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Sample records for canine pulmonary arterial

  1. Relationship between pulmonary arterial pressure and pulmonary thromboembolism associated with dead worms in canine heartworm disease.

    Science.gov (United States)

    Hirano, Y; Kitagawa, H; Sasaki, Y

    1992-10-01

    To examine effects of thromboemboli due to dead worms on pulmonary arterial pressure (PAP), 20 to 50 dead heartworms were inserted into the pulmonary arteries of 4 heartworm uninfected dogs (uninfected group) and 11 dogs infected with heartworms (infected group). In the uninfected group, the mean PAP rose 1 week after worm insertion (10.9 to 166. mmHg), but it recovered by the 4th week. Clinical signs, hemodynamics and blood gas findings also deteriorated at the 1st week, but recovered at the 4th week. Angiographic and pathological findings indicated that blood flow recovered through the spaces between thromboemboli and vessel walls at the 4th week. The infected dogs were divided into three groups. In the infected-I group (5 dogs), the intimal lesions of the pulmonary arteries were slight, and clinical and laboratory findings showed changes similar to those of the uninfected group. In the infected-II group (4 dogs), the pulmonary arterial lesions were severe and the mean PAP was higher (25.7 mmHg) than in the uninfected group before worm insertion. An increase in PAP (34.1 mmHg) and worsening of clinical and laboratory findings were noticed till the 4th week. Thromboemboli adhered extensively to the vessel walls. Two dogs in the infected-III group died of severe dyspnea on the 9th and 10th day, and the mean PAP rose remarkably at the 1st week (from 19.4 to 28.2 mmHg). Severe pulmonary parenchymal lesions with edema or perforation were observed. From the above results, it was clarified that effects of dead worms on PAP and clinical signs depended on the severity of pulmonary arterial lesions before worm insertion.(ABSTRACT TRUNCATED AT 250 WORDS)

  2. Canine pulmonary angiostrongylosis

    DEFF Research Database (Denmark)

    Koch, Jørgen; Willesen, Jakob Lundgren

    2009-01-01

    Canine pulmonary angiostrongylosis is an emerging snail-borne disease causing verminous pnemonia and coagulopathy in dogs. The parasite is fund in Europe, North and South America and Africa, covering tropical, subtropical and temperate regions. Its distribution has been characterised by isolated ...

  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 Arterial Hypertension

    Science.gov (United States)

    Pulmonary Arterial Hypertension What Is Pulmonary Hypertension? To understand pulmonary hypertension (PH) it helps to understand how blood ows throughout your body. While the heart is one organ, it ...

  5. Idiopathic pulmonary artery aneurysm.

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    Kotwica, Tomasz; Szumarska, Joanna; Staniszewska-Marszalek, Edyta; Mazurek, Walentyna; Kosmala, Wojciech

    2009-05-01

    Pulmonary artery aneurysm (PAA) is an uncommon lesion, which may be associated with different etiologies including congenital cardiovascular diseases, systemic vasculitis, connective tissue diseases, infections, and trauma. Idiopathic PAA is sporadically diagnosed by exclusion of concomitant major pathology. We report a case of a 56-year-old female with an idiopathic pulmonary artery dilatation identified fortuitously by echocardiography and confirmed by contrast-enhanced computed tomography. Neither significant pulmonary valve dysfunction nor pulmonary hypertension and other cardiac abnormalities which might contribute to the PAA development were found. Here, we describe echocardiographic and computed tomography findings and review the literature on PAA management.

  6. 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 a

  7. Pulmonary arterial hypertension in pregnancy.

    Science.gov (United States)

    Običan, Sarah G; Cleary, Kirsten L

    2014-08-01

    Pulmonary hypertension is a medical condition characterized by elevated pulmonary arterial pressure and secondary right heart failure. Pulmonary arterial hypertension is a subset of pulmonary hypertension, which is characterized by an underlying disorder of the pulmonary arterial vasculature. Pulmonary hypertension can also occur secondarily to structural cardiac disease, autoimmune disorders, and toxic exposures. Although pregnancies affected by pulmonary hypertension and pulmonary arterial hypertension are rare, the pathophysiology exacerbated by pregnancy confers both high maternal and fetal mortality and morbidity. In light of new treatment modalities and the use of a multidisciplinary approach to care, maternal outcomes may be improving.

  8. Pulmonary artery sling: Case report

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    Shin, Gil Hyun; Lee, Sun Wha; Cha, Sung Ho [Kyunghee University College of Medicine, Seoul (Korea, Republic of)

    1993-09-15

    Aberrant left-sided pulmonary artery(pulmonary artery sling) is an uncommon anomaly,which may cause significant respiratory abnormality. We report a case of pulmonary artery sling which is combined with persistent left superior vena cava and dextrocardia. This case were identified by esophagogram and CT and confirmed by MRI and angiography. We consider that MRI is a valuable new method for the diagnosis of aberrant left-sided pulmonary artery.

  9. Update in pulmonary arterial hypertension.

    Science.gov (United States)

    Mejía Chew, C R; Alcolea Batres, S; Ríos Blanco, J J

    2016-11-01

    Pulmonary arterial hypertension is a rare and progressive disease that mainly affects the pulmonary arterioles (precapillary), regardless of the triggering aetiology. The prevalence of pulmonary hypertension and pulmonary arterial hypertension in Spain is estimated at 19.2 and 16 cases per million inhabitants, respectively. The diagnosis of pulmonary arterial hypertension is based on haemodynamic criteria (mean pulmonary artery pressure ≥25mmHg, pulmonary capillary wedge pressure ≤15mmHg and pulmonary vascular resistance >3 Wood units) and therefore requires the implementation of right cardiac catheterisation. Sequential therapy with a single drug has been used in clinical practice. However, recent European guidelines recommend combined initial therapy in some situations. This review conducts a critical update of our knowledge of this disease according to the latest guidelines and recommendations.

  10. Genetics Home Reference: pulmonary arterial hypertension

    Science.gov (United States)

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

  11. Fatal dissection of the pulmonary artery in pulmonary arterial hypertension

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    B. Degano

    2009-09-01

    Full Text Available A 41-yr-old patient with chronic stable idiopathic pulmonary arterial hypertension (PAH presented with sudden chest pain and unusual dyspnoea during physical exertion. The patient had been diagnosed with PAH at the age of 12 yrs and was in New York Heart Association functional class I/II. The patient was being treated with an anticoagulant regimen, low-dose diuretics and continuous intravenous epoprostenol therapy. A computed tomography scan showed ancient massive thrombi in dilated central pulmonary arteries, which were not haemodynamically significant (perfusion lung scans did not demonstrate segmental or larger defects, and extensive dissection of the right pulmonary artery starting from the intermediate branch. Due to the extensiveness of the dissection, the patient was immediately considered for heart–lung transplantation, but died 72 h after the onset of symptoms. Permission for post mortem examination was denied. Pulmonary artery dissection should be suspected in PAH patients presenting with chest pain and worsening dyspnoea. In the current case, the factors possibly associated with increased risk for dissection may include dilatation of the pulmonary artery, local inflammation favoured by in situ thrombosis, and acute increase of pulmonary pressure secondary to physical exertion. Extensive pulmonary artery dissection is a life-threatening complication of PAH, and urgent heart/lung transplantation might be the treatment of choice in eligible patients. In addition, better identification of the risk factors for pulmonary artery dissection may help in considering transplantation for selected patients at risk.

  12. 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.

  13. Anomalous origin of right coronary artery from pulmonary artery

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    Rajat Gupta

    2012-01-01

    Full Text Available Anomalous origin of coronary artery from the pulmonary artery is a rare anomaly that most frequently involves the left coronary artery and very rarely the right coronary artery. These lesions can be missed on echocardiography unless carefully looked for. We describe a case of isolated anomalous origin of right coronary artery from pulmonary artery diagnosed on echocardiography and confirmed by computed tomography (CT angiography.

  14. Pulmonary Artery Intimal Sarcoma: A Case Report

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    Joseph P. Kriz

    2016-04-01

    Full Text Available Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung.

  15. Multiple coarctation of the pulmonary artery

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    Dicle, Oguz; Yilmaz, Erkan E-mail: eyilmaz@kordon.deu.edu.tr

    2000-12-01

    Pulmonary artery coarctation is a rare congenital anomaly characterized by single or multiple stenoses of the pulmonary arteries and their branches. There are only a few reports describing the radiological film of this entity. In this paper we report the case of a 68 year-old woman who presented with dyspnea. The scintigraphic, examination of the lungs gave the misleading impression of pulmonary embolism, and only angiography and magnetic resonance imaging established the correct diagnosis of multiple coarctations of the pulmonary artery.

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

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    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.

  17. 应用Swan-Ganz导管测定肺动脉高压犬模型肺循环阻力及心输出量的实验研究%Application of Swan-Ganz catheter to measure pulmonary vascular resistance and cardiac output of canine model with pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    林约瑟; 李淑娟; 李轩狄; 李运泉; 王慧深

    2015-01-01

    ObjectiveTo explore the value of Swan-Ganz catheter to measure pulmonary vascular resistance (PVR) and cardiac output (CO) of canine model with pulmonary arterial hypertension (PAH).MethodsSixteen Beagle dogs were randomly divided into two groups, the control group(n=8) and the pulmonary arterial hypertension group (n=8). Arterial blood was obtained to test arterial oxygen saturation. A Swan-Ganz catheter was advanced through the the right jugular vein way to the pulmonary artery and the pulmonary arterial pressure (PAP) were measured. Blood was obtained to test the pulmonary arterial oxygen saturation and plasma concentration of brain natriuretic peptide (BNP). Cardiac output and pulmonary vascular resistance were then calculated according to formula. The pulmonary arterial hypertension group were injected dehydromonocrataline 2.5 mg/kgto set up pulmonary arterial hypertension model. Hemodynamic measure was repeated after eight weeks.ResultsEight weeks after injection of dehydromonocrotaline, the pulmonary arterial systolic pressure, mean pulmonary arterial pressure of the pulmonary arterial hypertension group were significantly increased from (20.33±1.86) mmHg to (50.10±3.72) mmHg, (10.42±1.48) mmHg to (34.30±2.35) mmHg, the pulmonary arterial resistance, pulmonary vascular resistance were significantly increased from (1.10±0.24) Wood to (12.60±0.29) Wood, (2.34±0.33) Wood to (15.68±0.68) Wood, respectively, cardiac output was significantly decreased from (4.60±0.64) L/min to (2.30±0.35) L/min (P<0.05).ConclusionIt is an accurate, stable and repeatable method to apply Swan-Ganz catheter for measuring pulmonary vascular resistance and cardiac output in Beagle models with pulmonary arterial hypertension.%目的:探讨Swan-Ganz导管在肺动脉高压犬模型肺循环阻力及心输出量测定中的应用价值。方法16只比格犬随机分为2组,正常对照组(n=8)、肺动脉高压组(n=8)。穿刺动脉取血检测血氧饱和度。

  18. Hemoptysis and pulmonary artery agenesis: Case report

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    Lopez-Majano, V.; Sobti, P.

    1985-08-01

    The combination of a pulmonary scintigram using radioactively labeled albumin macroaggregates (MAA) and a study of the circulation in the bronchial artery was performed in one patient. This noninvasive methodology showed that there was increased circulation to the vascular territory of the lung in which the pulmonary artery was missing. This could have resulted from abnormal communications between the bronchial artery and the pulmonary vessels or an increased blood supply to the right lung from bronchial arteries arising from the aorta. The absence of pulmonary circulation in the right lung was proved by the absence of radioactivity in the right lung after an intravenous injection of labeled albumin MAA.

  19. 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.

    2012-01-01

    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 impo

  20. Pulmonary Artery Agenesis: A Case Series

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    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.

  1. Pulmonary arterial hypertension: Basic knowledge for clinicians.

    Science.gov (United States)

    Santos-Ribeiro, Diana; Mendes-Ferreira, Pedro; Maia-Rocha, Carolina; Adão, Rui; Leite-Moreira, Adelino F; Brás-Silva, Carmen

    2016-10-01

    Pulmonary arterial hypertension is a progressive syndrome based on diverse aetiologies, which is characterized by a persistent increase in pulmonary vascular resistance and overload of the right ventricle, leading to heart failure and death. Currently, none of the available treatments is able to cure pulmonary arterial hypertension; additional research is therefore needed to unravel the associated pathophysiological mechanisms. This review summarizes current knowledge related to this disorder, and the several experimental animal models that can mimic pulmonary arterial hypertension and are available for translational research.

  2. Left Anterior Descending Artery-Pulmonary Artery Fistula

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    Turan Ege

    2011-12-01

    Full Text Available Despite the fact that coronary arteriovenous fistulas constitute approximately half (48% of coronary artery anomalies, they are rarely seen anomalies. In this report,we aim to present a coronary arteriovenous fistula case detected during a coronary angiography between left anterior descending artery and pulmonary artery.

  3. Pulmonary arterial hypertension in children after neonatal arterial switch operation

    NARCIS (Netherlands)

    Zijlstra, Willemijn Mh; Elmasry, Ola; Pepplinkhuizen, Shari; Ivy, D Dunbar; Bonnet, Damien; Lévy, Marilyne; Gavilan, Jose Luis; Torrent-Vernetta, Alba; Mendoza, Alberto; Del Cerro, Maria Jesus; Moledina, Shahin; Berger, Rolf M. F.

    2017-01-01

    OBJECTIVES: Paediatric pulmonary arterial hypertension (PAH) after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA) is a clinically recognised entity with an estimated incidence of 0.6%-1.0%. Nevertheless, a clinical characterisation is lacking. We present an in

  4. Transcatheter rehabilitation of pulmonary arteries.

    Science.gov (United States)

    Thomson, John D R; Qureshi, Shakeel A

    2011-11-01

    Pulmonary arterial stenoses commonly occur in patients with congenital heart disease. Indications for treatment are based around reduction of right ventricular hypertension and equalization of flow to each lung and its constituent segments. There are many treatment approaches for these lesions depending on the anatomy, location and the age of the patient. Although surgical reconstruction remains an option, it is technically challenging and in many cases, the results are disappointing. In the modern era, the majority of these patients are treated with transcatheter therapy, including balloon angioplasty, and increasingly, the use of intravascular stents. In this article, we discuss these issues and deal with both the technical approach to transcatheter therapy and up-to-date results.

  5. Unilateral absence of a pulmonary artery: Report of 3 cases

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    Bae, Oh Keun; Choi, Chul Seung; Choi, Yo Won; Jeon, Seok Cheol; Seo, Heung Suk; Hahm, Chang Kok [College of Medicine, Hanyang University, Seoul (Korea, Republic of)

    1994-07-15

    Unilateral absence of a pulmonary artery is an uncommon anomaly, which presents as an isolated lesion or in combination with other congenital heart disease such as TOF or PDA. We encountered three cases of isolated unilateral absence of a pulmonary artery; one was left pulmonary artery agenesis with right sided aortic arch and the others were right pulmonary artery agenesis with left sided aortic arch. Plain chest radiograph showed considerable loss of unilateral lung volume and lack of ipsilateral hilar shadow. Pulmonary angiogram which was done in two cases, revealed proximal interruption of a pulmonary artery. Chest CT was done in only one case, on which right pulmonary artery was absent and was replaced by adipose tissue. CT with its clean demonstration pulmonary artery without any evidence of acquired obstruction of a pulmonary artery by pulmonary embolism or tumor invasion, maybe a valuable method for evaluation of the unilateral absence of a pulmonary artery.

  6. The arterial load in pulmonary hypertension

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    A. Vonk-Noordegraaf

    2010-09-01

    Full Text Available The anatomical differences between the pulmonary and systemic arterial system are the main cause of the difference in distribution of compliance. In the pulmonary arterial system compliance is distributed over the entire arterial system, and stands at the basis of the constancy of the RC-time. This distribution depends on the number of peripheral vessels, which is ∼8–10 times more in the pulmonary system than the systemic tree. In the systemic arterial tree the compliance is mainly located in the aorta (80% of total compliance in thoracic-abdominal aorta. The constant RC-time in the pulmonary bed results in proportionality of systolic and diastolic pressure with mean pressure and, in turn, in the constant ratio of oscillatory and mean power.

  7. Treatment options for paediatric pulmonary arterial hypertension

    NARCIS (Netherlands)

    Berger, R M F; Bonnet, D

    2010-01-01

    Pulmonary arterial hypertension (PAH) is a serious, progressive condition, which can present idiopathically or secondary to conditions such as systemic sclerosis or congenital heart disease. The condition exists in both adult and paediatric forms, which possess several similar characteristics. Adult

  8. Unusual migration of pulmonary artery catheter

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    Sanjay Kuravinakop

    2007-01-01

    Full Text Available Pulmonary artery catheter is widely used in intensive care. Distal migration of the catheter is a know complication. Diagnosis of such a migration is made by both clinical criteria and radiographs. A 55 year old septic lady was admitted to the intensive care unit. Pulmonary artery catheter introduced for cardiac output monitoring migrated from right lung to left lung. Diagnosis was made following a chest radiograph the following day of insertion with the clinical criteria remaining unaltered. Migration of pulmonary artery catheter can occur not only distally but from one lung to another. Clinical criteria alone cannot rule out migration. Chest radiographs form an important part in monitoring the position of the pulmonary artery catheter.

  9. Surgical repair of pulmonary artery branches.

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    Ghez, Olivier; Saeed, Imran; Serrato, Maria; Quintero, Diana Bernal; Kreitmann, Bernard; Fraisse, Alain; Uemura, Hideki; Seale, Anna; Daubeney, Piers; McCarthy, Karen; Ho, S Yen

    2013-01-01

    Surgical repair of pulmonary artery (PA) branches encompasses many different clinical scenarios and technical challenges. The most common, such as bifurcation and central PA reconstruction, are described, as well as the challenges of complex and peripheral reconstruction.

  10. Lineage Analysis in Pulmonary Arterial Hypertension

    Science.gov (United States)

    2013-06-01

    endothelial cells 12, 13. At the level of small pulmonary arteries, the occlusion by neointimal formation significantly exceeds muscularization of the...pyrrole. A, B) Normal muscular pulmonary artery (PA) adjacent to bronchiole (Br) A) hematoxylin and eosin stain (H&E), B) elastin-van Gieson stain (EVG...Iyer NV, Huso DL, Sun X, McWilliams R, Beaty T, Sham JS, Wiener CM, Sylvester JT, Semenza GL. Impaired physiological responses to chronic hypoxia in

  11. Isolated Left Pulmonary Artery Agenesis: A Case Report

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    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

  12. Anesthetic Management of Pediatric Pulmonary Arterial Hypertension

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    Mediha Turktan

    2015-06-01

    Full Text Available Pulmonary arterial hypertension is the most important cause of morbidity and mortality associated with congenital heart disease. Patients in this group have a greater peroperative cardiovascular risks including cardiac arrest, pulmonary hypertensive crisis and death compared the normal population. The main purpose of anesthesia is to avoid increased pulmonary vascular resistance and myocardial depression. [Archives Medical Review Journal 2015; 24(2.000: 149-158

  13. Idiopathic aneurysm of pulmonary artery

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    Pacheco, Julio B. Cota; Pimentel, Patricia N.; Knust, Beatriz S., E-mail: jcota@uol.com.br [Clinica de Cardiologia Cota Pacheco, Mogi das Cruzes, SP (Brazil)

    2015-07-15

    Because it is a very rare isolated lesion, we decided to present this case of idiopathic pulmonary artery aneurysm (IPAA) and review the cases published in the literature in order to correlate our clinical and imaging findings, as well as management based on patient data. IPAA was first described in a case of autopsy by Bristowe in 1860 and later in 1947 by Deterling and Claggett, whose prevalence was lower than eight to one hundred thousand. Although the use of diagnostic imaging methods has been applied in a very large population in recent decades, this lesion has been most often described in postmortem examinations. Therefore, it is important to be aware of possible clinical symptoms, at times non-specific, as well as the signs on imaging studies. In this study, therefore, the report of a case of an asymptomatic patient whose diagnosis was done through color Doppler echocardiography in a routine test in 2012, later confirmed by simple chest computed tomography (chest CT) and cardiac catheterization as IPAA and its branches. We discussed the literature available and the possibilities for treatment and the use of color Doppler echocardiography as an initial diagnostic tool for such a rare and intriguing disease. (author)

  14. Non-congenital heart disease associated pediatric pulmonary arterial hypertension

    OpenAIRE

    Ivy, D D; Feinstein, J. A.; Humpl, T; Rosenzweig, E. B.

    2009-01-01

    Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, he...

  15. Medical treatment update on pulmonary arterial hypertension.

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    Enderby, Cher Y; Burger, Charles

    2015-09-01

    Pulmonary arterial hypertension is a chronic, progressive disease of the pulmonary vasculature resulting in poor outcomes if left untreated. The management of group 1 pulmonary arterial hypertension has included the use of prostanoids, phosphodiesterase-5 inhibitors, and endothelin receptor antagonists targeting the prostacyclin, endothelin-1, and nitric oxide pathways. Three new medications have been approved by the US Food and Drug Administration over the past couple of years. Macitentan is the newest endothelin receptor antagonist, riociguat is a soluble guanylate cyclase stimulator, and treprostinil diolamine is the first oral prostanoid. This review will focus on the key trials leading to their approval, special considerations for each medication, and their potential place in therapy. The use of combination therapy as initial therapy in pulmonary arterial hypertension will also be discussed.

  16. Potassium channels in pulmonary arterial hypertension.

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    Boucherat, Olivier; Chabot, Sophie; Antigny, Fabrice; Perros, Frédéric; Provencher, Steeve; Bonnet, Sébastien

    2015-10-01

    Pulmonary arterial hypertension (PAH) is a devastating cardiopulmonary disorder with various origins. All forms of PAH share a common pulmonary arteriopathy characterised by vasoconstriction, remodelling of the pre-capillary pulmonary vessel wall, and in situ thrombosis. Although the pathogenesis of PAH is recognised as a complex and multifactorial process, there is growing evidence that potassium channels dysfunction in pulmonary artery smooth muscle cells is a hallmark of PAH. Besides regulating many physiological functions, reduced potassium channels expression and/or activity have significant effects on PAH establishment and progression. This review describes the molecular mechanisms and physiological consequences of potassium channel modulation. Special emphasis is placed on KCNA5 (Kv1.5) and KCNK3 (TASK1), which are considered to play a central role in determining pulmonary vascular tone and may represent attractive therapeutic targets in the treatment of PAH.

  17. Recent trends in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Rajagopalan Natarajan

    2011-01-01

    Full Text Available Pulmonary hypertension is a serious and unrelenting pulmonary vascular disorder that affects the functional quality of patients and significantly decreases their life span. If diagnosed early, with the number of new therapeutic options that are available, a better quality of life can be provided for a protracted length of time. It is likely that the available treatment will change the natural course of the disease and perhaps prolong survival. As symptoms are often subtle in the early stages of the disease it is imperative that physicians are aware of the manifestations of this condition. A thorough investigation of patients suspected of this condition is essential so that appropriate treatment can be initiated promptly. The routine workup of a patient suspected to have pulmonary hypertension could easily be carried out in any well-equipped peripheral hospital in many affluent and advanced countries. However, it must be mentioned that in some less advanced countries the necessary work up can only be done in major teaching hospitals. Both pulmonologists and cardiologists should be aware of the pathophysiology of pulmonary arterial hypertension, the workup and the treatment options that are available. Patients with refractory pulmonary hypertension should be referred to these research centers for enrolment into any ongoing drug trials as well as for evaluation for heart−lung, single lung, or double lung transplantation. This paper is primarily aimed at pulmonologists and cardiologists taking care of these patients. Unless indicated otherwise this paper mainly deals with WHO group 1 pulmonary hypertension which is designated pulmonary arterial hypertension. Extensive review of the literature spanning the last 30 years was made through Medline using titles such as primary pulmonary hypertension, pulmonary arterial hypertension, secondary pulmonary hypertension, and pulmonary vascular diseases.

  18. Pulmonary Artery Agenesis with Bronchial Asthma

    Directory of Open Access Journals (Sweden)

    Hassan Ghobadi

    2014-05-01

    Full Text Available Unilateral Pulmonary Artery Agenesis (UPAA is a rare congenital anomaly during the 4 th  week of gestational age. It is defined as an absence of pulmonary parenchyma and its supporting artery. A 9-year-old girl was admitted to our hospital because of chronic cough. Chest examination showed a decrement in lung sound of right hemi-thorax with expiratory wheeze. Chest radiography (CXR revealed a semi-opaque right hemi-thorax. Chest CT with intra-venous contrast demonstrated absence of the right pulmonary artery and lung parenchyma with hyper-inflated left lung and dextro-position of mediastinum. This case emphasizes that in patients with respiratory compliant and chronic cough CXR must be done to rule out similar diagnosis other than asthma.    

  19. Management of pulmonary arterial hypertension.

    Science.gov (United States)

    McLaughlin, Vallerie V; Shah, Sanjiv J; Souza, Rogerio; Humbert, Marc

    2015-05-12

    Pulmonary hypertension (PH) is common and may result from a number of disorders, including left heart disease, lung disease, and chronic thromboembolic disease. Pulmonary arterial hypertension (PAH) is an uncommon disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in elevated pulmonary vascular resistance and, eventually, in right ventricular failure. Over the past decades, knowledge of the basic pathobiology of PAH and its natural history, prognostic indicators, and therapeutic options has exploded. A thorough evaluation of a patient is critical to correctly characterize the PH. Cardiac studies, including echocardiography and right heart catheterization, are key elements in the assessment. Given the multitude of treatment options currently available for PAH, assessment of risk and response to therapy is critical in long-term management. This review also underscores unique situations, including perioperative management, intensive care unit management, and pregnancy, and highlights the importance of collaborative care of the PAH patient through a multidisciplinary approach.

  20. Role of oxidized lipids in pulmonary arterial hypertension

    OpenAIRE

    Sharma, Salil; Ruffenach, Grégoire; Umar, Soban; Motayagheni, Negar; Reddy, Srinivasa T.; Eghbali, Mansoureh

    2016-01-01

    Pulmonary arterial hypertension (PAH) is a multifactorial disease characterized by interplay of many cellular, molecular, and genetic events that lead to excessive proliferation of pulmonary cells, including smooth muscle and endothelial cells; inflammation; and extracellular matrix remodeling. Abnormal vascular changes and structural remodeling associated with PAH culminate in vasoconstriction and obstruction of pulmonary arteries, contributing to increased pulmonary vascular resistance, pul...

  1. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology

    Directory of Open Access Journals (Sweden)

    M. Humbert

    2010-03-01

    Full Text Available Pulmonary arterial hypertension (PAH and chronic thromboembolic pulmonary hypertension (CTEPH are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2, HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis, and exposure to particular drugs and toxins including fenfluramine derivatives. In contrast, CTEPH can be associated with anti-phospholipid antibodies, splenectomy and the presence of a ventriculo-atrial shunt or an infected pacemaker. The first-line therapies used to treat PAH and CTEPH also differ. While medical therapy tends to be used for patients with PAH, pulmonary endarterectomy is the treatment of choice for patients with CTEPH. However, there are possible common mechanisms behind the two diseases, including endothelial cell dysfunction and distal pulmonary artery remodelling. Further research into these similarities is needed to assist the development of targeted pharmacological therapies for patients with inoperable CTEPH and patients who have persistent pulmonary hypertension after endarterectomy.

  2. 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.

  3. Coronary Artery and Pulmonary Artery Fistula Originated from Significant Stenosis in the Left Anterior Descending Artery

    Directory of Open Access Journals (Sweden)

    Alper Sami Kunt

    2013-01-01

    Full Text Available Coronary artery fistula (CAF is defined as a rare anomalous connection between a coronary artery and a major vessel or a cardiac chamber. We report a case of a left anterior descending coronary (LAD stenosis and coronary artery fistula between the LAD coronary artery and the pulmonary artery (PA. CAF is often diagnosed by coronary angiogram. We describe our diagnostic approach and review the literature on the epidemiology, the pathophysiology, the diagnostic modalities, and the treatment options.

  4. Determinants of an elevated pulmonary arterial pressure in patients with pulmonary arterial hypertension.

    Science.gov (United States)

    Sakao, Seiichiro; Voelkel, Norbert F; Tanabe, Nobuhiro; Tatsumi, Koichiro

    2015-01-01

    Given the difficulty of diagnosing early-stage pulmonary arterial hypertension (PAH) due to the lack of signs and symptoms, and the risk of an open lung biopsy, the precise pathological features of presymptomatic stage lung tissue remain unknown. It has been suggested that the maximum elevation of the mean pulmonary arterial pressure (P pa) is achieved during the early symptomatic stage, indicating that the elevation of the mean P pa is primarily driven by the pulmonary vascular tone and/or some degree of pulmonary vascular remodeling completed during this stage. Recently, the examination of a rat model of severe PAH suggested that the severe PAH may be primarily determined by the presence of intimal lesions and/or the vascular tone in the early stage. Human data seem to indicate that intimal lesions are essential for the severely increased pulmonary arterial blood pressure in the late stage of the disease.However, many questions remain. For instance, how does the pulmonary hemodynamics change during the course of the disease, and what drives the development of severe PAH? Although it is generally acknowledged that both pulmonary vascular remodeling and the vascular tone are important determinants of an elevated pulmonary arterial pressure, which is the root cause of the time-dependent progression of the disease? Here we review the recent histopathological concepts of PAH with respect to the progression of the lung vascular disease.

  5. [Pulmonary arterial hypertension: a flavor of autoimmunity].

    Science.gov (United States)

    Perros, Frédéric; Humbert, Marc; Cohen-Kaminsky, Sylvia

    2013-01-01

    It is admitted that autoimmunity results from a combination of risks such as genetic background, environmental triggers, and stochastic events. Pulmonary arterial hypertension (PAH) shares with the so-called prototypic autoimmune diseases, genetic risk factors, female predominance and sex hormone influence, association with other chronic inflammatory and autoimmune diseases, defects in regulatory T cells function, and presence of autoantibodies. Case reports have been published indicating the beneficial effect of some immunosuppressive and anti-inflammatory therapies in PAH, supporting the potential role of immune mechanisms in the pathophysiology of the disease. In this review, we discuss the current knowledge on autoimmune mechanisms operating in PAH, especially mounting a local autoimmune response inside the pulmonary tissue, namely pulmonary lymphoid neogenesis. A better understanding of the role of autoimmunity in pulmonary vascular remodelling may help develop targeted immunomodulatory strategies in PAH.

  6. Left Coronary Artery-Pulmonary Artery Fistula in Coronary Artery Disease

    Directory of Open Access Journals (Sweden)

    Orhan Veli Doğan

    2012-08-01

    Full Text Available Coroner artery fistula which is rare and congenital or acquired arise from whole coroner artery drainage all of cardiac chamber and great artery. Although coroner artery fistula is uncommon, it can cause significant mortality and morbidity rates. The article is presented which coroner artery disease and coroner-pulmonary artery fistula was accomplishment committed. Sixty three year-old male patient admitted to the hospital with chest pain. Non-ST myocardial infarction was diagnosed in the examinations. After coroner angiography, it was found coronary artery disease in addition between LAD proximal portion and main pulmonary artery fistula. Fistula repair and coronary bypass were performed successfully under cardiopulmonary bypass. Without hemodynamic problem in intensive care and service follow-up, the patient was discharged from the hospital in the seventh postoperative day. We think that surgical treatment of coronary fistulas in patients with coronary artery lesion is done at the earliest time would enable improvement in mortality and morbidity rates.

  7. A case of left main pulmonary artery aneurysm associated with valvular pulmonary stenosis in a child.

    Science.gov (United States)

    Lee, Ran; Son, Jae Sung; Park, Yong Mean

    2011-10-01

    Aneurysm of the main pulmonary artery is a rare clinical entity that can be congenital or acquired. Most cases occur in association with other congenital malformations, severe pulmonary hypertension, vasculitides, infectious agents, or collagen vascular disorders. We report here a pediatric case of left pulmonary artery aneurysm associated with valvular pulmonary stenosis and a hypoplastic right pulmonary artery, which we confirmed via multidetector computed tomography angiography.

  8. Isolated Left Pulmonary Artery Agenesis: A Case Report

    OpenAIRE

    Tansel Ansal Balcı; Zehra Pınar Koç; Gamze Kırkıl; Ahmet Kürşad Poyraz

    2012-01-01

    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 w...

  9. Recent Strategies in Treatment of Pulmonary Arterial Hypertension, A Review

    OpenAIRE

    Fallah, Flora

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a disease characterized by an elevation in pulmonary artery pressure that can lead to right ventricular failure and death. The pulmonary circulation has to accommodate the entire cardiac output in each cardiac cycle and evolution has adapted to this by making it a low-pressure high-flow system. However, pathology can affect both the arterial and venous components of this system. Pulmonary venous hypertension mainly refers to diseases that result in ele...

  10. Endovascular Thrombin Injection for a Pulmonary Artery Pseudoaneurysm: Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Jin Ho; Shin, Ji Hoon; Yoon, Hyun Ki [Dept. of Radiology and Research Institute of Radiology, Asan Medical Center, Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2011-12-15

    Massive hemoptysis caused by pulmonary artery pseudoaneurysms is uncommon, and endovascular treatment such as coil embolization is the first choice for treating pulmonary artery pseudoaneurysms. Various embolic agents could be used according to the angiographic findings, yet embolization with thrombin injection is very rare. Herein, we describe a case of a pulmonary artery pseudoaneurysm that was successfully treated by endovascular thrombin injection using a microcatheter because of the difficulty in performing a coil embolization due to a short feeding artery.

  11. Iron deficiency in patients with idiopathic pulmonary arterial hypertension

    NARCIS (Netherlands)

    van Empel, Vanessa P M; Lee, Joy; Williams, Trevor J; Kaye, David M

    2014-01-01

    BACKGROUND: Iron deficiency has been reported to be highly prevalent in idiopathic pulmonary arterial hypertension (iPAH) patients, with the potential to influence cardiac performance, pulmonary artery pressures and the pulmonary vascular response to hypoxia. METHODS: Iron status was evaluated in 29

  12. Intimal sarcoma of the pulmonary artery--diagnostic challenge.

    Science.gov (United States)

    Fukuda, Wakako; Morohashi, Satoko; Fukuda, Ikuo

    2011-08-01

    Pulmonary artery intimal sarcoma is a rare tumour and the diagnosis is often delayed. We report the case of a woman with a primary pulmonary artery intimal sarcoma who presented with massive pulmonary embolism. The definitive diagnosis was elucidated after the patient's death by autopsy specimen. We discuss the diagnosis and lessons learned from this case.

  13. [Novel immunopathological approaches to pulmonary arterial hypertension].

    Science.gov (United States)

    Perros, Frédéric; Montani, David; Dorfmüller, Peter; Huertas, Alice; Chaumais, Marie-Camille; Cohen-Kaminsky, Sylvia; Humbert, Marc

    2011-04-01

    Inflammation is important for the initiation and the maintenance of vascular remodeling in the most commun animal models of pulmonary hypertension (PH), and its therapeutical targeting blocks PH development in these models. In human, pulmonary vascular lesions of PH are also the source of an intense chemokine production, linked to inflammatory cell recruitment. However, arteritis is uncommon in PH patients. Of note, current PH treatments have immunomodulatory properties. In addition, some studies have shown a correlation between levels of circulating inflammatory mediators and patients' survival. The study of autoimmunity in the pathophysiology of pulmonary arterial hypertension is becoming an area of intense investigation. New immunopathological approaches to PH should allow the development of innovative treatments for this very severe condition.

  14. Recurrent ischemia resulting from left internal mammary artery-to-pulmonary artery fistula.

    Science.gov (United States)

    Madu, E C; Hanumanthu, S K; Kim, C; Prudoff, A

    2001-03-01

    This report describes a case series of recurrent ischemia after coronary artery bypass grafting resulting from left internal mammary artery-to-pulmonary artery fistula. An angiographic demonstration of this fistula is presented.

  15. Pulmonary arterial wall distensibility assessed by intravascular ultrasound in children with congenital heart disease : an indicator for pulmonary vascular disease?

    NARCIS (Netherlands)

    Berger, Rolf M F; Cromme-Dijkhuis, Adri H; Hop, Wim C J; Kruit, Marco N; Hess, John; Berger, Rudolphus

    2002-01-01

    BACKGROUND: Both pulmonary hypertension and pulmonary overflow are associated with functional and structural changes of the pulmonary arterial wall. Current techniques to evaluate the pulmonary vasculature neglect the pulsatile nature of pulmonary flow. STUDY OBJECTIVES: To determine whether the dyn

  16. Pulmonary artery haemodynamic properties in patients with pulmonary hypertension secondary to rheumatic mitral stenosis.

    Science.gov (United States)

    Yan, Tao; Zhang, Guan-xin; Li, Bai-lin; Zhong, Keng; Xu, Zhi-yun; Han, Lin

    2012-12-01

    We sought to explore the pulmonary haemodynamic changes in rheumatic mitral stenosis patients with secondary pulmonary hypertension. The pulmonary artery resistance and compliance of 35 patients with rheumatic mitral stenosis and 12 controls without cardiopulmonary vascular disease were evaluated by using an improved method, which is based on making calculations with parameters obtained from right heart catheterisation. The results are as follows: (1) pulmonary artery compliance in patients with secondary pulmonary hypertension was significantly lower than that of the control group (P0.05) The walls of pulmonary artery vessels in patients with pulmonary hypertension secondary to rheumatic mitral stenosis appeared to be remodelled by varying degrees as indicated by their haemodynamic properties. Structural remodelling may be a factor affecting preoperative pulmonary artery pressure. Mitral stenosis patients with severe pulmonary hypertension have significantly lower responses to sodium nitroprusside possibly due to aggradation and deposition of collagen in the artery walls, decreasing constriction and dilation, or atrophy of smooth muscle cells.

  17. The Warburg effect: A new story in pulmonary arterial hypertension.

    Science.gov (United States)

    Peng, Hongyan; Xiao, Yunbin; Deng, Xicheng; Luo, Jingfei; Hong, Chenliang; Qin, Xuping

    2016-10-01

    Pulmonary arterial hypertension (PAH) is a rare yet fatal condition that is characterized by a continuous and notable elevation of pulmonary arterial pressure (PAP), resulting in right heart failure and death. Pulmonary arterial remodelling does not result from abnormal proliferation of pulmonary arterial vascular smooth muscle cells (PASMCs) but from pulmonary arterial endothelial cell (PAEC) dysfunction. However, the pathological mechanism of these two types of vascular cells in pulmonary artery remodelling is unclear. The Warburg effect describes aerobic glycolysis wherein cells commonly reprogram their energy metabolism to preferentially utilize glycolysis over oxidative phosphorylation for ATP production. Recent research has demonstrated that the Warburg effect plays a significant role in the development of PAH, which involves the abnormal proliferation of PASMCs and endothelial dysfunction. This review attempts to illustrate the functions of the Warburg effect in PAH, which may provide a new therapeutic target for PAH treatment.

  18. Recapitulation of Developing Artery Muscularization in Pulmonary Hypertension

    Directory of Open Access Journals (Sweden)

    Abdul Q. Sheikh

    2014-03-01

    Full Text Available Excess smooth muscle accumulation is a key component of many vascular disorders, including atherosclerosis, restenosis, and pulmonary artery hypertension, but the underlying cell biological processes are not well defined. In pulmonary artery hypertension, reduced pulmonary artery compliance is a strong independent predictor of mortality, and pathological distal arteriole muscularization contributes to this reduced compliance. We recently demonstrated that embryonic pulmonary artery wall morphogenesis consists of discrete developmentally regulated steps. In contrast, poor understanding of distal arteriole muscularization in pulmonary artery hypertension severely limits existing therapies that aim to dilate the pulmonary vasculature but have modest clinical benefit and do not prevent hypermuscularization. Here, we show that most pathological distal arteriole smooth muscle cells, but not alveolar myofibroblasts, derive from pre-existing smooth muscle. Furthermore, the program of distal arteriole muscularization encompasses smooth muscle cell dedifferentiation, distal migration, proliferation, and then redifferentiation, thereby recapitulating many facets of arterial wall development.

  19. A Review of Targeted Pulmonary Arterial Hypertension-Specific Pharmacotherapy

    OpenAIRE

    Ataya, Ali; Cope, Jessica; Alnuaimat, Hassan

    2016-01-01

    Significant advances in the understanding of the pathophysiology of pulmonary arterial hypertension over the past two decades have led to the development of targeted therapies and improved patient outcomes. Currently, a broad armamentarium of pulmonary arterial hypertension-specific drugs exists to assist in the treatment of this complex disease state. In this manuscript, we provide a comprehensive review of the current Food and Drug Administration (FDA)-approved pulmonary arterial hypertensi...

  20. Prognostic markers for idiopathic pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    Guo Xiaomin; Jin Hongfang; Du Junbao

    2014-01-01

    Objective The objective of this study is to review the research on the prognostic markers of idiopathic pulmonary arterial hypertension (IPAH).Date sources We searched literature from PubMed and CNKI databases both in English and Chinese up to 2013.Study selection Data about mortality and cut-off value are from clinical trials and identified by analysis.Results IPAH is an unexplained,progressive,and rare disease characterized by increased pulmonary artery pressure and pulmonary vascular resistance.The diagnosis is difficult,mortality of IPAH is high,and the survival periods are only 2-3 years after diagnosis.Investigations in recent years have identified a range of prognostic markers for IPAH,including the 6-minute walking test,red blood cell distribution width,and platelet levels,as well as imaging findings.Changes in these markers are important sources of information to predict the prognosis of patients with IPAH,which carries significant benefits for treatment planning.Conclusion Even though the prognosis of IPAH has been investigated,the mortality is also high.More accurate and meaningful assessment for the prognosis of IPAH is required.

  1. Isolated absence of right pulmonary artery

    Directory of Open Access Journals (Sweden)

    Singhi Anil

    2010-01-01

    Full Text Available Background : Absence of right pulmonary artery (RPA is a rare congenital abnormality with variable presentation. The diagnosis is often missed in infants Objective : The aim of this study was to describe infantile presentation of isolated absence of RPA along with a brief review of the literature. Methods : The details of five patients diagnosed with isolated absence of RPA from April 2007 to October 2009 were reviewed retrospectively Results : Five patients were identified with this anomaly. The median age of presentation was 86 days (range, 40-120 days and the median weight was 3.65 kg (range, 3.1-5.5 kg. All patients presented with breathing difficulty and had severe pulmonary hypertension (PHT along with absent RPA on echocardiography. A multidetector computed tomographic scan was performed to confirm the diagnosis in four cases. Three patients had major aortopulmonary collateral and the hilar RPA was not well developed in all. A correct diagnosis was made before referral in one patient only. Differential vascularity in chest X-ray, a useful clue, was seen in four of five cases. Surgical correction was not considered in view of the small hilar pulmonary artery. The patients were all managed medically with diuretics and Sildenafil. Conclusion : Isolated absence of RPA is a rare congenital abnormality with varied presentation. Infantile presentation is marked with congestive cardiac failure and PHT. Specific diagnostic clue includes differential vascularity on the chest X-ray.

  2. 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.

  3. Pulmonary artery agenesis associated with coronary collaterals among adults.

    Science.gov (United States)

    Darwazah, Ahmad K; Alhaddad, Imad A

    2016-07-16

    Unilateral agenesis of the pulmonary artery is a rare congenital anomaly, which commonly involves the right side. Cases are associated with systemic collaterals, that may also rarely arise from the coronary arteries.Two adult patients are presented with a right pulmonary artery agenesis associated with collaterals from the right coronary artery. The implications of such an anomaly on pulmonary artery pressure and lung pathology differs among both cases. The association of coronary collaterals is rare and its implication is variable among various patients.

  4. Extensive pulmonary sarcoid reaction in a patient with BMPR-2 associated idiopathic pulmonary arterial hypertension

    NARCIS (Netherlands)

    Braam, Evelien A J E; Quanjel, Marian J R; Van Haren-Willems, Jolanda H G M; Van Oosterhout, Matthijs F M; Vink, Aryan; Heijdra, Yvonne F; Kwakkel-van Erp, Johanna M

    2016-01-01

    Pulmonary arterial hypertension is a progressive life-threatening disease characterized by vascular remodeling. There is evidence that varied immune mechanism play an important role in progression of pulmonary hypertension. We describe a case of a 35-year-old woman with idiopathic pulmonary arterial

  5. Pulmonary oligemia maneuver can alleviate pulmonary artery injury during pulmonary thromboendarterectomy procedure

    Institute of Scientific and Technical Information of China (English)

    GAN Hui-li; ZHANG Jian-qun; LU Jia-kai; DONG Xiu-hua; HOU Xiao-tong; GAO Yuan-ming; ZHU Guang-fa

    2013-01-01

    Background Pulmonary thromboendarterectomy (PTE) has evolved as a treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH).This study aimed to characterize if pulmonary oligemia maneuver (POM) can alleviate pulmonary artery injury during PTE procedure.Methods A total of 112 cases of CTEPH admitted to Beijing Anzhen Hospital from March 2002 to August 2011 received PTE procedure.They were retrospectively classified as non-POM group (group A,n=55) or POM group (group B,n=57).Members from group B received POM during rewarming period,whereas members from group A did not.Results There were three (5.45%) early deaths in group A,no death in group B (0) (Fisher's exact test,P=-0.118).Six patients in group A needed extracorporeal membrane oxygenation (ECMO) as life support after the PTE procedure,no patients in group B needed ECMO (Fisher's exact test,P=0.013).The patients in group B had a shorter intubation and ICU stay,lower mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR),higher partial pressure of oxygen in artery (PaO2) and arterial oxygen saturation (SaO2) and less medical expenditure than patients in group A.With a mean follow-up time of (58.3 ± 30.6) months,two patients in group A and one patient in group B died.The difference of the actuarial survival after the procedure between the two groups did not reach statistical significance.Three months post the PTE procedure,the difference of residual occluded pulmonary segment between the two groups did not reach statistical significance (P=-0.393).Conclusion POM can alleviate pulmonary artery injury,shorten ICU stay and intubation time,and lower down the rate of ECMO after PTE procedure.

  6. "Nocturnal seizures" in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Izzo, Anthony; McSweeney, Julia; Kulik, Thomas; Khatwa, Umakanth; Kothare, Sanjeev V

    2013-10-15

    The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children.

  7. Treatment of idiopathic/hereditary pulmonary arterial hypertension.

    Science.gov (United States)

    Matsubara, Hiromi; Ogawa, Aiko

    2014-10-01

    Treatment of pulmonary hypertension has progressed by recently developed pulmonary arterial hypertension-targeted drugs. However, long-term survival of the patients with idiopathic/heritable pulmonary arterial hypertension is still suboptimal. To improve the outcomes, treatment goals of pulmonary hypertension were proposed at the 5th World Symposium on Pulmonary Hypertension held at Nice, France in 2013; parameters were obtained from cardiopulmonary exercise test, blood tests, echocardiography, and magnetic resonance imaging. In particular, parameters evaluating right ventricular function have been highlighted because survival of the patients with pulmonary arterial hypertension is closely related to right ventricular function. However, treatment specifically targeted to improve right ventricular function in pulmonary hypertension is not yet established. In this setting, we need to maintain or improve right ventricular function with available vasodilators. In this review, we focus on the following two points: (1) Why can pulmonary arterial hypertension-targeted drugs improve right ventricular function without an apparent decrease in pulmonary artery pressure? (2) Are proposed goals sufficient to improve long-term prognosis of the patients? Further, we will discuss what would be the appropriate goal in treating patients with pulmonary arterial hypertension.

  8. Association of pulmonary artery agenesis and hypoplasia of the lung.

    Science.gov (United States)

    Vitiello, Renato; Pisanti, Chiara; Pisanti, Antonello; Silberbach, Michael

    2006-09-01

    Pulmonary artery agenesis and hypoplasia of the homolateral lung occasionally occurs as an isolated lesion, but more often has associated congenital cardiac anomalies. We present a case where pulmonary artery agenesis was the sole lesion in an asymptomatic child. Pulmonary artery agenesis should be suspected in asymptomatic patients if a plain chest X-ray shows asymmetric lung fields, lung hypoplasia, or hyperinflation of the contralateral lung. Echocardiography is the best tool to establish the diagnosis. In our opinion, invasive procedures, such as cardiac catheterization, may be postponed if there is no echocardiographic evidence of pulmonary hypertension.

  9. Pathogenic Mechanisms of Pulmonary Arterial Hypertension

    Science.gov (United States)

    Chan, Stephen Y.; Loscalzo, Joseph

    2008-01-01

    Pulmonary arterial hypertension (PAH)1 is a complex disease that causes significant morbidity and mortality and is clinically characterized by an increase in pulmonary vascular resistance. The histopathology is marked by vascular proliferation/fibrosis, remodeling, and vessel obstruction. Development of PAH involves the complex interaction of multiple vascular effectors at all anatomic levels of the arterial wall. Subsequent vasoconstriction, thrombosis, and inflammation ensue, leading to vessel wall remodeling and cellular hyperproliferation as the hallmarks of severe disease. These processes are influenced by genetic predisposition as well as diverse endogenous and exogenous stimuli. Recent studies have provided a glimpse at certain molecular pathways that contribute to pathogenesis; these have led to the identification of attractive targets for therapeutic intervention. We will review our current understanding of the mechanistic underpinnings of the genetic and exogenous/acquired triggers of PAH. The resulting imbalance of vascular effectors provoking pathogenic vascular changes will also be discussed, with an emphasis on common and overarching regulatory pathways that may relate to the primary triggers of disease. The current conceptual framework should allow for future studies to refine our understanding of the molecular pathogenesis of PAH and improve the therapeutic regimen for this disease. PMID:17950310

  10. Regression of pulmonary artery hypertension due to development of a pulmonary arteriovenous malformation

    Science.gov (United States)

    Hasan, Ashfaq; Sastry, B.K.S.; Aleem, M.A.; Reddy, Gokul; Mahmood, Syed

    2014-01-01

    Idiopathic Pulmonary Hypertension (IPAH) is characterized by elevated pulmonary arterial pressure in the absence of an identifiable underlying cause. The condition is usually relentlessly progressive with a short survival in the absence of treatment.1 We describe a patient of IPAH in whom the pulmonary artery pressures significantly abated with complete disappearance of symptoms, following spontaneous development of a pulmonary arterio-venous malformation (PAVM). PMID:25443608

  11. Incidentally detected right pulmonary artery agenesis with right coronary artery collateralization.

    Science.gov (United States)

    Mikaberidze, Nino; Goldberg, Ythan; Khosraviani, Khashayar; Taub, Cynthia

    2014-01-01

    Unilateral pulmonary artery agenesis (UPAA) with pulmonary hypoplasia is a rare congenital anomaly. We describe a 71-year old male who was incidentally diagnosed with the right UPAA and a hypoplastic right lung supplied by collateralized right coronary.

  12. 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.

  13. Congenital right pulmonary artery agenesis with atrial septal defect and pulmonary hypertension.

    Science.gov (United States)

    Orun, Utku Arman; Yilmaz, Osman; Bilici, Meki; Karademir, Selmin; Uner, Cigdem; Senocak, Filiz; Dogan, Vehbi

    2012-01-01

    Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.

  14. Pulmonary artery dilatation: an overlooked mechanism for angina pectoris.

    Science.gov (United States)

    Ginghina, Carmen; Popescu, Bogdan A; Enache, Roxana; Ungureanu, Catalina; Deleanu, Dan; Platon, Pavel

    2008-07-01

    Dilatation of the pulmonary artery may lead to the compression of adjacent structures. Of those, the extrinsic compression of the left main coronary artery is the most worrisome. We present the case of a 48-year-old woman who was diagnosed with pulmonary artery dilatation due to severe, thromboembolic pulmonary hypertension. She also had angina and coronary angiography revealed a 70% ostial stenosis of the left main coronary artery. The presence of this isolated lesion in a young woman without risk factors for atherosclerosis suggests extrinsic compression of the left main coronary artery by the dilated pulmonary artery as the likely mechanism. The patient underwent direct stenting of the left main coronary stenosis with a good result.

  15. Sodium hydrosulfide alleviates pulmonary artery collagen remodeling in rats with high pulmonary blood flow.

    Science.gov (United States)

    Li, Xiaohui; Du, Junbao; Jin, Hongfang; Geng, Bin; Tang, Chaoshu

    2008-11-01

    This study aimed to explore the effect of sodium hydrosulfide (NaHS) on pulmonary artery collagen remodeling in rats with high pulmonary blood flow. Thirty-two Sprague-Dawley rats were randomly divided into a sham group, shunt group, sham + NaHS (an H2S donor) group, and shunt + NaHS group. After 11 weeks of shunting, mean pulmonary artery pressure (MPAP), relative median area (RMA) of pulmonary arteries, H2S concentration in lung tissues, plasma endothelin-1 (ET-1) levels, and ET-1 mRNA in lung tissues were investigated. Collagen I and collagen III were evaluated by immunohistochemistry. Hydroxyproline assay and Sirius-red staining were performed. Matrix metalloproteinase-13 (MMP-13), tissue inhibitor of metalloproteinase-1 (TIMP-1), and connective tissue growth factor (CTGF) were evaluated by immunohistochemistry. After 11 weeks of shunting, rats showed a significant pulmonary hypertension and pulmonary artery collagen remodeling in association with a decrease in lung tissue H2S content. After NaHS treatment for 11 weeks, lung tissue H(2)S content was increased, whereas MPAP was attenuated and RMA was reduced. Meanwhile, pulmonary artery collagen I and collagen III protein expressions of intra-acinar pulmonary arteries were inhibited, but MMP-13/TIMP-1 ratio was augmented with a decreased plasma ET-1 content and lung tissue ET-1mRNA and CTGF expressions. The downregulation of H(2)S is involved in the development of pulmonary artery collagen remodeling induced by high pulmonary blood flow.

  16. Low-pressure pulmonary artery aneurysm presenting with pulmonary embolism: a case series

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    Papoulidis Pavlos

    2011-04-01

    Full Text Available Abstract Introduction Pulmonary artery aneurysm is an uncommon disorder with severe complications. The diagnosis is often difficult, since the clinical manifestations are non-specific and the treatment is controversial, as the natural history of the disease is not completely understood. Case presentation We describe the cases of two patients with pulmonary artery aneurysms. The first patient was a 68-year-old Caucasian man with an idiopathic low-pressure pulmonary artery aneurysm together with a pulmonary embolism. The patient preferred a conservative approach and was stable at the 10-month follow-up visit after being placed on anti-coagulant treatment. The second patient was a 66-year-old Caucasian woman with a low-pressure pulmonary artery aneurysm also presented together with a pulmonary embolism. The aneurysm was secondary to pulmonary valve stenosis. She received anti-coagulants and, after stabilization, underwent percutaneous balloon valvuloplasty. Conclusion Pulmonary embolism may be the initial presentation of a low-pressure pulmonary artery aneurysm. No underlying cause for pulmonary embolism was found in either of our patients, suggesting a causal association with low-pressure pulmonary artery aneurysm.

  17. Arterial pulmonary hypertension in noncardiac intensive care unit

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    Mykola V Tsapenko

    2008-10-01

    Full Text Available Mykola V Tsapenko1,5, Arseniy V Tsapenko2, Thomas BO Comfere3,5, Girish K Mour1,5, Sunil V Mankad4, Ognjen Gajic1,51Division of Pulmonary and Critical Care Medicine; 3Division of Critical Care Medicine; 4Division of Cardiovascular Diseases, Mayo Epidemiology and Translational Research in Intensive Care (M.E.T.R.I.C, Mayo Clinic, Rochester, MN, USA; 2Division of Pulmonary and Critical Care Medicine, Brown University, Miriam Hospital, Providence, RI, USAAbstract: Pulmonary artery pressure elevation complicates the course of many complex disorders treated in a noncardiac intensive care unit. Acute pulmonary hypertension, however, remains underdiagnosed and its treatment frequently begins only after serious complications have developed. Significant pathophysiologic differences between acute and chronic pulmonary hypertension make current classification and treatment recommendations for chronic pulmonary hypertension barely applicable to acute pulmonary hypertension. In order to clarify the terminology of acute pulmonary hypertension and distinguish it from chronic pulmonary hypertension, we provide a classification of acute pulmonary hypertension according to underlying pathophysiologic mechanisms, clinical features, natural history, and response to treatment. Based on available data, therapy of acute arterial pulmonary hypertension should generally be aimed at acutely relieving right ventricular (RV pressure overload and preventing RV dysfunction. Cases of severe acute pulmonary hypertension complicated by RV failure and systemic arterial hypotension are real clinical challenges requiring tight hemodynamic monitoring and aggressive treatment including combinations of pulmonary vasodilators, inotropic agents and systemic arterial vasoconstrictors. The choice of vasopressor and inotropes in patients with acute pulmonary hypertension should take into consideration their effects on vascular resistance and cardiac output when used alone or in

  18. A CASE OF IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION IN MALE

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    Poongavanam Paranthaman

    2016-08-01

    Full Text Available 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 case of middle-aged male who presented with dyspnoea and on further evaluation found to have primary pulmonary hypertension, which is uncommon in male

  19. Longitudinal Deformation of Pulmonary Artery: A Case Series Study

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    Ali Hosseinsabet

    2013-12-01

    Full Text Available Objectives:: In this study, pulmonary artery longitudinal deformation was evaluated and attempted to clarify pulmonary artery deformation. Methods:: Eight healthy men and seven healthy women with structurally normal hearts were enrolled. In the parasternal short axis view at the aortic valve level, longitudinal axis of pulmonary artery was obtained and color coded Doppler myocardial imaging applied. Results:: The systolic strain was positive in most the pulmonary segments, velocity and strain rate waves had three defined peaks, one positive in systole and two negative in diastole. Conclusions:: This study revealed for first time, deformation pattern of pulmonary artery by color coded Doppler myocardial imaging and can be basis for future researches in cardiopulmonary diseases.

  20. Current insights on the pathogenesis of pulmonary arterial hypertension.

    Science.gov (United States)

    Perros, Frédéric; Dorfmüller, Peter; Humbert, Marc

    2005-08-01

    Regardless of the initial trigger, the elevated pulmonary arterial pressure and vascular resistance in patients with pulmonary arterial hypertension are primarily caused by remodeling and thrombosis of small- and medium-sized pulmonary arteries and arterioles, as well as sustained vasoconstriction. The process of pulmonary vascular remodeling involves all layers of the vessel wall and is complicated by cellular heterogeneity within each compartment. Indeed, each cell type (endothelial cells, smooth muscle cells, and fibroblasts), as well as inflammatory cells and platelets, may play significant roles in this condition. Recent studies have emphasized the relevance of several mediators in this condition, including prostaglandin-I (2) (prostacyclin), nitric oxide, endothelin-1, angiopoietin-1, 5-hydroxytryptamine (serotonin), cytokines, chemokines, and members of the transforming growth factor beta (TGF-beta) superfamily. Targeting some of these dysfunctional pathways (prostacyclin, nitric oxide, and endothelin-1) has been beneficial in subjects displaying pulmonary arterial hypertension.

