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Sample records for bovine pulmonary artery

  1. Roles for Nox4 in the contractile response of bovine pulmonary arteries to hypoxia

    OpenAIRE

    Ahmad, Mansoor; Kelly, Melissa R.; Zhao, Xiangmin; Kandhi, Sharath; Wolin, Michael S.

    2010-01-01

    Hypoxia appears to promote contraction [hypoxic pulmonary vasoconstriction (HPV)] of bovine pulmonary arteries (BPA) through removal of a peroxide-mediated relaxation. This study examines the roles of BPA Nox oxidases and mitochondria in the HPV response. Inhibitors of Nox2 (0.1 mM apocynin and 50 μM gp91-dstat) and mitochondrial electron transport (10 μM antimycin and rotenone) decreased superoxide generation in BPA without affecting contraction to 25 mM KCl or the HPV response. Transfection...

  2. 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.

  3. 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 ...

  4. 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

  5. Gestational pulmonary arterial hypertension

    OpenAIRE

    Moll, Matthew; Payne, Julie G.; Tukey, Melissa H.; Farber, Harrison W.

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a progressive disease marked by the irreversible pulmonary vascular changes of vasoconstriction, thrombosis, and proliferation of smooth muscle and endothelial cells. The untreated clinical course is characterized by progressive dyspnea and a median survival of less than 3 years. Many of these patients are of child-bearing age; however, pregnancy leads to physiologic changes that are particularly poorly tolerated in PAH, conferring a 30%–56% mortality....

  6. 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.

  7. Pulmonary arterial hypertension: an update

    OpenAIRE

    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 and specific histological changes. It is a progressive disease finally resulting in right heart failure and premature death. Typical symptoms are dyspnoea at exercise, chest pain and syncope; furthe...

  8. 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. ... All Open All Close All Description Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high ...

  9. Pulmonary arterial hypertension.

    Science.gov (United States)

    Montani, David; Günther, Sven; Dorfmüller, Peter; Perros, Frédéric; Girerd, Barbara; Garcia, Gilles; Jaïs, Xavier; Savale, Laurent; Artaud-Macari, Elise; Price, Laura C; Humbert, Marc; Simonneau, Gérald; Sitbon, Olivier

    2013-01-01

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

  10. Relaxation to bradykinin in bovine pulmonary supernumerary arteries can be mediated by both a nitric oxide-dependent and -independent mechanism

    Science.gov (United States)

    Tracey, A; Bunton, D; Irvine, J; MacDonald, A; Shaw, A M

    2002-01-01

    The aim of the present study was to determine the relative contribution of prostanoids, nitric oxide and K+ channels in the bradykinin-induced relaxation of bovine pulmonary supernumerary arteries. In endothelium-intact, but not denuded rings, bradykinin produced a concentration-dependent relaxation (pEC50, 9.6±0.1), which was unaffected by the cyclo-oxygenase inhibitor indomethacin. The nitric oxide scavenger hydroxocobalamin (200 μM, pEC50, 8.5±0.2) and the nitric oxide synthase inhibitor L-NAME (100 μM, pEC50, 8.9±0.1) and the combination of L-NAME and hydroxocobalamin (pEC50, 8.1±0.2) produced rightward shifts in the bradykinin concentration response curve. The guanylyl cyclase inhibitor ODQ (10 μM, pEC50, 9.6±0.4) did not affect the response to bradykinin. Elevating the extracellular [K+] to 30 mM did not affect the response to bradykinin but abolished the response when ODQ or L-NAME was present. The K+ channel blocker apamin (100 nM), combined with charybdotoxin (100 nM), produced a small reduction in the maximum response to bradykinin but they abolished the response to bradykinin when ODQ, L-NAME or hydroxocobalamin were present. Apamin (100 nM) combined with iberiotoxin (100 nM) also reduced the response to bradykinin in the presence of hydroxocobalamin or L-NAME. The concentration response curve for sodium nitroprusside-induced relaxation was abolished by ODQ (10 μM) and shifted to the right by apamin and charybdotoxin. These studies suggest that in bovine pulmonary supernumerary arteries bradykinin can stimulate the formation of nitric oxide and activate an EDHF-like mechanism and that either of these pathways alone can mediate the bradykinin-induced relaxation. In addition nitric oxide, acting through guanylyl cyclase, can activate an apamin/charbydotoxin-sensitive K+ channel in this tissue. PMID:12359636

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

    Directory of Open Access Journals (Sweden)

    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. Pulmonary Extramedullary Hematopoiesis Involving the Pulmonary Artery

    OpenAIRE

    Varun Monga; Margarida Silverman

    2015-01-01

    Extramedullary hematopoiesis (EMH) occurs as a complication of hematologic disorders such as myelofibrosis, sickle cell anemia and thalassemia. The extramedullary tissue usually involves liver, spleen and lymph nodes, less frequently the chest. We present a recent case of a man with myeloproliferative neoplasm who developed pulmonary hemorrhage secondary to EMH in the lung and pulmonary artery. Radiation therapy was considered the best approach, but it didn’t work and the patient died a week ...

  13. 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.

  14. Pulmonary Artery Denervation Reduces Pulmonary Artery Pressure and Induces Histological Changes in an Acute Porcine Model of Pulmonary Hypertension

    OpenAIRE

    Rothman, A.M.K.; Arnold, N D; Chang, W.; Watson, O.; Swift, A J; Condliffe, R; Elliot, C A; Kiely, D. G.; Suvarna, S K; Gunn, J.; Lawrie, A.

    2015-01-01

    Background— Pulmonary arterial hypertension is a devastating disease with high morbidity and mortality and limited treatment options. Recent studies have shown that pulmonary artery denervation improves pulmonary hemodynamics in an experimental model and in an early clinical trial. We aimed to evaluate the nerve distribution around the pulmonary artery, to determine the effect of radiofrequency pulmonary artery denervation on acute pulmonary hypertension induced by vasoconstriction, and to de...

  15. Drugs induced pulmonary arterial hypertension.

    Science.gov (United States)

    Seferian, Andrei; Chaumais, Marie-Camille; Savale, Laurent; Günther, Sven; Tubert-Bitter, Pascale; Humbert, Marc; Montani, David

    2013-09-01

    Pulmonary arterial hypertension (PAH) is a rare disorder characterized 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 appetite suppressant 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 arterial smooth muscle cells. Amphetamines, phentermine and mazindol were less frequently used but are also considered as possible risk factors for PAH. Dasatinib, a dual Src/Abl kinase inhibitor, used in the treatment of chronic myelogenous leukaemia was associated with cases of severe PAH, in part reversible after its withdrawal. Recently several studies raised the potential endothelial dysfunction that could be induced by interferon, and few cases of PAH have been reported with interferon therapy. Other possible risk factors for PAH include: nasal decongestants, like phenylpropanolamine, dietary supplement - L-Tryptophan, selective serotonin reuptake inhibitors, pergolide and other drugs that could act on 5HT2B receptors. Interestingly, PAH remains a rare complication of these drugs, suggesting possible individual susceptibility and further studies are needed to identify patients at risk of drugs induced PAH. PMID:23972547

  16. Aberrant left pulmonary artery associated with right pulmonary hypoplasia

    International Nuclear Information System (INIS)

    Aberrant left pulmonary artery (ALPA), or pulmonary artery sling, is an uncommon vascular malformation that is frequently associated with obstructive disorders of the tracheobronquial tree. In newborns, it produces severe respiratory problems. In contrast, in adults, it is usually discovered by change. ALPA has been associated with right pulmonary hypoplasia (RPH) in a small number of cases. We present a new case of ALP associated with right pulmonary hypoplasia in an adult woman, diagnosed by CT and MR. 12 refs

  17. An Update on Pulmonary Arterial Hypertension

    OpenAIRE

    Wapner, Joanna; Matura, Lea Ann

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a progressive disease that ultimately leads to right heart failure and death. PAH is defined as a mean pulmonary arterial pressure ≥ 25 mm Hg with a pulmonary capillary wedge pressure ≤ 15 mm Hg at rest. The diagnosis of PAH is one of exclusion; diagnostics include an extensive history, serology, chest radiograph, pulmonary function tests, ventilation/perfusion scan, transthoracic echocardiogram, and right heart catheterization. Treatment and care of p...

  18. Inflammation in pulmonary arterial hypertension.

    Science.gov (United States)

    Price, Laura C; Wort, S John; Perros, Frédéric; Dorfmüller, Peter; Huertas, Alice; Montani, David; Cohen-Kaminsky, Sylvia; Humbert, Marc

    2012-01-01

    Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling of the precapillary pulmonary arteries, with excessive proliferation of vascular cells. Although the exact pathophysiology remains unknown, there is increasing evidence to suggest an important role for inflammation. Firstly, pathologic specimens from patients with PAH reveal an accumulation of perivascular inflammatory cells, including macrophages, dendritic cells, T and B lymphocytes, and mast cells. Secondly, circulating levels of certain cytokines and chemokines are elevated, and these may correlate with a worse clinical outcome. Thirdly, certain inflammatory conditions such as connective tissue diseases are associated with an increased incidence of PAH. Finally, treatment of the underlying inflammatory condition may alleviate the associated PAH. Underlying pathologic mechanisms are likely to be "multihit" and complex. For instance, the inflammatory response may be regulated by bone morphogenetic protein receptor type 2 (BMPR II) status, and, in turn, BMPR II expression can be altered by certain cytokines. Although antiinflammatory therapies have been effective in certain connective-tissue-disease-associated PAH, this approach is untested in idiopathic PAH (iPAH). The potential benefit of antiinflammatory therapies in iPAH is of importance and requires further study. PMID:22215829

  19. Differential imaging features of pulmonary artery dissection from other intraluminal diseases of pulmonary artery: Two cases report

    International Nuclear Information System (INIS)

    Pulmonary artery dissection is rarer than other intraluminal diseases of pulmonary artery such as pulmonary thromboembolism or pulmonary artery sarcoma. We report two cases of pulmonary artery dissection mimicking pulmonary artery sarcoma. Computed tomography (CT) showed no enhancement of intrapulmonary arterial lesion or expansion of involved pulmonary artery. Magnetic resonance imaging (MRI) showed low-signal intensity intimal flap on T1- and T2-weighted images. There was no fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET)-CT. In this case report, we describe the imaging features of pulmonary artery dissection on CT, MRI, and PET-CT.

  20. Differential imaging features of pulmonary artery dissection from other intraluminal diseases of pulmonary artery: Two cases report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Joo Ho; Shin, Hyun Woong; Sohn, Kung Rak; Lee, Yil Gi [Daegu Fatima Hospital, Daegu(Korea, Republic of)

    2015-03-15

    Pulmonary artery dissection is rarer than other intraluminal diseases of pulmonary artery such as pulmonary thromboembolism or pulmonary artery sarcoma. We report two cases of pulmonary artery dissection mimicking pulmonary artery sarcoma. Computed tomography (CT) showed no enhancement of intrapulmonary arterial lesion or expansion of involved pulmonary artery. Magnetic resonance imaging (MRI) showed low-signal intensity intimal flap on T1- and T2-weighted images. There was no fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET)-CT. In this case report, we describe the imaging features of pulmonary artery dissection on CT, MRI, and PET-CT.

