mechanical support and lung transplantation

    Directory of Open Access Journals (Sweden)

    Sonja Bartolome

    2017-12-01

    Full Text Available The development of targeted therapies has transformed the outlook for patients with pulmonary arterial hypertension (PAH; however, some patients fail to achieve an adequate clinical response despite receiving maximal treatment. For these patients, lung transplantation remains an important therapeutic option, and recommendations for transplantation are included in the current European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension. Although lung transplantation is not without risk, overall long-term survival rates are good and substantial improvements in quality of life have been reported for lung transplant recipients. In this review, we describe the important considerations prior to, during and after transplantation, including the role of mechanical support, in patients with advanced PAH.

  13. Comparison of Radiofrequency Ablation with Saturated Saline Preinjection and Renal Artery Occlusion: In Vivo Study in Canine Kidneys Preinjection and Renal Artery Occlusion: In Vivo Study in Canine Kidneys

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Byung Seok; Ahn, Moon Sang [Chungnam National University Hospital, Daejeon (Korea, Republic of); Park, Mi Hyun [Dept. of Radiology, Dankook University Hospital, Cheonan (Korea, Republic of); Jeon, Gyeong Sik [Dept. of Radiology, CHA Bundang Medical Center, CHA University College of Medicine, Seongnam (Korea, Republic of); Lee, Byung Mo [Dept. of Surgery, Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of); Lee, Ki Chang [Dept. of Veterinary Radiology, Chonbuk National University College of VeterinaryMedicine, Seoul (Korea, Republic of); Kim, Ho Jun [Dept. of Radiology, Konyang University Hospital, Daejeon (Korea, Republic of); Ohm, Joon Young [Dept. of Radiology, Bucheon St. Mary Hospital, The Catholic University of Korea College of Medicine, Bucheon (Korea, Republic of)

    2012-04-15

    To compare the ablation zone after radiofrequency ablation (RFA) with saturated saline preinjection and renal artery occlusion in canine kidneys. RFA was induced in the kidneys of six mongrel dogs. A total of 24 ablation zones were induced using a 1-cm tip internally cooled needle electrode in three groups: RFA (Control group), RFA with 0.5 mL saturated saline preinjection (SS group), and RFA with renal artery occlusion by atraumatic vascular clamp (Occlusion group). Ablation zone diameters were measured along transverse and longitudinal sections of the needle axis, and volumes were calculated. Temperature, applied voltage, current, and impedance during RFA were recorded automatically. The RFA zone volume was the largest in the SS group (1.33 {+-} 0.34 cm{sup 3}), followed by the Occlusion group (1.07 {+-} 0.38 cm{sup 3}) and then the Control group (0.62 {+-} 0.09 cm{sup 3}). Volumes for the SS and Occlusion groups were significantly larger than those for the Control group (p = 0.001, p = 0.012). There was no significant difference in volumes between the SS and Occlusion groups (p = 0.178). Saturated saline preinjection is as effective as renal arterial occlusion for expanding the ablation zone. RFA with saturated saline preinjection could help to treat large renal tumors.

  14. Comparison of Radiofrequency Ablation with Saturated Saline Preinjection and Renal Artery Occlusion: In Vivo Study in Canine Kidneys Preinjection and Renal Artery Occlusion: In Vivo Study in Canine Kidneys

    International Nuclear Information System (INIS)

    Shin, Byung Seok; Ahn, Moon Sang; Park, Mi Hyun; Jeon, Gyeong Sik; Lee, Byung Mo; Lee, Ki Chang; Kim, Ho Jun; Ohm, Joon Young

    2012-01-01

    To compare the ablation zone after radiofrequency ablation (RFA) with saturated saline preinjection and renal artery occlusion in canine kidneys. RFA was induced in the kidneys of six mongrel dogs. A total of 24 ablation zones were induced using a 1-cm tip internally cooled needle electrode in three groups: RFA (Control group), RFA with 0.5 mL saturated saline preinjection (SS group), and RFA with renal artery occlusion by atraumatic vascular clamp (Occlusion group). Ablation zone diameters were measured along transverse and longitudinal sections of the needle axis, and volumes were calculated. Temperature, applied voltage, current, and impedance during RFA were recorded automatically. The RFA zone volume was the largest in the SS group (1.33 ± 0.34 cm 3 ), followed by the Occlusion group (1.07 ± 0.38 cm 3 ) and then the Control group (0.62 ± 0.09 cm 3 ). Volumes for the SS and Occlusion groups were significantly larger than those for the Control group (p = 0.001, p = 0.012). There was no significant difference in volumes between the SS and Occlusion groups (p = 0.178). Saturated saline preinjection is as effective as renal arterial occlusion for expanding the ablation zone. RFA with saturated saline preinjection could help to treat large renal tumors.

  15. Determinants of exercise-induced pulmonary arterial hypertension in systemic sclerosis.

    Science.gov (United States)

    Voilliot, Damien; Magne, Julien; Dulgheru, Raluca; Kou, Seisyou; Henri, Christine; Laaraibi, Saloua; Sprynger, Muriel; Andre, Béatrice; Pierard, Luc A; Lancellotti, Patrizio

    2014-05-15

    Exercise-induced pulmonary arterial hypertension (EIPH) in systemic sclerosis (SSc) has already been observed but its determinants remain unclear. The aim of this study was to determine the incidence and the determinants of EIPH in SSc. We prospectively enrolled 63 patients with SSc (age 54±3years, 76% female) followed in CHU Sart-Tilman in Liège. All patients underwent graded semi-supine exercise echocardiography. Systolic pulmonary arterial pressure (sPAP) was derived from the peak velocity of the tricuspid regurgitation jet and adding the estimation of right atrial pressure, both at rest and during exercise. Resting pulmonary arterial hypertension (PH) was defined as sPAP > 35 mmHg and EIPH as sPAP > 50 mmHg during exercise. The following formulas were used: mean PAP (mPAP) = 0.61 × sPAP + 2, left atrial pressure (LAP)=1.9+1.24 × left ventricular (LV) E/e' and pulmonary vascular resistance (PVR)=(mPAP-LAP)/LV cardiac output (CO) and slope of mPAP-LVCO relationship=changes in mPAP/changes in LVCO. Resting PH was present in 3 patients (7%) and 21 patients developed EIPH (47%). Patients with EIPH had higher resting LAP (10.3 ± 2.2 versus 8.8 ± 2.3 mmHg; p = 0.03), resting PVR (2.6 ± 0.8 vs. 1.4 ± 1.1 Woods units; p=0.004), exercise LAP (13.3 ± 2.3 vs. 9 ± 1.7 mmHg; p exercise PVR (3.6 ± 0.7 vs. 2.1 ± 0.9 Woods units; p = 0.02) and slope of mPAP-LVCO (5.8 ± 2.4 vs. 2.9 ± 2.1 mmHg/L/min; p age and gender, exercise LAP (β=3.1 ± 0.8; p=0.001) and exercise PVR (β=7.9 ± 1.7; p=0.0001) were independent determinants of exercise sPAP. EIPH is frequent in SSc patients and is mainly related to both increased exercise LV filling pressure and exercise PVR. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  16. Coronary to Bronchial Artery Fistula Causing Massive Hemoptysis in Patients with Longstanding Pulmonary Tuberculosis

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Ji Young; Jeon, Eui Yong; Lee, In Jae; Koh, Sung Hye [Hallym University College of Medicine, Anyang (Korea, Republic of)

    2012-01-15

    We report on three cases of longstanding pulmonary tuberculosis patients with coronary to bronchial artery fistula (CBF) who presented with recurrent massive hemoptysis. The first and second patients died because of decreased functional pulmonary volume plus massive hemoptysis and cannulation failure of CBF due to hypovolemic vasospasm, respectively. When recurrent hemoptysis occurs despite successful embolization treatment, CBF should be considered as a potential bleeding source. Moreover, a coronary angiography should be performed, especially in patients with longstanding cardiopulmonary disease such as pulmonary tuberculosis.

  17. Origem anômala de uma artéria pulmonar da aorta ascendente: resolução da hipertensão arterial pulmonar com a correção cirúrgica Anomalous origin of one pulmonary artery from the ascending aorta: surgical repair resolving pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Marco Aurélio Santos

    2004-12-01

    Full Text Available OBJETIVO: Enfatizar a possibilidade diagnóstica da origem anômala de uma artéria pulmonar da aorta ascendente, em lactentes com quadro de insuficiência cardíaca clinicamente intratável e sem defeito estrutural intracardíaco. MÉTODOS: Estudo retrospectivo em quatro lactentes com insuficiência cardíaca intratável, submetidos a estudo ecocardiográfico bidimensional com cortes subcostal, supraesternal e paraesternal, e a estudo hemodinâmico e angiocardiográfico na projeção ântero-posterior. RESULTADOS: Três dos quatro lactentes tinham a artéria pulmonar direita originando-se da aorta ascendente, como diagnóstico principal. No quarto paciente a artéria pulmonar esquerda originava-se da aorta ascendente associada à ampla comunicação interventricular. Os lactentes tinham pressão em ambas artérias pulmonares em nível sistêmico. Todos foram submetidos a tratamento cirúrgico que consistiu na translocação da artéria pulmonar anômala da aorta. Não houve óbitos cardíacos imediatos ou tardios. CONCLUSÃO: Uma vez estabelecido o diagnóstico de origem anômala de artéria pulmonar da aorta ascendente, na forma isolada, a correção cirúrgica deverá ser prontamente realizada, não só pelo desenvolvimento de doença vascular pulmonar, como pelos excelentes resultados cirúrgicos atualmente obtidos.OBJECTIVE: To emphasize the diagnostic possibility of the anomalous origin of one pulmonary artery from the ascending aorta in infants with clinically refractory heart failure and no intracardiac structural defect. METHODS: Retrospective study of 4 infants with refractory heart failure undergoing 2-dimensional echocardiographic study with subcostal, suprasternal, and parasternal views, and hemodynamic and angiocardiographic study in the anteroposterior projection. RESULTS: Three of the 4 infants had their right pulmonary artery originating from the ascending aorta as their major diagnosis. In the fourth patient, the left pulmonary

  18. The Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA: a Case Series and Brief Review

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    Aliasghar Moeinipour

    2016-02-01

    Full Text Available Background Anomalous left coronary artery from the pulmonary artery (ALCAPA is a rare congenital cardiovascular defect that occurs in approximately 1/300 000 live births or 0.5% of children with congenital heart disease. There are two types of ALCAPA syndrome: the infant type and the adult type. The most infants experience myocardial infarction and congestive heart failure, and approximately 90% die within the first year of life; also, without early surgical intervention they have a dismal prognosis. Materials and Methods We report 3- year experiences from January 2013 to January 2016 of Imam Reza Hospital center (a tertiary referral hospital North East of Iran that consist of all patients with ALCAPA syndrome. Results The Takeuchi procedure, were successfully performed in five children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA. There was no death and significant mitral regurgitation postoperative (n=0 in this short study. All of patients (n=5 had evidence of improving ischemic myocardium status by increasing of ejection fraction and regional wall motion of left ventricular in follow up echocardiography. Conclusion The only cure treatment for ALCAPA syndrome is surgical intervention that needs to be performed immediately after diagnosis to prevent myocardial infarction and chronic heart failure. Today, establishing a system with two coronary arteries is the goal in definitive surgical repair. The Takeuchi procedure is a prefer method to establish a two-coronary repair for ALCAPA.

  19. Multi-detector CT coronary angiographic findings of coronary-to-pulmonary artery fistula

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Jae Seok; Park, Eun Ah; Lim, Ji Yeon; Lee, Whal [Dept. of Radiology, Seoul National University Hospital, Seoul (Korea, Republic of); Park, Jae Hyung [Dept. of Radiology, Myongji Hospital, Seonam University College of Medicine, Goyang (Korea, Republic of)

    2017-01-15

    To evaluate multi-detector CT (MDCT) coronary angiographic findings of coronary-to-pulmonary artery fistula (CPAF). We retrospectively reviewed images of patients with CPAF from the coronary CT angiography (CCTA) database obtained with a 64-channel MDCT between January 2008 and March 2011. We analyzed the CCTA findings for feeding arteries, fistula, association with peripulmonary arterial aneurysms, and the presence of communication between the CPAF and bronchial arteries. Fifty-five of the 15042 (0.37%) patients were diagnosed with CPAFs. The feeding artery was single (n = 18) or multiple (n = 37). The fistula had a single drainage site (n = 54) or multiple drainage sites (n = 1). The mean diameter of the fistulous opening was 2.7 ± 1.4 mm. A peripulmonary arterial aneurysm was present in 24 (44%) patients. Communication between CPAF and bronchial arteries was present in eight (14.5%) patients. MDCT coronary angiography can provide comprehensive morphologic details on CPAF and may help in presurgical or preinterventional planning.

  20. Successful Retrieval of a Dismembered Central Venous Catheter Stuck to the Right Pulmonary Artery Using a Stepwise Approach

    Directory of Open Access Journals (Sweden)

    Keisuke Nakabayashi

    2016-01-01

    Full Text Available Recent advances in anticancer chemotherapy have resulted in an increase in the number of patients requiring a central venous port catheter, and the incidence of catheter pinch-off syndrome has been increasing. Catheter pinch-off syndrome is a rare and unusual complication. It is difficult to retrieve dislodged catheters from the pulmonary artery, especially if the catheter is stuck to the peripheral pulmonary artery. We herein describe the successful removal of a catheter stuck in the pulmonary artery with a stepwise approach. First, a pigtail catheter was used to tug the dislodged catheter in order to free the unilateral end. Then, a gooseneck snare was used to catch and pull the catheter out of the patient. The key to success is to free the end of the catheter.

  1. Pulmonary arteriography by digital subtraction angiographic method in cyanotic heart disease with pulmonary stenosis or pulmonary atresia

    International Nuclear Information System (INIS)

    Kobayashi, Junjiro; Hirose, Hajime; Nakano, Susumu

    1985-01-01

    Pulmonary arteriography was performed by digital subtraction angiographic (DSA) method in 10 patients with cyanotic heart disease associated with pulmonary stenosis or pulmonary atresia. Ten patients consisted of five patients with tetralogy of Fallot, three with single ventricle and pulmonary stenosis, and two with pseudotruncus arteriosus. Hepato-clavicular position was taken in four patients. Pulmonary artery and its main branches were opacified and recognized clearly, and their diameter could be measured accurately with a small amount of contrast medium. There was a good correlation between the diameter of pulmonary artery measured by DSA and that measured by conventional pulmonary arteriography. DSA is a useful method for evaluating the size and the stenosis of pulmonary artery especially in small cyanotic infants. (author)

  2. Pulmonary artery intravascular abscess: A rare complication of incomplete infective endocarditis treatment in the setting of injection drug use

    Directory of Open Access Journals (Sweden)

    Simran Gupta

    Full Text Available Infective endocarditis (IE is a serious complication of injection drug use. Right-sided IE encompasses 5–10% of all IE cases, with the majority involving the tricuspid valve (TV. The predominant causal organism is Staphylococcus aureus. Most cases of right-sided IE can be successfully treated with antimicrobials, but approximately 5–16% require eventual surgical intervention. We report the case of a 36-year-old female with active injection drug use who developed methicillin-sensitive Staphylococcus aureus IE of the tricuspid valve. Associated with poor adherence to medical therapy as a consequence of opioid addiction, she developed septic emboli to the lungs and an intravascular abscess in the left main pulmonary artery. These long-term potentially fatal, sequelae of incompletely treated IE require surgical intervention, as medical therapy is unlikely to be sufficient. Surgical management may involve TV replacement, pulmonary artery resection, and pneumonectomy. Prevention of these complications may have been achieved by concurrent opioid addiction therapy. An intravascular pulmonary artery abscess is a novel complication of advanced IE that has not been previously reported. This complication likely arose due to incomplete IE treatment as a consequence of opioid addiction, highlighting the need for concurrent addiction management. Intravenous antimicrobial therapy is likely not adequate, and surgical intervention, including pulmonary artery resection and pneumonectomy may be necessary. Keywords: Pulmonary artery abscess, Infective endocarditis, Injection drug use, Opioid use disorder

  3. Pulmonary arterial hypertension associated with congenital heart disease

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    Michele D'Alto

    2012-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a common complication of congenital heart disease (CHD, with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.

  4. Screening for pulmonary arterial hypertension in systemic sclerosis

    Directory of Open Access Journals (Sweden)

    J-L. Vachiéry

    2009-09-01

    Full Text Available The onset and progression of pulmonary arterial hypertension (PAH in patients with systemic sclerosis (SSc can be particularly aggressive; however, effective treatments are available. Therefore, early identification of patients with suspected PAH, confirmation of diagnosis, and intervention is essential. PAH may be challenging to diagnose in its earliest stages, particularly in populations that have multiple causes of breathlessness, and, therefore, screening is required. The optimal screening tools and methodology are, as yet, unknown, and this is confounded by a lack of consensus over which patients to screen. Current practice favours annual screening of all SSc patients using Doppler echocardiography to detect elevated right heart pressures. This will typically identify most patients with the various forms of pulmonary hypertension found in SSc. The optimum thresholds for Doppler echocardiography are still subject to investigation, especially for patients with mild pulmonary hypertension, and this technique may, therefore, yield a significant number of false-positives and a currently unknown number of false-negatives. Confirmatory right heart catheterisation remains necessary in all suspected cases. Further research is needed to identify the optimal tools and the screening approach with greatest specificity and selectivity.

  5. Pulmonary artery thrombosis in a patient with right‑sided heart failure

    African Journals Online (AJOL)

    2013-09-19

    Sep 19, 2013 ... features of right-sided heart failure accompanied with dizziness. He was diagnosed to have pulmonary artery thrombosis ... was no history of vertigo. He denied history of orthopnea or paroxysmal nocturnal ... hypertension, diabetes mellitus, or asthma. He was a reformed smoker and heavy alcohol drinker ...

  6. Pleural subxyphoid drain confers better pulmonary function and clinical outcomes in chronic obstructive pulmonary disease after off-pump coronary artery bypass grafting: a randomized controlled trial

    Directory of Open Access Journals (Sweden)

    Solange Guizilini

    2014-12-01

    Full Text Available Objective: To evaluate the lung function and clinical outcome in severe chronic obstructive pulmonary disease in patients undergoing off-pump coronary artery bypass grafting with left internal thoracic artery graft, comparing the pleural drain insertion in the intercostal versus subxyphoid region. Methods: A randomized controlled trial. Chronic obstructive pulmonary disease patients were randomized into two groups according pleural drain site: II group (n=27 - pleural drain in intercostal space; SI group (n=29 - pleural drain in the subxyphoid region. Spirometry values (Forced Vital Capacity - and Forced expiratory volume in 1 second were obtained on preoperative and 1, 3 and 5 postoperative days. Chest x-ray from preoperative until postoperative day 5 (POD5 was performed for monitoring respiratory events, such as atelectasis and pleural effusion. Pulmonary shunt fraction and pain score was evaluate preoperatively and on postoperative day 1. Results: In both groups there was a significant decrease of the spirometry values (Forced Vital Capacity and Forced expiratory volume in 1 second until POD5 (P<0.05. However, when compared, SI group presented less decrease in these parameters (P<0.05. Pulmonary shunt fraction was significantly lower in SI group (P<0.05. Respiratory events, pain score, orotracheal intubation time and postoperative length of hospital stay were lower in the SI group (P<0.05. Conclusion: Subxyphoid pleural drainage in severe Chronic obstructive pulmonary disease patients determined better preservation and recovery of pulmonary capacity and volumes with lower pulmonary shunt fraction and better clinical outcomes on early postoperative off-pump coronary artery bypass grafting.

  7. Vascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection.

    Science.gov (United States)

    Gu, Guangchao; Yang, Hang; Cui, Lijia; Fu, Yuanyuan; Li, Fangda; Zhou, Zhou; Zheng, Yuehong

    2018-02-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a life-threatening connective tissue disorder due to its high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. We present a young male patient with vEDS who developed severe pulmonary complications and severe rupture of the iliac artery at different stages of his life. Vascular Ehlers-Danlos syndrome was diagnosed based on clinical manifestations and confirmed by the identification of COL3A1 gene mutation. Due to high bleeding tendency and weak cardiopulmonary capacity, conservative treatment was taken for him. To our knowledge, this is the first report of vEDS case in which the patient developed both pulmonary complications and dissection of large arteries. Our report emphasizes the importance of considering vEDS when an adolescent develops unexplained pulmonary cysts with fragility of lung tissues. Genetic counseling and close monitoring should be performed for earlier diagnosis and prevention of severe complications of large arteries. The typical presentations of vEDS were also discussed by means of a review of case reports on vEDS with pulmonary complications.

  8. Evaluation of the angiographic findings in pulmonary atresia

    International Nuclear Information System (INIS)

    Choe, Kyu Ok; Sul, Jun Hee; Lee, Seung Kyu; Cho, Bum Koo; Hong, Pill Whoon

    1986-01-01

    We studied the angiographic findings in 65 patients with congenital pulmonary atresia, ages 4 days to 14 years (mean 3.3 yrs), from 1981 to 1986 at Severance Hospital Yonsei University. 1. 6 had pulmonary atresia with an intact interventricular septum, 38 had it with cardiac anomaly Renodynamically simulating TOF, and 21 associated with more complicated cardiac anomalies. 2. In the group with an intact ventricular septum, 5 showed confluent intrapericardial pulmonary artery, all segmental pulmonary arteries connected to intrapericardial artery. 3. In the group simulating TOF, aorta arose from RV with or without overriding in 35. In 27 patients with confluent intrapericardial pulmonary artery, 23 had more than 10 segmental pulmonary arteries connected to intrapericardial artery and 5 had severely hypoplastic hilar pulmonary arteries. In 11 with non confluent intrapericardial pulmonary artery, 4 had more than 10 segmental pulmonary arteries connected to central pulmonary artery and 9 had severely hypoplastic hilar pulmonary arteries. 4. In the group associated with more complicated cardiac anomaly, included 8 patients with atrioventricular discordance, 7 with univentricular heart and 6 with tricuspid atresia. In 17 patients with confluent intrapericardial artery, 16 had more than 10 segmental pulmonary arteries connected to intrapericardial artery, one showed severe hypoplasia of hilar pulmonary arteries. In another 4 with non confluence, no one showed more than 10 segmental arteries connected to intrapericardial or hilar pulmonary artery.

  9. Thallium-201 myocardial imaging in young adults with anomalous left coronary artery arising from the pulmonary artery

    International Nuclear Information System (INIS)

    Moodie, D.S.; Cook, S.A.; Gill, C.C.; Napoli, C.A.