  1. Sildenafil in pediatric pulmonary arterial hypertension.

    Science.gov (United States)

    Dhariwal, A K; Bavdekar, S B

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a life-threatening disease of varied etiologies. Although PAH has no curative treatment, a greater understanding of pathophysiology, technological advances resulting in early diagnosis, and the availability of several newer drugs have improved the outlook for patients with PAH. Sildenafil is one of the therapeutic agents used extensively in the treatment of PAH in children, as an off-label drug. In 2012, the United States Food and Drug Administration (USFDA) issued a warning regarding the of use high-dose sildenafil in children with PAH. This has led to a peculiar situation where there is a paucity of approved therapies for the management of PAH in children and the use of the most extensively used drug being discouraged by the regulator. This article provides a review of the use of sildenafil in the treatment of PAH in children.

  2. Epistatic interactions in idiopathic pulmonary arterial hypertension

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    Shivani Vadapalli

    2012-01-01

    Full Text Available Background : Idiopathic pulmonary arterial hypertension (IPAH is a poorly understood complex disorder, which results in progressive remodeling of the pulmonary artery that ultimately leads to right ventricular failure. A two-hit hypothesis has been implicated in pathogenesis of IPAH, according to which the vascular abnormalities characteristic of PAH are triggered by the accumulation of genetic and/or environmental insults in an already existing genetic background. The multifactor dimensionality reduction (MDR analysis is a statistical method used to identify gene-gene interaction or epistasis and gene-environment interactions that are associated with a particular disease. The MDR method collapses high-dimensional genetic data into a single dimension, thus permitting interactions to be detected in relatively small sample sizes. Aim: To identify and characterize polymorphisms/genes that increases the susceptibility to IPAH using MDR analysis. Materials and Methods: A total of 77 IPAH patients and 100 controls were genotyped for eight polymorphisms of five genes (5HTT, EDN1, NOS3, ALK-1, and PPAR-γ2. MDR method was adopted to determine gene-gene interactions that increase the risk of IPAH. Results : With MDR method, the single-locus model of 5HTT (L/S polymorphism and the combination of 5HTT(L/S, EDN1(K198N, and NOS3(G894T polymorphisms in the three-locus model were attributed to be the best models for predicting susceptibility to IPAH, with a P value of 0.05. Conclusion: MDR method can be useful in understanding the role of epistatic and gene-environmental interactions in pathogenesis of IPAH.

  3. Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery

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    Venkat Gangadharan MD

    2017-01-01

    Full Text Available A 36-year-old woman was seen with complaints of exertional chest pain and shortness of breath. Her medical history included atrial fibrillation and diabetes. Physical examination was unremarkable except for an irregular cardiac rhythm. Myocardial perfusion imaging revealed the presence of a large area of infarction involving the entire anterior and apical walls and part of the anteroseptal wall with minimal periinfarct ischemia. Computed tomography coronary angiogram revealed an anomalous left main coronary artery arising from the main pulmonary artery. Right and left heart catheterizations demonstrated moderate pulmonary hypertension with a slight step-up in oxygen saturation between the right ventricle and main pulmonary artery. Coronary angiography showed a large tortuous right coronary artery with collaterals to the left anterior descending artery that drained into the main pulmonary artery. She was referred for surgery. This case demonstrates a rare coronary artery anomaly in an adult where survival is dependent on collateral circulation.

  4. Unilateral Absence of the Pulmonary Artery Incidentally Found in Adulthood

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    Cenk Aypak

    2012-01-01

    Full Text Available Unilateral absence of the pulmonary artery (UAPA or pulmonary artery agenesis is a rare congenital disorder presenting with a wide spectrum of symptoms. UAPA is usually associated with cardiac anomalies and surgically treated in childhood. We report a rare case of a 50-year-old woman who was diagnosed with left pulmonary artery agenesis without any other cardiac anomalies. Clinicians should be aware of the possibility of undiagnosed cases of UAPA in patients through a chest radiograph that suggests the diagnosis. Confirmation of the diagnosis and anatomic details can be discerned by CT scanning.

  5. A Review of Targeted Pulmonary Arterial Hypertension-Specific Pharmacotherapy.

    Science.gov (United States)

    Ataya, Ali; Cope, Jessica; Alnuaimat, Hassan

    2016-12-06

    Significant advances in the understanding of the pathophysiology of pulmonary arterial hypertension over the past two decades have led to the development of targeted therapies and improved patient outcomes. Currently, a broad armamentarium of pulmonary arterial hypertension-specific drugs exists to assist in the treatment of this complex disease state. In this manuscript, we provide a comprehensive review of the current Food and Drug Administration (FDA)-approved pulmonary arterial hypertension-specific therapies, and their supporting evidence for adults, targeting the nitric oxide, soluble guanylate cyclase, endothelin, and prostacyclin pathways.

  6. Developments in pulmonary arterial hypertension-targeted therapy for chronic thromboembolic pulmonary hypertension.

    Science.gov (United States)

    Hadinnapola, Charaka; Pepke-Zaba, Joanna

    2015-10-01

    Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease characterised by the presence of organised chronic thromboembolic material occluding the proximal pulmonary arteries and a vasculopathy in the distal pulmonary arterial tree. Pulmonary endarterectomy (PEA) is a potential cure for many patients with CTEPH. However, PEA is not suitable for patients with a significant distal distribution of chronic thromboembolic material or with significant comorbidities. Also, a proportion of patients are left with residual CTEPH post PEA. Until recently, pulmonary arterial hypertension-targeted therapies have been used off licence to treat patients with inoperable or residual CTEPH. The CHEST1 study investigated the use of riociguat and was the first randomised controlled trial to show efficacy in inoperable or residual CTEPH. In this review, we explore the pathophysiology of CTEPH and review the current trial evidence for pulmonary arterial hypertension-targeted therapies. We also include a discussion of physiological considerations that require further investigation.

  7. The role of increased pulmonary blood flow in pulmonary arterial hypertension

    NARCIS (Netherlands)

    van Albada, ME; Schoemaker, RG; Kemna, MS; Cromme - Dijkhuis, A; van Veghel, R; Berger, RMF

    2005-01-01

    Chronic increased pulmonary blood flow is considered a pre-requisite for the induction of advanced vascular lesions in pulmonary arterial hypertension in congenital heart defects. The aim of the present study was to characterise the effects of increased pulmonary flow induced by an aortocaval shunt

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

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    Makker, H.K.; Barnes, P.C. (Manchester Univ. (United Kingdom). School of Medicine Salford Royal Hospital (United Kingdom))

    1991-08-01

    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).

  9. Effects of pravastatin on pulmonary arteries and aorta reactivity in monocrotalin-induced pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    PGUERARD; 0BARTHEZ; FGOIRAND; LROCHETTE; MBARDOU; MDUMAS

    2004-01-01

    AIM: Vascular injury caused by monocrotalin (MC) can affect endothelial regulation and induces pulmonary hypertension and heart failure. We showed previously that pravastatin prevented the development of MC-induced pulmonary hypertension by improving pulmonary arteries (PA) endothelium dependent vasodilation. The aims of this study were to compare the protective

  10. [Unilateral pulmonary artery agenesis with ipsilateral pulmonary hypoplasia as incidental finding in an asthmatic patient].

    Science.gov (United States)

    Contreras-Arias, Catalina; Duarte, Diana; Ramírez, Luis F; Serrano, Carlos D

    2014-01-01

    Unilateral absence of a pulmonary artery is an uncommon congenital heart disease. It can be related to respiratory symptoms such as asthma, an unusual finding 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.

  11. Niflumic Acid Attenuated Pulmonary Artery Tone and Vascular Structural Remodeling of Pulmonary Arterial Hypertension Induced by High Pulmonary Blood Flow In Vivo.

    Science.gov (United States)

    Wang, Kai; Ma, Jianfa; Pang, Yusheng; Lao, Jinquan; Pan, Xuanren; Tang, Qiaoyun; Zhang, Feng; Su, Danyan; Qin, Suyuan; Shrestha, Arnav Prasad

    2015-10-01

    Calcium-activated chloride channels (CaCCs) play a vital role in regulating pulmonary artery tone during pulmonary arterial hypertension (PAH) induced by high blood flow. The role of CaCCs inhibitor niflumic acid (NFA) in vivo during this process requires further investigation. We established the PAH model by abdominal shunt surgery and treated with NFA in vivo. Fifty rats were randomly divided into normal, sham, shunt, NFA group 1 (0.2 mg/kg), and NFA group 2 (0.4 mg/kg). Pathological changes, right ventricle hypertrophy index, arterial wall area/vessel area, and arterial wall thickness/vessel external diameter were analyzed. Then contraction reactions of pulmonary arteries were measured. Finally, the electrophysiological characteristics of pulmonary arterial smooth muscle cells were investigated using patch-clamp technology. After 11 weeks of shunting, PAH developed, accompanied with increased right ventricle hypertrophy index, arterial wall area/vessel area, and arterial wall thickness/vessel external diameter. In the NFA treatment groups, the pressure and pathological changes were alleviated. The pulmonary artery tone in the shunt group increased, whereas it decreased after NFA treatment. The current density of CaCC was higher in the shunt group, and it was decreased in the NFA treatment groups. In conclusion, NFA attenuated pulmonary artery tone and structural remodeling in PAH induced by high pulmonary blood flow in vivo. CaCCs were involved and the augmented current density was alleviated by NFA treatment.

  12. Pulmonary arterial hypertension: a current review of pharmacological management.

    Science.gov (United States)

    Sahni, Sonu; Ojrzanowski, Marcin; Majewski, Sebastian; Talwar, Arunabh

    2016-01-01

    Pulmonary hypertension (PHTN) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance, which eventually leads to right ventricular failure and death. At present there is no cure for pulmonary arterial hypertension (PAH); however over the past decade targeted pharmaceutical options have become available for the treatment of PAH. Prior to evaluation for therapeutic options a definitive diagnosis of pulmonary arterial hypertension must be made via comprehensive physical exam and definitive diagnostic testing. Screening test of choice remains echocardiography and gold standard for definitive diagnosis is right heart catheterization. Once the establishment of a diagnosis of PAH is made therapeutic options may be a possibility based on a diagnostic algorithm and disease severity of the PAH patient. There are different classes of medications available with different mechanisms of actions which net a vasodilatory effect and improve exercise tolerance, quality of life as well and survival.

  13. Surgical repair of an idiopathic pulmonary artery aneurysm.

    Science.gov (United States)

    Shiokawa, Yuichi; Ushijima, Tomoki; Oishi, Yasuhisa; Tominaga, Ryuji

    2011-02-01

    We report a rare case of idiopathic pulmonary artery aneurysm (PAA) in a 56-year-old woman without any causative conditions, such as congenital heart disease, inflammation, pulmonary artery hypertension, or systemic vasculitis. She presented with sudden back pain, and examination revealed the PAA. She electively underwent resection of the aneurysm and graft replacement. Pathology examination revealed cystic medial necrosis, which was considered the underlying pathology of the aneurysm.

  14. Optical coherence tomography of the pulmonary arteries: A systematic review.

    Science.gov (United States)

    Jorge, Elisabete; Baptista, Rui; Calisto, João; Faria, Henrique; Monteiro, Pedro; Pan, Manuel; Pêgo, Mariano

    2016-01-01

    Optical coherence tomography (OCT) is an imaging technique extensively used for visualizing the coronary circulation, where it assists clinical decision-making. Along with the new interventional procedures being introduced for pulmonary vascular disease, there is an increasing need for intravascular imaging of the pulmonary arteries. Additionally, measurements of the wall thickness of the pulmonary arteries of patients with various types of pulmonary hypertension (PH) may provide relevant diagnostic and prognostic information. The aim of this review is to summarize all the available evidence on the use of OCT for imaging the pulmonary bed and to describe a simple protocol for OCT image acquisition. We conducted a systematic review of the literature using electronic reference databases through February 2015 (MEDLINE, Cochrane Library, Web of Knowledge, and references cited in other studies) and the search terms "optical coherence tomography," "pulmonary hypertension," and "pulmonary arteries." Studies in which OCT was used to image the pulmonary vessels were considered for inclusion. We identified 14 studies reporting OCT imaging data from the pulmonary arteries. OCT was able to identify intravascular thrombi in patients with chronic thromboembolic PH (CTEPH), and an increase in vessel wall thickness was found in most patients with PH, compared with the controls. OCT has also been reported to be useful for the selection of balloon size in the setting of balloon pulmonary angioplasty for CTEPH. The main limitations include lack of standardization, little data on outcomes, cost, and the technical limitations involved in visualizing small-diameter (arteries, and may provide additional information in the assessment of patients with PH. Prospective high-quality studies assessing the safety, validity, and clinical impact of OCT imaging for pulmonary vessels are warranted.

  15. Genetics and genomics of pulmonary arterial hypertension.

    Science.gov (United States)

    Soubrier, Florent; Chung, Wendy K; Machado, Rajiv; Grünig, Ekkehard; Aldred, Micheala; Geraci, Mark; Loyd, James E; Elliott, C Gregory; Trembath, Richard C; Newman, John H; Humbert, Marc

    2013-12-24

    Major discoveries have been obtained within the last decade in the field of hereditary predisposition to pulmonary arterial hypertension (PAH). Among them, the identification of bone morphogenetic protein receptor type 2 (BMPR2) as the major predisposing gene and activin A receptor type II-like kinase-1 (ACVRL1, also known as ALK1) as the major gene when PAH is associated with hereditary hemorrhagic telangiectasia. The mutation detection rate for the known genes is approximately 75% in familial PAH, but the mutation shortfall remains unexplained even after careful molecular investigation of these genes. To identify additional genetic variants predisposing to PAH, investigators harnessed the power of next-generation sequencing to successfully identify additional genes that will be described in this report. Furthermore, common genetic predisposing factors for PAH can be identified by genome-wide association studies and are detailed in this paper. The careful study of families and routine genetic diagnosis facilitated natural history studies based on large registries of PAH patients to be set up in different countries. These longitudinal or cross-sectional studies permitted the clinical characterization of PAH in mutation carriers to be accurately described. The availability of molecular genetic diagnosis has opened up a new field for patient care, including genetic counseling for a severe disease, taking into account that the major predisposing gene has a highly variable penetrance between families. Molecular information can be drawn from the genomic study of affected tissues in PAH, in particular, pulmonary vascular tissues and cells, to gain insight into the mechanisms leading to the development of the disease. High-throughput genomic techniques, on the basis of next-generation sequencing, now allow the accurate quantification and analysis of ribonucleic acid, species, including micro-ribonucleic acids, and allow for a genome-wide investigation of epigenetic or

  16. A PROSPECTIVE STUDY OF PULMONARY ARTERIAL HYPERTENSION IN CHRONIC OBSTRUCTIVE PULMONARY DISEASES

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    Saptanaga Kumar

    2015-04-01

    Full Text Available BACKGROUND : Chronic obstructive pulmonary disease (COPD is a heterogeneous, multisystem disease with complexities that extend far beyond airway obstruction. OBJECTIVES : The purpose of this prospective study is to determine pulmonary arterial hypertension in chronic obstructi ve pulmonary disease non - invasively. METHODS : In this descriptive, prospective, observational, cross sectional study, all patients who presented to the department of Medicine and Respiratory medicine, during this study period of 12 months from January 2013 - December 2014 in Chennai were included. RESULTS : Total number of males in the study is 90(90%, females in the study is 10 (10%. Number of patients in the age group 25 - 35years was 06 (6%, 36 - 45years was 38(38%, 46 - 55 years was 30(30, number of patie nts in 56 - 65 years was 14 (14 and number of patients in the age group 66 - 75 years was 12(12. total number of males smoking in the study is 55(61.11% and total number of non - smokers were 35(38.88, total number of female smoking in the study is 1(10% an d total number of non - smokers were 9(90%. Pulmonary arterial systolic pressure in present study, Mild pulmonary arterial hypertension was seen in 26(26%, Moderate pulmonary arterial hypertension was seen in 54(54%, Severe pulmonary arterial hypertension was seen in 20(20%. CONCLUSION : This study shows the prevalence of pulmonary arterial hypertension in COPD patients.

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

    NARCIS (Netherlands)

    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 progeni

  18. Treatment options for paediatric pulmonary arterial hypertension

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    R.M.F. Berger

    2010-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a serious, progressive condition, which can present idiopathically or secondary to conditions such as systemic sclerosis or congenital heart disease. The condition exists in both adult and paediatric forms, which possess several similar characteristics. Adult and paediatric PAH can, however, be distinguished based on underlying pathology and the presence of age-specific conditions, some of which are related to poor lung development in children. Improved knowledge of vascular biology has led to the development of several PAH-specific therapies, which have demonstrated clinical benefits in adults, including improved exercise capacity and prolonged survival. Treatment data in paediatric PAH are scarce. Although limited, the existing data indicate that current treatments for paediatric PAH are well tolerated and effective, at least in the short- and medium-term. Nevertheless, the current guidelines for clinicians, which recommend use of the adult treatment algorithm in paediatric patients, appear justified when judged according to the available evidence. However, further randomised, controlled trials are necessary to increase the evidence base for treatment of paediatric PAH, especially in relation to age-specific conditions. At present, early initiation of treatment and combination pharmacological therapy may offer the most promising courses of action to improve outcomes in paediatric PAH.

  19. 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.

  20. The value of tools to assess pulmonary arterial hypertension

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    H. Gupta

    2011-12-01

    Full Text Available Pulmonary hypertension is a common but complex clinical problem. When suspected in an appropriate clinical setting or detected incidetally, an array of investigative tools are employed with an intent to confirm the diagnosis, define aetiology, evaluate the functional and haemodynamic impairment, define treatment options, monitor the therapy, and establish long-term prognosis. However, no single tool provides comprehensive information that encompasses the aforementioned aims. Therefore, judicious use of these tools is of paramount importance, in order to maximise outcome and cost-effectiveness, while minimising risks and redundancies. Furthermore, a number of promising tools and techniques are emerging rapidly in the arena of pulmonary hypertension. These tools augment our understanding of pathophysiology and natural history of pulmonary hypertension. There is, therefore, increasing need for validating these emerging paradigms in multicentre trials. In this review, we focus on the tools commonly used to evaluate pulmonary arterial hyertension and also define some of the new approaches to pulmonary arterial hypertension.

  1. [Physiopathology of pulmonary arterial hypertension. Cellular and molecular aspects].

    Science.gov (United States)

    Perros, Frédéric; Humbert, Marc

    2005-02-12

    The combined effects of vasoconstriction, remodelling of the pulmonary vessel walls and in situ thrombosis contribute to the increase in pulmonary vascular resistance during pulmonary arterial hypertension. Vascular remodelling involves all the sheaths of the vessel wall and all the cell types of which it is composed (endothelial cells, smooth muscle cells, fibroblasts, inflammatory cells and platelets). Excessive vasoconstriction has been related to a defect in the function of expression of the potassium channels and endothelial dysfunction. This leads to chronic insufficiency in the production of vasodilators, notably nitrogen monoxide and prostacyclin and the excessive production of vasoconstrictors such as endotheline-1. These defects contribute to the increase in vascular tonus and pulmonary vascular remodelling and represent pertinent pharmacological targets. Certain growth factors, including those of the super-family of transforming growth factor beta, angiopoietine-1 and serotonin, may play a part in the pathogenesis of pulmonary arterial hypertension.

  2. Pulmonary hypertension with a huge thrombosis in main stem of pulmonary artery

    Institute of Scientific and Technical Information of China (English)

    杨萍; 曾红; 孟繁波; 赵林阳

    2001-01-01

    @@A huge thrombosis in the main stem of the pulmonary artery (PA) with pulmonary hypertension has rarely been reported. We present two cases diagnosed and treated in our hospital. One suffered from polyarteritis with a huge thrombus in PA revealed at autopsy. The second case had congenital heart disease of the patent artery duct; and the huge thrombus was found on echocardiogram and extirpated in surgery.

  3. [Bilateral coronary artery-pulmonary artery fistulas in a case with unstable angina pectoris].

    Science.gov (United States)

    Kepez, Alper; Kaya, Ergün Bariş; Aytemir, Kudret; Oto, Ali

    2008-03-01

    Bilateral coronary artery fistulas originating from both right and left coronary arteries are rare congenital abnormalities. A 58-year-old man presented with chest pain unrelated to exertion. Coronary angiography showed a fistula originating from the level of the first diagonal branch of the left anterior descending (LAD) coronary artery and a 95% stenosis just distal to the fistula. Right coronary angiography showed another fistula originating from the ostium of the right coronary artery. Both fistulas drained into the pulmonary artery. Coronary bypass surgery was performed for the LAD lesion using the left internal mammary artery graft, during which both fistulas were ligated. No complications were encountered postoperatively.

  4. Moyamoya Disease with Peripheral Pulmonary Artery Stenoses and Coronary Artery Fistulae

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    Lindsay Reardon

    2009-01-01

    Full Text Available Moyamoya is a progressive disorder of the cerebral vasculature. Our report describes a rare case of Moyamoya disease with distal peripheral pulmonary artery stenoses and coronary fistulae in a 12-year-old Caucasian female patient.

  5. 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.

    2008-01-01

    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 hypertens

  6. Case Report: Chronic Recurrent Unilateral Pulmonary Infection: Result of Congenital Unilateral Agenesis of Pulmonary Artery.

    Science.gov (United States)

    Al Jabbari, Odeaa; Abu Saleh, Walid K; Ramchandani, Mahesh; Scheinin, Scott

    2016-01-01

    Unilateral agenesis of the pulmonary artery (UAPA) is a rare congenital anomaly. This report describes a 52-year-old female who gave a long history of chronic, recurrent, left-sided pulmonary infections related to UAPA. For many years, she was managed medically but the infection continued to recur. She eventually underwent left pneumonectomy and made a good recovery.

  7. Isolated agenesis of the right pulmonary artery with late manifestation of pulmonary hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Huebsch, P.; Pichler, W.; Lang, I.; Mlczoch, J.

    1987-01-01

    The case of a woman patient of 25 years of age with acute cardiac decompensation is presented. The chest X-ray as well as the lung scan showed the typical features of absence of the right pulmonary artery. The diagnosis was confirmed angiographically. The sudden and late onset of symptoms of pulmonary hypertension is a remarkable feature.

  8. 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.)

  9. Tropical pulmonary eosinophilia presenting as severe pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Shikha Jindal

    2013-01-01

    Full Text Available Tropical pulmonary eosinophilia (TPE is an easily diagnosed and treatable disease. Patients with TPE usually present with respiratory symptoms that include paroxysmal cough, breathlessness, wheeze and chest pain, often misdiagnosed as bronchial asthma. This case highlights one of the unusual presentations of TPE and discusses the association between TPE and pulmonary hypertension.

  10. Pulmonary Artery Aneurysm Thrombosis with Combined Pulmonary Fibrosis and Emphysema: A Case Report

    OpenAIRE

    Agrawal, Mitali Bharat; Awad, Nilkant Tukaram

    2016-01-01

    We report a rare case of Pulmonary Artery Aneurysm (PAA) thrombosis with Combined Pulmonary Fibrosis and Emphysema (CPFE) with pulmonary hypertension. A 75-year-old male presented with haemoptysis, dyspnoea, clubbing and bilateral fine end inspiratory rales on examination. He was diagnosed to have PAA thrombosis with CPFE on the basis of computed tomographical angiography and high resolution computed tomography. He was then managed conservatively with pirfenidone for the interstitial lung dis...

  11. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnosis with CT Angiography

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    Guray Oncel

    2013-01-01

    Full Text Available Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA is a rare congenital anomaly. It is associated with early infant mortality and sudden death in adults. Traditionally, ALCAPA has been diagnosed by angiography or autopsy; however, the development of cardiac computed tomography (CT and magnetic resonance imaging (MRI has allowed noninvasive evaluation of the coronary anatomy by direct visualization of the origin of the left coronary artery (LCA from the pulmonary artery. We report a case of 10-year-old girl who has been on follow up for dilated cardiomyopathy for 4 years. The definitive diagnosis of ALCAPA is reached by multislice computed tomography (MSCT. The MSCT scan showed an anomalous origin of LCA from the pulmonary trunk, with a tortuous and dilated right coronary artery and right-to-left collateralization. Consequently, the patient was successfully treated with surgery.

  12. 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.

  13. Pulmonary complications of transcatheter arterial chemoembolization for hepatocellular carcinoma

    Science.gov (United States)

    Nhu, Quan M; Knowles, Harry; Pockros, Paul J; Frenette, Catherine T

    2016-01-01

    Transarterial chemoembolization (TACE) is an effective palliative intervention that is widely accepted for the management of hepatocellular carcinoma (HCC). Post-TACE pulmonary complications resulting in acute lung injury (ALI) or acute respiratory distress syndrome (ARDS) are rare events. Pulmonary complications after TACE are thought to be related to chemical injury subsequent to the migration of the infused ethiodized oil or chemotherapeutic agent to the lung vasculature, facilitated by arteriovenous (AV) shunts within the hyper-vascular HCC. We review herein the literature on pulmonary complications related to TACE for HCC. Post-TACE pulmonary complications have included pulmonary oil embolism, interstitial pneumonitis, chemical pneumonitis, ALI, ARDS, lipoid pneumonia, acute eosinophilic and neutrophilic pneumonia, bilious pleuritis, pulmonary abscess, pulmonary tumor embolism, and possibly pulmonary metastasis with HCC. The risk factors associated with post-TACE pulmonary complications identified in the literature include large hyper-vascular HCC with AV shunts, large-volume Lipiodol infusion, and embolization via the right inferior phrenic artery. However, the absence of known risk factors is not a guarantee against serious complications. An astute awareness of the potential post-TACE pulmonary complications should expedite appropriate therapeutic interventions and increase potential for early recovery. PMID:27904836

  14. Pulmonary arterial dysfunction in insulin resistant obese Zucker rats

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    Cogolludo Angel

    2011-04-01

    Full Text Available Abstract Background Insulin resistance and obesity are strongly associated with systemic cardiovascular diseases. Recent reports have also suggested a link between insulin resistance with pulmonary arterial hypertension. The aim of this study was to analyze pulmonary vascular function in the insulin resistant obese Zucker rat. Methods Large and small pulmonary arteries from obese Zucker rat and their lean counterparts were mounted for isometric tension recording. mRNA and protein expression was measured by RT-PCR or Western blot, respectively. KV currents were recorded in isolated pulmonary artery smooth muscle cells using the patch clamp technique. Results Right ventricular wall thickness was similar in obese and lean Zucker rats. Lung BMPR2, KV1.5 and 5-HT2A receptor mRNA and protein expression and KV current density were also similar in the two rat strains. In conductance and resistance pulmonary arteries, the similar relaxant responses to acetylcholine and nitroprusside and unchanged lung eNOS expression revealed a preserved endothelial function. However, in resistance (but not in conductance pulmonary arteries from obese rats a reduced response to several vasoconstrictor agents (hypoxia, phenylephrine and 5-HT was observed. The hyporesponsiveness to vasoconstrictors was reversed by L-NAME and prevented by the iNOS inhibitor 1400W. Conclusions In contrast to rat models of type 1 diabetes or other mice models of insulin resistance, the obese Zucker rats did not show any of the characteristic features of pulmonary hypertension but rather a reduced vasoconstrictor response which could be prevented by inhibition of iNOS.

  15. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Report of an Adult Case

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    Celso K. Takimura

    2002-03-01

    Full Text Available We report the clinical findings, pathophysiology, diagnostic characteristics, and surgical repair of anomalous origin of the left coronary artery from the pulmonary artery in a 26-year-old female patient with a clinical diagnosis of coronary heart disease.

  16. Diffuse Pulmonary Arteriovenous Fistulas With Pulmonary Arterial Hypertension: Case Report and Review.

    Science.gov (United States)

    Jiang, Rong; Gong, Su-Gang; Pudasaini, Bigyan; Zhao, Qin-Hua; Wang, Lan; He, Jing; Liu, Jin-Ming

    2016-04-01

    Pulmonary arteriovenous fistulas (PAVFs) are rare. Diffuse type PAVFs with pulmonary arterial hypertension (PAH) are even rarer and can elude anatomy imaging like a plain chest film or a computed tomography. The rapid blood flow that ensues due to lack of a capillary bed leads to various degrees of ischemia depending on the number and size of the PAVF. This is a case report of diffuse PAVF in a patient with PAH.This case report describes a patient with recurrent hemoptysis and chest pain. Systemic examination was unremarkable except for P2 attenuation on auscultation. Echocardiograghy showed confirmed pulmonary hypertension with mild dilation of right atrium and ventricle and a tricuspid regurgitation pressure gradient of 40 mm Hg and ruled out congenital heart diseases. Right heart catheterization revealed precapillary PAH with mean pulmonary arterial pressure of 88 mm Hg. Pulmonary angiography showed enlarged pulmonary arterial trunk and diffuse spiral tortuous pulmonary arterial branches indicting diffuse PAVFs. The patient was diagnosed as PAH and began treatment of 25 mg tid of sildenafil.The case highlights a rare and unique presentation of PAH.

  17. 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...... starting CPB and longitudinally in a mixed-effects model (MEM). Secondary outcomes were tracheal intubation time, serious adverse events, mortality, days alive outside the intensive care unit (ICU) and outside the hospital. RESULTS: 21 hours after starting CPB patients receiving pulmonary artery perfusion...... with normothermic oxygenated blood had a higher oxygenation index compared with no pulmonary perfusion (mean difference (MD) 0.94; 95% CI 0.05 to 1.83; p=0.04). The blood group had also a higher oxygenation index both longitudinally (MEM, p=0.009) and at 21 hours (MD 0.99; CI 0.29 to 1.69; p=0.007) compared...

  18. Advanced multimodality imaging of an anomalous vessel between the ascending aorta and main pulmonary artery in a dog.

    Science.gov (United States)

    Markovic, Lauren E; Kellihan, Heidi B; Roldán-Alzate, Alejandro; Drees, Randi; Bjorling, Dale E; Francois, Chris J

    2014-03-01

    A 1-year-old male German shorthaired pointer was referred for evaluation of tachypnea and hemoptysis. A grade VI/VI left basilar continuous murmur was ausculted. Multimodality imaging consisting of thoracic radiographs, transthoracic and transesophageal echocardiography, fluoroscopy-guided selective angiography, computed tomography angiogram (CTA) and magnetic resonance angiogram (MRA), was performed on this patient. The defect included a left-to-right shunting anomalous vessel between the ascending aorta and main pulmonary artery, along with a dissecting aneurysm of the main and right pulmonary artery. An MRA post-processing technique (PC VIPR) was used to allow for high resolution angiographic images and further assessment of the patient's hemodynamics prior to surgical correction. This case report describes the clinical course of a canine patient with a rare form of congenital cardiac disease, and the multiple imaging modalities that were used to aid in diagnosis and treatment.

  19. STRAIN ELASTOGRAPHY USING DOBUTAMINE-INDUCED CAROTID ARTERY PULSATION IN CANINE THYROID GLAND.

    Science.gov (United States)

    Lee, Gahyun; Jeon, Sunghoon; Lee, Sang-Kwon; Kim, Hyunwoo; Yu, Dohyeon; Choi, Jihye

    2015-01-01

    Thyroid disease is common in dogs and conventional ultrasonography is a standard diagnostic test for diagnosis and treatment planning. Strain elastography can provide additional information about tissue stiffness noninvasively after applying external or internal compression. However, natural carotid artery pulsations in the canine thyroid gland are too weak to maintain sufficient internal compression force. The objective of the present study was to describe the feasibility of strain elastography for evaluating the canine thyroid gland and the repeatability of dobutamine-induced carotid artery pulsation as an internal compression method. In seven healthy Beagle dogs, strain on each thyroid lobe was induced by external compression using the ultrasound probe and internal compression using carotid artery pulsation after dobutamine infusion. The thyroid appeared homogeneously green and the subcutaneous fat superficial to the thyroid lobe appeared blue. Strain values and strain ratios did not differ among dogs or between the left and right lobes. Interobserver repeatability was excellent for both compression methods. Intraobserver repeatability of the strain ratio measured using the carotid artery pulsation method (intraclass coefficient correlation = 0.933) was higher than that measured using the external compression method (0.760). Mean strain values of thyroid lobes for the external compression method (142.93 ± 6.67) differed from the internal method (147.31 ± 8.24; P thyroid stiffness in dogs. Carotid artery pulsation induced by dobutamine infusion can be used for canine thyroid strain elastography with excellent repeatability.

  20. Tissue Doppler Findings in Patients with Pulmonary Arterial Hypertension

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    Firoozeh Abtahi

    2016-09-01

    Full Text Available In conclusion, our results suggested that increasing degrees of pulmonary artery systolic pressure affected timing of some tissue Doppler-derived intervals within the cardiac cycle, including IVC time, time to peak systolic myocardial velocity (Sm, and time to peak strain. Therefore, tissue Doppler imaging could be used in assessment of patients with suspected pulmonary arterial hypertension. Background: Pulmonary hypertension is an untreatable condition with poor prognosis and factors such as more elevated pulmonary arterial systolic pressure and right ventricular dysfunction are associated with a worse outcome. Objectives: Considering the limitations of the current modalities, this study aimed to find the relationship between tissue Doppler-derived systolic and diastolic parameters and elevated pulmonary arterial pressure in order to assess the routine application of tissue Doppler imaging in evaluation of pulmonary arterial hypertension. Patients and Methods: This study was conducted on 100 inpatient and outpatient individuals referred to the Department of Echocardiography in Shahid Faghihi hospital, Shiraz, Iran from July 2012 to March 2013. The individuals who had preserved right ventricular function in the presence of pulmonary arterial hypertension were included in the case group. On the other hand, the patients who did not have echocardiographic signs of pulmonary arterial hypertension were enrolled into the control group. All the patients underwent a complete transthoracic echocardiogram including 2-dimensional, color flow, and spectral Doppler as well as tissue Doppler imaging using a vivid E9 system, and the desired systolic and diastolic parameters were recorded. The relationship among these parameters was evaluated by independent sample t-test using the SPSS statistical software, version 16. Besides, P < 0.05 was considered to be statistically significant. Results: The mean time to peak strain was significantly longer in the case

  1. 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...

  2. 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

  3. Right pulmonary artery agenesis and coronary-to-bronchial artery aneurysm.

    Science.gov (United States)

    De Dominicis, Florence; Leborgne, Laurent; Raymond, Alexandre; Berna, Pascal

    2011-03-01

    Isolated unilateral pulmonary artery agenesis is a rare congenital anomaly that may be complicated with hemoptysis, recurrent pulmonary infections or pulmonary hypertension. To our knowledge the occurrence of a coronary syndrome associated with a coronary-to-bronchial artery saccular aneurysmal collateralization has never been described before. A 44-year-old female presented a congenital right pulmonary artery agenesis associated with a hypotrophic and multicystic right lung complicated with recurrent bronchitis. This patient had a coronary syndrome for which the coronary artery imaging showed a coronary-to-bronchial artery collateralization with an aneurysm at this level. It gives rise to a coronary syndrome by coronary steal. Two bronchial collaterals arising from a diaphragmatic artery and the subclavian artery were also found on the computed tomography (CT)-scan. This last collateral also showed another saccular aneurysm. We first performed an embolization of those two aneurysms in order to decrease the risk of hemorrhage and coronary steal, before performing a right pneumonectomy. In this case, the surgery was indicated because of the pathological lung and the risk of postembolization ischaemia. The postoperative course was uneventful and the patient was doing well six months later.

  4. Pulmonary arterial hypertension secondary to chronic left-sided cardiac dysfunction in dogs.

    Science.gov (United States)

    Stepien, Rebecca L

    2009-09-01

    Pulmonary arterial hypertension is a description of a physiological finding rather than a diagnosis. Pulmonary arterial pressure is the result of interactions among pulmonary blood flow (right ventricular cardiac output), pulmonary vascular impedance and post-capillary pressure (typically reflecting left atrial pressure). When elevations in pulmonary arterial pressure (systolic/diastolic pulmonary arterial pressure > approximately 30/19 mmHg at rest) are accompanied by increased left atrial pressure, pulmonary arterial hypertension may be considered secondary to left-heart failure. Introduction of Doppler methods to diagnose pulmonary arterial hypertension has increased the awareness of the prevalence and importance of pulmonary arterial hypertension dogs with left-heart failure. Increasing understanding of the mechanism of development of pulmonary venous hypertension and reactive pulmonary arterial hypertension in dogs with left-heart disease has led to the development of successful additive therapies for progressive clinical signs in the setting of chronic therapy for congestive heart failure due to left-sided valvular and myocardial dysfunction. Because effective therapies for pulmonary arterial hypertension secondary to chronic left-sided cardiac dysfunction are now available, screening for pulmonary arterial hypertension should be a regular part of the Doppler echocardiographic examination in a clinical setting of chronic therapy for left-sided congestive heart failure due to valvular or myocardial disease.

  5. Diaphragm weakness in pulmonary arterial hypertension: role of sarcomeric dysfunction

    NARCIS (Netherlands)

    Manders, E.; Man, F.S. de; Handoko, M.L.; Westerhof, N.; Hees, H.W.H. van; Stienen, G.J.; Vonk-Noordegraaf, A.; Ottenheijm, C.A.C.

    2012-01-01

    We previously demonstrated that diaphragm muscle weakness is present in experimental pulmonary arterial hypertension (PH). However, the nature of this diaphragm weakness is still unknown. Therefore, the aim of this study was to investigate whether changes at the sarcomeric level contribute to diaphr

  6. 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.

  7. Current and advancing treatments for pulmonary arterial hypertension in childhood

    NARCIS (Netherlands)

    Zijlstra, Willemijn M. H.; Ploegstra, Mark-Jan; Berger, Rolf M. F.

    2014-01-01

    Pulmonary arterial hypertension (PAH) is a severe and progressive intrinsic disease of the precapillary lung vasculature. Since the introduction of PAH-targeted drugs, survival of PAH patients seems to have improved. Randomized controlled trials have led to evidence-based guidelines to direct treatm

  8. Nesiritide for pulmonary arterial hypertension with decompensated cor pulmonale.

    Science.gov (United States)

    Kingman, Martha S; Thompson, Brenda S; Newkirk, Trixie; Torres, Fernando

    2005-01-01

    Pulmonary arterial hypertension complicated by decompensated cor pulmonale is a challenging clinical problem with few effective therapeutic options. B-type natriuretic peptide is a pluripotent hormone that promotes diuresis and natriuresis, vasodilates systemic and pulmonary vessels, and reduces circulating levels of endothelin and aldosterone. It may represent a possible therapeutic strategy for decompensated cor pulmonale in the same manner that it is used to treat decompensated left heart failure. The authors report their experience with B-type natriuretic peptide as adjunctive therapy for pulmonary arterial hypertension complicated by decompensated cor pulmonale. A detailed case report is presented followed by the evaluation of a series of 11 cases occurring in eight patients from December 2002 through April 2004.

  9. Application of Blocking Unilateral Main Pulmonary Artery in Pulmonary Lobectomy of Lung Cancer

    Institute of Scientific and Technical Information of China (English)

    Fei Gao; Yunchao Huang; Lixia Liang; Anning Chen; Tierong Zhao

    2009-01-01

    OBJECTIVE To explore the application of blocking the unilateral main pulmonary artery (MPA) in pulmonary lobectomy (PL) for patients with stage Ⅱand Ⅲ lung cancer, and to retrospectively analyze the methods of surgery for blocking the unilateral main pulmonary artery, perioperative indications, intraoperative concerns and postoperative cardio-pulmonary complications. METHODS During a period from January 2006 to January 2008, intra-pericardial, or extra-pericardial separation and blockade of the left or right MPA followed by completion of various PLs were conducted for 30 lung cancer patients in stage-Ⅱ to Ⅲ with ill- defined anatomic structure of the pulmonary hilum and difficult pulmonary angiodiastasis.RESULTS In the 30 patients, 5 were diagnosed as stage-Ⅱb, 11 stage-Ⅲa, and 14 stage-Ⅲb. During the surgery, giant tumors at the superior pulmonary lobe, with a diameter of over 10 cm, were seen in 13 cases, in which tumor invasion in the extra-pericardiac pulmonary artery was found in 5 cases. Hilar lymphadenectasis with severe tumor adhesion to pulmonary blood vessel could be seen in 20 cases and partial tumorous invasion in the pericardium in 7. In most of the cases, adhesions existed around the tumor, aorta, superior vena, and azygous vein. Invasion of the laryngeal and vagus nerves on the left side was found in 3 cases. Of the 30 patients, simple PL was conducted in 12, and sleeve lobectomy combined with a pulmonary arterioplasty in 18 cases. With a blockade of unilateral MPA, no intraoperative hemorrhea of pulmonary blood vessels occurred during surgery, when there was a clear surgical field of vision. Both PL and lymphadenectomy were smoothly completed in the 30 patients. The healthy pulmonary lobes with normal function were kept and total pneumonectomy was avoided. The time of blocking the pulmonary artery ranged from 10 to 30 min, and intraoperative blood loss was from 200 to 300 ml. Postoperative complicated acute pulmonary edema occurred in

  10. 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.)

  11. 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.)

  12. Left pulmonary artery banding to repair ipsilateral diffuse pulmonary arteriovenous fistula

    Directory of Open Access Journals (Sweden)

    Hirata Takuya

    2012-08-01

    Full Text Available Abstract Congenital pulmonary arteriovenous fistula (PAVF is a rare disease which causes hypoxemia by shunting deoxygenated blood from the pulmonary artery into pulmonary venous return. Lung transplantation is the most effective therapy to treat severe, diffuse PAVF. However, the availability of lungs for transplantation is limited in most parts in the world. For patients with diffuse PAVF affecting only one side of the lungs, ipsilateral pulmonary artery banding (PAB is an effective treatment, but not yet standard of care. We report successful treatment of a patient with diffuse left-sided PAVF with PAB. We believe that PAB is an effective therapy for severe unilateral PAVF and may serve as a bridge to lung transplantation.

  13. Rare case of truncus arteriosus with anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) and unilateral left pulmonary artery agenesis.

    Science.gov (United States)

    Mittal, Kartik; Dey, Amit K; Gadewar, Rohit; Sharma, Rajaram; Pandit, Nilesh; Rajput, Priya; Hira, Priya

    2015-04-01

    The incidence of congenital heart disease (CHD) is 2.4-3.8/1000 live births. Up to 70.7 % of all cases of CHD are reported to be benign; complex heart anomalies are extremely rare. Our case is extremely rare, as we report three very rare findings-truncus arteriosus, anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), and unilateral left pulmonary artery agenesis-in a single patient. Congenital complex cardiac abnormalities are very rare, and two-dimensional echocardiography screening should be supported by cardiac computed tomography (CT). We report a case of truncus arteriosus associated with ARCAPA and left pulmonary artery agenesis diagnosed by cardiac computed tomography; we believe that such an unusual case with all three of these entities has never been reported before.

  14. Effect of the phosphodiesterase type 5 inhibitor tadalafil on pulmonary hemodynamics in a canine model of pulmonary hypertension.

    Science.gov (United States)

    Hori, Yasutomo; Kondo, Chigusa; Matsui, Maho; Yamagishi, Maki; Okano, Shozo; Chikazawa, Seishiro; Kanai, Kazutaka; Hoshi, Fumio; Itoh, Naoyuki

    2014-11-01

    Phosphodiesterase type 5 (PDE5) inhibitors are used for treating pulmonary arterial hypertension (PAH) in dogs. The long-acting PDE5 inhibitor tadalafil was recently approved for treatment of PAH in humans. Basic information related to the pharmacological and hemodynamic effects of tadalafil in dogs is scarce. In this study, the hemodynamic effects of tadalafil after intravenous (IV) and oral administration were investigated in a healthy vasoconstrictive PAH Beagle dog model induced by U46619, a thromboxane A2 mimetic. Six healthy Beagle dogs were anesthetized with propofol and maintained with isoflurane. Fluid-filled catheters were placed into the descending aorta to measure systemic arterial pressure and in the pulmonary artery to measure pulmonary arterial pressure (PAP). U46619 was infused via the cephalic vein to induce PAH. IV infusion of U46619 significantly elevated PAP from baseline in a dose-dependent manner. U46619-elevated PAP and pulmonary vascular resistance was significantly attenuated by the simultaneous infusion of tadalafil at 100 and 200 µg/kg/h. Likewise, oral administration of tadalafil at 1.0, 2.0, and 4.0 mg/kg significantly attenuated U46619-elevated PAP in a dose-dependent manner. U46619-elevated systolic and mean PAP decreased significantly 1 h after oral tadalafil administration at 4.0 mg/kg, and this effect was maintained for 6 h. In conclusion, tadalafil had a pharmacological effect in dogs and IV infusion of tadalafil induced pulmonary arterial relaxation, while oral administration of tadalafil decreased PAP. These results suggest that tadalafil may offer a new therapeutic option for treating dogs with PAH.

  15. Apoptosis-based therapy to treat pulmonary arterial hypertension

    Science.gov (United States)

    Suzuki, Yuichiro J.; Ibrahim, Yasmine F.; Shults, Nataliia V.

    2016-01-01

    Pulmonary arterial hypertension (PAH) is rare, but patients who are diagnosed with this disease still suffer from a lack of satisfactory treatment strategies to prolong survival. While currently approved drugs for PAH have some benefits, these vasodilators only have limited efficacy for eliminating pulmonary vascular remodeling and reducing mortality. Thus, our laboratory has been exploring the use of aggressive drugs, which are capable of causing apoptotic cell death, to treat PAH. We have so far found that three classes of anti-tumor agents, including anthracyclines, taxanes, and proteasome inhibitors, are capable of reducing pulmonary vascular thickness in rats with PAH. These drugs kill cells in remodeled pulmonary vessels without affecting the normal, healthy pulmonary vasculature, revealing that proliferating vascular cells in PAH patients are more sensitive to drug-induced apoptosis compared to the differentiated phenotype that is physiologically important for smooth muscle contraction. Since many apoptosis-inducing drugs cause cardiotoxicity in cancer patients, and because PAH patients already have a weakened heart, we focus on finding biological mechanisms that may reverse pulmonary vascular remodeling without promoting cardiotoxicity. We found two agents, dexrazoxane and pifithrin-α, that selectively inhibit cardiac muscle apoptosis without affecting the drug-induced apoptosis of the proliferating pulmonary vascular cells. Thus, we propose that the addition of apoptosis-inducing drugs and cardioprotectants to PAH therapies may be effective in treating patients and preventing right heart failure.

  16. miR-223 reverses experimental pulmonary arterial hypertension.

    Science.gov (United States)

    Meloche, Jolyane; Le Guen, Marie; Potus, François; Vinck, Jérôme; Ranchoux, Benoit; Johnson, Ian; Antigny, Fabrice; Tremblay, Eve; Breuils-Bonnet, Sandra; Perros, Frederic; Provencher, Steeve; Bonnet, Sébastien

    2015-09-15

    Pulmonary arterial hypertension (PAH) is a devastating disease affecting lung vasculature. The pulmonary arteries become occluded due to increased proliferation and suppressed apoptosis of the pulmonary artery smooth muscle cells (PASMCs) within the vascular wall. It was recently shown that DNA damage could trigger this phenotype by upregulating poly(ADP-ribose)polymerase 1 (PARP-1) expression, although the exact mechanism remains unclear. In silico analyses and studies in cancer demonstrated that microRNA miR-223 targets PARP-1. We thus hypothesized that miR-223 downregulation triggers PARP-1 overexpression, as well as the proliferation/apoptosis imbalance observed in PAH. We provide evidence that miR-223 is downregulated in human PAH lungs, distal PAs, and isolated PASMCs. Furthermore, using a gain and loss of function approach, we showed that increased hypoxia-inducible factor 1α, which is observed in PAH, triggers this decrease in miR-223 expression and subsequent overexpression of PARP-1 allowing PAH-PASMC proliferation and resistance to apoptosis. Finally, we demonstrated that restoring the expression of miR-223 in lungs of rats with monocrotaline-induced PAH reversed established PAH and provided beneficial effects on vascular remodeling, pulmonary resistance, right ventricle hypertrophy, and survival. We provide evidence that miR-223 downregulation in PAH plays an important role in numerous pathways implicated in the disease and restoring its expression is able to reverse PAH.

  17. Existing drugs and agents under investigation for pulmonary arterial hypertension.

    Science.gov (United States)

    Sharma, Mala; Pinnamaneni, Sowmya; Aronow, Wilbert S; Jozwik, Bartosz; Frishman, William H

    2014-01-01

    Pulmonary arterial hypertension is a progressive and debilitating disorder with an associated high morbidity and mortality rate. Significant advances in our understanding of the epidemiology, pathogenesis, and pathophysiology of pulmonary hypertension have occurred over the past several decades. This has allowed the development of new therapeutic options in this disease. Today, our selection of therapeutic modalities is broader, including calcium channel blockers, prostanoids, endothelin receptor antagonists, phosphodiesterase inhibitors, and soluble guanylate cyclase stimulators, but the disease remains fatal. This underscores the need for a continued search for novel therapies. Several potential pharmacologic agents for the treatment of pulmonary arterial hypertension are under clinical development and some promising results with these treatments have been reported. These agents include rho-kinase inhibitors, long-acting nonprostanoid prostacyclin receptor agonists, tyrosine protein kinase inhibitors, endothelial nitric oxide synthase couplers, synthetically produced vasoactive intestinal peptide, antagonists of the 5-HT2 receptors, and others. This article will review several of these promising new therapies and will discuss the current evidence regarding their potential benefit in pulmonary arterial hypertension.

  18. 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.

  19. Echocardiographic assessment of pulmonary vascular resistance in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Bergot Emmanuel

    2010-06-01

    Full Text Available Abstract Background Echocardiographic ratio of peak tricuspid regurgitant velocity to the right ventricular outflow tract time-velocity integral (TRV/TVI rvot was presented as a reliable non-invasive method of estimating pulmonary vascular resistance (PVR. Studies using this technique in patients with moderate to high PVR are scarce. Left ventricular outflow tract time-velocity integral (TVI lvot can be easier to measure than TVI rvot, especially in patients with severe pulmonary hypertension (PH with significant anatomical modifications of the right structures. Aims We wanted to determine whether the TRV/TVI rvot and TRV/TVI lvot ratios would form a reliable non-invasive tool to estimate PVR in a cohort of patients with moderate to severe pulmonary vascular disease. Methods Doppler echocardiographic examination and right heart catheterisation were performed in 37 patients. Invasive PVR was compared with TRV/TVI rvot and TRV/TVI lvot ratios using regression analysis. Two equations were modelled and the results compared with invasive measurements using the Bland-Altman analysis. Using receiver-operating characteristics curve analysis, a cut-off value for the two ratios was generated. Results Correlation coefficients between invasive PVR and TRV/TVI rvot then TRV/TVI lvot were respectively 0.76 and 0.74. Two new equations were found but the Bland-Altman analysis showed wide standard deviations (respectively 3.8 and 3.9 Wood units. A TRV/TVI rvot then TRV/TVI lvot ratio cut-off value of 0.14 had a sensitivity of 93% and a specificity of 57% for the first and a sensitivity of 87% and a specificity of 57% for the second to determine PVR > 2 Wood units. Conclusion Echocardiography is useful for the screening of patients with pulmonary hypertension and PVR > 2 WU. It remains disappointing for accurate assessment of high PVR. TVI lvot may be an alternative to TVI rvot for patients for whom accurate TVI rvot measurement is not possible.

  20. Role of oxidized lipids in pulmonary arterial hypertension

    Science.gov (United States)

    Ruffenach, Grégoire; Umar, Soban; Motayagheni, Negar; Reddy, Srinivasa T.; Eghbali, Mansoureh

    2016-01-01

    Abstract Pulmonary arterial hypertension (PAH) is a multifactorial disease characterized by interplay of many cellular, molecular, and genetic events that lead to excessive proliferation of pulmonary cells, including smooth muscle and endothelial cells; inflammation; and extracellular matrix remodeling. Abnormal vascular changes and structural remodeling associated with PAH culminate in vasoconstriction and obstruction of pulmonary arteries, contributing to increased pulmonary vascular resistance, pulmonary hypertension, and right ventricular failure. The complex molecular mechanisms involved in the pathobiology of PAH are the limiting factors in the development of potential therapeutic interventions for PAH. Over the years, our group and others have demonstrated the critical implication of lipids in the pathogenesis of PAH. This review specifically focuses on the current understanding of the role of oxidized lipids, lipid metabolism, peroxidation, and oxidative stress in the progression of PAH. This review also discusses the relevance of apolipoprotein A-I mimetic peptides and microRNA-193, which are known to regulate the levels of oxidized lipids, as potential therapeutics in PAH. PMID:27683603

  1. Review of bosentan in the management of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Eli Gabbay

    2008-01-01

    Full Text Available Eli Gabbay1, John Fraser2, Keith McNeil31Western Australian Lung Transplant Unit and Pulmonary Hypertension Service, Royal Perth Hospital, Western Australia, Australia; 2Critical Care Research Group, The Prince Charles Hospital, Rode Road, Chermside, Queensland, Australia; 3Transplant and Pulmonary Vascular Disease Unit, The Prince Charles Hospital, Rode Road, Chermside, Queensland, AustraliaAbstract: The dual endothelin receptor antagonist, bosentan, is an orally active therapy, which is effective in the treatment of pulmonary arterial hypertension (PAH. This review critically appraises the evidence for the efficacy of bosentan in idiopathic and familial PAH, in PAH associated with connective tissue disease and in PAH which may develop in association with other conditions. Data from the pivotal placebo controlled studies and their open labeled extensions as well as long term survival and quality of life data is presented. Data is also presented on the potential benefit of bosentan in patients with inoperable chronic thromboembolic pulmonary hypertension. The safety and tolerability of bosentan as well as drug interactions are discussed. Dosage recommendations in adults and pediatrics are presented. An algorithm is provided to guide the reader in monitoring potential increases in alanine and aspartate transaminase levels that may occur with bosentan use and the dose adjustments that are recommended as a result of any increase in the levels of these enzymes are shown. Finally, the role of bosentan as part of combination therapy in PAH is examined.Keywords: bosentan, pulmonary arterial hypertension, review

  2. Role of oxidized lipids in pulmonary arterial hypertension.

    Science.gov (United States)

    Sharma, Salil; Ruffenach, Grégoire; Umar, Soban; Motayagheni, Negar; Reddy, Srinivasa T; Eghbali, Mansoureh

    2016-09-01

    Pulmonary arterial hypertension (PAH) is a multifactorial disease characterized by interplay of many cellular, molecular, and genetic events that lead to excessive proliferation of pulmonary cells, including smooth muscle and endothelial cells; inflammation; and extracellular matrix remodeling. Abnormal vascular changes and structural remodeling associated with PAH culminate in vasoconstriction and obstruction of pulmonary arteries, contributing to increased pulmonary vascular resistance, pulmonary hypertension, and right ventricular failure. The complex molecular mechanisms involved in the pathobiology of PAH are the limiting factors in the development of potential therapeutic interventions for PAH. Over the years, our group and others have demonstrated the critical implication of lipids in the pathogenesis of PAH. This review specifically focuses on the current understanding of the role of oxidized lipids, lipid metabolism, peroxidation, and oxidative stress in the progression of PAH. This review also discusses the relevance of apolipoprotein A-I mimetic peptides and microRNA-193, which are known to regulate the levels of oxidized lipids, as potential therapeutics in PAH.

  3. [Treatment of pulmonary arterial hypertension in children].

    Science.gov (United States)

    Fraisse, A; Habib, G

    2004-08-01

    Treatment strategies for pulmonary hypertension in children have dramatically evolved. Traditional therapy with calcium channel blockers and pulmonary transplantation is only indicated in selected patients and does not reduce mortality very significantly. New pulmonary vasodilators are emerging from recent trials in the adult population. Their indications are based on the patient's NYHA classification. The epoprostenol (prostacyclin, Flolan) has shown reduction in mortality and improvement in functional symptoms in pediatric patients. The frequent side effects and continuous intravenous infusion limit the indication of prostacyclin in NYHA class IV children. The endothelin receptor blocker bosentan (Tracleer) is an orally given agent. It improves functional symptoms in adults and hemodynamic measures in children. It can be started in children with moderate functional symptoms (NYHA class II and III). The type V phosphodiesterase inhibitor sildenafil (Viagra) is being evaluated and may represent a promising therapy in the future. Invasive strategies like catheter-based atrial septostomy may be useful in particular cases. Randomized-controlled studies are urgently needed to evaluate the safety and efficacy of these new therapies.

  4. Surgical treatment of anomalous origin of coronary artery from the pulmonary artery

    Institute of Scientific and Technical Information of China (English)

    WU Qing-yu; XU Zhong-hua

    2008-01-01

    Background Anomalous origin of coronary artery from the pulmonary artery is a rare congenial cardiac malformation with a mortality rate of up to 90% within the first year of life without surgical intervention. Direct implantation of the anomalous coronary artery (ACA)into the aorta is successful in early life, but it may have increased surgical difficulty and risk with age. This retrospective study summarized our operative experience in direct implantation for treatment of this coronary anomaly in pediatric and adult patients. Methods From August 2000 to January 2003. 4 consecutive patients aged from 9 months to 41 years underwent dual coronary repair. Among them, two children and one infant with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA)and one adult was anomalous origin of right coronary artery from the pulmonary artery(ARCAPA). Coronary arteries were directly implanted into the ascending aorta in 4 patients. In a boy with ALCAPA associated with moderate mitral insufficiency(MI), whose ACA arose remotely from the ascending aorta, we created a tube-shaped graft using part of the pulmonary arterial wall in continuity with the origin of the left coronary artery (LCA). Concomitant moderate MI was repaired in 2 patients, including this boy, after a dual-coronary repair. Results All patients survived. There were no hospital or late deaths and no major complications as well. Echocardiography revealed that the left ventricular (LV)function including LV end-diastolic dimension(EDD)and ejection fraction(EF)was markedly improved at hospital discharge. At 3-6 years follow-up after surgery all patients were asymptomatic and currently in NYHA class I. Conclusions The best results are achieved with direct implantation of the ACA into the ascending aorta and simultaneous mitral valve repair if needed. Direct implantation is feasible in pediatric and adult patients with ALCAPA or ARCAPA including the coronary artery in a location remote from

  5. 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.

  6. Anomalous origin of the left coronary artery from the pulmonary artery: case report and review

    OpenAIRE

    Sandhu, K; Barron, D.; Jones, H.; Clift, P. (Peter); Thorne, S.; BUTLER, R.

    2016-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital condition that proves to be fatal in most individuals during childhood due to significant left ventricular ischaemia. However, there are case reports of individuals surviving into adulthood that have varying presenting symptoms. We report a case of a young male, who presented to our cardiology clinic with typical ischaemic cardiac pain, with no established risk factors, and was found to have anomalous ...