  1. Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Jane A. Leopold

    2016-05-01

    Full Text Available Pulmonary arterial hypertension (PAH is a devastating disease that is precipitated by hypertrophic pulmonary vascular remodeling of distal arterioles to increase pulmonary artery pressure and pulmonary vascular resistance in the absence of left heart, lung parenchymal, or thromboembolic disease. Despite available medical therapy, pulmonary artery remodeling and its attendant hemodynamic consequences result in right ventricular dysfunction, failure, and early death. To limit morbidity and mortality, attention has focused on identifying the cellular and molecular mechanisms underlying aberrant pulmonary artery remodeling to identify pathways for intervention. While there is a well-recognized heritable genetic component to PAH, there is also evidence of other genetic perturbations, including pulmonary vascular cell DNA damage, activation of the DNA damage response, and variations in microRNA expression. These findings likely contribute, in part, to dysregulation of proliferation and apoptosis signaling pathways akin to what is observed in cancer; changes in cellular metabolism, metabolic flux, and mitochondrial function; and endothelial-to-mesenchymal transition as key signaling pathways that promote pulmonary vascular remodeling. This review will highlight recent advances in the field with an emphasis on the aforementioned molecular mechanisms as contributors to the pulmonary vascular disease pathophenotype.

  2. Anomalous Origin of Left Coronary Artery from the Pulmonary Trunk in a Mildly Symptomatic Adult Female

    Directory of Open Access Journals (Sweden)

    Kevin Fan-Ying Tseng

    2013-01-01

    Full Text Available Anomalous origin of the left coronary artery from the pulmonary trunk, also known as Garland-Bland-White syndrome, is an extremely rare but potentially fatal congenital cardiovascular anomaly, and it often exists as an isolated condition. We hereby report an adult female who was admitted for mild chest discomfort and was accidentally diagnosed to have anomalous origin of the left coronary artery from the pulmonary trunk. This anomaly was simply repaired by using a bovine pericardial patch to obliterate the anomalous opening in the pulmonary trunk and a single coronary artery bypass graft. This report highlights the characteristic events of the anomaly in an adult with only mild symptoms.

  3. The arterial load in pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    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.

  4. 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

  5. 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.

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

    OpenAIRE

    Humbert, M.

    2010-01-01

    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 cont...

  7. 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

  8. Metabolomic Heterogeneity of Pulmonary Arterial Hypertension

    OpenAIRE

    Zhao, Yidan; Peng, Jenny; Lu, Catherine; Hsin, Michael; Mura, Marco; Wu, Licun; Chu, Lei; Zamel, Ricardo; Machuca, Tiago; Waddell, Thomas; Liu, Mingyao; Keshavjee, Shaf; Granton, John; de Perrot, Marc

    2014-01-01

    Although multiple gene and protein expression have been extensively profiled in human pulmonary arterial hypertension (PAH), the mechanism for the development and progression of pulmonary hypertension remains elusive. Analysis of the global metabolomic heterogeneity within the pulmonary vascular system leads to a better understanding of disease progression. Using a combination of high-throughput liquid-and-gas-chromatography-based mass spectrometry, we showed unbiased metabolomic profiles of ...

  9. 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

  10. 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)

  11. Idiopathic aneurysm of pulmonary artery

    International Nuclear Information System (INIS)

    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)

  12. Left main coronary artery compression in pulmonary arterial hypertension

    DEFF Research Database (Denmark)

    Al-Badri, Kadhem Helo Abbas; Jensen, Jesper Møller; Christiansen, Evald H;

    2015-01-01

    In patients with pulmonary arterial hypertension (PAH), chest pain is most likely due to right ventricular demand ischemia. We report a patient with idiopathic PAH who developed severe angina due to extrinsic compression of the left main coronary artery (LMCA) from a dilated pulmonary artery trunk....... The diagnosis was verified by electrocardiogram after exercise, coronary angiography including intravascular ultrasound, and cardiac multidetector computed tomography (MDCT). The origin of the LMCA was high in the left coronary sinus, facilitating extrinsic compression. The patient was successfully treated...

  13. Potassium channels in pulmonary arterial hypertension.

    Science.gov (United States)

    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. PMID:26341985

  14. Recent trends in pulmonary arterial hypertension

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    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.

  15. 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.

  16. 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.

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

    Science.gov (United States)

    Humbert, M

    2010-03-01

    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. PMID:20956167

  18. 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.

  19. 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.

  20. [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. PMID:23859515

  1. Metabolomic heterogeneity of pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Yidan Zhao

    Full Text Available Although multiple gene and protein expression have been extensively profiled in human pulmonary arterial hypertension (PAH, the mechanism for the development and progression of pulmonary hypertension remains elusive. Analysis of the global metabolomic heterogeneity within the pulmonary vascular system leads to a better understanding of disease progression. Using a combination of high-throughput liquid-and-gas-chromatography-based mass spectrometry, we showed unbiased metabolomic profiles of disrupted glycolysis, increased TCA cycle, and fatty acid metabolites with altered oxidation pathways in the human PAH lung. The results suggest that PAH has specific metabolic pathways contributing to increased ATP synthesis for the vascular remodeling process in severe pulmonary hypertension. These identified metabolites may serve as potential biomarkers for the diagnosis of PAH. By profiling metabolomic alterations of the PAH lung, we reveal new pathogenic mechanisms of PAH, opening an avenue of exploration for therapeutics that target metabolic pathway alterations in the progression of PAH.

  2. 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...

  3. 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.

  4. Novel biomarkers for pulmonary arterial hypertension.

    Science.gov (United States)

    Anwar, Anjum; Ruffenach, Gregoire; Mahajan, Aman; Eghbali, Mansoureh; Umar, Soban

    2016-01-01

    Pulmonary arterial hypertension is a deadly disease characterized by elevated pulmonary arterial pressures leading to right ventricular hypertrophy and failure. The confirmatory gold standard test is the invasive right heart catheterization. The disease course is monitored by pulmonary artery systolic pressure measurement via transthoracic echocardiography. A simple non-invasive test to frequently monitor the patients is much needed. Search for a novel biomarker that can be detected by a simple test is ongoing and many different options are being studied. Here we review some of the new and unique pre-clinical options for potential pulmonary hypertension biomarkers. These biomarkers can be broadly categorized based on their association with endothelial cell dysfunction, inflammation, epigenetics, cardiac function, oxidative stress, metabolism,extracellular matrix, and volatile compounds in exhaled breath condensate. A biomarker that can be detected in blood, urine or breath condensate and correlates with disease severity, progression and response to therapy may result in significant cost reduction and improved patient outcomes. PMID:27439993

  5. Pulmonary Artery Cement Embolism after a Vertebroplasty

    Directory of Open Access Journals (Sweden)

    Anas Nooh

    2015-01-01

    Full Text Available Background Context. Vertebroplasty is a minimally invasive procedure most commonly used for the treatment of vertebral compression fractures. Although it is relatively safe, complications have been reported over time. Among those complications, massive cement pulmonary embolism is considered a rare complication. Here we report a case of massive diffuse cement pulmonary embolism following percutaneous vertebroplasty for a vertebral compression fracture. Study Design. Case report. Methods. This is a 70-year-old female who underwent vertebroplasty for T11 and T12 vertebral compression fracture. Results. CT-scan revealed an incidental finding of cement embolism in the pulmonary trunk and both pulmonary arteries. Since the patient was asymptomatic, she was monitored closely and she did not need any intervention. Conclusion. Vertebroplasty is a minimally invasive procedure used for treatment of vertebral compression fracture. Despite the low rate of complications, a pulmonary cement embolism can occur. The consequences of cement embolism range widely from being asymptomatic to embolism that can cause paralysis, radiculopathy, or a fatal pulmonary embolism.

  6. [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. PMID:21536178

  7. 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.

  8. Erythropoietin upregulation in pulmonary arterial hypertension.

    Science.gov (United States)

    Karamanian, Vanesa A; Harhay, Michael; Grant, Gregory R; Palevsky, Harold I; Grizzle, William E; Zamanian, Roham T; Ihida-Stansbury, Kaori; Taichman, Darren B; Kawut, Steven M; Jones, Peter L

    2014-06-01

    The pathophysiologic alterations of patients with pulmonary arterial hypertension (PAH) are diverse. We aimed to determine novel pathogenic pathways from circulating proteins in patients with PAH. Multianalyte profiling (MAP) was used to measure 90 specifically selected antigens in the plasma of 113 PAH patients and 51 control patients. Erythropoietin (EPO) functional activity was assessed via in vitro pulmonary artery endothelial cell networking and smooth muscle cell proliferation assays. Fifty-eight patients had idiopathic PAH, whereas 55 had other forms of PAH; 5 had heritable PAH, 18 had connective tissue disease (15 with scleroderma and 3 with lupus erythematosis), 13 had portopulmonary hypertension, 6 had PAH associated with drugs or toxins, and 5 had congenital heart disease. The plasma-antigen profile of PAH revealed increased levels of several novel biomarkers, including EPO. Immune quantitative and histochemical studies revealed that EPO not only was significantly elevated in the plasma of PAH patients but also promoted pulmonary artery endothelial cell network formation and smooth muscle cell proliferation. MAP is a hypothesis-generating approach to identifying novel pathophysiologic pathways in PAH. EPO is upregulated in the circulation and lungs of patients with PAH and may affect endothelial and smooth muscle cell proliferation. PMID:25006446

  9. 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

  10. Contribution of live heartworms harboring in pulmonary arteries to pulmonary hypertension in dogs with dirofilariasis.

    Science.gov (United States)

    Kitagawa, H; Sasaki, Y; Ishihara, K; Hirano, Y

    1990-12-01

    To investigate whether adult heartworms harboring in the pulmonary arteries contribute to pulmonary hypertension, we determined the cardio-pulmonary values immediately before and after removal of heartworms from the pulmonary arteries and before and after insertion of live worms in their place. In 10 heartworm-infected dogs, 8 to 46 worms were removed. The mean pulmonary arterial pressure fell significantly from 24.5 +/- 7.9 mmHg to 16.3 +/- 4.9 mmHg (p less than 0.01) immediately after removal. The right cardiac output decreased in 7 of the 10 cases. The total pulmonary resistance and right ventricular stroke work index also decreased. At 24 hours after removal, live heartworms were put back into the pulmonary arteries of their host dog. The mean pulmonary arterial pressure elevated significantly (p less than 0.01) immediately after insertion. The right cardiac output further decreased in 7 of the 10 dogs, and the total pulmonary resistance and right ventricular stroke work index increased. Separate from this, 12 to 42 heartworms were transplanted into the pulmonary arteries of 5 heartworm-free dogs. Immediately after transplantation, the pulmonary arterial pressure did not show any significant change. However, the stroke volume decreased, and the total pulmonary resistance increased. These facts suggest a contribution of live heartworms to the pulmonary hypertension, although there is a complicated interaction among the presence of heartworms, the pulmonary lesions and the pulmonary hypertension. PMID:2287128

  11. 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.

  12. 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.

  13. Prostanoid therapies in the management of pulmonary arterial hypertension

    OpenAIRE

    LeVarge, Barbara

    2015-01-01

    Barbara L LeVarge Department of Pulmonary and Critical Care Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA Abstract: Prostacyclin is an endogenous eicosanoid produced by endothelial cells; through actions on vascular smooth-muscle cells, it promotes vasodilation. Pulmonary arterial hypertension (PAH) is characterized by elevated mean pulmonary artery pressure due to a high pulmonary vascular resistance state. A relative decrease in prostacyclin presence has been associated ...