    1980-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery (Bland-Garland-White syndrome) may produce myocardial ischemia, infarction, and frequently death in infancy. Some patients, however, develop satisfactory coronary artery collaterals and are relatively asymptomatic into adulthood. Very little is known about their myocardial perfusion patterns. We studied three young adults with this condition using stress thallium-201 myocardial imaging. Electrocardiograms in two patients demonstrated old arterolateral myocardial infarctions. Preoperative stress exercise tests were positive in all three patients. Marked perfusion abnormalities were found in the proximal anterolateral wall in all patients, and one patient also had a posterolateral defect. Postoperatively, all stress tests returned to normal. Thallium imaging demonstrated improvement in ischemic areas, but old scars persisted

  10. Transcatheter Arterial Embolization With Spherical Embolic Agent for Pulmonary Metastases From Renal Cell Carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Seki, Akihiko, E-mail: sekia@igtc.jp; Hori, Shinichi, E-mail: horishin@igtc.jp; Sueyoshi, Satoru, E-mail: sueyoshis@igtc.jp; Hori, Atsushi, E-mail: horiat@igtc.jp; Kono, Michihiko, E-mail: konom@igtc.jp; Murata, Shinichi, E-mail: muratas@igtc.jp; Maeda, Masahiko, E-mail: maedam@igtc.jp [Gate Tower Institute for Image Guided Therapy, Department of Radiology (Japan)

    2013-12-15

    Purpose: This retrospective study aimed to evaluate the safety and local efficacy of transcatheter arterial embolization (TAE) with superabsorbent polymer microspheres (SAP-MS) in patients with pulmonary metastases from renal cell carcinoma (RCC). Methods: Sixteen patients with unresectable pulmonary metastases from RCC refractory to standard therapy were enrolled to undergo TAE with the purpose of mass reduction and/or palliation. The prepared SAP-MS swell to approximately two times larger than their dry-state size (100-150 {mu}m [n = 14], 50-100 {mu}m [n = 2]). Forty-nine pulmonary nodules (lung n = 22, mediastinal lymph node n = 17, and hilar lymph node n = 10) were selected as target lesions for evaluation. Local tumor response was evaluated 3 months after TAE according to Response Evaluation Criteria in Solid Tumors (RECIST; version 1.1). The relationship between tumor enhancement ratio by CT during selective angiography and local tumor response was evaluated. Results: The number of TAE sessions per patient ranged from 1 to 5 (median 2.9). Embolized arteries at initial TAE were bronchial arteries in 14 patients (87.5 %) and nonbronchial systemic arteries in 11 patients (68.8 %). Nodule-based evaluation showed that 5 (10.2 %) nodules had complete response, 17 (34.7 %) had partial response, 15 (30.6 %) had stable disease, and 12 (24.5 %) had progressive disease. The response rate was significantly greater in 22 lesions that had a high tumor enhancement ratio than in 27 lesions that had a slight or moderate ratio (90.9 vs. 7.4 %, p = 0.01). Severe TAE-related adverse events did not occur. Conclusion: TAE with SAP-MS might be a well-tolerated and locally efficacious palliative option for patients with pulmonary metastases from RCC.

  11. Correlative magnetic resonance imaging in the evaluation of aortic and pulmonary artery abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Risius, B.; O' Donnell, J.K.; Geisinger, M.A.; Zelch, M.G.; George, C.R.; Graor, R.A.; Moodie, D.S.

    1985-05-01

    Magnetic resonance imaging (MRI) yields excellent quality images of the cardiovascular system utilizing the inherent natural contrast between flowing blood and the surrounding anatomic structures. To evaluate the clinical usefulness of MRI in the noninvasive diagnosis of large vessel disorders, the authors have performed MRI on 40 pts with either aortic or pulmonary artery abnormalities (18 thoracic or abdominal aortic aneurysms, 8 aorto-occlusive disease, 6 dissecting aneurysms, 4 Marfan's syndrome, 2 pulmonary artery aneurysms 1 pulmonary artery occlusion, 1 aortic coarctation). Images were obtained in the transverse, coronal and sagital body planes utilizing a 0.6T superconductive magnet. Cardiac and/or respiratory gating was employed in most cases. Correlation was made for all studies with conventional or digital subtraction angiography, computed tomography, and/or ultrasound. The diagnostic information obtained by MRI equaled or exceeded that obtained by other imaging techniques except for the few cases where cardiac arrhythmias precluded adequate gated acquisition. All aneurysms and their relationships to adjacent structures were readily demonstrated as were the presence or absence of mural thrombi and dissecting intimal flaps. Angiographically demonstrated atherosclerotic plaques and luminal stenoses were seen by MRI in all patients without arrhythmias. The authors concluded that MRI is a powerful noninvasive diagnostic aid in the delineation of large vessel disorders, especially where knowledge of anatomic interrelationships can guide surgical or other interventional planning.

  12. Upfront triple combination therapy-induced pulmonary edema in a case of pulmonary arterial hypertension associated with Sjogren's syndrome

    Directory of Open Access Journals (Sweden)

    Kimikazu Takeuchi

    Full Text Available Clinical efficacy of combination therapy using vasodilators for pulmonary arterial hypertension (PAH is well established. However, information on its safety are limited. We experienced a case of primary Sjogren's syndrome associated with PAH where the patient developed pulmonary edema immediately after the introduction of upfront triple combination therapy. Although the combination therapy successfully stabilized her pre-shock state, multiple ground glass opacities (GGO emerged. We aborted the dose escalation of epoprostenol and initiated continuous furosemide infusion and noninvasive positive pressure ventilation (NPPV, but this did not prevent an exacerbation of pulmonary edema. Chest computed tomography showing diffuse alveolar infiltrates without inter-lobular septal thickening suggests the pulmonary edema was unlikely due to cardiogenic pulmonary edema and pulmonary venous occlusive disease. Acute respiratory distress syndrome was also denied from no remarkable inflammatory sign and negative results of drug-induced lymphocyte stimulation tests (DLST. We diagnosed the etiological mechanism as pulmonary vasodilator-induced trans-capillary fluid leakage. Following steroid pulse therapy dramatically improved GGO. We realized that overmuch dose escalation of epoprostenol on the top of dual upfront combination poses the risk of pulmonary edema. Steroid pulse therapy might be effective in cases of vasodilator-induced pulmonary edema in Sjogren's syndrome associated with PAH. Keywords: Steroid therapy, Ground glass opacity, Inter-lobular septal thickening, Epoprostenol, Acute respiratory distress syndrome, Trans-capillary fluid leakage

  13. Podoplanin Expression in Canine Melanoma

    OpenAIRE

    Ogasawara, Satoshi; Honma, Ryusuke; Kaneko, Mika K.; Fujii, Yuki; Kagawa, Yumiko; Konnai, Satoru; Kato, Yukinari

    2016-01-01

    A type I transmembrane protein, podoplanin (PDPN), is expressed in several normal cells such as lymphatic endothelial cells or pulmonary type I alveolar cells. We recently demonstrated that anticanine PDPN monoclonal antibody (mAb), PMab-38, recognizes canine PDPN of squamous cell carcinomas, but does not react with lymphatic endothelial cells. Herein, we investigated whether PMab-38 reacts with canine melanoma. PMab-38 reacted with 90% of melanoma cells (9/10 cases) using immunohistochemistr...

  14. The Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA): a Case Series and Brief Review

    OpenAIRE

    Aliasghar Moeinipour; Mohammad Abbassi Teshnisi; Hassan Mottaghi Moghadam; Nahid Zirak; Reihaneh Hassanzadeh; Hamid Hoseinikhah; Abbas Bahreini

    2016-01-01

    Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect that occurs in approximately 1/300 000 live births or 0.5% of children with congenital heart disease. There are two types of ALCAPA syndrome: the infant type and the adult type. The most infants experience myocardial infarction and congestive heart failure, and approximately 90% die within the first year of life; also, without early surgical intervention they have a dismal p...

  15. [Pulmonary arterial hypertension associated to human immunodeficiency virus].

    Science.gov (United States)

    Sandoval-Gutiérrez, José Luis; Santos-Martínez, Luis Efren; Rodríguez-Silverio, Juan; Baranda-Tovar, Francisco Martín; Rivera-Rosales, Rosa María; Flores-Murrieta, Francisco Javier

    2015-01-01

    From the advent of the highly effective antiretroviral treatment, the life expectancy of patients with human immunodeficiency virus has increased significantly. At present, the causes of death are non-infectious complications. Between them, the pulmonary arterial hypertension has a special importance. It is important early detection to establish the therapeutic, with the objective of preventing a fatal outcome to future. Copyright © 2013 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

  16. Detection of experimentally produced acute pulmonary arterial occlusion by methyl iodide-131 inhalation imaging

    International Nuclear Information System (INIS)

    Grossman, Z.D.; McAfee, J.G.; Subramanian, G.

    1981-01-01

    Methyl iodide-131 (CH 3 I-131) is described as an agent for detection of acute experimentally produced pulmonary arterial occlusion in dogs. When gaseous CH 3 I-131 is inhaled, radioactivity passes instantaneously from the alveoli to the lung capillary bed. Where pulmonary blood flow exists, activity is washed out into the systemic circulation, but in areas of blood stasis, a transient pulmonary hot spot remains. CH 3 I-131 is easily produced and inexpensive, but administration is awkward and strict radiation safety precautions are mandatory

  17. “Anomalous right pulmonary artery left atrial fistula”: Growth in vain

    Directory of Open Access Journals (Sweden)

    Vishal Kumar Jain

    2015-12-01

    Full Text Available We report a case of direct communication between the right pulmonary artery and the left atrium; a rare cause of central cyanosis in 10 year old boy, emphasizing the role of multislice computed tomography scanner in delineating the complex vascular abnormality over more invasive conventional angiography.

  18. Liquid breathing - Prevention of pulmonary arterial-venous shunting during acceleration.

    Science.gov (United States)

    Sass, D. J.; Ritman, E. L.; Caskey, P. E.; Banchero, N.; Wood, E. H.

    1972-01-01

    Dependent pulmonary atelectasis, arterial-venous shunting, and downward displacement of the heart caused by the gravitational-inertial force environment were prevented in dogs breathing oxygenated liquid fluorocarbon in a whole-body water-immersion respirator. Partial closure of the major airways during part of the expiratory phase of liquid respiration was a significant problem initially but was minimized in subsequent studies.

  19. [Supervised exercise training in patients with pulmonary arterial hypertension - analyses of the effectiveness and safety].

    Science.gov (United States)

    Saxer, S; Rhyner, M; Treder, U; Speich, R; van Gestel, A J R

    2012-02-01

    Both in today's scientific research and in clinical practice, there exists a need to address the uncertainty concerning the effectiveness and safety of cardiopulmonary exercise training (CPET) in patients with pulmonary arterial hypertension (PAH). It is commonly believed that CPET may be dangerous for patients with PAH, because increasing pressure on the pulmonary arteries may worsen right-sided heart failure. Recently, the first clinical trials on exercise training in patients with pulmonary hypertension reported promising results. Extension of the walking distance at the 6-minute walk test improved quality of life, endurance capacity and a reduction in symptoms were observed after CPET. Furthermore, CPET was well tolerated by the patients in five clinical trials. In conclusion, it may be postulated that CPET is an effective therapy in patients with PAH and was tendentially well tolerated by the patients.

  20. Pulmonary hyperinflation due to gas trapping and pulmonary artery size: The MESA COPD Study.

    Directory of Open Access Journals (Sweden)

    Hooman D Poor

    Full Text Available Pulmonary hypertension is associated with increased morbidity and mortality in chronic obstructive pulmonary disease (COPD. Since pulmonary artery (PA size increases in pulmonary hypertension, we measured PA cross-sectional area using magnetic resonance imaging (MRI to test the hypothesis that pulmonary hyperinflation due to gas trapping is associated with PA cross-sectional area in COPD.The MESA COPD Study recruited participants with COPD and controls from two population-based cohort studies ages 50-79 years with 10 or more pack-years and free of clinical cardiovascular disease. Body plethysmography was performed according to standard criteria. Cardiac MRI was performed at functional residual capacity to measure the cross-sectional area of the main PA. Percent emphysema was defined as the percentage of lung voxels less than -950 Hounsfield units as assessed via x-ray computed tomography. Analyses were adjusted for age, gender, height, weight, race-ethnicity, the forced expiratory volume in one second, smoking status, pack-years, lung function, oxygen saturation, blood pressure, left ventricular ejection fraction and percent emphysema.Among 106 participants, mean residual volume was 1.98±0.71 L and the mean PA cross-sectional area was 7.23±1.72 cm2. A one standard deviation increase in residual volume was independently associated with an increase in main PA cross-sectional area of 0.55 cm2 (95% CI 0.18 to 0.92; p = 0.003. In contrast, there was no evidence for an association with percent emphysema or total lung capacity.Increased residual volume was associated with a larger PA in COPD, suggesting that gas trapping may contribute to pulmonary hypertension in COPD.

  1. Pulmonary Artery Occlusion and Mediastinal Fibrosis in a Patient on Dopamine Agonist Treatment for Hyperprolactinemia

    DEFF Research Database (Denmark)

    Su, Junjing; Simonsen, Ulf; Carlsen, Jørn

    2017-01-01

    Unusual forms of pulmonary hypertension include pulmonary hypertension related to mediastinal fibrosis and the use of serotonergic drugs. Here, we describe a patient with diffuse mediastinal fibrosis and pulmonary hypertension while she was on dopamine agonist therapy. A young woman, who...... showed fibrosis and chronic inflammation. Subsequent investigations revealed that diffuse mediastinal fibrosis with concurrent pulmonary hypertension, and not CTEPH, was the most likely diagnosis and cabergoline and bromocriptine may have triggered the fibrotic changes. Both drugs are ergot...... was treated with cabergoline and bromocriptine for hyperprolactinemia, presented with progressive dyspnea over several months. Based on the clinical investigation results, in particular, elevated pulmonary arterial pressures and significant perfusion defects on computed tomography (CT) pulmonary angiography...

  2. Rapid Fatal Outcome from Pulmonary Arteries Compression in Transitional Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Ioannis A. Voutsadakis

    2009-01-01

    Full Text Available Transitional cell carcinoma of the urinary bladder is a malignancy that metastasizes frequently to lymph nodes including the mediastinal lymph nodes. This occurrence may produce symptoms due to compression of adjacent structures such as the superior vena cava syndrome or dysphagia from esophageal compression. We report the case of a 59-year-old man with metastatic transitional cell carcinoma for whom mediastinal lymphadenopathy led to pulmonary artery compression and a rapidly fatal outcome. This rare occurrence has to be distinguished from pulmonary embolism, a much more frequent event in cancer patients, in order that proper and prompt treatment be initiated.

  3. Endovascular Treatment of an Aneurysmal Aberrant Systemic Artery Supplying a Pulmonary Sequestrum

    Energy Technology Data Exchange (ETDEWEB)

    Kristensen, Katrine Lawaetz, E-mail: klk@dadlnet.dk [Odense University Hospital, Department of Thoracic and Vascular Surgery (Denmark); Duus, Louise Aarup, E-mail: louise.brodersen@gmail.com [Sygehus Lillebaelt Vejle, Department of Radiology (Denmark); Elle, Bo, E-mail: Bo.Elle@rsyd.dk [Odense University Hospital, Department of Radiology (Denmark)

    2015-10-15

    An aberrant systemic artery originating from the abdominal aorta supplying a pulmonary sequestration is a rare congenital malformation. This causes a left-to-left shunt. Symptoms include recurrent pneumonias, hemoptysis, and, in the long term, heart failure. Aneurysm of the aberrant vessel is rarely seen. Traditionally, treatment of pulmonary sequestrations includes ligation of the feeding vessel and lobectomy. A new promising treatment is an endovascular approach. Only a few cases describe endovascular treatment of pulmonary sequestration. This is the first published case of a giant aneurysmal branch from the abdominal aorta to the normal basal segments of the lung, successfully occluded with an Amplatzer Vascular Plug II (AVP II, St.Jude Medical, MN, USA) alone.

  4. Endovascular Treatment of an Aneurysmal Aberrant Systemic Artery Supplying a Pulmonary Sequestrum

    International Nuclear Information System (INIS)

    Kristensen, Katrine Lawaetz; Duus, Louise Aarup; Elle, Bo

    2015-01-01

    An aberrant systemic artery originating from the abdominal aorta supplying a pulmonary sequestration is a rare congenital malformation. This causes a left-to-left shunt. Symptoms include recurrent pneumonias, hemoptysis, and, in the long term, heart failure. Aneurysm of the aberrant vessel is rarely seen. Traditionally, treatment of pulmonary sequestrations includes ligation of the feeding vessel and lobectomy. A new promising treatment is an endovascular approach. Only a few cases describe endovascular treatment of pulmonary sequestration. This is the first published case of a giant aneurysmal branch from the abdominal aorta to the normal basal segments of the lung, successfully occluded with an Amplatzer Vascular Plug II (AVP II, St.Jude Medical, MN, USA) alone

  5. Randomized comparison of intra-arterial and intravenous thrombolysis in a canine model of acute basilar artery thrombosis

    International Nuclear Information System (INIS)

    Qureshi, A.I.; Yahia, A.M.; Boulos, A.S.; Hanel, R.A.; Suri, M.F.K.; Hopkins, L.N.; Alberico, R.A.

    2004-01-01

    We compared the rates of recanalization cerebral infarct and hemorrhage between intra-arterial (IA) reteplase and intravenous (IV) alteplase thrombolysis in a canine model of basilar artery thrombosis. Thrombosis was induced by injecting a clot in the basilar artery of 13 anesthetized dogs via superselective catheterization. The animals were randomized in a blinded fashion, 2 h after clot injection and verification of arterial occlusion, to receive IV alteplase 0.9 mg/kg over 60 min and IA placebo, or IA reteplase 0.09 units/kg over 20 min, equivalent to one-half the alteplase dose, and IV placebo. Recanalization was studied for 6 h after treatment with serial angiography; the images were later graded in a blinded fashion. Blinded interpretation of postmortem MRI was performed to assess the presence of brain infarcts and/or hemorrhage. At 3 h after initiation of treatment, partial or complete recanalization was observed in one of six dogs in the IV alteplase group and in five of seven in the IA reteplase group (P = 0.08). At 6 h, no significant difference in partial or complete recanalization was observed between the groups (two of six vs. five of seven; P = 0.20). Postmortem MRI revealed infarcts in four of six animals treated with IV alteplase and three of seven treated with IA reteplase (P = 0.4). Intracerebral hemorrhage was more common in the IV alteplase group (four of six vs. none of seven; P = 0.02). This study thus suggests that IA thrombolysis affords a recanalization rate similar to that of IV thrombolysis, but with a lower rate of intracerebral hemorrhage. (orig.)

  6. The emergence of oral tadalafil as a once-daily treatment for pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Jeremy A Falk

    2010-04-01

    Full Text Available Jeremy A Falk, Kiran J Philip, Ernst R SchwarzCedars Sinai Women’s Guild Lung Institute, Cedars Sinai Heart Institute, Cedars Sinai Medical Center, Los Angeles, CA, USAAbstract: Pulmonary hypertension (PH is found in a vast array of diseases, with a minority representing pulmonary arterial hypertension (PAH. Idiopathic PAH or PAH in association with other disorders has been associated with poor survival, poor exercise tolerance, progressive symptoms of dyspnea, and decreased quality of life. Left untreated, patients with PAH typically have a progressive decline in function with high morbidity ultimately leading to death. Advances in medical therapy for PAH over the past decade have made significant inroads into improved function, quality of life, and even survival in this patient population. Three classes of pulmonary artery-specific vasodilators are currently available in the United States. They include prostanoids, endothelin receptor antagonists, and phosphodiesterase type 5 (PDE5 inhibitors. In May 2009, the FDA approved tadalafil, the first once-daily PDE5 inhibitor for PAH. This review will outline the currently available data on tadalafil and its effects in patients with PAH.Keywords: PDE-5 inhibition, pulmonary hypertension, tadalafil

  7. Interventional and surgical therapeutic strategies for pulmonary arterial hypertension: Beyond palliative treatments.

    Science.gov (United States)

    Sandoval, Julio; Gomez-Arroyo, Jose; Gaspar, Jorge; Pulido-Zamudio, Tomas

    2015-10-01

    Despite significant advances in pharmacological treatments, pulmonary arterial hypertension remains an incurable disease with an unreasonably high morbidity and mortality. Although specific pharmacotherapies have shifted the survival curves of patients and improved exercise endurance as well as quality of life, it is also true that these pharmacological interventions are not always accessible (particularly in developing countries) and, perhaps most importantly, not all patients respond similarly to these drugs. Furthermore, many patients will continue to deteriorate and will eventually require an additional, non-pharmacological, intervention. In this review we analyze the role of atrial septostomy and Potts anastomosis in the management of patients with pulmonary arterial hypertension, we summarize the current worldwide clinical experience (case reports and case series), and discuss why these interventional/surgical strategies might have a therapeutic role beyond that of a "bridge" to transplantation. Copyright © 2015 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

  8. Intermittent hypoxia and hypercapnia induce pulmonary artery atherosclerosis and ventricular dysfunction in low density lipoprotein receptor deficient mice.

    Science.gov (United States)

    Douglas, Robert M; Bowden, Karen; Pattison, Jennifer; Peterson, Alexander B; Juliano, Joseph; Dalton, Nancy D; Gu, Yusu; Alvarez, Erika; Imamura, Toshihiro; Peterson, Kirk L; Witztum, Joseph L; Haddad, Gabriel G; Li, Andrew C

    2013-12-01

    Patients with obstructive sleep apnea, who experience episodic hypoxia and hypercapnia during sleep, often demonstrate increased inflammation, oxidative stress, and dyslipidemia. We hypothesized that sleep apnea patients would be predisposed to the development of atherosclerosis. To dissect the mechanisms involved, we developed an animal model in mice whereby we expose mice to intermittent hypoxia/hypercapnia (IHH) in normobaric environments. Two- to three-month-old low-density lipoprotein receptor deficient (Ldlr(-/-)) mice were fed a high-fat diet for 8 or 16 wk while being exposed to IHH for either 10 h/day or 24 h/day. Plasma lipid levels, pulmonary artery and aortic atherosclerotic lesions, and cardiac function were then assayed. Surprisingly, atherosclerosis in the aorta of IHH mice was similar compared with controls. However, in IHH mice, atherosclerosis was markedly increased in the trunk and proximal branches of the pulmonary artery of exposed mice; even though plasma cholesterol and triglycerides were lower than in controls. Hemodynamic analysis revealed that right ventricular maximum pressure and isovolumic relaxation constant were significantly increased in IHH exposed mice and left ventricular % fractional shortening was reduced. In conclusion, 1) Intermittent hypoxia/hypercapnia remarkably accelerated atherosclerotic lesions in the pulmonary artery of Ldlr(-/-) mice and 2) increased lesion formation in the pulmonary artery was associated with right and left ventricular dysfunction. These findings raise the possibility that patients with obstructive sleep apnea may be susceptible to atherosclerotic disease in the pulmonary vasculature, an observation that has not been previously recognized.