  7. Dopamine-induced cyclic AMP increase in canine myocardium, kidney and superior mesenteric artery.

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    Kazuno,Hiroshi

    1982-04-01

    Full Text Available The effect of dopamine on cyclic AMP levels in tissue slices of canine myocardium and kidney, and in chopped superior mesenteric arterial wall was investigated to identify dopamine receptors. Tissues were incubated in modified Krebs-Henseleit Ringer bicarbonate solution at 37 degrees C for 20 min with test drugs, after 20-min preincubation. In the presence of 3-isobutyl-1-methylxanthine (IBMX, dopamine and apomorphine caused dose-dependent increases in cyclic AMP levels in the myocardium, kidney and superior mesenteric artery. Phentolamine significantly intensified the cyclic AMP-increasing effect of dopamine in the superior mesenteric artery, but it did not influence the cyclic AMP increase caused by dopamine or apomorphine in the myocardium and kidney. Propranolol markedly blocked the effect of dopamine on cyclic AMP levels in all tissues studied. Haloperidol slightly inhibited the effect of dopamine and completely blocked the effect of apomorphine in the myocardium and kidney. These data suggest that dopamine increases cyclic AMP levels by activating predominantly beta-adrenergic receptors and partly dopamine receptors in the canine myocardium, kidney and superior mesenteric artery. The present results also suggest that dopamine acts not only on beta-adrenergic and dopamine receptors but also on alpha-adrenergic receptors in the superior mesenteric artery. Contrary to the activation of beta-adrenergic and dopamine receptors, the activation of alpha-adrenergic receptors resulted in a decrease in cyclic AMP levels in this tissue.

  8. 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.

  9. [Treatment of compression of the left main coronary artery in patients with pulmonary hypertension].

    Science.gov (United States)

    Talavera, María L; Diez, Mirta; Cáneva, Jorge O; Boughen, Roberto P; Valdivieso, León; Mendiz, Oscar

    2011-01-01

    Chest pain is a frequent symptom in patients with pulmonary hypertension of any etiology. Its pathophysiology has not been clearly established, the proposed causes are ischemia due to increased right ventricle wall stress, transient increased pulmonary hypertension resulting in acute pulmonary artery dilatation and external compression of the left main coronary artery (LMCA) by a dilated pulmonary artery. We report and discuss here three cases where the association between chest pain and compression of the LMCA by a dilated pulmonary artery could be shown, and they were treated with coronary stenting.

  10. 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...... of pulmonary arterial hypertension management, which places the patient at the centre of their own healthcare. Patients are thus becoming more proactive, involved and engaged in their self-care, and this engagement is an important factor if patient outcomes are to improve. In addition, involvement....... A multidisciplinary approach involving healthcare professionals, carers, patient associations and expert patient programmes can also encourage patients to engage. Strategies that promote patient engagement can help to achieve the best possible care and support for the patient and also benefit healthcare providers....

  11. 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 related...... to the shunt. METHODS: In a multicenter, double-blind, event-driven trial, we randomly assigned infants 92 days of age or younger with cyanotic congenital heart disease and a systemic-to-pulmonary-artery shunt to receive clopidogrel at a dose of 0.2 mg per kilogram of body weight per day (467 infants...... days of age. RESULTS: The rate of the composite primary end point did not differ significantly between the clopidogrel group (19.1%) and the placebo group (20.5%) (absolute risk difference, 1.4 percentage points; relative risk reduction with clopidogrel, 11.1%; 95% confidence interval, -19.2 to 33.6; P...

  12. Selexipag for the Treatment of Pulmonary Arterial Hypertension

    DEFF Research Database (Denmark)

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

    2015-01-01

    regimen prematurely because of adverse events. The most common adverse events in the selexipag group were consistent with the known side effects of prostacyclin, including headache, diarrhea, nausea, and jaw pain. CONCLUSIONS: Among patients with pulmonary arterial hypertension, the risk of the primary......-receptor antagonist, a phosphodiesterase type 5 inhibitor, or both. The primary end point was a composite of death from any cause or a complication related to pulmonary arterial hypertension up to the end of the treatment period (defined for each patient as 7 days after the date of the last intake of selexipag...... and hospitalization accounted for 81.9% of the events. The effect of selexipag with respect to the primary end point was similar in the subgroup of patients who were not receiving treatment for the disease at baseline and in the subgroup of patients who were already receiving treatment at baseline (including those...

  13. Endothelial GATA-6 Deficiency Promotes Pulmonary Arterial Hypertension

    Science.gov (United States)

    Ghatnekar, Angela; Chrobak, Izabela; Reese, Charlie; Stawski, Lukasz; Seta, Francesca; Wirrig, Elaine; Paez-Cortez, Jesus; Markiewicz, Margaret; Asano, Yoshihide; Harley, Russell; Silver, Richard; Feghali-Bostwick, Carol; Trojanowska, Maria

    2014-01-01

    Pulmonary arterial hypertension (PAH) is a chronic and progressive disease characterized by pulmonary vasculopathy with elevation of pulmonary artery pressure, often culminating in right ventricular failure. GATA-6, a member of the GATA family of zinc-finger transcription factors, is highly expressed in quiescent vasculature and is frequently lost during vascular injury. We hypothesized that endothelial GATA-6 may play a critical role in the molecular mechanisms underlying endothelial cell (EC) dysfunction in PAH. Here we report that GATA-6 is markedly reduced in pulmonary ECs lining both occluded and nonoccluded vessels in patients with idiopathic and systemic sclerosis-associated PAH. GATA-6 transcripts are also rapidly decreased in rodent PAH models. Endothelial GATA-6 is a direct transcriptional regulator of genes controlling vascular tone [endothelin-1, endothelin-1 receptor type A, and endothelial nitric oxide synthase (eNOS)], pro-inflammatory genes, CX3CL1 (fractalkine), 5-lipoxygenease-activating protein, and markers of vascular remodeling, including PAI-1 and RhoB. Mice with the genetic deletion of GATA-6 in ECs (Gata6-KO) spontaneously develop elevated pulmonary artery pressure and increased vessel muscularization, and these features are further exacerbated in response to hypoxia. Furthermore, innate immune cells including macrophages (CD11b+/F4/80+), granulocytes (Ly6G+/CD45+), and dendritic cells (CD11b+/CD11c+) are significantly increased in normoxic Gata6-KO mice. Together, our findings suggest a critical role of endothelial GATA-6 deficiency in development and disease progression in PAH. PMID:23583651

  14. [Intralobar pulmonary sequestration with multiple arterial blood supply].

    Science.gov (United States)

    Uroz Tristán, J; Mogueya, S A; Poenaru, D; Martínez Lagares, F; Arteaga García, R; Sanchís Solera, L; López-Pinto Ruiz, J

    1994-04-01

    We report the case of a 4 years old boy, who presented at our institution with reiterative neumonia affecting left basal lobe. Anomalous vascular appearance was detected in the chest x-ray. With the suspicion of pulmonary sequestration we carried on Digital Intravenous Angiography by Substraction (DIVAS) and aortogram. The anomalous systemic arterial supply was formed by 6 vessels coming from the thoracic aorta and going into the left lower lobe basal segment. Lobectomy was performed and previous diagnosis was confirmed pathologically.

  15. 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

  16. Epoprostenol sodium for treatment of pulmonary arterial hypertension

    OpenAIRE

    Saito Y.; Nakamura K; Akagi S; Sarashina T; Ejiri K; Miura A; Ogawa A.; Matsubara H; Ito H

    2015-01-01

    Yukihiro Saito,1 Kazufumi Nakamura,1 Satoshi Akagi,1 Toshihiro Sarashina,1 Kentaro Ejiri,1 Aya Miura,1 Aiko Ogawa,2 Hiromi Matsubara,2 Hiroshi Ito1 1Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan; 2Division of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan Abstract: The release of endogenous prostacyclin (PGI2) is depressed in patients with pulmonary arterial hyper...

  17. Pulmonary Vascular Capacitance as a Predictor of Vasoreactivity in Idiopathic Pulmonary Arterial Hypertension Tested by Adenosine

    Directory of Open Access Journals (Sweden)

    Shafie

    2015-09-01

    Full Text Available Background Acute pulmonary vasoreactivity testing has been recommended in the diagnostic work-up of patients with idiopathic pulmonary arterial hypertension (IPAH. Pulmonary arteriolar capacitance (Cp approximated by stroke volume divided by pulmonary pulse pressure (SV/PP is considered as an independent predictor of mortality in patients with IPAH. Objectives We sought to evaluate any differences in baseline and adenosine Cp between vasoreactive and non-vasoreactive IPAH patients tested with adenosine. Patients and Methods Fourteen patients with IPAH and a vasoreactive adenosine vasoreactivity testing according to the ESC guidelines were compared with 24 IPAH patients with nonreactive adenosine test results. Results There were no statistical significant differences between the two groups regarding NYHA class, body surface area, heart rate, and systemic blood pressure during right heart catheterization. Hemodynamic study showed no statistical significant differences in cardiac output/Index, mean pulmonary artery pressure, pulmonary vascular resistance, and baseline Cp between the two groups. There was a statistical significant but weak increase in adenosine Cp in vasoreactive group compared to non-reactive group (P = 0.04. Multivariable analysis showed an association between Cp and vasoreactivity (Beta = 2, P = 0.04, OR = 0.05 (95%CI = 0.003 - 0.9. Conclusions Cp could be considered as an index for the prediction of vasoreactivity in patients with IPAH. Prediction of long-term response to calcium channel blockers in patients with IPAH and a positive vasoreactive test by this index should be addressed in further studies.

  18. A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis.

    Science.gov (United States)

    Simsek, Pelin Ozlem; Ozcelik, Ugur; Celiker, Alpay; Yalcin, Ebru; Cobanoglu, Nazan; Pekcan, Sevgi; Alehan, Dursun; Ucar, Canan; Dogru, Deniz; Kiper, Nural

    2009-02-01

    Congenital unilateral absence of a pulmonary artery is a rare anomaly most frequently accompanied by other cardiovascular anomalies. We report a 10-year-old girl presenting with fatigue and recurrent hemoptysis who was initially misdiagnosed with idiopathic pulmonary hemosiderosis. Her symptoms did not resolve despite treatment so she was referred to our center for further evaluation. We carried out an angiography which revealed the absence of the right pulmonary artery and multiple collaterals originating from the right subclavian and right internal mammary arteries supplying the right lung. During the follow-up the patient developed a severe episode of pulmonary infection and pulmonary hypertension which responded well to medical treatment. Physicians should be aware of the congenital absence of the right pulmonary artery especially in patients presenting with recurrent respiratory symptoms. Although this condition is generally considered to have a good prognosis, close observation is mandatory in order to prevent further complications and comorbidities.

  19. Intravenous Epoprostenol for Management of Pulmonary Arterial Hypertension during Pregnancy

    Directory of Open Access Journals (Sweden)

    Julia Timofeev

    2013-10-01

    Full Text Available Background - Pulmonary arterial hypertension carries a high risk of mortality in pregnancy. Recent advances in treatment may improve disease course and allow for successful management of the pregnancy. Case Report - We present the case of a 20-year-old gravida 1, para 0 with diagnosis of severe primary pulmonary hypertension. The patient was managed with epoprostenol (prostacyclin infusion via an indwelling catheter, which was initiated at 23 weeks' gestation. The dose was adjusted to the patient's symptoms and a successful vaginal delivery was achieved at 36 weeks' gestation. Although maternal postpartum course was uncomplicated, unexplained neonatal demise occurred at 11 days of life. Conclusion - Successful management of pulmonary hypertension in pregnancy can be accomplished with a multidisciplinary approach and intensive therapy. Long-term effects of epoprostenol on fetal or neonatal well-being are unknown.

  20. Intravenous Epoprostenol for Management of Pulmonary Arterial Hypertension during Pregnancy.

    Science.gov (United States)

    Timofeev, Julia; Ruiz, George; Fries, Melissa; Driggers, Rita W

    2013-10-01

    Background Pulmonary arterial hypertension carries a high risk of mortality in pregnancy. Recent advances in treatment may improve disease course and allow for successful management of the pregnancy. Case Report We present the case of a 20-year-old gravida 1, para 0 with diagnosis of severe primary pulmonary hypertension. The patient was managed with epoprostenol (prostacyclin) infusion via an indwelling catheter, which was initiated at 23 weeks' gestation. The dose was adjusted to the patient's symptoms and a successful vaginal delivery was achieved at 36 weeks' gestation. Although maternal postpartum course was uncomplicated, unexplained neonatal demise occurred at 11 days of life. Conclusion Successful management of pulmonary hypertension in pregnancy can be accomplished with a multidisciplinary approach and intensive therapy. Long-term effects of epoprostenol on fetal or neonatal well-being are unknown.

  1. The Molecular Genetics and Cellular Mechanisms Underlying Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Rajiv D. Machado

    2012-01-01

    Full Text Available Pulmonary arterial hypertension (PAH is an incurable disorder clinically characterised by a sustained elevation of mean arterial pressure in the absence of systemic involvement. As the adult circulation is a low pressure, low resistance system, PAH represents a reversal to a foetal state. The small pulmonary arteries of patients exhibit luminal occlusion resultant from the uncontrolled growth of endothelial and smooth muscle cells. This vascular remodelling is comprised of hallmark defects, most notably the plexiform lesion. PAH may be familial in nature but the majority of patients present with spontaneous disease or PAH associated with other complications. In this paper, the molecular genetic basis of the disorder is discussed in detail ranging from the original identification of the major genetic contributant to PAH and moving on to current next-generation technologies that have led to the rapid identification of additional genetic risk factors. The impact of identified mutations on the cell is examined, particularly, the determination of pathways disrupted in disease and critical to pulmonary vascular maintenance. Finally, the application of research in this area to the design and development of novel treatment options for patients is addressed along with the future directions PAH research is progressing towards.

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

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    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.

  3. Postadulticide pulmonary hypertension of canine heartworm disease: successful treatment with oxygen and failure of antihistamines.

    Science.gov (United States)

    Rawlings, C A; Tackett, R L

    1990-10-01

    Postadulticide pulmonary hypertension mechanisms and treatment with antihistamines and supplemental oxygen were studied in eight dogs with heartworm disease. To ensure severe postadulticide thromboembolism, additional heartworms (either 20 or 40 into 4 dogs each) were transplanted into naturally infected dogs before thiacetarsamide treatment. During pentobarbital anesthesia, 2 pulmonary hemodynamic studies were conducted on each dog with a sequence of baseline, hypoxia with FlO2 = 10%, hyperoxia with FlO2 = 100%, a second baseline, treatment with either diphenhydramine (D) or cimetidine (C), and another hypoxia. All dogs were pulmonary hypertensive, with each dog having a mean pulmonary arterial pressure (PPA) greater than 20 mm of Hg. Mean PPA increased from baseline conditions (25.0 +/- 4.5 SD for D and 24.3 +/- 4.4 for C) to hypoxia (28.5 +/- 4.7 for D and 28.4 +/- 3.7 for C), and decreased during hyperoxia (16.9 +/- 3.0 for D and 17.4 +/- 3.0 for C), respectively. Neither antihistamine reduced PPA at normoxia. The degree of pulmonary hypertension when breathing room air increased even more during hypoxia, and this increase was not attenuated by either antihistamine. Histamine did not appear to mediate pulmonary hypertension during postadulticide thromboembolism, nor to modify the hypoxia-mediated pulmonary hypertension at this disease stage. Because baseline PO2 was low (66.6 +/- 11.7 mm of Hg for D and 69.4 +/- 14.2 for C) and because PPA decreased during administration of oxygen, the pulmonary hypertension was mostly hypoxia-induced. In addition to the arterial lesions, much of the pulmonary hypertensive mechanism was an active and reversible vasoconstriction in response to hypoxia caused by the secondary lung disease.(ABSTRACT TRUNCATED AT 250 WORDS)

  4. Imaging Features of Isolated Unilateral Pulmonary Artery Agenesis: a review of four cases

    OpenAIRE

    Batur, Abdussamet; Ödev, Kemal; Pekcan, Sevgi

    2015-01-01

    To highlight the variation in imaging and clinical manifestations of four cases of isolated unilateral absence of pulmonary artery presented in varied ages. Four patients with unilateral pulmonary artery agenesis were referred to our institution They underwent a series of investigations, including chest radiography, computed tomography and magnetic resonance imaging. Two of the four patients had absence of the right main pulmonary artery, while the remaining two patients had absence of the le...

  5. Visualization of peripheral pulmonary artery red thrombi utilizing optical coherence tomography

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Cheng; Wang, Wei; Zhong, Nan Shan; Zeng, Guang Qiao; Zhang, Nuo Fu [The First Affiliated Hospital of Guangzhou Medical College, Guangzhou (China)

    2013-10-15

    Optical coherence tomography (OCT) is a new imaging technique capable of obtaining high-resolution intravascular images and has been used in interventional cardiology. However, an application of OCT in pulmonary arteries had seldom been documented. In this case, OCT imaging is performed in peripheral pulmonary arteries and shows mural red thrombi. Subsequently, the red thrombi are aspirated and confirmed by a histological examination. These findings suggest that OCT may be a useful tool to depict peripheral pulmonary artery thrombi.

  6. Pulmonary artery rupture in a patient receiving an orthotopic heart transplant after total artificial heart explant.

    Science.gov (United States)

    Nomoto, Koichi; Weiner, Menachem M; Evans, Adam

    2014-02-01

    Our case illustrates a patient who suffered a pulmonary artery rupture despite previous total artificial heart implantation and replacement with orthotopic heart transplant. Pulmonary artery rupture during or following cardiac surgery has been reported to occur due to both pulmonary artery catheter use and surgical technique. Our case is the first to demonstrate the occurrence of this complication in the total artificial heart patient population.

  7. 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.

  8. A review of wave mechanics in the pulmonary artery with an emphasis on wave intensity analysis.

    Science.gov (United States)

    Su, J; Hilberg, O; Howard, L; Simonsen, U; Hughes, A D

    2016-12-01

    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 the dynamic compliance of the pulmonary arteries and the contribution of wave transmission. Wave intensity analysis offers an alternative way to assess the pulmonary vasculature in health and disease. Wave speed is a measure of arterial stiffness, and the magnitude and timing of wave reflection provide information on the degree of impedance mismatch between the proximal and distal circulation. Studies in the pulmonary artery have demonstrated distinct differences in arterial wave propagation between individuals with and without pulmonary vascular disease. Notably, greater wave speed and greater wave reflection are observed in patients with pulmonary hypertension and in animal models exposed to hypoxia. Studying wave propagation makes a valuable contribution to the assessment of the arterial system in pulmonary hypertension, and here, we briefly review the current state of knowledge of the methods used to evaluate arterial waves in the pulmonary artery.

  9. TEVAR for Flash Pulmonary Edema Secondary to Thoracic Aortic Aneurysm to Pulmonary Artery Fistula.

    Science.gov (United States)

    Bornak, Arash; Baqai, Atif; Li, Xiaoyi; Rey, Jorge; Tashiro, Jun; Velazquez, Omaida C

    2016-01-01

    Enlarging aneurysms in the thoracic aorta frequently remain asymptomatic. Fistulization of thoracic aortic aneurysms (TAA) to adjacent structures or the presence of a patent ductus arteriosus and TAA may lead to irreversible cardiopulmonary sequelae. This article reports on a large aneurysm of the thoracic aorta with communication to the pulmonary artery causing pulmonary edema and cardiorespiratory failure. The communication was ultimately closed after thoracic endovascular aortic aneurysm repair allowing rapid symptom resolution. Early diagnosis and closure of such communication in the presence of TAA are critical for prevention of permanent cardiopulmonary damage.

  10. Arterial desaturation due to pulmonary arteriovenous malformations after the Kawashima Operation

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    Rohit S Loomba

    2016-01-01

    Full Text Available Arterial desaturation may occur after the Kawashima procedure and, in the absence of venovenous collaterals is usually due to pulmonary arteriovenous malformations. Stenting of the pulmonary arteries, oxygen therapy, and pulmonary vasodilators such as sildenafil have not been able to resolve the arterial desaturation and the only way to do this has been Fontan completion. The time course of the formation of these malformations after the Kawashima and the progression of cyanosis and its resolution after the Fontan has only been demonstrated in case reports and small case series. We pool the available data to model arterial saturations in patients with pulmonary arteriovenous malformations after the Kawashima procedure.

  11. Medial defects of the small pulmonary arteries in fatal pulmonary hypertension in infants with trisomy 13 and trisomy 18.

    Science.gov (United States)

    Tahara, Masahiro; Shimozono, Saiko; Nitta, Tetsuya; Yamaki, Shigeo

    2014-02-01

    Congestive heart failure is a major cause of early death in patients with trisomy 13 or 18 and congenital heart disease (CHD). Pulmonary artery banding for these patients early in life is preferred to protect the lungs from high pulmonary flow rates and improve survival. We performed open lung biopsies in 11 patients with trisomy 13 or 18 accompanied by CHD and severe pulmonary artery hypertension (PAH) between 2009 and 2011. Two (18.2%) of these 11 patients had medial defects of the small pulmonary arteries. One patient with trisomy 13 and an atrial septal defect developed lung hemorrhage and lung edema at the age of 9 months and died at the age of 13 months. The lumens of the small pulmonary arteries of the other patient with trisomy 18 and a ventricular septal defect became occluded due to the intimal proliferation of fibrous tissues at the age of 2 months. This patient died at the age of 27 months. The deaths of both patients were associated with heart-related factors. Patients with medial defects are vulnerable to intimal proliferation in the small pulmonary arteries. More patients with trisomy 13 or 18 and CHD might have similar pulmonary vascular changes. The small pulmonary arteries of patients with trisomy 13 and 18 should be further analyzed.

  12. THE ROLES OF bcl-2 GENE FAMILY IN THE PULMONARY ARTERY REMODELING OF HYPOXIA PULMONARY HYPERTENSION IN RATS

    Institute of Scientific and Technical Information of China (English)

    杨成; 王胜发; 梁桃; 王巨; 王凯; 王柏春

    2001-01-01

    Objective. To investigate the roles of apoptosis in the pulmonary artery remodeling of pulmonary hypertension secondary to hypoxia and illustrate the relative genes expression.Methods. Thirty rats were divided into hypoxia group(10%O2, 8h/d) and normal control group. On the 15th day of hypoxia, pulmonary artery pressure and right ventricular hypertrophy index were measured and pulmonary artery vessels were studied by light microscope. Then terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling(TUNEL)technique was used to detect nucleosomal DNA fragmentation of apoptotic cells.In situ hybridization and RT-PCR were used to detect the expression level of bcl-2 and bax.``Results. The pulmonary artery pressure and right ventricular hypertrophy index of hypoxia group were increased significantly, the pulmonary artery wall of hypoxic group become incrassate than control group. Apoptotic cells can be found in lung with hypoxia or without hypoxia. Compared with control group, apoptotic index of hypoxic group decreased significantly. Through the methods of in situ hybridization and RT-PCR, we found the expression of bcl-2 increased whereas bax decreased significantly in the hypoxic group.``Conclusion. The alternation in bcl-2 and bax expression induced by hypoxia play an important role in the pulmonary artery remodeling which is the main pathologic change of pulmonary hypertension secondary to hypoxia.

  13. Effect of L-Arginine on Pulmonary Artery Smooth Muscle Cell Apoptosis in Rats with Hypoxic Pulmonary Vascular Structural Remodeling

    Institute of Scientific and Technical Information of China (English)

    Ingrid Karmane SUMOU; Jun-Bao DU; Bing WEI; Chun-Yu ZHANG; Jian-Guang QI; Chao-Shu TANG

    2006-01-01

    This study investigated the effect of L-arginine (L-Arg) on the apoptosis of pulmonary artery smooth muscle cells (PASMC) in rats with hypoxic pulmonary vascular structural remodeling, and its mechanisms. Seventeen Wistar rats were randomly divided into a control group (n=5), a hypoxia group (n=7), and a hypoxia+L-Arg group (n=5). The morphologic changes of lung tissues were observed under optical microscope. Using the terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphatebiotin nick end labeling assay, the apoptosis of PASMC was examined. Fas expression in PASMC was examined using immunohistochemistry. The results showed that the percentage of muscularized artery in small pulmonary vessels, and the relative medial thickness and relative medial area of the small and median pulmonary muscularized arteries in the hypoxic group were all significantly increased. Pulmonary vascular structural remodeling developed after hypoxia. Apoptotic smooth muscle cells of the small and median pulmonary arteries in the hypoxia group were significantly less than those in the control group. After 14 d of hypoxia, Fas expression by smooth muscle cells of median and small pulmonary arteries was significantly inhibited. L-Arg significantly inhibited hypoxic pulmonary vascular structural remodeling in association with an augmentation of apoptosis of smooth muscle cells as well as Fas expression in PASMC. These results showed that L-Arg could play an important role in attenuating hypoxic pulmonary vascular structural remodeling by upregulating Fas expression in PASMC, thus promoting the apoptosis of PASMC.

  14. [Successful pregnancy in a patient with idiopathic pulmonary arterial hypertension. Case report].

    Science.gov (United States)

    Szenczi, Orsolya; Karlócai, Kristóf; Bucsek, László; Rigó, János

    2016-04-10

    Idiopathic pulmonary arterial hypertension is characterized by progressive increase in pulmonary arterial pressure and pulmonary vascular resistance which lead to right ventricular failure and death. Pregnancy in patients with idiopathic pulmonary arterial hypertension is contraindicated because of the high maternal and fetal mortality. The authors present a case of successful pregnancy and delivery of a patient with idiopathic pulmonary arterial hypertension in Hungary for the first time. The aim of the report was to demonstrate that management and treatment of idiopathic pulmonary arterial hypertension in a pregnant woman is a complex and multidisciplinary task that should involve obstetrician, cardiologist and anesthesiologist. Those patients who become pregnant and do not wish to terminate the pregnancy must be referred to obstetric centers where a multidiciplinary approach is taken.

  15. 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.

  16. Echocardiographic presentation of anomalous origin of the left coronary artery from the pulmonary artery.

    Science.gov (United States)

    Silverman, Norman H

    2015-12-01

    In the 1970s, diagnosing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was often uncertain using imaging alone; however, with the advances in high-frequency transducers, advanced image processing, and other ultrasound modalities such as Doppler colour flow imaging, tissue Doppler imaging, and speckle tracking to asses regional wall motion abnormalities, modern echocardiography now permits accurate diagnosis of ALCAPA with greater certainty. Although many consider ultrasound to be the only imaging test necessary if there is a question as to the diagnosis, other imaging modalities such as MRI, CT, and cardiac catheterisation with angiography remain valuable complementary tests, especially in older patients.

  17. Left ventricular thrombus formation after repair of anomalous left coronary artery from the pulmonary artery.

    Science.gov (United States)

    Freud, Lindsay R; Koenig, Peter R; Russell, Hyde M; Patel, Angira

    2014-04-01

    Although thrombus formation following myocardial infarction in adults is well known, intracardiac thrombosis in children is uncommon. We report the case of a large left ventricular thrombus in an infant with ischemic cardiomyopathy secondary to anomalous origin of the left coronary artery from the pulmonary artery. Given its mobility and protrusion across the aortic valve, the patient underwent urgent thrombus removal through a transaortic approach. There were no embolic or neurologic complications. This case highlights that thrombectomy may be performed safely and successfully in critically ill pediatric patients.

  18. Characterization of optimal resting tension in human pulmonary arteries

    Science.gov (United States)

    Hussain, Azar; Bennett, Robert T; Chaudhry, Mubarak A; Qadri, Syed S; Cowen, Mike; Morice, Alyn H; Loubani, Mahmoud

    2016-01-01

    AIM To determine the optimum resting tension (ORT) for in vitro human pulmonary artery (PA) ring preparations. METHODS Pulmonary arteries were dissected from disease free sections of the resected lung in the operating theatre and tissue samples were directly sent to the laboratory in Krebs-Henseleit solution (Krebs). The pulmonary arteries were then cut into 2 mm long rings. PA rings were mounted in 25 mL organ baths or 8 mL myograph chambers containing Krebs compound (37 °C, bubbled with 21% O2: 5% CO2) to measure changes in isometric tension. The resting tension was set at 1-gram force (gf) with vessels being left static to equilibrate for duration of one hour. Baseline contractile reactions to 40 mmol/L KCl were obtained from a resting tension of 1 gf. Contractile reactions to 40 mmol/L KCl were then obtained from stepwise increases in resting tension (1.2, 1.4, 1.6, 1.8 and 2.0 gf). RESULTS Twenty PA rings of internal diameter between 2-4 mm were prepared from 4 patients. In human PA rings incrementing the tension during rest stance by 0.6 gf, up to 1.6 gf significantly augmented the 40 mmol/L KCl stimulated tension. Further enhancement of active tension by 0.4 gf, up to 2.0 gf mitigate the 40 mmol/L KCl stimulated reaction. Both Myograph and the organ bath demonstrated identical conclusions, supporting that the radial optimal resting tension for human PA ring was 1.61 g. CONCLUSION The radial optimal resting tension in our experiment is 1.61 gf (15.78 mN) for human PA rings. PMID:27721938

  19. 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.

  20. A comparison of pulmonary arterial occlusion algorithms for estimation of pulmonary capillary pressure.

    Science.gov (United States)

    Pellett, A A; Johnson, R W; Morrison, G G; Champagne, M S; deBoisblanc, B P; Levitzky, M G

    1999-07-01

    Using the arterial occlusion method, we compared five literature-based estimates of pulmonary capillary pressure (Ppc) with the corresponding double occlusion pressures (Pdo) in anesthetized dogs whose chests had been closed after sternotomy for instrumentation. Arterial occlusions were performed with a balloon-tipped pulmonary artery catheter that housed pressure transducers immediately proximal and distal to the balloon. Separation of the proximal and distal pressure waveforms during balloon inflation allowed us to precisely define the moment of occlusion. We fit a monoexponential curve to pressure data beginning 200 ms after the onset of occlusion and a biexponential curve to data beginning at the instant of occlusion, with data obtained over a range of vascular states (control, serotonin infusion, histamine infusion). In addition, we investigated the use of sampling of the raw data to estimate capillary pressure. Three of the five literature-based estimates of Ppc yielded values similar to Pdo. The optimal (least average difference from Pdo) interpolation/extrapolation time points of the curve fits varied, depending on the type of curve fitting and the state of the pulmonary vasculature. We also determined that a close approximation of Pdo may be derived from the raw data, as an alternative to exponential curve fitting.

  1. Gene Expression Profiling of Pulmonary Artery in a Rabbit Model of Pulmonary Thromboembolism

    Science.gov (United States)

    Huang, Jianfei; Zhou, Xiaoyu; Xie, Hao; Zhu, Qilin; Huang, Minjie

    2016-01-01

    Acute pulmonary thromboembolism (PTE) refers to the obstruction of thrombus in pulmonary artery or its branches. Recent studies have suggested that PTE-induced endothelium injury is the major physiological consequence of PTE. And it is reasonal to use PTE-induced endothelium injury to stratify disease severity. According to the massive morphologic and histologic findings, rabbit models could be applied to closely mimic the human PE. Genomewide gene expression profiling has not been attempted in PTE. In this study, we determined the accuracy of rabbit autologous thrombus PTE model for human PTE disease, then we applied gene expression array to identify gene expression changes in pulmonary arteries under PTE to identify potential molecular biomarkers and signaling pathways for PTE. We detected 1343 genes were upregulated and 923 genes were downregulated in PTE rabbits. The expression of several genes (IL-8, TNF-α, and CXCL5) with functional importance were further confirmed in transcript and protein levels. The most significantly differentially regulated genes were related to inflammation, immune disease, pulmonary disease, and cardiovascular diseases. Totally 87 genes were up-regulated in the inflammatory genes. We conclude that gene expression profiling in rabbit PTE model could extend the understanding of PTE pathogenesis at the molecular level. Our study provides the fundamental framework for future clinical research on human PTE, including identification of potential biomarkers for prognosis or therapeutic targets for PTE. PMID:27798647

  2. Gene Expression Profiling of Pulmonary Artery in a Rabbit Model of Pulmonary Thromboembolism.

    Science.gov (United States)

    Tang, Zhiyuan; Wang, Xudong; Huang, Jianfei; Zhou, Xiaoyu; Xie, Hao; Zhu, Qilin; Huang, Minjie; Ni, Songshi

    2016-01-01

    Acute pulmonary thromboembolism (PTE) refers to the obstruction of thrombus in pulmonary artery or its branches. Recent studies have suggested that PTE-induced endothelium injury is the major physiological consequence of PTE. And it is reasonal to use PTE-induced endothelium injury to stratify disease severity. According to the massive morphologic and histologic findings, rabbit models could be applied to closely mimic the human PE. Genomewide gene expression profiling has not been attempted in PTE. In this study, we determined the accuracy of rabbit autologous thrombus PTE model for human PTE disease, then we applied gene expression array to identify gene expression changes in pulmonary arteries under PTE to identify potential molecular biomarkers and signaling pathways for PTE. We detected 1343 genes were upregulated and 923 genes were downregulated in PTE rabbits. The expression of several genes (IL-8, TNF-α, and CXCL5) with functional importance were further confirmed in transcript and protein levels. The most significantly differentially regulated genes were related to inflammation, immune disease, pulmonary disease, and cardiovascular diseases. Totally 87 genes were up-regulated in the inflammatory genes. We conclude that gene expression profiling in rabbit PTE model could extend the understanding of PTE pathogenesis at the molecular level. Our study provides the fundamental framework for future clinical research on human PTE, including identification of potential biomarkers for prognosis or therapeutic targets for PTE.

  3. 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.

  4. THE ROLES OF bcl-2 GENE FAMILY IN THE PULMONARY ARTERY REMODELING OF HYPOXIA PULMONARY HYPERTENSION IN RATS

    Institute of Scientific and Technical Information of China (English)

    王巨; 王凯; 王柏春; 杨成; 王胜发; 梁桃

    2001-01-01

    Objective. To investigate the roles of apeptosis in the pulmonary artery remodeling of pulmonary hypertension secondary to hypoxia and illustrate the relative genes expression. Methods. Thirty rats were divided into hypoxia group(10%O2, 8h/d) and normal control group. On the 15th day of hypoxia, pulmonary artery pressure and fight ventricular hypertrophy index were measured and pulmonary artery vessels were studied by light microscope. Then terminal deoxynucleotidyl transferase-mediateddUTP nick-end labeling(TUNEL)technique was used to detect nucleosomal DNA fragmentation of apeptotic cells.In situ hybridization and RT-PCR were used to detect the expression level of bel-2 and bax. Results. The pulmonary artery pressure and right ventricular hypertrophy index of hypoxia group were increased significantly, the pulmonary artery wall of hypoxic group become incrassate than control group. Apeptotic cells can be found in lung with hypoxia or without hypexia. Compared with control group, apeptotic index of hypoxic group decreased significantly. Through the methods of in situ hybridization and RT-PCR, we found the expression of bel-2 increased whereas bax decreased significantly in the hypoxic group. Conclusion. The alternation in bel-2 and bax expression induced by hypoxia play an important role in the pulmonary artery remodeling which is the main pathologic change of p~monary hypertension secondary to hypoxia.

  5. Effect of low-molecular-weight heparin and urokinase on pulmonary arteries involved in pulmonary embolism

    Institute of Scientific and Technical Information of China (English)

    WU Jun-ping; SUN Xin; WU Qi; DU Zhong-zhen; LI Li; WU Qian; SUN Hong-fen

    2013-01-01

    Background Pulmonary embolism (PE) is a common and often fatal disease.Early after pulmonary thromboembolism,inflammation and associated intimal hyperplasia occur within the pulmonary arteries,similar to what is observed with chronic thromboembolic pulmonary hypertension.This study tested the hypothesis that thrombolytic and anticoagulant agents would have anti-inflammatory effects or inhibit intimal hyperplasia of involved pulmonary arteries.Methods Seventy-two male New Zealand white rabbits were randomly divided into two groups (54 rabbits in the PE group and 18 in the sham group).Experimental PE was induced in 54 rabbits by femoral vein injection of autologous blood clots and confirmed with pulmonary angiography,and other 18 rabbits underwent sham operations.Fifty-four rabbits in the PE group were randomly divided into three groups:a control group (treated with normal saline),a low-molecularweight heparin (LMWH) group (treated with LMWH),and a urokinase (UK) group (treated with UK).Arterial blood gas was analyzed at 2,7,and 28 days (n=6 per time point by random group division),then lung tissues were removed and were analyzed for pro-inflammatory cytokines and chemokines,and were stained for intimal hyperplasia.Results The overall survival of rabbits undergoing PE was 100%.PE distribution detected on digital signal angiography (DSA) and histopathology was shown in 67% of rabbits (36/54) in the bilateral low lobar pulmonary arteries (PAs).The results showed that alveolar-arterial partial pressure of oxygen (PO2) difference (PA-aO2) significantly increased and PO2 decreased in the control group compared with the sham group.Compared with controls,the UK group had a decreased level of PA-aO2 on day 2 (P <0.05),however,there was no significant difference in the LMWH group.Compared with controls,the LMWH group had a decreased level of monocyte chemoattractant protein-1 (MCP-1) in affected tissue and serum samples on days 7 and 28 (P <0.05),and the UK group had

  6. Severe pulmonary arterial hypertension due to Angiostrongylosus vasorum in a dog.

    Science.gov (United States)

    Nicolle, Audrey P; Chetboul, Valérie; Tessier-Vetzel, Dominique; Carlos Sampedrano, Carolina; Aletti, Edouard; Pouchelon, Jean-Louis

    2006-08-01

    A dog was presented with a history of dyspnea, coughing, and ascites. Angiostrongylosis and severe pulmonary arterial hypertension (PAH) were found, as well as a marked discordance between the electrical and mechanical events of the heart. Pulmonary arterial hypertension related to Angiostrongylus vasorum has rarely been reported.

  7. The role of inflammation and autoimmunity in the pathophysiology of pulmonary arterial hypertension.

    Science.gov (United States)

    Kherbeck, Nada; Tamby, Mathieu C; Bussone, Guillaume; Dib, Hanadi; Perros, Frederic; Humbert, Marc; Mouthon, Luc

    2013-02-01

    Pulmonary arterial hypertension is characterized by a remodeling of pulmonary arteries with endothelial cell, fibroblast, and vascular smooth muscle cell activation and proliferation. Since pulmonary arterial hypertension occurs frequently in autoimmune conditions such as systemic sclerosis, inflammation and autoimmunity have been suspected to play a critical role in both idiopathic pulmonary arterial hypertension and systemic sclerosis-associated pulmonary arterial hypertension. High levels of pro-inflammatory cytokines such as interleukin-1 and interleukin-6, platelet-derived growth factor, or macrophage inflammatory protein 1 have been found in lung samples of patients with pulmonary arterial hypertension, along with inflammatory cell infiltrates mainly composed of macrophages and dendritic cells, T and B lymphocytes. In addition, circulating autoantibodies are found in the peripheral blood of patients. Thus, autoimmunity and inflammation probably play a role in the development of pulmonary arterial hypertension. In this setting, it would be important to set-up new experimental models of pulmonary arterial hypertension, in order to define novel therapeutics that specifically target immune disturbances in this devastating condition.

  8. 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

  9. Intrapulmonary arteries respond to serotonin and adenosine triphosphate in broiler chickens susceptible to idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Kluess, H A; Stafford, J; Evanson, K W; Stone, A J; Worley, J; Wideman, R F

    2012-06-01

    This study examined factors contributing to increased vascular resistance and plexiform lesion formation in broiler chickens susceptible to idiopathic pulmonary arterial hypertension (IPAH). A diet supplemented with excess tryptophan (high-Trp diet), the precursor for serotonin, was used to accelerate the development of IPAH. Broilers fed the high-Trp diet had higher pulmonary arterial pressures than broilers fed the control diet, and plexiform lesion incidences tended to be higher (P = 0.11) in the high-Trp group than in the control group at 30 d of age. The intrapulmonary arteries were assessed for vasoconstriction in response to serotonin and adenosine triphosphate (ATP) and for activities of key metabolic enzymes for serotonin and ATP. The pulmonary artery (defined as the first major branch of the pulmonary artery inside the lung) and the primary pulmonary arterial rami (defined as the second major branch of the pulmonary artery inside the lung) both exhibited vasoconstriction in response to serotonin and ATP. This is the first study to demonstrate purinergic-mediated vasoconstriction in intrapulmonary arteries from broilers. Arteriole responsiveness did not differ between broilers fed the control diet or the high-Trp diet. Therefore, the high-Trp diet enhanced the development of IPAH but did not affect the artery's sensitivity to serotonin or ATP. Monoamine oxidase activity, responsible for the breakdown of serotonin, was severely impaired in pulmonary arteries from broilers in the high-Trp group. Accordingly, serotonin may persist longer and elicit an amplified response in broilers fed the high-Trp diet.

  10. Comparison of clinical characteristics and survival on patients with idiopathic pulmonary arterial hypertension and familial pulmonary arterial hypertension during conventional therapy era and targeted therapy era

    Institute of Scientific and Technical Information of China (English)

    徐希奇

    2014-01-01

    Objective To compare the clinical characteristics and survival on Chinese patients with idiopathic pulmonary arterial hypertension(IPAH)and familiar pulmonary arterial hypertension(FPAH)during conventional therapy era and targeted therapy era.Methods IPAH and FPAH patients who were referred between Jan 1999and Oct 2004 in Fuwai Hospital were defined as conventional therapy era group(before 2005 no PAH-specific drug was available in China).All patients in this group

  11. Initial experience with tadalafil in pediatric pulmonary arterial hypertension.

    Science.gov (United States)

    Takatsuki, Shinichi; Calderbank, Michelle; Ivy, David Dunbar

    2012-06-01

    This study aimed to investigate the safety, tolerability, and effects of tadalafil on children with pulmonary arterial hypertension (PAH) after transition from sildenafil or after tadalafil received as initial therapy. A total of 33 pediatric patients with PAH were retrospectively evaluated. Of the 33 patients, 29 were switched from sildenafil to tadalafil. The main reason for the change from sildenafil was once-daily dosing. The average dose of sildenafil was 3.4 ± 1.1 mg/kg/day, and that of tadalafil was 1.0 ± 0.4 mg/kg/day. For 14 of the 29 patients undergoing repeat catheterization, statistically significant improvements were observed after transition from sildenafil to tadalafil in terms of mean pulmonary arterial pressure (53.2 ± 18.3 vs. 47.4 ± 13.7 mmHg; p sildenafil to tadalafil including headache, nausea, myalgia, nasal congestion, flushing, and allergic reaction. Two patients discontinued tadalafil due to migraine or allergic reaction. One patient receiving sildenafil had no breakthrough syncope after transition to tadalafil. Tadalafil can be safely used for pediatric patients with PAH and may prevent disease progression.

  12. Histamine releases PGI2 from human pulmonary artery.

    Science.gov (United States)

    Schellenberg, R R; Duff, M J; Foster, A; Paddon, H B

    1986-08-01

    Histamine caused a triphasic response of human pulmonary artery strips in vitro, consisting of a small initial contraction followed by pronounced relaxation preceding a second contractile response. These characteristics were not seen with other contractile stimuli including 5-hydroxytryptamine, leukotriene D4, and KCl. The relaxant component of this response was ablated by removal of endothelium from the vascular strips or by pretreatment of the tissue with 1 microM indomethacin. Measurement of the PGI2 degradation product 6-keto-PGF1 alpha in supernatants from histamine-challenged tissues confirmed the synthesis of PGI2. Supernatants from unstimulated or leukotriene-challenged tissues contained no detectable amounts of 6-keto-PGF1 alpha. The histamine H1 antagonist diphenydramine inhibited both the contractile and relaxant responses to histamine whereas the H2 antagonist cimetidine affected neither component. The released PGI2 significantly altered the dose-response curve to histamine without inhibiting the maximal contractile responses. We conclude that histamine induces PGI2 formation from pulmonary arterial endothelium via an H1 receptor.

  13. H2S inhibits pulmonary arterial endothelial cell inflammation in rats with monocrotaline-induced pulmonary hypertension.

    Science.gov (United States)

    Feng, Shasha; Chen, Siyao; Yu, Wen; Zhang, Da; Zhang, Chunyu; Tang, Chaoshu; Du, Junbao; Jin, Hongfang

    2017-03-01

    This study aimed to determine whether hydrogen sulfide (H2S) inhibits pulmonary arterial endothelial inflammation in rats with monocrotaline (MCT)-induced pulmonary hypertension and its possible mechanisms. Twenty-four male Wistar rats were divided randomly into control, MCT, and MCT+H2S treatment groups. Human pulmonary arterial endothelial cells (HPAEC) were cultured and divided into four groups: control, MCT, MCT+H2S, and H2S. Pulmonary artery pressure was determined using a right cardiac catheterization procedure 3 weeks after MCT administration. Pulmonary vascular morphological changes and inflammatory infiltration were measured. Endogenous H2S levels, cystathionine-γ-lyase (CSE) expression, and inflammatory cytokines were determined both in vivo and in vitro. In addition, phosphorylation of NF-κB p65 and IκBα was detected by western blotting, and NF-κB p65 nuclear translocation, as well as its DNA-binding activity, was determined. Pulmonary hypertension and vascular remolding developed 3 wks after MCT administration, with elevated lung tissue inflammatory infiltration and cytokine level associated with activation of the NF-κB pathway, both in vivo and in vitro. However, the endogenous H2S/CSE pathway was downregulated in MCT rats. By contrast, an H2S donor markedly reduced pulmonary artery pressure, pulmonary vascular structural remolding, and increased lung inflammatory infiltration and cytokine levels of MCT-treated rats. Meanwhile, H2S reversed the activation of the NF-κB pathway successfully. The downregulated pulmonary arterial endothelial H2S/CSE pathway is involved in the pulmonary inflammatory response in MCT-treated pulmonary hypertensive rats. H2S attenuated endothelial inflammation by inhibiting the NF-κB pathway.

  14. Changing demographics of pulmonary arterial hypertension in congenital heart disease

    Directory of Open Access Journals (Sweden)

    B.J.M. Mulder

    2010-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a serious complication of congenital heart disease (CHD. Without early surgical repair, around one-third of paediatric CHD patients develop significant PAH. Recent data from the Netherlands suggest that >4% of adult CHD patients have PAH, with higher rates in those with septal defects. A spectrum of cardiac defects is associated with PAH-CHD, although most cases develop as a consequence of large systemic-to-pulmonary shunts. Eisenmenger's syndrome, characterised by reversed pulmonary-to-systemic (right-to-left shunt, represents the most advanced form of PAH-CHD and affects as many as 50% of those with PAH and left-to-right shunts. It is associated with the poorest outcome among patients with PAH-CHD. 40 yrs ago, ∼50% of children with CHD requiring intervention died within the first year, and <15% survived to adulthood. Subsequent advances in paediatric cardiology have seen most patients with CHD survive to adulthood, with resulting shifts in the demographics of CHD and PAH-CHD. The number of adults presenting with CHD is increasing and, although mortality is decreasing, morbidity is increasing as older patients are at increased risk of arrhythmia, heart failure, valve regurgitation and PAH. Data show that probability of PAH increases with age in patients with cardiac defects.

  15. 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.

  16. Pulmonary arterial hypertension associated with congenital heart disease

    Directory of Open Access Journals (Sweden)

    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.

  17. 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.

  18. Increased Mutagen Sensitivity and DNA Damage in Pulmonary Arterial Hypertension

    Science.gov (United States)

    Federici, Chiara; Drake, Kylie M.; Rigelsky, Christina M.; McNelly, Lauren N.; Meade, Sirena L.; Comhair, Suzy A. A.; Erzurum, Serpil C.

    2015-01-01

    Rationale: Pulmonary arterial hypertension (PAH) is a serious lung condition characterized by vascular remodeling in the precapillary pulmonary arterioles. We and others have demonstrated chromosomal abnormalities and increased DNA damage in PAH lung vascular cells, but their timing and role in disease pathogenesis is unknown. Objectives: We hypothesized that if DNA damage predates PAH, it might be an intrinsic cell property that is present outside the diseased lung. Methods: We measured DNA damage, mutagen sensitivity, and reactive oxygen species (ROS) in lung and blood cells from patients with Group 1 PAH, their relatives, and unrelated control subjects. Measurements and Main Results: Baseline DNA damage was significantly elevated in PAH, both in pulmonary artery endothelial cells (P < 0.05) and peripheral blood mononuclear cells (PBMC) (P < 0.001). Remarkably, PBMC from unaffected relatives showed similar increases, indicating this is not related to PAH treatments. ROS levels were also higher (P < 0.01). DNA damage correlated with ROS production and was suppressed by antioxidants (P < 0.001). PBMC from patients and relatives also showed markedly increased sensitivity to two chemotherapeutic drugs, bleomycin and etoposide (P < 0.001). Results were consistent across idiopathic, heritable, and associated PAH groups. Conclusions: Levels of baseline and mutagen-induced DNA damage are intrinsically higher in PAH cells. Similar results in PBMC from unaffected relatives suggest this may be a genetically determined trait that predates disease onset and may act as a risk factor contributing to lung vascular remodeling following endothelial cell injury. Further studies are required to fully characterize mutagen sensitivity, which could have important implications for clinical management. PMID:25918951

  19. Galectin-3 induces pulmonary artery endothelial cell morphogenesis and angiogenesis

    Institute of Scientific and Technical Information of China (English)

    ZHANG Li; LI Yu-mei; WANG Xiao-yan; ZHU Da-ling

    2016-01-01

    AIM:Increasing evidence suggests that carbohydrate-binding proteins play an essential role in tumor growth and metastasis .Ga-lectin-3, a multifunctional protein of an expanding family of β-galactoside-binding animal lectins , is the major nonintegrin cellular laminin-binding protein , and is implicated in a variety of biologic events , such as inflammation and angiogenesis .Because galectin-3 expression was shown to participate in mediating tumor angiogenesis and initiate signaling cascades in several diseases .We hypothe-sized that galectin-3 may promote pulmonary vascular endothelial neovascularization .METHODS:Hypoxic and MCT rat model of pul-monary artery remodeling was used .The mRNA and protein levels of galectin-3 in rats were measured by in situ hybrization and West-ern blot analysis.Endothelial cell (EC) proliferation, migration and tube formation were measured using MTT , cell scratch and Matri-gel assays, respectively.Protein expression was quantitated by Western blot analysis .LC 3A/B staining was detected with cellular im-munofluorescence staining .RESULTS:We found that galectin-3 was localized on the intima and adventitial wall .Galectin-3 was in-creased after rat hypoxia and MCT administration .Galectin-3 promoted EC proliferation , migration and tube formation , while its roles were reversed by RNA interference.Galectin-3 induced Atg 5, Beclin-1, LAMP-2, and LC 3A/B expression increases.Galectin-3 al-so increased LC 3A/B staining in ECs.Akt/mTOR and GSK-3βsignaling pathways were activated after galectin-3 treated ECs using its specific phosphorylation antibodies , while blocked it with LY294002 inhibited cell autophagy and EC dynamic alterations induced by galectin-3.CONCLUSION:These findings demonstrate that galectin-3 can induce an Akt signaling cascade leading to cell autoph-agy, and then the differentiation and angiogenesis of pulmonary artery endothelial cells .

  20. 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.

  1. 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.

  2. Management of a child with pulmonary arterial hypertension presenting with systemic hypertension.

    Science.gov (United States)

    Flores, Saul; Daily, Joshua; Pratap, Jayant Nick; Cash, Michelle C; Hirsch, Russel

    2016-02-01

    We describe the course and management of a 12-year-old girl with severe pulmonary arterial hypertension who initially presented with severe systemic hypertension. Successful therapy included pulmonary vasodilators and an atrial septostomy, while ensuring adequate maintenance of her systemic vascular resistance to maintain cardiac output. Clear understanding of the physiology and judicious medical management in patients with severe pulmonary arterial hypertension using extreme compensatory mechanisms is vitally important.

  3. Effects of platelet-derived growth factor on the function of smooth muscle cells from different orders of pulmonary artery

    Institute of Scientific and Technical Information of China (English)

    国桓

    2014-01-01

    Objective To explore the functional responses of normal rat pulmonary artery smooth muscle cells(PASMCs)from different orders of pulmonary artery to the platelet-derived growth factor(PDGF).Methods The pulmonary artery branches were gently isolated from Sprague-Dawley rats(250-350 g)and eventually cut into three groups according to the vascular grading:the

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

    Energy Technology Data Exchange (ETDEWEB)

    Cowles, Robert A. [Morgan Stanley Children' s Hospital of New York-Presbyterian, Division of Pediatric Surgery,Columbia University College of Physicians and Surgeons, New York, NY (United States); Berdon, Walter E. [Morgan Stanley Children' s Hospital of New York-Presbyterian, Department of Pediatric Radiology, Columbia University College of Physicians and Surgeons, New York, NY (United States)

    2007-09-15

    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.)

  5. Why there is a need to discuss pulmonary hypertension other than pulmonary arterial hypertension?

    Science.gov (United States)

    Papathanasiou, Athanasios; Nakos, George

    2015-11-04

    Pulmonary hypertension (PH) is a condition characterized by the elevation of the mean pulmonary artery pressure above 25 mmHg and the pulmonary vascular resistance above 3 wood units. Pulmonary arterial hypertension (PAH) is an uncommon condition with severe morbidity and mortality, needing early recognition and appropriate and specific treatment. PH is frequently associated with hypoxemia, mainly chronic obstructive pulmonary disease and DPLD and/or left heart diseases (LHD), mainly heart failure with reduced or preserved ejection fraction. Although in the majority of patients with PH the cause is not PAH, a significant number of published studies are still in regard to group I PH, leading to a logical assumption that PH due to other causes is not such an important issue. So, is there a reason to discuss PH other than PAH? Chronic lung diseases, mainly chronic obstructive lung disease and DPLD, are associated with a high incidence of PH which is linked to exercise limitations and a worse prognosis. Although pathophysiological studies suggest that specific PAH therapy may benefit such patients, the results presented from small studies in regard to the safety and effectiveness of the specific PAH therapy are discouraging. PH is a common complication of left heart disease and is related to disease severity, especially in patients with reduced ejection fraction. There are two types of PH related to LHD based on diastolic pressure difference (DPD, defined as diastolic pulmonary artery pressure - mean PAWP): Isolated post-capillary PH, defined as PAWP > 15 mmHg and DPD 15 mmHg and DPD ≥ 7 mmHg. The potential use of PAH therapies in patients with PH related to left heart disease is based on a logical pathobiological rationale. In patients with heart failure, endothelial dysfunction has been proposed as a cause of PH and hence as a target for treatment, supported by the presence of increased endothelin-1 activity and impaired nitric oxide-dependent vasodilation

  6. Anomalous Origins of Coronary Arteries From the Pulmonary Artery: A Comprehensive Review of Literature and Surgical Options.

    Science.gov (United States)

    Karimi, Mohsen; Kirshbom, Paul M

    2015-10-01

    Anomalous origins of coronary arteries from the pulmonary artery are rare malformations in which the coronary arteries originate from pulmonary artery sinuses or branches. The consequences are variable although, in most cases, these anomalies lead to severe coronary hypoperfusion and ventricular dysfunction. Surgical correction is indicated once the diagnosis is established due to high early mortality associated with the disease. In nearly all cases, the anomalous artery can be excised from its pulmonary origin, mobilized, and reimplanted directly into the ascending aorta using different surgical techniques. In rare circumstances, technical modifications must be used to restore a normal dual coronary perfusion. The emphasis of this article is to provide a collective review of surgical options published in the literature.

  7. Clinical characteristics of in-situ pulmonary artery thrombosis in Korea.

    Science.gov (United States)

    Cha, Seung-Ick; Choi, Keum-Ju; Shin, Kyung-Min; Lim, Jae-Kwang; Yoo, Seung-Soo; Lee, Jaehee; Lee, Shin-Yup; Kim, Chang-Ho; Park, Jae-Yong

    2015-12-01

    Little is known regarding the clinical features and course of in-situ pulmonary artery thrombosis (PAT). The aim of the present study was to investigate the clinical characteristics of PAT. Patients with PAT were retrospectively identified from a tertiary referral center in South Korea. A control group consisted of patients with pulmonary embolism in whom the right or left pulmonary artery was the largest pulmonary embolism-involved site. We compared various clinical parameters between the two groups. Of the 23 PAT patients, the most common underlying condition was tuberculosis-destroyed lung [11 (47.8%)], followed by pulmonary artery stump after lobectomy or pneumonectomy [7 (30.4%)]. In all patients except one, PAT was located in the right or left pulmonary artery. Computed tomography scans demonstrated that clots were completely or partially resolved less frequently in the PAT group than in the control group [4 (25%) versus 62 (90%); P PAT group, three of the seven patients (43%) who had undergone anticoagulation therapy exhibited improvement, and one of the nine patients (11%) who had not received anticoagulation therapy experienced improvement; however, the difference was not significant. Tuberculosis-destroyed lung was the most common underlying condition in Korean PAT patients, followed by pulmonary artery stump after lung resection. The clots in patients with PAT were mostly located in the right or left pulmonary artery, and clot resolution was less frequent in the PAT group compared to the pulmonary embolism group.

  8. Copper dependence of angioproliferation in pulmonary arterial hypertension in rats and humans.

    Science.gov (United States)

    Bogaard, Harm J; Mizuno, Shiro; Guignabert, Christophe; Al Hussaini, Aysar A; Farkas, Daniela; Ruiter, Gerrina; Kraskauskas, Donatas; Fadel, Elie; Allegood, Jeremy C; Humbert, Marc; Vonk Noordegraaf, Anton; Spiegel, Sarah; Farkas, Laszlo; Voelkel, Norbert F

    2012-05-01

    Obliteration of the vascular lumen by endothelial cell growth is a hallmark of many forms of severe pulmonary arterial hypertension. Copper plays a significant role in the control of endothelial cell proliferation in cancer and wound-healing. We sought to determine whether angioproliferation in rats with experimental pulmonary arterial hypertension and pulmonary microvascular endothelial cell proliferation in humans depend on the proangiogenic action of copper. A copper-depleted diet prevented, and copper chelation with tetrathiomolybdate reversed, the development of severe experimental pulmonary arterial hypertension. The copper chelation-induced reopening of obliterated vessels was caused by caspase-independent apoptosis, reduced vessel wall cell proliferation, and a normalization of vessel wall structure. No evidence was found for a role of super oxide-1 inhibition or lysyl-oxidase-1 inhibition in the reversal of angioproliferation. Tetrathiomolybdate inhibited the proliferation of human pulmonary microvascular endothelial cells, isolated from explanted lungs from control subjects and patients with pulmonary arterial hypertension. These data suggest that the inhibition of endothelial cell proliferation by a copper-restricting strategy could be explored as a new therapeutic approach in pulmonary arterial hypertension. It remains to be determined, however, whether potential toxicity to the right ventricle is offset by the beneficial pulmonary vascular effects of antiangiogenic treatment in patients with pulmonary arterial hypertension.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  10. Left ventricular dysfunction in patients with suspected pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Francisca Gavilanes

    2014-12-01

    Full Text Available OBJECTIVE: To evaluate the role of right heart catheterization in the diagnosis of pulmonary arterial hypertension (PAH. METHODS: We evaluated clinical, functional, and hemodynamic data from all patients who underwent right heart catheterization because of diagnostic suspicion of PAH-in the absence of severe left ventricular dysfunction (LVD, significant changes in pulmonary function tests, and ventilation/perfusion lung scintigraphy findings consistent with chronic pulmonary thromboembolism-between 2008 and 2013 at our facility. RESULTS: During the study period, 384 patients underwent diagnostic cardiac catheterization at our facility. Pulmonary hypertension (PH was confirmed in 302 patients (78.6%. The mean age of those patients was 48.7 years. The patients without PH showed better hemodynamic profiles and lower levels of B-type natriuretic peptide. Nevertheless, 13.8% of the patients without PH were categorized as New York Heart Association functional class III or IV. Of the 218 patients who met the inclusion criteria, 40 (18.3% and 178 (81.7% were diagnosed with PH associated with LVD (PH-LVD and with PAH, respectively. The patients in the HP-LVD group were significantly older than were those in the PAH group (p < 0.0001. CONCLUSIONS: The proportional difference between the PAH and PH-LVD groups was quite significant, considering the absence of echocardiographic signs suggestive of severe LVD during the pre-catheterization investigation. Our results highlight the fundamental role of cardiac catheterization in the diagnosis of PAH, especially in older patients, in whom the prevalence of LVD that has gone undiagnosed by non-invasive tests is particularly relevant.