  14. 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.

  15. 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.

  16. Mechanical properties of porcine intralobar pulmonary arteries.

    Science.gov (United States)

    Ohtaka, H; Hogg, J C; Moreno, R H; Paré, P D; Schellenberg, R R

    1988-04-01

    The isobaric and isovolumetric properties of intrapulmonary arteries were evaluated by placing a highly compliant balloon inside arterial segments. The passive pressure-volume (P-V) curve was obtained by changing volume (0.004 ml/s) and measuring pressure. The isobaric active volume change (delta V) or isovolumetric active pressure change (delta P) generated by submaximal histamine was measured at four different transmural pressures (Ptm's) reached by balloon inflation. The maximal delta P = 11.2 +/- 0.6 cmH2O (mean +/- SE) was achieved at 30.8 +/- 1.2 cmH2O Ptm and maximal delta V = 0.20 +/- 0.02 ml at 16.7 +/- 1.7 cmH2O Ptm. The P-V relationships were similar when volume was increased after either isobaric or isovolumetric contraction. The calculated length-tension (L-T) relationship showed that the active tension curve was relatively flat and that the passive tension at the optimal length was 149 +/- 11% of maximal active tension. These data show that 1) a large elastic component operates in parallel with the smooth muscle in intralobar pulmonary arteries, and 2) the change in resistance associated with vascular expansion of the proximal arteries is independent of the type of contraction that occurs in the more distal arterial segments. PMID:3378988

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

    International Nuclear Information System (INIS)

    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

  18. A case of pulmonary artery sarcoma presented as cavitary pulmonary lesions.

    Science.gov (United States)

    Min, Daniel; Lee, Ji-Hyun; Jeong, Hye-Cheol; Kim, Jung-Hyun; Shin, Suk-Pyo; Kim, Hong-Min; Han, Kyu Hyun; Jeong, Hye Yun; Kim, Eun-Kyung

    2014-03-01

    Pulmonary artery sarcoma (PAS) is a rare, poorly differentiated malignancy arising from the intimal layer of the pulmonary artery. Contrast-enhanced chest computed tomography (CT) is a good diagnostic modality that shows a low-attenuation filling defect of the pulmonary artery in PAS patients. An 18-year-old man was referred to our hospital for the evaluation and management of cavitary pulmonary lesions that did not respond to treatment. A contrast-enhanced CT of the chest was performed, which showed a filling defect within the right interlobar pulmonary artery. The patient underwent a curative right pneumonectomy after confirmation of PAS. Although lung parenchymal lesions of PAS are generally nonspecific, it can be presented as cavities indicate pulmonary infarcts. Clinicians must consider the possibility of PAS as well as pulmonary thromboembolism in patients with pulmonary infarcts. So, we report the case with PAS that was diagnosed during the evaluation of cavitary pulmonary lesions and reviewed the literatures. PMID:24734102

  19. Interruption of pulmonary arterial flow with inadequate ventilation leads to pulmonary infection.

    Science.gov (United States)

    Urano; Shibayama, Y; Fukunshi, K; Nariyama, K; Ohsawa, N

    1996-03-01

    We examined the effect of interruption of pulmonary arterial flow and inadequate ventilation on the development of pulmonary infarction in rats. Pulmonary arterial flow was blocked by the injection of agar into the inferior vena cava and inadequate ventilation was produced by obstructing the left main bronchus with a polypropylene tip. Histological and angiographic examination of the lung demonstrated that: pulmonary artery embolism alone does not induce pulmonary infarction; obstruction of a bronchus does not induce significant changes, but that pulmonary infarction develops when pulmonary artery embolism and obstruction of a bronchus occur simultaneously. It has been thought that pulmonary infarction is caused by acute obstruction of a pulmonary artery, however, the alveolar walls are supplied with oxygen by both the pulmonary circulation and by ventilation. Interruption of pulmonary arterial flow alone is probably not sufficient to induce pulmonary infarction, which is probably caused by deficiency of oxygen supply to the alveolar walls by a synergy between interruption of pulmonary arterial flow and inadequate ventilation. PMID:8605572

  20. Coanda effect on ductal flow in the pulmonary artery.

    Science.gov (United States)

    Guntheroth, W; Miyaki-Hull, C

    1999-03-01

    The Coanda effect (the tendency of a jet stream to adhere to a boundary wall), and the relevant anatomy, may explain the location of ductal jets within the main pulmonary artery. With the usual insertion of the duct close to the left pulmonary artery, during right ventricular ejection, the ductal jet adheres to the left wall of the main pulmonary artery. When right ventricular ejection is absent in pulmonary atresia, the ductal jet streams down the right wall of the pulmonary artery to the pulmonary valve, reverses, and maintains a parallel column back toward the bifurcation. If the reversed flow is mistaken for ejection from the right ventricle, the diagnosis of pulmonary atresia may be missed. PMID:10082354

  1. Pulmonary artery stent for bronchial adenoid cystic carcinoma causing pulmonary artery stenosis

    DEFF Research Database (Denmark)

    Smith, Corey Allister; Kotlyar, Eugene; Mellemkjaer, Soren;

    2014-01-01

    A 46-year-old woman presented with a 6-month history of dyspnea and weight loss on a background of previous pneumonectomy for bronchial adenoid cystic carcinoma 14 years beforehand. Several years prior to this presentation, she had developed left vocal cord palsy and a metastatic lesion to the ri......A 46-year-old woman presented with a 6-month history of dyspnea and weight loss on a background of previous pneumonectomy for bronchial adenoid cystic carcinoma 14 years beforehand. Several years prior to this presentation, she had developed left vocal cord palsy and a metastatic lesion...... improved both pulmonary artery pressures and the patient's symptoms. The diagnosis of pulmonary artery stenosis due to mediastinal infiltration by metastatic bronchial adenoid cystic carcinoma was based on these findings as well as the presence of the pulmonary nodules and the previous mediastinal...

  2. Pulmonary rehabilitation and exercise in pulmonary arterial hypertension: An underutilized intervention

    OpenAIRE

    Sahni, Sonu; Capozzi, Barbara; Iftikhar, Asma; Sgouras, Vasiliki; Ojrzanowski, Marcin; Talwar, Arunabh

    2015-01-01

    Pulmonary arterial hypertension (PAH) 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. Early thought process was that exercise and increased physical activity may be detrimental to PAH patients however many small cohort trials have proven otherwise. In addition to the many pharmaceutical options, exercise and pulmonary rehabilitation have also...

  3. 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

  4. The right ventricle in pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Robert Naeije

    2014-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a right heart failure syndrome. In early-stage PAH, the right ventricle tends to remain adapted to afterload with increased contractility and little or no increase in right heart chamber dimensions. However, less than optimal right ventricular (RV–arterial coupling may already cause a decreased aerobic exercise capacity by limiting maximum cardiac output. In more advanced stages, RV systolic function cannot remain matched to afterload and dilatation of the right heart chamber progressively develops. In addition, diastolic dysfunction occurs due to myocardial fibrosis and sarcomeric stiffening. All these changes lead to limitation of RV flow output, increased right-sided filling pressures and under-filling of the left ventricle, with eventual decrease in systemic blood pressure and altered systolic ventricular interaction. These pathophysiological changes account for exertional dyspnoea and systemic venous congestion typical of PAH. Complete evaluation of RV failure requires echocardiographic or magnetic resonance imaging, and right heart catheterisation measurements. Treatment of RV failure in PAH relies on: decreasing afterload with drugs targeting pulmonary circulation; fluid management to optimise ventricular diastolic interactions; and inotropic interventions to reverse cardiogenic shock. To date, there has been no report of the efficacy of drug treatments that specifically target the right ventricle.

  5. Pulmonary arterial remodeling in chronic obstructive pulmonary disease is lobe dependent.

    Science.gov (United States)

    Wrobel, Jeremy P; McLean, Catriona A; Thompson, Bruce R; Stuart-Andrews, Christopher R; Paul, Eldho; Snell, Gregory I; Williams, Trevor J

    2013-09-01

    Abstract Pulmonary arterial remodeling has been demonstrated in patients with severe chronic obstructive pulmonary disease (COPD), but it is not known whether lobar heterogeneity of remodeling occurs. Furthermore, the relationship between pulmonary hypertension (PH) and pulmonary arterial remodeling in COPD has not been established. Muscular pulmonary arterial remodeling in arteries 0.10-0.25 mm in diameter was assessed in COPD-explanted lungs and autopsy controls. Remodeling was quantified as the percentage wall thickness to vessel diameter (%WT) using digital image analysis. Repeat measures mixed-effects remodeling for %WT was performed according to lobar origin (upper and lower), muscular pulmonary arterial size (small, medium, and large), and echocardiography-based pulmonary arterial pressure (no PH, mild PH, and moderate-to-severe PH). Lobar perfusion and emphysema indices were determined from ventilation-perfusion and computed tomography scans, respectively. Overall, %WT was greater in 42 subjects with COPD than in 5 control subjects ([Formula: see text]). Within the COPD group, %WT was greater in the upper lobes ([Formula: see text]) and in the small muscular pulmonary arteries ([Formula: see text]). Lobar differences were most pronounced in medium and large arteries. Lobar emphysema index was not associated with arterial remodeling. However, there was a significant positive relationship between the lobar perfusion index and pulmonary arterial remodeling ([Formula: see text]). The presence of PH on echocardiography showed only a trend to a small effect on lower lobe remodeling. The pattern of pulmonary arterial remodeling in COPD is complicated and lobe dependent. Differences in regional blood flow partially account for the lobar heterogeneity of pulmonary arterial remodeling in COPD. PMID:24618551

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. Arterial pulmonary hypertension in noncardiac intensive care unit

    Directory of Open Access Journals (Sweden)

    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

  9. 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.

  10. Exercise Intolerance in Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Robin M. Fowler

    2012-01-01

    Full Text Available Pulmonary arterial hypertension (PAH is associated with symptoms of dyspnea and fatigue, which contribute to exercise limitation. The origins and significance of dyspnea and fatigue in PAH are not completely understood. This has created uncertainly among healthcare professionals regarding acceptable levels of these symptoms, on exertion, for patients with PAH. Dysfunction of the right ventricle (RV contributes to functional limitation and mortality in PAH; however, the role of the RV in eliciting dyspnea and fatigue has not been thoroughly examined. This paper explores the contribution of the RV and systemic and peripheral abnormalities to exercise limitation and symptoms in PAH. Further, it explores the relationship between exercise abnormalities and symptoms, the utility of the cardiopulmonary exercise test in identifying RV dysfunction, and offers suggestions for further research.

  11. 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.

  12. 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.

  13. 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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2016-04-15

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

  15. 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.