  9. Right ventricular to pulmonary artery conduit instead of modified Blalock-Taussig shunt improves postoperative hemodynamics in newborns after the Norwood operation.

    Science.gov (United States)

    Mair, Rudolf; Tulzer, Gerald; Sames, Eva; Gitter, Roland; Lechner, Evelyn; Steiner, Jürgen; Hofer, Anna; Geiselseder, Gertraud; Gross, Christoph

    2003-11-01

    Perioperative mortality, prolonged postoperative recovery after the Norwood procedure, and mortality between stage I and stage II might be related to shunt physiology. A right ventricular to pulmonary artery conduit offers a banded physiology in contrast to a Blalock-Taussig shunt. The purpose of this study was to assess the hemodynamic differences and their consequences in the postoperative course between Norwood patients with a Blalock-Taussig shunt and those with a right ventricular to pulmonary artery conduit. From October 1999 until May 2002, 32 unselected consecutive patients underwent a Norwood procedure at the General Hospital Linz. The first 18 patients received a Blalock-Taussig shunt. In the remaining 14 patients we performed a right ventricular to pulmonary artery conduit. Both groups were compared. The diastolic blood pressure was significantly higher in the right ventricular to pulmonary artery conduit group (P <.001). Despite a higher FIO(2), PO(2) levels tended to be lower in the first 5 postoperative days. At the age of 3 months, catheterization laboratory data showed a lower Qp/Qs ratio in the same group (0.86 [0.78; 1] versus 1.55 [1.15; 1.6]; P =.005) and a higher dp/dt (955 [773; 1110] vs 776 [615; 907]; P =.018). (Descriptive data reflect medians and quartiles [in brackets].) Hospital survival was 72% in the Blalock-Taussig shunt group versus 93% in the right ventricular to pulmonary artery conduit group. Mortality between stage I and stage II was 23% in the Blalock-Taussig shunt group versus 0% in the right ventricular to pulmonary artery conduit group. A higher diastolic blood pressure and a lower Qp/Qs ratio were associated with a more stable and efficient circulation in patients with a right ventricular to pulmonary artery conduit. More intensive ventilatory support was necessary during the first postoperative days. We did not note any adverse effects of the ventriculotomy on ventricular performance.

  10. Cardiac MRI in pulmonary artery hypertension: correlations between morphological and functional parameters and invasive measurements

    Energy Technology Data Exchange (ETDEWEB)

    Alunni, Jean-Philippe; Otal, Philippe; Rousseau, Herve; Chabbert, Valerie [CHU Rangueil, Department of Radiology, Toulouse (France); Degano, Bruno; Tetu, Laurent; Didier, Alain [CHU Larrey, Department of Pneumology, Toulouse (France); Arnaud, Catherine [CHU Rangueil, Department of Methods in Clinical Research, Toulouse (France); Blot-Souletie, Nathalie [CHU Rangueil, Department of Cardiology, Toulouse (France)

    2010-05-15

    To compare cardiac MRI with right heart catheterisation in patients with pulmonary hypertension (PH) and to evaluate its ability to assess PH severity. Forty patients were included. MRI included cine and phase-contrast sequences, study of ventricular function, cardiac cavity areas and ratios, position of the interventricular septum (IVS) in systole and diastole, and flow measurements. We defined four groups according to the severity of PH and three groups according to IVS position: A, normal position; B, abnormal in diastole; C, abnormal in diastole and systole. IVS position was correlated with pulmonary artery pressures and PVR (pulmonary vascular resistance). Median pulmonary artery pressures and resistance were significantly higher in patients with an abnormal septal position compared with those with a normal position. Correlations were good between the right ventricular ejection fraction and PVR, right ventricular end-systolic volume and PAP, percentage of right ventricular area change and PVR, and diastolic and systolic ventricular area ratio and PVR. These parameters were significantly associated with PH severity. Cardiac MRI can help to assess the severity of PH. (orig.)

  11. Asthma causes inflammation of human pulmonary arteries and decreases vasodilatation induced by prostaglandin I2 analogs.

    Science.gov (United States)

    Foudi, Nabil; Badi, Aouatef; Amrane, Mounira; Hodroj, Wassim

    2017-12-01

    Asthma is a chronic inflammatory disease associated with increased cardiovascular events. This study assesses the presence of inflammation and the vascular reactivity of pulmonary arteries in patients with acute asthma. Rings of human pulmonary arteries obtained from non-asthmatic and asthmatic patients were set up in organ bath for vascular tone monitoring. Reactivity was induced by vasoconstrictor and vasodilator agents. Protein expression of inflammatory markers was detected by western blot. Prostanoid releases and cyclic adenosine monophosphate (cAMP) levels were quantified using specific enzymatic kits. Protein expression of cluster of differentiation 68, intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and cyclooxygenase-2 was significantly increased in arteries obtained from asthmatic patients. These effects were accompanied by an alteration of vasodilatation induced by iloprost and treprostinil, a decrease in cAMP levels and an increase in prostaglandin (PG) E 2 and PGI 2 synthesis. The use of forskolin (50 µmol/L) has restored the vasodilatation and cAMP release. No difference was observed between the two groups in reactivity induced by norepinephrine, angiotensin II, PGE 2 , KCl, sodium nitroprusside, and acetylcholine. Acute asthma causes inflammation of pulmonary arteries and decreases vasodilation induced by PGI 2 analogs through the impairment of cAMP pathway.

  12. Serotonin Signaling Through the 5-HT1B Receptor and NADPH Oxidase 1 in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Hood, Katie Y; Mair, Kirsty M; Harvey, Adam P; Montezano, Augusto C; Touyz, Rhian M; MacLean, Margaret R

    2017-07-01

    Serotonin can induce human pulmonary artery smooth muscle cell (hPASMC) proliferation through reactive oxygen species (ROS), influencing the development of pulmonary arterial hypertension (PAH). We hypothesize that in PASMCs, serotonin induces oxidative stress through NADPH-oxidase-derived ROS generation and reduced Nrf-2 (nuclear factor [erythroid-derived 2]-like 2) antioxidant systems, promoting vascular injury. HPASMCs from controls and PAH patients, and PASMCs from Nox1 -/- mice, were stimulated with serotonin in the absence/presence of inhibitors of Src kinase, the 5-HT 1B receptor, and NADPH oxidase 1 (Nox1). Markers of fibrosis were also determined. The pathophysiological significance of our findings was examined in vivo in serotonin transporter overexpressing female mice, a model of pulmonary hypertension. We confirmed thatserotonin increased superoxide and hydrogen peroxide production in these cells. For the first time, we show that serotonin increased oxidized protein tyrosine phosphatases and hyperoxidized peroxiredoxin and decreased Nrf-2 and catalase activity in hPASMCs. ROS generation was exaggerated and dependent on cellular Src-related kinase, 5-HT 1B receptor, and the serotonin transporter in human pulmonary artery smooth muscle cells from PAH subjects. Proliferation and extracellular matrix remodeling were exaggerated in human pulmonary artery smooth muscle cells from PAH subjects and dependent on 5-HT 1B receptor signaling and Nox1, confirmed in PASMCs from Nox1 -/- mice. In serotonin transporter overexpressing mice, SB216641, a 5-HT 1B receptor antagonist, prevented development of pulmonary hypertension in a ROS-dependent manner. Serotonin can induce cellular Src-related kinase-regulated Nox1-induced ROS and Nrf-2 dysregulation, contributing to increased post-translational oxidative modification of proteins and activation of redox-sensitive signaling pathways in hPASMCs, associated with mitogenic responses. 5-HT 1B receptors contribute to

  13. The study of risk in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Gerald Simonneau

    2012-09-01

    Full Text Available A growing body of published evidence exists on the risk factors for disease progression in pulmonary arterial hypertension (PAH. The Scientific Steering Committee for the Study of Risk in PAH was established to bring together leading clinical and statistical experts in PAH and risk modelling, for the purpose of advancing the understanding of the risk of development and progression of PAH. Herein, we discuss the impact of this information on three key areas: 1 clinical decision-making; 2 policy and reimbursement; and 3 future trials and research.

  14. Measurement of pulmonary arterial elastance in patients with systolic heart failure using Doppler echocardiography

    Science.gov (United States)

    Taghavi, Sepideh; Esmaeilzadeh, Maryam; Amin, Ahmad; Naderi, Nasim; Abkenar, Hooman Bakhshandeh; Maleki, Majid; Mitra, Chitsazan

    2016-01-01

    Objective: A reliable and easy-to-perform method for measuring right ventricular (RV) afterload is desirable when scheduling patients with systolic heart failure to undergo heart transplantation. The present study aimed to investigate the accuracy of echocardiographically-derived pulmonary arterial elastance as a measurement of pulmonary vascular resistance by comparing it with invasive measures. Methods: Thirty-one patients with moderate to severe systolic heart failure, including 22 (71%) male patients, with a mean age of 41.16±15.9 years were enrolled in the study. Right heart catheterization and comprehensive echocardiography during the first hour after completion of cardiac catheterization were performed in all the patients. The pulmonary artery elastance was estimated using the ratio of end-systolic pressure (Pes) over the stroke volume (SV) by both cardiac catheterization [Ea (PV)-C] and echocardiography [Ea (PV)-E]. Results: The mean Ea (PV)-C and Ea (PV)-E were estimated to be 0.73±0.49 mm Hg/mL and 0.67±0.44 mm Hg/mL, respectively. There was a significant relation between Ea (PV)-E and Ea (PV)-C (r=0.897, pechocardiography and catheterization methods for estimating Ea (PV), investigated by the Bland-Altman method, showed a mean bias of -0.06, with 95% limits of agreement from -0.36 mm Hg/mL to 0.48 mm Hg/mL. Conclusion: Doppler echocardiography is an easy, non-invasive, and inexpensive method for measuring pulmonary arterial elastance, which provides accurate and reliable estimation of RV afterload in patients with systolic heart failure. PMID:26467379

  15. Pulmonary Arterial Hypertension: Use of Delayed Contrast-Enhanced Cardiovascular Magnetic Resonance in Risk Assessment

    International Nuclear Information System (INIS)

    Bessa, Luiz Gustavo Pignataro; Junqueira, Flávia Pegado; Bandeira, Marcelo Luiz da Silva; Garcia, Marcelo Iorio; Xavier, Sérgio Salles; Lavall, Guilherme; Torres, Diego; Waetge, Daniel

    2013-01-01

    Pulmonary arterial hypertension is a severe and progressive disease. Its early diagnosis is the greatest clinical challenge. To evaluate the presence and extension of the delayed myocardial contrast-enhanced cardiovascular magnetic resonance, as well as to verify if the percentage of the myocardial fibrosis mass is a severity predictor. Cross-sectional study with 30 patients with pulmonary arterial hypertension of groups I and IV, subjected to clinical, functional and hemodynamic evaluation, and to cardiac magnetic resonance. The mean age of patients was 52 years old, with female predominance (77%). Among the patients, 53% had right ventricular failure at diagnosis, and 90% were in functional class II/III. The mean of the 6-minute walk test was 395m. In hemodynamic study with right catheterism, the mean average pulmonary arterial pressure was 53.3mmHg, of the cardiac index of 2.1L/ min.m 2 , and median right atrial pressure was 13.5 mmHg. Delayed myocardial contrast enhanced cardiovascular magnetic resonance was found in 28 patients. The mean fibrosis mass was 9.9 g and the median percentage of fibrosis mass was 6.17%. The presence of functional class IV, right ventricular failure at diagnosis, 6-minute walk test < 300 meters and right atrial pressure ≥ 15 mmHg, with cardiac index < 2.0 L/ min.m 2 , there was a relevant association with the increased percentage of myocardial fibrosis. The percentage of the myocardial fibrosis mass indicates a non-invasive marker with promising perspectives in identifying patients with high risk factors for pulmonary hypertension

  16. Pulmonary Arterial Hypertension: Use of Delayed Contrast-Enhanced Cardiovascular Magnetic Resonance in Risk Assessment

    Energy Technology Data Exchange (ETDEWEB)

    Bessa, Luiz Gustavo Pignataro, E-mail: lgpignataro@ig.com.br; Junqueira, Flávia Pegado; Bandeira, Marcelo Luiz da Silva; Garcia, Marcelo Iorio; Xavier, Sérgio Salles; Lavall, Guilherme; Torres, Diego; Waetge, Daniel [Hospital Universitário Clementino Fraga Filho, Ilha do Fundão, RJ (Brazil)

    2013-10-15

    Pulmonary arterial hypertension is a severe and progressive disease. Its early diagnosis is the greatest clinical challenge. To evaluate the presence and extension of the delayed myocardial contrast-enhanced cardiovascular magnetic resonance, as well as to verify if the percentage of the myocardial fibrosis mass is a severity predictor. Cross-sectional study with 30 patients with pulmonary arterial hypertension of groups I and IV, subjected to clinical, functional and hemodynamic evaluation, and to cardiac magnetic resonance. The mean age of patients was 52 years old, with female predominance (77%). Among the patients, 53% had right ventricular failure at diagnosis, and 90% were in functional class II/III. The mean of the 6-minute walk test was 395m. In hemodynamic study with right catheterism, the mean average pulmonary arterial pressure was 53.3mmHg, of the cardiac index of 2.1L/ min.m{sup 2}, and median right atrial pressure was 13.5 mmHg. Delayed myocardial contrast enhanced cardiovascular magnetic resonance was found in 28 patients. The mean fibrosis mass was 9.9 g and the median percentage of fibrosis mass was 6.17%. The presence of functional class IV, right ventricular failure at diagnosis, 6-minute walk test < 300 meters and right atrial pressure ≥ 15 mmHg, with cardiac index < 2.0 L/ min.m{sup 2}, there was a relevant association with the increased percentage of myocardial fibrosis. The percentage of the myocardial fibrosis mass indicates a non-invasive marker with promising perspectives in identifying patients with high risk factors for pulmonary hypertension.

  17. The relationship between pulmonary artery wedge pressure and pulmonary blood volume derived from contrast echocardiography: A proof-of-concept study.

    Science.gov (United States)

    Monahan, Ken; Lenihan, Daniel; Brittain, Evan L; Saliba, Linda; Piana, Robert N; Robison, Leslie L; Hudson, Melissa M; Armstrong, Gregory T

    2018-05-14

    Pulmonary transit time (PTT) obtained from contrast echocardiography is a marker of global cardiopulmonary function. Pulmonary blood volume (PBV), derived from PTT, may be a noninvasive surrogate for left-sided filling pressures, such as pulmonary artery wedge pressure (PAWP). We sought to assess the relationship between PBV obtained from contrast echocardiography and PAWP. Participants were adult survivors of childhood cancer that had contrast echocardiography performed nearly simultaneously with right-heart catheterization. PTT was derived from time-intensity curves of contrast passage through the right ventricle (RV) and left atrium (LA). PBV relative to overall stroke volume (rPBV) was estimated from the product of PTT and heart rate during RV-LA transit. PAWP was obtained during standard right-heart catheterization. The Spearman correlation coefficient was used to assess the relationship between rPBV and PAWP. The study population consisted of 7 individuals who had contrast echocardiography and right-heart catheterization within 3 hours of each other. There was a wide range of right atrial (1-17 mm Hg), mean pulmonary artery (18-42 mm Hg), and PAW pressures (4-26 mm Hg) as well as pulmonary vascular resistance (<1-6 Wood Units). We observed a statistically significant correlation between rPBV and PAWP (r = .85; P = .02). Relative PBV derived from contrast echocardiography correlates with PAWP. If validated in larger studies, rPBV could potentially be used as an alternative to invasively determine left-sided filling pressure. © 2018 Wiley Periodicals, Inc.

  18. Large and medium-sized pulmonary artery obstruction does not play a role of primary importance in the etiology of sickle-cell disease-associated pulmonary hypertension

    NARCIS (Netherlands)

    van Beers, Eduard J.; van Eck-Smit, Berthe L. F.; Mac Gillavry, Melvin R.; van Tuijn, Charlotte F. J.; van Esser, Joost W. J.; Brandjes, Dees P. M.; Kappers-Klunne, Mies C.; Duits, Ashley J.; Biemond, Bart J.; Schnog, John-John B.

    2008-01-01

    Background: Pulmonary hypertension (PHT) occurs in approximately 30% of adult patients with sickle-cell disease (SCD) and is a risk factor for early death. The potential role of pulmonary artery obstruction, whether due to emboli or in situ thrombosis, in the etiology of SCD-related PHT is unknown.

  19. Vaccine-induced canine distemper in a lesser panda.

    Science.gov (United States)

    Bush, M; Montali, R J; Brownstein, D; James, A E; Appel, M J

    1976-11-01

    A fatal disease occurred in a lesser panda (Ailurus fulgens) 2 weeks after vaccination with modified live distemper vaccine. The disease clinically resembled canine distemper. Pathologically there was giant cell pneumonia, with canine distemper viral inclusion bodies in pulmonary and digestive tract epithelium. Viral isolates were indicative of an attenuated strain rather than virulent types.

  20. Alpha-1-antitrypsin studies: canine serum and canine surfactant protein

    International Nuclear Information System (INIS)

    Tuttle, W.C.; Slauson, D.O.; Dahlstrom, M.; Gorman, C.

    1974-01-01

    Canine serum alpha-1-antitrypsin was isolated by gel filtration and affinity chromatography and characterized by polyacrylamide gel electrophoresis and immunoelectrophoresis. Measurement of the trypsin inhibitory capacity of the separated protein indicated a ninefold concentration of functional trypsin inhibitor during the isolation procedure. Electrophoresis demonstrated the presence of a single protein with alpha-globulin mobility and a molecular weight near that of human alpha-1-antitrypsin. The trypsin inhibitory capacity of pulmonary surfactant protein from five Beagle dogs was measured, related to total surfactant protein concentration, and compared with similar measurements on whole serum from the same animals. Results indicated a variable concentration of trypsin inhibitor in the canine pulmonary surfactant protein. However, the concentration in the surfactant protein was always significantly higher than that in the corresponding serum sample. Preliminary experiments designed to separate the trypsin inhibitory fraction(s) from the other surfactant proteins by gel filtration chromatography indicated that the trypsin inhibitor was probably a single protein with a molecular weight near that of alpha-1-antitrypsin. (U.S.)

  1. CHRONIC OBSTRUCTIVE PULMONARY DISEASE AND ARTERIAL HYPERTENSION: VASCULAR WALL AS THE TARGET ORGAN IN COMORBID PATIENTS

    OpenAIRE

    N. A. Karoli; A. P. Rebrov

    2017-01-01

    Studies of endothelial dysfunction in patients with respiratory diseases have become relevant in recent years. Perhaps endothelial dysfunction and high arterial stiffness bind bronchopulmonary and cardiovascular diseases.Aim. To reveal features of disturbances of arterial wall vasoregulatory function in patients with chronic obstructive pulmonary disease (COPD) in the presence and absence of arterial hypertension (HT).Material and methods. The study included 50 patients with COPD with normal ...

  2. Use of mathematic modeling to compare and predict hemodynamic effects of the modified Blalock-Taussig and right ventricle-pulmonary artery shunts for hypoplastic left heart syndrome.

    Science.gov (United States)

    Bove, Edward L; Migliavacca, Francesco; de Leval, Marc R; Balossino, Rossella; Pennati, Giancarlo; Lloyd, Thomas R; Khambadkone, Sachin; Hsia, Tain-Yen; Dubini, Gabriele

    2008-08-01

    Stage one reconstruction (Norwood operation) for hypoplastic left heart syndrome can be performed with either a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. Both methods have certain inherent characteristics. It is postulated that mathematic modeling could help elucidate these differences. Three-dimensional computer models of the Blalock-Taussig shunt and right ventricle-pulmonary artery shunt modifications of the Norwood operation were developed by using the finite volume method. Conduits of 3, 3.5, and 4 mm were used in the Blalock-Taussig shunt model, whereas conduits of 4, 5, and 6 mm were used in the right ventricle-pulmonary artery shunt model. The hydraulic nets (lumped resistances, compliances, inertances, and elastances) were identical in the 2 models. A multiscale approach was adopted to couple the 3-dimensional models with the circulation net. Computer simulations were compared with postoperative catheterization data. Good correlation was found between predicted and observed data. For the right ventricle-pulmonary artery shunt modification, there was higher aortic diastolic pressure, decreased pulmonary artery pressure, lower Qp/Qs ratio, and higher coronary perfusion pressure. Mathematic modeling predicted minimal regurgitant flow in the right ventricle-pulmonary artery shunt model, which correlated with postoperative Doppler measurements. The right ventricle-pulmonary artery shunt demonstrated lower stroke work and a higher mechanical efficiency (stroke work/total mechanical energy). The close correlation between predicted and observed data supports the use of mathematic modeling in the design and assessment of surgical procedures. The potentially damaging effects of a systemic ventriculotomy in the right ventricle-pulmonary artery shunt modification of the Norwood operation have not been analyzed.

  3. Impact of evolving strategy on clinical outcomes and central pulmonary artery growth in patients with bilateral superior vena cava undergoing a bilateral bidirectional cavopulmonary shunt.

    Science.gov (United States)

    Honjo, Osami; Tran, Kim-Chi D; Hua, Zhongdong; Sapra, Priya; Alghamdi, Abdullah A; Russell, Jennifer L; Caldarone, Christopher A; Van Arsdell, Glen S

    2010-09-01

    We reported a high incidence of thrombosis, central pulmonary artery hypoplasia, and mortality for bilateral bidirectional cavopulmonary shunts. We hypothesized that technical modifications in the cavopulmonary anastomosis and anticoagulation would limit thrombus and central pulmonary artery hypoplasia, and thereby improve outcomes. Sixty-one patients (median age, 8.4 months; weight, 6.6 kg) underwent bilateral bidirectional cavopulmonary shunt from 1990 to 2007. The cohort was divided into 2 groups: 1) the conventional group (1990-1999, n = 37) and 2) the V-shaped group, with a hemi-Fontan or modification in which the cavae were anastomosed to the pulmonary artery adjacent to each other so they formed the appearance of a V (1999-2007, n = 24). Central and branch pulmonary artery growth, survival, and reinterventions were determined. The pre-Fontan study showed equivalent superior venae cavae and Nakata indices. The central pulmonary artery index and central pulmonary artery/Nakata index ratio were significantly higher in the V-shaped group (P analysis showed anastomotic strategy, low saturation, and thrombosis were predictors for death. Anastomotic strategy, lack of anticoagulation, thrombosis, and small superior venae cavae were predictors for reintervention (P strategy affected reintervention. Anastomotic strategy and postoperative thrombus affected mortality. 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  4. An efficient parallel simulation of unsteady blood flows in patient-specific pulmonary artery.