  11. Appendicular arterial tumor embolization in two cats with pulmonary carcinoma.

    Science.gov (United States)

    Ibarrola, Patricia; German, Alexander J; Stell, Anneliese J; Fox, Richard; Summerfield, Nuala J; Blackwood, Laura

    2004-10-01

    A 13-year-old neutered male Persian cat and an 11-year-old neutered female Persian cat were examined because of an acute onset of lameness. In both cats, conscious proprioception and reflexes were diminished in the affected limb. In 1 cat, no blood flow was detected in the left brachial artery with a Doppler ultrasonic flow detector, whereas blood flow in the right brachial artery was easily documented. In the other cat, the right femoral pulse was not palpable. Neither cat had any echocardiographic evidence of cardiac disease. In both cats, treatment was primarily supportive. One cat died, and the other was euthanatized. At necropsy, lung lobe consolidation was seen. Microscopically, there was multifocal infiltration of the lung parenchyma with cuboidal to columnar neoplastic epithelial cells. Neoplastic epithelial cells of similar morphology were identified in nodular masses in sections of muscle, and intravascular tumor emboli were identified obliterating small and large arterioles. Immunohistochemical staining of pulmonary and muscular tissue for pan-cytokeratin antigen revealed intense cytoplasmic staining of neoplastic cells. Staining for factor VIII-related antigen confirmed that clusters of neoplastic cells represented intravascular emboli. Clinical signs in the cats were attributed to arterial occlusion by tumor emboli.

  12. Pulmonary vascular response during phases of canine heartworm disease: scanning electron microscopic study.

    Science.gov (United States)

    Schaub, R G; Rawlings, C A

    1980-07-01

    Pulmonary arteries and veins of 14 dogs in phases of heartworm disease (Dirofilaria immitis infection) were examined by scanning electron microscopy. Two dogs were infected with D immitis microfilaria only, whereas 12 dogs were infected with adult D immitis. Seven of the dogs infected with adult worms were untreated. Two of these 7 dogs had natural infections of unknown duration introduced by mosquito bite, whereas 5 were experimentally infected for 30 days. The remaining five dogs were experimentally infected for 1 year and had worms removed by drug therapy. These five dogs were maintained 12 months after treatment. Arteries and veins from dogs infected with microfilaria had a continuous sheet of endothelial cells. Arterial endothelium from the seven nontreated dogs infected with adult heartworms exhibited swirling patterns, pore formation, and separation of intercellular junctions. Arteries from all dogs had numerous endothelialized villus protrusions; veins had similar, less extensive changes. Arteries and veins from experimentally infected dogs were similar to naturally infected dogs, indicating the infection procedure produced lesions similar to that normally seen in heartworm disease. The extent of vascular lesions was reduced in four of the five treated dogs that had been infected with adult worms. Adult worms, not microfilaria, may produce the vascular lesions seen in heartworm disease. Lesions will regress if worms are removed from the circulation. Lesions may be caused by generation of humoral factors initiated by the presence of adult worms.

  13. Acute effect of hydralazine administration on pulmonary artery hemodynamics in dogs with chronic heartworm disease.

    Science.gov (United States)

    Atkins, C E; Keene, B W; McGuirk, S M; Sato, T

    1994-02-01

    In an effort to better understand the role of vasodilators in the management of pulmonary hypertension associated with chronic heartworm disease (HWD), pulmonary hemodynamic measurements were obtained from 7 experimentally infected, anesthetized dogs before and after hydralazine administration (mean dose, 1.96 mg/kg of body weight). Five dogs were maintained on room air, while 2 were maintained on 100% oxygen during the hydralazine study. The hemodynamic effect of hydralazine in dogs with HWD was evaluated, using heart rate, cardiac index, mean pulmonary artery pressure, mean arterial pressure, total pulmonary resistance, total systemic resistance, total systemic resistance/total pulmonary resistance, left ventricular dP/dtmax, left ventricular end diastolic pressure, and left and right ventricular double products ([mean arterial pressure x heart rate] and [mean pulmonary artery pressure x heart rate], respectively). Responders were defined as those in which total pulmonary resistance decreased > or = 20% without an increase in mean pulmonary arterial pressure and in which heart rate increase was < or = 10%. Comparison was also made between maximal hemodynamic effect of hydralazine with that after 100% oxygen administration for 15 minutes to previously normoxemic dogs (n = 5). Significance was determined if P < 0.05, using the paired t-test. Hydralazine induced significant reductions in mean pulmonary and systemic arterial pressures and total pulmonary resistance, with no significant change in heart rate, cardiac index, total systemic resistance, left ventricular dP/dtmax, left ventricular end diastolic pressure, or right and left ventricular double products. Four (57%) of the 7 dogs studied were considered responders. Pretreatment cardiac index, mean pulmonary artery pressure, and total pulmonary resistance did not allow differentiation of responders from nonresponders.(ABSTRACT TRUNCATED AT 250 WORDS)

  14. 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

  15. Safety, efficacy, and clinical utility of macitentan in the treatment of pulmonary arterial hypertension

    OpenAIRE

    Monaco TJ; Davila CD

    2016-01-01

    Thomas J Monaco,1 Carlos D Davila2 1Division of Pulmonary and Critical Care Medicine, Baylor College of Medicine, Houston, TX, 2Department of Medicine, Einstein Medical Center, Philadelphia, PA, USA Abstract: Pulmonary arterial hypertension is a progressive, debilitating disease caused by a dysregulation of the pulmonary vascular tone that inevitably leads to right heart failure and death without treatment. Until relatively recently, the treatment options for those afflicted by pulmonary ar...

  16. Right Aortic Arch with a Retroesophageal Left Subclavian Artery and an Anomalous Origin of the Pulmonary Artery from the Aorta

    Science.gov (United States)

    Jeon, Chang-Seok; Shim, Man-shik; Yang, Ji-Hyuk; Jun, Tae-Gook

    2017-01-01

    We report the case of a newborn with a rare anatomic variation: a right aortic arch with a retroesophageal left subclavian artery and an anomalous origin of the pulmonary artery from the aorta. This variation was diagnosed using echocardiography and computed tomography, and we treated the condition surgically. PMID:28180103

  17. 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.

  18. 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

  19. Proximal pulmonary arterial obstruction decreases the time constant of the pulmonary circulation and increases right ventricular afterload.

    Science.gov (United States)

    Pagnamenta, Alberto; Vanderpool, Rebecca; Brimioulle, Serge; Naeije, Robert

    2013-06-01

    The time constant of the pulmonary circulation, or product of pulmonary vascular resistance (PVR) and compliance (Ca), called the RC-time, has been reported to remain constant over a wide range of pressures, etiologies of pulmonary hypertension, and treatments. We wondered if increased wave reflection on proximal pulmonary vascular obstruction, like in operable chronic thromboembolic pulmonary hypertension, might also decrease the RC-time and thereby increase pulse pressure and right ventricular afterload. Pulmonary hypertension of variable severity was induced either by proximal obstruction (pulmonary arterial ensnarement) or distal obstruction (microembolism) eight anesthetized dogs. Pulmonary arterial pressures (Ppa) were measured with high-fidelity micromanometer-tipped catheters, and pulmonary flow with transonic technology. Pulmonary ensnarement increased mean Ppa, PVR, and characteristic impedance, decreased Ca and the RC-time (from 0.46 ± 0.07 to 0.30 ± 0.03 s), and increased the oscillatory component of hydraulic load (Wosc/Wtot) from 25 ± 2 to 29 ± 2%. Pulmonary microembolism increased mean Ppa and PVR, with no significant change in Ca and characteristic impedance, increased RC-time from 0.53 ± 0.09 to 0.74 ± 0.05 s, and decreased Wosc/Wtot from 26 ± 2 to 13 ± 2%. Pulse pressure increased more after pulmonary ensnarement than after microembolism. Concomitant measurements with fluid-filled catheters showed the same functional differences between the two types of pulmonary hypertension, with, however, an underestimation of Wosc. We conclude that pulmonary hypertension caused by proximal vs. distal obstruction is associated with a decreased RC-time and increased pulsatile component of right ventricular hydraulic load.

  20. Isolated right pulmonary artery agenesis with aplasia of right upper lobe and with anomalous arterial supply from celiac axis, anomalous venous drainage

    Directory of Open Access Journals (Sweden)

    Tushar Madhav Kalekar

    2015-01-01

    Full Text Available Pulmonary artery agenesis is the complete absence of the right or left pulmonary artery. This lesion is commonly associated with other cardiac anomalies and is usually diagnosed incidentally. Unilateral absence of pulmonary artery with lung hypoplasia is very rare. Ipsilateral hypoplastic or absent central pulmonary artery in these patients will have a systemic arterial supply to peripheral pulmonary arteries of the affected lung from the descending thoracic or upper abdominal aorta. These findings are usually characterized as pulmonary veno lobar syndrome or scimitar syndrome when associated with ipsilateral total or partial anomalous pulmonary venous return from the lower lobe which drains into the inferior vena cava or less commonly to the hepatic, azygous, or portal vein, or into the right atrium and lung agenesis/hypoplasia.

  1. New trial designs and potential therapies for pulmonary artery hypertension.

    Science.gov (United States)

    Gomberg-Maitland, Mardi; Bull, Todd M; Saggar, Rajeev; Barst, Robyn J; Elgazayerly, Amany; Fleming, Thomas R; Grimminger, Friedrich; Rainisio, Maurizio; Stewart, Duncan J; Stockbridge, Norman; Ventura, Carlo; Ghofrani, Ardeschir H; Rubin, Lewis J

    2013-12-24

    A greater understanding of the epidemiology, pathogenesis, and pathophysiology of pulmonary artery hypertension (PAH) has led to significant advances, but the disease remains fatal. Treatment options are neither universally available nor always effective, underscoring the need for development of novel therapies and therapeutic strategies. Clinical trials to date have provided evidence of efficacy, but were limited in evaluating the scope and duration of treatment effects. Numerous potential targets in varied stages of drug development exist, in addition to novel uses of familiar therapies. The pursuit of gene and cell-based therapy continues, and device use to help acute deterioration and chronic management is emerging. This rapid surge of drug development has led to multicenter pivotal clinical trials and has resulted in novel ethical and global clinical trial concerns. This paper will provide an overview of the opportunities and challenges that await the development of novel treatments for PAH.

  2. Optical studies of oxidative stress in pulmonary artery endothelial cells

    Science.gov (United States)

    Ghanian, Zahra; Sepehr, Reyhaneh; Eis, Annie; Kondouri, Ganesh; Ranji, Mahsa

    2015-03-01

    Reactive oxygen species (ROS) play an essential role in facilitating signal transduction processes within the cell and modulating the injuries. However, the generation of ROS is tightly controlled both spatially and temporally within the cell, making the study of ROS dynamics particularly difficult. This study present a novel protocol to quantify the dynamic of the mitochondrial superoxide as a precursor of reactive oxygen species. To regulate the mitochondrial superoxide level, metabolic perturbation was induced by administration of potassium cyanide (KCN). The presented method was able to monitor and measure the superoxide production rate over time. Our results demonstrated that the metabolic inhibitor, potassium cyanide (KCN) induced a significant increase in the rate of superoxide production in mitochondria of fetal pulmonary artery endothelial cells (FPAEC). Presented method sets the stage to study different ROS mediated injuries in vitro.

  3. Translating Research into Improved Patient Care in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Bonnet, Sebastien; Provencher, Steeve; Guignabert, Christophe; Perros, Frédéric; Boucherat, Olivier; Schermuly, Ralph Theo; Hassoun, Paul M; Rabinovitch, Marlene; Nicolls, Mark R; Humbert, Marc

    2016-09-20

    Despite important advances in its therapeutic management, pulmonary arterial hypertension (PAH) remains an incurable disease. Although numerous drugs exhibited beneficial effects in preclinical settings, only few have reached clinical trial phases, highlighting the challenges of translating preclinical investigations into clinical trials. Potential reasons for delayed PAH drug development may include the inherent limitations of the currently available animal and in vitro models, potential lack of appropriate standardization of the experimental design, regulatory agencies requirements, competing clinical trials and insufficient funding. Although this is not unique to PAH, there is urgency for reducing the number of false positive signals in preclinical studies and optimizing the development of innovative therapeutic targets through performance of clinical trials based on more robust experimental data. The current review discusses the challenges and opportunities in preclinical research to foster drug development in PAH.

  4. Oral treprostinil for the treatment of pulmonary arterial hypertension.

    Science.gov (United States)

    de Lartigue, J

    2014-08-01

    Pulmonary arterial hypertension (PAH) is a rare yet progressive and life-threatening condition that, despite the availability of FDA-approved therapies, remains incurable. Prostacyclin analogues are a mainstay of therapy for patients with PAH, but in spite of demonstrated improvements in survival, exercise capacity and hemodynamics, these agents have been limited by poor pharmacokinetics and complex administration requirements. Treprostinil diolamine (Orenitram™; United Therapeutics) is a novel oral formulation that joins the approved parenteral and inhaled formulations (Remodulin® and Tyvaso®; United Therapeutics). It displays similar pharmacokinetic properties, while offering the potential for improved patient compliance through the convenience of oral dosing. Following the demonstration of improved exercise capacity as monotherapy in patients with de novo PAH (FREEDOM-M), treprostinil diolamine was recently approved by the FDA for the treatment of patients with WHO group 1 PAH and continues to be evaluated in a number of clinical trials in this patient population.

  5. Optimising the management of pulmonary arterial hypertension patients: emergency treatments

    Directory of Open Access Journals (Sweden)

    R. Naeije

    2010-09-01

    Full Text Available Pulmonary arterial hypertension (PAH is a rare and potentially fatal disease whose management is usually restricted to a few specialised centres. As patients do not necessarily live in the neighbourhood of these centres, daily care and emergencies have to be delegated to first and second lines. Treatment guidelines do not usually provide recommendations for acute emergency situations as evidence is scarce. This short review provides a description of our therapeutic protocols based on available data. A model of transmural organisation of care for PAH patients, currently applied in Belgium, is described. Thereafter, based on an analysis of the reasons of death in the PAH population, a review of the main emergencies is provided. Cardiac arrest and resuscitation, decompensated right heart failure, respiratory failure, arrhythmia, pericardial effusion, haemoptysis, surgery and drug-related adverse events will be discussed successively. Case reports showing the precariousness of PAH patients will enforce our thesis of the need for optimal patient management organisation.

  6. RELAXANT EFFECTS OF VASOACTIVE INTESTINAL PEPTIDE ON PULMONARY ARTERY IN CHRONICALLY HYPOXIC RATS

    Institute of Scientific and Technical Information of China (English)

    陈玉玲; 罗慰慈; 蔡英年

    1996-01-01

    The object of this study is to investigate the effect of VIP on pulmonary artery of chronically hypoxic rats. It was shown that chronic hypoxla depressed significantly pulmnonary artery relaxation induced by VIP as compared with those of control (Ppulmonary arteries in rats was endotbelium-independent, and was not prevented by indomethaein or nordihydroguaiaretie acid, but was abolished completely by methylene blue. These results suggest that the lower relaxation of pulmonary artery in rats might not be due to the endothelial injury caused by chronic hypoxxia, and chronic hypoxiamay inhibit directly the soluble guanylate cyclase in vascular smooth muscle cells invioved in synthesis of cGMP and thus reduced the sensitivity and reactivity of pulmonary artery to VIP.

  7. Pulmonary artery aneurysm with patent arterial duct: resection of aneurysm and ductal division.

    Science.gov (United States)

    Tefera, Endale; Teodori, Michael

    2013-10-01

    Congenital or acquired aneurysm of the pulmonary artery (PA) is rare. Although aneurysms are described following surgical treatment of patent ductus arteriosus (PDA), occurrence of this lesion in association with PDA without previous surgery is extremely uncommon. An eight-year-old patient with PDA and aneurysm of the main PA is described in this report. Clinical diagnosis of PDA was made upon presentation. Diagnosis of PA aneurysm was suspected on chest x-ray and was confirmed on transthoracic echocardiography. Successful surgical resection of the aneurysm and division of the duct were performed under cardiopulmonary bypass. The patient did well on follow-up both from clinical and echocardiographic point of view.

  8. 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.

  9. Heterogeneous mechanics of the mouse pulmonary arterial network.

    Science.gov (United States)

    Lee, Pilhwa; Carlson, Brian E; Chesler, Naomi; Olufsen, Mette S; Qureshi, M Umar; Smith, Nicolas P; Sochi, Taha; Beard, Daniel A

    2016-10-01

    Individualized modeling and simulation of blood flow mechanics find applications in both animal research and patient care. Individual animal or patient models for blood vessel mechanics are based on combining measured vascular geometry with a fluid structure model coupling formulations describing dynamics of the fluid and mechanics of the wall. For example, one-dimensional fluid flow modeling requires a constitutive law relating vessel cross-sectional deformation to pressure in the lumen. To investigate means of identifying appropriate constitutive relationships, an automated segmentation algorithm was applied to micro-computerized tomography images from a mouse lung obtained at four different static pressures to identify the static pressure-radius relationship for four generations of vessels in the pulmonary arterial network. A shape-fitting function was parameterized for each vessel in the network to characterize the nonlinear and heterogeneous nature of vessel distensibility in the pulmonary arteries. These data on morphometric and mechanical properties were used to simulate pressure and flow velocity propagation in the network using one-dimensional representations of fluid and vessel wall mechanics. Moreover, wave intensity analysis was used to study effects of wall mechanics on generation and propagation of pressure wave reflections. Simulations were conducted to investigate the role of linear versus nonlinear formulations of wall elasticity and homogeneous versus heterogeneous treatments of vessel wall properties. Accounting for heterogeneity, by parameterizing the pressure/distention equation of state individually for each vessel segment, was found to have little effect on the predicted pressure profiles and wave propagation compared to a homogeneous parameterization based on average behavior. However, substantially different results were obtained using a linear elastic thin-shell model than were obtained using a nonlinear model that has a more

  10. Epoprostenol sodium for treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Saito Y

    2015-05-01

    Full Text Available Yukihiro Saito,1 Kazufumi Nakamura,1 Satoshi Akagi,1 Toshihiro Sarashina,1 Kentaro Ejiri,1 Aya Miura,1 Aiko Ogawa,2 Hiromi Matsubara,2 Hiroshi Ito1 1Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan; 2Division of Cardiology, National Hospital Organization Okayama Medical Center, Okayama, Japan Abstract: The release of endogenous prostacyclin (PGI2 is depressed in patients with pulmonary arterial hypertension (PAH. PGI2 replacement therapy by epoprostenol infusion is one of the best treatments available for PAH. Here, we provide an overview of the current clinical data for epoprostenol. Epoprostenol treatment improves symptoms, exercise capacity, and hemodynamics, and is the only treatment that has been shown to reduce mortality in patients with idiopathic PAH (IPAH in randomized clinical trials. We have reported that high-dose epoprostenol therapy (>40 ng/kg/min also results in marked hemodynamic improvement in some patients with IPAH. High-dose epoprostenol has a pro-apoptotic effect on PAH-PASMCs via the IP receptor and upregulation of Fas ligand (FasL in vitro. However, long-term intravenous administration of epoprostenol is sometimes associated with catheter-related infections and leads to considerable inconvenience for the patient. In the future, the development of new routes of administration or the development of powerful PGI2 analogs, IP-receptor agonists, and gene and cell-based therapy enhancing PGI2 production with new routes of administration is required. Keywords: pulmonary arterial hypertension, prostacyclin, apoptosis

  11. The role of polymorphonudear cells in lung ischemia-reperfusion injury in a canine model of pulmonary thromboembolism

    Institute of Scientific and Technical Information of China (English)

    邓朝胜

    2006-01-01

    Objective To explore the effects of polymoronuclear cells (PMN) on lung ischemia-reperfusion(I/R) injury in a canine model of pulmonary thromboembolism. Methods Fifteen dogs were divided into three groups; a sham group (n=5), an ischemia group (n=5) and a reperfusion group (n=5). PMN in the whole blood were isolated with density gradient centrifugation. Apoptosis rate of the PMN was measured through flow cytome-

  12. 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.

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

    Institute of Scientific and Technical Information of China (English)

    Mehmet; Yaman; Ugur; Arslan; Ahmet; Hakan; Ates; Aytekin; Aksakal

    2015-01-01

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    Coche, Emmanuel; Pawlak, Sebastien; Dechambre, Stephane; Maldague, Baudouin [Department of Radiology, St. Luc Hospital, UCL, Avenue Hippocrate, 10, 1200 Brussels (Belgium)

    2003-04-01

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

    Science.gov (United States)

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

    2003-04-01

    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.

  16. Diagnosis and Rescue of a Kinked Pulmonary Artery Catheter

    Directory of Open Access Journals (Sweden)

    Nicolas J. Mouawad

    2015-01-01

    Full Text Available Invasive hemodynamic monitoring with a pulmonary catheter has been relatively routine in cardiovascular and complex surgical operations as well as in the management of critical illnesses. However, due to multiple potential complications and its invasive nature, its use has decreased over the years and less invasive methods such as transesophageal echocardiography and hemodynamic sensors have gained widespread favor. Unlike these less invasive forms of hemodynamic monitoring, pulmonary artery catheters require an advanced understanding of cardiopulmonary physiology, anatomy, and the potential for complications in order to properly place, manage, and interpret the device. We describe a case wherein significant resistance was encountered during multiple unsuccessful attempts at removing a patient’s catheter secondary to kinking and twisting of the catheter tip. These attempts to remove the catheter serve to demonstrate potential rescue options for such a situation. Ultimately, successful removal of the catheter was accomplished by simultaneous catheter retraction and sheath advancement while gently pulling both objects from the cannulation site. In addition to being skilled in catheter placement, it is imperative that providers comprehend the risks and complications of this invasive monitoring tool.

  17. Robotic-Assisted Endovascular Pulmonary Artery Foreign Body Retrieval: A Case Report.

    Science.gov (United States)

    Wolujewicz, Michael

    2016-04-01

    As intravascular robotics technology continues to evolve, so do the potential applications. The present case describes the use of the Magellan Robotic System within the pulmonary artery. A 61-year-old female was referred for retrieval of a transected port catheter, which had embolized into the pulmonary artery. After a failed attempt using a conventional technique, retrieval was ultimately successful with assistance of the Magellan system. The unique navigational capabilities and stability of the system may make it a valuable tool in pulmonary artery interventions.

  18. Right pulmonary artery agenesis presenting with uncontrolled asthma in an adult: a case report

    Directory of Open Access Journals (Sweden)

    Thammasitboon Supat

    2011-08-01

    Full Text Available Abstract Introduction Unilateral absence of the pulmonary artery (UAPA or pulmonary artery agenesis is a rare congenital disorder presenting with a wide spectrum of symptoms. The clinical presentation is variable and many patients can be asymptomatic for many years and even throughout their lives. Case presentation We report the case of a 53-year-old African-American woman who was diagnosed with right pulmonary artery agenesis after presenting with uncontrolled asthma and recurrent bronchopulmonary infections. Conclusion In an unexplained case of recurrent respiratory infections and shortness of breath, the possibility of a rare congenital anomaly like UAPA should be considered and an appropriate evaluation should be done.

  19. Main pulmonary artery aneurysm: a case report and review of the literature.

    Science.gov (United States)

    Imazio, Massimo; Cecchi, Enrico; Giammaria, Massimo; Pomari, Franco; Tabasso, Maria Donata; Ghisio, Aldo; Forsennati, Pier Giuseppe; Beqaraj, Federico; Favro, Elisa; Trinchero, Rita; Brusca, Antonio

    2004-03-01

    Main pulmonary artery aneurysms are a rare entity with few available published data. As reported in the literature, operative treatment is commonly recommended but the relation between the size of the aneurysm, its localization, and the risk of rupture is not as well defined as for aortic aneurysms. Proximal lesions that involve the main branches of the pulmonary artery are usually apparent on chest radiographs and must be taken into consideration in the differential diagnosis of mediastinal masses. An early diagnosis allows timely surgical treatment. We report an unusual case of a main pulmonary artery aneurysm presenting with persistent non-productive cough and provide a review of the pertinent published data.

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

    Science.gov (United States)

    Hardin, Elizabeth Ashley; Chin, Kelly M

    2016-01-01

    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.

  1. Dramatic response of a patient with pregnancy induced idiopathic pulmonary arterial hypertension to sildenafil treatment.

    Science.gov (United States)

    Taçoy, Gülten; Ekim, Numan Nadir; Cengel, Atiye

    2010-04-01

    Idiopathic pulmonary arterial hypertension (IPAH) is characterized by a progressive increase in pulmonary vascular resistance, which may lead to right ventricular failure and death. Major cardiovascular and pulmonary alterations occur during pregnancy and therefore worsen or increase the complications of pulmonary arterial hypertension (PAH). A patient diagnosed with IPAH after a successful full-term pregnancy and cesarean section with epidural anesthesia is presented. The postoperative course was complicated by progressive dyspnea, and lower limb edema. The outcome of treatment with sildenafil during puerperium was favorable in this patient. The clinical course was complicated by an unexpected spontaneous pregnancy after primary infertility.

  2. Pulmonary embolism caused by a carbon dioxide blower during off-pump coronary artery bypass grafting.

    Science.gov (United States)

    Hirata, Naoyuki; Kanaya, Noriaki; Yamazaki, Yutaka; Sonoda, Hajime; Namiki, Akiyoshi

    2010-02-01

    We report a rare case of pulmonary embolism (PE) caused by a carbon dioxide (CO2) blower during off-pump coronary artery bypass grafting (OPCAB). When the anastomosis of the right internal thoracic artery to left anterior descending artery was performed, the operator tore the right ventricle outflow track (RVOT) that was adjacent to the left anterior descending artery. Immediately after the anastomosis and repair of the torn RVOT with CO2 blower, the systolic pulmonary artery pressure (PAP) increased from 28 to 64 mmHg, and end-tidal CO2 decreased from 32 to 12 mmHg. Because transesophageal echocardiograph (TEE) showed numerous gas bubbles in the main pulmonary artery, we diagnosed PE caused by invasion of CO2 gas bubbles via the torn RVOT. Although a CO2 blower is useful to enhance visualization of the anastomosis during OPCAB, it should not be used for the venous system because it may cause CO2 embolism.

  3. [Acute Postoperative Negative Pressure Pulmonary Edema Caused by the Compression of Brachiocephalic Artery].

    Science.gov (United States)

    Tagawa, Miki; Iwai, Hidetaka; Fukatsu, Ken; Shimada, Mami; Hirabayashi, Yoshihiro

    2016-06-01

    We report a case of negative-pressure pulmonary edema occurring by tracheal obstruction caused by the brachiocephalic artery. The patient had deformed thorax with cerebral palsy, which deformed thorax placing the brachiocephalic artery high over the trachea, resulting in close and tight contact between the artery and trachea. Additional deformity of the thorax associated with myotonic attacks after general anesthesia might shorten the distance between the sternal notch and the vertebral body, resulting in the tracheal obstruction by the artery.

  4. Response to pulmonary arterial hypertension drug therapies in patients with pulmonary arterial hypertension and cardiovascular risk factors.

    Science.gov (United States)

    Charalampopoulos, Athanasios; Howard, Luke S; Tzoulaki, Ioanna; Gin-Sing, Wendy; Grapsa, Julia; Wilkins, Martin R; Davies, Rachel J; Nihoyannopoulos, Petros; Connolly, Susan B; Gibbs, J Simon R

    2014-12-01

    The age at diagnosis of pulmonary arterial hypertension (PAH) and the prevalence of cardiovascular (CV) risk factors are increasing. We sought to determine whether the response to drug therapy was influenced by CV risk factors in PAH patients. We studied consecutive incident PAH patients (n = 146) between January 1, 2008, and July 15, 2011. Patients were divided into two groups: the PAH-No CV group included patients with no CV risk factors (obesity, systemic hypertension, type 2 diabetes mellitus, permanent atrial fibrillation, mitral and/or aortic valve disease, and coronary artery disease), and the PAH-CV group included patients with at least one. The response to PAH treatment was analyzed in all the patients who received PAH drug therapy. The PAH-No CV group included 43 patients, and the PAH-CV group included 69 patients. Patients in the PAH-No CV group were younger than those in the PAH-CV group (P < 0.0001). In the PAH-No CV group, 16 patients (37%) improved on treatment and 27 (63%) did not improve, compared with 11 (16%) and 58 (84%) in the PAH-CV group, respectively (P = 0.027 after adjustment for age). There was no difference in survival at 30 months (P = 0.218). In conclusion, in addition to older age, CV risk factors may predict a reduced response to PAH drug therapy in patients with PAH.

  5. The dynamic study of the pulmonary artery obstruction degree and the right ventricular function in massive pulmonary embolism on CT pulmonary angiography

    Institute of Scientific and Technical Information of China (English)

    Jianguo Wang; Yulin Guo; Xiaojuan Guo; Min Liu; Youmin Guo; Chen Wang; Yuanhua Yang; Zhenguo Zhai; Li Zhu; Hongxia Ma

    2008-01-01

    Objective: To analyze the value of CTPA in assessing the dissolve of embolus and the function of the fight ventricle dynamically.Methods:Twenty-three cases of massive pulmonary embolism were analyzed retrospectively. The pulmonary artery obstruction index and the fight ventricular function parameters were collected and analyzed on CTPA before thrombolytic therapy, 24 hours and 14 days after therapy, respectively. Results:The pulmonary artery obstruction index decreased gradually, and there was significant difference before therapy, 24 hours and 2 weeks after therapy. Twenty-four hours after therapy, the maximal short axes diameter and the maximal transverse area of fight ventricle(RVd, RVs) decreased significantly, the maximal short axes diameter and the maximal transverse area of left ventricle(LVd, LVs) increased significantly, and the RVd/LVd, RVs/LVs decreased apparently. The pulmonary artery symbolic pressure before and 24 hours after therapy were apparently different. There was no significant difference between azygos vein, the super vena cava, the main pulmonary artery and vein reflux before and after therapy. Conclusion:CTPA can evaluate the pulmonary artery obstruction degree and right ventricular function dynamically.

  6. [Surgical treatment for tetralogy of Fallot with unilateral absence of a pulmonary artery (author's transl)].

    Science.gov (United States)

    Beyer, J; Brunner, L; Klinner, W

    1976-10-01

    Among 843 patients with Tetralogy of Fallot, 10 showed unilateral absence of a pulmonary artery. In 7 cases the cause was congenital aplasia, whereas thrombosis of one of the pulmonary arteries following systemic-pulmonary anastomosis was the cause in the remaining 3 patients. According to the reports of other authors and to our own experiences, this rare malformation is treated best with palliative operations, the technique depending on the patient's age and on the individual anatomical condition. Only in very few cases with an acceptable pathologic anatomy there will be a reasonable chance for successful repair with reconstruction of the continuity from the right ventricle to the affected lung. However, if complete repair is performed, reconstruction of the absent pulmonary artery usually will be necessary for avoidance of pulmonary hypertension of the contralateral lung.

  7. A neonate with left pulmonary artery thrombosis and left lung hypoplasia: a case report

    Directory of Open Access Journals (Sweden)

    ElHassan Nahed O

    2010-08-01

    Full Text Available Abstract Introduction Spontaneous intrauterine arterial thrombosis and congenital pulmonary hypoplasia are rare conditions and have not been reported to occur together. The literature rather includes two reports of babies with neonatal pulmonary artery occlusion and post-infarction cysts of the lungs. Case presentation We report a case of a live Caucasian male newborn with left lung hypoplasia that occurred in association with left pulmonary artery thrombosis. Despite a critical neonatal course, including extracorporeal membrane oxygenation, this infant is alive and well at 18 months of age without any neurodevelopmental sequelae or reactive airway disease. Conclusion This association suggests the possibility of an intrauterine vascular event between the fifth and eighth weeks of gestation during early pulmonary artery and lung development.

  8. Emergent Unilateral Renal Artery Stenting for Treatment of Flash Pulmonary Edema: Fact or Fiction?

    Directory of Open Access Journals (Sweden)

    Asaad Akbar Khan

    2015-01-01

    Full Text Available Flash pulmonary edema is characteristically sudden in onset with rapid resolution once appropriate therapy has been instituted (Messerli et al., 2011. Acute increase of left ventricular (LV end diastolic pressure is the usual cause of sudden decompensated cardiac failure in this patient population. Presence of bilateral renal artery stenosis or unilateral stenosis in combination with a single functional kidney in the susceptible cohort is usually blamed for this condition. We describe a patient who presented with flash pulmonary edema in the setting of normal coronary arteries. Our case is distinct as our patient developed flash pulmonary edema secondary to unilateral renal artery stenosis in the presence of bilateral functioning kidneys. Percutaneous stent implantation in the affected renal artery resulted in rapid resolution of pulmonary edema.

  9. Emergent Unilateral Renal Artery Stenting for Treatment of Flash Pulmonary Edema: Fact or Fiction?

    Science.gov (United States)

    Khan, Asaad Akbar; McFadden, Eugene Patrick

    2015-01-01

    Flash pulmonary edema is characteristically sudden in onset with rapid resolution once appropriate therapy has been instituted (Messerli et al., 2011). Acute increase of left ventricular (LV) end diastolic pressure is the usual cause of sudden decompensated cardiac failure in this patient population. Presence of bilateral renal artery stenosis or unilateral stenosis in combination with a single functional kidney in the susceptible cohort is usually blamed for this condition. We describe a patient who presented with flash pulmonary edema in the setting of normal coronary arteries. Our case is distinct as our patient developed flash pulmonary edema secondary to unilateral renal artery stenosis in the presence of bilateral functioning kidneys. Percutaneous stent implantation in the affected renal artery resulted in rapid resolution of pulmonary edema. PMID:25793128

  10. Pulmonary artery catheter insertion in a patient of dextrocardia with anomalous venous connections

    Directory of Open Access Journals (Sweden)

    Tripathi Mukesh

    2004-08-01

    Full Text Available In a young adult patient having situs solitus with dextrocardia the attempted pulmonary artery catheter placement for emergency mitral valve replacement required an unduly long length (50cm of catheter insertion to get into right ventricle and then into pulmonary artery. Although catheter coiling was suspected initially, chest x-ray taken after successfully placement revealed an uncommon congenital anomalous venous connection i.e. right internal jugular opening into left sided superior vena cava then into inferior vena cava after running all along the left border of the heart. With the result, it required to pass 50cm of PA catheter to get into right ventricle in our patient. This emphasizes the need to look for abnormal venous connections during echocardiography and x-ray screening in congenital heart disease. Fluoroscopy is recommended when an unusual length of pulmonary artery catheter insertion is required to enter the pulmonary artery.

  11. Intra-arterial pulmonary thrombolysis at the postoperative period of brain aneurysm clamping: case report.

    Science.gov (United States)

    Rojas, Salomón Soriano Ordinola; Veiga, Viviane Cordeiro; Carvalho, Júlio César de; Campodônico, Luis Enrique Amaya; Assis, Fabrizio Rodrigues; Shimizu, Sandra Patrícia; Morais, Elaine Aparecida; Buesio, Roberto; Marchesini, Andréia Maria; Junqueira, Ligia Maria Coscrato; Holanda, Carlos Vanderlei

    2008-09-01

    Pulmonary thromboembolism is a major cause of morbidity and mortality of patients undergoing neurosurgical procedures. The purpose of this study was to present a case of intra-arterial pulmonary thrombolysis in recent neurosurgery postoperative period. Male patient, undergoing neurosurgery, presented as a complication on the seventh day of postoperative massive pulmonary embolism with hemodynamic instability and intraarterial pulmonary thrombolysis with alteplase was indicated. Evolution was satisfactory without bleeding complications and patient was discharged. Pulmonary thromboembolism is a high morbidity and mortality condition at neurosurgical postoperative period and thrombolysis should be an alternative therapy in cases refractory to clinical treatment.

  12. Biophysical and molecular comparison of sodium current in cells isolated from canine atria and pulmonary vein.

    Science.gov (United States)

    Barajas-Martinez, Hector; Goodrow, Robert J; Hu, Dan; Patel, Payal; Desai, Mayurika; Panama, Brian K; Treat, Jacqueline A; Aistrup, Gary L; Cordeiro, Jonathan M

    2017-02-27

    The collar of the pulmonary vein (PV) is the focal point for the initiation of atrial arrhythmias, but the mechanisms underlying how PV cells differ from neighboring left atrial tissue are unclear. We examined the biophysical and molecular properties of INa in cells isolated from the canine pulmonary sleeve and compared the properties to left atrial tissue. PV and left atrial myocytes were isolated and patch clamp techniques were used to record INa. Action potential recordings from either tissue type were made using high-resistance electrodes. mRNA was determined using quantitative RT-PCR and proteins were determined by Western blot. Analysis of the action potential characteristics showed that PV tissue had a lower Vmax compared with left atrial tissue. Fast INa showed that current density was slightly lower in PV cells compared with LA cells (-96 ± 18.7 pA/pF vs. -120 ± 6.7 pA/pF, respectively, p < 0.05). The recovery from inactivation of INa in PV cells was slightly slower but no marked difference in steady-state inactivation was noted. Analysis of late INa during a 225-ms pulse showed that late INa was significantly smaller in PV cells compared to LA cells at all measured time points into the pulse. These results suggest PV cells have lower density of both peak and late INa. Molecular analysis of Nav1.5 and the four beta subunits showed lower levels of Nav1.5 as well as Navβ1 subunits, confirming the biophysical findings. These data show that a lower density of INa may lead to depression of excitability and predispose the PV collar to re-entrant circuits under pathophysiological conditions.

  13. 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.

  14. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

    OpenAIRE

    Tselios K; Gladman DD; Urowitz MB

    2016-01-01

    Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz, University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada Abstract: Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (&...

  15. 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.

  16. Isolated Anomalous Origin of Left Pulmonary Artery From the Descending Aorta: An Embryologic Ambiguity.

    Science.gov (United States)

    Gnanappa, Ganesh Kumar; Laohachai, Karina; Orr, Yishay; Ayer, Julian

    2016-11-01

    Anomalous origin of a branch pulmonary artery from the aorta is a rare malformation, accounting for 0.12% of all congenital heart defects. Anomalous origin of the left pulmonary artery from the aorta (ALPA) constitutes a small proportion of these cases. ALPA has been reported to arise from the ascending aorta with various embryologic postulates. We report a case of isolated ALPA arising from the descending aorta in association with a patent ductus arteriosus, to emphasize its embryologic ambiguity.

  17. 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....

  18. Indications, Results and Mortality of Pulmonary Artery Banding Procedure: a Brief Review and Five- year Experiences

    OpenAIRE

    Hamid Hoseinikhah; Aliasghar Moeinipour; Ahmadreza Zarifian; Mohammad Sobhan Sheikh Andalibi; Yasamin Moeinipour; Mohammad Abbassi Teshnisi; Abbas Bahreini

    2016-01-01

    Background Pulmonary artery banding (PAB) is a technique of palliative surgical therapy used by congenital heart surgeons as a staged approach to operative correction of congenital heart defects. Materials and Methods We report 5- year experiences from January 2011 to January 2016 of Imam Reza Hospital center (a tertiary referral hospital in Mashhad city, North East of Iran) that consist of 50 patients with congenital heart disease with left to right shunt that pulmonary artery banding proced...

  19. Pulmonary artery dissection in a patient with Eisenmenger syndrome treated with heart and lung transplantation

    DEFF Research Database (Denmark)

    Tønder, Niels; Køber, Lars; Hassager, Christian

    2004-01-01

    We report the case of a patient with known Eisenmenger syndrome due to congenital ventricular septal defect, who developed pulmonary artery dissection. The patient was successfully treated with heart and lung transplantation.......We report the case of a patient with known Eisenmenger syndrome due to congenital ventricular septal defect, who developed pulmonary artery dissection. The patient was successfully treated with heart and lung transplantation....

  20. Non-invasive assessment of pulmonary arterial pressure in healthy neonates.

    OpenAIRE

    Skinner, J. R.; Boys, R J; Hunter, S; Hey, E N

    1991-01-01

    Doppler echocardiograms were carried out on 51 healthy babies three times during the first 72 hours of life to estimate pulmonary arterial systolic pressure by measuring regurgitant tricuspid jet velocity and applying the Bernoulli equation. Tricuspid regurgitation was detected at some stage in all preterm babies and most of those born at full term. Pulmonary arterial pressure could be measured from peak regurgitant velocity in babies with pansystolic regurgitation. The incidence of pansystol...

  1. Percutaneous transhepatic venous embolization of pulmonary artery aneurysm in Hughes-Stovin syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Kyung Ah; Kim, Man Deuk; Oh, Do Yun; Park, Pil Won [Bundang CHA General Hospital, Pochon CHA University, Seongnam (Korea, Republic of)

    2007-08-15

    Hughes-Stovin syndrome is an extremely rare entity. We present a case of a 42-year-old man, who developed deep vein and inferior vena cava (IVC) thrombosis, repeated internal bleeding and pulmonary artery aneurysms (PAAs). The patient presented with massive hemoptysis and with PAAs of a 2.5 cm maximum diameter. We describe the successful percutaneous transhepatic venous embolization of the PAAs due to occluded common vascular pathways to the pulmonary artery.

  2. Identification of G-Protein-Coupled Receptors (GPCRs) in Pulmonary Artery Smooth Muscle Cells as Novel Therapeutic Targets

    Science.gov (United States)

    2015-10-01

    enhanced proliferation of pulmonary arterial smooth muscle cells (PASMCs). The underlying idea of this project is that the currently limited...Pulmonary arterial hypertension (PAH) is associated with increased vascular resistance, sustained contraction, and enhanced proliferation of pulmonary... CRISPR /Cas9) to knock down receptor expression in cells of interest and then to assess the impact of receptor knock down on functional activity

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  4. Early teatment with hepatocyte growth factor improves pulmonary artery and right ventricular remodeling in rats with pulmonary artery hypertension by modulating cytokines expression

    Institute of Scientific and Technical Information of China (English)

    王晓林

    2014-01-01

    Objective To investigate the effect of early treatment with hepatocyte growth factor(HGF)on the cytokine expression and pulmonary artery,right ventricular(RV)remodeling in the rat model of pulmonary artery hypertension(PAH).Methods The rat model of PAH was produced by injecting monocrotaline,and the model rats were randomly divided into empty adenovirus transfection group(MCT group,n=10)and HGF gene transfection group(HGF group,n=10).Another group of rats served as the Sham operation group(Sham group n=10).After 4 weeks of HGF gene transfection,the histological sections of the lungs and right ventricular(RV)

  5. Integrated care and optimal management of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Geoff Strange

    2009-05-01

    Full Text Available Geoff Strange1, Robin Fowler2, Corina Jary2, Brad Dalton3, Simon Stewart4, Eli Gabbay51Epidemiology and Preventative Medicine, Monash University, VIC, Australia; 2Royal Perth Hospital and Curtin University, Perth, WA, Australia; 3University of Tasmania, Launceston, TAS, Australia; 4Baker Heart Research Institute, Melbourne, VIC, Australia; 5Royal Perth Hospital and University of Western Australia, Perth, WA, AustraliaAbstract: Pulmonary arterial hypertension (PAH may occur as an idiopathic process or as a component of a variety of diseases, including connective tissue diseases, congenital heart disease, and exposure to appetite suppressants or infectious agents such as HIV. Untreated, it is a potentially devastating disease; however, diagnosis can be difficult due to the non-specific nature of symptoms during the early stages, and the fact that patients often present to a range of different medical specialties. The past decade has seen remarkable improvements in our understanding of the pathology associated with the condition and the development of PAH-specific therapies with the ability to alter the natural history of the disease. This article reviews the evidence for screening and diagnosis of susceptible patient groups and discusses treatment selection and recommendations based on data available from randomized controlled trials. In addition, due to the complexity of the diagnostic evaluation required and the treatment options available, this review mandates for a multidisciplinary approach to the management of PAH. We discuss the roles and organizational structure of a specialized PAH center in Perth, Western Australia to highlight these issues. Keywords: pulmonary hypertension, multidisciplinary care, systemic sclerosis, diagnostic protocol

  6. Upregulated Genes In Sporadic, Idiopathic Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Yacoub Magdi H

    2006-01-01

    Full Text Available Abstract Background To elucidate further the pathogenesis of sporadic, idiopathic pulmonary arterial hypertension (IPAH and identify potential therapeutic avenues, differential gene expression in IPAH was examined by suppression subtractive hybridisation (SSH. Methods Peripheral lung samples were obtained immediately after removal from patients undergoing lung transplant for IPAH without familial disease, and control tissues consisted of similarly sampled pieces of donor lungs not utilised during transplantation. Pools of lung mRNA from IPAH cases containing plexiform lesions and normal donor lungs were used to generate the tester and driver cDNA libraries, respectively. A subtracted IPAH cDNA library was made by SSH. Clones isolated from this subtracted library were examined for up regulated expression in IPAH using dot blot arrays of positive colony PCR products using both pooled cDNA libraries as probes. Clones verified as being upregulated were sequenced. For two genes the increase in expression was verified by northern blotting and data analysed using Student's unpaired two-tailed t-test. Results We present preliminary findings concerning candidate genes upregulated in IPAH. Twenty-seven upregulated genes were identified out of 192 clones examined. Upregulation in individual cases of IPAH was shown by northern blot for tissue inhibitor of metalloproteinase-3 and decorin (P Conclusion Four of the up regulated genes, magic roundabout, hevin, thrombomodulin and sucrose non-fermenting protein-related kinase-1 are expressed specifically by endothelial cells and one, muscleblind-1, by muscle cells, suggesting that they may be associated with plexiform lesions and hypertrophic arterial wall remodelling, respectively.

  7. Loss of alveolar membrane diffusing capacity and pulmonary capillary blood volume in pulmonary arterial hypertension

    Science.gov (United States)

    2013-01-01

    Background Reduced gas transfer in patients with pulmonary arterial hypertension (PAH) is traditionally attributed to remodeling and progressive loss of pulmonary arterial vasculature that results in decreased capillary blood volume available for gas exchange. Methods We tested this hypothesis by determination of lung diffusing capacity (DL) and its components, the alveolar capillary membrane diffusing capacity (Dm) and lung capillary blood volume (Vc) in 28 individuals with PAH in comparison to 41 healthy individuals, and in 19 PAH patients over time. Using single breath simultaneous measure of diffusion of carbon monoxide (DLCO) and nitric oxide (DLNO), DL and Dm were respectively determined, and Vc calculated. Dm and Vc were evaluated over time in relation to standard clinical indicators of disease severity, including brain natriuretic peptide (BNP), 6-minute walk distance (6MWD) and right ventricular systolic pressure (RVSP) by echocardiography. Results Both DLCO and DLNO were reduced in PAH as compared to controls and the lower DL in PAH was due to loss of both Dm and Vc (all p DLNO decreased by 24 ml/min/mmHg/year (p = 0.01). Consequently, Dm decreased and Vc tended to increase over time, which led to deterioration of the Dm/Vc ratio, a measure of alveolar-capillary membrane functional efficiency without changes in clinical markers. Conclusions The findings indicate that lower than normal gas transfer in PAH is due to loss of both Dm and Vc, but that deterioration of Dm/Vc over time is related to worsening membrane diffusion. PMID:23339456

  8. Successful pregnancy in pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report

    Directory of Open Access Journals (Sweden)

    Streit Michael

    2009-06-01

    Full Text Available Abstract Introduction Pulmonary arterial hypertension is a complication of systemic lupus erythematosus. Mortality in pregnant patients with pulmonary arterial hypertension related to connective tissue disease is as high as 56%. The authors report the first case of a successful maternal-fetal outcome in a pregnant patient with systemic lupus erythematosus-associated pulmonary arterial hypertension treated with sildenafil and inhaled iloprost during pregnancy and until several weeks after caesarean section. Case presentation The case presented is of a 29-year-old woman with systemic lupus erythematosus and associated severe pulmonary arterial hypertension. Vasodilator therapy with bosentan and sildenafil, immunosuppressive therapy with prednisone, hydroxychloroquine and azathioprine and oral anticoagulation (phenprocoumon had normalized her right ventricular over right atrial pressure when she was diagnosed in her 5th week of pregnancy. The teratogenic drugs bosentan and phenprocoumon were stopped, the latter replaced by low molecular weight heparin. During the 35th week, a slight increase in pulmonary pressure was found. Therapy with inhaled iloprost was established. A caesarean section was performed in the 37th week and a healthy baby was delivered. The patient remained stable until 11 weeks after delivery, when an increase in right ventricular over right atrial pressure was noted. Bosentan was reintroduced and prednisone and azathioprine doses were increased. The patient has remained stable until the present time. Conclusion Pulmonary arterial hypertension has been considered a contraindication for pregnancy. Novel vasodilator therapy, combined with immunosuppressants in this patient with systemic lupus erythematosus, may "cure" pulmonary arterial hypertension and permit pregnancy with successful outcome. However, postpartum exacerbation of systemic lupus erythematosus and pulmonary arterial hypertension have to be considered.

  9. Dynamic respiratory mechanics and exertional dyspnoea in pulmonary arterial hypertension.

    Science.gov (United States)

    Laveneziana, Pierantonio; Garcia, Gilles; Joureau, Barbara; Nicolas-Jilwan, Fadia; Brahimi, Toufik; Laviolette, Louis; Sitbon, Olivier; Simonneau, Gérald; Humbert, Marc; Similowski, Thomas

    2013-03-01

    Patients with pulmonary arterial hypertension (PAH) may exhibit reduced expiratory flows at low lung volumes, which could promote exercise-induced dynamic hyperinflation (DH). This study aimed to examine the impact of a potential exercise-related DH on the intensity of dyspnoea in patients with PAH undergoing symptom-limited incremental cardiopulmonary cycle exercise testing (CPET). 25 young (aged mean±sd 38±12 yrs) nonsmoking PAH patients with no evidence of spirometric obstruction and 10 age-matched nonsmoking healthy subjects performed CPET to the limit of tolerance. Ventilatory pattern, operating lung volumes (derived from inspiratory capacity (IC) measurements) and dyspnoea intensity (Borg scale) were assessed throughout CPET. IC decreased (i.e. DH) progressively throughout CPET in PAH patients (average 0.15 L), whereas it increased in all the healthy subjects (0.45 L). Among PAH patients, 15 (60%) exhibited a decrease in IC throughout exercise (average 0.50 L), whereas in the remaining 10 (40%) patients IC increased (average 0.36 L). Dyspnoea intensity and ventilation were greater in PAH patients than in controls at any stage of CPET, whereas inspiratory reserve volume was lower. We conclude that DH-induced mechanical constraints and excessive ventilatory demand occurred in these young nonsmoking PAH patients with no spirometric obstruction and was associated with exertional dyspnoea.

  10. [New trial designs and potential therapies for pulmonary artery hypertension].

    Science.gov (United States)

    Gomberg-Maitland, Mardi; Bull, Todd M; Saggar, Rajeev; Barst, Robyn J; Elgazayerly, Amany; Fleming, Thomas R; Grimminger, Friedrich; Rainisio, Maurizio; Stewart, Duncan J; Stockbridge, Norman; Ventura, Carlo; Ghofrani, Ardeschir H; Rubin, Lewis J

    2014-10-01

    A greater understanding of the epidemiology, pathogenesis, and pathophysiology of pulmonary artery hypertension (PAH) has led to significant advances, but the disease remains fatal. Treatment options are neither universally available nor always effective, underscoring the need for development of novel therapies and therapeutic strategies. Clinical trials to date have provided evidence of efficacy, but were limited in evaluating the scope and duration of treatment effects. Numerous potential targets in varied stages of drug development exist, in addition to novel uses of familiar therapies. The pursuit of gene and cell-based therapy continues, and device use to help acute deterioration and chronic management is emerging. This rapid surge of drug development has led to multicenter pivotal clinical trials and has resulted in novel ethical and global clinical trial I concerns. This paper will provide an overview of the opportunities and challenges that await the development of novel treatments for PAH. (J Am Coil Cardiol 2013;62:D82-91) ©2013 by the American College of Cardiology Foundation.

  11. Early detection and management of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Marc Humbert

    2012-12-01

    Full Text Available The long-term prognosis for patients with pulmonary arterial hypertension (PAH remains poor, despite advances in treatment options that have been made in the past few decades. Recent evidence suggests that World Health Organization functional class I or II patients have significantly better long-term survival rates than patients in higher functional classes, thus providing a rationale for earlier diagnosis and treatment of PAH. However, early diagnosis is challenging and there is frequently a delay between symptom onset and diagnosis. Screening programmes play an important role in PAH detection and expert opinion favours echocardiographic screening of asymptomatic patients who may be predisposed to the development of PAH (i.e. those with systemic sclerosis or sickle cell disease, although current guidelines only recommend annual echocardiographic screening in symptomatic patients. This article reviews the currently available screening programmes, including their limitations, and describes alternative screening approaches that may identify more effectively those patients who require right heart catheterisation for a definitive PAH diagnosis.

  12. Challenges in the diagnosis and treatment of pulmonary arterial hypertension.

    LENUS (Irish Health Repository)

    2012-12-01

    Advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have resulted in significant improvements in outcomes for patients with this devastating and progressive disease. However, because of the non-specific nature of its symptoms, and the low level of suspicion among clinicians, prompt and accurate diagnosis of PAH as a rare disease remains a challenge. This article explains some of the issues that need to be addressed when faced with a patient with suspected PAH and describes how noninvasive and invasive techniques can be used effectively to ensure an accurate diagnosis. The availability of PAH-specific therapy means that once diagnosed, patients have a much greater chance of survival than they would have had in the past. However, despite improved survival, mortality is still high and, therefore, there is still room for improvement. It is currently recommended that patients with an inadequate clinical response to treatment receive sequential combination therapy; however, supportive data are still scarce. Although there is no clear explanation, these findings may be explained by the design and end-points chosen in clinical trials, the changing population of PAH and a need to improve the management strategy in this disease. Indeed, there is a clear need for randomised controlled studies that investigate whether adopting individualised treatment strategies, including upfront combination therapy, could help to optimise long-term management of patients with PAH.

  13. Paradoxical emboli: demonstration using helical computed tomography of the pulmonary artery associated with abdominal computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Delalu, P.; Ferretti, G.R.; Bricault, I.; Ayanian, D.; Coulomb, M. [Service Central de Radiologie et Imagerie Medicale, CHU Grenoble (France)

    2000-02-01

    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.)

  14. Management of Sepsis in Patients with Pulmonary Arterial Hypertension in the Intensive Care Unit.

    Science.gov (United States)

    Tartavoulle, Todd M

    2017-03-01

    Pulmonary arterial hypertension is a lethal condition, and the management of sepsis in patients with pulmonary arterial hypertension is challenging. As the disease progresses, the right ventricle is susceptible to failure due to a high pulmonary vascular resistance. The limited ability of the right ventricle to increase cardiac output in septic shock makes it difficult to deliver oxygen to the organ and tissues. Intravascular volume replacement and vasoactive drugs should only be considered after a thorough assessment. Priorities of care include improving cardiac output and oxygen delivery by optimizing preload, reducing afterload, and improving contractility.

  15. The vasorelaxing effect of hydrogen sulfide on isolated rat aortic rings versus pulmonary artery rings

    Institute of Scientific and Technical Information of China (English)

    Yan SUN; Chao-shu TANG; Hong-fang JIN; Jun-bao DU

    2011-01-01

    Aim:To compare the vasorelaxing effects of hydrogen sulfide(H2S)on isolated aortic and pulmonary artery rings and to determine their action mechanisms.Methods:H2S-induced vasorelaxation of isolated rat aortic versus pulmonary artery tings under 95%02 and 5%CO2 was analyzed.The expression of cystathinonine gamma-lyase(CSE),cystathionine beta synthase(CBS),3-memaptopyruvate sulfurtransferase(3MST),SUR2B and Kir6.1 was examined.Results:NaHS caused vasorelaxation of rat aortic and pulmonary artery rings in a dose-dependent mannener NaHS dilated aortic rings to a greater extent(16.4%,38.4%,64.1%,84.3%,and 95.9%at concentrations of 50,100.200, 500,and 1000 μmol/L,respectively)than pulmonary artery rings(10.1%,22.2%.50.6%,73.6%,and 84.6%at concentrations of 50,100,200, 500 and 1000 μmol/L,respectively).The EC50 of the vasorelaxant effect for aortic rings was 152.17 pmol/L,whereas the EC50 for pulmonary artery tings was233.65 μmol/L.The vasorelaxing effect of H2was markedly blocked b y cellular and mitochondrial membrane Km channel blockers in aortic rings(P<0.01).In contrast.only the cellular membrane KATP channel blocker inhibited H2S-induced vasorelaxation in pulmonary artery rings.SUR2B mRNA and protein expression was higher in aortic rings than in pulmonary artery rings.Cystathinonine gamma-lyase(CSE)but not cystathionine beta synthase(CBS)expression in aortic rings was higher than in pulmonary artery rings.3.Mercapto PYruvate sulfurtransferase(3MST)mRNA was lower in aortic rings than in pulmonary artery rings.Conclusion:The vasorelaxing effect of H2S on isolated aortic rings was more pronounced than the effect on pulmonary artery rings at specific concentrations,which might be associated with increased expression of the KATP channel subunit SUR2B.

  16. Long-term therapy of interferon-alpha induced pulmonary arterial hypertension with different PDE-5 inhibitors: a case report

    Directory of Open Access Journals (Sweden)

    Baumann Gert

    2005-09-01

    Full Text Available Abstract background Interferon alpha2 is widely used in hepatitis and high-risk melanoma. Interferon-induced pulmonary arterial hypertension as a side effect is rare. Case presentation We describe a melanoma patient who developed severe pulmonary arterial hypertension 30 months after initiation of adjuvant interferon alpha2b therapy. Discontinuation of interferon did not improve pulmonary arterial hypertension. This patient could be treated successfully with phosphodiesterase-5 inhibitor therapy. Conclusion This is only the 5th case of interferon-induced pulmonary arterial hypertension and the first documented case where pulmonary arterial hypertension was not reversible after termination of interferon alpha2 therapy. If interferon alpha2 treated patients develop respiratory symptoms, pulmonary arterial hypertension should be considered in the differential diagnosis. For these patients phosphodiesterase-5 inhibitors, e.g. sildenafil or vardenafil, could be an effective therapeutic approach.