  16. Proximal Interruption of the Pulmonary Artery: A Case Series.

    Science.gov (United States)

    Anand, S H; Jasper, Anitha; Mani, Sunithi Elizabeth; Joseph, Elizabeth; Mathai, John

    2015-12-01

    We present a few cases of Proximal Interruption of the Pulmonary Artery, an uncommon developmental anomaly associated with congenital heart disease. The cases had varied clinical presentations. Chest radiograph showed a hypoplastic lung with an ipsilateral small hilum on the side of the interruption and hyperinflation of the contralateral lung. Contrast CT confirmed the diagnosis, demonstrating non-visualization of the left or right pulmonary artery, and other related findings. PMID:26816968

  17. Baseline Characteristics of the Korean Registry of Pulmonary Arterial Hypertension

    OpenAIRE

    Chung, Wook-Jin; Park, Yong Bum; Jeon, Chan Hong; Jung, Jo Won; Ko, Kwang-Phil; Choi, Sung Jae; Seo, Hye Sun; Lee, Jae Seung; Jung, Hae Ok; ,

    2015-01-01

    Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December ...

  18. 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.

  19. The linear relationship between systolic pulmonary artery pressure and mean pulmonary artery pressure is maintained regardless of autonomic or rhythm disturbances

    OpenAIRE

    Vanden Eynden, Frédéric; Racapé, Judith; Vincent, Jame; Vachiéry, Jean-Luc; Bové, Thierry; Van Nooten, Guido

    2016-01-01

    Background In the pulmonary circulation, there is a linear relationship between systolic pulmonary arterial pressure (SPAP) and mean pulmonary arterial pressure (MPAP). The aim of this study was to determine the passive or active nature of this mechanism by exploring the relationship in patients with and without autonomic rhythm control of the heart and pulmonary circulation. Methods Pulmonary arterial pressure recordings from non-transplanted patients and patients with heart transplants or d...

  20. Chronic intrauterine pulmonary hypertension increases main pulmonary artery stiffness and adventitial remodeling in fetal sheep

    OpenAIRE

    Dodson, R. Blair; Morgan, Matthew R.; Galambos, Csaba; Hunter, Kendall S.; Abman, Steven H.

    2014-01-01

    Persistent pulmonary hypertension of the newborn (PPHN) is a clinical syndrome that is characterized by high pulmonary vascular resistance due to changes in lung vascular growth, structure, and tone. PPHN has been primarily considered as a disease of the small pulmonary arteries (PA), but proximal vascular stiffness has been shown to be an important predictor of morbidity and mortality in other diseases associated with pulmonary hypertension (PH). The objective of this study is to characteriz...

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

    OpenAIRE

    Shafie, Davood; Dohaei, Abolfazl; AMIN, Ahmad; Taghavi, Sepideh; Naderi, Nasim

    2015-01-01

    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...

  2. Congenital Pulmonary Artery Stenoses Masquerading as Chronic Thromboembolic Disease

    OpenAIRE

    Hemnes, Anna R.; Doyle, Thomas P.; Janssen, Dana; Robbins, Ivan M.

    2009-01-01

    Stenotic pulmonary vascular lesions, often amenable to balloon angioplasty, can erroneously appear to suggest chronic thromboembolic disease on ventilation/perfusion (V/Q) scan. We report a case of multiple peripheral pulmonary artery stenoses and describe radiologic findings and treatment options.

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

    OpenAIRE

    Gérald Simonneau; Laurent Savale; Andrei Seferian; David Montani; Marc Humbert

    2013-01-01

    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...

  4. The role of pulmonary arterial stiffness in COPD

    OpenAIRE

    Weir-McCall, Jonathan R.; Struthers, Allan D.; Lipworth, Brian J; Houston, J. Graeme

    2015-01-01

    COPD is the second most common cause of pulmonary hypertension, and is a common complication of severe COPD with significant implications for both quality of life and mortality. However, the use of a rigid diagnostic threshold of a mean pulmonary arterial pressure (mPAP) of ≥25mHg when considering the impact of the pulmonary vasculature on symptoms and disease is misleading. Even minimal exertion causes oxygen desaturation and elevations in mPAP, with right ventricular hypertrophy and dilatat...

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

    Directory of Open Access Journals (Sweden)

    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.

  6. Rarefaction and blood pressure in systemic and pulmonary arteries.

    Science.gov (United States)

    Olufsen, Mette S; Hill, N A; Vaughan, Gareth D A; Sainsbury, Christopher; Johnson, Martin

    2012-08-01

    The effects of vascular rarefaction (the loss of small arteries) on the circulation of blood are studied using a multiscale mathematical model that can predict blood flow and pressure in the systemic and pulmonary arteries. We augmented a model originally developed for the systemic arteries (Olufsen et al. 1998, 1999, 2000, 2004) to (a) predict flow and pressure in the pulmonary arteries, and (b) predict pressure propagation along the small arteries in the vascular beds. The systemic and pulmonary arteries are modelled as separate, bifurcating trees of compliant and tapering vessels. Each tree is divided into two parts representing the `large' and `small' arteries. Blood flow and pressure in the large arteries are predicted using a nonlinear cross-sectional area-averaged model for a Newtonian fluid in an elastic tube with inflow obtained from magnetic resonance measurements. Each terminal vessel within the network of the large arteries is coupled to a vascular bed of small `resistance' arteries, which are modelled as asymmetric structured trees with specified area and asymmetry ratios between the parent and daughter arteries. For the systemic circulation, each structured tree represents a specific vascular bed corresponding to major organs and limbs. For the pulmonary circulation, there are four vascular beds supplied by the interlobar arteries. This manuscript presents the first theoretical calculations of the propagation of the pressure and flow waves along systemic and pulmonary large and small arteries. Results for all networks were in agreement with published observations. Two studies were done with this model. First, we showed how rarefaction can be modelled by pruning the tree of arteries in the microvascular system. This was done by modulating parameters used for designing the structured trees. Results showed that rarefaction leads to increased mean and decreased pulse pressure in the large arteries. Second, we investigated the impact of decreasing vessel

  7. 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

  8. 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.

  9. Treatment options for paediatric pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    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.

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

    International Nuclear Information System (INIS)

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

  11. Pulmonary arterial remodeling revealed by microfocal x-ray tomography

    Science.gov (United States)

    Karau, Kelly L.; Molthen, Robert C.; Johnson, Roger H.; Dhyani, Anita H.; Haworth, Steven T.; Dawson, Christopher A.

    2001-05-01

    Animal models and micro-CT imaging are useful for understanding the functional consequences of, and identifying the genes involved in, the remodeling of vascular structures that accompanies pulmonary vascular disease. Using a micro-CT scanner to image contrast-enhanced arteries in excised lungs from fawn hooded rats (a strain genetically susceptible to hypoxia induced pulmonary hypertension), we found that portions of the pulmonary arterial tree downstream from a given diameter were morphometrically indistinguishable. This 'self-consistency' property provided a means for summarizing the pulmonary arterial tree architecture and mechanical properties using a parameter vector obtained from measurements of the contiguous set of vessel segments comprising the longest (principal) pathway and its branches over a range of vascular pressures. This parameter vector was used to characterize the pulmonary vascular remodeling that occurred in rats exposed to a hypoxic (11.5% oxygen) environment and provided the input to a hemodynamic model relating structure to function. The major effect of the remodeling was a longitudinally (pulmonary artery to arterioles) uniform decrease in vessel distensibility that resulted in a 90% increase in arterial resistance. Despite the almost uniform change in vessel distensibility, over 50% of the resistance increase was attributable to vessels with unstressed diameters less than 125 microns.

  12. 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.

  13. The diagnosis value of pulmonary perfusion/ventilation imaging for pulmonary embolism: in comparison with pulmonary artery angiography

    International Nuclear Information System (INIS)

    Objective: To evaluate the value of the pulmonary perfusion/ventilation imaging in diagnosis of pulmonary embolism (PE). Methods: Forty-five patients undergone pulmonary radionuclide imaging and pulmonary artery angiography in Fuwai Hospital were analyzed retrospectively. Results: Twenty-six patients were diagnosed as with PE by pulmonary angiography. For the pulmonary segments with complete filling defect on pulmonary artery angiography, the coincidence rate was 92.7% between the angiography and radionuclide pulmonary imaging, while the pulmonary segments with partial filling defect, the coincidence rate was 73.2% (P<0.01). The sensitivity and specificity were 92.3% and 84.2%, respectively. Both the positive predictive value and negative predictive value were 88.9%. Out of 26 patients with PE, 23 patients had also pulmonary ventilation imaging. The results showed mismatch between pulmonary perfusion and ventilation imaging in all of the 23 patients. Conclusion: The pulmonary perfusion plus pulmonary ventilation imaging plays an important role in diagnosing PE

  14. Endovascular covered stent repair of an iatrogenic subclavian artery-to-pulmonary artery fistula and pseudoaneurysm.

    Science.gov (United States)

    Wheeler, Shane C; Zinn, Kenneth M; Hughes, Terence W

    2007-06-01

    An iatrogenic fistula and consequent pseudoaneurysm developed between the right subclavian artery and right pulmonary artery as a result of misplacement of a hemodialysis access catheter. The patient, who was considered to be at high risk for surgical repair, successfully underwent endovascular treatment that involved insertion of two nitinol stents covered with expanded polytetrafluoroethylene (stent-grafts), one into the right subclavian artery and the other into a right upper lobe pulmonary artery. Multi-detector row computed tomographic angiography played an integral role in the evaluation of the patient's vascular injury and treatment planning. PMID:17538141

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

    OpenAIRE

    Farha Samar; Laskowski Daniel; George Deepa; Park Margaret M; Tang WH Wilson; Dweik Raed A; Erzurum Serpil C

    2013-01-01

    Abstract 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 c...

  16. 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.

  17. 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

  18. Cardiac and pulmonary artery mensuration in feline heartworm disease

    International Nuclear Information System (INIS)

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

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

    International Nuclear Information System (INIS)

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

  20. Increasing pulmonary artery pulsatile flow improves hypoxic pulmonary hypertension in piglets.

    Science.gov (United States)

    Courboulin, Audrey; Kang, Chantal; Baillard, Olivier; Bonnet, Sebastien; Bonnet, Pierre

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a disease affecting distal pulmonary arteries (PA). These arteries are deformed, leading to right ventricular failure. Current treatments are limited. Physiologically, pulsatile blood flow is detrimental to the vasculature. In response to sustained pulsatile stress, vessels release nitric oxide (NO) to induce vasodilation for self-protection. Based on this observation, this study developed a protocol to assess whether an artificial pulmonary pulsatile blood flow could induce an NO-dependent decrease in pulmonary artery pressure. One group of piglets was exposed to chronic hypoxia for 3 weeks and compared to a control group of piglets. Once a week, the piglets underwent echocardiography to assess PAH severity. At the end of hypoxia exposure, the piglets were subjected to a pulsatile protocol using a pulsatile catheter. After being anesthetized and prepared for surgery, the jugular vein of the piglet was isolated and the catheter was introduced through the right atrium, the right ventricle and the pulmonary artery, under radioscopic control. Pulmonary artery pressure (PAP) was measured before (T0), immediately after (T1) and 30 min after (T2) the pulsatile protocol. It was demonstrated that this pulsatile protocol is a safe and efficient method of inducing a significant reduction in mean PAP via an NO-dependent mechanism. These data open up new avenues for the clinical management of PAH. PMID:25993379

  1. HRCT score in bronchiectasis: Correlation with pulmonary function tests and pulmonary artery pressure

    OpenAIRE

    Alzeer Abdulaziz

    2008-01-01

    Background: High resolution CT scan (HRCT) and its score have an important role in delineating pathological changes and pulmonary functional impairment in patients with bronchiectasis. Aims: To assess pulmonary function tests (PFTs) in patients with cystic and cylindrical bronchiectasis. To correlate HRCT score with PFTs and systolic pulmonary artery pressure (SPAP) in both radiological types. Materials and Methods: A cross-sectional study of patients with bronchiectasis diagnosed by HR...