    Science.gov (United States)

    Kong, Fande; Kheyfets, Vitaly; Finol, Ender; Cai, Xiao-Chuan

    2018-04-01

    Simulation of blood flows in the pulmonary artery provides some insight into certain diseases by examining the relationship between some continuum metrics, eg, the wall shear stress acting on the vascular endothelium, which responds to flow-induced mechanical forces by releasing vasodilators/constrictors. V. Kheyfets, in his previous work, studies numerically a patient-specific pulmonary circulation to show that decreasing wall shear stress is correlated with increasing pulmonary vascular impedance. In this paper, we develop a scalable parallel algorithm based on domain decomposition methods to investigate an unsteady model with patient-specific pulsatile waveforms as the inlet boundary condition. The unsteady model offers tremendously more information about the dynamic behavior of the flow field, but computationally speaking, the simulation is a lot more expensive since a problem which is similar to the steady-state problem has to be solved many times, and therefore, the traditional sequential approach is not suitable anymore. We show computationally that simulations using the proposed parallel approach with up to 10 000 processor cores can be obtained with much reduced compute time. This makes the technology potentially usable for the routine study of the dynamic behavior of blood flows in the pulmonary artery, in particular, the changes of the blood flows and the wall shear stress in the spatial and temporal dimensions. Copyright © 2017 John Wiley & Sons, Ltd.

  5. The role of disturbed blood flow in the development of pulmonary arterial hypertension : lessons from preclinical animal models

    NARCIS (Netherlands)

    Dickinson, Michael G.; Bartelds, Beatrijs; Borgdorff, Marinus A. J.; Berger, Rolf M. F.

    Pulmonary arterial hypertension (PAH) is a progressive pulmonary vasoproliferative disorder characterized by the development of unique neointimal lesions, including concentric laminar intima fibrosis and plexiform lesions. Although the histomorphology of neointimal lesions is well described, the

  6. Intermittent hypoxia and hypercapnia induce pulmonary artery atherosclerosis and ventricular dysfunction in low density lipoprotein receptor deficient mice

    Science.gov (United States)

    Bowden, Karen; Pattison, Jennifer; Peterson, Alexander B.; Juliano, Joseph; Dalton, Nancy D.; Gu, Yusu; Alvarez, Erika; Imamura, Toshihiro; Peterson, Kirk L.; Witztum, Joseph L.; Haddad, Gabriel G.; Li, Andrew C.

    2013-01-01

    Patients with obstructive sleep apnea, who experience episodic hypoxia and hypercapnia during sleep, often demonstrate increased inflammation, oxidative stress, and dyslipidemia. We hypothesized that sleep apnea patients would be predisposed to the development of atherosclerosis. To dissect the mechanisms involved, we developed an animal model in mice whereby we expose mice to intermittent hypoxia/hypercapnia (IHH) in normobaric environments. Two- to three-month-old low-density lipoprotein receptor deficient (Ldlr−/−) mice were fed a high-fat diet for 8 or 16 wk while being exposed to IHH for either 10 h/day or 24 h/day. Plasma lipid levels, pulmonary artery and aortic atherosclerotic lesions, and cardiac function were then assayed. Surprisingly, atherosclerosis in the aorta of IHH mice was similar compared with controls. However, in IHH mice, atherosclerosis was markedly increased in the trunk and proximal branches of the pulmonary artery of exposed mice; even though plasma cholesterol and triglycerides were lower than in controls. Hemodynamic analysis revealed that right ventricular maximum pressure and isovolumic relaxation constant were significantly increased in IHH exposed mice and left ventricular % fractional shortening was reduced. In conclusion, 1) Intermittent hypoxia/hypercapnia remarkably accelerated atherosclerotic lesions in the pulmonary artery of Ldlr−/− mice and 2) increased lesion formation in the pulmonary artery was associated with right and left ventricular dysfunction. These findings raise the possibility that patients with obstructive sleep apnea may be susceptible to atherosclerotic disease in the pulmonary vasculature, an observation that has not been previously recognized. PMID:23990245

  7. Recanalization of an occluded left pulmonary artery: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Ali Ibrahim Elarabi

    2017-01-01

    Full Text Available We report an 8-year-old male child with tetralogy of Fallot (TOF, who developed left pulmonary artery (LPA atresia, following surgical repair of TOF and left pulmonary arterioplasty at the age of 6 years. He underwent successful radiofrequency recanalization and stenting of the LPA. The LPA exhibited satisfactory growth for 3 months, following recanalization and stenting.

  8. Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions

    Directory of Open Access Journals (Sweden)

    Cha Gon Lee

    2010-02-01

    Full Text Available Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH. Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (?#248; Wood unit from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD, VSD and patent ductus arteriosus (PDA, and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ?#241;.5. Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

  9. Prevalence of renal artery stenosis in flash pulmonary oedema: determination using gadolinium-enhanced MRA.

    LENUS (Irish Health Repository)

    McMahon, Colm J

    2012-02-01

    PURPOSE: The primary purpose was to determine the prevalence of renal artery stenosis (RAS) in patients presenting with acute ("flash") pulmonary oedema (FPE), without identifiable cause using contrast-enhanced magnetic resonance angiography (CE-MRA) of renal arteries. A secondary goal was to correlate clinical parameters at presentation with the presence or absence of RAS. MATERIALS AND METHODS: Patients presenting with acute pulmonary oedema without identifiable cause prospectively underwent CE-MRA. >50% renal artery stenosis was considered significant. Clinical parameters (blood pressure, serum creatinine, history of hypertension\\/hyperlipidaemia) were compared in patients with and without RAS using an unpaired t-test. Results expressed; mean (+\\/-SD). RESULTS: 20 patients (4 male, 16 female, age 78.5+\\/-11 years) underwent CE-MRA. 9 patients (45%) had significant RAS (6 (30%) bilateral, 3 (15%) unilateral). Systolic BP was higher in patients with RAS (192+\\/-38 mm Hg) than those without (134+\\/-30 mm Hg) (p<.005). Diastolic BP was higher in patients with RAS (102+\\/-23 mm Hg) than those without (76+\\/-17 mm Hg) (p<.01). All patients with RAS and 6\\/11(55%) patients without RAS had a history of hypertension. No significant difference in creatinine or hyperlipidaemia history was observed. CONCLUSION: The prevalence of RAS in patients presenting with FPE is 45%. The diagnosis should be considered in patients presenting with unexplained acute pulmonary oedema, particularly if hypertensive at presentation.

  10. Podoplanin Expression in Canine Melanoma.

    Science.gov (United States)

    Ogasawara, Satoshi; Honma, Ryusuke; Kaneko, Mika K; Fujii, Yuki; Kagawa, Yumiko; Konnai, Satoru; Kato, Yukinari

    2016-12-01

    A type I transmembrane protein, podoplanin (PDPN), is expressed in several normal cells such as lymphatic endothelial cells or pulmonary type I alveolar cells. We recently demonstrated that anticanine PDPN monoclonal antibody (mAb), PMab-38, recognizes canine PDPN of squamous cell carcinomas, but does not react with lymphatic endothelial cells. Herein, we investigated whether PMab-38 reacts with canine melanoma. PMab-38 reacted with 90% of melanoma cells (9/10 cases) using immunohistochemistry. Of interest, PMab-38 stained the lymphatic endothelial cells and cancer-associated fibroblasts in melanoma tissues, although it did not stain any lymphatic endothelial cells in normal tissues. PMab-38 could be useful for uncovering the function of PDPN in canine melanomas.

  11. The measurement of blood speed in the pulmonary artery trunk

    Energy Technology Data Exchange (ETDEWEB)

    Saro, J P; Bula-Cruz, J [UTAD - 5000 Vila Real (Spain); Rafael, J A [Dep. Electronica e Telecomunicacoes da Univ. de Aveiro - 3800 Aveiro (Spain); Botelho, M F; Lima, J P [IBILI - Faculdade de Medicina da Univ. de Coimbra - 3000 Coimbra (Spain)

    1999-12-31

    The paper describes a non invasive methodology for the measurement of blood speed in the pulmonary artery trunk. The methodology has been tested with a moving radioactive tracer (nuclear medicine). An image processing technique is proposed, for detection and analysis of a moving object with variable shape and intensity over time (radioactive bolus). Experiments on the application of the technique in nuclear medicine are critically analysed. (authors) 9 refs., 7 figs.

  12. Estimation of pulmonary artery pressure in patients with primary pulmonary hypertension by quantitative analysis of magnetic resonance images.

    Science.gov (United States)

    Murray, T I; Boxt, L M; Katz, J; Reagan, K; Barst, R J

    1994-01-01

    The use of magnetic resonance (MR) images for estimating mean pulmonary artery pressure (PAP) was tested by comparing main pulmonary artery (MPA) and middescending thoracic aorta (AO) caliber in 12 patients with primary pulmonary hypertension (PPH) with measurements made in eight other patients who were observed for diseases other than heart disease (controls). The ratio MPA/AO and the ratios of vessel caliber normalized to body surface area (MPAI and AOI, respectively) were computed. The PAP was obtained in all PPH patients and compared with caliber measurements. The PPH MPA (3.6 +/- 0.8 cm) was significantly larger than the control MPA (2.9 +/- 0.3 cm, p = 0.02); the PPH MPAI (2.8 +/- 0.7 cm/M2) was significantly greater than the control MPA (1.7 +/- 0.2 cm/M2, p < 0.0001). Control AO (2.2 +/- 0.3 cm) was significantly greater than PPH AO (1.6 +/- 0.4 cm, p < 0.0001); there was no significant difference between control AOI (1.3 +/- 0.2 cm/M2) and PPH AOI (1.2 +/- 0.2 cm/M2, p = 0.25). The PPH MPA/AO (2.3 +/- 0.6) was significantly greater than the control MPA/AO (1.3 +/- 0.1, p < 0.0001); overlap between MPA in the two groups was eliminated by indexing values to AO caliber (MPA/AO). Among PPH patients there was strong correlation between PAP and MPA/AO (PAP = 24 x MPA/AO + 3.7, r = 0.7, p < 0.01). Increased MPA/AO denotes the presence of pulmonary hypertension and may be used to estimate PAP.

  13. Do pulmonary artery catheters cause or increase tricuspid or pulmonic valvular regurgitation?

    Science.gov (United States)

    Sherman, S V; Wall, M H; Kennedy, D J; Brooker, R F; Butterworth, J

    2001-05-01

    There are few quantitative data on the extent or mechanism of pulmonary artery catheter (PAC)-induced valvular dysfunction. We hypothesized that PACs cause or worsen tricuspid and pulmonic valvular regurgitation, and tested this hypothesis by using transesophageal echocardiography. In 54 anesthetized adult patients, we measured color Doppler jet areas of tricuspid regurgitation (TR) in two planes (midesophageal [ME] 4-chamber and right ventricular inflow-outflow views) and pulmonic insufficiency (PI) in one plane (ME aortic valve long-axis view), both before and after we advanced a PAC into the pulmonary artery. Regurgitant jet areas and hemodynamic measurements were compared by using paired t-test. There were no significant changes in blood pressure or heart rate after passage of the PAC. After PAC placement, the mean PI jet area was not significantly increased. The mean TR jet area increased significantly in the right ventricular inflow-outflow view (+0.37 +/- 0.11 cm(2)) (P = 0.0014), but did not increase at the ME 4-chamber view. Seventeen percent of patients had an increase in TR jet area > or =1 cm(2); 8% of patients had an increase in PI jet area >/=1 cm(2). In patients without pulmonic or tricuspid valvular pathology, placement of a pulmonary artery catheter (PAC) worsened tricuspid regurgitation, which is consistently visualized in the right ventricular inflow-outflow view, and often not seen in the midesophageal 4-chamber view. This is consistent with malcoaptation of the anterior and posterior leaflets. PAC-induced pulmonic insufficiency was rarely detected in the midesophageal aortic valve long-axis view. We conclude that a PAC is very unlikely to be the sole cause of severe tricuspid regurgitation or pulmonic insufficiency.

  14. Low pulmonary artery flush perfusion pressure combined with high positive end-expiratory pressure reduces oedema formation in isolated porcine lungs

    International Nuclear Information System (INIS)

    Schumann, Stefan; Schließmann, Stephan J; Wagner, Giskard; Goebel, Ulrich; Priebe, Hans-Joachim; Guttmann, Josef; Kirschbaum, Andreas

    2010-01-01

    Flush perfusion of the pulmonary artery with organ protection solution is a standard procedure before lung explantation. However, rapid flush perfusion may cause pulmonary oedema which is deleterious in the lung transplantation setting. In this study we tested the hypotheses that high pulmonary perfusion pressure contributes to the development of pulmonary oedema and positive end-expiratory pressure (PEEP) counteracts oedema formation. We expected oedema formation to increase weight and decrease compliance of the lungs on the basis of a decrease in alveolar volume as fluid replaces alveolar air spaces. The pulmonary artery of 28 isolated porcine lungs was perfused with a low-potassium dextrane solution at low (mean 27 mmHg) or high (mean 40 mmHg) pulmonary artery pressure (PAP) during mechanical ventilation at low (4 cmH 2 O) or high (8 cmH 2 O) PEEP, respectively. Following perfusion and storage, relative increases in lung weight were smaller (p < 0.05) during perfusion at low PAP (62 ± 32% and 42 ± 26%, respectively) compared to perfusion at high PAP (133 ± 54% and 87 ± 30%, respectively). Compared to all other PAP–PEEP combinations, increases in lung weight were smallest (44 ± 9% and 27 ± 12%, respectively), nonlinear intratidal lung compliance was largest (46% and 17% respectively, both p < 0.05) and lung histology showed least infiltration of mononuclear cells in the alveolar septa, and least alveolar destruction during the combination of low perfusion pressure and high PEEP. The findings suggest that oedema formation during pulmonary artery flush perfusion in isolated and ventilated lungs can be reduced by choosing low perfusion pressure and high PEEP. PAP–PEEP titration to minimize pulmonary oedema should be based on lung mechanics and PAP monitoring

  15. Retrograde pulmonary arteriography

    International Nuclear Information System (INIS)

    Calcaterra, G.; Lam, J.; Losekoot, T.G.

    1984-01-01

    The authors performed retrograde pulmonary arteriography by means of a pulmonary venous wedge injection in 10 patients with no demonstrable intrapericardial pulmonary arteries by 'conventional' angiographic techniques. In all cases but one, the procedure demonstrated the feasibility of a further operation. No complications were observed. Retrograde pulmonary arteriography is an important additional method for determining the existence of surgically accessible pulmonary arteries when other techniques have failed. (Auth.)

  16. Pulmonary arterial hypertension in adult congenital heart disease.

    Science.gov (United States)

    Brida, Margarita; Gatzoulis, Michael A

    2018-05-02

    Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care. Patients with PAH-CHD and systemic to pulmonary shunting may benefit from late defect closure if pulmonary vascular resistance (PVR) is still normal or near normal. Patients with PAH and coincidental defects, or previous repair of CHD should be managed as those with idiopathic PAH. Patients with a Fontan circulation, despite not strictly fulfilling criteria for PAH, may have elevated PVR; recent evidence suggests that they may also benefit from DTT, but more data are required before general recommendations can be made. CHD-PAH is a lifelong, progressive disease; patients should receive tertiary care and benefit from a proactive DTT approach. Novel biomarkers and genetic advances may identify patients with CHD who should be referred for late defect closure and/or patients at high risk of developing PAH despite early closure in childhood. Ongoing vigilance for PAH and further controlled studies are clearly warranted in CHD. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  17. Extending the use of the pacing pulmonary artery catheter for safe minimally invasive cardiac surgery.

    Science.gov (United States)

    Levin, Ricardo; Leacche, Marzia; Petracek, Michael R; Deegan, Robert J; Eagle, Susan S; Thompson, Annemarie; Pretorius, Mias; Solenkova, Nataliya V; Umakanthan, Ramanan; Brewer, Zachary E; Byrne, John G

    2010-08-01

    In this study, the therapeutic use of pacing pulmonary artery catheters in association with minimally invasive cardiac surgery was evaluated. A retrospective study. A single institutional university hospital. Two hundred twenty-four consecutive patients undergoing minimally invasive cardiac surgery through a small (5-cm) right anterolateral thoracotomy using fibrillatory arrest without aortic cross-clamping. Two hundred eighteen patients underwent mitral valve surgery (97%) alone or in combination with other procedures. Six patients underwent other cardiac operations. In all patients, the pacing pulmonary artery catheter was used intraoperatively to induce ventricular fibrillation during the cooling period, and in the postoperative period it also was used in 37 (17%) patients who needed to be paced, mainly for bradyarrhythmias (51%). There were no complications related to the insertion of the catheters. Six (3%) patients experienced a loss of pacing capture, and 2 (1%) experienced another complication requiring the surgical removal of the catheter. Seven (3%) patients needed postoperative implantation of a permanent pacemaker. In combination with minimally invasive cardiac surgery, pacing pulmonary artery catheters were therapeutically useful to induce ventricular fibrillatory arrest intraoperatively and for obtaining pacing capability in the postoperative period. Their use was associated with a low number of complications. Copyright 2010 Elsevier Inc. All rights reserved.

  18. Left atrium and pulmonary artery compression due to aortic aneurysm causing heart failure symptoms.

    Science.gov (United States)

    Jorge, Antonio José Lagoeiro; Martins, Wolney de Andrade; Moutinho, Victor M; Rezende, Juliano M; Alves, Patricia Y; Villacorta, Humberto; Silveira, Pedro F; Couto, Antonio A

    2018-05-09

    Patients with thoracic aortic aneurysm (TAA) are mostly asymptomatic and TAA is rarely related to heart failure (HF). We report the case of an 80-year-old female patient, with type A TAA without dissection, with right pulmonary artery and left atrium compression, who presented with HF, preserved ejection fraction and acute pulmonary edema. Copyright © 2018 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  19. Ischemic preconditioning of the lower extremity attenuates the normal hypoxic increase in pulmonary artery systolic pressure.

    Science.gov (United States)

    Foster, Gary P; Westerdahl, Daniel E; Foster, Laura A; Hsu, Jeffrey V; Anholm, James D

    2011-12-15

    Ischemic pre-condition of an extremity (IPC) induces effects on local and remote tissues that are protective against ischemic injury. To test the effects of IPC on the normal hypoxic increase in pulmonary pressures and exercise performance, 8 amateur cyclists were evaluated under normoxia and hypoxia (13% F(I)O(2)) in a randomized cross-over trial. IPC was induced using an arterial occlusive cuff to one thigh for 5 min followed by deflation for 5 min for 4 cycles. In the control condition, the resting pulmonary artery systolic pressure (PASP) increased from a normoxic value of 25.6±2.3 mmHg to 41.8±7.2 mmHg following 90 min of hypoxia. In the IPC condition, the PASP increased to only 32.4±3.1 mmHg following hypoxia, representing a 72.8% attenuation (p=0.003). No significant difference was detected in cycle ergometer time trial duration between control and IPC conditions with either normoxia or hypoxia. IPC administered prior to hypoxic exposure was associated with profound attenuation of the normal hypoxic increase of pulmonary artery systolic pressure. Published by Elsevier B.V.

  20. Unilateral absence of lung perfusion resulting from endarteritis of the pulmonary artery

    International Nuclear Information System (INIS)

    Calegaro, J.U.M.; Turini, T.L.

    This case shows endarteritis of left pulmonary artery in the angiographic study with absent perfusion of the left lung in the MAA 131 I scintigraphy. Considerations are made conderning the presumable diagnosis of infection by 'toxocara canis' larval form. The literature review don't point out a similar case. (author) [pt

  1. Clinical use of extended-release oral treprostinil in the treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Pugliese SC

    2016-01-01

    Full Text Available Steven C Pugliese,1 Todd M Bull1,2 1Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, 2UCD Pulmonary Vascular Disease Center, Division of Pulmonary Sciences and Critical Care Medicine and Cardiology, Department of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA Abstract: The development of parenteral prostacyclin therapy marked a dramatic breakthrough in the treatment of pulmonary arterial hypertension (PAH. Intravenous (IV epoprostenol was the first PAH specific therapy and to date, remains the only treatment to demonstrate a mortality benefit. Because of the inherent complexities and risks of treating patients with continuous infusion IV therapy, there is great interest in the development of an oral prostacyclin analog that could mimic the benefits of IV therapy. Herein, we highlight the development of oral prostacyclin therapy, focusing on oral treprostinil, the only US Food and Drug Administration approved oral prostacyclin. Recent Phase III clinical trials have shown the drug to improve exercise tolerance in treatment-naïve PAH patients, but not patients on background oral therapy. Oral treprostinil appears to be most efficacious at higher doses, but its side effect profile and complexities with dosing complicate its use. While oral treprostinil’s current therapeutic role in PAH remains unclear, ongoing studies of this class of medication should help clarify their role in the treatment of PAH. Keywords: oral treprostinil, pulmonary arterial hypertension, selexipag

  2. Prognostic factors in pediatric pulmonary arterial hypertension : A systematic review and meta-analysis

    NARCIS (Netherlands)

    Ploegstra, Mark-Jan; Zijlstra, Willemijn M. H.; Douwes, Johannes M.; Hillege, Hans L.; Berger, Rolf M. F.