  17. 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.

    2013-01-01

    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 pat

  18. 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.

  19. Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis

    Science.gov (United States)

    Schwaiger, Johannes P.; Loder, Christopher D.; Dobarro, David; Kaier, Thomas; Reddecliffe, Sally; Schreiber, Benjamin E.; Handler, Clive; Denton, Christopher P.; Coghlan, John G.

    2017-01-01

    Our current understanding of the pathophysiology of pulmonary vascular disease is incomplete, since information about alterations of the pulmonary vasculature in pulmonary arterial hypertension (PAH) is primarily provided by autopsy or tissue specimens. The aim of this study was to compare the distal pulmonary vasculature of <2 mm in diameter in Systemic Sclerosis (SSc) patients with (n = 17) and without (n = 5) associated PAH using Optical Coherence Tomography during Right Heart catheterization. SSc-PAH patients showed significant thickening of Intima Media Thickening Area compared to patients without PAH (27 +/− 5.8% vs. 21 +/− 1.4%, p = 0.024). A good haemodynamic response to previous targeted PAH treatment was associated with a significantly greater number of small pulmonary artery side branches <300 μm per cm vessel (3.8 +/− 1.1 vs. 1.8 +/− 1.1; p = 0.010) and not associated with Intima Media thickening Area (26 +/− 5.4% vs. 28 +/− 6.7%; p = 0.6). Unexpected evidence of pulmonary artery thrombus formation was found in 19% of SSc-PAH patients. This is the first in-vivo study demonstrating a direct link between a structural abnormality of pulmonary arteries and a response to targeted treatment in PAH. Intravascular imaging may identify subgroups that may benefit from anticoagulation. PMID:28233825

  20. A clinical study on velocity patterns of pulmonary venous flow in canine heartworm disease.

    Science.gov (United States)

    Shibata, T; Wakao, Y; Takahashi, M

    2000-02-01

    In this study, we evaluated methods of determining the velocity patterns of pulmonary venous flow (PVF) in dogs and then investigated the relationship of the patterns to cardiac functions in heartworm disease (HD) by transthoracic echocardiography (TTE). The results revealed that there was a good correlation between PVF patterns determined by transesophageal echocardiography (TEE) and TTE in animals lying on their left sides. The measurement of S and D wave velocities (PVS and PVD) by TTE was shown to allow clinical determination of the velocity patterns of PVF in dogs. The HD groups showed significant increases in PVS and PVD, and S and D wave time-velocity integrals (S-TVI and D-TVI) of the right cranial lobe PVF, when compared with the normal group, as determined by TTE (P<0.05). In contrast, the HD groups produced significant decreases in PVD and D-TVI of the right caudal lobe PVF compared with the normal group (P<0.05), and a significant increase in the ratio of S-TVI to (S-TVI + D-TVI) (P<0.05). It is, therefore, suggested that measurement of the velocity patterns of the right cranial and caudal lobe PVF could be one method of assessing the stages of obstructive lesions in the pulmonary artery.

  1. Adult presentation of right lung agenesis and left pulmonary artery sling.

    Science.gov (United States)

    Espinosa, L; Agarwal, P

    2008-02-01

    The combination of right lung agenesis and left pulmonary artery (LPA) sling is a rare entity that has been described only in the pediatric population. Cross-sectional imaging modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) have an advantage over echocardiography and pulmonary angiography in demonstrating the anomalous left pulmonary artery, particularly in the presence of coexisting lung agenesis, as exemplified in this case. We report the first case of this rare entity in an adult. It is important to be aware that this abnormality, though rare, can present even in adulthood, and therefore close attention should be paid to the course of the pulmonary artery to ensure detection of a sling in association with lung agenesis.

  2. Adult Presentation of Right Lung Agenesis and Left Pulmonary Artery Sling

    Energy Technology Data Exchange (ETDEWEB)

    Espinosa, L.; Agarwal, P. (Div. of Cardiothoracic Radiology, Dept. of Radiology, Univ. of Michigan, Ann Arbor, MI (US))

    2008-02-15

    The combination of right lung agenesis and left pulmonary artery (LPA) sling is a rare entity that has been described only in the pediatric population. Cross-sectional imaging modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) have an advantage over echocardiography and pulmonary angiography in demonstrating the anomalous left pulmonary artery, particularly in the presence of coexisting lung agenesis, as exemplified in this case. We report the first case of this rare entity in an adult. It is important to be aware that this abnormality, though rare, can present even in adulthood, and therefore close attention should be paid to the course of the pulmonary artery to ensure detection of a sling in association with lung agenesis

  3. Hipertensão arterial pulmonar associada à anemia falciforme Sickle cell anemia-associated pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Roberto Ferreira Pinto Machado

    2007-10-01

    Full Text Available A hipertensão pulmonar é uma complicação comum em pacientes com anemia falciforme. A despeito das elevações leves das pressões pulmonares desses pacientes, a morbimortalidade é alta e, em pacientes adultos com anemia falciforme, a hipertensão pulmonar é um fator de risco muito importante. A patogênese da hipertensão pulmonar relacionada à anemia falciforme é multifatorial e inclui hemólise, baixos níveis de óxido nítrico, hipóxia crônica, tromboembolismo, doença hepática crônica e asplenia. Na maioria dos pacientes, a hipertensão arterial pulmonar é a causa principal para as elevações na pressão arterial pulmonar, mas a hipertensão pulmonar venosa também é um fator contribuinte em alguns pacientes. Existem poucos estudos específicos avaliando os efeitos de tratamento para a hipertensão pulmonar em pacientes com anemia falciforme. É provável que a intensificação da terapia para a anemia hemolítica em todos os pacientes e o tratamento específico para a hipertensão pulmonar em pacientes com doença severa sejam benéficos. Estudos de grande porte avaliando o efeito do tratamento da hipertensão pulmonar em pacientes com anemia falciforme estão em andamento.Pulmonary hypertension is a common complication of sickle cell anemia. Despite the fact that the elevations in pulmonary artery pressures are slight, morbidity and mortality are high. In adult sickle cell anemia patients, pulmonary hypertension is emerging as a major risk factor for death. The pathogenesis of sickle cell anemia-related pulmonary hypertension is multifactorial, including hemolysis, impaired nitric oxide bioavailability, chronic hypoxemia, thromboembolism, chronic liver disease and asplenia. In the majority of patients, pulmonary arterial hypertension is the main cause of elevated pulmonary artery pressures. However, pulmonary venous hypertension also plays a role in a subgroup of patients. Specific data on the effects of treatment

  4. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA in an Old Adult

    Directory of Open Access Journals (Sweden)

    Maryam Esmaeilzadeh

    2011-09-01

    Full Text Available The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA is a rare congenital cardiac malformation. It presents predominantly in infancy and its main presenting feature is myocardial ischemia or heart failure. Survival to adulthood is quite uncommon. If untreated, mortality from ALCAPA approaches 90% in infancy; early recognition and surgical correction are, therefore, essential. With early surgical correction, the prognosis is good. There are two types of ALCAPA syndrome: the infant type and the adult type, each of which has different manifestations and outcomes. Infants experience myocardial infarction and congestive heart failure, and approximately 90% die within the first year of life. A literature review regarding this anomaly in teenagers and adults show that only 25 cases have been diagnosed during life and 18 additional cases of ALCAPA in these age groups have been diagnosed post mortem. We present a rare case of a 60-year-old man, who referred to our center due to dyspnea on exertion from the previous year without any history of chest pain and diagnosed as ALCAPA. Given the absence of ischemia and the patient’s age, only medical therapy was recommended.

  5. Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension.

    Science.gov (United States)

    Courand, Pierre-Yves; Pina Jomir, Géraldine; Khouatra, Chahéra; Scheiber, Christian; Turquier, Ségolène; Glérant, Jean-Charles; Mastroianni, Bénédicte; Gentil, Béatrice; Blanchet-Legens, Anne-Sophie; Dib, Alfred; Derumeaux, Geneviève; Humbert, Marc; Mornex, Jean-François; Cordier, Jean-François; Cottin, Vincent

    2015-01-01

    Right ventricle ejection fraction (RVEF) evaluated with magnetic resonance imaging is a strong determinant of patient outcomes in pulmonary arterial hypertension. We evaluated the prognostic value of RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change 3-6 months after initiating pulmonary arterial hypertension-specific therapy. In a prospective cohort of newly diagnosed patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension, RVEF was measured at baseline (n=100) and 3-6 months after initiation of therapy (n=78). After a median follow-up of 4.1 years, 41 deaths occurred, including 35 from cardiovascular causes. Patients with a (median) baseline RVEF >25% had better survival than those with a RVEF arterial hypertension.

  6. Reference Values of Arterial Gases and Electrolytes in Canines from the Bogota Savanna

    Directory of Open Access Journals (Sweden)

    Carlos Andrés Trujillo Jurado

    2014-06-01

    Full Text Available Due to the lack of arterial gas values that meet our environmental conditions and the wide variety of out-of-date data in literature and used in everyday practice, obtained with different heights, breeds and equipment to the ones available in our context, it becomes necessary to generate our own knowledge adjusted to our reality. A random systematic measurement of arterial and venous blood was made on 100 healthy canines in the Bogota Savanna (Chia: 2652 m.a.s.l.; Cajica: 2558 m.a.s.l.; Sopo: 2650 m.a.s.l.; La Calera: 2718 m.a.s.l.; and Bogota: 2630 m.a.s.l.;, using the I-STAT® analyzer with EG7+ cartridge. Reference values were found for gasometry, electrolytes, effective basis and pH. The values were compared between four groups by weight. Descriptive statistics were made for each parameter based on average, standard deviation and standard error, and the trust limits (95% and the intervals with standard deviation (one and two deviations were found. A decrease on PCO2, PO2, SO2, bicarbonate and BE was evident. The pH was found to be slightly increased, similar to the one reported by authors at great heights and different (not statistically confirmed from reports in literature, where height was not a variable. Even so, they are frequently used as reference in the practice of small animals; only PO2 presented statistical differences by groups of weight. As a conclusion, effective regulation of blood pH was evident at 2600 m.a.s.l., with different bicarbonate values, PCO2, PO2, BE, which suggest the need to obtain and use reference values consistent with local conditions.

  7. Percutaneous pulmonary valve implantation in a single artery branch: A preliminary experience

    Institute of Scientific and Technical Information of China (English)

    Massimo; Chessa; Gianfranco; Butera; Luca; Giugno; Angelo; Micheletti; Diana; G; Negura; Mario; Carminati

    2015-01-01

    To describe preliminary experience of percutaneous pulmonary valve implantation, in a single pulmonary branch position. Two procedures in 2 patients from a single center are described, where implantation of percutaneous valves within a single pulmonary artery branch was technically successful. The procedural indication was pulmonary valve regurgitation and/or residual stenosis. The 2 patients were symptomatic. An Edwards Sapien? valve(Patient 1), and a Medtronic Melody? valve(Patient 2) were implanted. Both pts were discharged with an excellent valve function. In this report it is underlined that this modality is technically feasible and may be considered an option in patients with congenital heart defect under special circumstances.

  8. Echocardiography may help detect pulmonary vasculopathy in the early stages of pulmonary artery hypertension associated with systemic sclerosis

    Directory of Open Access Journals (Sweden)

    Serra Walter

    2010-07-01

    Full Text Available Abstract Background Pulmonary arterial hypertension (PAH in patients with systemic sclerosis is associated with a poor prognosis, but this can be improved by early disease detection. Abnormal pulmonary and cardiac function can be detected early by means of echocardiography, whereas right heart catheterization is usually performed later. Objectives The purpose of this prospective study was to detect early the presence of pulmonary artery vasculopathy in patients with verified systemic sclerosis without significant pulmonary fibrosis, normal lung volumes and a mildly reduced lung diffusion capacity of carbon monoxide (DLCO. Methods Nineteen consecutive female NYHA class I-II patients with scleroderma and a PAPs of 2. They all underwent complete Doppler echocardiography, CPET, a pulmonary ventilation test (carbon monoxide lung diffusion, DLCO, HRCT. To investigate PAH by means of complete resting Doppler echocardiography estimates of systolic pulmonary artery pressure (PAPs derived from tr icuspid regurgitation, mean PAP derived from pulmonary regurgitation, pulmonary vessel resistance (PVR derived from the acceleration time of the pulmonary outflow tract (ACTpo, and right ventricular function derived from tricuspid annular plane systolic excursion (TAPSE. Right heart catheterisation was conducted only, if pulmonary hypertension was suggested by echocardiography and an abnormal ventilator test. The data are given as mean values ± SD, unless otherwise stated. The correlations between the variables were analysed using Pearson's r coefficient, and the predictive value of the variables was calculated using linear regression analysis. A p value of > 0.05 was considered significant. Results Right heart catheterization detected PAH in 15/19 patients; mean PAP was 30.5 mm/Hg and RVP 3.6 UW. Coronary angiography of the patients aged more than 55 years showed some evidence of significant coronary artery disease. Echocardiography showed high systolic PAP

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

    OpenAIRE

    2011-01-01

    Objective 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). Materials and Methods 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, a...

  10. Cardiovascular magnetic resonance of pulmonary artery growth and ventricular function after Norwood procedure with Sano modification

    Directory of Open Access Journals (Sweden)

    Matherne G Paul

    2008-07-01

    Full Text Available Abstract For hypoplastic left heart syndrome (HLHS, there have been concerns regarding pulmonary artery growth and ventricular dysfunction after first stage surgery consisting of the Norwood procedure modified with a right ventricle-to-pulmonary artery conduit. We report our experience using cardiovascular magnetic resonance (CMR to determine and follow pulmonary arterial growth and ventricular function in this cohort. Following first stage palliation, serial CMR was performed at 1 and 10 weeks post-operatively, followed by cardiac catheterization at 4 – 6 months. Thirty-four of 47 consecutive patients with HLHS (or its variations underwent first stage palliation. Serial CMR was performed in 20 patients. Between studies, ejection fraction decreased (58 ± 9% vs. 50 ± 5%, p In this cohort, reasonable growth of pulmonary arteries occurred following first stage palliation with this modification, although that growth was preferential to the left. Serial studies demonstrate worsening of ventricular function for the cohort. CMR was instrumental for detecting pulmonary artery stenosis and right ventricular dysfunction.

  11. Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights.

    Science.gov (United States)

    Dardi, Fabio; Manes, Alessandra; Palazzini, Massimiliano; Bachetti, Cristina; Mazzanti, Gaia; Rinaldi, Andrea; Albini, Alessandra; Gotti, Enrico; Monti, Enrico; Bacchi Reggiani, Maria Letizia; Galiè, Nazzareno

    2015-08-01

    Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option.This study aimed to assess the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real-world setting.Patients with idiopathic/heritable pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease or connective tissue disease and who were not meeting treatment goals on either first-line bosentan or sildenafil monotherapy, were given additional sildenafil or bosentan and assessed after 3-4 months. Double combination therapy significantly improved clinical and haemodynamic parameters, independent of aetiology or the order of drug administration. Significant improvements in functional class were observed in patients with idiopathic/heritable pulmonary arterial hypertension. The 1-, 3- and 5-year overall survival estimates were 91%, 69% and 59%, respectively. Patients with pulmonary arterial hypertension associated with connective tissue disease had significantly poorer survival rates compared to other aetiologies (p<0.003).The favourable short-term haemodynamic results and good survival rates, observed in patients receiving both bosentan and sildenafil, supports the use of sequential combination therapy in patients failing on monotherapy in a real-world setting.

  12. Pioglitazone alleviates cardiac and vascular remodelling and improves survival in monocrotaline induced pulmonary arterial hypertension.

    Science.gov (United States)

    Behringer, Arnica; Trappiel, Manuela; Berghausen, Eva Maria; Ten Freyhaus, Henrik; Wellnhofer, Ernst; Odenthal, Margarete; Blaschke, Florian; Er, Fikret; Gassanov, Natig; Rosenkranz, Stephan; Baldus, Stephan; Kappert, Kai; Caglayan, Evren

    2016-04-01

    Pulmonary arterial hypertension (PAH) is a fatal disease with limited therapeutic options. Pathophysiological changes comprise obliterative vascular remodelling of small pulmonary arteries, elevated mean pulmonary arterial systolic pressure (PASP) due to elevated resistance of pulmonary vasculature, adverse right ventricular remodelling, and heart failure. Recent findings also indicate a role of increased inflammation and insulin resistance underlying the development of PAH. We hypothesized that treatment of this condition with the peroxisome proliferator-activated receptor-γ (PPARγ) activator pioglitazone, known to regulate the expression of different genes addressing insulin resistance, inflammatory changes, and vascular remodelling, could be a beneficial approach. PAH was induced in adult rats by a single subcutaneous injection of monocrotaline (MCT). Pioglitazone was administered for 2 weeks starting 3 weeks after MCT-injection. At day 35, hemodynamics, organ weights, and -indices were measured. We performed morphological and molecular characterization of the pulmonary vasculature, including analysis of the degree of muscularization, proliferation rates, and medial wall thickness of the small pulmonary arteries. Furthermore, markers of cardiac injury, collagen content, and cardiomyocyte size were analyzed. Survival rates were monitored throughout the experimental period. Pioglitazone treatment improved survival, reduced PASP, muscularization of small pulmonary arteries, and medial wall thickness. Further, MCT-induced right ventricular hypertrophy and fibrosis were attenuated. This was accompanied with reduced cardiac expression of brain natriuretic peptide, as well as decreased cardiomyocyte size. Finally, pulmonary macrophage content and osteopontin gene expression were attenuated. Based on the beneficial impact of pioglitazone, activation of PPARγ might be a promising treatment option in PAH.

  13. A novel multiscale topo-morphometric approach for separating arteries and veins via pulmonary CT imaging

    Science.gov (United States)

    Saha, Punam K.; Gao, Zhiyun; Alford, Sara; Sonka, Milan; Hoffman, Eric

    2009-02-01

    Distinguishing arterial and venous trees in pulmonary multiple-detector X-ray computed tomography (MDCT) images (contrast-enhanced or unenhanced) is a critical first step in the quantification of vascular geometry for purposes of determining, for instance, pulmonary hypertension, using vascular dimensions as a comparator for assessment of airway size, detection of pulmonary emboli and more. Here, a novel method is reported for separating arteries and veins in MDCT pulmonary images. Arteries and veins are modeled as two iso-intensity objects closely entwined with each other at different locations at various scales. The method starts with two sets of seeds -- one for arteries and another for veins. Initialized with seeds, arteries and veins grow iteratively while maintaining their spatial separation and eventually forming two disjoint objects at convergence. The method combines fuzzy distance transform, a morphologic feature, with a topologic connectivity property to iteratively separate finer and finer details starting at a large scale and progressing towards smaller scales. The method has been validated in mathematically generated tubular objects with different levels of fuzziness, scale and noise. Also, it has been successfully applied to clinical CT pulmonary data. The accuracy of the method has been quantitatively evaluated by comparing its results with manual outlining. For arteries, the method has yielded correctness of 81.7% at the cost of 6.7% false positives and 11.6% false negatives. Our method is very promising for automated separation of arteries and veins in MDCT pulmonary images even when there is no mark of intensity variation at conjoining locations.

  14. Home-made fenestrated amplatzer occluder for atrial septal defect and pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    Fabio Dell'Avvocata; Gianluca Rigatelli; Paolo Cardaioli; Massimo Giordan

    2011-01-01

    We report the management of a patient with secundum atrial septal defect (ASD)and severe pulmonary hypertension.A 65-year-old male with recently diagnosed atrial serital defect was referred to our centre for decompensated right heart failure with rest and exercise induced dispnea and severe pulmonary hypertension.Right heart catheterization confirmed a mean pulmonary pressure of about 55 mmHg and a Qp/Qs of 2.7.An occlusion test with a compliant large balloon demonstrated partial fall of pulmonary arterial pressure.The implantation of a home-made fenestrated Amplatzer ASD Occluder (ASO) was planned in order to decrease lefttoright shunt and promote further decrease of pulmonary arterial pressure in the long-term.Thus,by means of mechanical intracardiac echocardiography study with a 9F 9 MHz Ultralce catheter(Boston Scientific Corp.),we selected a 34 mm ASO for implantation.Four millimeter fenestration was made inflating a 4 ntm non-compliant coronary balloon throughout the waist of the ASO,which was successfully implanted under intracardiac echocardiography.After six months,a decrease of pulmonary arterial pressure to 24 mmHg and full compensated right heart failure was observed on transthoracic echocardiography and clinical examination.This case suggests that transcatheter closure with home-made fenestrated ASD in elderly patients with severe pulmonary hypertension is feasible.

  15. Chronic thromboembolic pulmonary arterial hypertension: a review of the literature and novel therapeutic approaches.

    Science.gov (United States)

    Androulakis, Emmanuel; Lioudaki, Eirini; Christophides, Theodoros; Ahmad, Mahmood; Fayed, Hossam; Laskar, Nabila; Schreiber, Benjamin

    2015-06-01

    Chronic thromboembolic pulmonary hypertension is defined as pulmonary hypertension (PH) caused by single or recurrent pulmonary emboli and is characterized by chronic obstruction of the pulmonary arteries leading to increased vascular resistance and PH. Also, progressive remodeling may occur in occluded and nonoccluded territories. Better understanding of the underlying mechanisms and risk factors could improve diagnosis and allow appropriate interventions. Pulmonary endarterectomy is an established approach and is considered the definitive treatment for chronic PH, resulting from thromboembolic disease. Furthermore, percutaneous transluminal pulmonary angioplasty is technically feasible, especially for those with peripheral-type of the disease. In addition, several agents, including prostanoids, endothelin receptor antagonists and phosphodiesterase type-5 inhibitors, have been tested in selected patients yielding promising results. Several novel agents are under investigation, and extensive research is currently in progress aiming to resolve uncertainties in the understanding and treatment of the disease.

  16. Dysregulated renin-angiotensin-aldosterone system contributes to pulmonary arterial hypertension

    Science.gov (United States)

    De Man, Frances; Tu, Ly; Handoko, Louis; Rain, Silvia; Ruiter, Gerrina; François, Charlène; Schalij, Ingrid; Dorfmüller, Peter; Simonneau, Gérald; Fadel, Elie; Perros, Frederic; Boonstra, Anco; Postmus, Piet; Van Der Velden, Jolanda; Vonk-Noordegraaf, Anton; Humbert, Marc; Eddahibi, Saadia; Guignabert, Christophe

    2012-01-01

    Rationale Patients with idiopathic pulmonary arterial hypertension (iPAH) often have a low cardiac output. To compensate, neurohormonal systems like renin-angiotensin-aldosterone system (RAAS) and sympathetic nervous system are upregulated but this may have long-term negative effects on the progression of iPAH. Objectives Assess systemic and pulmonary RAAS-activity in iPAH-patients and determine the efficacy of chronic RAAS-inhibition in experimental PAH. Measurements and Main Results We collected 79 blood samples from 58 iPAH-patients in the VU University Medical Center Amsterdam (between 2004–2010), to determine systemic RAAS-activity. We observed increased levels of renin, angiotensin (Ang) I and AngII, which was associated with disease progression (p<0.05) and mortality (p<0.05). To determine pulmonary RAAS-activity, lung specimens were obtained from iPAH-patients (during lung transplantation, n=13) and controls (during lobectomy or pneumonectomy for cancer, n=14). Local RAAS-activity in pulmonary arteries of iPAH-patients was increased, demonstrated by elevated ACE-activity in pulmonary endothelial cells and increased AngII type 1 (AT1) receptor expression and signaling. In addition, local RAAS- upregulation was associated with increased pulmonary artery smooth muscle cell proliferation via enhanced AT1-receptor signaling in iPAH-patients compared to controls. Finally, to determine the therapeutic potential of RAAS-activity, we assessed the chronic effects of an AT1-receptor antagonist (losartan) in the monocrotaline PAH-rat model (60 mg/kg). Losartan delayed disease progression, decreased RV afterload and pulmonary vascular remodeling and restored right ventricular-arterial coupling in PAH-rats. Conclusions Systemic and pulmonary RAAS-activities are increased in iPAH-patients and associated with increased pulmonary vascular remodeling. Chronic inhibition of RAAS by losartan is beneficial in experimental PAH. PMID:22859525

  17. Anomalous origin of the right coronary artery from the pulmonary artery: an autopsied sudden death case with severe atherosclerotic disease of the left coronary artery.

    Science.gov (United States)

    Nagai, T; Mukai, T; Takahashi, S; Takada, A; Saito, K; Harada, K; Mori, S; Abe, N

    2014-03-01

    Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare anomaly. It may contribute to myocardial ischemia or sudden death, although the lesion is usually asymptomatic. We report a sudden death case of a 58-year-old man with ARCAPA coexisting with severe atherosclerotic coronary artery disease. He had been healthy until he complained of chest pain, several days before death, despite the discovery of heart murmur in childhood and suspicion of valvular heart disease. The autopsy revealed not only typical findings of the right coronary anomaly with well-developed collateral circulations but also severe atherosclerotic lesions of the left coronary artery, and ischemic change of the myocardium in the left and right coronary arterial perfusion territory. In addition to the "coronary steal" phenomenon primarily caused by ARCAPA, the reduced flow of both coronary arteries and further increase of "coronary steal" due to atherosclerotic obstructive coronary disease might have contributed to the patient's death.

  18. Combination of constant-flow and continuous positive-pressure ventilation in canine pulmonary edema.

    Science.gov (United States)

    Sznajder, J I; Becker, C J; Crawford, G P; Wood, L D

    1989-08-01

    Constant-flow ventilation (CFV) maintains alveolar ventilation without tidal excursion in dogs with normal lungs, but this ventilatory mode requires high CFV and bronchoscopic guidance for effective subcarinal placement of two inflow catheters. We designed a circuit that combines CFV with continuous positive-pressure ventilation (CPPV; CFV-CPPV), which negates the need for bronchoscopic positioning of CFV cannula, and tested this system in seven dogs having oleic acid-induced pulmonary edema. Addition of positive end-expiratory pressure (PEEP, 10 cmH2O) reduced venous admixture from 44 +/- 17 to 10.4 +/- 5.4% and kept arterial CO2 tension (PaCO2) normal. With the innovative CFV-CPPV circuit at the same PEEP and respiratory rate (RR), we were able to reduce tidal volume (VT) from 437 +/- 28 to 184 +/- 18 ml (P less than 0.001) and elastic end-inspiratory pressures (PEI) from 25.6 +/- 4.6 to 17.7 +/- 2.8 cmH2O (P less than 0.001) without adverse effects on cardiac output or pulmonary exchange of O2 or CO2; indeed, PaCO2 remained at 35 +/- 4 Torr even though CFV was delivered above the carina and at lower (1.6 l.kg-1.min-1) flows than usually required to maintain eucapnia during CFV alone. At the same PEEP and RR, reduction of VT in the CPPV mode without CFV resulted in CO2 retention (PaCO2 59 +/- 8 Torr). We conclude that CFV-CPPV allows CFV to effectively mix alveolar and dead spaces by a small bulk flow bypassing the zone of increased resistance to gas mixing, thereby allowing reduction of the CFV rate, VT, and PEI for adequate gas exchange.

  19. Brain natriuretic peptide in pulmonary arterial hypertension: biomarker and potential therapeutic agent

    Directory of Open Access Journals (Sweden)

    Brian Casserly

    2009-11-01

    Full Text Available Brian Casserly, James R KlingerDivision of Pulmonary and Critical Care Medicine, The Memorial Hospital of Rhode Island, Pawtucket, RI, Rhode Island Hospital, Providence, RI, Alpert Medical School of Brown University, Providence, RI, USAAbstract: B-type natriuretic peptide (BNP is a member of the natriuretic peptide family, a group of widely distributed, but evolutionarily conserved, polypeptide mediators that exert myriad cardiovascular effects. BNP is a potent vasodilator with mitogenic, hypertrophic and pro-inflammatory properties that is upregulated in pulmonary hypertensive diseases. Circulating levels of BNP correlate with mean pulmonary arterial pressure (mPAP and pulmonary vascular resistance (PVR in patients with pulmonary arterial hypertension (PAH. Elevated plasma BNP levels are associated with increased mortality in patients with PAH and a fall in BNP levels after therapy is associated with improved survival. These findings have important clinical implications in that a noninvasive blood test may be used to identify PAH patients at high-risk of decompensation and to guide pulmonary vasodilator therapy. BNP also has several biologic effects that could be beneficial to patients with PAH. However, lack of a convenient method for achieving sustained increases in circulating BNP levels has impeded the development of BNP as a therapy for treating pulmonary hypertension. New technologies that allow transdermal or oral administration of the natriuretic peptides have the potential to greatly accelerate research into therapeutic use of BNP for cor pulmonale and pulmonary vascular diseases. This review will examine the basic science and clinical research that has led to our understanding of the role of BNP in cardiovascular physiology, its use as a biomarker of right ventricular function and its therapeutic potential for managing patients with pulmonary vascular disease.Keywords: brain natriuretic peptide, pulmonary artery hypertension

  20. Cigarette smoke exposure induced pulmonary artery pressure increase through inhibiting Kv1.5 and Kv2.1 mRNA expression in rat pulmonary artery smooth muscles

    Institute of Scientific and Technical Information of China (English)

    林纯意

    2012-01-01

    Objective To investigate the effect of cigarette smoke exposure on Kv1.5 and Kv2.1 mRNA expression in rat pulmonary arterial smooth muscle cells(PASMCs), and further to clarify the possible mechanism of cigarette smoking induced pulmonary arterial hypertension. Methods Primary

  1. 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.

  2. AMBITION: An important piece in the therapeutic puzzle of pulmonary arterial hypertension.

    Science.gov (United States)

    Said, Karim

    2015-01-01

    It is believed that simultaneous targeting of two or more of the three pathogenic pathways of pulmonary arterial hypertension (the endothelin, nitric oxide, and prostacyclin pathways) is associated with additive or synergistic effects with subsequent increasing efficacy and improving outcomes. However, there is lack of evidence to guide the use of combination strategy among pulmonary arterial hypertension patients and many questions remain to be answered. One of these vital questions is whether the strategy of upfront initiation of combination therapy could improve patients outcomes compared to the strategy of initial monotherapy. The recently published AMBITION trial represents an important forward step towards answering this question by comparing a strategy of first-line combination therapy (ambrisentan and tadalafil) versus first-line monotherapy (ambrisentan or tadalafil) in patients with pulmonary arterial hypertension.

  3. Effect of ivaradine on hyperpolarization activated cation current in canine pulmonary vein sleeve cardiomyocytes with atrial fibrillation

    Institute of Scientific and Technical Information of China (English)

    Yang Li; Yan Huang; Zongbin Li; Hao Wang; Jianguo Song; Yuqi Liu; Lei Gao; Shiwen Wang

    2008-01-01

    Objective To study the effect of ivabradine on hyperpolarization activated cation current in canine pulmonary vein(PY) sleeve cardiomyocytes with atrial fibrillation.Methods Dissociation of PVs yielded single cardiomyocytes from a Landengorff column without or with pacemaker activity from long-term rapidly atrial pacing (RAP) canines.If current was measured with the whole-cell patch-clamp technique.Results Compared with the control group,the rapidly atrial pacing canine PV cardiomyocytes had spontaneous diastolic depolarization and had larger If densities.Ivabradine (Iva,1 μM),a selective inhibitor of the If current,markedly reduced If currents in the RAP from -2.66±0.4 pA/pF to -1.58±0.1 pA/pF at the test potential of-120 mV (P<0.01,n=12).Inhibition effect of Iva of If current showed concentration-dependent range from 0.1 to 10.0μM,with IC50 of 2.2 μ M ( 1.8-2.9 μM,95% CL).Furthermore,V1/ of steady-state activated curve was shifted from -84.3±4.9 mV to -106.9±3.4 mV and k value of steady-state activated curve was changed from 12.1+2.6 mV to 9.9±3.4 mV by the application of.1.0 μM Iva ( P<0.01,n=12).Conclusions Our study revealed that Ivarbadine may significantly decrease If of rapidly atrial pacing pulmonary vein sleeve ceUs with atrial fibdllation.(J Geriatr Cardiol 2008;5:39-42)

  4. Lipopolysaccharide and Interleukin 1 Augment the Effects of Hypoxia and Inflammation in Human Pulmonary Arterial Tissue

    Science.gov (United States)

    Ziesche, Rolf; Petkov, Venzeslav; Williams, John; Zakeri, Schaker M.; Mosgoller, Wilhelm; Knofler, Martin; Block, Lutz H.

    1996-10-01

    The combined effects of hypoxia and interleukin 1, lipopolysaccharide, or tumor necrosis factor α on the expression of genes encoding endothelial constitutive and inducible nitric oxide synthases, endothelin 1, interleukin 6, and interleukin 8 were investigated in human primary pulmonary endothelial cells and whole pulmonary artery organoid cultures. Hypoxia decreased the expression of constitutive endothelial nitric oxide synthase (NOS-3) mRNA and NOS-3 protein as compared with normoxic conditions. The inhibition of expression of NOS-3 corresponded with a reduced production of NO. A combination of hypoxia with bacterial lipopolysaccharide, interleukin 1β , or tumor necrosis factor α augmented both effects. In contrast, the combination of hypoxia and the inflammatory mediators superinduced the expression of endothelin 1, interleukin 6, and interleukin 8. Here, we have shown that inflammatory mediators aggravate the effect of hypoxia on the down-regulation of NOS-3 and increase the expression of proinflammatory cytokines in human pulmonary endothelial cells and whole pulmonary artery organoid cultures.

  5. Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients.

    Science.gov (United States)

    Seyahi, Emire; Melikoglu, Melike; Akman, Canan; Hamuryudan, Vedat; Ozer, Harun; Hatemi, Gulen; Yurdakul, Sebahattin; Tuzun, Hasan; Oz, Buge; Yazici, Hasan

    2012-01-01

    Pulmonary artery aneurysms (PAAs) are well known causes of mortality and morbidity in Behçet disease (BD). However, pulmonary artery involvement in BD is not limited to PAA; the other main type of pulmonary artery involvement is pulmonary artery thrombus (PAT), with or without associated PAA. In addition, other types of lung disease like nodules and cavities in the lung parenchyma are frequently associated with pulmonary artery involvement, and can be misinterpreted as being due to infection. We surveyed the clinical, radiologic, and laboratory characteristics and outcome of 47 BD patients with pulmonary artery involvement and the associated findings, all seen and followed at a single dedicated tertiary care center.We identified 47 (41 male, 6 female) patients in whom pulmonary artery involvement was diagnosed, who were registered in the multidisciplinary clinic at Cerrahpasa Medical Faculty between January 2000 and December 2007. Mean age at diagnosis was 29 ± 8 years, and mean disease duration to the onset of pulmonary artery involvement was 3.6 ± 4.8 years. Hemoptysis was the most common presenting symptom (79%) followed by cough, fever, dyspnea, and pleuritic chest pain. Thirty-four of 47 patients (72%) presented with PAA, including 8 with associated PAT. The remaining 13 patients (28%) had isolated PAT. Patients with isolated PAT in general have clinical features similar to patients with PAA. However, hemoptysis was less frequent and voluminous in patients with isolated PAT. Most (91%) of the patients had active disease outside the lungs when they presented with pulmonary artery involvement.Forty (85%) patients had nodules and 6 (13%) had cavities when first seen. Peripheral venous thrombosis was present in 36 of 47 (77%) patients, and intracardiac thrombi in 12 of the 36 (33%) patients. Nodules, cavities, and intracardiac thrombi were mainly present in the acute stages of pulmonary artery involvement.Pulmonary artery involvement is usually multiple, and

  6. Lung Aplasia with Pulmonary Artery Sling Like Anomaly

    Directory of Open Access Journals (Sweden)

    Onur Isik

    2016-04-01

    Full Text Available Unilateraly pulmonary agenesis is an extremely rare documented congenital anomaly. It may be defined as total absence of the pulmonary parencilyma, its vascular supporting structure, and bronchi beyond the bifurcation. The present case with reconstructed images is one of the different cases of agenesis reported in the literature.

  7. A Rare Case of Pulmonary Artery Dissection Associated With Infective Endocarditis.

    Science.gov (United States)

    Shi, Xiaoqing; Wang, Xiaoqin; Wang, Chuan; Zhou, Kaiyu; Li, Yifei; Hua, Yimin

    2016-05-01

    Pulmonary artery dissection (PAD) is a rare condition with high mortality and has not been reported in patient with infective endocarditis (IE). Here, we report the first case of such patient who experienced PDA and survived after surgical intervention.A 10-year-old female child was diagnosed as IE with a patent ductus arteriosis (PDA) and a vegetation on the left side of pulmonary artery trunk (10 × 5 mm). Following 3-week antibacterial treatment, the body temperature of patient returned to normal, and the size of vegetation reduced (7 × 3 mm). However, the patient had a sudden attack of sustained and crushing right chest pain, orthopnea with increasing respiratory rate (> 60/min), and acute high fever. Echocardiography revealed the detachment of vegetation on the first day and dissection of pulmonary artery on the next day. The patient received immediate surgical intervention. It was found that aneurysm had a size of 28 × 20 mm and its orifice (the dissecting site) located on the opposite side of the PDA opening (right side of the pulmonary artery trunk). The dissected left wall of pulmonary artery trunk was reconstructed followed by the closure of PDA with suture. The patient recovered uneventfully.From this case, we learned that the surgical intervention should be considered at an early time for IE patients who have a vegetation in pulmonary artery and PDA. After the infection is under control, the earlier surgery may prevent severe complications.

  8. Erythropoietin attenuates pulmonary vascular remodeling in experimental pulmonary arterial hypertension through interplay between endothelial progenitor cells and heme-oxygenase

    Directory of Open Access Journals (Sweden)

    Rosa L.E. Loon

    2015-08-01

    Full Text Available BackgroundPulmonary 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 (HO1.MethodsRats with flow-associated PAH, resembling pediatric PAH, were treated with HO-1 inducer EPO in the presence or absence of the selective HO-activity-inhibitor tin-mesoporphyrin (SnMP. HO-activity, circulating EPCs and pulmonary vascular lesions were assessed after 3 weeks.ResultsIn PAH-rats, circulating EPCs were decreased and HO-activity was increased compared to control. EPO-treatment restored circulating EPCs and improved pulmonary vascular remodeling, as shown by a reduced wall thickness and occlusion rate of the intra-acinar vessels. Inhibition of HO-activity with SnMP aggravated PAH. Moreover, SnMP treatment abrogated EPO-induced amelioration of pulmonary vascular remodeling, while surprisingly further increasing circulating EPCs as compared with EPO alone.ConclusionsIn experimental PAH, EPO treatment restored the number of circulating EPC’s to control level, improved pulmonary vascular remodeling, and showed important interplay with HO-activity. Inhibition of increased HO-activity in PAH-rats exacerbated progression of pulmonary vascular remodeling, despite the presence of restored numbers of circulating EPC’s. We suggest that both EPO-induced HO1 and EPCs are promising targets to ameliorate the pulmonary vasculature in PAH.

  9. A method for quantitative characterization of growth in the 3-D structure of rat pulmonary arteries.

    Science.gov (United States)

    Razavi, Hedi; Dusch, Marie N; Zarafshar, Shahrzad Y; Taylor, Charles A; Feinstein, Jeffrey A

    2012-03-01

    Understanding mechanisms causing pulmonary vascular disease (PVD) frequently requires a thorough understanding of the underlying structural changes in the pulmonary circulation. Animal models have been used extensively to study different forms of PVD but conventional experimental techniques are limited in their ability to allow the study of the whole pulmonary vasculature at once. In this study, we introduce novel techniques of arterial casting, high-resolution imaging and tree analysis to study the pulmonary circulation in rodent models. Male Sprague-Dawley rats were used at 20, 36, 52, 100 and 160 days of age. A technique involving arterial casting with Microfil silicone polymer, high-resolution micro-computed tomography (micro-CT) at 12.5 μm resolution and image data analysis involving segmentation and skeletonization was developed to both qualitatively and quantitatively describe the changes in the pulmonary vasculature with increasing age. Parameters identified to affect the quality of pulmonary arterial casting included polymer flow rate, total injected volume, polymer viscosity and polymerization time. By optimizing these parameters, we successfully created arterial casts of the pulmonary circulation in rats of different ages and demonstrated the feasibility of quantitatively characterizing the changes in the number of vessels with postnatal growth. These preliminary data suggest that the number of vessels with lumen diameters of 50-200 μm increases rapidly in both lungs between 52 and 100 days of age. With this new technique, the pulmonary vasculature can now be studied in a whole lung animal model to better understand the global effects of disease on vascular structure.

  10. Clinical Analysis of Pulmonary Lipiodol Embolism in Patients with Hepatic Carcinoma after Transcatheter Arterial Chemoembolization

    OpenAIRE

    2015-01-01

    Objective:To explore the clinical manifestations, therapeutic methods and preventive measures of pulmonary lipiodol embolism (PLE) induced by transcatheter arterial chemoembolization (TACE) so as to improve the cognition and management of PLE. Methods:A total of 2 613 patients with hepatic cancer without history of pulmonary disease who were treated with TACE in our hospital from Sept., 2004 to Mar., 2013 were selected. The clinical manifestations, therapeutic methods and preventing measures ...

  11. Dynamic cone beam CT angiography of carotid and cerebral arteries using canine model

    Energy Technology Data Exchange (ETDEWEB)

    Cai Weixing; Zhao Binghui; Conover, David; Liu Jiangkun; Ning Ruola [Department of Imaging Sciences, University of Rochester, 601 Elmwood Avenue, Rochester, New York 14642 (United States); Department of Radiology, Shanghai 6th People' s Hospital, 600 Yishan Road, Xuhui, Shanghai (China); Koning Corporation, Lennox Tech Enterprise Center, 150 Lucius Gordon Drive Suite 112, West Henrietta, New York 14586 (United States); Department of Imaging Sciences, University of Rochester, 601 Elmwood Avenue, Rochester, New York 14642 (United States); Department of Imaging Sciences, University of Rochester, 601 Elmwood Avenue, Rochester, New York 14642 (United States) and Koning Corporation, Lennox Tech Enterprise Center, 150 Lucius Gordon Drive Suite 112, West Henrietta, New York 14586 (United States)

    2012-01-15

    Purpose: This research is designed to develop and evaluate a flat-panel detector-based dynamic cone beam CT system for dynamic angiography imaging, which is able to provide both dynamic functional information and dynamic anatomic information from one multirevolution cone beam CT scan. Methods: A dynamic cone beam CT scan acquired projections over four revolutions within a time window of 40 s after contrast agent injection through a femoral vein to cover the entire wash-in and wash-out phases. A dynamic cone beam CT reconstruction algorithm was utilized and a novel recovery method was developed to correct the time-enhancement curve of contrast flow. From the same data set, both projection-based subtraction and reconstruction-based subtraction approaches were utilized and compared to remove the background tissues and visualize the 3D vascular structure to provide the dynamic anatomic information. Results: Through computer simulations, the new recovery algorithm for dynamic time-enhancement curves was optimized and showed excellent accuracy to recover the actual contrast flow. Canine model experiments also indicated that the recovered time-enhancement curves from dynamic cone beam CT imaging agreed well with that of an IV-digital subtraction angiography (DSA) study. The dynamic vascular structures reconstructed using both projection-based subtraction and reconstruction-based subtraction were almost identical as the differences between them were comparable to the background noise level. At the enhancement peak, all the major carotid and cerebral arteries and the Circle of Willis could be clearly observed. Conclusions: The proposed dynamic cone beam CT approach can accurately recover the actual contrast flow, and dynamic anatomic imaging can be obtained with high isotropic 3D resolution. This approach is promising for diagnosis and treatment planning of vascular diseases and strokes.

  12. Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: a rare case diagnosed during the postpartum period.

    Science.gov (United States)

    Beker-Acay, Mehtap; Ozkececi, Gulay; Unlu, Ebru; Hocaoglu, Elif; Kacar, Emre; Onrat, Ersel

    2014-01-01

    Unilateral absence of a pulmonary artery a very rare congenital disorder. We here present a case of a 22-year-old female patient with agenesis of the right pulmonary artery accompanying patent ductus arteriosus and Eisenmenger syndrome, diagnosed by chest X-ray and multidetector computed tomography 5 days after giving birth.

  13. [Primary pulmonary artery sarcoma in 36-year-old women: 3-years follow-up after partial resection and radiotherapy].

    Science.gov (United States)

    Drożdż, Jarosław; Warchoł, Ewa; Fijuth, Jacek; Filipiak, Krzysztof; Spych, Michał; Maciejewski, Marek; Piestrzeniewicz, Katarzyna; Ludomir, Stafańczyk; Janaszek-Sitkowska, Hanna; Januszewicz, Andrzej; Zembala, Marian

    2013-01-01

    Intimal sarcoma of the heart and pulmonary artery is a very rare, malignant, primary tumour. The prognosis in patients with primary sarcoma of the pulmonary artery, including intimal sarcoma, is poor. We present the case and 3-years follow-up of 36-year-old woman who was successfully treated with surgical, partial resection of the tumour followed by radiotherapy.

  14. Pulmonary Arterial Hypertension in Adults: Novel Drugs and Catheter Ablation Techniques Show Promise? Systematic Review on Pharmacotherapy and Interventional Strategies

    OpenAIRE

    Salvatore Rosanio; Francesco Pelliccia; Carlo Gaudio; Cesare Greco; Abdul M. Keylani; D’Agostino, Darrin C.

    2014-01-01

    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 t...

  15. Indications, Results and Mortality of Pulmonary Artery Banding Procedure: a Brief Review and Five- year Experiences

    Directory of Open Access Journals (Sweden)

    Hamid Hoseinikhah

    2016-05-01

    Full Text Available Background Pulmonary artery banding (PAB is a technique of palliative surgical therapy used by congenital heart surgeons as a staged approach to operative correction of congenital heart defects. Materials and Methods We report 5- year experiences from January 2011 to January 2016 of Imam Reza Hospital center (a tertiary referral hospital in Mashhad city, North East of Iran that consist of 50 patients with congenital heart disease with left to right shunt that pulmonary artery banding procedure was performed for them were studied. Results Age of patients (n=50 was 1to 9 months (mean=4.6 + 1.3. In this study, the most common disease that need to PAB procedure was Ventricular septal defect (VSD with twenty-eight patients (56%. Mean of extubation time (hour was 10.4 + 0.8 and mean of hospital stay (day was 13.3 + 2.4 respectively. Conclusion Although the number of pulmonary artery banding palliation surgery was decreased, but in selected group of congenital heart disease, this palliation to reduce over circulation of Pulmonary system, can use successfully with acceptable results and low mortality. We suggest pulmonary artery banding palliative surgery in these selected patients.

  16. Pulmonary thromboembolism associated with renal insufficiency due to renal artery thrombosis

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    Vučičević-Trobok Jadranka

    2002-01-01

    Full Text Available Pulmonary thromboembolism is a clinical and pathophysiological condition caused by occlusion of pulmonary arteries by thrombotic embolus. Deep venous thrombosis needn't necessarily be evident. Acute renal insufficiency is a syndrome manifested by rapid decrease or even complete urinary retention which may be due to obturation of renal arteries by a thrombus. The condition is clinically manifested by hematuria, oliguria, anuria and death due to uremia. Case Report The patient had a history of disease and a chest X-ray finding both irrelevant for establishing the working diagnosis. Blood gas analysis presented prominent hypoxemia with hyperventilation and ECG finding exhibited right heart overload, pointing to pulmonary thromboembolism. On the third day of treatment with heparin, the patient developed hematuria, oliguria and excessive elevation of nitrogen in blood as a sign of acute renal insufficiency, leading to fatal outcome. Autopsy revealed deep venous thrombosis of the left femoral vein, with massive pulmonary thromboembolism and thrombosis of both femoral arteries, inducing acute renal failure. Discussion Although the patient had a typical radiologic presentation of pulmonary thromboembolism, treatment with heparin was initiated on the basis of his blood gas analysis and ECG findings. Deep venous thrombosis was not clinically evident and acute renal failure could not be explained during patient's lifetime. Conclusion This is a rare case of acute renal failure due to a rare occurrence of bilateral acute thrombosis of renal arteries.

  17. Novel Model of Pulmonary Artery Banding Leading to Right Heart Failure in Rats

    OpenAIRE

    Masataka Hirata; Daiki Ousaka; Sadahiko Arai; Michihiro Okuyama; Suguru Tarui; Junko Kobayashi; Shingo Kasahara; Shunji Sano

    2015-01-01

    Background. Congenital heart diseases often involve chronic pressure overload of the right ventricle (RV) which is a major cause of RV dysfunction. Pulmonary artery (PA) banding has been used to produce animal models of RV dysfunction. We have devised a new and easier method of constricting the PA and compared it directly with the partial ligation method. Methods. Eight-week-old male Sprague-Dawley rats (240–260 g) were divided into three groups: sham operation, partial pulmonary artery ligat...

  18. A conservative surgical approach for management of iatrogenic pulmonary artery perforation

    Directory of Open Access Journals (Sweden)

    Monodeep Biswas

    2012-01-01

    Full Text Available Accidental malposition of a chest tube in the pulmonary artery is a potentially fatal complication. Here we describe a 66 year-old obese woman with prior cardiac transplantation, intubated for respiratory failure and felt to have a large left pleural effusion. A chest tube was inserted using a trocar tube, and resulted in near-exsanguinating hemorrhage immediately, with development of hypovolemic shock. Prompt clamping of the tube proved life-saving, and CT scan confirmed placement of the tube in the main pulmonary artery. Initial stabilization, followed by a planned surgical approach, resulted in successful removal of the tube.

  19. Successful retrieval of a knotted pulmonary artery catheter trapped in the tricuspid valve apparatus

    OpenAIRE

    Muhammad Ishaq; Nicki Alexander; David H. T. Scott

    2013-01-01

    We report the case of a 64-year-old patient in whom a pulmonary artery catheter formed a knot fixed within the right ventricle in the region of the tricuspid valve apparatus. Knot formation is a recognized complication associated with pulmonary artery catheters (PAC) insertion. This problem is usually dealt with by simply withdrawing the PAC until the knot impacts onto the introducer and after enlarging the puncture site by a small skin incision removing the introducer-PAC as one unit. Howeve...

  20. Precontrol of the pulmonary artery during thoracoscopic left upper lobectomy and systemic lymph node dissection

    Science.gov (United States)

    Ma, Lin; Mei, Jiandong; Liu, Chengwu

    2016-01-01

    The main reasons of emergent conversion during thoracoscopic lobectomy included tumor invasion, hilar lymph node (LN) metastasis, and lymphoid tuberculosis. We presented a video which depicted a three-portal thoracoscopic left upper lobectomy with dissection of large LNs. Severe adhesions between a large LN and the first branch of the left pulmonary artery (PA) were found during surgery. For safety reasons, the left main pulmonary artery (LMPA) was clamped with an endoscopic bulldog clamp prior to lymph nodal dissection. The strategy of precontrolling the LMPA during thoracoscopic lobectomy is useful for these patients with complicated hilum, and may assure the safety of the operation. PMID:27162690

  1. Is cardiac resynchronization therapy for right ventricular failure in pulmonary arterial hypertension of benefit?

    Science.gov (United States)

    Rasmussen, Jason T; Thenappan, Thenappan; Benditt, David G; Weir, E Kenneth; Pritzker, Marc R

    2014-12-01

    Pulmonary arterial hypertension is a manifestation of a group of disorders leading to pulmonary vascular remodeling and increased pulmonary pressures. The right ventricular (RV) response to chronic pressure overload consists of myocardial remodeling, which is in many ways similar to that seen in left ventricular (LV) failure. Maladaptive myocardial remodeling often leads to intraventricular and interventricular dyssychrony, an observation that has led to cardiac resynchronization therapy (CRT) for LV failure. CRT has proven to be an effective treatment strategy in subsets of patients with LV failure resulting in improvement in LV function, heart failure symptoms, and survival. Current therapy for pulmonary arterial hypertension is based on decreasing pulmonary vascular resistance, and there is currently no effective therapy targeting the right ventricle or maladaptive ventricular remodeling in these patients. This review focuses on the RV response to chronic pressure overload, its effect on electromechanical coupling and synchrony, and how lessons learned from left ventricular cardiac resynchronization might be applied as therapy for RV dysfunction in the context of pulmonary arterial hypertension.

  2. 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.

  3. Quantitative 3D pulmonary MR-perfusion in patients with pulmonary arterial hypertension: Correlation with invasive pressure measurements

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    Ley, Sebastian [Department of Pediatric Radiology, Children' s Hospital University Heidelberg, Im Neuenheimer Feld 153, 69120 Heidelberg (Germany) and Department of Radiology (E010), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg (Germany)]. E-mail: ley@gmx.net; Mereles, Derliz [Internal Medicine III, University Hospital Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg (Germany); Risse, Frank [Medical Physics in Radiology (E020), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Gruenig, Ekkehard [Internal Medicine III, University Hospital Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg (Germany); Ley-Zaporozhan, Julia [Department of Radiology (E010), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Tecer, Zueleyha [Department of Radiology (E010), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Puderbach, Michael [Department of Radiology (E010), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Fink, Christian [Department of Radiology (E010), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg (Germany); Department of Clinical Radiology, University Medical Center Grosshadern, Ludwigs-Maximilians-University, Munich (Germany); Kauczor, Hans-Ulrich [Department of Radiology (E010), German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg (Germany)

    2007-02-15

    Purpose: Pathological changes of the peripheral pulmonary arteries induce pulmonary arterial hypertension (PAH). Aim of this study was to quantitatively assess the effect of PAH on pulmonary perfusion by 3D-MR-perfusion techniques and to compare findings to healthy controls. Furthermore, quantitative perfusion data were correlated with invasive pressure measurements. Material and methods: Five volunteers and 20 PAH patients (WHO class II or III) were examined using a 1.5 T MR scanner. Measurement of pulmonary perfusion was done in an inspiratory breathhold (FLASH3D; 3.5 mm x 1.9 mm x 4 mm; TA per 3D dataset 1.5 s). Injection of contrast media (0.1 mmol Gd-DTPA/kg BW) and image acquisition were started simultaneously. Evaluation of 3D perfusion was done using singular value decomposition. Lung borders were outlined manually. Each lung volume was divided into three regions (anterior, middle, posterior), and the following parameters were assessed: Time-to-Peak (TTP), blood flow (PBF), blood volume (PBV), and mean transit time (MTT). In 10 patients invasive pulmonary artery pressure measurements were available and correlated to the perfusion measurements. Results: In both, controls and patients, an anterior-to-posterior gradient with higher PBF and PBV posterior was observed. In the posterior lung region, a significant difference (p < 0.05) was found for TTP (12 s versus 16 s) and MTT (4 s versus 6 s) between volunteers and patients. PBF and PBV were lower in patients than in volunteers (i.e. dorsal regions: 124 versus 180 ml/100 ml/min and 10 versus 12 ml/100 ml), but the difference failed to be significant. The ratio of PBF and PBV between the posterior and the middle or ventral regions showed no difference between both groups. A moderate linear correlation between mean pulmonary arterial pressure (mPAP) and PBV (r = 0.51) and MTT (r = 0.56) was found. Conclusion: The only measurable effect of PAH on pulmonary perfusion is a prolonging of the MTT. There is only a

  4. Severe stenosis of a long tracheal segment, with agenesis of the right lung and left pulmonary arterial sling.

    Science.gov (United States)

    Munro, Hamish M; Sorbello, Andrea M C; Nykanen, David G

    2006-02-01

    A baby presented at term with respiratory distress was managed with extracorporeal membrane oxygenation. Bronchoscopy revealed tracheal hypoplasia, complete tracheal rings, and agenesis of the right main bronchus. Echocardiography showed a left pulmonary arterial sling arising from the proximal part of the right pulmonary artery. Cardiac catheterization demonstrated abnormal pulmonary vasculature in the left lung which would have prevented survival, even after surgical repair. Diagnostic catheterization was important in delineating the anatomy, and aided in the decision not to proceed with surgical repair.

  5. Sodium hydrosulfide prevents hypoxia-induced pulmonary arterial hypertension in broilers.

    Science.gov (United States)

    Yang, Y; Zhang, B K; Liu, D; Nie, W; Yuan, J M; Wang, Z; Guo, Y M

    2012-01-01

    1. The aim of the study was to determine if H(2)S is involved in the development of hypoxia-induced pulmonary hypertension in broilers, a condition frequently observed in a variety of cardiac and pulmonary diseases. 2. Two-week-old broilers were reared under normoxic conditions or exposed to normobaric hypoxia (6 h/day) with tissue levels of H(2)S adjusted by administering sodium hydrosulfide (NaHS, 10 µmol/kg body weight/day). Mean pulmonary arterial pressure, right ventricular mass, plasma and tissue H(2)S levels, the expression of cystathionine-β-synthase (CSE) and vascular remodeling were determined at 35 d of age. 3. Exposure to hypoxia-induced pulmonary arterial hypertension was characterized by elevated pulmonary pressure, right ventricular hypertrophy and vascular remodeling. This was accompanied by decreased expression of CSE and decreased concentrations of plasma and tissue H(2)S. 4. Hypoxia-induced pulmonary hypertension was significantly reduced by administration of NaHS but this protective effect was largely abolished by D, L-propargylglycerine, an inhibitor of CSE. 5. The results indicate that H(2)S is involved in the development of hypoxia-induced pulmonary hypertension. Supplementing NaHS or H(2)S could be a strategy for reducing hypoxia-induced hypertension in broilers.

  6. A rapid decrease in pulmonary arterial pressure by noninvasive positive pressure ventilation in a patient with chronic obstructive pulmonary disease

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    Dursunoglu Nese

    2007-01-01

    Full Text Available The natural history of chronic obstructive pulmonary disease (COPD is characterized by progressive decrements in expiratory airflow, increments in end-expired pulmonary volume, hypoxaemia, hypercapnia and the progression of pulmonary arterial hypertension (PAH. Noninvasive positive pressure ventilation (NPPV treatment is increasingly used for the treatment of acute and chronic respiratory failure in patients with COPD. NPPV can increase PaO2 and decrease PaCO2 by correcting the gas exchange in such patients. The acute effect of NPPV on decreasing PAP is seen in patients with respiratory failure, probably due to the effect on cardiac output. Here, a case with COPD whose respiratory acidosis and PAH rapidly improved by NPPV was presented and therefore we suggested to perform an echocardiographic assessment to reveal an improvement of PAH as well as respiratory acidosis, hypercapnia and hypoxemia with that treatment.