  2. Computational Simulation of the Pulmonary Arteries and its Role in the Study of Pediatric Pulmonary Hypertension

    OpenAIRE

    Hunter, Kendall S.; Feinstein, Jeffrey A.; Ivy, D. Dunbar; Shandas, Robin

    2010-01-01

    The hemodynamic state of the pulmonary arteries is challenging to routinely measure in children due to the vascular circuit's position in the lungs. The resulting relative scarcity of quantitative clinical diagnostic and prognostic information impairs management of diseases such as pulmonary hypertension, or high blood pressure of the pulmonary circuit, and invites new techniques of measurement. Here we examine recent applications of macro-scale computational mechanics methods for fluids and ...

  3. Selexipag for the Treatment of Pulmonary Arterial Hypertension

    DEFF Research Database (Denmark)

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

    2015-01-01

    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...... patients with pulmonary arterial hypertension to receive placebo or selexipag in individualized doses (maximum dose, 1600 μg twice daily). Patients were eligible for enrollment if they were not receiving treatment for pulmonary arterial hypertension or if they were receiving a stable dose of an endothelin......-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...

  4. Non-invasive measurement of pulmonary arterial pressure

    International Nuclear Information System (INIS)

    Pulmonary artery pulse pressure (Psub(p)) and diastolic pressure (Psub(d)) may be obtained by applying a haemodynamic model of blood flow kinetics and wall mechanics to the pulmonary artery: Psub(P) = ρ(Wsub(s)/(Ssub(s)/Ssub(d) -1))2 log(Ssub(s)/Ssub(d)) - 1/2 ρw2sub(s) Psub(d) (Ssub(d)/Ssub(s))sup(1/2)Psub(p) where ρ is blood density, Wsub(s) is peak ejection velocity, and Ssub(s) and Ssub(d) are peak maximal and end diastolic cross-sectional areas of the main pulmonary artery. The different parameters of the equations were measured from radionuclide Tcsup(m) first pass and equilibrium studies. (author)

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

    Directory of Open Access Journals (Sweden)

    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.

  6. Therapeutic pulmonary artery stenting for metastatic bronchial carcinoid

    OpenAIRE

    Vawdrey, Daniel B F; Fitzsimmons, Samantha; Veldtman, Gruschen R; Carpenter, John-Paul

    2013-01-01

    We present a case of a middle-aged man with a 3-month history of progressive shortness of breath and peripheral oedema. Ten years prior to this, he had undergone a left pneumonectomy for metastatic bronchial carcinoid. Clinical examination revealed significant right heart failure, supported by transthoracic echocardiography. CT pulmonary angiogram revealed the cause to be marked progression of the bronchial carcinoid causing severe external compression of right pulmonary artery (RPA). In view...

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

    OpenAIRE

    Mulder, B. J. M.

    2010-01-01

    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, ch...

  8. A Huge Thrombosed Pulmonary Artery Aneurysm without Pulmonary Hypertension in a Patient with Hepatosplenic Schistosomiasis

    Science.gov (United States)

    Abo-Salem, Elsayed S.; Ramadan, Mahmoud M.

    2015-01-01

    Patient: Male, 55 Final Diagnosis: Thrombosed pulmonary artery aneurysm Symptoms: Cough productive • fever • shortness of breath Medication: — Clinical Procedure: Pericardiocentesis Specialty: Cardiology Objective: Rare disease Background: We herein report a case of huge pulmonary artery aneurysm in a 55-year-old male farmer from the Nile delta (Lower-Egypt), mostly due to infestation with Schistosoma mansoni, which is the parasite causing hepatosplenic schistosomiasis. Case Report: This male patient was admitted with a month-long history of progressive shortness of breath, 2-month history of fever, and a cough with mucoid sputum for 10 days. On examination, he had normal temperature and blood pressure, but he had tachypnea, tachycardia, and congested neck veins. Electrocardiography showed multifocal atrial tachycardia and right bundle branch block. Conclusions: The present case is unique in that it shows the presence of a huge pulmonary artery aneurysm despite the absence of pulmonary hypertension. PMID:25746428

  9. 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.

  10. Stenting of Variant Left Carotid Artery Using Brachial Artery Approach in a Patient with Unusual Type of Bovine Aortic Arch

    Directory of Open Access Journals (Sweden)

    Emre Gürel

    2016-01-01

    Full Text Available Bovine aortic arch is the most frequently encountered variation in human aortic arch branching. A 63-year-old Asian male presented with symptomatic severe stenosis of left carotid artery originating from the brachiocephalic trunk. Selective engagement to the left carotid artery was unsuccessful using transfemoral approach. We reported on a successful left carotid artery stenting case using right brachial artery approach in a bovine aortic arch. This paper is worthy of reporting in terms of guiding physicians for interventional procedures in these types of challenging cases.

  11. Leptin levels predict survival in pulmonary arterial hypertension

    OpenAIRE

    Tonelli, Adriano R.; Aytekin, Metin; Ariel E Feldstein; Dweik, Raed A

    2012-01-01

    Evidence suggests that leptin is involved in relevant processes in the cardiovascular system. Low serum leptin levels have been associated with increased cardiovascular events and mortality in patients with coronary artery, diabetes, or chronic kidney disease. We hypothesized that leptin is increased in pulmonary arterial hypertension (PAH) and provides prognostic information. We correlated leptin levels with clinical data and assessed its association with survival. Sixty-seven patients with ...

  12. A Microstructurally-Driven Model for Pulmonary Artery Tissue

    OpenAIRE

    Kao, Philip H; Lammers, Steve; Tian, Lian; Hunter, Kendall; Stenmark, Kurt R.; Shandas, Robin; Qi, H. Jerry

    2011-01-01

    A new constitutive model for elastic, proximal pulmonary artery tissue is presented here, called the Total Crimped Fiber Model. This model is based on the material and micro-structural properties of the two main, passive, load-bearing components of the artery wall, elastin and collagen. Elastin matrix proteins are modeled with an orthotropic neo-Hookean material. High stretch behavior is governed by an orthotropic crimped fiber material, modeled as a planar sinusoidal linear elastic beam, whi...

  13. 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...

  14. Arterial pulmonary hypertension in noncardiac intensive care unit

    OpenAIRE

    Tsapenko, Mykola

    2008-01-01

    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 elev...

  15. Endothelial cells and pulmonary arterial hypertension: apoptosis, proliferation, interaction and transdifferentiation

    OpenAIRE

    Sakao Seiichiro; Tatsumi Koichiro; Voelkel Norbert F

    2009-01-01

    Abstract Severe pulmonary arterial hypertension, whether idiopathic or secondary, is characterized by structural alterations of microscopically small pulmonary arterioles. The vascular lesions in this group of pulmonary hypertensive diseases show actively proliferating endothelial cells without evidence of apoptosis. In this article, we review pathogenetic concepts of severe pulmonary arterial hypertension and explain the term "complex vascular lesion ", commonly named "plexiform lesion", wit...

  16. Pulmonary blood flow distribution in transposition of the great arteries

    International Nuclear Information System (INIS)

    Pulmonary blood flow distribution was studied by scintillation scanning of the lungs after the infusion of iodine-131-labeled macroaggregates of human albumin before and after the Mustard operation in 53 patients with transposition of the great arteries. The patients were classified as follows: Group 1 (24 infants with uncomplicated transposition of the great arteries); Group II (18 patients with transposition and ventricular septal defect); and Group III (11 patients with transposition, ventricular septal defect and pulmonary obstruction). Before operation, 21 patients had a normal distribution of pulmonary blood flow, 10 had preferential flow to the right lung and 2 had preferential flow to the left lung. After operation, 19 had a normal pattern of pulmonary blood flow, 21 had preferential flow to the right lung and 3 had preferential flow to the left lung. The scanning studies have proved helpful in follow-up of patients to rule out recurrence of the shunt, pulmonary or systemic venous obstruction, development of pulmonary hypertension and occlusion of a palliative systemic-pulmonary shunt

  17. Tissue Doppler Findings in Patients with Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    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

  18. Recent trends in pulmonary arterial hypertension

    OpenAIRE

    Rajagopalan Natarajan

    2011-01-01

    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 ...

  19. Effect of Parachlamydia acanthamoebae on pulmonary function parameters in a bovine respiratory model.

    Science.gov (United States)

    Lohr, M; Prohl, A; Ostermann, C; Diller, R; Greub, G; Reinhold, P

    2016-07-01

    The aim of this study was to evaluate pulmonary dysfunction induced by experimental infection with Parachlamydia acanthamoebae in calves. Intrabronchial inoculation with P. acanthamoebae was performed in 31 calves aged 2-3 months old at two different challenge doses of 10(8) and 10(10) inclusion-forming units (IFU) per animal. Control animals received heat inactivated bacteria. The effects on pulmonary gas exchange were determined by arterial blood gas analysis and haemoximetry during the 7 days post inoculation (DPI). For pulmonary function testing (PFT), impulse oscillometry, capnography, and measurement of O2 uptake were undertaken in spontaneously breathing animals 7 and 3 days before inoculation and were repeated until 10 DPI. In the early phase after challenge (1-3 DPI), mild hypoxaemia occurred, which was accompanied by a significant reduction in both tidal and alveolar volumes (each related to bodyweight, BW). In parallel, expiratory flow rate and specific ventilation (i.e. minute ventilation related to O2 uptake) were significantly increased. Minute and alveolar ventilations (each related to metabolic BW) increased significantly due to higher respiratory rates, lasting until 4 and 5 DPI, respectively. Oxygen uptake was slightly reduced during the first 2 days after challenge, but increased significantly during the recovery phase, from 4 to 8 DPI. No deterioration in respiratory mechanics or acid-base balance was observed. Respiratory infection with 10(10) IFU P. acanthamoebae per calf induced mild respiratory dysfunction, mainly characterised by hypoxaemia. The study's findings do not indicate severe pathophysiological consequences of P. acanthamoebae infection on pulmonary function in the bovine host. PMID:27240907

  20. 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.

  1. 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

  2. Clopidogrel in infants with systemic-to-pulmonary-artery shunts

    DEFF Research Database (Denmark)

    Wessel, David L; Berger, Felix; Li, Jennifer S;

    2013-01-01

    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...

  3. SECONDARY PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC DISEASES OF CONNECTIVE TISSUE

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2009-01-01

    Full Text Available Modern definition of pulmonary arterial hypertension (PAH as well as data on prevalence and incidence of secondary PAH in systemic disease of connective tissue is presented,  including data of USA, France and Scotland registers. The main chains of pathogenesis, classification approaches, clinical features and diagnostics are described. 

  4. SECONDARY PULMONARY ARTERIAL HYPERTENSION IN SYSTEMIC DISEASES OF CONNECTIVE TISSUE

    Directory of Open Access Journals (Sweden)

    N. A. Shostak

    2016-01-01

    Full Text Available Modern definition of pulmonary arterial hypertension (PAH as well as data on prevalence and incidence of secondary PAH in systemic disease of connective tissue is presented,  including data of USA, France and Scotland registers. The main chains of pathogenesis, classification approaches, clinical features and diagnostics are described. 