    2015-01-01

    BACKGROUND: Despite the introduction of targeted therapies in pediatric pulmonary arterial hypertension (PAH), prognosis remains poor. For the definition of treatment strategies and guidelines, there is a high need for an evidence-based recapitulation of prognostic factors. The aim of this study was

  3. “Denervation” of autonomous nervous system in idiopathic pulmonary arterial hypertension by low-dose radiation: a case report with an unexpected outcome

    Directory of Open Access Journals (Sweden)

    Hohenforst-Schmidt W

    2014-03-01

    Full Text Available Wolfgang Hohenforst-Schmidt,1 Paul Zarogoulidis,2 Filiz Oezkan,3 Christian Mahnkopf,4 Gerhard Grabenbauer,5 Alfons Kreczy,6 Rolf Bartunek,7 Kaid Darwiche,3 Lutz Freitag,3 Qiang Li,8 Haidong Huang,8 Thomas Vogl,9 Patrick LePilvert,10 Theodora Tsiouda,11 Kosmas Tsakiridis,12 Konstantinos Zarogoulidis,2 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Würzburg, Coburg, Germany; 2Pulmonary Department-Oncology Unit, G Papanikolaou General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 3Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 4II Medizinische Klinik, Klinik für Kardiologie, Angiologie, Pneumologie, Klinikum Coburg, 5Department of Radiotherapy, 6Department of Pathology, Cytology and Molecular Diagnostics, 7Institute of Diagnostic and Interventional Radiology, Coburg Clinic, University of Wüerzburg, Coburg, Germany; 8Department of Respiratory Diseases, Changhai Hospital/First Affiliated Hospital of the Second Military Medical University, Shanghai, People’s Republic of China; 9Department of Diagnostic and Interventional Radiology, Goethe University of Frankfurt, Frankfurt, Germany; 10Interventional Drug Delivery Systems and Strategies (ID2S2, Medical Cryogenics, Lakeland Court Jupiter, FL, USA; 11Internal Medicine Unit, Theagenio Cancer Hospital, Thessaloniki, 12Cardiothoracic Surgery Department, Saint Luke Private Hospital, Thessaloniki, GreeceAbstract: Vasointestinal peptide metabolism plays a key physiological role in multimodular levels of vasodilatory, smooth muscle cell proliferative, parenchymal, and inflammatory lung reactions. In animal studies, vasointestinal peptide relaxes isolated pulmonary arterial segments from several mammalian species in vitro and neutralizes the pulmonary vasoconstrictor effect of endothelin. In some animal models, it reduces pulmonary vascular resistance in vivo and

  4. Correlation of 64 row MDCT, echocardiography and cardiac catheterization angiography in assessment of pulmonary arterial anatomy in children with cyanotic congenital heart disease

    International Nuclear Information System (INIS)

    Chandrashekhar, Guruprasadh; Sodhi, Kushaljit Singh; Saxena, Akshay Kumar; Rohit, Manoj Kumar; Khandelwal, Niranjan

    2012-01-01

    Objective: To study the correlation of low-dose 64-row multi-detector computed tomography (MDCT) with echocardiography and cardiac catheterization angiography (CCA) in the assessment of pulmonary arterial anatomy in children with cyanotic congenital heart disease (CCHD). Materials and methods: This prospective study included 105 children (74 males, 31 females) with CCHD, in the age group of 2 months to 20 years, who underwent 64-row MDCT examination (low-dose CT protocol), echocardiography and CCA for the assessment of pulmonary arteries, including visualization, presence of confluence, stenosis and collaterals. Statistical analysis was performed using the non-parametric statistical analysis test to evaluate the concordance or discordance between echocardiography, MDCT and CCA. Results: 64-row MDCT detected significantly more main and branch pulmonary arteries, patent pulmonary confluences, and more cases of pulmonary artery stenosis. CCA detected more major aorto-pulmonary collaterals than MDCT, whereas echocardiography failed to identify these major aorto-pulmonary collaterals. The effective CT radiation dose to patients less than 2 years of age was in the range of 0.7–2.5 mSv, where as the dose in patients more than 2 years of age ranged from that of 2.1 to 4.2 mSv, which is much less than the radiation dose reported in cardiac catheterization angiography. Conclusion: In cases where cardiac MRI cannot be performed, or is not sufficiently informative, low-dose 64-row MDCT correlates well with CCA and can provide adequate information about pulmonary arterial anatomy in children with cyanotic congenital heart disease, and can replace invasive cardiac catheterization angiography with markedly reduced radiation dosage to the patient.

  5. Depression in pulmonary arterial hypertension: An undertreated comorbidity

    Directory of Open Access Journals (Sweden)

    Sameer Verma

    2016-01-01

    Full Text Available Pulmonary arterial hypertension (PAH is a debilitating condition leading to progressive decline in functional capacity. As a result, PAH can lead to psychological impairment that can impact the overall disease status. The medical community has developed several screening questionnaires in order to assess depression in their patients allowing physicians to be at the forefront of recognizing clinical depression. There is a suggestion that depression symptomatology is more prevalent in the PAH population. The aim of this article is to review the current thought process about diagnosis and management of depression in PAH patients.

  6. MicroRNA-143 Activation Regulates Smooth Muscle and Endothelial Cell Crosstalk in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Deng, Lin; Blanco, Francisco J; Stevens, Hannah; Lu, Ruifang; Caudrillier, Axelle; McBride, Martin; McClure, John D; Grant, Jenny; Thomas, Matthew; Frid, Maria; Stenmark, Kurt; White, Kevin; Seto, Anita G; Morrell, Nicholas W; Bradshaw, Angela C; MacLean, Margaret R; Baker, Andrew H

    2015-10-23

    The pathogenesis of pulmonary arterial hypertension (PAH) remains unclear. The 4 microRNAs representing the miR-143 and miR-145 stem loops are genomically clustered. To elucidate the transcriptional regulation of the miR-143/145 cluster and the role of miR-143 in PAH. We identified the promoter region that regulates miR-143/145 microRNA expression in pulmonary artery smooth muscle cells (PASMCs). We mapped PAH-related signaling pathways, including estrogen receptor, liver X factor/retinoic X receptor, transforming growth factor-β (Smads), and hypoxia (hypoxia response element), that regulated levels of all pri-miR stem loop transcription and resulting microRNA expression. We observed that miR-143-3p is selectively upregulated compared with miR-143-5p during PASMC migration. Modulation of miR-143 in PASMCs significantly altered cell migration and apoptosis. In addition, we found high abundance of miR-143-3p in PASMC-derived exosomes. Using assays with pulmonary arterial endothelial cells, we demonstrated a paracrine promigratory and proangiogenic effect of miR-143-3p-enriched exosomes from PASMC. Quantitative polymerase chain reaction and in situ hybridization showed elevated expression of miR-143 in calf models of PAH and in samples from PAH patients. Moreover, in contrast to our previous findings that had not supported a therapeutic role in vivo, we now demonstrate a protective role of miR-143 in experimental pulmonary hypertension in vivo in miR-143-/- and anti-miR-143-3p-treated mice exposed to chronic hypoxia in both preventative and reversal settings. MiR-143-3p modulated both cellular and exosome-mediated responses in pulmonary vascular cells, whereas inhibition of miR-143-3p blocked experimental pulmonary hypertension. Taken together, these findings confirm an important role for the miR-143/145 cluster in PAH pathobiology. © 2015 American Heart Association, Inc.

  7. Intravascular pulmonary metastases

    International Nuclear Information System (INIS)

    Shepard, J.A.O.; Moore, E.H.; Templeton, P.A.; McLoud, T.C.

    1988-01-01

    The diagnosis of intravascular metastatic tumor emboli to the lungs is rarely made. The authors present a characteristic radiographic finding of intravascular lung metastases that they observed in four patients with diagnoses or right atrial myoxoma, invasive renal cell carcinoma, invasive pelvic osteosarcoma, and recurrent pelvic chondrosarcoma. Substantiation of intravascular pulmonary metastases was achieved by means of autopsy, pulmonary artery biopsy, and surgical documentation of tumor invasion of the inferior vena cava or pelvic veins. In all four cases, chest computed tomography (CT) demonstrated branching, beaded opacities extending from the hila into the periphery of the lung in the distribution of pulmonary arteries. In one case, similar findings were observed in magnetic resonance (MR) images of the chest. Follow-up studies in three cases showed progressive enlargement and varicosity of the abnormal pulmonary artery consistent with proliferation of intravascular tumor. In the case of metastatic osteosarcoma, intraluminal ossification was also observed at CT. In three of four cases, pulmonary infarction was demonstrated in the distribution of the abnormal pulmonary arteries seen at CT as small, peripheral, wedge-shaped opacities. The demonstration of progressively dilated and beaded pulmonary arteries in patients with extrathoracic malignancies is suggestive of intravascular lung metastases, particularly when accompanied by peripheral infarction

  8. Isorhynchophylline protects against pulmonary arterial hypertension and suppresses PASMCs proliferation

    International Nuclear Information System (INIS)

    Guo, Haipeng; Zhang, Xin; Cui, Yuqian; Deng, Wei; Xu, Dachun; Han, Hui; Wang, Hao; Chen, Yuguo; Li, Yu; Wu, Dawei

    2014-01-01

    Highlights: • We focus on PASMCs proliferation in the pathogenesis of PAH. • Isorhynchophylline inhibited PASMCs proliferation and alleviated PAH. • IRN blocked PDGF-Rβ phosphorylation and its downstream signal transduction. • IRN regulated cyclins and CDKs to arrest cell cycle in the G0/G1 phase. • We reported IRN has the potential to be a candidate for PAH treatment. - Abstract: Increased pulmonary arterial smooth muscle cells (PASMCs) proliferation is a key pathophysiological component of pulmonary vascular remodeling in pulmonary arterial hypertension (PAH). Isorhynchophylline (IRN) is a tetracyclic oxindole alkaloid isolated from the Chinese herbal medicine Uncaria rhynchophylla. It has long been used clinically for treatment of cardiovascular and cerebrovascular diseases. However, very little is known about whether IRN can influence the development of PAH. Here we examined the effect of IRN on monocrotaline (MCT) induced PAH in rats. Our data demonstrated that IRN prevented MCT induced PAH in rats, as assessed by right ventricular (RV) pressure, the weight ratio of RV to (left ventricular + septum) and RV hypertrophy. IRN significantly attenuated the percentage of fully muscularized small arterioles, the medial wall thickness, and the expression of smooth muscle α-actin (α-SMA) and proliferating cell nuclear antigen (PCNA). In vitro studies, IRN concentration-dependently inhibited the platelet-derived growth factor (PDGF)-BB-induced proliferation of PASMCs. Fluorescence-activated cell-sorting analysis showed that IRN caused G0/G1 phase cell cycle arrest. IRN-induced growth inhibition was associated with downregulation of Cyclin D1 and CDK6 as well as an increase in p27Kip1 levels in PDGF-BB-stimulated PASMCs. Moreover, IRN negatively modulated PDGF-BB-induced phosphorylation of PDGF-Rβ, ERK1/2, Akt/GSK3β, and signal transducers and activators of transcription 3 (STAT3). These results demonstrate that IRN could inhibit PASMCs proliferation and

  9. Isorhynchophylline protects against pulmonary arterial hypertension and suppresses PASMCs proliferation

    Energy Technology Data Exchange (ETDEWEB)

    Guo, Haipeng; Zhang, Xin [Department of Critical Care Medicine, Qilu Hospital of Shandong University, Jinan 250012 (China); Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Cui, Yuqian [Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Deng, Wei [Department of Cardiology, Renmin Hospital of Wuhan University, Wuhan 430060 (China); Xu, Dachun [Department of Cardiology, Shanghai Tenth People’s Hospital of Tongji University, Shanghai 200072 (China); Han, Hui; Wang, Hao [Department of Critical Care Medicine, Qilu Hospital of Shandong University, Jinan 250012 (China); Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Chen, Yuguo [Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China); Li, Yu, E-mail: qlliyu@126.com [Department of Respiratory, Qilu Hospital of Shandong University, Jinan 250012 (China); Wu, Dawei, E-mail: wdwu55@163.com [Department of Critical Care Medicine, Qilu Hospital of Shandong University, Jinan 250012 (China); Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education and Chinese Ministry of Health, Qilu Hospital of Shandong University, Jinan 250012 (China)

    2014-07-18

    Highlights: • We focus on PASMCs proliferation in the pathogenesis of PAH. • Isorhynchophylline inhibited PASMCs proliferation and alleviated PAH. • IRN blocked PDGF-Rβ phosphorylation and its downstream signal transduction. • IRN regulated cyclins and CDKs to arrest cell cycle in the G0/G1 phase. • We reported IRN has the potential to be a candidate for PAH treatment. - Abstract: Increased pulmonary arterial smooth muscle cells (PASMCs) proliferation is a key pathophysiological component of pulmonary vascular remodeling in pulmonary arterial hypertension (PAH). Isorhynchophylline (IRN) is a tetracyclic oxindole alkaloid isolated from the Chinese herbal medicine Uncaria rhynchophylla. It has long been used clinically for treatment of cardiovascular and cerebrovascular diseases. However, very little is known about whether IRN can influence the development of PAH. Here we examined the effect of IRN on monocrotaline (MCT) induced PAH in rats. Our data demonstrated that IRN prevented MCT induced PAH in rats, as assessed by right ventricular (RV) pressure, the weight ratio of RV to (left ventricular + septum) and RV hypertrophy. IRN significantly attenuated the percentage of fully muscularized small arterioles, the medial wall thickness, and the expression of smooth muscle α-actin (α-SMA) and proliferating cell nuclear antigen (PCNA). In vitro studies, IRN concentration-dependently inhibited the platelet-derived growth factor (PDGF)-BB-induced proliferation of PASMCs. Fluorescence-activated cell-sorting analysis showed that IRN caused G0/G1 phase cell cycle arrest. IRN-induced growth inhibition was associated with downregulation of Cyclin D1 and CDK6 as well as an increase in p27Kip1 levels in PDGF-BB-stimulated PASMCs. Moreover, IRN negatively modulated PDGF-BB-induced phosphorylation of PDGF-Rβ, ERK1/2, Akt/GSK3β, and signal transducers and activators of transcription 3 (STAT3). These results demonstrate that IRN could inhibit PASMCs proliferation and

  10. Aortopulmonary collateral flow is related to pulmonary artery size and affects ventricular dimensions in patients after the fontan procedure.

    Directory of Open Access Journals (Sweden)

    Heiner Latus

    Full Text Available BACKGROUND: Aortopulmonary collaterals (APCs are frequently found in patients with a single-ventricle (SV circulation. However, knowledge about the clinical significance of the systemic-to-pulmonary shunt flow in patients after the modified Fontan procedure and its potential causes is limited. Accordingly, the aim of our study was to detect and quantify APC flow using cardiovascular magnetic resonance (CMR and assess its impact on SV volume and function as well as to evaluate the role of the size of the pulmonary arteries in regard to the development of APCs. METHODS: 60 patients (mean age 13.3 ± 6.8 years after the Fontan procedure without patent tunnel fenestration underwent CMR as part of their routine clinical assessment that included ventricular functional analysis and flow measurements in the inferior vena cava (IVC, superior vena cava (SVC and ascending aorta (Ao. APC flow was quantified using the systemic flow estimator: (Ao - (IVC + SVC. Pulmonary artery index (Nakata index was calculated as RPA + LPA area/body surface area using contrast enhanced MR angiography. The patient cohort was divided into two groups according to the median APC flow: group 1 0.495 l/min/m(2. RESULTS: Group 1 patients had significant smaller SV enddiastolic (71 ± 16 vs 87 ± 25 ml/m(2; p=0.004 and endsystolic volumes (29 ± 11 vs 40 ± 21 ml/m(2; p=0.02 whereas ejection fraction (59 ± 9 vs 56 ± 13%; p=0.38 differed not significantly. Interestingly, pulmonary artery size showed a significant inverse correlation with APC flow (r=-0.50, p=0.002. CONCLUSIONS: Volume load due to APC flow in Fontan patients affected SV dimensions, but did not result in an impairment of SV function. APC flow was related to small pulmonary artery size, suggesting that small pulmonary arteries represent a potential stimulus for the development of APCs.

  11. Relationship between interatrial communication, ductus arteriosus, and pulmonary flow patterns in fetuses with transposition of the great arteries: prediction of neonatal desaturation.

    Science.gov (United States)

    Vaujois, Laurence; Boucoiran, Isabelle; Preuss, Christophe; Brassard, Myriam; Houde, Christine; Fouron, Jean C; Raboisson, Marie-Josée

    2017-09-01

    The relationship between interatrial communication, ductus arteriosus, and pulmonary flow in transposition of the great arteries and intact ventricular septum may help predict postnatal desaturation. Echocardiographic data of 45 fetuses with transposition of the great arteries and intact ventricular septum and 50 age-matched controls were retrospectively reviewed. Interatrial communication, left and right ventricular output, flow in the ductus arteriosus, as well as effective pulmonary flow were measured. Patients were divided into two groups on the basis of postnatal saturations: group 1 had saturations ⩽50% and group 2 >50%. Of 45 fetuses, 13 (26.7%) were classified into group 1. Compared with fetuses in group 2, they had a smaller interatrial communication (2.9 versus 4.0 mm, p=0.004) and more retrograde diastolic flow in the ductus arteriosus (92 versus 23%, p=0.002). Both groups showed a significant decrease in ductal flow compared with controls. Patients in group 2 had a higher effective pulmonary flow compared with controls. There was a mild correlation between left ventricular output and size of the interatrial communication (Spearman's rank correlation 0.44). A retrograde diastolic flow is present in most of the fetuses with postnatal desaturation. Fetuses with transposition of the great arteries have a lower flow through the ductus arteriosus compared with controls. Fetuses without restrictive foramen ovale have higher effective pulmonary flow. Peripheral pulmonary vasodilatation due to higher oxygen saturation in pulmonary arteries in the case of transposition of the great arteries could be one possible cause.

  12. Evaluation of C-reactive protein as a clinical biomarker in naturally heartworm-infected dogs: a field study.

    Science.gov (United States)

    Venco, Luigi; Bertazzolo, Walter; Giordano, Guglielmo; Paltrinieri, Saverio

    2014-11-15

    Canine heartworm disease caused by Dirofilaria immitis is considered a pulmonary disease, which leads to pulmonary hypertension, and in the late stage, may induce right cardiac insufficiency. Adult worms are localized in the pulmonary arteries, which undergo endothelial damage (proliferative endoarteritis), the severity of which depends on the duration of infection and the worm burden. C-reactive protein (CRP) is a major canine acute-phase protein that rapidly increases in a wide range of inflammatory conditions and rapidly decreases when inflammation resolves. CRP is therefore considered a sensitive but nonspecific marker of inflammation. Pulmonary arterial damage in canine heartworm may induce an increase in CRP concentrations similar to what occurs in humans with endoarteritis. The aim of the present study was to investigate whether CRP may be a diagnostic and/or prognostic marker in canine heartworm, whether it may be used for staging and monitoring canine heartworm, and whether its concentration depends on worm burden or on pulmonary arterial damage. Serum CRP concentrations were determined in 57 dogs with heartworm disease, 47 of which were grouped according to parasite burden (low: n=11; high: n=10) or on severity of pulmonary hypertension (mild: n=16; severe: n=10). An additional 23 heartworm-free cardiopathic dogs were grouped on the absence of pulmonary hypertension (n=8), presence of dilated cardiomyopathy (DCM) (n=6), or presence of cardiomyopathy and pulmonary hypertension (n=3) due to previous heartworm disease that had been treated (n=6). Twenty control dogs also were sampled for CRP concentrations. Results show that CRP was significantly increased (pcardiomyopathy compared with concentrations in controls. In the heartworm group, CRP was significantly increased (p29.8 mg/L). In conclusion, CRP can be used as a marker of endothelial arteritis and pulmonary hypertension in dogs with heartworm. Copyright © 2014 Elsevier B.V. All rights reserved.

  13. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

    Directory of Open Access Journals (Sweden)

    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  14. Covered Stent and Coils Embolization of a Pulmonary Artery Pseudoaneurysm After Gunshot Wound

    International Nuclear Information System (INIS)

    Huet, Nicolas; Rodiere, Mathieu; Badet, Michel; Michoud, Marie; Brichon, Pierre-Yves; Ferretti, Gilbert; Thony, Frédéric

    2016-01-01

    We report the first case of endovascular covered stent implantation for the treatment of a large pulmonary artery pseudoaneurysm (PAPA) following a right thoracic gunshot wound. After resuscitation and hemodynamic stabilization, a CT angiography was performed to analyze the neck size of the PAPA and its position relative to the branches of the parent artery. Covered stent implantation with additional coil embolization was successfully performed. At the 4-year follow-up, the stents remained patent and there was neither pseudoaneurysm recurrence nor treatment-related complication.

  15. Covered Stent and Coils Embolization of a Pulmonary Artery Pseudoaneurysm After Gunshot Wound

    Energy Technology Data Exchange (ETDEWEB)

    Huet, Nicolas, E-mail: nhuet@chu-grenoble.fr; Rodiere, Mathieu, E-mail: mrodiere@chu-grenoble.fr [Hôpital Universitaire de Grenoble and Université Grenoble Alpes, Department of Radiology and Medical Imaging (France); Badet, Michel, E-mail: michel.badet@ch-chambery.fr [Centre Hospitalier Métropôle Savoie, site de Chambéry, Intensive Care Unit (France); Michoud, Marie, E-mail: marie.michoud@ch-chambery.fr [Centre Hospitalier Métropôle Savoie, Site de Chambéry, Department of Radiology (France); Brichon, Pierre-Yves, E-mail: pybrichon@chu-grenoble.fr [Hôpital Universitaire de Grenoble and Université Grenoble Alpes, Department of Thoracic and Vascular Surgery (France); Ferretti, Gilbert, E-mail: gferretti@chu-grenoble.fr; Thony, Frédéric, E-mail: fthony@chu-grenoble.fr [Hôpital Universitaire de Grenoble and Université Grenoble Alpes, Department of Radiology and Medical Imaging (France)

    2016-05-15

    We report the first case of endovascular covered stent implantation for the treatment of a large pulmonary artery pseudoaneurysm (PAPA) following a right thoracic gunshot wound. After resuscitation and hemodynamic stabilization, a CT angiography was performed to analyze the neck size of the PAPA and its position relative to the branches of the parent artery. Covered stent implantation with additional coil embolization was successfully performed. At the 4-year follow-up, the stents remained patent and there was neither pseudoaneurysm recurrence nor treatment-related complication.

  16. Pulmonary artery and right ventricle assessment in pulmonary hypertension. Correlation between functional parameters of ECG-gated CT and right-side heart catheterization

    International Nuclear Information System (INIS)

    Abel, Elodie; Jankowski, Adrien; Pison, Christophe; Bosson, Jean Luc; Bouvaist, Helene; Ferretti, Gilbert R.