  7. The relationship of pulmonary vascular resistance and compliance to pulmonary artery wedge pressure during submaximal exercise in healthy older adults

    Science.gov (United States)

    Wright, Stephen P.; Granton, John T.; Esfandiari, Sam; Goodman, Jack M.

    2016-01-01

    Key points A consistent inverse hyperbolic relationship has been observed between pulmonary vascular resistance and compliance, although changes in pulmonary artery wedge pressure (PAWP) may modify this relationship.This relationship predicts that pulmonary artery systolic, diastolic and mean pressure maintain a consistent relationship relative to the PAWP.We show that, in healthy exercising human adults, both pulmonary vascular resistance and compliance decrease in relation to exercise‐associated increases in PAWP.Pulmonary artery systolic, diastolic and mean pressures maintain a consistent relationship with one another, increasing linearly with increasing PAWP.Increases in PAWP in the setting of exercise are directly related to a decrease in pulmonary vascular compliance, despite small decreases in pulmonary vascular resistance, thereby increasing the pulsatile afterload to the right ventricle. Abstract The resistive and pulsatile components of right ventricular afterload (pulmonary vascular resistance, Rp; compliance, Cp) are related by an inverse hyperbolic function, expressed as their product known as RpCp‐time. The RpCp‐time exhibits a narrow range, although it may be altered by the pulmonary artery wedge pressure (PAWP). Identifying the determinants of RpCp‐time should improve our understanding of the physiological behaviour of pulmonary arterial systolic (PASP), diastolic (PADP) and mean (mPAP) pressures in response to perturbations. We examined the effect of exercise in 28 healthy non‐athletic adults (55 ± 6 years) who underwent right heart catheterization to assess haemodynamics and calculate Rp and Cp. Measurements were made at rest and during two consecutive 8–10 min stages of cycle ergometry, at targeted heart‐rates of 100 beats min–1 (Light) and 120 beats min–1 (Moderate). Cardiac output increased progressively during exercise. PASP, PADP, mPAP and PAWP increased for Light exercise, without any further rise for Moderate

  8. Use of pulmonary artery catheter in coronary artery bypass graft. Costs and long-term outcomes.

    Directory of Open Access Journals (Sweden)

    Fei Xu

    Full Text Available Pulmonary artery catheters (PAC are used widely to monitor hemodynamics in patients undergoing coronary bypass graft (CABG surgery. However, recent studies have raised concerns regarding both the effectiveness and safety of PAC. Therefore, our aim was to determine the effects of the use of PAC on the short- and long-term health and economic outcomes of patients undergoing CABG.1361 Chinese patients who consecutively underwent isolated, primary CABG at the Cardiovascular Institute of Fuwai Hospital from June 1, 2012 to December 31, 2012 were included in this study. Of all the patients, 453 received PAC during operation (PAC group and 908 received no PAC therapy (control group. Short-term and long-term mortality and major complications were analyzed with multivariate regression analysis and propensity score matched-pair analysis was used to yield two well-matched groups for further comparison.The patients who were managed with PAC more often received intraoperative vasoactive drugs dopamine (70.9% vs. 45.5%; P<0.001 and epinephrine (7.7% vs. 2.6%; P<0.001. In addition, costs for initial hospitalization were higher for PAC patients ($14,535 vs. $13,873, respectively, p = 0.004. PAC use was neither associated with the perioperative mortality or major complications, nor was it associated with long-term mortality and major adverse cardiac and cerebrovascular events. In addition, comparison between two well-matched groups showed no significant differences either in baseline characteristics or in short-term and long-term outcomes.There is no clear indication of any benefit or harm in managing CABG patients with PAC. However, use of PAC in CABG is more expensive. That is, PAC use increased costs without benefit and thus appears unjustified for routine use in CABG surgery.

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

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    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.

  10. Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension.

    Science.gov (United States)

    Lang, Irene M; Gaine, Sean P

    2015-12-01

    Pulmonary arterial hypertension (PAH) is a severe disease characterised by increased pulmonary vascular resistance, which leads to restricted pulmonary arterial blood flow and elevated pulmonary arterial pressure. In patients with PAH, pulmonary concentrations of prostacyclin, a prostanoid that targets several receptors including the IP prostacyclin receptor, are reduced. To redress this balance, epoprostenol, a synthetic prostacyclin, or analogues of prostacyclin have been given therapeutically. These therapies improve exercise capacity, functional class and haemodynamic parameters. In addition, epoprostenol improves survival among patients with PAH. Despite their therapeutic benefits, treatments that target the prostacyclin pathway are underused. One key factor is their requirement for parenteral administration: continuous intravenous administration can lead to embolism and thrombosis; subcutaneous administration is associated with infusion-site pain; and inhalation is time consuming, requiring multiple daily administrations. Nevertheless, targeting the prostacyclin pathway is an important strategy for the management of PAH. The development of oral therapies for this pathway, as well as more user-friendly delivery devices, may alleviate some of the inconveniences. Continued improvements in therapeutic options will enable more patients with PAH to receive medication targeting the prostacyclin pathway.

  11. 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.

  12. Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Irene M. Lang

    2015-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a severe disease characterised by increased pulmonary vascular resistance, which leads to restricted pulmonary arterial blood flow and elevated pulmonary arterial pressure. In patients with PAH, pulmonary concentrations of prostacyclin, a prostanoid that targets several receptors including the IP prostacyclin receptor, are reduced. To redress this balance, epoprostenol, a synthetic prostacyclin, or analogues of prostacyclin have been given therapeutically. These therapies improve exercise capacity, functional class and haemodynamic parameters. In addition, epoprostenol improves survival among patients with PAH. Despite their therapeutic benefits, treatments that target the prostacyclin pathway are underused. One key factor is their requirement for parenteral administration: continuous intravenous administration can lead to embolism and thrombosis; subcutaneous administration is associated with infusion-site pain; and inhalation is time consuming, requiring multiple daily administrations. Nevertheless, targeting the prostacyclin pathway is an important strategy for the management of PAH. The development of oral therapies for this pathway, as well as more user-friendly delivery devices, may alleviate some of the inconveniences. Continued improvements in therapeutic options will enable more patients with PAH to receive medication targeting the prostacyclin pathway.

  13. Bone morphogenetic protein receptor II regulates pulmonary artery endothelial cell barrier function.

    Science.gov (United States)

    Burton, Victoria J; Ciuclan, Loredana I; Holmes, Alan M; Rodman, David M; Walker, Christoph; Budd, David C

    2011-01-06

    Mutations in bone morphogenetic protein receptor II (BMPR-II) underlie most heritable cases of pulmonary arterial hypertension (PAH). However, less than half the individuals who harbor mutations develop the disease. Interestingly, heterozygous null BMPR-II mice fail to develop PAH unless an additional inflammatory insult is applied, suggesting that BMPR-II plays a fundamental role in dampening inflammatory signals in the pulmonary vasculature. Using static- and flow-based in vitro systems, we demonstrate that BMPR-II maintains the barrier function of the pulmonary artery endothelial monolayer suppressing leukocyte transmigration. Similar findings were also observed in vivo using a murine model with loss of endothelial BMPR-II expression. In vitro, the enhanced transmigration of leukocytes after tumor necrosis factor α or transforming growth factor β1 stimulation was CXCR2 dependent. Our data define how loss of BMPR-II in the endothelial layer of the pulmonary vasculature could lead to a heightened susceptibility to inflammation by promoting the extravasation of leukocytes into the pulmonary artery wall. We speculate that this may be a key mechanism involved in the initiation of the disease in heritable PAH that results from defects in BMPR-II expression.

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

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    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. Endobronchial mucosal blanching due to a post-lung transplantation pulmonary artery stenosis.

    Science.gov (United States)

    Slebos, Dirk-Jan; Wolff, Rienhart F E; van der Bij, Wim

    2011-03-01

    A 45-year-old woman underwent a bronchoscopy shortly after lung transplantation. The airway mucosal appearance significantly differed between both lungs, with a pale aspect of the left bronchial tree. Computed tomography (CT) and perfusion scan confirmed a left pulmonary artery stenosis, improving with conservative treatment.

  16. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease

    NARCIS (Netherlands)

    Zijlstra, Willemijn M H; Douwes, Johannes M; Ploegstra, Mark-Jan; Krishnan, Usha; Roofthooft, Marcel; Hillege, Hans L; Ivy, D Dunbar; Rosenzweig, Erika B; Berger, Rolf M F

    2016-01-01

    Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore

  17. 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

  18. Pulmonary artery location during microgravity activity: Potential impact for chest-mounted Doppler during space travel

    Science.gov (United States)

    Hadley, A. T., III; Conkin, J.; Waligora, J. M.; Horrigan, D. J., Jr.

    1984-01-01

    Doppler, or ultrasonic, monitoring for pain manifestations of decompression sickness (the bends) is accomplished by placing a sensor on the chest over the pulmonary artery and listening for bubbles. Difficulties have arisen because the technician notes that the pulmonary artery seems to move with subject movement in a one-g field and because the sensor output is influenced by only slight degrees of sensor movement. This study used two subjects and mapped the position of the pulmonary artery in one-g, microgravity, and two-g environments using ultrasound. The results showed that the pulmonary artery is fixed in location in microgravity and not affected by subject position change. The optimal position corresponded to where the Doppler signal is best heard with the subject in a supine position in a one-g environment. The impact of this result is that a proposed multiple sensor array on the chest proposed for microgravity use may not be necessary to monitor an astronaut during extravehicular activities. Instead, a single sensor of approximately 1 inch diameter and mounted in the position described above may suffice.

  19. Outcome of Pediatric Patients With Pulmonary Arterial Hypertension in the Era of New Medical Therapies

    NARCIS (Netherlands)

    van Loon, Rosa Laura E.; Roofthooft, Marcus T. R.; Delhaas, Tammo; van Osch-Gevers, Magdalena; ten Harkel, Arend D. J.; Strengers, Jan L. M.; Backx, Ad; Hillege, Hans L.; Berger, Rolf M. F.

    2010-01-01

    Little is known about the effects of "second-generation drugs" (prostanoids, endothelin receptor antagonists, 5-phosphodiesterase inhibitors) in children with pulmonary arterial hypertension (PAH). This study describes the outcome of a national cohort of children with PAH in an era when these drugs

  20. Growth in children with pulmonary arterial hypertension : a longitudinal retrospective multiregistry study

    NARCIS (Netherlands)

    Ploegstra, Mark-Jan; Ivy, D Dunbar; Wheeler, Jeremy G; Brand, Monika; Beghetti, Maurice; Rosenzweig, Erika B; Humpl, Tilman; Iriart, Xavier; Rouzic, Erwan Muros-Le; Bonnet, Damien; Berger, Rolf M F

    2016-01-01

    BACKGROUND: To enable adequate interpretation of growth measurements in the management of children with pulmonary arterial hypertension (PAH), we assessed growth and its associated determinants in children with PAH. METHODS: We did a retrospective longitudinal study of height and body-mass index in

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

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    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.

  2. “Anomalous right pulmonary artery left atrial fistula”: Growth in vain

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    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.

  3. Rare Anomalous Origin of Superior Left Pulmonary Artery from Left Subclavian Vein

    Energy Technology Data Exchange (ETDEWEB)

    Lv, Tian-shi, E-mail: TerrenceLv@126.com; Wang, Chao, E-mail: wangchaoxs@163.com; Song, Li, E-mail: song9981@163.com; Lv, Yong-xing, E-mail: lyongxing@msn.com; Zou, Ying-hua, E-mail: yinghzou@139.com [Peking University First Hospital, Department of Interventional Radiology and Vascular Surgery (China)

    2013-10-15

    We report for the first time an extremely rare anomalous origin of the superior left pulmonary artery in a 60 year-old man. Although it was occult in clinical indications, such a malformation still ought to be considered, especially during endovascular procedures.

  4. Left Pulmonary Artery Thrombosis in a Neonate with Left Lung Hypoplasia

    Directory of Open Access Journals (Sweden)

    Matthias P. van Schendel

    2012-01-01

    Full Text Available Thrombotic events in neonates may origin from fetal life. A 4-day-old newborn infant with a family history of heterozygous type 1 protein C deficiency was diagnosed with left lung hypoplasia and left pulmonary artery thrombosis. Its source was prenatally closed ductus arteriosus. Surgical removal of the thrombus was performed.

  5. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study

    NARCIS (Netherlands)

    Coghlan, J.G.; Denton, C.P.; Grunig, E.; Bonderman, D.; Distler, O.; Khanna, D.; Muller-Ladner, U.; Pope, J.E.; Vonk, M.C.; Doelberg, M.; Chadha-Boreham, H.; Heinzl, H.; Rosenberg, D.M.; McLaughlin, V.V.; Seibold, J.R.

    2014-01-01

    OBJECTIVE: Earlier detection of pulmonary arterial hypertension (PAH), a leading cause of death in systemic sclerosis (SSc), facilitates earlier treatment. The objective of this study was to develop the first evidence-based detection algorithm for PAH in SSc. METHODS: In this cross-sectional, intern

  6. Role of curcumin in idiopathic pulmonary arterial hypertension treatment: a new therapeutic possibility.

    Science.gov (United States)

    Bronte, Enrico; Coppola, Giuseppe; Di Miceli, Riccardo; Sucato, Vincenzo; Russo, Antonio; Novo, Salvatore

    2013-11-01

    The idiopathic pulmonary arterial hypertension is a complex disease that mainly affects pulmonary arterial circulation. This undergoes a remodeling with subsequent reduction of flow in the small pulmonary arteries. Because of this damage an increased vascular resistance gradually develops, and over time it carries out in heart failure. The inflammatory process is a key element in this condition, mediated by various cytokines. The inflammatory signal induces activation of NF-κB, and prompts TGF-β-related signaling pathway. Clinical evolution leads to progressive debilitation, greatly affecting the patient quality of life. The actual therapeutic approaches, are few and expensive, and include systemic drugs such as prostanoids, phosphodiesterase inhibitors and antagonists of endothelin-1 (ERBs). Some researchers have long investigated the anti-inflammatory effects of curcumin. It shows a role for inactivation of NF-κB-mediated inflammation. On the basis of these findings we propose a potential role of curcumin and its pharmacologically fit derivatives for treatment of idiopathic pulmonary arterial hypertension.

  7. Association between airway obstruction and peripheral arterial stiffness in elderly patients with chronic obstructive pulmonary disease

    Institute of Scientific and Technical Information of China (English)

    付志方

    2014-01-01

    Objective To evaluate the relationship between se-verity of airway obstruction and peripheral arterial stiffness in patients with chronic obstructive pulmonary disease(COPD).Methods 81 COPD patients[aged(78.32±6.98)yrs,73 males,8 females]from Jan2008 to Oct 2012 were enrolled in Geriatric Department

  8. Transforming growth factor-beta receptor mutations and pulmonary arterial hypertension in childhood

    NARCIS (Netherlands)

    Harrison, RE; Berger, R; Haworth, SG; Tulloh, R; Mache, CJ; Morrell, NW; Aldred, MA; Trembath, RC

    2005-01-01

    BACKGROUND: Pulmonary arterial hypertension (PAH) is a potentially fatal vasculopathy that can develop at any age. Adult-onset disease has previously been associated with mutations in BMPR2 and ALK-1. Presentation in early life may be associated with congenital heart disease but frequently is idiopa

  9. Pulmonary arterial hypertension in congenital heart disease : An epidemiologic perspective from a Dutch registry

    NARCIS (Netherlands)

    Duffels, M. G. J.; Engelfriet, P. M.; Berger, R. M. F.; van Loon, R. L. E.; Hoendermis, E.; Vriend, J. W. J.; Bresser, P.; Mulder, B. J. M.; van der Velde, Enno T.

    2007-01-01

    Background: Pulmonary arterial hypertension (PAH) associated with congenital heart disease is usually the result of a large systemic-topulmonary shunt, and often leads to right ventricular failure and early death. The purpose of this study was to determine the prevalence of PAH among adult patients

  10. Egr-1 identifies neointimal remodeling and relates to progression in human pulmonary arterial hypertension

    NARCIS (Netherlands)

    van der Feen, Diederik E; Dickinson, Michael G; Bartelds, Beatrijs; Borgdorff, Marinus A J; Sietsma, Hannie; Lévy, Marilyne; Berger, Rolf M F

    2015-01-01

    BACKGROUND: Pulmonary arterial hypertension (PAH) is hallmarked by the development of neointimal lesions. The transcription factor Egr-1 seems to play a critical role in neointimal formation in experimental PAH and was identified as a putative target for intervention. In this study we investigated w

  11. Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification

    NARCIS (Netherlands)

    Riel, A.C. van; Schuuring, M.J.; Hessen, I.D. van; Zwinderman, A.H.; Cozijnsen, L.; Reichert, C.L.; Hoorntje, J.C.A.; Wagenaar, L.J.; Post, M.C.; Dijk, A.P.J. van; Hoendermis, E.S.; Mulder, B.J.; Bouma, B.J.

    2014-01-01

    BACKGROUND: The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigat

  12. Lobectomy with Bronchoplasty and Reconstruction of Pulmonary Artery by Minitrauma-technique for Lung Cancer

    Directory of Open Access Journals (Sweden)

    Zhongmin PENG

    2009-11-01

    Full Text Available Background and objective To research the effect and practicalbility of lobectomy with bronchoplasty and reconstruction of pulmonary artery by minitrauma-technique for lung cancer. Methods We retrospectibely reviewed our experience on 61 cases being lobectomy with bronchoplasty and bronchoplasty with or without video assisted thoracic small incision surgery for lung cancer from July 2005 to June 2009 from Shandong Provincal Hospital and 46 cases simultaneously by routine posterolateral incision. All patients whose bronchus and/or pulmonary artery were involved underwent the operation and experienced the bronchial sleeve/wedge resection or reconstruction of the pulmonary artery. Results All patients were done operation successfully and there were no operative mortality and no occurrence of anastomosis stenosis as well as fistula. The small incisions’ length was from 8 cm-15 cm while the routine posterolateral incision’s length was 25 cm-35 cm. The patients done the operation of small incision had less postoperative shoulder joint dysfunction and had better quality of life compaired to the patients done the routine posterolateral incision. Conclusion Lobectomy with bronchoplasty and reconstruction of pulmonary artery by minitrauma-technique for lung cancer could finished the same work with the traditional thoracic lateral incision and had less trauma, less pain, less recovery time.

  13. Robust extraction of the aorta and pulmonary artery from 3D MDCT image data

    Science.gov (United States)

    Taeprasartsit, Pinyo; Higgins, William E.

    2010-03-01

    Accurate definition of the aorta and pulmonary artery from three-dimensional (3D) multi-detector CT (MDCT) images is important for pulmonary applications. This work presents robust methods for defining the aorta and pulmonary artery in the central chest. The methods work on both contrast enhanced and no-contrast 3D MDCT image data. The automatic methods use a common approach employing model fitting and selection and adaptive refinement. During the occasional event that more precise vascular extraction is desired or the method fails, we also have an alternate semi-automatic fail-safe method. The semi-automatic method extracts the vasculature by extending the medial axes into a user-guided direction. A ground-truth study over a series of 40 human 3D MDCT images demonstrates the efficacy, accuracy, robustness, and efficiency of the methods.

  14. Isolated Hypoplasia of Left Pulmonary Artery with Agenesis of Left Lobe of Thyroid: A Case Report.

    Science.gov (United States)

    Khadir, Mohammed Abdul; Narayana, Ganesh; Ramagopal, Ganavi; Nayar, Pradeep G

    2016-12-01

    Isolated Unilateral hypoplasia or agenesis of a branch of pulmonary artery is very rare. It is usually seen associated with congenital heart diseases such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia. It occurs as a result of lack of embryological development of either the left or right sixth aortic arch and has been found to present itself with various clinical manifestations as during childhood it presents as contralateral pulmonary hypertension and in adults as haemoptysis. Early diagnosis and early surgical indication avoids the evolution of pulmonary hypertension to unfavourble state of more severe and progressive degrees and also prevents the development of pulmonary systemic collateral circulation, which is mainly responsible for subsequent haemoptysis in the adulthood. We hereby, report the case of an infant who presented with features of lower respiratory tract infection and later diagnosed as isolated congenital hypoplasia of left pulmonary artery and hence planned for proper follow-up for early surgery thereby preventing complications in the future.

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

    Science.gov (United States)

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

    2015-12-01

    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 arterial hypertension is associated with a markedly increased morbidity and mortality. It is important to identify patients with a poor prognosis using disease specific markers. Echocardiography and biomarkers arise as practical tools to determine the risk of mortality. Although pulmonary arterial hypertension cannot be cured, four classes of disease-targeting therapies are currently available and several promising therapies are being studied. There is a shift in drug studies towards more clinically relevant endpoints such as time to clinical worsening and morbidity and mortality events.

  16. Pulmonary artery dissection causing haemothorax in a cat: potential role of Dirofilaria immitis infection and literature review.

    Science.gov (United States)

    Biasato, I; Tursi, M; Zanet, S; Longato, E; Capucchio, M T

    2017-02-01

    A 7-year-old male castrated domestic short-haired cat suddenly died. Gross examination revealed severe right-sided haemothorax with blood clots, four adult filarial nematodes in the blood clots and the caudal vena cava and haemorrhage dissecting into the tunica media of the right pulmonary artery. Histopathological investigation showed fibrosis of the tunica intima and disorganization/fragmentation of the elastic fibres accompanied by fibrous tissue deposition in the tunica media of both branches of pulmonary artery. Degenerative vasculopathy (intimal fibromuscular hyperplasia and medial hypertrophy/hyperplasia) involving pulmonary arteries was also observed. The polymerase chain reaction amplification and sequencing confirmed the identification of the parasite as Dirofilaria immitis. A diagnosis of pulmonary artery dissection with haemothorax and concomitant heartworm disease was formulated. Degenerative processes of the tunica media have been reported to cause pulmonary artery dissection in both humans and animals. Pulmonary artery remodelling induced by heartworms may be considered the underlying cause in the first case of feline pulmonary artery dissection, herein described.

  17. Coronary Artery Calcification Is Often Unreported in CT Pulmonary Angiograms in Patients With Suspected Pulmonary Embolism: An Opportunity to Improve Diagnosis of Acute Coronary Syndrome

    OpenAIRE

    Johnson, Patrick Connor

    2015-01-01

    Objective: In patients with suspected pulmonary thromboembolism (PTE), coronary artery calcification (CAC) can be an incidental finding in CT pulmonary angiograms. We evaluated the frequency of unreported CAC and its association with diagnosis of acute coronary syndrome (ACS). Methods: The data of 469 consecutive patients who were referred to the emergency radiology department for CT pulmonary angiography because of suspicion for PTE were reviewed. Radiology reports were rechecked, and pos...

  18. In situ expression of Bcl-2 in pulmonary artery endothelial cells associates with pulmonary arterial hypertension relative to heart failure with preserved ejection fraction

    Science.gov (United States)

    Benza, Raymond L.; Williams, Gretchen; Wu, Changgong; Shields, Kelly J.; Raina, Amresh; Murali, Srinivas

    2016-01-01

    Abstract We have previously reported that pulmonary artery endothelial cells (PAECs) can be harvested from the tips of discarded Swan-Ganz catheters after right heart catheterization (RHC). In this study, we tested the hypothesis that the existence of an antiapoptotic phenotype in PAECs obtained during RHC is a distinctive feature of pulmonary arterial hypertension (PAH; World Health Organization group 1) and might be used to differentiate PAH from other etiologies of pulmonary hypertension. Specifically, we developed a flow cytometry-based measure of Bcl-2 activity, referred to as the normalized endothelial Bcl-2 index (NEBI). We report that higher NEBI values are associated with PAH to the exclusion of heart failure with preserved ejection fraction (HFpEF) and that this simple diagnostic measurement is capable of differentiating PAH from HFpEF without presenting addition risk to the patient. If validated in a larger, multicenter study, the NEBI has the potential to assist physicians in the selection of appropriate therapeutic interventions in the common and dangerous scenario wherein patients present a clinical and hemodynamic phenotype that makes it difficult to confidently differentiate between PAH and HFpEF. PMID:28090298

  19. A loud right-apical systolic murmur is associated with the diagnosis of secondary pulmonary arterial hypertension: retrospective analysis of data from 201 consecutive client-owned dogs (2006-2007).

    Science.gov (United States)

    Ohad, D G; Lenchner, I; Bdolah-Abram, T; Segev, G

    2013-12-01

    Canine pulmonary arterial hypertension (PAH) remains under-recognized and under-treated despite being prevalent. This retrospective study investigated whether selected historical and physical examination findings were associated with the diagnosis of canine PAH, defined as tricuspid regurgitation (TR) with a confirmed systolic pressure gradient ≥ 35 mm Hg. Two hundred and one client-owned dogs (PAH group, n=96; control group, n=105) were studied. Dogs in the control group had TR with a confirmed systolic gradient dogs underwent a complete physical examination and a complete echocardiographic assessment. A loud systolic right-apical murmur (RAM) was significantly associated with TR ≥ 35 mm Hg. The proportion of dogs with PAH significantly increased as the RAM grade increased, with odds ratios of 4.4-37.6 for Grades 3/6-5/6 (P=0.004 to dogs with degenerative valve disease is highly suggestive of concurrent PAH.

  20. Exhaled and arterial levels of endothelin-1 are increased and correlate with pulmonary systolic pressure in COPD with pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    Dragonieri Silvano

    2008-09-01

    Full Text Available Abstract Background Endothelin-1 (ET-1 and Nitric Oxide (NO are crucial mediators for establishing pulmonary artery hypertension (PAH. We tested the hypothesis that their imbalance might also occur in COPD patients with PAH. Methods The aims of the study were to measure exhaled breath condensate (EBC and circulating levels of ET-1, as well as exhaled NO (FENO levels by, respectively, a specific enzyme immunoassay kit, and by chemiluminescence analysis in 3 groups of subjects: COPD with PAH (12, COPD only (36, and healthy individuals (15. In order to evaluate pulmonary-artery systolic pressure (PaPs, all COPD patients underwent Echo-Doppler assessment. Results Significantly increased exhaled and circulating levels of ET-1 were found in COPD with PAH compared to both COPD (p 1%, (r = -0.59, p = 0.043, and PaPs negatively correlated to PaO2 (r = -0.618; p = 0.032. Significantly reduced levels of FENO were found in COPD associated with PAH, compared to COPD only (22.92 ± 11.38 vs.35.07 ± 17.53 ppb; p = 0.03. Thus, we observed an imbalanced output in the breath between ET-1 and NO, as expression of pulmonary endothelium and epithelium impairment, in COPD with PAH compared to COPD only. Whether this imbalance is an early cause or result of PAH due to COPD is still unknown and deserves further investigations.

  1. [Treatment for pulmonary arterial hypertension under the new French hospital financing system. Recommendations of the Pulmonary Vascular Diseases Working Group of the French Society of Pulmonary Medicine].

    Science.gov (United States)

    Sitbon, O; Humbert, M; Simonneau, G

    2005-11-05

    Activity-based financing (that is, casemix-based hospital payments, known as T2A) is intended to harmonize and improve the fairness of remuneration of public and private hospitals. T2A will ultimately rely mainly on a flat rate per admission, set according to the diagnosis-related group (DRG). Although payment for drugs is usually included in the DRG price, some expensive drugs will be reimbursed on an additional cost basis after implementation of a "best practices" agreement. Four drugs used for treatment of pulmonary arterial hypertension are eligible for this additional reimbursement: 3 prostacyclin derivatives (intravenous epoprostenol, inhaled iloprost, and subcutaneous treprostinil), and oral bosentan, an endothelin receptor antagonist. The Pulmonary Vascular Diseases working group of the French Society of Pulmonary Medicine has developed guidelines for the best practices in use of these drugs.

  2. The effect of ACE inhibition on the pulmonary vasculature in combined model of chronic hypoxia and pulmonary arterial banding in Sprague Dawley rats

    Science.gov (United States)

    Clarke, Shanelle; Baumgardt, Shelley; Molthen, Robert

    2010-03-01

    Microfocal CT was used to image the pulmonary arterial (PA) tree in rodent models of pulmonary hypertension (PH). CT images were used to measure the arterial tree diameter along the main arterial trunk at several hydrostatic intravascular pressures and calculate distensibility. High-resolution planar angiographic imaging was also used to examine distal PA microstructure. Data on pulmonary artery tree morphology improves our understanding of vascular remodeling and response to treatments. Angiotensin II (ATII) has been identified as a mediator of vasoconstriction and proliferative mitotic function. ATII has been shown to promote vascular smooth muscle cell hypertrophy and hyperplasia as well as stimulate synthesis of extracellular matrix proteins. Available ATII is targeted through angiotensin converting enzyme inhibitors (ACEIs), a method that has been used in animal models of PH to attenuate vascular remodeling and decrease pulmonary vascular resistance. In this study, we used rat models of chronic hypoxia to induce PH combined with partial left pulmonary artery occlusion (arterial banding, PLPAO) to evaluate effects of the ACEI, captopril, on pulmonary vascular hemodynamic and morphology. Male Sprague Dawley rats were placed in hypoxia (FiO2 0.1), with one group having underwent PLPAO three days prior to the chronic hypoxia. After the twenty-first day of hypoxia exposure, treatment was started with captopril (20 mg/kg/day) for an additional twenty-one days. At the endpoint, lungs were excised and isolated to examine: pulmonary vascular resistance, ACE activity, pulmonary vessel morphology and biomechanics. Hematocrit and RV/LV+septum ratio was also measured. CT planar images showed less vessel dropout in rats treated with captopril versus the non-treatment lungs. Distensibility data shows no change in rats treated with captopril in both chronic hypoxia (CH) and CH with PLPAO (CH+PLPAO) models. Hemodynamic measurements also show no change in the pulmonary vascular

  3. Constitutively active form of natriuretic peptide receptor 2 ameliorates experimental pulmonary arterial hypertension.

    Science.gov (United States)

    Nawa, Nobutoshi; Ishida, Hidekazu; Katsuragi, Shinichi; Baden, Hiroki; Takahashi, Kunihiko; Higeno, Ryota; Torigoe, Fumiko; Mihara, Seiko; Narita, Jun; Miura, Kohji; Nakamura, Kazufumi; Kogaki, Shigetoyo; Ozono, Keiichi

    2016-01-01

    We recently found a constitutively active mutant of natriuretic peptide receptor 2 (caNPR2; V883M), which synthesizes larger amounts of cyclic guanosine monophosphate (cGMP) intracellularly without any ligand stimulation than existing drugs. The aim of this study was to investigate the therapeutic effects of gene transduction using caNPR2 for pulmonary arterial hypertension (PAH). In vitro gene transduction into human pulmonary arterial smooth muscle cells using Sendai virus (SeV) vectors carrying caNPR2 induced 10,000-fold increases in the synthesis of cGMP without ligand stimulation, and the proliferation of caNPR2-expressing cells was significantly attenuated. The PAH model rats generated by hypoxia and the administration of SU5416 were then treated with SeV vectors through a direct injection into the left pulmonary artery. Right ventricular systolic pressure was significantly decreased 2 weeks after the treatment, while systemic blood pressure remained unchanged. Histological analyses revealed that the medial wall thickness and occlusion rate of pulmonary arterioles were significantly improved in caNPR2-treated lungs. Neither the systemic integration of virus vectors nor side effects were observed. The massive stimulation of cGMP synthesis by gene therapy with caNPR2 was safe and effective in a PAH rat model and, thus, has potential as a novel therapy for patients with severe progressive PAH.

  4. Clinical utility of tadalafil in the treatment of pulmonary arterial hypertension: an evidence-based review

    Directory of Open Access Journals (Sweden)

    Henrie AM

    2015-11-01

    Full Text Available Adam M Henrie, James J Nawarskas, Joe R Anderson College of Pharmacy, University of New Mexico, Albuquerque, NM, USA Abstract: Pulmonary arterial hypertension (PAH is a chronic and disabling condition characterized by an elevated pulmonary vascular resistance and an elevated mean pulmonary arterial pressure. Despite recent improvements in treatment availability, PAH remains challenging to treat, burdensome for patients, and ultimately incurable. Tadalafil is a phosphodiesterase-5 inhibitor that is administered once daily by mouth for the treatment of PAH. Current treatment guidelines recommend tadalafil as an option for patients with World Health Organization functional class II or III PAH. In a placebo-controlled clinical trial, patients taking tadalafil demonstrated significantly improved exercise capacity as measured by the 6-minute walk distance. Patients also experienced decreased incidence of clinical worsening, increased quality of life, and improved cardiopulmonary hemodynamics. Uncontrolled studies and smaller trials have indicated a possible role for tadalafil as a suitable alternative to sildenafil and as a beneficial add-on option when used in combination with other treatments for PAH. Tadalafil is generally safe and well tolerated. Adverse events are typically mild-to-moderate in intensity, and discontinuation rates are usually low. The purpose of this review is to provide an evidence-based evaluation of the clinical utility of tadalafil in the treatment of PAH. Keywords: tadalafil, phosphodiesterase-5 inhibitor, pulmonary arterial hypertension

  5. Pulmonary Interstitial Glycogenosis: A Reversible Underlying Condition Associated With D-Transposition of the Great Arteries and Severe Persistent Pulmonary Hypertension.

    Science.gov (United States)

    Sanchez-de-Toledo, Joan; González-Peris, Sebastià; Gran, Ferran; Gregoraci, Angela; Ferreres, Joan Carles; Ruiz, Cèsar W; Balcells, Joan; Abella, Raul F

    2015-07-01

    Transposition of the great arteries with intact ventricular septum and persistent pulmonary hypertension (TGA-IVS PPHN) is a rare association with a poor prognosis. We report the case of a term newborn with TGA-IVS PPHN successfully managed with perioperative extracorporeal membrane oxygenation (ECMO) and aggressive pulmonary vasodilation therapy that underwent successful arterial switch procedure. A lung biopsy obtained during the surgical procedure showed pulmonary interstitial glycogenosis, a reversible condition. Concerns over left ventricle deconditioning after ECMO could be minimized with appropriate management and monitoring of the ductus arteriosus and appropriate timing of surgery.

  6. Computed tomography angiography with pulmonary artery thrombus burden and right-to-left ventricular diameter ratio after pulmonary embolism.

    Science.gov (United States)

    Ouriel, Kenneth; Ouriel, Richard L; Lim, Yeun J; Piazza, Gregory; Goldhaber, Samuel Z

    2017-02-01

    Purpose Computed tomography angiography is used for quantifying the significance of pulmonary embolism, but its reliability has not been well defined. Methods The study cohort comprised 10 patients randomly selected from a 150-patient prospective trial of ultrasound-facilitated fibrinolysis for acute pulmonary embolism. Four reviewers independently evaluated the right-to-left ventricular diameter ratios using the standard multiplanar reformatted technique and a simplified (axial) method, and thrombus burden with the standard modified Miller score and a new, refined Miller scoring system. Results The intraclass correlation coefficient for intra-observer variability was .949 and .970 for the multiplanar reformatted and axial methods for estimating right-to-left ventricular ratios, respectively. Inter-observer agreement was high and similar for the two methods, with intraclass correlation coefficient of .969 and .976. The modified Miller score had good intra-observer agreement (intraclass correlation coefficient .820) and was similar to the refined Miller method (intraclass correlation coefficient .883) for estimating thrombus burden. Inter-observer agreement was also comparable between the techniques, with intraclass correlation coefficient of .829 and .914 for the modified Miller and refined Miller methods. Conclusions The reliability of computed tomography angiography for pulmonary embolism was excellent for the axial and multiplanar reformatted methods for quantifying the right-to-left ventricular ratio and for the modified Miller and refined Miller scores for quantifying of pulmonary artery thrombus burden.

  7. Effect of peroxynitrite on reactivity of rabbit pulmonary artery in vitro

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    In order to investigate the possible role of ONOO- in regulatory disorder of pulmonary arterial tension in endotoxic shock, the responses of rabbit pulmonary arterial rings (PARs) preincubated with ONOO- to endothelial dependent and receptor dependent relaxants acetylcholine (ACh) and adenosine diphosphate (ADP), endothelial dependent and receptor independent relaxant A23187, endothelial independent relaxant sodium nitroprusside (SNP) and α1-adrenoceptor agonist phenylephrine (PE) were observed in vitro in accumulative manner. Results were as follow: (1) Relaxations of PARs to ACh, A23187 and ADP were markedly impaired with shift of accumulative dose response curve of each agonist to the right. Inhibition of endothelial dependent and receptor dependent or independent relaxation by ONOO- was dose dependent. (2) ONOO- incubation inhibited SNP-induced relaxation in a dose dependent manner. Accumulative dose response curve of SNP was right shift to some degree depending on the doses of ONOO-. (3) Contractile response of PARs to PE varied with the different doses of ONOO-. In PARs preincubated with 0.5 mmol/L ONOO-, contractile reponse was significantly enhanced with shift of PE accumulative dose response curve to the left, while in PARs preincubated with 1.0 mmol/L or 2.0 mmol/L ONOO-, it was markedly reduced with right shift of PE accumulative dose response curve. (4) Vehicle of ONOO- had no effect on responses to every agonist, whereas decomposed ONOO- had minimal effect on the response to PE and ADP. In contrast, relaxation of PARs to ACh, A23187 and SNP were enhanced. These results suggested that direct effect of ONOO- on pulmonary artery may be a key factor contributing to regulatory disorder of pulmonary arterial tension induced by LPS and pulmonary hypertension in the early stage of endotoxic shock.

  8. Controlled Release Inhalable Polymeric Microspheres for Treatment of Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Saigal, Aparna; Ng, Wai Kiong; Tan, Reginald B H; Chan, Sui Yung

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a chronic ailment of the lungs, exhibiting elevated arterial pressure and vascular resistance; with a mean arterial pressure above 25 mmHg at rest and above 30 mmHg during exercise. It is associated with poor prognosis, and its prevalence is estimated to be 15 cases per one million. The current treatment options for PAH are discussed with the prostanoid class of drugs being the most effective. The latter drugs act by dilating systemic and pulmonary arterial vascular beds and, with sustained long-term usage, altering pulmonary remodelling. They are administered as IV infusions or inhalation solutions. Despite their clinical effectiveness, prostanoids have short half-lives requiring frequent administration of 6-9 times daily and thus suffer from poor compliance. Controlled release inhalation delivery systems for treatment of PAH, ranging from liposomes, biodegradable nano- and microparticles, formation of co-precipitates and complexation with cyclodextrins, are explored. Arising from these formulation strategies, we developed novel polymeric microspheres for inhalation to reduce dosing frequency and improve medication compliance. These microspheres are designed with release modifiers, to reside in the lung which is the site of drug action for a longer duration so as to release the drug slowly and consistently over a prolonged period. This could lead to the development of the first commercially available controlled release inhalation product.

  9. Diseases of the thoracic aorta and pulmonary arteries

    NARCIS (Netherlands)

    Salgado, R.; Habets, J.; Budde, R. P J; Leiner, T.

    2015-01-01

    Many acute and chronic clinical entities can affect the great thoracic arteries. As clinical findings are often unreliable, (mostly) noninvasive imaging plays a crucial role both in the initial assessment of critically ill patients, and in the follow-up of chronic conditions. Imaging also forms an i

  10. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies.

    Science.gov (United States)

    Tselios, Konstantinos; Gladman, Dafna D; Urowitz, Murray B

    2017-01-01

    Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud's phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.

  11. 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

  12. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

    Science.gov (United States)

    Tselios, Konstantinos; Gladman, Dafna D; Urowitz, Murray B

    2017-01-01

    Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively. PMID:28053559

  13. A review of sitaxsentan sodium in patients with pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Aaron B Waxman

    2007-03-01

    Full Text Available Aaron B WaxmanDepartment of Internal Medicine, Pulmonary Critical Care Unit, Harvard Medical School, Massachusetts General Hospital, Boston, MA, USAAbstract: Pulmonary arterial hypertension (PAH is a life threatening, progressive condition which eventually leads to fatal right heart failure. Endothelin-1 (ET-1, a potent vasoconstrictor peptide, is increased in the pulmonary arteries of patients with pulmonary hypertension. Endothelin-1 acts through the stimulation of 2 subtypes of receptors (endothelin receptor subtypes A [ETA] and B [ETB]. In PAH patients, ETRAs block the deleterious vasoconstrictor effects of ET-1, and ETRA treatment in PAH patients has been shown to be safe and efficacious. Sitaxsentan is an orally active, highly ETA selective ETRA that, in clinical trials, has demonstrated improvements in exercise capacity, functional class and hemodynamics in PAH patients. Sitaxsentan has been shown to be safe, well tolerated, and associated with a lower incidence of liver toxicity than other approved ETRAs.Keywords: endothelin receptor antagonist, endothelin receptor inhibitor, endothelin A, sitaxsentan, pulmonary hypertension, endothelin

  14. Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

    Directory of Open Access Journals (Sweden)

    Tselios K

    2016-12-01

    Full Text Available Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz, University of Toronto Lupus Clinic, Centre for Prognosis Studies in the Rheumatic Diseases, University Health Network, Toronto, ON, Canada Abstract: Systemic lupus erythematosus (SLE is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH, after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg with normal pulmonary capillary wedge pressure (≤15 mmHg and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan, phosphodiesterase type 5 inhibitors (sildenafil, and vasodilators (epoprostenol. Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively. Keywords: systemic lupus erythematosus, pulmonary arterial hypertension, immunosuppressive, transthoracic echocardiogram, endothelin receptor antagonists

  15. Effects of terlipressin on systolic pulmonary artery pressure of patients with liver cirrhosis:An echocardiographic assessment

    Institute of Scientific and Technical Information of China (English)

    Engin Altintas; Necdet Akkus; Ramazan Gen; M. Rami Helvaci; Orhan Sezgin; Dilek Oguz

    2004-01-01

    AIM: Portopulmonary hypertension is a serious complication of chronic liver disease. Our aim was to search into the effect of terlipressin on systolic pulmonary artery pressure among cirrhotic patients.METHODS: Twelve patients (6 males and 6 females) with liver cirrhosis were recruited in the study. Arterial blood gas samples were obtained in sitting position at rest. Contrast enhanced echocardiography and measurements of systolic pulmonary artery pressure were performed before and after the intravenous injection of 2 mg terlipressin.RESULTS: Of 12 patients studied, the contrast enhanced echocardiography was positive in 5, and the positive findings in contrast enhanced echocardiography were reversed to normal in two after terlipressin injection. The mean systolic pulmonary artery pressure was 25.5±3.6 mmHg before terlipressin injection, and was 22.5±2.5 mmHg after terlipressin (P=0.003). The systolic pulmonary artery pressure was above 25 mmHg in seven of these 12 patients.After the terlipressin injection, systolic pulmonary artery pressure was <25 mmHg in four of these cases (58.3% vs 25%, P=0.04).CONCLUSION: Terlipressin can decrease the systolic pulmonary artery pressure in patients with liver cirrhosis.

  16. 超声引导下犬自体血栓栓塞性急性肺动脉高压模型的建立及意义%Establishment of a canine model with acute thrombo-embolic pulmonary hypertension guided by echocardiography

    Institute of Scientific and Technical Information of China (English)

    孙丹丹; 陈洪茂; 段云友; 尚福军; 梁宁南; 曹玮; 刘禧; 曹铁生

    2010-01-01

    目的 探讨超声引导下建立肺动脉血栓栓塞所致犬急性肺动脉高压模型的可行性.方法 模拟人体急性肺动脉血栓栓塞的病理生理过程,建立肺动脉血栓栓塞所致的犬急性肺动脉高压模型.超声引导下经股静脉穿刺,放置右心导管监测肺动脉压力,同时对侧股静脉推注自体血栓,以肺动脉收缩压超过30 mmHg为模型建立成功的标准.结果 超声可以准确引导右心导管的放置.实验用犬27只,成功建立急性肺动脉高压模型24只,成功率88.9%.结论 超声引导下建立肺动脉血栓栓塞所致犬急性肺动脉高压模型的方法具有良好的可行性和可重复性.%Objective To establish a canine model with acute thrombo-embolic pulmonary hypertension monitored by echocardiography. Methods Simulated the pathophysiologic process of acute thrombo-embolic pulmonary hypertension in human, the canine model was developed. The femoral vein puncture and the right heart catheterization to monitor pulmonary artery pressure were operated guided by echocardiography, while the autologous blood clot was injected to the contralateral femoral vein. The criteria of model was the pulmonary artery systolic pressure was higher than 30 mmHg. Results Echocardiography can accurately guide the placement of right heart catheterization and ancillarily observe the pressure testing of pulmonary artery. Of twenty seven healthy experiment dogs, twenty four models with acute thromboembolic pulmonary hypertension were successfully developed. The successful rate was 88.9 %. Conclusions This canine model with acute thrombo-embolic pulmonary hypertension guided by echocardiography is easy to operate and its repeatability is good.

  17. Missed diagnosis of atresia of the right pulmonary artery in woman with left-sided pneumothorax

    DEFF Research Database (Denmark)

    Dagnegård, Hanna; Ryom, Philip

    2016-01-01

    Isolated pulmonary atresia is an uncommon condition, which can go undiagnosed for a long time in asymptomatic patients. Sometimes, diagnosis can be made at pregnancy due to respiratory symptoms. There is no known increased risk of pneumothorax. We here present a case where a second-time pregnant ...... woman with an unknown atresia of the right pulmonary artery received a left-sided pneumothorax. The diagnosis was initially missed in spite of adequate imaging and the condition progressed to respiratory stop. We describe the course of diagnostics and the chosen strategy of treatment....

  18. Pulmonary Artery Agenesis Associated With Emphysema and Multiple Invasive Non-Small Cell Lung Cancers.

    Science.gov (United States)

    Makdisi, George; Edell, Eric S; Maleszewski, Joseph J; Molina, Julian R; Deschamps, Claude

    2015-06-01

    Pulmonary artery (PA) agenesis in the absence of associated cardiac abnormalities is a rare congenital abnormality. It may remain undiagnosed until adulthood when patients present with respiratory symptoms such as hemoptysis, dyspnea, repeated respiratory infections, or pulmonary hypertension. Herein we present a case of a 50-year-old woman who was found to have multiple, morphologically distinct non-small cell lung cancers in association with agenesis of the PA. This instance represents the fourth reported case of such association in the English literature.

  19. 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.

  20. 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.

  1. Cardiac magnetic resonance findings predicting mortality in patients with pulmonary arterial hypertension: a systematic review and meta-analysis

    NARCIS (Netherlands)

    V.J.M. Baggen (Vivan J. M.); I. Leiner; M.C. Post (Martijn); A.P.J. van Dijk (Arie); J.W. Roos-Hesselink (Jolien); H. Boersma (Eric); J. Habets; G.T. Sieswerda (Gertjan)

    2016-01-01

    textabstractObjectives: To provide a comprehensive overview of all reported cardiac magnetic resonance (CMR) findings that predict clinical deterioration in pulmonary arterial hypertension (PAH). Methods: MEDLINE and EMBASE electronic databases were systematically searched for longitudinal studies p

  2. KCa3.1 channel downregulation and impaired EDH-type relaxation in pulmonary arteries from chronic 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......Calcium-activated potassium channels of small (K(Ca)2, SK) and intermediate (K(Ca)3.1, IK) conductance are involved in endothelium-dependent relaxation of pulmonary arteries. We hypothesized that the function and expression of K(Ca)2 and K(Ca)3.1 increase as a compensatory mechanism to counteract......-induced relaxations were abolished by the combined inhibition of nitric oxide synthase (by L-nitro-arginine, L-NOARG), cyclo-oxygenase (by indomethacin) and soluble guanylate cyclase (by ODQ) in pulmonary arteries from hypoxic rats, whereas 20 ± 6% (n = 8) maximal relaxation in response to ACh persisted in arteries...

  3. Automated segmentation of the pulmonary arteries in low-dose CT by vessel tracking

    CERN Document Server

    Wala, Jeremiah; Lee, Jaesung; Jirapatnakul, Artit; Biancardi, Alberto; Reeves, Anthony

    2011-01-01

    We present a fully automated method for top-down segmentation of the pulmonary arterial tree in low-dose thoracic CT images. The main basal pulmonary arteries are identified near the lung hilum by searching for candidate vessels adjacent to known airways, identified by our previously reported airway segmentation method. Model cylinders are iteratively fit to the vessels to track them into the lungs. Vessel bifurcations are detected by measuring the rate of change of vessel radii, and child vessels are segmented by initiating new trackers at bifurcation points. Validation is accomplished using our novel sparse surface (SS) evaluation metric. The SS metric was designed to quantify the magnitude of the segmentation error per vessel while significantly decreasing the manual marking burden for the human user. A total of 210 arteries and 205 veins were manually marked across seven test cases. 134/210 arteries were correctly segmented, with a specificity for arteries of 90%, and average segmentation error of 0.15 mm...

  4. Relationship between chronic obstructive pulmonary disease and subclinical coronary artery disease in long-term smokers

    DEFF Research Database (Denmark)

    Rasmussen, Thomas; Køber, Lars; Pedersen, Jesper Holst;

    2013-01-01

    Cardiovascular conditions are reported to be the most frequent cause of death in patients with chronic obstructive pulmonary disease (COPD). However, it remains unsettled whether severity of COPD per se is associated with coronary artery disease (CAD) independent of traditional cardiovascular risk...... factors. The aim of this study was to examine the relationship between the presence and severity of COPD and the amount of coronary artery calcium deposit, an indicator of CAD and cardiac risk, in a large population of current and former long-term smokers....

  5. Influence of changes in the pulmonary artery pressure on ventilation requirements in patients undergoing mitral valve replacement.

    Directory of Open Access Journals (Sweden)

    Muralidhar K

    2004-01-01

    Full Text Available The study was designed to evaluate the influence of changes in pulmonary artery pressure on the ventilation requirements in patients undergoing mitral valve surgery. Thirty patients with mitral valve disease with significant pulmonary arterial hypertension undergoing mitral valve replacement under cardiopulmonary bypass were included in this prospective study. All patients had a pulmonary artery catheter placed after the anaesthetic induction. The minute ventilation was adjusted to achieve an arterial carbon dioxide tension (PaCO2 of 35-40 mm Hg. After a stabilisation period of 15 minutes, the pulmonary artery pressure and the minute volume needed for maintaining a PaCO2 of 35-40 mm Hg in the precardiopulmonary bypass, post-cardiopulmonary bypass and six hours postoperatively were measured after ensuring stable haemodynamics and normothermia. There was a significant decrease in the mean pulmonary artery pressure from pre-cardiopulmonary bypass value of 41.3+/-15 mm Hg to 29.3+/-8 mm Hg in the postcardiopulmonary bypass period and subsequently to 25.5+/-7 mm Hg in the intensive care unit. There was a corresponding increase in the minute volume requirements from a pre-cardiopulmonary bypass value of 6.8+/-1 L/min to 8.0+/-1 L/min in the post cardiopulmonary bypass period and then to 9.4+/-1.2 L/min in the postoperative period. We conclude that there is a significant decrease in the pulmonary blood volume and a subsequent decrease in the pulmonary artery pressure after a successful mitral valve replacement in patients with pulmonary arterial hypertension. This is associated with a significant increase in the requirement of minute ventilation to maintain normocarbia.

  6. Hemodynamic response to treatment of iron deficiency anemia in pulmonary arterial hypertension: longitudinal insights from an implantable hemodynamic monitor

    OpenAIRE

    Mehmood, Muddassir; Agarwal, Richa; Raina, Amresh; Correa-Jaque, Priscilla; Benza, Raymond L.

    2016-01-01

    Despite new therapeutic options, pulmonary arterial hypertension (PAH) remains a progressive disease associated with substantial morbidity and mortality. As such, additional strategies for monitoring and adjunctive management of this disease are important. A 59-year-old woman with scleroderma-associated PAH received an implantable hemodynamic monitor (IHM) as part of a research protocol at our institution. Pulmonary artery pressures, heart rate, and cardiac output (sensor-based algorithm) wer...

  7. Isolated right pulmonary artery agenesis with agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply from celiac axis with normal venous drainage.

    Science.gov (United States)

    Chaudhry, A; Rathore, M; Banavaliker, J N

    2014-01-01

    Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly. When detected in infancy, the condition is commonly associated with cardiovascular defects which are more frequently associated with left pulmonary artery agenesis. Patients with isolated right pulmonary artery agenesis survive into adulthood with minimal or no symptoms and are diagnosed incidentally on the chest radiographs. We report a case of a 19-year-old female patient who presented to us with recurrent haemoptysis. She was symptomatic since the age of four years. We report the rare occurrence of UAPA on right side, agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply of right lung from coeliac axis in this patient.

  8. Value of MR phase-contrast flow measurements for functional assessment of pulmonary arterial hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Ley, Sebastian [German Cancer Research Center DKFZ, Department of Radiology (E010), Heidelberg (Germany); University Heidelberg, Department of Pediatric Radiology, Heidelberg (Germany); Mereles, Derliz; Gruenig, Ekkehard [University Heidelberg, Department of Internal Medicine III, Heidelberg (Germany); Puderbach, Michael; Schoeck, Helena; Eichinger, Monika; Ley-Zaporozhan, Julia; Fink, Christian; Kauczor, Hans-Ulrich [German Cancer Research Center DKFZ, Department of Radiology (E010), Heidelberg (Germany)

    2007-07-15

    Goals of our study were to compare the pulmonary hemodynamics between healthy volunteers and patients with pulmonary arterial hypertension (PAH) and correlate MR flow measurements with echocardiography. Twenty-five patients with PAH and 25 volunteers were examined at 1.5 T. Phase-contrast flow measurements were performed in the ascending aorta and pulmonary trunk, resulting in the following parameters: peak velocity (cm/s), average blood flow (l/min), time to peak velocity (ms), velocity rise gradient and pulmonary distensibility (cm{sup 2}). The bronchosystemic shunt was calculated. In PAH patients transthoracic echocardiography and right-heart catheterization (RHC) served as the gold standard. In comparison to volunteers, the PAH patients showed significantly reduced pulmonary velocities (P = 0.002), blood flow (P = 0.002) and pulmonary distensibility (P = 0.008). In patients, the time to peak velocity was shorter (P<0.001), and the velocity rise gradient was steeper (P = 0.002) than in volunteers. While in volunteers the peak velocity in the aorta was reached earlier, it was the reverse in patients. Patients showed a significant bronchosystemic shunt (P = 0.01). No meaningful correlation was found between MRI measurements and echocardiography or RHC. MRI is a feasible technique for the differentiation between PAH and volunteers. Further studies have to be conducted for the absolute calculation of pressure estimates. (orig.)

  9. Clinical results of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries

    Institute of Scientific and Technical Information of China (English)

    FAN Xiang-ming; ZHU Yao-bin; SU Jun-wu; ZHANG Jing; LI Zhi-qiang; XU Yao-qiang; LI Xiao-feng

    2013-01-01

    Background Congenital heart defects with intractable hypoplasia of the pulmonary arteries without intercourse or with intercourse stenosis is unsuitable for surgical correction or regular palliative procedures.We reported our experience with combined palliative procedures for congenital heart defects with intractable hypoplasia pulmonary arteries.Methods From 2001 to 2012,a total of 41 patients with cyanotic congenital heart defects and intractable hypoplasia of the pulmonary arteries underwent surgical procedures.From among them,31 patients had pulmonary atresia with ventricular septal defect (VSD) and the other 10 cases had complicated congenital heart defects with pulmonary stenosis.Different kinds of palliative procedures were performed according to the morphology of the right and left pulmonary arteries in every patient.If the pulmonary artery was well developed,a Glenn procedure was performed.A modified Blalock-Taussi9 shunt or modified Waterston shunt was performed if pulmonary arteries were hypoplastic.If the pulmonary arteries were severely hypoplastic,a Melbourne shunt was performed.Systemic pulmonary artery shunts were performed bilaterally in 25 cases.A systemic-pulmonary shunt was performed on one side and a Glenn procedure was performed contralaterally in 16 cases.Major aortopulmonary collateral arteries were unifocalized in six cases,ligated in two cases and interventionally embolized in two cases.There was one early death because of cardiac arrest and the hospital mortality was 2.4%.Results Five patients suffered from postoperative low cardiac output syndrome,three had perfusion of the lungs,and two pulmonary infections.Systemic pulmonary shunts were repeated after the original operation in three cases due to the occlusion of conduits.The mean follow-up time was 25 months.The pre-and the post-operation left pulmonary indices were (8.13±3.68) vs.(14.9±6.21) mm2/m2.The pre-and post-operation right pulmonary indices were (12.7±8.13) vs.(17.7±7

  10. 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.

  11. Observation of right descending pulmonary arterial size and frontal cardiac area in patent ductus arteriosus patients

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, Kyung Soo; Seol, Hae Yung; Suh, Won Huk; Lee, Min Jae [Korea University College of Medicine, Seoul (Korea, Republic of)

    1979-12-15

    Frontal cardiac area, right descending pulmonary arterial size and size of the ductus arteriosus were measured on 29 patients with patent ductus arteriosus,21 females and 8 males. The results are as follows: 1. Frontal cardiac area in PDA patient in this series is increased in 0.63% to 100.11 % and the average is 38.91-23.54%. 2. Central branch of the right descending pulmonary artery is dilated in 28 cases (96.55%) on pre-operative stage, and combination with dilation of central branch and constriction of peripheral branch is in 18 cases (62.1%). 3. Degree of dilation of central branch is correlated with increase of age, but it is not related with size of ductus arteriosus. 4. Increase rate of frontal cardiac area is not related with size of ductus arteriosus.

  12. Therapeutic effect of low-dose imatinib on pulmonary arterial hypertension in dogs.

    Science.gov (United States)

    Arita, Shinji; Arita, Noboru; Hikasa, Yoshiaki

    2013-03-01

    This was a pilot study to determine the effectiveness of low-dose imatinib therapy for hemodynamic disturbances, including pulmonary arterial hypertension (PAH), and clinical manifestations caused by chronic heart failure in dogs. Six client-owned dogs with PAH were administered imatinib mesylate orally, 3 mg/kg body weight q24h, for 30 d. Physical examination, blood biochemical tests, radiography, and Doppler echocardiography were performed prior to imatinib administration and again 30 days after administration. Clinical scores were significantly reduced after imatinib treatment. Systolic pulmonary arterial pressure, heart rate, maximum tricuspid regurgitation velocity, left atrium/aorta ratio, right and left ventricular Tei indexes, early diastolic transmitral flow wave/mitral annulus velocity ratio, and plasma atrial natriuretic peptide concentration decreased significantly after therapy. Diastolic blood pressure, stroke volume, cardiac output, and left ventricular fractional shortening increased significantly after therapy. These results indicate that low-dose imatinib therapy was effective for heart failure in dogs with PAH.

  13. Renal sympathetic denervation prevents the development of pulmonary arterial hypertension and cardiac dysfunction in dogs.