  5. Bullet embolism of pulmonary artery: a case report

    Directory of Open Access Journals (Sweden)

    Mauricio Gustavo Ieiri Yamanari

    2014-04-01

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

  6. Pulmonary arterial hypertension as a manifestation of lupus erythematosus

    International Nuclear Information System (INIS)

    We present five patients with systemic lupus erythematosus (SLE) who developed pulmonary arterial hypertension and cor pulmonale in the course of their disease. The clinical features, as well as, the radiological manifestations of this rare manifestation of SLE are discussed. A vasculitic process is the most likely cause of this complication. Therapy is ineffective and the prognosis is poor. (orig.)

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

    International Nuclear Information System (INIS)

    Increased stiffness of the pulmonary vascular bed is known to increase mortality in patients with pulmonary arterial hypertension (PAH); and pulmonary artery (PA) stiffness is also thought to be associated with exercise capacity. The purpose of the present study was to investigate whether cardiac magnetic resonance imaging (CMRI)-derived PA distensibility index correlates with PA stiffness estimated on right heart catheterization (RHC) and predicts functional capacity (FC) in patients with PAH. Thirty-five consecutive PAH patients (23% male, mean age, 44±13 years; 69% idiopathic) underwent CMRI, RHC, and 6-min walk test (6MWT). PA distensibility indices were derived from cross-sectional area change (%) in the transverse view, perpendicular to the axis of the main PA, on CMRI [(maximum area-minimum area)/minimum area during cardiac cycle]. Among the PA stiffness indices, pulmonary vascular resistance (PVR) and PA capacitance were calculated using hemodynamic dataset from RHC. CMRI-derived PA distensibility was inversely correlated with PVR (R2=0.34, P2=0.35, P2=0.61, P<0.001). Furthermore, PA distensibility <20% predicted poor FC (<400 m in 6MWT) with a sensitivity of 82% and a specificity of 94%. Non-invasive CMRI-derived PA distensibility index correlates with PA stiffness and can predict FC in patients with PAH. (author)

  8. 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.)

  9. Pulmonary Root Translocation with the Lecompte Maneuver: For Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis

    OpenAIRE

    Yoon, Dong Woog; Kim, Tae Ho; Shim, Man-shik; Jun, Tae-Gook; Jang, Jae Seok

    2015-01-01

    A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed we...

  10. [Pulmonary arterial hypertension, bone marrow, endothelial cell precursors and serotonin].

    Science.gov (United States)

    Ayme-Dietrich, Estelle; Banas, Sophie M; Monassier, Laurent; Maroteaux, Luc

    2016-01-01

    Serotonin and bone-marrow-derived stem cells participate together in triggering pulmonary hypertension. Our work has shown that the absence of 5-HT2B receptors generates permanent changes in the composition of the blood and bone-marrow in the myeloid lineages, particularly in endothelial cell progenitors. The initial functions of 5-HT2B receptors in pulmonary arterial hypertension (PAH) are restricted to bone-marrow cells. They contribute to the differentiation/proliferation/mobilization of endothelial progenitor cells from the bone-marrow. Those bone-marrow-derived cells have a critical role in the development of pulmonary hypertension and pulmonary vascular remodeling. These data indicate that bone-marrow derived endothelial progenitors play a key role in the pathogenesis of PAH and suggest that interactions involving serotonin and bone morphogenic protein type 2 receptor (BMPR2) could take place at the level of the bone-marrow. PMID:27687599

  11. Pulmonary artery denervation for treatment of a patient with pulmonary hypertension secondary to left heart disease

    OpenAIRE

    Zhang, Hang; Zhang, Juan; Xie, Du-Jiang; Jiang, Xiaoming; Zhang, Feng-Fu; Chen, Shao-Liang

    2016-01-01

    Pulmonary hypertension (PH) predicts poor outcome in patients with left heart disease. A 62-year-old man was referred for heart failure associated with ischemic cardiomyopathy. He received a diagnosis of combined postcapillary and precapillary PH secondary to left heart disease on the basis of hemodynamic parameters. After the pulmonary artery denervation procedure was performed, hemodynamic parameters were markedly improved, which resulted in a significant increase in functional capacity.

  12. Acute Effects of Vardenafil on Pulmonary Artery Responsiveness in Pulmonary Hypertension

    Directory of Open Access Journals (Sweden)

    Edibe Karasu-Minareci

    2012-01-01

    Full Text Available Phosphodiesterase type-5 (PDE-5 inhibitors are novel and important options for the treatment of pulmonary arterial hypertension (PAH. Therefore, we aimed to examine effects of vardenafil, a PDE-5 inhibitor, on the pulmonary arteries isolated from rats with monocrotaline- (MCT- induced pulmonary hypertension. MCT (60 mg/kg or its vehicle was administered by a single intraperitoneal injection to 6-week-old male Sprague Dawley rats. Rats were sacrificed 21 days after MCT injection, and the main pulmonary arteries were isolated and then mounted in 20 mL organ baths. Concentration-response curves for vardenafil (10−10–10−5 M were constructed in phenylephrine- (Phe- precontracted rings. PAH caused marked rightward shift in the curves to vardenafil whereas maximal responses were not affected. Inhibition of NO synthase (L-NAME, 10−4 M or guanylyl cyclase (ODQ, 10−5 M caused similar attenuation in responses evoked by vardenafil. Moreover, contraction responses induced by CaCl2 (3×10−5–3×10−2 M were significantly reduced in concentration-dependent manner by vardenafil. In conclusion, vardenafil induced pulmonary vasodilatation via inhibition of extracellular calcium entry in addition to NO-cGMP pathway activation. These results provide evidence that impaired arterial relaxation in PAH can be prevented by vardenafil. Thus, vardenafil represents a valuable therapeutic approach in PAH besides other PDE-5 inhibitors.

  13. 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. PMID:26084306

  14. 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.

  15. 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

  16. 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

  17. 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.

  18. The limits of oral therapy in pulmonary arterial hypertension management

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    Liu QQ

    2015-11-01

    Full Text Available Qian-Qian Liu,1,2 Zhi-Cheng Jing1,3 1Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, People’s Republic of China; 2Department of Echocardiography, Fu Wai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China; 3State Key Laboratory of Cardiovascular Disease, Fu Wai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China Abstract: Pulmonary arterial hypertension (PAH is a devastating disease in which remodeling of the small pulmonary arteries leads to a progressive increase in pulmonary vascular resistance and right-sided heart failure. Over the past decade, new treatments for PAH, such as the use of ERAs, PDE-5 inhibitors and prostacyclin analogs, have brought about dramatic improvements in clinical outcomes. Epoprostenol infusion therapy has been shown to improve hemodynamics, functional status, and survival, and it remains the gold standard for treatment of patients with severe PAH. Many agents, approved for PAH are always delivered in pill form. Although oral therapy occupies an important position, it has some drawbacks and limitations in PAH management. For patients in World Health Organization functional class IV and with severe right heart failure, there are few data on the long-term survival of patients treated with oral medications. Further research, exploration, and clinical experience with oral therapy in severe PAH and combination therapy will redefine its position in PAH management. Keywords: pulmonary arterial hypertension, right heart failure, oral therapy, survival

  19. Endovascular embolization through pulmonary artery access for refractory massive hemoptysis

    International Nuclear Information System (INIS)

    Objective: To determine the effectiveness of endovascular embolization through pulmonary artery access in patients with refractory massive hemoptysis in whom systemic artery (SA) embolization is ineffective or contraindicated. Methods: A total of 102 patients were treated with SA embolization for hemoptysis. Of the 102 patients,6 patients had severe persistent hemoptysis despite complete SA embolization and 1 patient had severe hemoptysis following complete bronchial artery embolization and other SA embolization was contraindicated. The underlying diseases were chronic cavitary pulmonary tuberculosis (n=3), chronic cavitary pulmonary tuberculosis complicated with aspergilloma (n=1), tuberculous bronchiectasis (n=1), severe necrotizing pneumonia (n=1) and bronchiectasis complicated with pneumatocele (n=1). The findings of SA angiography, main pulmonary angiography and selective pulmonary angiography were analyzed. Endovascular embolization was performed in patients with the detectable pathology in PA and the clinical results were observed. Results: The findings of SA angiography showed bronchopulmonary shunting in all cases, and pseudoaneurysm of PA in 2 cases and hypertrophy of peripheral PA in 2 cases. The main PA angiography demonstrated pseudoaneurysm of PA in 1 case and hypoperfusion of the diseased PA in other case. The selective PA angiography demonstrated pseudoaneurysm of PA in 4 cases (1 case with extravasation of contrast medium) and hypertrophy of peripheral PA in 2 cases. Coil embolization of the pathologic PA were successfully performed and bleeding ceased in all patients. During follow-up, 1 patient had episodic bloody sputum after embolization, and 2 died day 6 and 15 of severe infection and respiratory failure and the remaining patients were all stable, Conclusions: In patients with refractory massive hemoptysis after systemic embolization, the possibility of PA pathology, especially pseudoaneurysm of PA should be considered. Selective pulmonary

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

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    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.

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

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    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.

  2. Adenocarcinoma pulmonar em um bovino Pulmonary adenocarcinoma in a bovine

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    Aline de Marco Viott

    2010-02-01

    bronchial and mediastinal lymph nodes, parietal pericardium, epicardium, and adventitia of the pulmonary artery. Microscopically, the pulmonary tissue mass was composed of neoplastic epithelial cells arranged in acini lined by two or more layers, some with intraluminal papillary projections. Anisokaryosis was marked, and mitotic index was moderate (2-3 mitosis in high field. Abundant fibrous connective tissue surrounded the neoplastic cell aggregates. Foci of necrosis and mineralization were moderate. Similar microscopic changes were observed in bronchial and mediastinal lymph nodes, visceral and parietal pericardium and adventitia of the pulmonary artery. With liver chronic generalized congestion exception, no other macro or microscopic lesions were observed. Microscopic findings were consistent with pulmonary adenocarcinoma with regional metastases. The right-sided congestive heart failure was probably due to obstruction of lymphatic drainage by metastases.

  3. Pulmonary stenosis development and reduction of pulmonary arterial hypertension in atrioventricular septal defect: a case report

    Directory of Open Access Journals (Sweden)

    Ninet Gérard

    2009-09-01

    Full Text Available Abstract A 24-year-old patient was admitted for dyspnoea and syncope. He had a previous history of complete atrio-ventricular septal defect and trisomy 21. At the age of 6 months, in 1984, cardiac catheterization revealed a quasi-systemic pulmonary arterial hypertension with a bidirectional shunt corresponding to an Eisenmenger syndrome. Corrective cardiac surgery was not performed at this time because surgical risk was considered too high. Until the age of 20 years old, he showed few symptoms while under medical treatment. But since 2006, his functional status became worse with an increased dyspnoea, syncopes, and severe cyanosis. In these conditions, haemodynamic parameters have been re-evaluated in 2006 and 2008. They highlighted a late and progressive development of a valvular and infundibular pulmonary stenosis leading to a normalisation of pulmonary arterial pressures. At the age of 24 , the patient underwent corrective cardiac surgery which was successful. Late development of both infundibular and valvular pulmonary stenosis have not been described before in non operated congenital ventricular septal defects, but development of one or the other abnormality would be found in 8% of patients. The physiopathological mechanism of this obstruction is unclear. Nevertheless, in unoperated congenital cardiac shunt lesions, reversibility of severe pulmonary arterial hypertension should be reconidered and re-assessed during follow up.