    2012-01-01

    Background: Right ventricular function predicts outcome in patients with pulmonary hypertension (PH). Therefore accurate assessment of right ventricular function is essential to graduate severity, assess follow-up, and response to therapy. Purpose: To evaluate whether PH severity could be assessed using electrocardiography-gated CT (ECG-gated CT) functional parameters. A further objective was to evaluate cardiac output (CO) using two ECG-gated CT methods: the reference Simpson technique and the fully automatic technique generated by commercially available cardiac software. Material and Methods: Our institutional review board approved this study; patient consent was not required. Twenty-seven patients who had undergone ECG-gated CT and right heart catheterization (RHC) were included. Two independent observers measured pulmonary artery (PA) diameter, PA distensibility, aorta diameter, right ventricular cardiac output (CT-RVCO) and right ventricular ejection fraction (CT-RVEF) with automatic and Simpson techniques on ECG-gated CT. RHC-CO and mean pulmonary arterial pressure (mPAP) were measured on RHC. Relationship between ECG-gated CT and RHC measurements was tested with linear regression analysis. Results: Inter-observer agreement was good for all measurements (r > 0.7) except for CT-RVCO calculated with Simpson's technique (r = 0.63). Pulmonary artery (PA) distensibility was significantly correlated to mPAP (r = -0.426, P = 0.027). CT-RVEF was correlated with mPAP only when issued from Simpson technique (r = -0.417, P = 0.034). CT-RVEF was not significantly correlated to RHC-CO (P > 0.2). CT-RVCO measured with Simpson technique (r = 0.487, P = 0.010) and automatic segmentation (r = 0.549, P 0.005) correlated equally with RHC-CO. Conclusion: CT-RVEF and CT-RVCO measured on ECG-gated CT are significantly correlated, respectively, to mPAP and RHC-CO in this population with severe reduction of the right ventricular ejection fraction and could be useful for evaluating

  17. Ginsenoside Rb1 Attenuates Agonist-Induced Contractile Response via Inhibition of Store-Operated Calcium Entry in Pulmonary Arteries of Normal and Pulmonary Hypertensive Rats

    Directory of Open Access Journals (Sweden)

    Rui-Xing Wang

    2015-03-01

    Full Text Available Background: Pulmonary hypertension (PH is characterized by sustained vasoconstriction, enhanced vasoreactivity and vascular remodeling, which leads to right heart failure and death. Despite several treatments are available, many forms of PH are still incurable. Ginsenoside Rb1, a principle active ingredient of Panax ginseng, exhibits multiple pharmacological effects on cardiovascular system, and suppresses monocrotaline (MCT-induced right heart hypertrophy. However, its effect on the pulmonary vascular functions related to PH is unknown. Methods: We examined the vasorelaxing effects of ginsenoside Rb1 on endothelin-1 (ET-1 induced contraction of pulmonary arteries (PAs and store-operated Ca2+ entry (SOCE in pulmonary arterial smooth muscle cells (PASMCs from chronic hypoxia (CH and MCT-induced PH. Results: Ginsenoside Rb1 elicited concentration-dependent relaxation of ET-1-induced PA contraction. The vasorelaxing effect was unaffected by nifedipine, but abolished by the SOCE blocker Gd3+. Ginsenoside Rb1 suppressed cyclopiazonic acid (CPA-induced PA contraction, and CPA-activated cation entry and Ca2+ transient in PASMCs. ET-1 and CPA-induced contraction, and CPA-activated cation entry and Ca2+ transients were enhanced in PA and PASMCs of CH and MCT-treated rats; the enhanced responses were abolished by ginsenoside Rb1. Conclusion: Ginsenoside Rb1 attenuates ET-1-induced contractile response via inhibition of SOCE, and it can effectively antagonize the enhanced pulmonary vasoreactivity in PH.

  18. [Pulmonary artery pressure evaluation in adults by Doppler echocardiography].

    Science.gov (United States)

    Campos Filho, O; Andrade, J L; Carvalho, A C; Luna Filho, B; Pfeferman, A; Arroyo, J B; Leão, L E; Martinez Filho, E E

    1991-04-01

    To assess the role of pulsed Doppler echocardiography (PDE) in the indirect assessment of pulmonary artery (PA) pressure (P), analysing the pulmonary velocity blood flow curves (PVBFC) profile. Sixty-one adults with several kinds of heart disease were submitted to heart catheterization to obtain PAP (systolic, diastolic, mean), and other hemodynamic variables. A PDE examination was performed in all to obtain the PVBFC at the level of the pulmonic annulus. Qualitative features of the curve were analysed (morphological pattern, presence of pulmonic regurgitation) as well as quantitative data (acceleration time = AT, right ventricle ejection time = RVET index, AT/RVET index AT corrected for heart rate = ATC), which were compared to the invasive measurements. An abnormal rapid acceleration of the PVBFC, with triangular configuration, was noted in patient with pulmonary hypertension (PH), in contrast to the dome-like shape of the PVBFC in normal PAP. Pulmonary regurgitation was more frequent (p less than 0.05) in patients with severe PH (mean PAP greater than or equal to 40 mmHg), comparing with patients with PAP less than 40 mmHg. Inverse linear correlations were observed between AT and mean PAP, particularly when sinus rhythm was present (r = 0.89; p less than 0.05) excluding patients with atrial fibrilation (19 cases). PDE is an useful and noninvasive method for indirect evaluation of PAP in adults, especially during stable sinus rhythm, in heart rate range from 60 to 115 bpm.

  19. Central extracorporeal membrane oxygenation requiring pulmonary arterial venting after near-drowning.

    Science.gov (United States)

    Kimura, Mitsutoshi; Kinoshita, Osamu; Fujimoto, Yoshifumi; Murakami, Arata; Shindo, Takahiro; Kashiwa, Koichi; Ono, Minoru

    2014-02-01

    Extracorporeal membrane oxygenation (ECMO) is an effective respiratory and circulatory support in patients in refractory cardiogenic shock or cardiac arrest. Peripheral ECMO sometimes requires left heart drainage; however, few reports state that pulmonary arterial (PA) venting is required during ECMO support. We present a case of a 14-year-old boy who required PA venting during ECMO support after resuscitation from near-drowning in freshwater. A biventricular assist device with an oxygenator implantation was intended on day 1; however, we were unable to proceed because of increasing of pulmonary vascular resistance from the acute lung injury. Central ECMO with PA venting was then performed. On day 13, central ECMO was converted to biventricular assist device with an oxygenator, which was removed on day 16. This case suggests that PA venting during ECMO support may be necessary in some cases of respiratory and circulatory failure with high pulmonary vascular resistance after near-drowning.

  20. Postoperative pulmonary and aortic 3D haemodynamics in patients after repair of transposition of the great arteries

    Energy Technology Data Exchange (ETDEWEB)

    Geiger, Julia; Buerk, Jonas; Jung, Bernd; Langer, Mathias [University Medical Center Freiburg, Department of Radiology, Medical Physics, Freiburg (Germany); Hirtler, Daniel; Stiller, Brigitte [University Medical Center Freiburg-Bad Krozingen, Department of Congenital Heart Disease and Paediatric Cardiology, Freiburg (Germany); Arnold, Raoul [University Medical Center Heidelberg, Department of Congenital Heart Disease and Paediatric Cardiology, Heidelberg (Germany); Markl, Michael [Northwestern University, Department of Radiology, Feinberg School of Medicine, Chicago (United States); Northwestern University, Department Biomedical Engineering, McCormick School of Engineering, Chicago (United States)

    2014-01-15

    To characterise aortic and pulmonary haemodynamics and investigate the correlation with post-surgical anatomy in patients with dextro-transposition of the great arteries (d-TGA). Four-dimensional (4D) MRI was performed in 17 patients after switch repair of TGA and 12 healthy controls (age, 11.9 ± 5.4 vs 23.3 ± 1.6 years). Patients were divided according to the pulmonary trunk (TP) position in relation to the ascending aorta (AAo): anterior (n = 10) and right/left anterior position (n = 7). Analysis included visual grading (ranking 0-2) of pulmonary and aortic vortical and helical flow, flow velocity quantification, blood-flow distribution to the right and left pulmonary arteries (flow ratio rPA:lPA), and vessel lumen areas. Anterior TP position was associated with increased vortices in six out of ten patients compared with right anterior TP position (one out of seven) and controls (none). Reduced systolic lPA and TP lumina in patients resulted in significantly increased peak systolic velocities (P < 0.001). Flow ratio rPA:lPA was more heterogeneous in patients (rPA:lPA = 1.56 ± 0.78 vs volunteers 1.09 ± 0.15; P < 0.05) with predominant flow to the rPA. Eleven patients presented increased helices in the AAo (grade 1.6). Evaluation of post-surgical haemodynamics in TGA patients revealed increased vortical flow for anterior TP position, asymmetric flow and increased systolic flow velocity in the pulmonary arteries owing to reduced vascular lumina. (orig.)

  1. [POL-PAVTI--Polish report on transcatheter pulmonary artery valve implantation of Melody-Medtronic prosthesis in the first 14 patients in Poland].

    Science.gov (United States)

    Ruzyłło, Witold; Demkow, Marcin; Włodarska, Elzbieta K; Kowalski, Mirosław; Spiewak, Mateusz; Siudalska, Hanna; Wolski, Piotr; Miśko, Jolanta; Hoffman, Piotr; Kusa, Jacek; Szkutnik, Małgorzata; Białkowski, Jacek; Fiszer, Roland; Urbańska, Ewa; Sondergaard, Lars

    2009-10-01

    To assess the early results of the pulmonary artery valve transcatheter implantation (PAVTI) in pts included into POL-PAVTI registry. Detailed medical and economic analyses were performed. Pulmonary artery valve implantation was performed in 14 pts (9 men), aged 16-31 (mean 24.6 +/- 4.8) years, with pulmonary homograft dysfunction after total repair of tetralogy of Fallot (4 pts), pulmonary atresia (2 pts), pulmonary stenosis (1 pt), common arterial trunk type I (1 pt), Ross procedure (3 pts) and TGA - Rastelli operation (3 pts). Eleven pts underwent in the past 2-5 surgical or/and catheter interventions. Indication for PAVTI was based on clinical evaluation and echocardiographic studies. Assessment of morphological and functional features of the right ventricle (RV) and homograft with the use of cardiac magnetic resonance (CMR) was performed in 10 cases. Pulmonary stenosis (max. pulmonary gradient 32-119, mean 72 +/- 28 mmHg) was observed in 13 pts and/or significant pulmonary regurgitation in 10 pts. The procedure was performed in general anesthesia. The deployment of a valved stent in the pulmonary valve position was preceded by a metal stent implantation. Results were evaluated by echocardiography two days after the procedure and one month later. Four patients were evaluated 6 months after procedure. Time of the procedure varied 60-190 (mean 127 +/- 35) min, time of fluoroscopy ranged 12-31 (mean 21 +/- 11) min. PAVTI was successfully performed in all pts without serious complications. Patients were discharged from the hospital 48-293 (mean 120 +/- 71) h after procedure. Significant reduction of pulmonary gradient after the procedure assessed by echocardiography was observed on the second day (20-60, mean 38 +/- 12 mmHg, p < 0.0001) and one month (19-52, mean 34 +/- 9 mmHg, p < 0.0001). Mild pulmonary regurgitation was observed in 2 pts. In 5 pts evaluated 6 months after procedure haemodynamic parameters were unchanged; no late complications were observed

  2. Pulmonary artery closure in combination with patch technique for treating congenital heart disease combined with large patent ductus arteriosus: A clinical study of 9 cases.

    Science.gov (United States)

    Wen, Bing; Yang, Junya; Liu, Huiruo; Jiao, Zhouyang; Zhao, Wenzeng

    2016-01-01

    To document clinical experience of treating congenital heart disease combined with large patent ductus arteriosus with pulmonary artery closure in combination with patch technique. Thirty-six patients (8 males and 28 females) who suffered from congenital heart disease and underwent hybrid surgery in the First Affiliated Hospital of Zhengzhou University from October 2010 to February 2014 were selected for this study. They aged 14 to 39 years and weighed 32.20 to 61.50 kg. Diameter of arterial duct was between 10 mm and 13 mm; 28 cases were tube type, 4 cases were funnel type and four cases were window type. All patients had moderate or severe pulmonary arterial hypertension; besides, there were 28 cases of ventricular septal defect, 16 cases of atrial septal defect, eight cases of aortic insufficiency, four cases of mitral stenosis and insufficiency and four cases of infectious endocarditis. Cardz Pulmonary Bypass (CPB) was established after chest was opened along the middle line. With the help of Transesophageal echocardiography, large patent ductus arteriosus was blocked off through pulmonary artery. Pulmonary artery was cut apart after blocking of heart. Large patent ductus arteriosus on the side of pulmonary artery was strengthened with autologous pericardial patch. Of 36 patients, 32 patients had patent ductus arteriosus closure device and four patients had atrial septal defect closure device. Pulmonary arteries of 36 cases were all successfully closed. Systolic pressure declined after closure ((54.86±19.23) mmHg vs (96.05±23.07) mmHg, pclosure ((39.15±14.83) mmHg vs (72.88±15.76) mmHg, ppatent ductus arteriosus and congenital heart disease, which decreases surgical problems, shortens surgical time and lowers the incidence of complications.

  3. Mortality in pulmonary arterial hypertension due to congenital heart disease: Serial changes improve prognostication

    NARCIS (Netherlands)

    Schuijt, M.T.U.; Blok, I.M.; Zwinderman, A.H.; Riel, A. van; Schuuring, M.J.; Winter, R.J. de; Duijnhouwer, A.L.; Dijk, A.P.J. van; Mulder, B.J.; Bouma, B.J.

    2017-01-01

    BACKGROUND: Adult patients with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) suffer from high mortality. This underlines the importance of adequate risk stratification to guide treatment decisions. Several baseline parameters are associated with mortality, however, their

  4. Mortality in pulmonary arterial hypertension due to congenital heart disease: Serial changes improve prognostication

    NARCIS (Netherlands)

    Schuijt, M. T. U.; Blok, I. M.; Zwinderman, A. H.; van Riel, A. C. M. J.; Schuuring, M. J.; de Winter, R. J.; Duijnhouwer, A. L.; van Dijk, A. P. J.; Mulder, B. J. M.; Bouma, B. J.

    2017-01-01

    Background: Adult patients with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) suffer from high mortality. This underlines the importance of adequate risk stratification to guide treatment decisions. Several baseline parameters are associated with mortality, however, their

  5. 2014 Guidelines of Taiwan Society of Cardiology (TSOC) for the Management of Pulmonary Arterial Hypertension

    Science.gov (United States)

    Hsu, Chih-Hsin; Ho, Wan-Jing; Huang, Wei-Chun; Chiu, Yu-Wei; Hsu, Tsu-Shiu; Kuo, Ping-Hung; Hsu, Hsao-Hsun; Chang, Jia-Kan; Cheng, Chin-Chang; Lai, Chao-Lun; Liang, Kae-Woei; Lin, Shoa-Lin; Sung, Hsao-Hsun; Tsai, Wei-Chuan; Weng, Ken-Pen; Hsieh, Kai-Sheng; Yin, Wei-Hsian; Lin, Shing-Jong; Wang, Kuo-Yang

    2014-01-01

    Pulmonary hypertension (PH) is a hemodynamic and pathophysiologic condition, defined as a mean pulmonary arterial pressure exceeding 25 mmHg at rest. According to the recent classifications, it is grouped into pulmonary arterial hypertension (PAH), heart-related, lung-related, thromboembolic, and miscellaneous PH. In the past two decades, tremendous advances have occurred in the field of PH. These include (1) development of clinical diagnostic algorithm and a monitoring strategy dedicated to PAH, (2) defining strong rationales for screening at-risk populations, (3) advent of pulmonary specific drugs which makes PAH manageable, (4) recognition of needs of having proper strategy of combining existing pulmonary specific drugs, and/or potential novel drugs, (5) pursuit of clinical trials with optimal surrogate endpoints and study durations, (6) recognition of critical roles of PH/right ventricular function, as well as interdependence of ventricles in different conditions, especially those with various phenotypes of heart failure, and (7) for rare diseases, putting equal importance on carefully designed observation studies, various registries, etc., besides double blind randomized studies. In addition, ongoing basic and clinical research has led to further understanding of relevant physiology, pathophysiology, epidemiology and genetics of PH/PAH. This guidelines from the working group of Pulmonary Hypertension of the Taiwan Society of Cardiology is to provide updated guidelines based on the most recent international guidelines as well as Taiwan’s domestic research on PH. The guidelines are mainly for the management of PAH (Group 1) ; however the majority of content can be helpful for managing other types of PH. PMID:27122817

  6. 8,9-Epoxyeicosatrienoic acid analog protects pulmonary artery smooth muscle cells from apoptosis via ROCK pathway

    International Nuclear Information System (INIS)

    Ma, Jun; Zhang, Lei; Li, Shanshan; Liu, Shulin; Ma, Cui; Li, Weiyang; Falck, J.R.; Manthati, Vijay L.; Reddy, D. Sudarshan; Medhora, Meetha; Jacobs, Elizabeth R.; Zhu, Daling

    2010-01-01

    Epoxyeicosatrienoic acids (EETs), metabolites of arachidonic acid (AA) catalyzed by cytochrome P450 (CYP), have many essential biologic roles in the cardiovascular system including inhibition of apoptosis in cardiomyocytes. In the present study, we tested the potential of 8,9-EET and derivatives to protect pulmonary artery smooth muscle cells (PASMCs) from starvation induced apoptosis. We found 8,9-epoxy-eicos-11(Z)-enoic acid (8,9-EET analog (214)), but not 8,9-EET, increased cell viability, decreased activation of caspase-3 and caspase-9, and decreased TUNEL-positive cells or nuclear condensation induced by serum deprivation (SD) in PASMCs. These effects were reversed after blocking the Rho-kinase (ROCK) pathway with Y-27632 or HA-1077. Therefore, 8,9-EET analog (214) protects PASMC from serum deprivation-induced apoptosis, mediated at least in part via the ROCK pathway. Serum deprivation of PASMCs resulted in mitochondrial membrane depolarization, decreased expression of Bcl-2 and enhanced expression of Bax, all effects were reversed by 8,9-EET analog (214) in a ROCK dependent manner. Because 8,9-EET and not the 8,9-EET analog (214) protects pulmonary artery endothelial cells (PAECs), these observations suggest the potential to differentially promote apoptosis or survival with 8,9-EET or analogs in pulmonary arteries.

  7. 8,9-Epoxyeicosatrienoic acid analog protects pulmonary artery smooth muscle cells from apoptosis via ROCK pathway

    Energy Technology Data Exchange (ETDEWEB)

    Ma, Jun; Zhang, Lei; Li, Shanshan [Department of Biopharmaceutical Sciences, College of Pharmacy, Harbin Medical University, 157 Baojian Road, Nangang District, Harbin, Heilongjiang 150081 (China); Liu, Shulin [Department of Biopharmaceutical Sciences, College of Pharmacy, Harbin Medical University, 157 Baojian Road, Nangang District, Harbin, Heilongjiang 150081 (China); Bio-pharmaceutical Key Laboratory of Heilongjiang Province, Harbin 150081 (China); Ma, Cui [Department of Biopharmaceutical Sciences, College of Pharmacy, Harbin Medical University, 157 Baojian Road, Nangang District, Harbin, Heilongjiang 150081 (China); Li, Weiyang [Mudanjiang Medical College, Mudanjiang 157011 (China); Falck, J.R.; Manthati, Vijay L.; Reddy, D. Sudarshan [University of Texas Southwestern Medical Center, Dallas, TX 75390 (United States); Medhora, Meetha; Jacobs, Elizabeth R. [Division of Pulmonary and Critical Care, Department of Medicine, Cardiovascular Center, Medical College of Wisconsin, Milwaukee, WI 53226 (United States); Zhu, Daling, E-mail: dalingz@yahoo.com [Department of Biopharmaceutical Sciences, College of Pharmacy, Harbin Medical University, 157 Baojian Road, Nangang District, Harbin, Heilongjiang 150081 (China); Bio-pharmaceutical Key Laboratory of Heilongjiang Province, Harbin 150081 (China)

    2010-08-15

    Epoxyeicosatrienoic acids (EETs), metabolites of arachidonic acid (AA) catalyzed by cytochrome P450 (CYP), have many essential biologic roles in the cardiovascular system including inhibition of apoptosis in cardiomyocytes. In the present study, we tested the potential of 8,9-EET and derivatives to protect pulmonary artery smooth muscle cells (PASMCs) from starvation induced apoptosis. We found 8,9-epoxy-eicos-11(Z)-enoic acid (8,9-EET analog (214)), but not 8,9-EET, increased cell viability, decreased activation of caspase-3 and caspase-9, and decreased TUNEL-positive cells or nuclear condensation induced by serum deprivation (SD) in PASMCs. These effects were reversed after blocking the Rho-kinase (ROCK) pathway with Y-27632 or HA-1077. Therefore, 8,9-EET analog (214) protects PASMC from serum deprivation-induced apoptosis, mediated at least in part via the ROCK pathway. Serum deprivation of PASMCs resulted in mitochondrial membrane depolarization, decreased expression of Bcl-2 and enhanced expression of Bax, all effects were reversed by 8,9-EET analog (214) in a ROCK dependent manner. Because 8,9-EET and not the 8,9-EET analog (214) protects pulmonary artery endothelial cells (PAECs), these observations suggest the potential to differentially promote apoptosis or survival with 8,9-EET or analogs in pulmonary arteries.

  8. Interstage evaluation of homograft-valved right ventricle to pulmonary artery conduits for palliation of hypoplastic left heart syndrome.

    Science.gov (United States)

    Sandeep, Nefthi; Punn, Rajesh; Balasubramanian, Sowmya; Smith, Shea N; Reinhartz, Olaf; Zhang, Yulin; Wright, Gail E; Peng, Lynn F; Wise-Faberowski, Lisa; Hanley, Frank L; McElhinney, Doff B

    2018-04-01

    Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling. This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed. On competing risk analysis, survival over time was better in the valved conduit group (P = .040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion (P = .15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance (P = .16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn. In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier

  9. Pulmonary capillary pressure in pulmonary hypertension.

    Science.gov (United States)

    Souza, Rogerio; Amato, Marcelo Britto Passos; Demarzo, Sergio Eduardo; Deheinzelin, Daniel; Barbas, Carmen Silvia Valente; Schettino, Guilherme Paula Pinto; Carvalho, Carlos Roberto Ribeiro

    2005-04-01

    Pulmonary capillary pressure (PCP), together with the time constants of the various vascular compartments, define the dynamics of the pulmonary vascular system. Our objective in the present study was to estimate PCPs and time constants of the vascular system in patients with idiopathic pulmonary arterial hypertension (IPAH), and compare them with these measures in patients with acute respiratory distress syndrome (ARDS). We conducted the study in two groups of patients with pulmonary hypertension: 12 patients with IPAH and 11 with ARDS. Four methods were used to estimate the PCP based on monoexponential and biexponential fitting of pulmonary artery pressure decay curves. PCPs in the IPAH group were considerably greater than those in the ARDS group. The PCPs measured using the four methods also differed significantly, suggesting that each method measures the pressure at a different site in the pulmonary circulation. The time constant for the slow component of the biexponential fit in the IPAH group was significantly longer than that in the ARDS group. The PCP in IPAH patients is greater than normal but methodological limitations related to the occlusion technique may limit interpretation of these data in isolation. Different disease processes may result in different times for arterial emptying, with resulting implications for the methods available for estimating PCP.