    Science.gov (United States)

    Hu, Wei; Yu, Sheng-Bo; Chen, Liao; Guo, Rui-Qiang; Zhao, Qing-Yan

    2015-08-01

    The renin-angiotensin-aldosterone system is activated in pulmonary arterial hypertension (PAH) patients, and this activation may have long-term negative effects on the progression of PAH. The purpose of this study was to evaluate the effects of transcatheter renal sympathetic denervation (RSD) on the development of pulmonary arterial hypertension and cardiac dysfunction in dogs using two-dimensional speckle tracking imaging. Twenty-two dogs were randomly divided into three groups: control group (n = 7), PAH group (n = 8), and PAH + RSD group (n = 7). All dogs were assessed using two-dimensional speckle tracking imaging. The ventricular strain, ventricular synchrony, left ventricular (LV) twist, and torsion rate were analyzed to evaluate cardiac function. After 8 weeks, the right ventricular lateral longitudinal strain and the septum longitudinal strain were reduced in the PAH group compared with the control group (p dogs.

  14. Successful retrieval of a knotted pulmonary artery catheter trapped in the tricuspid valve apparatus.

    Science.gov (United States)

    Ishaq, Muhammad; Alexander, Nicki; Scott, David H T

    2013-04-01

    We report the case of a 64-year-old patient in whom a pulmonary artery catheter formed a knot fixed within the right ventricle in the region of the tricuspid valve apparatus. Knot formation is a recognized complication associated with pulmonary artery catheters (PAC) insertion. This problem is usually dealt with by simply withdrawing the PAC until the knot impacts onto the introducer and after enlarging the puncture site by a small skin incision removing the introducer-PAC as one unit. However, we recently encountered a situation where the PAC was knotted around the tricuspid valve apparatus and could not be withdrawn. An interventional radiologist was able to unknot the catheter and release it from the tricuspid valve. We reviewed the literature related to this topic. We believe our experience could be of use to others.

  15. Successful retrieval of a knotted pulmonary artery catheter trapped in the tricuspid valve apparatus

    Directory of Open Access Journals (Sweden)

    Muhammad Ishaq

    2013-01-01

    Full Text Available We report the case of a 64-year-old patient in whom a pulmonary artery catheter formed a knot fixed within the right ventricle in the region of the tricuspid valve apparatus. Knot formation is a recognized complication associated with pulmonary artery catheters (PAC insertion. This problem is usually dealt with by simply withdrawing the PAC until the knot impacts onto the introducer and after enlarging the puncture site by a small skin incision removing the introducer-PAC as one unit. However, we recently encountered a situation where the PAC was knotted around the tricuspid valve apparatus and could not be withdrawn. An interventional radiologist was able to unknot the catheter and release it from the tricuspid valve. We reviewed the literature related to this topic. We believe our experience could be of use to others.

  16. Treatment with anti-gremlin 1 antibody ameliorates chronic hypoxia/SU5416-induced pulmonary arterial hypertension in mice.

    Science.gov (United States)

    Ciuclan, Loredana; Sheppard, Kellyann; Dong, Liqun; Sutton, Daniel; Duggan, Nicholas; Hussey, Martin; Simmons, Jenny; Morrell, Nicholas W; Jarai, Gabor; Edwards, Matthew; Dubois, Gerald; Thomas, Matthew; Van Heeke, Gino; England, Karen

    2013-11-01

    The expression of the bone morphogenetic protein antagonist, Gremlin 1, was recently shown to be increased in the lungs of pulmonary arterial hypertension patients, and in response to hypoxia. Gremlin 1 released from the vascular endothelium may inhibit endogenous bone morphogenetic protein signaling and contribute to the development of pulmonary arterial hypertension. Here, we investigate the impact of Gremlin 1 inhibition in disease after exposure to chronic hypoxia/SU5416 in mice. We investigated the effects of an anti-Gremlin 1 monoclonal antibody in the chronic hypoxia/SU5416 murine model of pulmonary arterial hypertension. Chronic hypoxic/SU5416 exposure of mice induced upregulation of Gremlin 1 mRNA in lung and right ventricle tissue compared with normoxic controls. Prophylactic treatment with an anti-Gremlin 1 neutralizing mAb reduced the hypoxic/SU5416-dependent increase in pulmonary vascular remodeling and right ventricular hypertrophy. Importantly, therapeutic treatment with an anti-Gremlin 1 antibody also reduced pulmonary vascular remodeling and right ventricular hypertrophy indicating a role for Gremlin 1 in the progression of the disease. We conclude that Gremlin 1 plays a role in the development and progression of pulmonary arterial hypertension in the murine hypoxia/SU5416 model, and that Gremlin 1 is a potential therapeutic target for pulmonary arterial hypertension.

  17. Effects of cyclic intermittent hypoxia on ET-1 responsiveness and endothelial dysfunction of pulmonary arteries in rats.

    Directory of Open Access Journals (Sweden)

    Zhuo Wang

    Full Text Available Obstructive sleep apnoea (OSA is a risk factor for cardiovascular disorders and in some cases is complication of pulmonary hypertension. We simulated OSA by exposing rats to cyclic intermittent hypoxia (CIH to investigate its effect on pulmonary vascular endothelial dysfunction. Sprague-Dawley Rats were exposed to CIH (FiO2 9% for 1 min, repeated every 2 min for 8 h/day, 7 days/wk for 3 wk, and the pulmonary arteries of normoxia and CIH treated rats were analyzed for expression of endothelin-1 (ET-1 and ET receptors by histological, immunohistochemical, RT-PCR and Western Blot analyses, as well as for contractility in response to ET-1. In the pulmonary arteries, ET-1 expression was increased, and ET-1 more potently elicited constriction of the pulmonary artery in CIH rats than in normoxic rats. Exposure to CIH induced marked endothelial cell damage associated with a functional decrease of endothelium-dependent vasodilatation in the pulmonary artery. Compared with normoxic rats, ETA receptor expression was increased in smooth muscle cells of the CIH rats, while the expression of ETB receptors was decreased in endothelial cells. These results demonstrated endothelium-dependent vasodilation was impaired and the vasoconstrictor responsiveness increased by CIH. The increased responsiveness to ET-1 induced by intermittent hypoxia in pulmonary arteries of rats was due to increased expression of ETA receptors predominantly, meanwhile, decreased expression of ETB receptors in the endothelium may also participate in it.

  18. Adipokines: A Possible Contribution to Vascular and Bone Remodeling in Idiopathic Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Kochetkova, Evgenia A; Ugai, Ludmila G; Maistrovskaia, Yuliya V; Nevzorova, Vera A

    2017-04-01

    Osteoporosis is a major comorbidity of cardio-respiratory diseases, but the mechanistic links between pulmonary arterial hypertension and bone remain elusive. The purpose of the stud was to evaluate serum adipokines and endothelin-1 (ET-1) levels in the patients with idiopathic pulmonary arterial hypertension (IPAH) NYHA class III-IV and to determine its associations with bone mineral density (BMD). Pulmonary and hemodynamic parameters, BMD Z-scores at the lumbar spine (LS) and femoral neck (FN), serum leptin, adiponectin, visfatin and endothelin-1 (ET-1), were evaluated in 32 patients with IPAH NYHA class III-IV and 30 healthy volunteers. Leptin, adiponectin and ET-1 were higher in the patients with IPAH than in healthy subjects. Visfatin level showed a tendency to increase compared to that of healthy subjects (p = 0.076). The univariate analysis revealed a positive correlation between BMD Z-scores at both sites and 6-min walk test, and inverse relation with pulmonary vascular resistance (PVR) and mean pulmonary arterial pressure (mPAP). Adiponectin and visfatin showed positive correlations with PVR (p = 0.009 and p = 0.006). Serum adiponectin, visfatin and leptin were inversely associated with Z-scores. After adjusting for BMI and FMI, such associations persisted between visfatin and adiponectin levels and Z-scores at both sites. ET-1 related to mPAP, cardiac index and PVR. Negative correlation was observed between ET-1 and FN BMD (p = 0.01). Positive correlations have revealed between ET-1 and adiponectin (p = 0.02), visfatin (p = 0.004) in IPAH patients. These results provide further evidence that adipokine and endothelial dysregulation may cause not only a decrease in BMD, but also an increase in hemodynamic disorders of IPAH.

  19. 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.

  20. Diagnosis of unilateral pulmonary arterial agenesis using scintiangiography - a case report -

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok Hwa; Kim, Choon Yul; Shinn, Kyung Sub; Bahk, Yong Whee [Catholic Medical College, Seoul (Korea, Republic of)

    1986-08-15

    A 7-year-old boy presented with productive cough and mild dyspne a. He had a history of intermittant attacks of bronchopneumonia, but otherwise he was healthy. He had a small right lung on chest roentgenograms. The diagnosis of developmental hypoplasia of the right lung due to congenital absence of the right pulmonary artery was made by radionuclide angiocardiography and subsequently confirmed by cardiac catheterization and angiography.

  1. Early Diagnosis of Rare Diseases with a Focus on Pulmonary Arterial Hypertension: A Narrative Review

    OpenAIRE

    Bonaguro, Russell

    2015-01-01

    Health outcomes for rare diseases can be greatly affected by timely diagnosis.This paper presents a narrative review of current literature on rare diseases, with a focuson Pulmonary Arterial Hypertension (PAH), to identify needs for early diagnosisinitiatives. The review assessed: what needs to be done, what is currently being done,and what are the approaches or change theories that underlie these initiatives.Literature from online key-word searches included academic articles pertaining todia...

  2. Safety and tolerability considerations in the use of sildenafil for children with pulmonary arterial hypertension

    OpenAIRE

    Dodgen AL; Hill KD

    2015-01-01

    Andrew L Dodgen,1 Kevin D Hill1,2 1Department of Pediatrics, Duke University Medical Center, 2Duke Clinical Research Institute, Durham, NC, USA Abstract: Sildenafil is a phosphodiesterase type-5 inhibitor approved for treatment of pulmonary arterial hypertension (PAH) in adults. Data from pediatric trials demonstrate a similar acute safety profile to the adult population but have raised concerns regarding the safety of long-term use in children. Interpretation of these trials remains controve...

  3. Compound BMPR2 gene mutations in a malignant variant of idiopathic pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Walter Serra

    2014-12-01

    Full Text Available Pulmonary arterial hypertension (PAH; MIM 600799 is frequently associated with concomitant diseases, including congenital heart disease. 6% of patients with PAH show a family history of the disease [hereditary PAH (HPAH], with the major genetic determinants of HPAH being heterozygous germline mutations in the bone morphogenetic protein type II receptor (BMPR2. We present the case of a 38-year-old woman of Indian descent; initially admitted with progressive dyspnea [New York Heart Association (NYHA class III]. The results of the proband’s clinical assessments are presented here. Cardiac catheterization confirmed idiopathic PAH with severe right ventricular hypertrophy associated with pulmonary arteriopathy. Initial treatment comprised the dual endothelin receptor antagonist, bosentan, furosemide, warfarin and intravenous infusion of prostaglandin I2 (PGI2 for 3 days. Despite this, the patient died of pulmonary hemorrhagic edema and cardiogenic shock after 6 days of intensive care. After relatives’ consent, post mortem assessments confirmed a diagnosis of PAH; the heart displayed significant right ventricular hypertrophy and it was particularly noted that the right atrial appendage had undergone extreme dilation. Pulmonary arteriopathy was characterized by medial hypertrophy, arterialization of muscular arteries and muscularization of non-muscularized distal arteries. Molecular genetic analyses revealed the presence of cis-mutations in the BMPR2 gene (p.Cys123Arg and p.Arg332X. Cosegregation studies were not available. Our findings suggest that mutations of the BMPR2 gene gave rise to the onset of PAH in this patient and that the severity of the onset and progression could be attributed to the presence of multiple mutations in a genedosage manner.

  4. Pulmonary arterial hypertension associated with hereditary spherocytosis and splenectomy in a patient with a mutation in the BMPR2 gene.

    Science.gov (United States)

    Baloira, Adolfo; Bastos, María; Pousada, Guillermo; Valverde, Diana

    2016-08-01

    There is some question about the relationship between hereditary spherocytosis (HS) and pulmonary arterial hypertension, even associated with splenectomy. The finding of BMPR2 mutations in our patient suggests that other factors are necessary for the development of the disease, and perhaps, the incidence of pulmonary hypertension is not increased in patients with HS.

  5. Up-regulation of the mammalian target of rapamycin complex 1 subunit Raptor by aldosterone induces abnormal pulmonary artery smooth muscle cell survival patterns to promote pulmonary arterial hypertension.

    Science.gov (United States)

    Aghamohammadzadeh, Reza; Zhang, Ying-Yi; Stephens, Thomas E; Arons, Elena; Zaman, Paula; Polach, Kevin J; Matar, Majed; Yung, Lai-Ming; Yu, Paul B; Bowman, Frederick P; Opotowsky, Alexander R; Waxman, Aaron B; Loscalzo, Joseph; Leopold, Jane A; Maron, Bradley A

    2016-07-01

    Activation of the mammalian target of rapamycin complex 1 (mTORC1) subunit Raptor induces cell growth and is a downstream target of Akt. Elevated levels of aldosterone activate Akt, and, in pulmonary arterial hypertension (PAH), correlate with pulmonary arteriole thickening, which suggests that mTORC1 regulation by aldosterone may mediate adverse pulmonary vascular remodeling. We hypothesized that aldosterone-Raptor signaling induces abnormal pulmonary artery smooth muscle cell (PASMC) survival patterns to promote PAH. Remodeled pulmonary arterioles from SU-5416/hypoxia-PAH rats and monocrotaline-PAH rats with hyperaldosteronism expressed increased levels of the Raptor target, p70S6K, which provided a basis for investigating aldosterone-Raptor signaling in human PASMCs. Aldosterone (10(-9) to 10(-7) M) increased Akt/mTOR/Raptor to activate p70S6K and increase proliferation, viability, and apoptosis resistance in PASMCs. In PASMCs transfected with Raptor-small interfering RNA or treated with spironolactone/eplerenone, aldosterone or pulmonary arterial plasma from patients with PAH failed to increase p70S6K activation or to induce cell survival in vitro Optimal inhibition of pulmonary arteriole Raptor was achieved by treatment with Staramine-monomethoxy polyethylene glycol that was formulated with Raptor-small interfering RNA plus spironolactone in vivo, which decreased arteriole muscularization and pulmonary hypertension in 2 experimental animal models of PAH in vivo Up-regulation of mTORC1 by aldosterone is a critical pathobiologic mechanism that controls PASMC survival to promote hypertrophic vascular remodeling and PAH.-Aghamohammadzadeh, R., Zhang, Y.-Y., Stephens, T. E., Arons, E., Zaman, P., Polach, K. J., Matar, M., Yung, L.-M., Yu, P. B., Bowman, F. P., Opotowsky, A. R., Waxman, A. B., Loscalzo, J., Leopold, J. A., Maron, B. A. Up-regulation of the mammalian target of rapamycin complex 1 subunit Raptor by aldosterone induces abnormal pulmonary artery smooth

  6. 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.

  7. Endothelin-1 receptor antagonists in fetal development and pulmonary arterial hypertension.

    Science.gov (United States)

    de Raaf, Michiel Alexander; Beekhuijzen, Manon; Guignabert, Christophe; Vonk Noordegraaf, Anton; Bogaard, Harm Jan

    2015-08-15

    The Pregnancy Prevention Program (PPP) is in place to prevent drug-induced developmental malformations. Remarkably, among the ten PPP-enlisted drugs are three endothelin-1 (ET-1) receptor antagonists (ERA's: ambrisentan, bosentan and macitentan), which are approved for the treatment of Pulmonary Arterial Hypertension (PAH). This review describes the effects of ERA's in PAH pathobiology and cardiopulmonary fetal development. While ERA's hamper pathological remodeling of the pulmonary vasculature and as such exert beneficial effects in PAH, they disturb fetal development of cardiopulmonary tissues. By blocking ET-1-mediated positive inotropic effects and myocardial fetal gene induction, ERA's may affect right ventricular adaptation to the increased pulmonary vascular resistance in both the fetus and the adult PAH patient.

  8. Endovascular Treatment of an Aneurysmal Aberrant Systemic Artery Supplying a Pulmonary Sequestrum.

    Science.gov (United States)

    Kristensen, Katrine Lawaetz; Duus, Louise Aarup; Elle, Bo

    2015-10-01

    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.

  9. The functional expression of extracellular calcium-sensing receptor in rat pulmonary artery smooth muscle cells

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    Zhang Wei-hua

    2011-02-01

    Full Text Available Abstract Background The extracellular calcium-sensing receptor (CaSR belongs to family C of the G protein coupled receptors. Whether the CaSR is expressed in the pulmonary artery (PA is unknown. Methods The expression and distribution of CaSR were detected by RT-PCR, Western blotting and immunofluorescence. PA tension was detected by the pulmonary arterial ring technique, and the intracellular calcium concentration ([Ca2+]i was detected by a laser-scanning confocal microscope. Results The expressions of CaSR mRNA and protein were found in both rat pulmonary artery smooth muscle cells (PASMCs and PAs. Increased levels of [Ca2+]o (extracellular calcium concentration or Gd3+ (an agonist of CaSR induced an increase of [Ca2+]i and PAs constriction in a concentration-dependent manner. In addition, the above-mentioned effects of Ca2+ and Gd3+ were inhibited by U73122 (specific inhibitor of PLC, 2-APB (specific antagonist of IP3 receptor, and thapsigargin (blocker of sarcoplasmic reticulum calcium ATPase. Conclusions CaSR is expressed in rat PASMCs, and is involved in regulation of PA tension by increasing [Ca2+]i through G-PLC-IP3 pathway.

  10. 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.

  11. Correlative magnetic resonance imaging in the evaluation of aortic and pulmonary artery abnormalities

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    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. Pulmonary artery catheter complications: report on a case of a knot accident and literature review

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    Lopes Marcelo Cruz

    2004-01-01

    Full Text Available A particular event concerning a Swan-Ganz catheter complication is reported. A 41-year-old woman was admitted at the emergency room of our hospital with massive gastrointestinal bleeding. A total gastrectomy was performed. During the postoperative period in the intensive care unit , the patient maintained hemodynamic instability. Invasive hemodynamic monitoring with a pulmonary artery catheter was then indicated. During the maneuvers to insert the catheter, a true knot formation was identified at the level of the superior vena cava. Several maneuvers by radiological endovascular invasive techniques allowed removal of the catheter. The authors describe the details of this procedure and provide comments regarding the various techniques that were employed in overcoming this event. A comprehensive review of evidence regarding the benefits and risks of pulmonary artery catheterization was performed. The consensus statement regarding the indications, utilization, and management of the pulmonary artery catheterization that were issued by a consensus conference held in 1996 are also discussed in detail.

  13. Riociguat as a treatment regime for pulmonary arterial hypertension: a review.

    Science.gov (United States)

    Narang, Bawneet K; Roy, Subhajit; Sharma, Rajiv; Singh, Virender; Rawal, Ravindra K

    2015-01-01

    Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening condition distinguished by elevated pressure of pulmonary arteries and increased vascular resistance. The management of patients with PAH and CTEPH has advanced rapidly over last decade but despite the progress in the treatment, the survival of suffering patients remain unsatisfactory and there is no cure for the diseases. However, surgery is not a first choice for patients. Furthermore, some patients who undergo surgery have persistent pulmonary hypertension (HTN) as a side effect after surgery. Therefore, the search for an "ideal" therapy still goes on and it lead to the approval of riociguat as a potential agent for the treatment. It acts directly on soluble guanylate cyclase, exciting the enzyme, and elevating sensitivity to lower levels of NO. Riociguat, therefore, has potential as a novel therapy for PAH and CTEPH. This review is focused on various aspects of the recently approved "riociguat" including its efficacy and safety profiles with the clinical data highlighting its importance in the present scenario.

  14. Puerarin induces mitochondria-dependent apoptosis in hypoxic human pulmonary arterial smooth muscle cells.

    Directory of Open Access Journals (Sweden)

    Chan Chen

    Full Text Available BACKGROUND: Pulmonary vascular medial hypertrophy in hypoxic pulmonary arterial hypertension (PAH is caused in part by decreased apoptosis in pulmonary artery smooth muscle cells (PASMCs. Puerarin, an isoflavone purified from the Chinese medicinal herb kudzu, ameliorates chronic hypoxic PAH in animal models. Here we investigated the effects of puerarin on apoptosis of hypoxic human PASMCs (HPASMCs, and to determine the possible underlying mechanisms. METHODOLOGY/PRINCIPAL FINDINGS: HPASMCs were cultured for 24 h in normoxia or hypoxia (5% O₂ conditions with and without puerarin. Cell number and viability were determined with a hemacytometer or a cell counting kit. Apoptosis was detected with a TUNEL test, rhodamine-123 (R-123 fluorescence, a colorimetric assay, western blots, immunohistochemical staining and RT-PCR. Hypoxia inhibited mitochondria-dependent apoptosis and promoted HPASMC growth. In contrast, after puerarin (50 µM or more intervention, cell growth was inhibited and apoptosis was observed. Puerarin-induced apoptosis in hypoxic HPASMCs was accompanied by reduced mitochondrial membrane potential, cytochrome c release from the mitochondria, caspase-9 activation, and Bcl-2 down-regulation with concurrent Bax up-regulation. CONCLUSIONS/SIGNIFICANCE: Puerarin promoted apoptosis in hypoxic HPASMCs by acting on the mitochondria-dependent pathway. These results suggest a new mechanism of puerarin relevant to the management of clinical hypoxic pulmonary hypertension.

  15. Puerarin Induces Mitochondria-Dependent Apoptosis in Hypoxic Human Pulmonary Arterial Smooth Muscle Cells

    Science.gov (United States)

    Chen, Chan; Chen, Chun; Wang, Zhiyi; Wang, Liangxing; Yang, Lehe; Ding, Minjiao; Ding, Cheng; Sun, Yu; Lin, Quan; Huang, Xiaoying; Du, Xiaohong; Zhao, Xiaowei; Wang, Chuangyi

    2012-01-01

    Background Pulmonary vascular medial hypertrophy in hypoxic pulmonary arterial hypertension (PAH) is caused in part by decreased apoptosis in pulmonary artery smooth muscle cells (PASMCs). Puerarin, an isoflavone purified from the Chinese medicinal herb kudzu, ameliorates chronic hypoxic PAH in animal models. Here we investigated the effects of puerarin on apoptosis of hypoxic human PASMCs (HPASMCs), and to determine the possible underlying mechanisms. Methodology/Principal Findings HPASMCs were cultured for 24 h in normoxia or hypoxia (5% O2) conditions with and without puerarin. Cell number and viability were determined with a hemacytometer or a cell counting kit. Apoptosis was detected with a TUNEL test, rhodamine-123 (R-123) fluorescence, a colorimetric assay, western blots, immunohistochemical staining and RT-PCR. Hypoxia inhibited mitochondria-dependent apoptosis and promoted HPASMC growth. In contrast, after puerarin (50 µM or more) intervention, cell growth was inhibited and apoptosis was observed. Puerarin-induced apoptosis in hypoxic HPASMCs was accompanied by reduced mitochondrial membrane potential, cytochrome c release from the mitochondria, caspase-9 activation, and Bcl-2 down-regulation with concurrent Bax up-regulation. Conclusions/Significance Puerarin promoted apoptosis in hypoxic HPASMCs by acting on the mitochondria-dependent pathway. These results suggest a new mechanism of puerarin relevant to the management of clinical hypoxic pulmonary hypertension. PMID:22457823

  16. 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

  17. Analysis of volatile compounds in exhaled breath condensate in patients with severe pulmonary arterial hypertension.

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    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.

  18. Vascular relaxation of canine visceral arteries after ischemia by means of supraceliac aortic cross-clamping followed by reperfusion

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    Dalio Marcelo B

    2010-07-01

    Full Text Available Abstract Background The supraceliac aortic cross-clamping can be an option to save patients with hipovolemic shock due to abdominal trauma. However, this maneuver is associated with ischemia/reperfusion (I/R injury strongly related to oxidative stress and reduction of nitric oxide bioavailability. Moreover, several studies demonstrated impairment in relaxation after I/R, but the time course of I/R necessary to induce vascular dysfunction is still controversial. We investigated whether 60 minutes of ischemia followed by 30 minutes of reperfusion do not change the relaxation of visceral arteries nor the plasma and renal levels of malondialdehyde (MDA and nitrite plus nitrate (NOx. Methods Male mongrel dogs (n = 27 were randomly allocated in one of the three groups: sham (no clamping, n = 9, ischemia (supraceliac aortic cross-clamping for 60 minutes, n = 9, and I/R (60 minutes of ischemia followed by reperfusion for 30 minutes, n = 9. Relaxation of visceral arteries (celiac trunk, renal and superior mesenteric arteries was studied in organ chambers. MDA and NOx concentrations were determined using a commercially available kit and an ozone-based chemiluminescence assay, respectively. Results Both acetylcholine and calcium ionophore caused relaxation in endothelium-intact rings and no statistical differences were observed among the three groups. Sodium nitroprusside promoted relaxation in endothelium-denuded rings, and there were no inter-group statistical differences. Both plasma and renal concentrations of MDA and NOx showed no significant difference among the groups. Conclusion Supraceliac aortic cross-clamping for 60 minutes alone and followed by 30 minutes of reperfusion did not impair relaxation of canine visceral arteries nor evoke biochemical alterations in plasma or renal tissue.

  19. Hydrogen sulfide induces apoptosis of pulmonary artery smooth muscle cell in rats with pulmonary hypertension induced by high pulmonary blood flow

    Institute of Scientific and Technical Information of China (English)

    LI Wei; JIN Hong-fang; LIU Die; SUN Jing-hui; JIAN Pei-jun; LI Xiao-hui; TANG Chao-shu; DU Jun-bao

    2009-01-01

    Background Abnormal apoptosis of pulmonary artery smooth muscle cells (PASMCs) is an important pathophysiological process in the pulmonary artery structural remodeling and pulmonary hypertension. We investigated possible effect of endogenous hydrogen sulfide (H_2S) on apoptosis of PASMCs during the development of pulmonary hypertension induced by high pulmonary blood flow.Methods Thirty-nine male Sprague-Dawley rats were randomly assigned to 4-week control, 4-week shunt, 4-week shunt+propargylglycine (PPG), 11-week control, 11-week shunt and 11-week shunt+sodium hydrosulfide (NaHS) groups. Rats in 4-week shunt, 4-week shunt+PPG, 11-week shunt and 11-week shunt+NaHS groups underwent an abdominal aorta-inferior vena cava shunt. Rats in 4-week shunt+PPG group were intraperitoneally injected with PPG, an inhibitor of endogenous H_2S production, for 4 weeks. Rats in 11-week shunt+NaHS group were intraperitoneally injected with NaHS, a H_2S donor, for 11 weeks. Lung tissue H_2S was evaluated by sulfide-sensitive electrode. Apoptosis of PASMCs were detected by terminal deoxynucleotidyl transferase mediated dUTP nick end labelling (TUNEL). Expressions of Fas, bcl-2 and caspase-3 in the PASMCs were analyzed with immunochemical staining.Results Four weeks after the shunting operation, the apoptosis of PASMCs and expression of Fas and caspase-3 were significantly decreased (P<0.01), but expression of bcl-2 increased significantly (P<0.01). PPG administration further inhibited the apoptosis of PASMCs, downregulated the expression of Fas and caspase-3 (P <0.01), but increased the expression of bcl-2 (P<0.01). After 11 weeks of shunting operation, the apoptosis of PASMCs and expression of Fas and caspase-3 were significantly decreased (P <0.01), but expression of bcl-2 increased obviously (P <0.01). NaHS administration significantly increased the apoptosis of PASMCs, upregulated the expression of Fas and caspase-3, but inhibited the expression of bcl-2.Conclusions H_2S induces

  20. Elevated pulmonary artery pressure and brain natriuretic peptide in high altitude pulmonary edema susceptible non-mountaineers

    Science.gov (United States)

    Gupta, Rajinder K.; Himashree, G.; Singh, Krishan; Soree, Poonam; Desiraju, Koundinya; Agrawal, Anurag; Ghosh, Dishari; Dass, Deepak; Reddy, Prassana K.; Panjwani, Usha; Singh, Shashi Bala

    2016-01-01

    Exaggerated pulmonary pressor response to hypoxia is a pathgonomic feature observed in high altitude pulmonary edema (HAPE) susceptible mountaineers. It was investigated whether measurement of basal pulmonary artery pressure (Ppa) and brain natriuretic peptide (BNP) could improve identification of HAPE susceptible subjects in a non-mountaineer population. We studied BNP levels, baseline hemodynamics and the response to hypoxia (FIo2 = 0.12 for 30 min duration at sea level) in 11 HAPE resistant (no past history of HAPE, Control) and 11 HAPE susceptible (past history of HAPE, HAPE-S) subjects. Baseline Ppa (19.31 ± 3.63 vs 15.68 ± 2.79 mm Hg, p < 0.05) and plasma BNP levels (52.39 ± 32.9 vs 15.05 ± 9.6 pg/ml, p < 0.05) were high and stroke volume was less (p < 0.05) in HAPE-S subjects compared to control. Acute hypoxia produced an exaggerated increase in heart rate (p < 0.05), mean arterial pressure (p < 0.05) and Ppa (28.2 ± 5.8 vs 19.33 ± 3.74 mm Hg, p < 0.05) and fall in peripheral oxygen saturation (p < 0.05) in HAPE-S compared to control. Receiver operating characteristic (ROC) curves showed that Ppa response to acute hypoxia was the best variable to identify HAPE susceptibility (AUC 0.92) but BNP levels provided comparable information (AUC 0.85). BNP levels are easy to determine and may represent an important marker for the determination of HAPE susceptibility. PMID:26892302

  1. Late presentation of an anomalous left coronary artery from the pulmonary artery treated with conservative surgical management with long-term cardiac magnetic resonance imaging follow-up

    Science.gov (United States)

    Gouda, Pishoy; Gouda, John; Butler, Craig; Welsh, Robert C

    2017-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery is rare congenital abnormality that most commonly presents in childhood and is associated with a high mortality. In the elderly, patients may present acutely with arrhythmias or signs of ischemia or with vague chronic presentations of shortness of breath and fatigue. In the high-risk elderly population, it is unclear as to whether conservative surgical management by means of suture ligation of the left coronary artery is associated with positive long-term outcomes. We present a case of a 69-year-old patient diagnosed with anomalous origin of the left coronary artery from the pulmonary artery, which was treated with conservative surgical management and followed up for 15 years with cardiovascular magnetic resonance imaging, with positive outcomes.

  2. Effect of stellate block on vasomotor factor, vascular endothelial nitricoxide synthase and pulmonary arterial pressure in rabbits with hypoxic pulmonary artery hypertension

    Institute of Scientific and Technical Information of China (English)

    Shunhou He; Qing Li; Sen Chen; Qingxiu Wang

    2007-01-01

    BACKGROUND: At present, inhalation of nitrogen monoxidum (NO) or other angiotenic is widely used to cure hypoxic pulmonary artery hypertension. In addition, recent researches demonstrate that postganglionic fiber of stellate ganglion can regulate contents of blood vessel endothelium-calcitonin gene-related peptide (BE-CGRP) and nitricoxide synthase (NOS) in lung tissue. Therefore, stellate ganglion which is blocked with the local anesthetic may cause therapeutic effects on hypoxic pulmonary artery hypertension.OBJECTIVE: To observe the effects of stellate block on calcitonin gene-related peptide (CGRP) of vasodilation factors, prostacyclin, endothelin-1 of vasoconstriction factors, thromboxan, blood vessel endothelium-nitricoxide synthase (BE-NOS) and mean arterial pressure of lung tissue in rabbits with hypoxic pulmonary artery hypertension.DESIGN: Randomly controlled animal study.SETTING: Neurological Institute of Taihe Hospital Affiliated to Yunyang Medical College.MATERIALS: A total of 24 adult Japanese rabbits of both genders and weighing 2.3 - 2.6 kg were provided by Animal Experimental Center of Hubei Academy of Medical Science. SP kit was provided by Beijing Zhongshan Biotechnology Co., Ltd.; moreover, kits of endothelin-1, CGRP, prostacyclin and thromboxan were provided by Radioimmunity Institute, Scientific and Technological Developing Center, General Hospital of Chinese PLA, and color image analytical system (Leica-Q500IW) was made in Germany.METHODS: The experiment was carried out in the Neurological Institute of Taihe Hospital affiliated to Yunyang Medical College from February to December 2002, ① Rabbits were performed with aseptic manipulation to exposure left stellate ganglion and then it was put in epidural catheter for 1 week. In addition,one end of epidural catheter was fixed near by stellate ganglion and the other end was fixed through dorsal neck. All rabbits were randomly divided into 4 groups, including normal control group, stellate block

  3. Effect of the sphingosine kinase 1 selective inhibitor, PF-543 on arterial and cardiac remodelling in a hypoxic model of pulmonary arterial hypertension.

    Science.gov (United States)

    MacRitchie, Neil; Volpert, Giora; Al Washih, Mohammed; Watson, David G; Futerman, Anthony H; Kennedy, Simon; Pyne, Susan; Pyne, Nigel J

    2016-08-01

    Recent studies have demonstrated that the expression of sphingosine kinase 1, the enzyme that catalyses formation of the bioactive lipid, sphingosine 1-phosphate, is increased in lungs from patients with pulmonary arterial hypertension. In addition, Sk1(-/-) mice are protected from hypoxic-induced pulmonary arterial hypertension. Therefore, we assessed the effect of the sphingosine kinase 1 selective inhibitor, PF-543 and a sphingosine kinase 1/ceramide synthase inhibitor, RB-005 on pulmonary and cardiac remodelling in a mouse hypoxic model of pulmonary arterial hypertension. Administration of the potent sphingosine kinase 1 inhibitor, PF-543 in a mouse hypoxic model of pulmonary hypertension had no effect on vascular remodelling but reduced right ventricular hypertrophy. The latter was associated with a significant reduction in cardiomyocyte death. The protection involves a reduction in the expression of p53 (that promotes cardiomyocyte death) and an increase in the expression of anti-oxidant nuclear factor (erythroid-derived 2)-like 2 (Nrf-2). In contrast, RB-005 lacked effects on right ventricular hypertrophy, suggesting that sphingosine kinase 1 inhibition might be nullified by concurrent inhibition of ceramide synthase. Therefore, our findings with PF-543 suggest an important role for sphingosine kinase 1 in the development of hypertrophy in pulmonary arterial hypertension.

  4. Dynamic Changes of Pulmonary Arterial Pressure and Ductus Arteriosus in Human Newborns From Birth to 72 Hours of Age

    OpenAIRE

    2016-01-01

    Abstract Normal pulmonary artery pressure and pulmonary hypertension assessment of newborns is rarely reported. The aim of the study is to explore dynamic changes of pulmonary arterial pressure and ductus arteriosus in human newborns from birth to 72 h of age with echocardiography. A total of 76 cases of normal newborns were prospectively detected by echocardiography after birth of 2 h, 6 h, 12 h, 24 h, 48 h, and 72 h, respectively. Ductus arteriosus diameter, blood shunt direction, blood flo...

  5. Ionic radiocontrast inhibits endothelium-dependent vasodilation of the canine renal artery in vitro: possible mechanism of renal failure following contrast medium infusion

    Directory of Open Access Journals (Sweden)

    B. Discigil

    2004-02-01

    Full Text Available To determine if radiocontrast impairs vascular relaxation of the renal artery, segments (4-5 mm in length of canine renal artery were suspended in vitro in organ chambers to measure isometric force (95% O2/5% CO2, at 37ºC. Arterial segments with and without endothelium were placed at the optimal point of their length-tension relation and incubated with 10 µM indomethacin to prevent synthesis of endogenous prostanoids. The presence of nonionic radiocontrast (iohexol, Omnipaque 350, 1 ml in 25 ml control solution, 4% (v/v did not alter endothelium-dependent relaxation to acetylcholine in rings precontracted with both norepinephrine and prostaglandin F2alpha (N = 6. When the rings were precontracted with prostaglandin F2alpha, the presence of ionic contrast did not inhibit the relaxation of the arteries. However, in canine renal arteries contracted with norepinephrine, the presence of ionic radiocontrast (diatrizoate meglumine and diatrizoate sodium, MD-76, 1 ml in 25 ml control solution, 4% (v/v inhibited relaxation in response to acetylcholine, sodium nitroprusside (N = 6 in each group, and isoproterenol (N = 5; P < 0.05. Rings were relaxed less than 50% of norepinephrine contraction. Following removal of the contrast, vascular relaxation in response to the agonists returned to normal. These results indicate that ionic radiocontrast nonspecifically inhibits vasodilation (both cAMP-mediated and cGMP-mediated of canine renal arteries contracted with norepinephrine. This reversible impairment of vasodilation could inhibit normal renal perfusion and act as a mechanism of renal failure following radiocontrast infusion. In the adopted experimental protocol the isoproterenol-induced relaxation of renal arteries precontracted with norepinephrine was more affected, suggesting a pivotal role of the cAMP system.

  6. MDCT and 3D evaluation of type 2 hypoplastic pulmonary artery sling associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis.

    Science.gov (United States)

    Lee, Edward Y

    2007-11-01

    The early diagnosis and complete anatomic evaluation of pulmonary artery sling, a congenital vascular anomaly in which left pulmonary artery arises from the right pulmonary artery, is paramount for proper patient management, because patients with this disorder frequently have other congenital anomalies resulting in high morbidity and mortality. Until recently, pulmonary artery sling in the neonate has been established with standard radiologic imaging studies such as plain radiographs, barium swallow studies, fluoroscopy-guided airway studies, and echocardiograms. However, with the development and widespread availability of multidetector computed tomography, pulmonary artery sling is increasingly evaluated with this newer technology. This case report presents a rare incidence of type 2 hypoplastic pulmonary artery sling in a neonate associated with right lung agenesis, hypoplastic aortic arch, and long segment tracheal stenosis. Multidetector computed tomography combined with 3-dimensional evaluation was particularly helpful in making a correct diagnosis of the complicated anatomic anomalies found in this case.

  7. Efficacy of pimobendan on survival and reoccurrence of pulmonary edema in canine congestive heart failure.

    Science.gov (United States)

    Mizuno, Masashi; Yamano, Shigeki; Chimura, Shuichi; Hirakawa, Atsushi; Takusagawa, Yoshimi; Sawada, Tamotsu; Maetani, Shigeki; Takahashi, Arane; Mizuno, Takeshi; Harada, Kayoko; Shinoda, Asako; Uchida, Shuhei; Takeuchi, Junichiro; Mizukoshi, Takahiro; Endo, Masaaki; Uechi, Masami

    2017-01-20

    The aim of this study was to evaluate the efficacy of pimobendan with conventional therapies on survival and reocurrence of pulmonary edema in dogs with congestive heart failure (CHF) caused by myxomatous mitral valve disease (MMVD). Records of 197 client-owned dogs from 14 veterinary hospitals were included in this study. Dogs were administered conventional treatments with or without pimobendan. Sixty-four dogs received a standard dose of pimobendan (0.20-0.48 mg/kg every 12 hr (q12hr)), 49 dogs received a low dose of pimobendan (0.05-0.19 mg/kg q12hr), and 84 dogs received conventional therapy alone. Dogs in the standard-dose and low-dose pimobendan groups had significantly longer median survival times than dogs in the conventional group (334, 277 and 136 days, respectively; P<0.001). The reoccurrence rate of pulmonary edema in the standard-dose group was significantly lower than in the low-dose and conventional groups (43%, 59% and 62%, respectively; P<0.05). Combination of pimobendan with a conventional treatment regimen significantly prolonged survival time after an initial episode of pulmonary edema in dogs with CHF caused by MMVD. There was no difference in survival between dogs administered standard and low doses of pimobendan, but pimobendan did prevent the reoccurrence of pulmonary edema in a dose-dependent manner.

  8. Unusual presentation of renal vein thrombosis with pulmonary artery embolism.

    Science.gov (United States)

    Mzayen, Khaled; Al-Said, Jafar; Nayak-Rao, Shobhana; Catacutan, Maria Teresa; Kamel, Olfat

    2013-05-01

    A young 23-year-old male patient presented with a two-day history of right flank pain. He had no history of any significant illnesses in the past. His investigations showed nephrotic range proteinuria with hypoalbuminemia. The patient developed cough and shortness of breath after having a left kidney biopsy. He did not respond to regular respiratory tract infection treatment. The kidney biopsy revealed membranoproliferative glomerulonephritis. Further investigations for the cough showed thromboembolism of the posterior and lateral basal segments of the right lower lobe. Moreover he was found to have thrombosis of the right upper pole renal vein. The patient was started on full anticoagulation along with three days pulse steroid, followed by 1 mg/kg oral steroid. Clinical improvement was noticed within 48 h. After eight weeks the proteinuria decreased from 8.5 gm/day to 1.1 gm/day. The kidney function was normal with eGFR 145 mL/min through the course of the disease. This case represent one of the unusual presentation of nephrotic syndrome with pulmonary and renal vascular thromboembolic events. The response to the combination of anticoagulation and steroid was remarkable.

  9. Unusual presentation of renal vein thrombosis with pulmonary artery embolism

    Directory of Open Access Journals (Sweden)

    Khaled Mzayen

    2013-01-01

    Full Text Available A young 23-year-old male patient presented with a two-day history of right flank pain. He had no history of any significant illnesses in the past. His investgations showed nephrotic range proteinuria with hypoalbuminemia. The patient developed cough and shortness of breath after having a left kidney biopsy. He did not respond to regular respiratory tract infection treat-ment. The kidney biopsy revealed membranoproliferative glomerulonephritis. Further investigations for the cough showed thromboembolism of the posterior and lateral basal segments of the right lower lobe. Moreover he was found to have thrombosis of the right upper pole renal vein. The patient was started on full anticoagulation along with three days pulse steroid, followed by 1 mg/kg oral steroid. Clinical improvement was noticed within 48 h. After eight weeks the proteinuria decreased from 8.5 gm/day to 1.1 gm/day. The kidney function was normal with eGFR 145 mL/min through the course of the disease. This case represent one of the unusual presentation of nephrotic syndrome with pulmonary and renal vascular thromboembolic events. The response to the combination of anticoagulation and steroid was remarkable.

  10. Up-front combination therapy in pulmonary arterial hypertension: from clinical trials to 'real world' observational studies.

    Science.gov (United States)

    Grignola, Juan C

    2014-05-15

    Combination therapy in pulmonary arterial hypertension is widely used in daily clinical practice. There is a wide variation among reference centers, with respect to not only when and how combination therapy should be initiated, but also what constitutes the most effective multidrug regimen. Presently, no combination therapy has proven to be more effective than the other. However, add-on therapy, in case of unsatisfactory improvements after initiation of specific monotherapy, seems to have more evidence of effectiveness than up-front therapy at the diagnosis. Data from national pulmonary arterial hypertension registries provide valuable information on practice patterns on diagnosis and treatment in 'real world'. In the study of Bergot et al. the current use of epoprostenol in newly diagnosed patients with severe idiopathic, heritable or anorexigen-use associated pulmonary arterial hypertension enrolled in the French pulmonary hypertension registry was investigated. Up-front combination of epoprostenol and oral pulmonary arterial hypertension tended to be more beneficial compared with epoprostenol alone. New drug trials with well-defined morbidity and mortality end-points are likely to help not only identify novel agents that may help in the treatment of pulmonary arterial hypertension but also determine the most useful combination strategies to maximize efficacy of existing drugs.

  11. Two congenital coronary abnormalities affecting heart function: anomalous origin of the left coronary artery from the pulmonary artery and congenital left main coronary artery atresia

    Institute of Scientific and Technical Information of China (English)

    Xiao Yanyan; Jin Mei; Han Ling; Ding Wenhong; Zheng Jianyong; Sun Chufan; Lyu Zhenyu

    2014-01-01

    Background The anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) and congenital left main coronary artery atresia (CLMCA-A) are two kinds of very rare coronary heart diseases which affect heart function profoundly.This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients.Methods From April 1984 to July 2012,in Beijing Anzhen Hospital,23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A.We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation,diagnosis,and treatments of these two kinds of congenital coronary abnormalities.Results The 23 patients (13 males and 10 females,aged ranging from 2.5 months to 65 years) identified with ALCAPA were classified into infantile type (age of onset younger than 12 months,16 cases) and adult type (age of onset older than 12 months,7 cases).Four patients were diagnosed with CLMCA-A (three males and one female,aged ranging from 3 months to 2 years).The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection,heart failure,dyspnea,feeding intolerance,diaphoresis,and failure to thrive.And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis,dilated cardiomyopathy,and acute myocardial infarction.As for the adult-type ALCAPA,cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease,myocarditis,or patent ductus arteriosus.In ECG examination:Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I,avL,and V4-V6,especially in lead avL.However,ECG of adult-type ALCAPA lacked distinct features.In chest radiography:pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A,while pulmonary artery segment dilation was more common in

  12. 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.

  13. 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.

  14. Pulmonary artery perfusion with HTK solution prevents lung injury in infants after cardiopulmonary bypass

    Institute of Scientific and Technical Information of China (English)

    LI Jian-an; LIU Ying-long; LIU Jin-ping; LI Xiao-feng

    2010-01-01

    Background Pulmonary artery perfusion during cardiopulmonary bypass (CPB) is a novel adjunctive method, which can minimize the lung ischemic-reperfusion injury and inflammatory response. This study evaluated the protective effect of pulmonary perfusion with hypothermic HTK solution in corrections of congenital heart defects with pulmonary hypertension.Methods Between June 2009 and December 2009, 24 consecutive infants with congenital heart defects and pulmonary hypertension were randomly divided into perfused group (n=12) and control group (n=12). Oxygen index, alveolar-arterial O2gradient, serum levels of malondialchehyche (MDA), interleukin (IL)-6, -8, -10, soluble intercellular adhesion molecule-1(slCAM-1), and P-selectin were measured before commencement and serially for 48 hours after termination of bypass.Results Oxygenation values were better preserved in the perfused group than in the control group. The serum levels of IL-6 increased immediately after CPB in both groups and returned to baseline at 48 hours after CPB, but it was restored faster and earlier in the perfused group. The serum levels of IL-8, slCAM-1, and MDA remained at baseline at each point after CPB in the perfused group and elevated significantly immediately after CPB in the control group, except for sICAM-1.The serum level of IL-10 increased immediately after CPB and decreased to baseline at 48 hours after CPB in both groups, but the IL-10 level in the perfused group was significantly higher than in the control group at 12 hours after CPB.The serum P-selectin levels in the control group immediately after CPB were significantly higher than prebypass levels.Moreover, there were no significant differences in postoperative clinical characters, except for the intubated time.Conclusion In infants with congenital heart defects, pulmonary perfusion with hypothermic HTK solution during cardiopulmonary bypass could ameliorate lung function and reduce the inflammatory response.

  15. Hypoxia-inducible factor-1 alpha regulates the role of vascular endothelial growth factor on pulmonary arteries of rats with hypoxia-induced pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    李启芳; 戴爱国

    2004-01-01

    Background Hypoxia-inducible factor-1α (HIF-1α) is one of the pivotal mediators in the response of lungs to decreased oxygen availability, and increasingly has been implicated in the pathogenesis of pulmonary hypertension. Vascular endothelial growth factor (VEGF), a downstream target gene of HIF-1α, plays an important role in the pathogenesis of hypoxic pulmonary hypertension and hypoxic pulmonary artery remodelling. In this study, we investigated the dynamic expression of HIF-1α and VEGF in pulmonary artery of rats with hypoxia-induced pulmonary hypertension. Methods Forty male Wistar rats were exposed to hypoxia for 0, 3, 7, 14 or 21 days. Mean pulmonary arterial pressure (mPAP), vessel morphometry and right ventricle hypertrophy index (RVHI) were estimated. Lungs were inflated and fixed for in situ hybridisation and immunohistochemistry. Results mPAP values were significantly higher than the control values after 7days of hypoxia [(18.4±0.4) mmHg, P<0.05]. RVHI developed significantly after 14 days of hypoxia. Expression of HIF-1α protein increased in pulmonary arterial tunica intima of all hypoxic rats. In pulmonary arterial tunica media, HIF-1α protein was markedly increased by day 3 (0.20±0.02, P<0.05), reached the peak by day 7, then declined after day 14 of hypoxia. HIF-1α mRNA increased significantly after day 14 of hypoxia (0.20±0.02, P<0.05). VEGF protein began to increase markedly after day 7 of hypoxia, reaching its peak around day 14 of hypoxia (0.15±0.02, P<0.05). VEGF mRNA began to increase after day 7 of hypoxia, then remained more or less stable from day 7 onwards. VEGF mRNA is located mainly in tunica intima and tunica media, whereas VEGF protein is located predominantly in tunica intima. Linear analysis showed that HIF-1α mRNA, VEGF and mPAP were correlated with hypoxic pulmonary artery remodelling. HIF-1α mRNA was positively correlated with VEGF mRNA and protein (P<0.01). Conclusion HIF-1α and VEGF are both involved in the

  16. The role of modern imaging techniques in the diagnosis of malposition of the branch pulmonary arteries and possible association with microdeletion 22q11.2.

    Science.gov (United States)

    Cuturilo, Goran; Drakulic, Danijela; Krstic, Aleksandar; Gradinac, Marija; Ilisic, Tamara; Parezanovic, Vojislav; Milivojevic, Milena; Stevanovic, Milena; Jovanovic, Ida

    2013-04-01

    Malposition of the branch pulmonary arteries is a rare malformation with two forms. In the typical form, pulmonary arteries cross each other as they proceed to their respective lungs. The “lesser form” is characterised by the left pulmonary artery ostium lying directly superior to the ostium of the right pulmonary artery, without crossing of the branch pulmonary arteries. Malposition of the branch pulmonary arteries is often associated with other congenital heart defects and extracardiac anomalies, as well as with 22q11.2 microdeletion. We report three infants with crossed pulmonary arteries and one adolescent with “lesser form” of the malformation. The results suggest that diagnosis of malposition of the branch pulmonary arteries could be challenging if based solely on echocardiography, whereas modern imaging technologies such as contrast computed tomography and magnetic resonance angiography provide reliable establishment of diagnosis. In addition, we performed the first molecular characterisation of the 22q11.2 region among patients with malposition of the branch pulmonary arteries and revealed a 3-megabase deletion in two out of four patients

  17. /sup 111/In-chloride thorax scintigraphic study for demonstration of pulmonary edema: Canine model

    Energy Technology Data Exchange (ETDEWEB)

    Shih Weijen; Deland, F.H.; Simmons, G.H.; Coupa, J.; Domstad, P.A.; Lee Luyuan

    1986-04-01

    Pulmonary edema induced by injections of oleic acid was demonstrated using /sup 111/In-chloride lung/heart image in five anesthetized dogs. The anesthetized dogs were positioned under a gamma camera interfaced to a computer. After /sup 111/In-chloride IV injections, dynamic data were recorded at 1 frame/min before and after induced pulmonary edema. The computer generated the curve of the lung-to-heart activity ratio; the ratio rose from 0.5-0.6 at baseline to 0.85-1.5 at th end of the study in the dogs. At the end of each study, the removed lungs demonstrated edematous fluid, and a few red blood cells were seen in the alveoli. The results indicating pulmoinary edema exhibited in the /sup 111/In-chloride lung-to-heart ratio obtained from a computerized gamma camera were compatable with the pathological results. Since /sup 111/In-chloride instantly binds to plasma transferrin, an excellent intravascular imaging agent, leakage of this tracer into alveoli indicates loss of membranous wall competence. Pulmonary edema, as in the adult respiratory distress syndrome, is sometimes difficult to diagnose; and this /sup 111/In-chloride lung-to-heart imaging technique may be potentially useful.

  18. Comparative Transcriptome Analysis Identifies CCDC80 as a Novel Gene Associated with Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Yuhei eNishimura

    2016-06-01

    Full Text Available Pulmonary arterial hypertension (PAH is a heterogeneous disorder associated with a progressive increase in pulmonary artery resistance and pressure. Although various therapies have been developed, the 5-year survival rate of PAH patients remains low. There is thus an important need to identify novel genes that are commonly dysregulated in PAH of various etiologies and could be used as biomarkers and/or therapeutic targets. In this study, we performed comparative transcriptome analysis of five mammalian PAH datasets downloaded from a public database. We identified 228 differentially expressed genes (DEGs from a rat PAH model caused by inhibition of vascular endothelial growth factor receptor under hypoxic conditions, 379 DEGs from a mouse PAH model associated with systemic sclerosis, 850 DEGs from a mouse PAH model associated with schistosomiasis, 1598 DEGs from one cohort of human PAH patients, and 4260 DEGs from a second cohort of human PAH patients. Gene-by-gene comparison identified four genes that were differentially upregulated or downregulated in parallel in all five sets of DEGs. Expression of coiled-coil domain containing 80 (CCDC80 and anterior gradient 2 genes was significantly increased in the five datasets, whereas expression of SMAD family member 6 and granzyme A was significantly decreased. Weighted gene co-expression network analysis revealed a connection between CCDC80 and collagen type I alpha 1 (COL1A1 expression. To validate the function of CCDC80 in vivo, we knocked out ccdc80 in zebrafish using the clustered regularly interspaced short palindromic repeats (CRISPR/Cas9 system. In vivo imaging of zebrafish expressing a fluorescent protein in endothelial cells showed that ccdc80 deletion significantly increased the diameter of the ventral artery, a vessel supplying blood to the gills. We also demonstrated that expression of col1a1 and endothelin-1 mRNA was significantly decreased in the ccdc80-knockout zebrafish. Finally, we

  19. Systemic-pulmonary artery shunts in infants: modified Blalock-Taussig and central shunt procedures.

    Science.gov (United States)

    Brooks, Andre

    2014-01-01

    Access is gained through a midline sternotomy, the thymus partially excised and the superior part of the pericardium is opened. The innominate vein is retracted and the innominate artery is mobilized up to the bifurcation. The aorta is retracted to the left, the superior vena cavae to the right and the right atrial appendage inferiorly. The adventitia around the right pulmonary artery (PA) is dissected, taking care to incise the bulky pericardial reflection between the superior vena cavae and the trachea. Heparin is administrated. An occlusive clamp is applied to the right PA to test for haemodynamic tolerance prior to proceeding with the interposition of a suitable size artificial vascular prosthesis, based on the weight of the patient, between the innominate artery, or proximal subclavian artery and the right PA. Alternatively, if a sufficient main PA is present and adequate flow from a patent ductus arteriosus an end-to-side interposition shunt may be constructed between the ascending aorta and the main PA, provided the patient is stable with the test occlusion of the main PA. The management of the patent arterial ductus depends on whether or not there is forward flow through the PA.

  20. Deciphering the genetic and modular connections between coronary heart disease, idiopathic pulmonary arterial hypertension and pulmonary heart disease.

    Science.gov (United States)

    Yuan, Ye; Zhang, Yingying; Zhang, Xiaoxu; Yu, Yanan; Li, Bing; Wang, Pengqian; Li, Haixia; Zhao, Yijun; Shen, Chunti; Wang, Zhong

    2016-07-01

    Coronary heart disease (CHD), idiopathic pulmonary arterial hypertension (IPAH) and pulmonary heart disease (PHD) are circulatory system diseases that may simultaneously emerge in a patient and they are often treated together in clinical practice. However, the molecular mechanisms connecting these three diseases remain unclear. In order to determine the multidimensional characteristic correlations between these three diseases based on genomic networks to aid in medical decision-making, genes from the Online Mendelian Inheritance in Man database were obtained, and applied network construction and modularized analysis were conducted. Functional enrichment analysis was conducted to explore the associations between overlapping genes, modules and pathways. A total of 29 overlapping genes and 3 common modules were identifed for the 3 diseases. Glycosphingolipid biosynthesis and the arachidonic acid metabolism are common pathways, and the biosynthetic process is suggested to be the major function involved in the three diseases. The current study reported, to the best of our knowledge for the first time, the role of glycosphingolipid biosynthesis in IPAH and PHD. The present study provided an improved understanding of the pathological mechanisms underlying CHD, IPAH and PHD. The overlapping genes, modules and pathways suggest novel areas for further research, and drug targets. The observations of the current study additionally suggest that drug indications can be broadened because of the presence of common targets.

  1. Measurements of Pulmonary Artery Size for Assessment of Pulmonary Hypertension by Cardiovascular Magnetic Resonance and Clinical Application

    Directory of Open Access Journals (Sweden)

    Fan YANG

    2017-02-01

    Full Text Available Background and objective Pulmonary hypertension (PH often leads to dilatation of main pulmonary artery (MPA. MPA measurements can be used to predict PH. This aim of this study is to investigate power of MPA vessel indices, which are acquired from cardiovascular magnetic resonance, to evaluate PH. Methods Cardiovascular-magnetic-resonance-determined parameters of MPA were acquired and calculated in 83 PH patients, whose diagnosis were confirmed with right heart catheterization and 49 healthy volunteers; these parameters included MPA diameter (DPA, ratio of DPA and ascending aorta diameter (DPA/DAo, max mean diameter (MDmax, min mean diameter (MDmin, fraction transverse diameter (fTD, fraction longitudinal diameter (fLD, and distensibility. Results Compared with control group, DPA, DPA/DAo, MDmax, and MDmin were significantly higher in patients with PH (P28.4 mm, and MDmax>32.4 mm (area under the curve, AUC=0.979, 0.981 showed best performance in predicting PH, yielding highest specificity at 100%. Conclusion Noninvasive cardiovascular-magnetic-resonance-derived MPA measurements provide excellent and practical reference in clinical settings for detecting PH.

  2. Effect of Feikangning composition in improving the pulmonary function in patients with COPD merged with pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    Li-Yun Wang

    2016-01-01

    Objective:To explore the effect of Feikangning composition in improving the pulmonary function in patients with COPD merged with pulmonary arterial hypertension (PAH), and observe the changes of ET-1 and D-D levels before and after treatment.Methods:A total of 52 patients with COPD merged with PAH who were admitted in our hospital were included in the study and randomized into the treatment group (n=31) and the control group (n=21). The patients in the two groups were given routine treatments after admission. On this basis, the patients in the treatment group were given Feikangning composition. ET-1 and D-D levels before treatment and 1 month after treatment in the two groups were detected. FEV1, FVC, FEV1%, mPAP, TPR, and SteO2 before and after treatment in the two groups were compared. Results:FEV1, FVC, and FEV1% after treatment in the treatment group were significantly higher than those in the control group. PAP and SteO2 after treatment in the treatment group were significantly higher than those in the control group, while TPR was significantly lower than that in the control group. ET-1 and D-D levels after treatment in the treatment group were significantly lower than those in the control group.Conclusions: Feikangning composition can effectively improve the ventilation function in patients with COPD merged with PAH, and regulate the endothelin balance, with a significant efficacy.

  3. How Is Pulmonary Hypertension Treated?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Pulmonary Hypertension Treated? Pulmonary hypertension (PH) has no cure. However, ... Types of Pulmonary Hypertension." ) Group 1 Pulmonary Arterial Hypertension Group 1 pulmonary arterial hypertension (PAH) includes PH ...