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

    International Nuclear Information System (INIS)

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

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

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    Yunus Nazli

    2013-08-01

    Full Text Available Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA, also known as Garland-Bland-White syndrome, is an extremely rare but potentially fatal congenital cardiovascular anomaly and it often exists as an isolated condition. We report an unusual case of a 32 years-old patient with ALCAPA presenting with chest pain and dyspnea who underwent surgical correction of this rare anomaly. This anomaly was simply repaired by the combination of LMCA ligation and coronary artery bypass grafting.

  6. [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.

  7. Versican accumulates in vascular lesions in pulmonary arterial hypertension.

    Science.gov (United States)

    Chang, Ya-Ting; Chan, Christina K; Eriksson, Inger; Johnson, Pamela Y; Cao, Xiaofang; Westöö, Christian; Norvik, Christian; Andersson-Sjöland, Annika; Westergren-Thorsson, Gunilla; Johansson, Staffan; Hedin, Ulf; Kjellén, Lena; Wight, Thomas N; Tran-Lundmark, Karin

    2016-09-01

    Pulmonary arterial hypertension (PAH) is a lethal condition for which there is no effective curative pharmacotherapy. PAH is characterized by vasoconstriction, wall thickening of pulmonary arteries, and increased vascular resistance. Versican is a chondroitin sulfate proteoglycan in the vascular extracellular matrix that accumulates following vascular injury and promotes smooth-muscle cell proliferation in systemic arteries. Here, we investigated whether versican may play a similar role in PAH. Paraffin-embedded lung sections from patients who underwent lung transplantation to treat PAH were used for immunohistochemistry. The etiologies of PAH in the subjects involved in this study were idiopathic PAH, scleroderma, and congenital heart disease (atrial septal defect) with left-to-right shunt. Independent of the underlying etiology, increased versican immunostaining was observed in areas of medial thickening, in neointima, and in plexiform lesions. Western blot of lung tissue lysates confirmed accumulation of versican in patients with PAH. Double staining for versican and CD45 showed only occasional colocalization in neointima of high-grade lesions and plexiform lesions. In vitro, metabolic labeling with [(35)S]sulfate showed that human pulmonary artery smooth-muscle cells (hPASMCs) produce mainly chondroitin sulfate glycosaminoglycans. In addition, hypoxia, but not cyclic stretch, was demonstrated to increase both versican messenger RNA expression and protein synthesis by hPASMCs. Versican accumulates in vascular lesions of PAH, and the amount of versican correlates more with lesion severity than with underlying etiology or inflammation. Hypoxia is a possible regulator of versican accumulation, which may promote proliferation of pulmonary smooth-muscle cells and vascular remodeling in PAH. PMID:27683612

  8. Secondary Pulmonary Arterial Hypertension Treated with Endothelin Receptor Blockade

    OpenAIRE

    Sharma, Sat; Kashour, Tarek; Philipp, Roger

    2005-01-01

    Secondary pulmonary arterial hypertension (SPAH) is an adverse outcome of a variety of systemic disorders. These include collagen vascular diseases, chronic thromboembolism, human immunodeficiency virus, portopulmonary hypertension, and other diseases. Progression of SPAH may persist despite stabilization of the causative disease, thereby contributing to the poor quality of life and unfavorable survival in these patients. Treatment of the underlying cause and oxygen supplementation may allevi...

  9. The measurement of blood speed in the pulmonary artery trunk

    International Nuclear Information System (INIS)

    The paper describes a non invasive methodology for the measurement of blood speed in the pulmonary artery trunk. The methodology has been tested with a moving radioactive tracer (nuclear medicine). An image processing technique is proposed, for detection and analysis of a moving object with variable shape and intensity over time (radioactive bolus). Experiments on the application of the technique in nuclear medicine are critically analysed. (authors)

  10. 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.

  11. 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.

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

    Science.gov (United States)

    Montani, David; Seferian, Andrei; Savale, Laurent; Simonneau, Gérald; Humbert, Marc

    2013-09-01

    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. PMID:23997051

  13. Mitochondrial Haplogroups and Risk of Pulmonary Arterial Hypertension.

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    Samar Farha

    Full Text Available Pulmonary arterial hypertension (PAH is a serious and often fatal disease. It is a panvasculopathy of the pulmonary microcirculation characterized by vasoconstriction and arterial obstruction due to vascular proliferation and remodeling and ultimately right ventricular failure. Mitochondrial dysfunction is a universal finding in pulmonary vascular cells of patients with PAH, and is mechanistically linked to disease origins in animal models of pulmonary hypertension. Mitochondria have their own circular DNA (mtDNA, which can be subgrouped into polymorphic haplogroup variants, some of which have been identified as at-risk or protective from cardiovascular and/or neurodegenerative diseases. Here, we hypothesized that mitochondrial haplogroups may be associated with PAH. To test this, mitochondrial haplogroups were determined in a cohort of PAH patients and controls [N = 204 Caucasians (125 PAH and 79 controls and N = 46 African Americans (13 PAH and 33 controls]. Haplogroup L was associated with a lower rate of PAH as compared to macrohaplogroups N and M. When haplogroups were nested based on ancestral inheritance and controlled for age, gender and race, haplogroups M and HV, JT and UK of the N macro-haplogroup had significantly higher rates of PAH compared to the ancestral L (L0/1/2 and L3 (all p ≤ 0.05. Overall, the findings suggest that mitochondrial haplogroups influence risk of PAH and that a vulnerability to PAH may have emerged under the selective enrichment of specific haplogroups that occurred with the migration of populations out of Africa.

  14. Mitochondrial Haplogroups and Risk of Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Farha, Samar; Hu, Bo; Comhair, Suzy; Zein, Joe; Dweik, Raed; Erzurum, Serpil C; Aldred, Micheala A

    2016-01-01

    Pulmonary arterial hypertension (PAH) is a serious and often fatal disease. It is a panvasculopathy of the pulmonary microcirculation characterized by vasoconstriction and arterial obstruction due to vascular proliferation and remodeling and ultimately right ventricular failure. Mitochondrial dysfunction is a universal finding in pulmonary vascular cells of patients with PAH, and is mechanistically linked to disease origins in animal models of pulmonary hypertension. Mitochondria have their own circular DNA (mtDNA), which can be subgrouped into polymorphic haplogroup variants, some of which have been identified as at-risk or protective from cardiovascular and/or neurodegenerative diseases. Here, we hypothesized that mitochondrial haplogroups may be associated with PAH. To test this, mitochondrial haplogroups were determined in a cohort of PAH patients and controls [N = 204 Caucasians (125 PAH and 79 controls) and N = 46 African Americans (13 PAH and 33 controls)]. Haplogroup L was associated with a lower rate of PAH as compared to macrohaplogroups N and M. When haplogroups were nested based on ancestral inheritance and controlled for age, gender and race, haplogroups M and HV, JT and UK of the N macro-haplogroup had significantly higher rates of PAH compared to the ancestral L (L0/1/2 and L3) (all p ≤ 0.05). Overall, the findings suggest that mitochondrial haplogroups influence risk of PAH and that a vulnerability to PAH may have emerged under the selective enrichment of specific haplogroups that occurred with the migration of populations out of Africa. PMID:27224443

  15. SGLT inhibitors attenuate NO-dependent vascular relaxation in the pulmonary artery but not in the coronary artery.

    Science.gov (United States)

    Han, Ying; Cho, Young-Eun; Ayon, Ramon; Guo, Rui; Youssef, Katia D; Pan, Minglin; Dai, Anzhi; Yuan, Jason X-J; Makino, Ayako

    2015-11-01

    Inhibitors of sodium-glucose cotransporter (SGLT)2 are a new class of oral drugs for type 2 diabetic patients that reduce plasma glucose levels by inhibiting renal glucose reabsorption. There is increasing evidence showing the beneficial effect of SGLT2 inhibitors on glucose control; however, less information is available regarding the impact of SGLT2 inhibitors on cardiovascular outcomes. The present study was designed to determine whether SGLT inhibitors regulate vascular relaxation in mouse pulmonary and coronary arteries. Phlorizin (a nonspecific SGLT inhibitor) and canagliflozin (a SGLT2-specific inhibitor) relaxed pulmonary arteries in a dose-dependent manner, but they had little or no effect on coronary arteries. Pretreatment with phlorizin or canagliflozin significantly inhibited sodium nitroprusside (SNP; a nitric oxide donor)-induced vascular relaxation in pulmonary arteries but not in coronary arteries. Phlorizin had no effect on cGMP-dependent relaxation in pulmonary arteries. SNP induced membrane hyperpolarization in human pulmonary artery smooth muscle cells, and pretreatment of cells with phlorizin and canagliflozin attenuated SNP-induced membrane hyperpolarization by decreasing K(+) activities induced by SNP. Contrary to the result observed in ex vivo experiments with SGLT inhibitors, SNP-dependent relaxation in pulmonary arteries was not altered by chronic administration of canagliflozin. On the other hand, canagliflozin administration significantly enhanced SNP-dependent relaxation in coronary arteries in diabetic mice. These data suggest that SGLT inhibitors differentially regulate vascular relaxation depending on the type of arteries, duration of the treatment, and health condition, such as diabetes. PMID:26361875

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

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Young Hi [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1984-09-15

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

  17. 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.

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

    OpenAIRE

    Streit Michael; Speich Rudolf; Fischler Manuel; Ulrich Silvia

    2009-01-01

    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 caes...

  19. 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.

  20. Ambrisentan for the treatment of pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Brian Casserly

    2008-12-01

    Full Text Available Brian Casserly1,3, James R Klinger2,31Division of Pulmonary Medicine, The Memorial Hospital of Rhode Island, Pawtucket, RI, USA; 2Division of Pulmonary Sleep and Critical Care Medicine, Rhode Island Hospital; 3Waren Alpert Medical School of Brown University, Providence, RI, USAAbstract: Ambrisentan is an endothelin receptor antagonist (ERA that was recently approved for treatment of pulmonary arterial hypertension (PAH. Endothelin (ET is a potent vasoconstrictor with mitogenic, hypertrophic and pro-inflammatory properties that is upregulated in pulmonary hypertensive diseases. The biologic effects of ET are mediated by 2 cell surface receptors termed ETA and ETB. ETA mediates the vasoconstrictor effect of ET on vascular smooth muscle, whereas ETB is expressed primarily on vascular endothelial cells where it induces nitric oxide synthesis and acts to clear ET from the circulation. Ambrisentan is the first ETA selective ERA approved for use in the US. Recently published clinical trials in patients with PAH demonstrate improvement in functional capacity and pulmonary hemodynamics similar to other ETA selective and non-selective ERAs. Its once daily dosing and lower incidence of serum aminotransferase elevation offer potential advantages over other ERAs, but further experience with this agent is needed to fully understand its long-term efficacy and safety. This review discusses the endothelin family of proteins and receptors and their role in the pathophysiology of pulmonary hypertensive diseases. It also examines the development process, safety profile and clinical trials that have resulted in ambrisentan being approved for treatment of PAH.Keywords: ambrisentan, endothelin receptor antagonist, pulmonary hypertension, endothelin

  1. Interim prostacyclin therapy for an isolated disconnected pulmonary artery: a case report

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    Grixti Cynthia

    2010-06-01

    Full Text Available Abstract Introduction Disconnected pulmonary arteries are unusual and may result in pulmonary hypertension with acute right heart failure. Case presentation We report a case of a three-month-old Asian girl who presented with heart failure and severe pulmonary hypertension due to a disconnected right pulmonary artery. An epoprostenol (prostacyclin infusion was instrumental in lowering pulmonary artery pressures and stabilizing the child prior to surgery. Conclusions This is, to the best of our knowledge, the first report of successful prostacyclin usage in such a situation.