  10. miR-1 is increased in pulmonary hypertension and downregulates Kv1.5 channels in rat pulmonary arteries.

    Science.gov (United States)

    Mondejar-Parreño, Gema; Callejo, María; Barreira, Bianca; Morales-Cano, Daniel; Esquivel-Ruiz, Sergio; Moreno, Laura; Cogolludo, Angel; Perez-Vizcaino, Francisco

    2018-05-02

    ■The expression of miR-1 is increased in lungs from the Hyp/Su5416 PAH rat model. ■PASMC from this animal model are more depolarised and show decreased expression and activity of Kv1.5. ■miR-1 directly targets Kv1.5 channels, reduces Kv1.5 activity and induces membrane depolarization. ■Antagomir-1 prevents Kv1.5 channel downregulation and the depolarization induced by hypoxia/Su5416 exposition. Impairment of voltage-dependent potassium channel (Kv) plays a central role in the development of cardiovascular diseases, including pulmonary arterial hypertension (PAH). MicroRNAs (miRNAs) are non-coding RNAs that regulate gene expression by binding to the 3'-UTR region of specific mRNAs. The aim of this study was to analyze the effects of miR-1 on Kv channel function in pulmonary arteries (PA). Kv channel activity was studied in PA from healthy animals transfected with miR-1 or scrambled-miR. Kv currents were studied using the whole-cell configuration of patch-clamp technique. The characterization of the Kv1.5 currents was performed with the selective inhibitor DPO-1. miR-1 expression was increased and Kv1.5 channels were decreased in lungs from a rat model of PAH induced by hypoxia and Su5416. miR-1 transfection increased cell capacitance, reduced Kv1.5 currents and induced membrane depolarization in isolated pulmonary artery smooth muscle cells (PASMCs). Luciferase reporter assay indicated that KCNA5, which encodes Kv1.5 channels, is a direct target gene of miR-1. Incubation of PA with Su5416 and hypoxia (3% O 2 ) increased miR-1 and induced a decline in Kv1.5 currents, which was prevented by antagomiR-1. In conclusion, these data indicate that miR-1 induces PASMC hypertrophy and reduces the activity and expression of Kv channels, suggesting a pathophysiological role in PAH. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  11. Intrauterine growth restriction decreases pulmonary alveolar and vessel growth and causes pulmonary artery endothelial cell dysfunction in vitro in fetal sheep

    Science.gov (United States)

    Seedorf, Gregory J.; Brown, Alicia; Roe, Gates; O'Meara, Meghan C.; Gien, Jason; Tang, Jen-Ruey; Abman, Steven H.

    2011-01-01

    Intrauterine growth restriction (IUGR) increases the risk for bronchopulmonary dysplasia (BPD). Abnormal lung structure has been noted in animal models of IUGR, but whether IUGR adversely impacts fetal pulmonary vascular development and pulmonary artery endothelial cell (PAEC) function is unknown. We hypothesized that IUGR would decrease fetal pulmonary alveolarization, vascular growth, and in vitro PAEC function. Studies were performed in an established model of severe placental insufficiency and IUGR induced by exposing pregnant sheep to elevated temperatures. Alveolarization, quantified by radial alveolar counts, was decreased 20% (P growth by 68% (P growth was reduced in IUGR PAECs by 29% at baseline (P growth and PAEC dysfunction in vitro. This may contribute to the increased risk for adverse respiratory outcomes and BPD in infants with IUGR. PMID:21873446

  12. Endothelium-dependent relaxation induced by cathepsin G in porcine pulmonary arteries

    Science.gov (United States)

    Glusa, Erika; Adam, Christine

    2001-01-01

    Serine proteinases elicit profound cellular effects in various tissues mediated by activation of proteinase-activated receptors (PAR). In the present study, we investigated the vascular effects of cathepsin G, a serine proteinase that is present in the azurophil granules of leukocytes and is known to activate several cells that express PARs. In prostaglandin F2α (3 μM)-precontracted rings from porcine pulmonary arteries with intact endothelium, cathepsin G caused concentration-dependent relaxant responses (pEC50=9.64±0.12). The endothelium-dependent relaxant effect of cathepsin G could also be demonstrated in porcine coronary arteries (pEC50=9.23±0.07). In pulmonary arteries the cathepsin G-induced relaxation was inhibited after blockade of nitric oxide synthesis by L-NAME (200 μM) and was absent in endothelium-denuded vessels. Bradykinin- and cathepsin G-induced relaxant effects were associated with a 5.7 fold and 2.4 fold increase in the concentration of cyclic GMP, respectively. Compared with thrombin and trypsin, which also produced an endothelium-dependent relaxation in pulmonary arteries, cathepsin G was 2.5 and four times more potent, respectively. Cathepsin G caused only small homologous desensitization. In cathepsin G-challenged vessels, thrombin was still able to elicit a relaxant effect. The effects of cathepsin G were blocked by soybean trypsin inhibitor (IC50=0.043 μg ml−1), suggesting that proteolytic activity is essential for induction of relaxation. Recombinant acetyl-eglin C proved to be a potent inhibitor (IC50=0.14 μg ml−1) of the cathepsin G effect, whereas neither indomethacin (3 μM) nor the thrombin inhibitor hirudin (5 ATU ml−1) elicited any inhibitory activity. Due to their polyanionic structure defibrotide (IC50=0.11 μg ml−1), heparin (IC50=0.48 μg ml−1) and suramin (IC50=1.85 μg ml−1) diminished significantly the relaxation in response to the basic protein cathepsin G. In conclusion, like

  13. Radiological diagnosis of pulmonary hypertension

    International Nuclear Information System (INIS)

    Huebsch, P.; Jenny, C.; Schwaighofer, B.; Seidl, G.; Burghuber, O.C.

    1987-01-01

    In 43 patients with obstructive and restrictive lung disease a catheterisation of the right heart with measurement of pulmonary artery pressure was performed. In a retrospective study several radiological parameters of pulmonary hypertension were evaluated on the chest radiographs of these patients. Considering those parameters on the p.a. and lateral chest radiograph, the diagnosis of pulmonary hypertension in patients with elevated pulmonary artery pressure at rest can be made with great accuracy. When pulmonary artery pressure is elevated only during exercise, the accuracy of radiological diagnosis is much lower. (orig.) [de

  14. Pulmonary artery aneurysm in Bechcet's disease: helical computed tomography study; Aneurisma de la arteria pulmonar en la enfermedad de Behcet. Estudio con tomografia computarizada helicoidal

    Energy Technology Data Exchange (ETDEWEB)

    Munoz, J.; Caballero, P.; Olivera, M. J.; Cajal, M. L.; Caniego, J. L. [Hospital de la Princesa. Iniversidad Autonoma. Madrid (Spain)

    2000-07-01

    Behcet's disease is a vasculitis of unknown etiology that affects arteries and veins of different sizes and can be associated with pulmonary artery aneurysms. We report the case of a patient with Behcet's disease and a pulmonary artery aneurysm who was studied by means of plain chest X ray, helical computed tomography and pulmonary arteriography. Helical computed tomography is a reliable technique for the diagnosis and follow-up of these patients. (Author) 9 refs.

  15. Balloon pulmonary angioplasty: a treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    Aiko eOgawa

    2015-02-01

    Full Text Available In chronic thromboembolic pulmonary hypertension, stenoses or obstructions of the pulmonary arteries due to organized thrombi can cause an elevation in pulmonary artery resistance, which in turn can result in pulmonary hypertension. Chronic thromboembolic pulmonary hypertension can be cured surgically by pulmonary endarterectomy; however, patients deemed unsuitable for pulmonary endarterectomy due to lesion, advanced age, or comorbidities have a poor prognosis and limited treatment options. Recently, advances have been made in balloon pulmonary angioplasty for these patients, and this review highlights this recent progress.

  16. Variations in pulmonary artery occlusion pressure to estimate changes in pleural pressure.

    Science.gov (United States)

    Bellemare, Patrick; Goldberg, Peter; Magder, Sheldon A

    2007-11-01

    A readily available assessment of changes in pleural pressure would be useful for ventilator and fluid management in critically ill patients. We examined whether changes in pulmonary artery occlusion pressure (Ppao) adequately reflect respiratory changes in pleural pressure as assessed by changes in intraesophageal balloon pressure (Peso). We studied patients who had a pulmonary catheter and esophageal balloon surrounding a nasogastric tube as part of their care (n=24). We compared changes in Ppao (dPpao) to changes in Peso (dPeso) by Bland-Altman and regression analysis. Adequacy of balloon placement was assessed by performing Mueller maneuvers and adjusting the position to achieve a ratio of dPeso to change in tracheal pressure (dPtr) of 0.85 or higher. This was achieved in only 14 of the 24 subjects. We also compared dCVP to dPeso. The dPpao during spontaneous breaths and positive pressure breaths gave a good estimate of Peso but generally underestimated dPeso (bias=2.2 +8.2 and -3.9 cmH2O for the whole group). The dCVP was not as good a predictor (bias=2.9 +10.3 and -4.6). In patients who have a pulmonary artery catheter in place dPpao gives a lower estimate of changes in pleural pressure and may be more reliable than dPeso. The dCVP is a less reliable predictor than changes in pleural pressure.

  17. CT of the chest in the evaluation of idiopathic pulmonary arterial hypertension in children

    Energy Technology Data Exchange (ETDEWEB)

    Chaudry, Gulraiz [University of Toronto, Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto (Canada); Children' s Hospital Boston, Department of Radiology, Boston, MA (United States); MacDonald, Cathy; Gundogan, Munire; Manson, David [University of Toronto, Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto (Canada); Adatia, Ian [UCSF Children' s Hospital, Department of Pediatric Cardiology, San Francisco, CA (United States)

    2007-04-15

    Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease in children. By definition it is a diagnosis of exclusion, and CT of the chest is primarily performed to exclude other causes. Previous studies have defined CT features suggestive of the diagnosis of IPAH, but these have all been limited to the adult population. Contrast-enhanced chest CT and high-resolution CT findings in IPAH were evaluated in an attempt to define features consistently seen in children with this condition. The chest CT scans performed at initial presentation were reviewed in 17 children with echocardiographic or angiographic evidence of IPAH. There were nine boys and eight girls, ranging in age from 1 month to 17 years. The extrapulmonary findings included cardiomegaly with right-sided cardiac enlargement, which was seen in 13 children. The central pulmonary arteries were enlarged in 15 children, with peripheral enlargement in two. In six children this resulted in bronchial compression. In addition, mediastinal and hilar lymphadenopathy was noted in three children. Prominent intrapulmonary features included a peripheral vasculopathy, with enlarged tortuous vessels, seen in eight children. Ill-defined ground-glass centrilobular opacities were also noted in eight children, representing the most common parenchymal abnormality. Other findings included septal lines in five, diffuse ground-glass opacification in four and focal hyperlucent zones in three. Mosaic attenuation was seen in one child. A variety of imaging findings are identified in IPAH. Features particularly consistent with the diagnosis include peripheral vasculopathy and centrilobular opacities in the setting of cardiomegaly and central pulmonary arterial enlargement. (orig.)

  18. CT of the chest in the evaluation of idiopathic pulmonary arterial hypertension in children

    International Nuclear Information System (INIS)

    Chaudry, Gulraiz; MacDonald, Cathy; Gundogan, Munire; Manson, David; Adatia, Ian

    2007-01-01

    Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease in children. By definition it is a diagnosis of exclusion, and CT of the chest is primarily performed to exclude other causes. Previous studies have defined CT features suggestive of the diagnosis of IPAH, but these have all been limited to the adult population. Contrast-enhanced chest CT and high-resolution CT findings in IPAH were evaluated in an attempt to define features consistently seen in children with this condition. The chest CT scans performed at initial presentation were reviewed in 17 children with echocardiographic or angiographic evidence of IPAH. There were nine boys and eight girls, ranging in age from 1 month to 17 years. The extrapulmonary findings included cardiomegaly with right-sided cardiac enlargement, which was seen in 13 children. The central pulmonary arteries were enlarged in 15 children, with peripheral enlargement in two. In six children this resulted in bronchial compression. In addition, mediastinal and hilar lymphadenopathy was noted in three children. Prominent intrapulmonary features included a peripheral vasculopathy, with enlarged tortuous vessels, seen in eight children. Ill-defined ground-glass centrilobular opacities were also noted in eight children, representing the most common parenchymal abnormality. Other findings included septal lines in five, diffuse ground-glass opacification in four and focal hyperlucent zones in three. Mosaic attenuation was seen in one child. A variety of imaging findings are identified in IPAH. Features particularly consistent with the diagnosis include peripheral vasculopathy and centrilobular opacities in the setting of cardiomegaly and central pulmonary arterial enlargement. (orig.)

  19. Spatial differences of cellular origins and in vivo hypoxia modify contractile properties of pulmonary artery smooth muscle cells: lessons for arterial tissue engineering.

    Science.gov (United States)

    Hall, S M; Soueid, A; Smith, T; Brown, R A; Haworth, S G; Mudera, V

    2007-01-01

    Tissue engineering of functional arteries is challenging. Within the pulmonary artery wall, smooth muscle cells (PASMCs) have site-specific developmental and functional phenotypes, reflecting differing contractile roles. The force generated by PASMCs isolated from the inner 25% and outer 50% of the media of intrapulmonary elastic arteries from five normal and eight chronically hypoxic (hypertensive) 14 day-old piglets was quantified in a three-dimensional (3D) collagen construct, using a culture force monitor. Outer medial PASMCs from normal piglets exerted more force (528 +/- 50 dynes) than those of hypoxic piglets (177 +/- 42 dynes; p engineering of major blood vessels.

  20. Cytoskeletal defects in Bmpr2-associated pulmonary arterial hypertension.

    Science.gov (United States)

    Johnson, Jennifer A; Hemnes, Anna R; Perrien, Daniel S; Schuster, Manfred; Robinson, Linda J; Gladson, Santhi; Loibner, Hans; Bai, Susan; Blackwell, Tom R; Tada, Yuji; Harral, Julie W; Talati, Megha; Lane, Kirk B; Fagan, Karen A; West, James

    2012-03-01

    The heritable form of pulmonary arterial hypertension (PAH) is typically caused by a mutation in bone morphogenic protein receptor type 2 (BMPR2), and mice expressing Bmpr2 mutations develop PAH with features similar to human disease. BMPR2 is known to interact with the cytoskeleton, and human array studies in PAH patients confirm alterations in cytoskeletal pathways. The goal of this study was to evaluate cytoskeletal defects in BMPR2-associated PAH. Expression arrays on our Bmpr2 mutant mouse lungs revealed cytoskeletal defects as a prominent molecular consequence of universal expression of a Bmpr2 mutation (Rosa26-Bmpr2(R899X)). Pulmonary microvascular endothelial cells cultured from these mice have histological and functional cytoskeletal defects. Stable transfection of different BMPR2 mutations into pulmonary microvascular endothelial cells revealed that cytoskeletal defects are common to multiple BMPR2 mutations and are associated with activation of the Rho GTPase, Rac1. Rac1 defects are corrected in cell culture and in vivo through administration of exogenous recombinant human angiotensin-converting enzyme 2 (rhACE2). rhACE2 reverses 77% of gene expression changes in Rosa26-Bmpr2(R899X) transgenic mice, in particular, correcting defects in cytoskeletal function. Administration of rhACE2 to Rosa26-Bmpr2(R899X) mice with established PAH normalizes pulmonary pressures. Together, these findings suggest that cytoskeletal function is central to the development of BMPR2-associated PAH and that intervention against cytoskeletal defects may reverse established disease.

  1. Analysis of volatile compounds in exhaled breath condensate in patients with severe pulmonary arterial hypertension.

    Science.gov (United States)

    Mansoor, J K; Schelegle, Edward S; Davis, Cristina E; Walby, William F; Zhao, Weixiang; Aksenov, Alexander A; Pasamontes, Alberto; Figueroa, Jennifer; Allen, Roblee

    2014-01-01

    An important challenge to pulmonary arterial hypertension (PAH) diagnosis and treatment is early detection of occult pulmonary vascular pathology. Symptoms are frequently confused with other disease entities that lead to inappropriate interventions and allow for progression to advanced states of disease. There is a significant need to develop new markers for early disease detection and management of PAH. Exhaled breath condensate (EBC) samples were compared from 30 age-matched normal healthy individuals and 27 New York Heart Association functional class III and IV idiopathic pulmonary arterial hypertenion (IPAH) patients, a subgroup of PAH. Volatile organic compounds (VOC) in EBC samples were analyzed using gas chromatography/mass spectrometry (GC/MS). Individual peaks in GC profiles were identified in both groups and correlated with pulmonary hemodynamic and clinical endpoints in the IPAH group. Additionally, GC/MS data were analyzed using autoregression followed by partial least squares regression (AR/PLSR) analysis to discriminate between the IPAH and control groups. After correcting for medicaitons, there were 62 unique compounds in the control group, 32 unique compounds in the IPAH group, and 14 in-common compounds between groups. Peak-by-peak analysis of GC profiles of IPAH group EBC samples identified 6 compounds significantly correlated with pulmonary hemodynamic variables important in IPAH diagnosis. AR/PLSR analysis of GC/MS data resulted in a distinct and identifiable metabolic signature for IPAH patients. These findings indicate the utility of EBC VOC analysis to discriminate between severe IPAH and a healthy population; additionally, we identified potential novel biomarkers that correlated with IPAH pulmonary hemodynamic variables that may be important in screening for less severe forms IPAH.

  2. Analysis of volatile compounds in exhaled breath condensate in patients with severe pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    J K Mansoor

    Full Text Available BACKGROUND: An important challenge to pulmonary arterial hypertension (PAH diagnosis and treatment is early detection of occult pulmonary vascular pathology. Symptoms are frequently confused with other disease entities that lead to inappropriate interventions and allow for progression to advanced states of disease. There is a significant need to develop new markers for early disease detection and management of PAH. METHODOLGY AND FINDINGS: Exhaled breath condensate (EBC samples were compared from 30 age-matched normal healthy individuals and 27 New York Heart Association functional class III and IV idiopathic pulmonary arterial hypertenion (IPAH patients, a subgroup of PAH. Volatile organic compounds (VOC in EBC samples were analyzed using gas chromatography/mass spectrometry (GC/MS. Individual peaks in GC profiles were identified in both groups and correlated with pulmonary hemodynamic and clinical endpoints in the IPAH group. Additionally, GC/MS data were analyzed using autoregression followed by partial least squares regression (AR/PLSR analysis to discriminate between the IPAH and control groups. After correcting for medicaitons, there were 62 unique compounds in the control group, 32 unique compounds in the IPAH group, and 14 in-common compounds between groups. Peak-by-peak analysis of GC profiles of IPAH group EBC samples identified 6 compounds significantly correlated with pulmonary hemodynamic variables important in IPAH diagnosis. AR/PLSR analysis of GC/MS data resulted in a distinct and identifiable metabolic signature for IPAH patients. CONCLUSIONS: These findings indicate the utility of EBC VOC analysis to discriminate between severe IPAH and a healthy population; additionally, we identified potential novel biomarkers that correlated with IPAH pulmonary hemodynamic variables that may be important in screening for less severe forms IPAH.

  3. Right ventricular stroke work correlates with outcomes in pediatric pulmonary arterial hypertension.

    Science.gov (United States)

    Yang, Weiguang; Marsden, Alison L; Ogawa, Michelle T; Sakarovitch, Charlotte; Hall, Keeley K; Rabinovitch, Marlene; Feinstein, Jeffrey A

    2018-01-01

    Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). Optimizing treatment strategies and timing for transplant remains challenging. Thus, a quantitative measure to predict disease progression would be greatly beneficial in treatment planning. We devised a novel method to assess right ventricular (RV) stroke work (RVSW) as a potential biomarker of the failing heart that correlates with clinical worsening. Pediatric patients with idiopathic PAH or PAH secondary to congenital heart disease who had serial, temporally matched cardiac catheterization and magnetic resonance imaging (MRI) data were included. RV and PA hemodynamics were numerically determined by using a lumped parameter (circuit analogy) model to create pressure-volume (P-V) loops. The model was tuned using optimization techniques to match MRI and catheterization derived RV volumes and pressures for each time point. RVSW was calculated from the corresponding P-V loop and indexed by ejection fraction and body surface area (RVSW EF ) to compare across patients. Seventeen patients (8 boys; median age = 9.4 years; age range = 4.4-16.3 years) were enrolled. Nine were clinically stable; the others had clinical worsening between the time of their initial matched studies and their most recent follow-up (mean time = 3.9 years; range = 1.1-8.0 years). RVSW EF and the ratio of pulmonary to systemic resistance (Rp:Rs) values were found to have more significant associations with clinical worsening within one, two, and five years following the measurements, when compared with PVR index (PVRI). A receiver operating characteristic analysis showed RVSW EF outperforms PVRI, Rp:Rs and ejection fraction for predicting clinical worsening. RVSW EF correlates with clinical worsening in pediatric PAH, shows promising results towards predicting adverse outcomes, and may serve as an indicator of future clinical worsening.