  4. Supplementation of iron in pulmonary hypertension: Rationale and design of a phase II clinical trial in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Howard, Luke S G E; Watson, Geoffrey M J; Wharton, John; Rhodes, Christopher J; Chan, Kakit; Khengar, Rajeshree; Robbins, Peter A; Kiely, David G; Condliffe, Robin; Elliott, Charlie A; Pepke-Zaba, Joanna; Sheares, Karen; Morrell, Nicholas W; Davies, Rachel; Ashby, Deborah; Gibbs, J Simon R; Wilkins, Martin R

    2013-01-01

    Our aim is to assess the safety and potential clinical benefit of intravenous iron (Ferinject) infusion in iron deficient patients with idiopathic pulmonary arterial hypertension (IPAH). Iron deficiency in the absence of anemia (1) is common in patients with IPAH; (2) is associated with inappropriately raised levels of hepcidin, the key regulator of iron homeostasis; and (3) correlates with disease severity and worse clinical outcomes. Oral iron absorption may be impeded by reduced absorption due to elevated hepcidin levels. The safety and benefits of parenteral iron replacement in IPAH are unknown. Supplementation of Iron in Pulmonary Hypertension (SIPHON) is a Phase II, multicenter, double-blind, randomized, placebo-controlled, crossover clinical trial of iron in IPAH. At least 60 patients will be randomized to intravenous ferric carboxymaltose (Ferinject) or saline placebo with a crossover point after 12 weeks of treatment. The primary outcome will be the change in resting pulmonary vascular resistance from baseline at 12 weeks, measured by cardiac catheterization. Secondary measures include resting and exercise hemodynamics and exercise performance from serial bicycle incremental and endurance cardiopulmonary exercise tests. Other secondary measurements include serum iron indices, 6-Minute Walk Distance, WHO functional class, quality of life score, N-terminal pro-brain natriuretic peptide (NT-proBNP), and cardiac anatomy and function from cardiac magnetic resonance. We propose that intravenous iron replacement will improve hemodynamics and clinical outcomes in IPAH. If the data supports a potentially useful therapeutic effect and suggest this drug is safe, the study will be used to power a Phase III study to address efficacy.

  5. Interleukin-6 as a Potential Therapeutic Target for Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Yoshiaki Furuya

    2010-01-01

    Full Text Available Interleukin-6 (IL-6 is a pleiotropic cytokine with a wide range of biologic activities in immune regulation, hematopoiesis, inflammation, and oncogenesis. Recent accumulating evidence indicates a pathologic role for IL-6 in promoting proliferation of both smooth muscle and endothelial cells in the pulmonary arterioles, resulting in development of pulmonary arterial hypertension (PAH. Here, we describe a patient with mixed connective tissue disease and severe, refractory PAH. Her functional activity and hemodynamic parameters dramatically responded to tocilizumab, a humanized monoclonal antibody to human IL-6 receptor, which was aimed at treating multicentric Castleman's disease. It appears that IL-6 blockade may hold promise as an adjunct drug in treatment of PAH in idiopathic form as well as in association with connective tissue disease.

  6. [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.

  7. Safety and tolerability considerations in the use of sildenafil for children with pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Dodgen AL

    2015-12-01

    Full Text Available Andrew L Dodgen,1 Kevin D Hill1,2 1Department of Pediatrics, Duke University Medical Center, 2Duke Clinical Research Institute, Durham, NC, USA Abstract: Sildenafil is a phosphodiesterase type-5 inhibitor approved for treatment of pulmonary arterial hypertension (PAH in adults. Data from pediatric trials demonstrate a similar acute safety profile to the adult population but have raised concerns regarding the safety of long-term use in children. Interpretation of these trials remains controversial with major regulatory agencies differing in their recommendations – the US Food and Drug Administration recommends against the use of sildenafil for treatment of PAH in children, while the European Medicines Agency supports its use at “low doses”. Here, we review the available pediatric data regarding dosing, acute, and long-term safety and efficacy of sildenafil for the treatment of PAH in children. Keywords: phosphodiesterase inhibitor, pulmonary vasodilator, STARTS trials

  8. Focal adhesion kinase antisense oligodeoxynucleotides inhibit human pulmonary artery smooth muscle cells proliferation and promote human pulmonary artery smooth muscle cells apoptosis

    Institute of Scientific and Technical Information of China (English)

    LIN Chun-long; ZHANG Zhen-xiang; XU Yong-jian; NI Wang; CHEN Shi-xin

    2005-01-01

    Background Pulmonary artery smooth muscle cell (PASMC) proliferation plays an important role in pulmonary vessel structural remodelling. At present, the mechanisms related to proliferation of PASMCs are not clear. Focal adhesion kinase (FAK) is a widely expressed nonreceptor protein tyrosine kinase. Recent research indicates that FAK is implicated in signalling pathways which regulate cytoskeletal organization, adhesion, migration, survival and proliferation of cells. Furthermore, there are no reports about the role of FAK in human pulmonary artery smooth muscle cells (HPASMCs). We investigated whether FAK takes part in the intracellular signalling pathway involved in HPASMCs proliferation and apoptosis, by using antisense oligodeoxynucleotides (ODNs) to selectively suppress the expression of FAK protein.Methods Cultured HPASMCs stimulated by fibronectin (40 μg/ml) were passively transfected with ODNs, sense FAK, mismatch sense and antisense-FAK respectively. Expression of FAK, Jun NH2-terminal kinase (JNK), cyclin-dependent kinase 2 (CDK 2) and caspase-3 proteins were detected by immunoprecipitation and Western blots. Cell cycle and cell apoptosis were analysed by flow cytometry. In addition, cytoplasmic FAK expression was detected by immunocytochemical staining.Results When compared with mismatch sense group, the protein expressions of FAK, JNK and CDK 2 in HPASMCs decreased in antisense-FAK ODNs group and increased in sense-FAK ODNs group significantly. Caspase-3 expression upregulated in HPASMCs when treated with antisense ODNs and downregulated when treated with sense ODNs. When compared with mismatch sense ODNs group, the proportion of cells at G1 phase decreased significantly in sense ODNs group, while the proportion of cells at S phase increased significantly. In contrast, compared with mismatch sense ODNs group, the proportion of cells at G1 phase was increased significantly in antisense-FAK ODNs group. The level of cell apoptosis in antisense-FAK group

  9. The effects of oral nafazatrom (= BAY g 6575) on canine coronary artery thrombosis and myocardial ischemia.

    Science.gov (United States)

    Fiedler, V B

    1983-01-01

    The in-vivo effects of the new antithrombotic compound nafazatrom on experimental thrombosis of the left circumflex coronary artery, on hemodynamics and on ultimate infarct size were studied in pentobarbital-anesthetized, open-chest dogs. Coronary artery thrombosis was induced by low amperage stimulation (150 microA, DC for 6 hr) of the circumflex artery intimal lining. The effects of oral pretreatment of 1%-Tylose suspension as drug diluent and 5 mg/kg nafazatrom plus vehicle were determined. Both agents were administered twice a day before onset of current stimulation. In the drug vehicle group, coronary thrombosis caused severe hemodynamic alterations, e.g. blood pressure and left ventricular pressure decrease, as well as reduction in the LV dP/dtmax associated with increases in end-diastolic filling pressure and heart rate. Time to coronary artery occlusion was delayed by nafazatrom (5.2 +/- 1.1 vs 3.1 +/- 0.4 hr, p less than 0.05). Smaller blood pressure and LV dP/dtmax reductions and minor heart rate and filling pressure increases around the time of thrombus formation suggested cardioprotection with the drug. Smaller R wave changes and S-T segment elevation indicated minor ischemia at the time of occlusive coronary artery occlusion in nafazatrom-treated hearts (24 +/- 0.5 vs 72 +/- 7% ST segment elevation, p less than 0.01). Thrombus wet weight was 18.4 +/- 2.6 mg in the nafazatrom group, but 63.7 +/- 3.1 mg in controls (p less than 0.01). Thus, ultimate infarct size was smaller in nafazatrom-treated hearts as related to left ventricular mass (8.4 +/- 1.4 vs 32.3 +/- 3.1%, p less than 0.02) or to the occluded artery perfusion area at risk for infarction (16 +/- 3.4 vs 53 +/- 6.2%, p less than 0.05). No ex-vivo effect of nafazatrom on collagen-induced platelet aggregation was observed. These results may indicate efficacy of the drug in prevention of acute coronary artery disease as one cause of ischemic jeopardy of the myocardium and/or therapeutic value in

  10. Noninvasive assessment of pulmonary arterial pressure by krypton-81m right cardiac ventriculography in patients with chronic obstructive pulmonary disease

    Energy Technology Data Exchange (ETDEWEB)

    Yamaoka, Shimpachi; Kuriyama, Takanobu; Hirai, Masashi; Nishimura, Kouichi; Kuno, Kenshi; Yonekura, Yoshiharu

    1987-10-01

    In twenty patients with COPD, the following pulmonary function test values were obtained (mean +- SD): FEV/sub 1.0/ = 1070 +- 490 ml;FEV/sub 1.0/ % = 47.7 +- 8.9 %;PaO/sub 2/ = 65.8 +- 8.4 torr;PaCO/sub 2/ = 39.7 +- 4.4 torr. All patients underwent right heart catheterization and pulmonary arterial mean pressures (PAMP) were obtained. The PAMP value was 20.2 +- 4.1 torr (range 12 - 28), RVEF was 52.2 +- 8.4 % (range 31 - 63) and RVRFR was 25.5 +- 4.7 %100 msec (range 18 - 34). The PAMP negatively correlated with the RVEF (r = -0.69, p < 0.001) and RVRFR (r = -0.82, p < 0.001). In ten healthy male subjects, the RVEF was 56.6 +- 5.1 % (range 50 - 64) and RVRFR was 37.7 +- 3.0 %100 msec (range 33 - 42). The lower limit of normal for the RVEF was regarded as 46.4 % and that of the RVRFR as 31.7 %100 msec;these are the values two standard deviations below the means. Nine of the twenty patients has a PAMP value of more than 20 torr (the sine qua non of cor pulmonale). Five of these nine patients had RVEF values less than 46.4 % and all of them had RVRFR values of less than 31.7 %100 msec. On the other hand, all of the remaining eleven patients (PAMP 20 torr or less) had RVEF values within the normal range, but ten of the eleven had RVRFR values of less than 31.7 %100 msec. Therefore, the RVRFR value, which showed excellent correlation with the PAMP, provided a noninvasive assessment of pulmonary arterial pressure with good sensitivity in detecting elevated pressure. The RVEF value supplemented the RVRFR with good specificity for detection of elevation of pressure;that is, it eliminated false positive results. (J.P.N.)

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

    Institute of Scientific and Technical Information of China (English)

    LIU Jia-ming; SHEN Jian-xiong; ZHANG Jian-guo; ZHAO Hong; LI Shu-gang; ZHAO Yu; QIU Giu-xing

    2012-01-01

    Background 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.Methods This study involved scoliotic patients with moderate or severe pulmonary dysfunction (forced vital capacity <60%) who underwent surgical 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.Results 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

  12. Right heart function during simulated altitude in patients with pulmonary arterial hypertension

    Science.gov (United States)

    Seccombe, Leigh M; Chow, Vincent; Zhao, Wei; Lau, Edmund M T; Rogers, Peter G; Ng, Austin C C; Veitch, Elizabeth M; Peters, Matthew J; Kritharides, Leonard

    2017-01-01

    Objective Patients with pulmonary arterial hypertension (PAH) are often recommended supplemental oxygen for altitude travel due to the possible deleterious effects of hypoxia on pulmonary haemodynamics and right heart function. This includes commercial aircraft travel; however, the direct effects and potential risks are unknown. Methods Doppler echocardiography and gas exchange measures were investigated in group 1 patients with PAH and healthy patients at rest breathing room air and while breathing 15.1% oxygen, at rest for 20 min and during mild exertion. Results The 14 patients with PAH studied were clinically stable on PAH-specific therapy, with functional class II (n=11) and III (n=3) symptoms when tested. Measures of right ventricular size and function were significantly different in the PAH group at baseline as compared to 7 healthy patients (p<0.04). There was no evidence of progressive right ventricular deterioration during hypoxia at rest or under exertion. Pulmonary arterial systolic pressure (PASP) increased in both groups during hypoxia (p<0.01). PASP in hypoxia correlated strongly with baseline PASP (p<0.01). Pressure of arterial oxygen correlated with PASP in hypoxia (p<0.03) but not at baseline, with three patients with PAH experiencing significant desaturation. The duration and extent of hypoxia in this study was tolerated well despite a mild increase in symptoms of breathlessness (p<0.01). Conclusions Non-invasive measures of right heart function in group 1 patients with PAH on vasodilator treatment demonstrated a predictable rise in PASP during short-term simulated hypoxia that was not associated with a deterioration in right heart function. PMID:28123765

  13. Role of pulmonary artery reactivity and nitric oxide in injury and inflammation following lung contusion.

    Science.gov (United States)

    Lakshminrusimha, Satyan; Suresh, Madathilparambil V; Knight, Paul R; Gugino, Sylvia F; Davidson, Bruce A; Helinski, Jadwiga D; Nielsen, Lori C; Russell, James A; Yu, Bi; Zeng, Lixia; Pennathur, Subramaniam; Raghavendran, Krishnan

    2013-03-01

    The mechanisms contributing to hypoxia in lung contusion (LC) remain unclear and not temporally associated with the peak onset of acute inflammation. We investigated the role of oxidative stress in alteration of pulmonary arterial (PA) reactivity following LC. In addition, the role of antioxidants in reversing this process was examined. PaO2 and PA reactivity were measured in rats subjected to bilateral LC. Rings were pretreated with a nitric oxide synthase (NOS) inhibitor, L-nitro arginine (10(-3) M), or PEG-superoxide dismutase (SOD) and PEG-catalase (CAT), or both (L-nitro arginine + SOD/CAT). Rings were constricted with norepinephrine and relaxed with an NOS agonist (A23187) or NO donor (SNAP [S-nitrosyl amino penicillamine]). Immunochemical and mass spectrometric quantification for nitrotyrosine was performed. Rats were hypoxemic at 4 h after contusion compared with controls, but recovered by 24 h (PaO(2)/FIO(2) ratio: baseline, 443 ± 28; 4 h, 288 ± 46; and 24 h, 417 ± 23). Pulmonary arterial constriction to NOS inhibition and relaxation to A23187 were impaired 4 h after LC. Pulmonary arterial relaxation to SNAP was decreased at 4 and 24 h after LC. These alterations in PA reactivity were reversed by SOD/CAT pretreatment. SOD1 and 2 mRNA were upregulated, and soluble guanylyl cyclase mRNA was downregulated 24 h after LC. Immunohistochemistry and mass spectrometry revealed that levels of 3-nitrotyrosine were increased markedly at 4 h following LC consistent with superoxide generation and formation of peroxynitrite. Collectively, these data suggest that consumption of NO due to excess superoxide resulting in peroxynitrite formation leads to diminished vascular reactivity following LC.

  14. 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.)

  15. Clinical utility of treprostinil in the treatment of pulmonary arterial hypertension: an evidence-based review

    Directory of Open Access Journals (Sweden)

    Buckley MS

    2014-06-01

    Full Text Available Mitchell S Buckley,1 Andrew J Berry,1 Nadine H Kazem,2 Shardool A Patel,3 Paul A Librodo41Department of Pharmacy, Banner Good Samaritan Medical Center, Phoenix, AZ, USA; 2Department of Pharmacy, St Joseph’s Hospital and Medical Center, Phoenix, AZ, USA; 3Department of Pharmacy, Banner Estrella Medical Center, Phoenix, AZ, USA; 4Department of Pharmacy, San Francisco VA Medical Center, San Francisco, CA, USAAbstract: Pulmonary arterial hypertension (PAH remains a progressive disease without a cure, despite the development of several treatment options over the past several decades. Its management strategy consists of the endothelin receptor antagonists (ambrisentan, bosentan, macitentan, phosphodiesterase-5 inhibitors (sildenafil, tadalafil, vardenafil, and prostacyclin analogs (epoprostenol, treprostinil, iloprost. Treprostinil, a stable prostacyclin analog, displays vasodilatory effects in the pulmonary vasculature, as well as antiplatelet aggregation properties. Clinical practice guidelines recommend oral endothelin receptor antagonist or phosphodiesterase inhibitor therapy in mild to moderate PAH. Epoprostenol is specifically suggested as first-line therapy in moderate to severe PAH patients (ie, World Health Organization/New York Heart Association functional class III–IV. However, treprostinil may be an alternative option in these severe PAH patients. The longer half-life and stability at room temperature with treprostinil may be associated with lower risk of pulmonary hemodynamic worsening as a result of abrupt infusion discontinuation and less frequent drug preparation. These characteristics make treprostinil an attractive alternative to continuous infusion of epoprostenol, due to convenience and patient safety. The purpose of this review is to evaluate the safety and efficacy of continuous infusion of treprostinil as well as the inhaled and oral routes of administration in PAH.Keywords: treprostinil, prostacyclin, pulmonary arterial

  16. Circulating ghrelin was negatively correlated with pulmonary arterial pressure in atrial septal defect patients

    Institute of Scientific and Technical Information of China (English)

    LI Zhao-feng; ZHOU Da-xin; PAN Wen-zhi; ZHANG Lei; GE Jun-bo

    2013-01-01

    Background Ghrelin was found to attenuate the magnitude of pulmonary arterial hypertension and pulmonary vascular remodeling in rats.The objective of this study was to explore the fasting plasma ghrelin level and the relationships between ghrelin and pulmonary arterial pressure (PAP) in atrial septal defect (ASD) patients with pulmonary arterial hypertension (PAH).Methods Fasting plasma ghrelin,obestatin,and insulin levels were measured by enzyme linked immunosorbent assay (ELISA) method in ASD patients with or without PAH according to the manufacturer's instructions.Insulin resistance was calculated by the homeostasis model of assessment for insulin resistance (HOMA-IR) approach,calculated as fasting insulin (microunits/ml)× fasting blood glucose (mmol/L)/22.5.Comparisons between the parameters of patients with PAH and those of patients with normal PAP were performed with an unpaired Student's t test.The relationships between ghrelin and various clinical parameters were examined by bivariate correlations and multiple regression analysis.Results We found that the fasting plasma ghrelin level and the ratio of ghrelin to obestatin were significantly lower in the PAH group compared with the control group ((582.4±12.8) pg/ml vs.(1045.2±95.5) pg/ml,P <0.05 and 30.5±4.9 vs.70.0±9.7,P <0.01).The fasting plasma obestatin level was higher in the PAH group compared with the control group,but the difference between them was not significant ((23.2±3.1) pg/ml vs.(16.3±1.6) pg/ml,P >0.05).In a multiple regression model analysis,only mean PAP was an independent predictor of ghrelin and the ratio of ghrelin to obestatin (standardized coefficient=0.737,P <0.001 and standardized coefficient=-0.588,P=0.006,respectively).Conclusion Ghrelin is negatively correlated with mean PAP and this suggests that circulating ghrelin might predict the severity of pulmonary hypertension in ASD patients with PAH.

  17. Respiratory and limb muscle dysfunction in pulmonary arterial hypertension: a role for exercise training?

    Science.gov (United States)

    Panagiotou, Marios; Peacock, Andrew J; Johnson, Martin K

    2015-09-01

    Respiratory and limb muscle dysfunction is emerging as an important pathophysiological abnormality in pulmonary arterial hypertension (PAH). Muscle abnormalities appear to occur frequently and promote dyspnea, fatigue, and exercise limitation in patients with PAH. Preliminary data suggest that targeted muscle training may be of benefit, although further evidence is required to consolidate these findings into specific recommendations for exercise training in patients with PAH. This article reviews the current evidence on prevalence, risk factors, and implications of respiratory and limb muscle dysfunction in patients with PAH. It also reviews the impact of exercise rehabilitation on morphologic, metabolic, and functional muscle profile and outcomes in PAH. Future research priorities are highlighted.

  18. Long-term effects with ambrisentan monotherapy in patients with pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    文莉

    2014-01-01

    Objective To investigate long-term efficacy and safety of ambrisentan monotherapy in patients with pulmonary arterial hypertension(PAH).Methods Patients with PAH who received 2.5 mg or 5 mg of ambrisentan once daily between July 10,2011 and August 30,2012for at least 6 months were enrolled.The efficacy endpoints were changes in exercise capacity,World Health Organization(WHO)functional class and N-terminal probrain natriuretic peptide(NT-pro BNP)level,echocardiographic parameters.The safety endpoint was the safety of long-term ambrisentan administration,as defined by

  19. Simple mechanical thrombectomy with intrapulmonary arterial thrombolysis in pulmonary thromboembolism:a small case series

    Institute of Scientific and Technical Information of China (English)

    Khurshid Ahmed; Muhammad Munawar; Dian Andina Munawar; Beny Hartono

    2014-01-01

    Pulmonary thromboembolism (PTE) is a life-threatening condition with a high early mortality rate caused by acute right ventricular failure and cardiogenic shock. We report a series of three patients who presented with acute and subacute submassive PTE. They were suc-cessfully treated by simple catheter-based mechanical thrombectomy and intrapulmonary arterial thrombolysis. Mechanical fragmentation and aspiration of thrombus was performed by commonly used J-wire, multi-purpose and Judkin Right guiding catheters and this obviated the need of specific thrombectomy devices.

  20. Axillary artery transection and bilateral pulmonary embolism after anterior shoulder dislocation: case report

    Directory of Open Access Journals (Sweden)

    Leclerc Betty

    2017-01-01

    Full Text Available Introduction: Anterior shoulder dislocation can be associated with vascular and neurological complications. However, axillary artery injury associated with shoulder dislocation is rare and extremely rare without bone fracture. An early diagnosis of these complications allows predicting long-term functional outcomes. Methods: This article reports the case of a 66-year-old patient who presented an anterior shoulder dislocation after a ski fall without any neurological dysfunction or pulse deficit. Results: The first reduction attempts were unsuccessful and during the new attempt, we observed a hematoma. A CT scan showed a disruption of the axillary artery and a bilateral pulmonary embolism. Conclusion: Neurovascular injury must be systematically sought before and after reduction, and a multidisciplinary approach is always necessary.

  1. INCREASED EXPRESSION OF PDGF AND C-MYC GENES IN LUNGS AND PULMONARY ARTERIES OF PULMONARY HYPERTENSIVE RATS INDUCED BY HYPOXIA

    Institute of Scientific and Technical Information of China (English)

    蔡英年; 韩梅; 罗兰; 宋为; 周晓梅

    1996-01-01

    The role of growth factors and proto-oncogene in pulmonary vascular structural remodelling is not well known.The present study examined gene expression of plateler-derived growth factor(PDGF)-A and -B chain and proto-oncogene,c-myc,in lung tissue and pulmonary artery of rats exposed to hypoxia and compred to those levels of gene expression in normal rats.Normal lungs and pulmonary artery expressed PDGF-A chain transcripr of 1.7kb and PDGF-B chain transcript of 3.5 Kb. The c-myc transcript of 2.2 kb was expressed as well.After hypoxic exposure for 7 and 14 days mRNA levels of PDGF-B chain and c-myc were elevated significantly compared with those of control rats.PDGF-A chain mRNA increased after hypoxia for 7 days,and then declined.These results suggest that activation of sutocrine and /or paracrine is important in proliferation mechanism of pulmonary artery smooth muscle cells in bypoxic pulmonary hypertensive rats.

  2. Utility of intra-operative capnogram to detect branch pulmonary artery obstruction following total correction of tetralogy of fallot

    Directory of Open Access Journals (Sweden)

    Garg Rajnish

    2011-01-01

    Full Text Available Branch pulmonary artery obstruction is one of the prime reasons for re-operation in patients who have undergone repair for tetralogy of Fallot. Branch pulmonary artery obstruction may develop over a period of time due to dilation of right ventricular outflow tract or it may be caused by residual stenosis after inadequate repair. This may lead to differential lung perfusion causing morbidity. Intra-operative capnogram monitoring reveals ventilation−perfusion relationship. We report two cases where the capnogram helped the diagnosis and management of branch pulmonary artery obstruction. We found a redundant patch in the first and an extra length of the homograft in second case which led to the obstruction. However, but for the changes in the intraoperative capnogram, this condition may by far remain undiagnosed considering the fact that it does not produce hemodynamic changes but can lead to postoperative morbidity.

  3. 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.

  4. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner's syndrome.

    Science.gov (United States)

    David, Jane Jackie; Mohanlal, Smilu; Sankhe, Punam; Ghildiyal, Radha

    2016-01-01

    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).

  5. Myosin heavy chain 15 is associated with bovine pulmonary arterial pressure

    Science.gov (United States)

    Neary, Joseph M.; Lund, Gretchen K.; Holt, Timothy N.; Garry, Franklyn B.; Mohun, Timothy J.; Breckenridge, Ross A.

    2014-01-01

    Abstract Bovine pulmonary hypertension, brisket disease, causes significant morbidity and mortality at elevations above 2,000 m. Mean pulmonary arterial pressure (mPAP) is moderately heritable, with inheritance estimated to lie within a few major genes. Invasive mPAP measurement is currently the only tool available to identify cattle at risk of hypoxia-induced pulmonary hypertension. A genetic test could allow selection of cattle suitable for high altitude without the need for invasive testing. In this study we evaluated three candidate genes (myosin heavy chain 15 [MYH15], NADH dehydrogenase flavoprotein 2, and FK binding protein 1A) for association with mPAP in 166 yearling Angus bulls grazing at 2,182 m. The T allele (rs29016420) of MYH15 was linked to lower mPAP in a dominant manner (CC 47.2 ± 1.6 mmHg [mean ± standard error of the mean]; CT/TT 42.8 ± 0.7 mmHg; P = 0.02). The proportions of cattle with MYH15 CC, CT, and TT genotypes were 55%, 41%, and 4%, respectively. Given the high frequency of the deleterious allele, it is likely that the relative contribution of MYH15 polymorphisms to pulmonary hypertension is small, supporting previous predictions that the disease is polygenic. We evaluated allelic frequency of MYH15 in the Himalayan yak (Bos grunniens), a closely related species adapted to high altitude, and found 100% prevalence of T allele homozygosity. In summary, we identified a polymorphism in MYH15 significantly associated with mPAP. This finding may aid selection of cattle suitable for high altitude and contribute to understanding human hypoxia-induced pulmonary hypertension. PMID:25621163

  6. Myosin heavy chain 15 is associated with bovine pulmonary arterial pressure.

    Science.gov (United States)

    Neary, Marianne T; Neary, Joseph M; Lund, Gretchen K; Holt, Timothy N; Garry, Franklyn B; Mohun, Timothy J; Breckenridge, Ross A

    2014-09-01

    Bovine pulmonary hypertension, brisket disease, causes significant morbidity and mortality at elevations above 2,000 m. Mean pulmonary arterial pressure (mPAP) is moderately heritable, with inheritance estimated to lie within a few major genes. Invasive mPAP measurement is currently the only tool available to identify cattle at risk of hypoxia-induced pulmonary hypertension. A genetic test could allow selection of cattle suitable for high altitude without the need for invasive testing. In this study we evaluated three candidate genes (myosin heavy chain 15 [MYH15], NADH dehydrogenase flavoprotein 2, and FK binding protein 1A) for association with mPAP in 166 yearling Angus bulls grazing at 2,182 m. The T allele (rs29016420) of MYH15 was linked to lower mPAP in a dominant manner (CC 47.2 ± 1.6 mmHg [mean ± standard error of the mean]; CT/TT 42.8 ± 0.7 mmHg; P = 0.02). The proportions of cattle with MYH15 CC, CT, and TT genotypes were 55%, 41%, and 4%, respectively. Given the high frequency of the deleterious allele, it is likely that the relative contribution of MYH15 polymorphisms to pulmonary hypertension is small, supporting previous predictions that the disease is polygenic. We evaluated allelic frequency of MYH15 in the Himalayan yak (Bos grunniens), a closely related species adapted to high altitude, and found 100% prevalence of T allele homozygosity. In summary, we identified a polymorphism in MYH15 significantly associated with mPAP. This finding may aid selection of cattle suitable for high altitude and contribute to understanding human hypoxia-induced pulmonary hypertension.

  7. 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.

  8. 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.

  9. Vasoconstriction induced by ouabain in the canine coronary artery: contribution of adrenergic and nonadrenergic responses.

    Science.gov (United States)

    Cooke, J P; Shepherd, J T; Vanhoutte, P M

    1988-07-01

    Ouabain, when applied to rings of the left circumflex coronary artery of the dog (which contains both alpha 1-adrenoceptors leading to contraction and beta 1-adrenoceptors leading to relaxation) caused an initial contraction which peaked within 15 minutes and a later secondary increase in tension which peaked within 60 minutes. These contractions were prevented by Ca2+ removal or by verapamil. Adrenergic denervation with 6-hydroxydopamine did not affect the initial contraction. Thus it is due to a nonadrenergic effect of the glycoside. Since the secondary increase in tension was prevented by adrenergic denervation and prazosin, it is likely to be due to norepinephrine released from adrenergic nerves acting on alpha-adrenoceptors. This interpretation was confirmed by the finding that ouabain, after a latent period of about 35 minutes, augmented the output of 3H-norepinephrine from helical strips of the artery previously incubated with tritiated transmitter. In rings contracted with prostaglandin F2 alpha, ouabain reduced beta-adrenergic relaxations caused by isoproterenol or exogenous norepinephrine, but not those caused by sodium nitroprusside. Thus, in this artery, ouabain depresses the responses of the beta-adrenoceptors to the norepinephrine which it releases, thereby permitting the neurotransmitter to cause contraction by activating postjunctional alpha 1-adrenoceptors.

  10. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report

    DEFF Research Database (Denmark)

    Kristensen, T.; Kofoed, Klaus Fuglsang; Helqvist, S.;

    2008-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from...... the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion....... Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery...

  11. [Tunnelled central venous line-associated infections in patients with pulmonary arterial hypertension treated with intravenous prostacyclin].

    Science.gov (United States)

    Boucly, Athénaïs; O'Connell, Caroline; Savale, Laurent; O'Callaghan, Dermot S; Jaïs, Xavier; Montani, David; Humbert, Marc; Simonneau, Gérald; Sitbon, Olivier

    2016-01-01

    Intravenous prostacyclins are a valuable treatment for patients with severe pulmonary arterial hypertension, leading to improved exercise capacity, haemodynamics, quality of life and survival. Unfortunately, due to the short half-life of these drugs, they need to be administered continuously through central venous catheters. Despite aseptic technique, regular dressing changes, tunneled central venous catheters and patient education, patients are exposed to central venous catheter associated infections. These infections cause significant morbidity and mortality. The clinical presentation, microbiology, consequences and management of these central venous catheter associated infections in pulmonary arterial hypertension patients treated with intravenous prostacyclins are discussed.

  12. PULMONARY ARTERIAL DISEASE ASSOCIATED WITH RIGHT-SIDED CARDIAC HYPERTROPHY AND CONGESTIVE HEART FAILURE IN ZOO MAMMALS HOUSED AT 2,100 M ABOVE SEA LEVEL.

    Science.gov (United States)

    Juan-Sallés, Carles; Martínez, Liliana Sofía; Rosas-Rosas, Arely G; Parás, Alberto; Martínez, Osvaldo; Hernández, Alejandra; Garner, Michael M

    2015-12-01

    Subacute and chronic mountain sickness of humans and the related brisket disease of cattle are characterized by right-sided congestive heart failure in individuals living at high altitudes as a result of sustained hypoxic pulmonary hypertension. Adaptations to high altitude and disease resistance vary among species, breeds, and individuals. The authors conducted a retrospective survey of right-sided cardiac hypertrophy associated with pulmonary arterial hypertrophy or arteriosclerosis in zoo mammals housed at Africam Safari (Puebla, México), which is located at 2,100 m above sea level. Seventeen animals with detailed pathology records matched the study criterion. Included were 10 maras (Dolichotis patagonum), 2 cotton-top tamarins (Saguinus oedipus oedipus), 2 capybaras (Hydrochaeris hydrochaeris), and 1 case each of Bennet's wallaby (Macropus rufogriseus), nilgai antelope (Boselaphus tragocamelus), and scimitar-horned oryx (Oryx dammah). All had right-sided cardiac hypertrophy and a variety of arterial lesions restricted to the pulmonary circulation and causing arterial thickening with narrowing of the arterial lumen. Arterial lesions most often consisted of medial hypertrophy or hyperplasia of small and medium-sized pulmonary arteries. All maras also had single or multiple elevated plaques in the pulmonary arterial trunk consisting of fibrosis, accompanied by chondroid metaplasia in some cases. Both antelopes were juvenile and died with right-sided congestive heart failure associated with severe pulmonary arterial lesions. To the authors' knowledge, this is the first description of cardiac and pulmonary arterial disease in zoo mammals housed at high altitudes.

  13. The Use of Pulmonary Artery Catheter in Sepsis Patients: A Literature Review

    Science.gov (United States)

    Velissaris, Dimitrios; Karamouzos, Vasilios; Kotroni, Ioanna; Pierrakos, Charalampos; Karanikolas, Menelaos

    2016-01-01

    This article was to review the literature regarding the use of the pulmonary artery catheter (PAC) in the management of patients with sepsis and septic shock. A PubMed search was conducted in order to identify publications evaluating the use of PAC as a tool for management and therapeutic guidance in patients with sepsis. The bibliographies of all identified publications were reviewed for additional relevant references. Much information is identified in the literature regarding the indications for pulmonary artery catheterization in the assessment and treatment of patients with sepsis. Although the PAC has been widely used for many years, there is no clear benefit with regard to outcome, and there is controversy regarding its use. It is not clear that use of the PAC contributes to reduced morbidity and mortality in patients with sepsis. The role of the PAC is becoming less clear, as newer, non-invasive techniques are developed for hemodynamic assessment of sepsis patients. Large, well-designed clinical trials are needed to better assess the role and potential benefit from use of the PAC in sepsis. PMID:27738477

  14. Pulmonary arterial hypertension: the burden of disease and impact on quality of life.

    Science.gov (United States)

    Delcroix, Marion; Howard, Luke

    2015-12-01

    Pulmonary arterial hypertension (PAH) is a debilitating disease that pervades all aspects of a patient's daily life. It is also increasingly acknowledged that the burden of PAH extends to older patients and carers. Until recently, the adverse effect of disease symptoms on the physical, emotional and social factors governing patient health-related quality of life (HRQoL) remained largely unrecognised. With a shift in therapeutic objectives to longer term improvements and HRQoL benefits, clinical trials now frequently include HRQoL measures as study end-points. Most HRQoL instruments used in patients with PAH are generic or non-disease-specific questionnaires and therefore may not accurately capture PAH disease burden. New PAH-specific HRQoL instruments currently undergoing validation include emPHasis-10 and Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT; Actelion Pharmaceuticals Ltd, Allschwil, Switzerland). Using various HRQoL measures, pharmacological therapies have been shown to improve HRQoL in patients with PAH. Patients also derive HRQoL benefits from nonpharmacological strategies, which include the emotional support provided by multidisciplinary care and support groups that is fundamental to patient wellbeing. Looking to the future, validated PAH-specific HRQoL instruments together with dedicated guidelines and procedures are essential to support the translation of HRQoL scores to the clinic, thus enabling a holistic treatment approach to the management of patients with PAH.

  15. Quercetin reverses experimental pulmonary arterial hypertension by modulating the TrkA pathway.

    Science.gov (United States)

    He, Yuanzhou; Cao, Xiaopei; Liu, Xiansheng; Li, Xiaochen; Xu, Yongjian; Liu, Jin; Shi, Jing

    2015-11-15

    Pulmonary arterial hypertension (PAH) is characterized by excessive proliferation, resistance to apoptosis, and increased migration of pulmonary artery smooth muscle cells (PASMCs). We hypothesized that quercetin exerts protective effects against this disease; thus, a chronic hypoxia model of PAH was generated using male Sprague-Dawley rats, which were treated with quercetin. In this model, quercetin prevented the development of PAH, right ventricular hypertrophy, and vascular remodeling after exposure to hypoxia. Quercetin inhibited PASMC proliferation and increased the apoptosis of PASMCs in vivo. In vitro, quercetin significantly inhibited hypoxia-induced PASMC proliferation, arrested cells in G1/G0 and inhibited cell migration in a dose-dependent manner. Moreover, our results showed that quercetin increased cyclin D1 protein levels and decreased the protein expression of cyclin B1 and Cdc2. Additionally, quercetin altered the Bax/Bcl-2 ratio and reduced MMP2, MMP9, CXCR4, integrin β1, and integrin α5 expression. Using genome-wide microarray analysis, we found that factors regulating proliferation, apoptosis, cell cycle, and migration were related to the tyrosine receptor kinase A (TrkA) pathway. In addition, activation of the TrkA/AKT signaling cascade during hypoxia was inhibited by quercetin in a dose-dependent manner. Moreover, quercetin alone inhibited the TrkA/AKT signaling pathway, resulting in decreased PASMC migration, cell cycle arrest and the induction of apoptosis. Our data suggest that quercetin is a potential candidate for the treatment of hypoxia-induced PAH.

  16. Epigenetic mechanisms in pulmonary arterial hypertension: the need for global perspectives

    Directory of Open Access Journals (Sweden)

    Prakash Chelladurai

    2016-06-01

    Full Text Available Pulmonary arterial hypertension (PAH is a severe and progressive disease, characterised by high pulmonary artery pressure that usually culminates in right heart failure. Recent findings of alterations in the DNA methylation state of superoxide dismutase 2 and granulysin gene loci; histone H1 levels; aberrant expression levels of histone deacetylases and bromodomain-containing protein 4; and dysregulated microRNA networks together suggest the involvement of epigenetics in PAH pathogenesis. Thus, PAH pathogenesis evidently involves the interplay of a predisposed genetic background, epigenetic state and injurious events. Profiling the genome-wide alterations in the epigenetic mechanisms, such as DNA methylation or histone modification pattern in PAH vascular cells, may explain the great variability in susceptibility and disease severity that is frequently associated with pronounced remodelling and worse clinical outcome. Moreover, the influence of genetic predisposition and the acquisition of epigenetic alterations in response to environmental cues in PAH progression and establishment has largely been unexplored on a genome-wide scale. In order to gain insights into the molecular mechanisms leading to the development of PAH and to design novel therapeutic strategies, high-throughput approaches have to be adopted to facilitate systematic identification of the disease-specific networks using next-generation sequencing technologies, the application of these technologies in PAH has been relatively trivial to date.

  17. Raynaud's phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Kasparian, A; Floros, A; Gialafos, E; Kanakis, M; Tassiopoulos, S; Kafasi, N; Vaiopoulos, G

    2007-01-01

    In patients with Systemic lupus erythematosus (SLE), Raynaud phenomenon (RP) is frequently present and associated with pulmonary hypertension (PHT). Elevated pulmonary artery systolic pressure (PASP) is an indicator of PHT and can be estimated noninvasively. We attempt to explore the significance of RP in SLE and to correlate it with clinical and serological parameters of the disease. The study population consisted of 34 patients (age, sex and disease duration matched) who fulfilled the revised SLE criteria of the American College of Rheumatology, and were categorized into two groups: Group 1 had patients having SLE and RP (2 males/15 females, mean age 45 +/- 18 years) and group 2 had patients with SLE but without RP (3 males/14 females, mean age 40 +/- 14 years. Detailed cardiac ultrasound was performed including measurement of PASP, while clinical and serological features of both groups were collected and correlated. Significant differences were shown in the presence of arterial hypertension (P < 0.05), arthralgias (P < 0.005), arthritis (P < 0.05), myalgias (P < 0.05), alopecia (P < 0.05) and PASP (P < 0.0001). No difference was observed among the cardiac ultrasound indices and the ejection fraction between the two groups. PASP was significantly correlated with RP, while no correlation was observed regarding the disease duration. In patients with SLE, the presence of RP was associated with elevation in PASP. Further investigation is needed to clarify the significance of this relation.

  18. Successful Treatment of Suspected Pulmonary Arterial Hypertension in a Mealy Amazon Parrot (Amazona farinose).

    Science.gov (United States)

    Brady, Sean M; Burgdorf-Moisuk, Anne; Silverman, Sarah; Wack, Raymund F

    2016-12-01

    A 25-year-old, male mealy Amazon parrot (Amazona farinose) with a history of polycythemia, hepatomegaly, and epistaxis was evaluated for progressive lethargy and anorexia. Clinical laboratory testing revealed severe polycythemia (71%), hypophosphatemia (1.6 mg/dL), and mild hypokalemia (2.8 mEq/L). Radiographs showed marked hepatomegaly and loss of air sac space. Despite supportive treatments, the bird's condition deteriorated, and it developed ataxia, was unable to fly, and became oxygen dependent. An echocardiogram, including an air bubble study, revealed a right-to-left atrial shunt and presumed pulmonary arterial hypertension. The bird was started on periodic phlebotomy (5-10 mL/kg q6wk) to reduce packed cell volume and sildenafil citrate (2.5 mg/kg PO q8h) for treatment of suspected pulmonary arterial hypertension. One week later, the patient was weaned off oxygen, and 24 days after initial presentation, the parrot was returned to its outdoor exhibit. Intermittent periods of increased respiratory rate and effort have been reported but have resolved without additional treatments. Epistaxis, once common in this bird, has not been noted since initiating treatment with sildenafil citrate 15 months ago.

  19. Gender-related differences in pulmonary arterial hypertension targeted drugs administration.

    Science.gov (United States)

    Marra, Alberto M; Benjamin, Nicola; Eichstaedt, Christina; Salzano, Andrea; Arcopinto, Michele; Gargani, Luna; D Alto, Michele; Argiento, Paola; Falsetti, Lorenzo; Di Giosia, Paolo; Isidori, Andrea M; Ferrara, Francesco; Bossone, Eduardo; Cittadini, Antonio; Grünig, Ekkehard

    2016-12-01

    During the last 15 years, a real "paradigm-shift" occurred, due to the development of PAH-targeted drugs, leading to crucial improvements in symptoms, exercise capacity, hemodynamics and outcome of PAH patients. In order to describe differences regarding epidemiology and therapy in PAH according to gender, we performed a review of the available literature in "PubMed" and "Web of Science" databases. In order to find relevant articles, we combined each of the following the keywords "pulmonary arterial hypertension", "gender", "sex", "men", "woman", "male", "female", "phosphodiesterase inhibitors", "endothelin receptor antagonists", "prostanoids". While there is a substantial agreement among epidemiological studies in reporting an increased prevalence of pulmonary arterial hypertension (PAH) among women, male PAH patients are affected by a higher impairment of the right ventricular function and consequently experience poorer outcomes. With regards to PAH-targeted drug administration, endothelin receptor antagonists (ERAs) and prostacyclin analogues (PC) show better treatment results in female PAH patients, while phosphodiesterase-5 inhibitors (PD5-I) seem to exert a more beneficial effect on male patients. However, to date no clear consensus could be formed by the available literature, which is constituted mainly by retrospective studies. Females with PAH are more prone to develop PAH, while males experience poorer outcomes. Females PAH might benefit more from ERAs and PC, while males seem to have more beneficial effects from PD5-I administration. However, more research is warranted in order to assess the most effective treatment for PAH patients according to gender.

  20. Left Ventricular Diastolic Function Assessment of a Heterogeneous Cohort of Pulmonary Arterial Hypertension Patients

    Science.gov (United States)

    Hernandez-Suarez, Dagmar F.; Lopez Menendez, Francisco R.; Palm, Denada; Lopez-Candales, Angel

    2017-01-01

    Background Pulmonary arterial hypertension (PAH) is known to trigger right ventricular (RV) remodeling that might compromise left ventricular (LV) filling due to inter-ventricular interdependence. In this study, we aimed to examine standard echocardiographic measurements of LV diastolic function in PAH patients. Methods In this retrospective study, we identified clinical as well as complete echocardiographic data from 128 chronic PAH patients to fully assess LV diastolic dysfunction (LVDD) using standard recommended Doppler guidelines. Accordingly, patients were divided into three groups: LVDD 0, LVDD 1 and LVDD 2. Results The mean age of the studied population was 57 ± 14 years with a mean pulmonary artery systolic pressure (PASP) of 55 ± 21 mm Hg. A total of 36% of the study patients had normal LV diastolic function. However, 64% had LVDD with LVDD stage 1 being the most common (48%). In terms of echocardiographic data, significant differences were found among the three LVDD groups in regards to PASP, LV end systolic and diastolic volumes, tricuspid annular plane systolic excursion, right ventricular fractional area change as well as many other tissue Doppler imaging parameters. Finally, just age and PASP were predictors of abnormal LV diastolic function (P < 0.05). Conclusions Impaired relaxation is a common abnormality in PAH patients. Additional studies are warranted to determine whether LVDD alters prognosis or is related to changes in the symptomatic profile of this group of patients. PMID:28270896

  1. Hypoxic pulmonary vasoconstriction in isolated rat pulmonary arteries is not inhibited by antagonists of H2 S-synthesizing pathways.

    Science.gov (United States)

    Prieto-Lloret, Jesus; Shaifta, Yasin; Ward, Jeremy P T; Aaronson, Philip I

    2015-01-15

    An increase in the H2 S (hydrogen sulphide, hereafter sulphide) concentration in pulmonary artery smooth muscle cells (PASMCs) has been proposed to mediate hypoxic pulmonary vasoconstriction (HPV). We evaluated this hypothesis in isolated rat intrapulmonary arteries (IPAs) by examining the effects of the sulphide precursor cysteine and sulphide-synthesis blockers on HPV and also on normoxic pulmonary vasoconstriction (NPV) stimulated by prostaglandin F2α (PGF2α ) and by the drug LY83583, which causes contraction in IPAs by increasing cellular reactive oxygen species levels. Experiments with several blockers of cystathionine γ-lyase (CSE), the enzyme responsible for sulphide synthesis in the vasculature, demonstrated that propargylglycine (PAG, 1 mm) had little or no effect on the NPV caused by PGF2α or LY83583. Conversely, other CSE antagonists tested, aminooxyacetic acid (AOAA, 100 μm), β-cyanoalanine (BCA, 500 μm) and hydroxylamine (HA, 100 μm), altered the NPV to PGF2α (BCA increased, HA inhibited) and/or LY83583 (BCA increased, AOAA and HA inhibited). Preincubating IPAs in physiological saline solution (PSS) containing 1 mm cysteine increased the amplitude of the NPV to PGF2(α) by ∼50%, and had a similar effect on HPV elicited by hypoxic challenge with 0% O2 . The enhancement of both responses by cysteine was abolished by pretreatment with 1 mm PAG. Measurements carried out with an amperometric electrode demonstrated that incubation with 1 mm cysteine under anoxic conditions (to minimize sulphide oxidation) greatly potentiated the release of sulphide from pieces of rat liver and that this release was strongly antagonized by PAG, indicating that at this concentration PAG could enter cells intact and antagonize CSE. PAG at 1 mm had no effect on HPV recorded in control PSS, or in PSS supplemented with physiological concentrations of cysteine (10 μm), cystine (50 μm) and glutamate (100 μm) in order to prevent the possible depletion of intracellular

  2. Multiple giant coronary aneurysms arising from coronary istula to the pulmonary artery revealed in aorta CT angiography

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Eun Ju; Lee, Ki Nam [Dept. of Radiology, Dong A University Hospital, Dong-A University College of Medicine, Busan (Korea, Republic of); Lee, Jong Min [Dept. of Radiology, Kyungpook National University Hospital, Kyungpook National University College of Medicine, Daegu (Korea, Republic of)

    2015-12-15

    Coronary fistula is a rare coronary abnormality through which blood drains into the cardiac chamber, great vessel or other vessels. In addition, giant aneurysm arising from coronary fistula is rare pathologic manifestation. Herein, we presented a rare case of multiple giant coronary artery aneurysms arising from coronary to pulmonary artery fistula in a 79-year-old woman presenting with sudden loss of consciousness. The aneurysms were detected using thoracic computed tomography angiography and consequently confirmed by invasive coronary angiography.

  3. Pulmonary intravascular blood volume changes through the cardiac cycle in healthy volunteers studied by cardiovascular magnetic resonance measurements of arterial and venous flow

    Directory of Open Access Journals (Sweden)

    Arheden Hakan

    2009-10-01

    Full Text Available Abstract Background This study aims to present a novel method for using cardiovascular magnetic resonance (CMR to non-invasively quantify the variation in pulmonary blood volume throughout the cardiac cycle in humans. Methods 10 healthy volunteers (7 males, 3 female, age range 21-32 years were studied. The blood flow in the pulmonary artery and all pulmonary veins was quantified during free breathing using phase contrast velocity encoded CMR. The difference in flow between the pulmonary artery and the pulmonary veins was integrated to calculate the change in pulmonary blood volume throughout the cardiac cycle. Results The stroke volumes in the pulmonary artery and the sum of the pulmonary veins were (mean ± SEM 103 ± 6 ml and 95 ± 6 ml, respectively. The pulmonary blood volume variation (PBVV was 48 ± 5 ml, and the PBVV expressed as percent of the pulmonary artery stroke volume was 46 ± 3%. The maximum increase in pulmonary blood volume occurred 310 ± 12 ms after the R-wave from the ECG (32 ± 2% of the cardiac cycle. PBVV did not correlate to change in cross-sectional area in the pulmonary artery (R2 = 0.03, p = 0.66. Conclusion It is feasible to non-invasively quantify the change in pulmonary blood volume during the cardiac cycle in humans using CMR. The average pulmonary blood volume variation in healthy volunteers was approximately 50 ml and this was approximately 50% of the stroke volume. Further studies are needed to assess the utility of the pulmonary blood volume variation as a measure for identifying cardiac and pulmonary vascular disease.

  4. The Effect of Erigeron Breviscapus on Proliferation of Pulmonary Artery Smooth Muscle Cells in Hypoxic Porcines

    Institute of Scientific and Technical Information of China (English)

    DING; Yipeng; XU; Yongjian; ZHANG; Zhenxiang

    2001-01-01

    In order to study the effect of Erigeron Breviscapus (EB) on proliferation of pulmonary artery smooth muscle cells (PASMC) in hypoxic porcines, immunohistochemical and MTT methods were employed to measure the proliferation of PASMC. It was found that the proliferation of PASMC in porcines was obvious, and the expression of proliferating cell nuclear antigen (PCNA)was significantly high within 48 h after exposure to hypoxia. The EB could inhibit the proliferation and the expression of PCNA in PASMC under hypoxia, but it had no effect on the proliferation and expression of PCNA in PASMC under normal condition. The EB could inhibit the proliferation and the expression of PCNA in PASMC induced by phorbol 12-myristate 13-acetate (PMA), an agonist of PKC in normal and hypoxic conditions. It was concluded that the hypoxia could enhance the proliferation and expression of PCNA in PASMC. The EB can inhibit the proliferation and expression of PCNA in PASMC under hypoxia through PKC-signal way. The EB may be used in treating the pulmonary hypertension by inhibiting the proliferation of PASMC and the pulmonary vascular remodeling.

  5. HYPOXIA AND ENDOTHELIN-1 STIMULATE DNA SYNTHESIS OF PULMONARY ARTERY SMOOTH MUSCLE CELLS

    Institute of Scientific and Technical Information of China (English)

    冯晓东; 蔡英年

    1996-01-01

    Hypoxia and endothelin-1 (ET-1) are associated with constriction of pulmonary vasculature both in vivo and in vitro. However, the role of hypoxia and ET-1 in the vascular remodelling during the development of pulmonary, hypertension is unciear. This study demonstrated that ET-1 (0.1nmol/L to 100nmol./L)increased the 3H]thymldine uptake in a dose-dependent manner in cuhured bovine pulmonary artery smooth muscle cells(PASMC), which was enhanced by exposing PASMC to hypoxia (2% O2, 93%N2,5%CO2). BQ123, the specific antagonist of endot helin receptor subtype A, eiLmLnated the ET-1 medicated proliferati0n of PASMC and the cooperative effect of hypoxia. Some dilatory drugs could inhibite the mitogenic effect of ET-1. We also observed that hypoxia significantiy increased [3H]thymldine uptake in PASMC without ET-1 and BQ123 could inhibite this effect. Radioimmunoassay suggested that there was an autocrine of ET-1 in cultured PASMC which was enhanced by hypoxia significantly.

  6. Role of Carnitine Acetyl Transferase in Regulation of Nitric Oxide Signaling in Pulmonary Arterial Endothelial Cells

    Directory of Open Access Journals (Sweden)

    Stephen M. Black

    2012-12-01

    Full Text Available Congenital heart defects with increased pulmonary blood flow (PBF result in pulmonary endothelial dysfunction that is dependent, at least in part, on decreases in nitric oxide (NO signaling. Utilizing a lamb model with left-to-right shunting of blood and increased PBF that mimics the human disease, we have recently shown that a disruption in carnitine homeostasis, due to a decreased carnitine acetyl transferase (CrAT activity, correlates with decreased bioavailable NO. Thus, we undertook this study to test the hypothesis that the CrAT enzyme plays a major role in regulating NO signaling through its effect on mitochondrial function. We utilized the siRNA gene knockdown approach to mimic the effect of decreased CrAT activity in pulmonary arterial endothelial cells (PAEC. Our data indicate that silencing the CrAT gene disrupted cellular carnitine homeostasis, reduced the expression of mitochondrial superoxide dismutase-and resulted in an increase in oxidative stress within the mitochondrion. CrAT gene silencing also disrupted mitochondrial bioenergetics resulting in reduced ATP generation and decreased NO signaling secondary to a reduction in eNOS/Hsp90 interactions. Thus, this study links the disruption of carnitine homeostasis to the loss of NO signaling observed in children with CHD. Preserving carnitine homeostasis may have important clinical implications that warrant further investigation.

  7. Reversible Pulmonary Arterial Hypertension Associated with Interferon-Beta Treatment for Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    E Gibbons

    2015-01-01

    Full Text Available Interferon (IFN therapy has an important role in the treatment of multiple sclerosis and chronic hepatitis C infection. A few case reports have described an association between IFN therapy and the development of irreversible pulmonary arterial hypertension (PAH, and it is currently listed as a possible drug-induced cause of PAH in the most recent classification of pulmonary hypertension. A causal link between IFN use and PAH remains to be elucidated; many reports of PAH resulting from IFN occur in individuals with some other risk factor for PAH. The authors present a case involving a patient with multiple sclerosis with no known risk factors for PAH, who developed severe PAH after exposure to IFN therapy. The patient experienced significant clinical and hemodynamic improvement, with normalization of her pulmonary pressures after the initiation of combination therapy for PAH. At 28 months after diagnosis, she remains asymptomatic with no hemodynamic evidence of PAH and has been off all PAH therapy for 10 months.

  8. Inhaled sildenafil as an alternative to oral sildenafil in the treatment of pulmonary arterial hypertension (PAH).

    Science.gov (United States)

    Rashid, Jahidur; Patel, Brijeshkumar; Nozik-Grayck, Eva; McMurtry, Ivan F; Stenmark, Kurt R; Ahsan, Fakhrul

    2017-03-28

    The practice of treating PAH patients with oral or intravenous sildenafil suffers from the limitations of short dosing intervals, peripheral vasodilation, unwanted side effects, and restricted use in pediatric patients. In this study, we sought to test the hypothesis that inhalable poly(lactic-co-glycolic acid) (PLGA) particles of sildenafil prolong the release of the drug, produce pulmonary specific vasodilation, reduce the systemic exposure of the drug, and may be used as an alternative to oral sildenafil in the treatment of PAH. Thus, we prepared porous PLGA particles of sildenafil using a water-in-oil-in-water double emulsion solvent evaporation method with polyethyleneimine (PEI) as a porosigen and characterized the formulations for surface morphology, respirability, in-vitro drug release, and evaluated for in vivo absorption, alveolar macrophage uptake, and safety. PEI increased the particle porosity, drug entrapment, and produced drug release for 36h. Fluorescent particles showed reduced uptake by alveolar macrophages. The polymeric particles were safe to rat pulmonary arterial smooth muscle cell and to the lungs, as evidenced by the cytotoxicity assay and analyses of the injury markers in the bronchoalveolar lavage fluid, respectively. Intratracheally administered sildenafil particles elicited more pulmonary specific and sustained vasodilation in SUGEN-5416/hypoxia-induced PAH rats than oral, intravenous, or intratracheal plain sildenafil did, when administered at the same dose. Overall, true to the hypothesis, this study shows that inhaled PLGA particles of sildenafil can be administered, as a substitute for oral form of sildenafil, at a reduced dose and longer dosing interval.

  9. Nuclear Factor κ-B Is Activated in the Pulmonary Vessels of Patients with End-Stage Idiopathic Pulmonary Arterial Hypertension

    Science.gov (United States)

    Price, Laura C.; Caramori, Gaetano; Perros, Frederic; Meng, Chao; Gambaryan, Natalia; Dorfmuller, Peter; Montani, David; Casolari, Paolo; Zhu, Jie; Dimopoulos, Konstantinos; Shao, Dongmin; Girerd, Barbara; Mumby, Sharon; Proudfoot, Alastair; Griffiths, Mark; Papi, Alberto; Humbert, Marc; Adcock, Ian M.; Wort, S. John

    2013-01-01

    Objectives To assess activation of the inflammatory transcription factor NF-kappa B (NF-κB) in human idiopathic pulmonary arterial hypertension (PAH). Background Idiopathic PAH is a severe progressive disease characterized by pulmonary vascular remodeling and excessive proliferation of vascular cells. Increasing evidence indicates that inflammation is important in disease pathophysiology. Methods NF-κB-p65 and CD68, CD20 and CD45 were measured by immunohistochemistry and confocal microscopy on lung specimens from patients with idiopathic PAH (n = 12) and controls undergoing lung surgery (n = 14). Clinical data were recorded for all patients including invasive pulmonary hemodynamics for the PAH patients. Immunohistochemical images were analyzed by blinded observers to include standard pulmonary vascular morphometry; absolute macrophage counts/mm2 and p65-positivity (p65+) using composite images and image-analysis software; and cytoplasmic:nuclear p65+ of individual pulmonary arterial endothelial and smooth muscle cells (PASMC) in 10–20 pulmonary arteries or arterioles per subject. The expression of ET-1 and CCL5 (RANTES) in whole lung was determined by RT-qPCR. Results Macrophage numbers were increased in idiopathic PAH versus controls (49.0±4.5 vs. 7.95±1.9 macrophages/100 mm2, p<0.0001): these macrophages demonstrated more nuclear p65+ than in macrophages from controls (16.9±2.49 vs. 3.5±1.25%, p<0.001). An increase in p65+ was also seen in perivascular lymphocytes in patients with PAH. Furthermore, NF-κB activation was increased in pulmonary arterial endothelial cells (62.3±2.9 vs. 14.4±3.8, p<0.0001) and PASMC (22.6±2.3 vs. 11.2±2.0, p<0.001) in patients with PAH versus controls, with similar findings in arterioles. Gene expression of both ET-1 mRNA ((0.213±0.069 vs. 1.06±0.23, p<0.01) and CCL5 (RANTES) (0.16±0.045 vs. 0.26±0.039, p<0.05) was increased in whole lung homogenates from patients with PAH. Conclusions NF-κB is activated in

  10. 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.

  11. 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