  4. Bronchial arteries: anatomy, function, hypertrophy, and anomalies.

    Science.gov (United States)

    Walker, Christopher M; Rosado-de-Christenson, Melissa L; Martínez-Jiménez, Santiago; Kunin, Jeffrey R; Wible, Brandt C

    2015-01-01

    The two main sources of blood supply to the lungs and their supporting structures are the pulmonary and bronchial arteries. The bronchial arteries account for 1% of the cardiac output but can be recruited to provide additional systemic circulation to the lungs in various acquired and congenital thoracic disorders. An understanding of bronchial artery anatomy and function is important in the identification of bronchial artery dilatation and anomalies and the formulation of an appropriate differential diagnosis. Visualization of dilated bronchial arteries at imaging should alert the radiologist to obstructive disorders that affect the pulmonary circulation and prompt the exclusion of diseases that produce or are associated with pulmonary artery obstruction, including chronic infectious and/or inflammatory processes, chronic thromboembolic disease, and congenital anomalies of the thorax (eg, proximal interruption of the pulmonary artery). Conotruncal abnormalities, such as pulmonary atresia with ventricular septal defect, are associated with systemic pulmonary supply provided by aortic branches known as major aortopulmonary collaterals, which originate in the region of the bronchial arteries. Bronchial artery malformation is a rare left-to-right or left-to-left shunt characterized by an anomalous connection between a bronchial artery and a pulmonary artery or a pulmonary vein, respectively. Bronchial artery interventions can be used successfully in the treatment of hemoptysis, with a low risk of adverse events. Multidetector computed tomography helps provide a vascular road map for the interventional radiologist before bronchial artery embolization. RSNA, 2015

  5. Load Adaptability in Patients With Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Amsallem, Myriam; Boulate, David; Aymami, Marie; Guihaire, Julien; Selej, Mona; Huo, Jennie; Denault, Andre Y; McConnell, Michael V; Schnittger, Ingela; Fadel, Elie; Mercier, Olaf; Zamanian, Roham T; Haddad, Francois

    2017-09-01

    Right ventricular (RV) adaptation to pressure overload is a major prognostic factor in patients with pulmonary arterial hypertension (PAH). The objectives were first to define the relation between RV adaptation and load using allometric modeling, then to compare the prognostic value of different indices of load adaptability in PAH. Both a derivation (n = 85) and a validation cohort (n = 200) were included. Load adaptability was assessed using 3 approaches: (1) surrogates of ventriculo-arterial coupling (e.g., RV area change/end-systolic area), (2) simple ratio of function and load (e.g., tricuspid annular plane systolic excursion/right ventricular systolic pressure), and (3) indices assessing the proportionality of adaptation using allometric pressure-function or size modeling. Proportional hazard modeling was used to compare the hazard ratio for the outcome of death or lung transplantation. The mean age of the derivation cohort was 44 ± 11 years, with 80% female and 74% in New York Heart Association class III or IV. Mean pulmonary vascular resistance index (PVRI) was 24 ± 11 with a wide distribution (1.6 to 57.5 WU/m 2 ). Allometric relations were observed between PVRI and RV fractional area change (R 2  = 0.53, p adaptation. In right heart parameters, RVESAI was the strongest predictor of outcomes (hazard ratio per SD = 1.93, 95% confidence interval 1.37 to 2.75, p adaptability indices provided stronger discrimination of outcome than simple RV adaptation metrics in either the derivation or the validation cohort. In conclusion, allometric modeling enables quantification of the proportionality of RV load adaptation but offers small incremental prognostic value to RV end-systolic dimension in PAH. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. Experimental studies on canine terminal carotid arterial aneurysm models pre- and post-embolized with microcoil

    International Nuclear Information System (INIS)

    Wang Qihong; Zhou Liangfu; Gong Jianqiu; Dai Jianhua; Zhang Xiaojun; Zhang Xinyuan

    2005-01-01

    Objective: To establish canine terminal saccular aneurysm model of common carotid artery (CCA), and evaluate the images and hemodynamic changes of aneurysm cavities pre- and post-embolized with microcoil (MC). Methods: Seven CCA terminal saccular aneurysm models had been created by using improved microsurgical technique. Seven to fourteen days after surgery, models were undergone color Doppler, Magnetic resonance image (MRI), Magnetic resonance angiography (MRA), digital subtraction angiography (DSA), and then the analysis of computational fluid dynamics (CFD) on these animal models were conducted. Afterwards they were compactly packed with MC, and then aneurysms again were examed under these methods, and simulation of pathological changes. Results: Models were created successfully with good MC compactly pack aneurysm cavities. DSA combined with other imaging methods can improve the diagnosis of aneurysms completely. CFD models can show the imitation hemodynamics of canine carotid aneurysms. Conclusions: This kind of aneurysm model is an ideal one on the area for the research of aneurysm embolization. Non-DSA imaging examination can noninvasively, repeatedly, clearly evaluate aneurysms pre- and post-embolized with MC. The interference on the inflow zone of the aneurysm is an effective method for cure and preventing recurrence of aneurysms. (authors)

  7. Validation of a brief telephone battery for neurocognitive assessment of patients with pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Palevsky Harold I

    2005-04-01

    Full Text Available Abstract Background The effects of pulmonary arterial hypertension on brain function are not understood, despite patients' frequent complaints of cognitive difficulties. Using clinical instruments normally administered during standard in-person assessment of neurocognitive function in adults, we assembled a battery of tests designed for administration over the telephone. The purpose was to improve patient participation, facilitate repeated test administration, and reduce the cost of research on the neuropsychological consequences of acute and chronic cardiorespiratory diseases. We undertook this study to validate telephone administration of the tests. Methods 23 adults with pulmonary arterial hypertension underwent neurocognitive assessment using both standard in-person and telephone test administration, and the results of the two methods compared using interclass correlations. Results For most of the tests in the battery, scores from the telephone assessment correlated strongly with those obtained by in-person administration of the same tests. Interclass correlations between 0.5 and 0.8 were observed for tests that assessed attention, memory, concentration/working memory, reasoning, and language/crystallized intelligence (p ≤ 0.05 for each. Interclass correlations for the Hayling Sentence Completion test of executive function approached significance (p = 0.09. All telephone tests were completed within one hour. Conclusion Administration of this neurocognitive test battery by telephone should facilitate assessment of neuropsychological deficits among patients with pulmonary arterial hypertension living across broad geographical areas, and may be useful for monitoring changes in neurocognitive function in response to PAH-specific therapy or disease progression.

  8. Combined radiodiagnosis of pulmonary artery coarctation in Fallot's tetrad

    International Nuclear Information System (INIS)

    Dynnik, I.B.; Shalenkov, V.P.

    1986-01-01

    The paper is concerned with a comparative analysis of the results of routine X-ray examination and those of intubation and angiocardiography in 36 patients with Fallot's tetrad and pulmonary artery coarctation (PAC). The chief method of PAC diagnosis was selective angiocardiography from the right ventricular orifice. The authors presented angiocardiographic semiotics of the PAC main classes according to modern morphological classification which could be of practical value in a cardiosurgical hospital for the planning of operations. In some forms of PAC, transmission chest roentgenograms revealed pathognomonic roentgenologic signs permitting a preliminary diagnosis with a subsequent roentgenosurgical investigation

  9. MRI-based flow measurements in the main pulmonary artery to detect pulmonary arterial hypertension in patients with cystic fibrosis

    International Nuclear Information System (INIS)

    Wolf, T.; Anjorin, A.; Abolmaali, N.; Posselt, H.; Smaczny, C.; Vogl, T.J.

    2009-01-01

    Development of pulmonary arterial hypertension (PH) is a common problem in the course of patients suffering from cystic fibrosis (CF). This study was performed to evaluate MRI based flow measurements (MR venc ; Velocity ENCoding) to detect signs of an evolving PH in patients suffering from CF. 48 patients (median age: 16 years, range: 10 - 40 years, 25 female) suffering from CF of different severity (mean FEV1: 74 % ± 23, mean Shwachman-score: 63 ± 10) were examined using MRI based flow measurements of the main pulmonary artery (MPA). Phase-contrast flash sequences (TR: 9.6 ms, TE: 2.5 ms, bandwidth: 1395 Hertz/Pixel) were utilized. Results were compared to an age- and sex-matched group of 48 healthy subjects. Analyzed flow data where: heart frequency (HF), cardiac output (HZV), acceleration time (AT), proportional acceleration time related to heart rate (ATr), mean systolic blood velocity (MFG), peak velocity (Peak), maximum flow (Fluss max ), mean flow (Fluss mitt ) and distensibility (Dist). The comparison of means revealed significant differences only for MFG, Fluss max and Dist, but overlap was marked. However, using a scatter-plot of AT versus MFG, it was possible to identify five CF-patients demonstrating definite signs of PH: AT = 81 ms ± 14, MFG = 46 ± 11 cm/s, Dist = 41 % ± 7. These CF-patients where the most severely affected in the investigated group, two of them were listed for complete heart and lung transplantation. The comparison of this subgroup and the remaining CF-patients revealed a highly significant difference for the AT (p = 0.000001) without overlap. Screening of CF-patients for the development of PH using MR venc of the MPA is not possible. In later stages of disease, the quantification of AT, MFG and Dist in the MPA may be useful for the detection, follow-up and control of therapy of PH. MR venc of the MPA completes the MRI-based follow-up of lung parenchyma damage in patients suffering from CF. (orig.)

  10. K(Ca)3.1 channel downregulation and impaired endothelium-derived hyperpolarization-type relaxation in pulmonary arteries from chronically hypoxic rats

    DEFF Research Database (Denmark)

    Kroigaard, Christel; Kudryavtseva, Olga; Dalsgaard, Thomas

    2013-01-01

    hypoxia-induced pulmonary hypertension in rats. For functional studies, pulmonary arteries were mounted in microvascular myographs for isometric tension recordings. The K(Ca) channel expression was evaluated by immunoblotting and quantitative PCR. Although ACh induced similar relaxations, the ACh...

  11. Effects of exercise training in patients with idiopathic pulmonary arterial hypertension

    OpenAIRE

    de Man, F.S.; Handoko, M.L.; Groepenhoff, H.; van 't Hul, A.J.; Abbink, J.; Koppers, R.J.H.; Grotjohan, H.P.; Twisk, J.W.R.; Bogaard, H.J.; Boonstra, A.; Postmus, P.E.; Westerhof, N.; van der Laarse, W.J.; Vonk Noordegraaf, A.

    2009-01-01

    We determined the physiological effects of exercise training on exercise capacity and quadriceps muscle function in patients with idiopathic pulmonary arterial hypertension (iPAH). In total, 19 clinically stable iPAH patients (New York Heart Association II-III) underwent a supervised exercise training programme for the duration of 12 weeks. Maximal capacity, endurance capacity and quadriceps function were assessed at baseline and after 12 weeks. In 12 patients, serial quadriceps muscle biopsi...

  12. A Dual Lung Scan for the Evaluation of Pulmonary Function in Patients with Pulmonary Tuberculosis before and after Treatment

    International Nuclear Information System (INIS)

    Rhee, Chong Heon

    1967-01-01

    In 20 normal cases and 39 pulmonary tuberculosis cases, regional pulmonary arterial blood flow measurement and lung perfusion scans by 131 I-Macroaggregated albumin, lung inhalation scans by colloidal 198 Au and spirometries by respirometer were done at the Radiological Research Institute. The measured lung function tests were compared and the results were as the following: 1) The normal distribution of pulmonary blood flow was found to be 54.5±2.82% to the right lung and 45.5±2.39% to the left lung. The difference between the right and left pulmonary arterial blood flow was significant statistically (p 131 I-MAA in patients with pulmonary tuberculosis was as follows: a) In the pretreated minimal pulmonary tuberculosis, the decreased area of pulmonary arterial blood flow was corresponding to the chest roentgenogram, but the decrease of pulmonary arterial blood flow was more extensive than had been expected from the chest roentgenogram in the apparently healed minimal pulmonary tuberculosis. b) In the pretreated moderately advanced pulmonary tuberculosis, the decrease of pulmonary arterial blood flow to the diseased area was corresponding to the chest roentgenogram, but the decrease of pulmonary arterial blood flow was more extensive in the treated moderately advanced pulmonary tuberculosis as in the treated minimal pulmonary tuberculosis. c) Pulmonary arterial blood flow in the patients with far advanced pulmonary tuberculosis both before and after chemotherapy were almost similar to the chest roentgenogram. Especially the decrease of pulmonary arterial blood flow to the cavity was usually greater than had been expected from the chest roentgenogram. 3) Lung inhalation scan by colloidal 198 Au in patients with pulmonary tuberculosis was as follows: a) In the minimal pulmonary tuberculosis, lung inhalation scan showed almost similar decrease of radioactivity corresponding to the chest roentgenogram. b) In the moderately advanced pulmonary tuberculosis the decrease

  13. Enhanced magnetic resonance pulmonary perfusion imaging in diagnosing pulmonary embolism: preliminary investigation

    International Nuclear Information System (INIS)

    Huang Xiaoyong; Du Jing; Zhang Zhaoqi; Guo Xi; Yan Zixu; Jiang Hong; Wang Wei

    2005-01-01

    Objective: This study was designed to investigate the sensitivity and specificity of magnetic resonance pulmonary perfusion imaging (MRPP) in diagnosing pulmonary embolism (PE) compared with enhanced magnetic resonance pulmonary angiography (MRPA) and pulmonary radionuclide perfusion imaging. Methods: Fourteen patients were definitely diagnosed as PE, whose ages were from 19 to 71 years old and mean 45.5 ± 19.8 years old. All patients under went MRPA and MRPP and 3 patients were examined again after thrombolytic treatment. Five patients underwent pulmonary radionuclide perfusion imaging. Setting ROI in top, middle, bottom of lung area and abnormal area respectively, we detected signal intensity and time-signal curve to obtain the transformation rate of signal (TROS) during perfusion peak value. Results: In 14 pulmonary embolism patients, MRPA found 62 branches of pulmonary artery obstruction. Fifty-five abnormal pulmonary perfusion zones were found by MRPP, and the above results were very alike. The coincidence was 88.71%. In 14 cases, MRPP could show 25 subsegments lesion below segments. In 5 patients who had both results Of MRPP and ECT at the same time. MRPP shows 33 perfusion defect zones and 37 segments were found by ECT, the sensitivity was 89.19%. After thrombolytic treatment, both the status of the affected pulmonary artery improved markedly and perfusion defect zones reduced obviously in 3 cases by MRPP and MRPA. TROS in normal perfusion zones perfusion defect zones and low perfusion zones had significant difference (t=22.882, P<0.01). Conclusion: Contrast enhanced MR pulmonary perfusion can show both perfusion defect zones and low perfusion zones in pulmonary embolism. Time-signal curve can show the period of maximum no perfusion zones in pulmonary artery embolism zones. And the amplitude of fluctuation is small with miminum TROS. MRPP has significant values especially in showing pulmonary artery embolism in segments and subsegments. Using both MRPP and

  14. Hypoxia increases pulmonary arterial thromboxane receptor internalization independent of receptor sensitization.

    Science.gov (United States)

    Fediuk, J; Sikarwar, A S; Lizotte, P P; Hinton, M; Nolette, N; Dakshinamurti, S

    2015-02-01

    Persistent Pulmonary Hypertension of the Newborn (PPHN) is characterized by sustained vasospasm and an increased thromboxane:prostacyclin ratio. Thromboxane (TP) receptors signal via Gαq to mobilize IP3 and Ca(2+), causing pulmonary arterial constriction. We have previously reported increased TP internalization in hypoxic pulmonary arterial (PA) myocytes. Serum-deprived PA myocytes were grown in normoxia (NM) or hypoxia (HM) for 72 h. TP localization was visualized in agonist-naïve and -challenged NM and HM by immunocytochemistry. Pathways for agonist-induced TP receptor internalization were determined by inhibiting caveolin- or clathrin-mediated endocytosis, and caveolar fractionation. Roles of actin and tubulin in TP receptor internalization were assessed using inhibitors of tubulin, actin-stabilizing or -destabilizing agents. PKA, PKC or GRK activation and inhibition were used to determine the kinase responsible for post-agonist receptor internalization. Agonist-naïve HM had decreased cell surface TP, and greater TP internalization after agonist challenge. TP protein did not sort with caveolin-rich fractions. Inhibition of clathrin prevented TP internalization. Both actin-stabilizing and -destabilizing agents prevented TP endocytosis in NM, while normalizing TP internalization in HM. Velocity of TP internalization was unaffected by PKA activity, but PKC activation normalized TP receptor internalization in HM. GRK inhibition had no effect. We conclude that in hypoxic myocytes, TP is internalized faster and to a greater extent than in normoxic controls. Internalization of the agonist-challenged TP requires clathrin, dynamic actin and is sensitive to PKC activity. TP receptor trafficking and signaling in hypoxia are pivotal to understanding increased vasoconstrictor sensitivity. Copyright © 2014 Elsevier Ltd. All rights reserved.

  15. HMGB1 promotes the development of pulmonary arterial hypertension in rats.

    Directory of Open Access Journals (Sweden)

    Yukari Sadamura-Takenaka

    Full Text Available Pulmonary arterial hypertension (PAH is characterized by increased pulmonary vascular resistance leading to right ventricular failure and death. Recent studies have suggested that chronic inflammatory processes are involved in the pathogenesis of PAH. However, the molecular and cellular mechanisms driving inflammation have not been fully elucidated.To elucidate the roles of high mobility group box 1 protein (HMGB1, a ubiquitous DNA-binding protein with extracellular pro-inflammatory activity, in a rat model of PAH.Male Sprague-Dawley rats were administered monocrotaline (MCT. Concentrations of HMGB1 in bronchoalveolar lavage fluid (BALF and serum, and localization of HMGB1 in the lung were examined over time. The protective effects of anti-HMGB1 neutralizing antibody against MCT-induced PAH were tested.HMGB1 levels in BALF were elevated 1 week after MCT injection, and this elevation preceded increases of other pro-inflammatory cytokines, such as TNF-α, and the development of PAH. In contrast, serum HMGB1 levels were elevated 4 weeks after MCT injection, at which time the rats began to die. Immunohistochemical analyses indicated that HMGB1 was translocated to the extranuclear space in periarterial infiltrating cells, alveolar macrophages, and bronchial epithelial cells of MCT-injected rats. Anti-HMGB1 neutralizing antibody protected rats against MCT-induced lung inflammation, thickening of the pulmonary artery wall, and elevation of right ventricular systolic pressure, and significantly improved the survival of the MCT-induced PAH rats.Our results identify extracellular HMGB1 as a promoting factor for MCT-induced PAH. The blockade of HMGB1 activity improved survival of MCT-induced PAH rats, and thus might be a promising therapy for the treatment of PAH.

  16. Key articles and guidelines in the management of pulmonary arterial hypertension: 2011 update.

    Science.gov (United States)

    Johnson, Samuel G; Kayser, Steven R; Attridge, Rebecca L; Duvall, Laura; Kiser, Tyree H; Moote, Rebecca; Reed, Brent N; Rodgers, Jo E; Erstad, Brian

    2012-06-01

    Pharmacotherapeutic approaches for the management of pulmonary arterial hypertension (PAH) have expanded greatly in the last 10 years. Pulmonary arterial hypertension is a relatively rare disease and is associated with myriad disease processes. The older term for PAH, primary PAH, has been changed to represent these differences and to distinguish it from postcapillary PAH associated with left-sided heart failure. Limitations in evaluating treatment approaches for PAH include its rarity, the small number of patients included in clinical trials, and issues regarding the use of placebo-controlled trials in a disease with such a high mortality rate if left untreated. Management options include the use of prostacyclin and prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase inhibitors, as well as traditional background therapy with diuretics, digoxin, calcium channel blockers, and warfarin. Numerous drugs are under investigation to evaluate their possible roles in management. Combination therapy is increasingly becoming a standard approach to therapy, with mounting literature to document effectiveness. Current or emerging roles for the pharmacist in the management of PAH largely involves ensuring access to drug therapy, facilitating specialty pharmacy dispensing, and providing patient counseling. Newer roles may include future drug development, optimized use of investigational drugs, and specialized disease management programs. This compilation includes a series of articles identifying important literature in cardiovascular pharmacotherapy. This bibliography focuses on pharmacotherapeutic management of pulmonary arterial hypertension (PAH). Most of the cited works present the results of significant human clinical studies that have shaped the management of patients with PAH. Limited primary literature is available for some topics, so in addition, consensus documents prepared by expert panels are reviewed. This compilation may serve as a

  17. [Pulmonary involvement in systemic sclerosis. Alveolitis, fibrosis and pulmonar arterial hypertension].

    Science.gov (United States)

    Navarro, Carmen

    2006-11-01

    Pulmonary involvement in systemic sclerosis. Alveolitis, fibrosis and pulmonar arterial hypertension Lung disease is present in most of the patients with systemic sclerosis and is now the most important cause of mortality. Interstitial lung disease and pulmonary hypertension are, so far, the main disorders found and both are difficult to detect at the earliest stages. However, diagnostic tools such as immunological test, lung function test, high resolution CT, bronchoalveolar lavage, echocardiography, right-side cardiac catheterization, or lung biopsy are necessary to accurately evaluate the clinical status and allow to improve the management organ-specific ad hoc. Progress in immunological and vascular therapies as well as other emergence drugs offer new expectations to scleroderma patients. Copyright © 2006 Elsevier España S.L. Barcelona. Published by Elsevier Espana. All rights reserved.

  18. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature

    NARCIS (Netherlands)

    A.D.J. ten Harkel (Arend); N.A. Blom (Nico); J. Ottenkamp (Jaap)

    2002-01-01

    textabstractOBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case

  19. Hipertensão pulmonar secundária à fístulas coronarianas para tronco da pulmonar Pulmonary hypertension secondary to coronary-to-pulmonary artery fistula

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    José Ramos Filho

    2008-08-01

    Full Text Available A fístula coronariana é uma anomalia caracterizada por comunicação entre uma artéria coronária e uma câmara cardíaca, artéria pulmonar, seio coronariano e veias pulmonares. Representa 0,2% a 0,4 % das cardiopatias congênitas e 0,1% a 0,2% da população adulta submetida a angiografia coronariana. Relatamos o caso clínico de uma paciente com 64 anos, cuja anomalia foi diagnosticada durante investigação clínica por desconforto torácico, dispnéia e síncope, sendo indicada correção cirúrgica com abertura da artéria pulmonar através de circulação extracorpórea.The coronary fistula is an anomaly characterized by the communication between a coronary artery and a cardiac chamber, pulmonary artery, coronary sinus and pulmonary veins. It represents 0.2 to 0.4% of the congenital cardiopathies and 0.1% to 0.2% of the adult population submitted to coronary angiography. We report the clinical case of a 64-year-old female patient, whose anomaly was diagnosed during a clinical investigation due to chest discomfort, dyspnea and syncope; the surgical correction was indicated, with opening of the pulmonary artery through extracorporeal circulation.

  20. Echocardiographic diagnosis of transposition of the great arteries associated with anomalous pulmonary venous connection

    Directory of Open Access Journals (Sweden)

    Lilian Maria Lopes

    2001-07-01

    Full Text Available We report 2 cases of transposition of the great arteries associated with anomalous pulmonary venous connection emphasizing the clinical findings, the diagnosis, and the evolution of the association. One of the patients had the anomalous pulmonary venous connection in its total infradiaphragmatic form, in the portal system, and the other patient had a partial form, in which an anomalous connection of the left superior lobar vein with the innominate vein existed. At the time of hospital admission, the patients had cyanosis and respiratory distress with clinical findings suggesting transposition of the great arteries. The diagnosis in 1 of the cases, in which the anomalous connection was partial, was established only with echocardiography, without invasive procedures that would represent risk for the patient; in the other case, in which the anomalous connection was total, the malformation was only evidenced with catheterization. The patients underwent surgery for anatomical correction of the heart disease. Only 1 patient had a good outcome.