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

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    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.

  3. 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.

  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)

    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.

  5. Pulmonary arterial dimensions and right ventricular function by cardiac MRI

    International Nuclear Information System (INIS)

    Objective: To evaluate the diagnostic value of cardiac magnetic resonance imaging (CMRI) for pulmonary arterial hypertension (PAH). Methods: One hundred and thirty patients with PAH confirmed by right cardiac catheterization were examined by CMRI and the results were compared with that of 31 healthy control participants. The main pulmonary artery diameter (MPAD), aortic diameter (AOD), main pulmonary artery diameter/aortic diameter (MPAD/AOD), right ventricular end-diastolic volume (RVEDV), right ventricular end-systolic volume (RVESV), right ventricular ejection fraction (RVEF) and right ventricular mass (RVM) were measured. The independent samples t-test was used to compare the PAH group with the control group. The Pearson correlation analysis and linear regression analysis were used to evaluate the relationship between cardiac and arterial measurements and pulmonary arterial pressure (PAP). Results: The MPAD, MPAD/AOD, RVEDV, RVESV, RVM in PAH group [(3.88 ±0.57) cm,1.36 ±0.17,(161.63 ±56.37) ml,(112.61 ±41.46) ml,(82.70 ± 20.73) g, respectively] were increased compared with those in normal control group [(2.74 ±0.31 ) cm, 0.90 ±0.07, (131.31 ± 15.14) ml, (61.33±9.00) ml, (44.39±5.87) g, respectively]. The RVSV and RVEF in PAH group[(49.02 ±19.20) ml, (30.76 ± 5.85 )%, respectively] were decreased compared with those in normal control group [(69.95 ± 9.63 )ml, (53.28 ± 4.14)%, respectively]. The MPAD, MPAD/AOD, RVEDV, RVESV, RVSV, RVEF, RVM were significantly different between PAH patients and control participants (tMPAD=10.82, tMPAD/AOD=14.93, tRVEDV=2.96, tRVESV=6.83, tRVSV=-5.89, tRVEF=-20.22, tRVM=10.12, respectively, P<0.01). There were no significant correlations between MPAD, RVEDV, RVESV, RVSV and PAP (r=0.299 for MPAD, r=0.127 for RVEDV, r=0.278 for RVESV, r=-0.229 for RVSV). Moderate positive correlations were found between MPAD/AOD, RVM and PAP (r=0.702 for MPAD/AOD, r=0.683 for RVM ). A moderate negative correlation was found between

  6. 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.

  7. [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.

  8. [Drug therapy of pulmonary arterial hypertension in 2014].

    Science.gov (United States)

    Aschermann, Michael; Jansa, Pavel

    2014-04-01

    Pulmonary arterial hypertension (PAH) is a primary pulmonary arteriolar disease, characterized by a progressive increase in pulmonary vascular resistance and pressure in the pulmonary circulation. It progressively leads to hypertrophy of the right ventricle and with no treatment to its failure and patient´s death. Etiology of pulmonary hypertension (PH) has been reclassified repeatedly, most recently during the 4th World Symposium on Pulmonary Hypertension held in 2008 [1]. Currently, the first group contains PAH with either unknown or known cause (systemic connective tissue disease, liver disease, congenital heart disease, HIV infection, abuse of anorexic agents). Current drug therapy of PAH is divided into conventional (anticoagulant therapy, calcium channel blockers, therapy of chronic heart failure) and specific (prostanoids, endothelium receptor antagonists, phosphodiesterase 5 inhibitors). Patients with positive vasodilator test are indicated for the high doses treatment of calcium channel blockers. Patients with negative vasodilator test are indicated for chronic anticoagulant therapy and specific drug therapy either as mono-therapy, or as combined therapy. Recent years have brought a wide range of new treatments modalities, especially in the field of pharmacotherapy. In addition, other treatment modalities have been tested, for example application of stem cells. Drugs in research include several groups: 1. vasodilators: fasudil, adrenonedullin, activators and stimulators of guanylate cyclase, vasoactive intestinal peptide (VIP); 2. Anti-inflammatory agents: inhibitor of elastase, antagonist of B cells, immunosuppressive agents, inhibitor of HDAC1; 3. agents affecting metabolism: nitrites, PPAR antagonists, antioxidants, serotonin receptor antagonist and serotonin transporter blockers, statins, inhibitors of Rho-kinase; 4. apoptosis inductors of smooth muscle cells: tyrosine-kinase inhibitors, elastase inhibitors; 5. agents influencing vascular regeneration

  9. 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.

  10. 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.

  11. Abnormal branch of right pulmonary artery (A7): a case report and literature review.

    Science.gov (United States)

    Atari, Maiko; Nakajima, Yuki; Fukuhara, Mitsuro; Iijima, Yoshihito; Kinoshita, Hiroyasu; Akiyama, Hirohiko; Minamiya, Yoshihiro; Uramoto, Hidetaka

    2016-12-01

    In thoracic surgery, anatomic variations of pulmonary artery increase the risks for vessel injury and critical mistakes during pulmonary artery resection. We report a case of lung cancer with an extremely rare branch, a mediastinal A7 pulmonary artery. Some case reports of the mediastinal pulmonary artery exist until now. However, to the best of our knowledge, this is the first case of a medial basal segmental artery (from the following, it is referred to as A7) branching directly from main pulmonary artery in the literature. Therefore, there is no report that showed three-dimensional computed tomography (3D-CT) and operative findings. So, these information is very useful for thoracic surgeon. A 67-year-old man was admitted to our hospital in order to undergo operation for the treatment of lung cancer. We detected the anomalies preoperatively by 3D-CT. The 3D-CT shows the A7 pulmonary artery branches from the right main pulmonary artery directly. According to previous literature, the cases of a single branch from main pulmonary artery to lower lobe are only five cases. And, the only two of them are right side including our case. In spite of an extremely rare case, we were able to successfully perform a right middle lobectomy because the information obtained from the 3D-CT findings was sufficiently understood preoperatively. PMID:26943692

  12. Inaccuracy of doppler echocardiographic estimates of pulmonary artery pressures in adult atrial septal defect patients with pulmonary arterial hypertension

    Institute of Scientific and Technical Information of China (English)

    Zhang Caojin; Huang Tao; Huang Xinsheng; Huang Yigao; Chen Jimei; Chen Jiyan; Wu Shulin

    2014-01-01

    Background While echocardiography has been a pivotal screening test in pulmonary arterial hypertension (PAH),the presence of structural cardiac defects may affect the ability to reliably predict pulmonary artery pressures (PAPs).This study sought to evaluate the accuracy of Doppler echocardiography (DE) for estimating PAPs in adult atrial septal defect (ASD) patients with PAH.Methods A prospective study was carried out to compare the echocardiographic assessment of PAP with the same pressures obtained by right heart catheterization (RHC) in adult ASD patients with PAH who underwent simultaneous DE and RHC.Bland-Altman analyses were performed to evaluate the agreement between DE and RHC measurements of PAPs.Results Two hundred and fifty-seven patients were included in the study.A significant overestimation of the systolic pulmonary arterial pressure (sPAP) and mean pulmonary artery pressure (mPAP) was reported by echocardiography compared with those by catheterization ((81.8±26.9) mmHg vs.(72.9±26.9) mmHg,P <0.01; (51.9±16.4) mmHg vs.(41.4±17.2) mmHg,P <0.01,respectively).Twenty-one percent (55/257) of the patients had PAH when estimated by echocardiography whereas showed normal results in the subsequent catheterization test.Using Bland-Altman analytic methods,the bias for the echocardiographic assessment of the sPAP was 9.1 mmHg with 95% limits of agreement ranging from-24.4 to 42.6 mmHg.For mPAP measurement,the bias was 10.5 mmHg with 95% limits of agreement ranging from-12.4 to 33.4 mmHg.On multiple linear regression analysis,age,gender,body surface area,ASDs' diameter,PVR,diastolic blood pressure,and echocardiographic assessment of right atrial pressure (RAP) explained 68.8% of the total variability in the model (r2=0.688,P <0.01).Conclusion Inaccuracy was frequently reported in Doppler echocardiographic assessment of the PAP in adult ASD patients with PAH and was often associated with age,gender,body surface area,ASDs' diameter,pulmonary

  13. A Semi-Automated Quantification of Pulmonary Artery Dimensions in Computed Tomography Angiography Images

    OpenAIRE

    Berty, Holly L.; Simon, Marc; Chapman, Brian E.

    2012-01-01

    Quantifying vascular dimensions may provide a non-invasive means of diagnosing a variety of vascular diseases, including pulmonary hypertension, a progressive, potentially fatal disease that results in the remodeling of the pulmonary vasculature. Currently the gold standard for diagnosis of pulmonary hypertension is through right heart catheterization, an invasive and costly procedure. Since pulmonary hypertension is associated with the remodeling of the pulmonary arteries, quantifying vascul...

  14. 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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-03-15

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

  16. Isorhynchophylline protects against pulmonary arterial hypertension and suppresses PASMCs proliferation.

    Science.gov (United States)

    Guo, Haipeng; Zhang, Xin; Cui, Yuqian; Deng, Wei; Xu, Dachun; Han, Hui; Wang, Hao; Chen, Yuguo; Li, Yu; Wu, Dawei

    2014-07-18

    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 attenuate pulmonary vascular remodeling after MCT induction. These beneficial effects were at least through the inhibition of PDGF-Rβ phosphorylation and its downstream signaling pathways. Therefore, IRN might be a potential candidate for the treatment of PAH. PMID:24950404

  17. 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.

  18. [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. PMID:8086288

  19. Protein losing enteropathy secondary to a pulmonary artery stent

    International Nuclear Information System (INIS)

    A 2-year-old patient with hypoplastic left heart syndrome presented 6 months following Fontan completion with protein-losing enteropathy (PLE). He had undergone stent implantation in the left pulmonary artery after the Norwood procedure, followed by redilation of the stent prior to Fontan completion. Combined bronchoscopic and catheterization studies during spontaneous breathing confirmed left bronchial stenosis behind the stent, and diastolic systemic ventricular pressure during expiration of 25 mm Hg. We postulate that the stent acts as a valve, against which the patient generates high expiratory pressures, which are reflected in the ventricular diastolic pressure. This may be the cause of PLE

  20. Radiographic morphometry of alveoli and small pulmonary arteries

    International Nuclear Information System (INIS)

    This paper reports on quantitative analysis of radiographic fine structure of the lung which is an approach used to get functional information from anatomic data (e.g., a correlation of alveolar surface area, membrane thickness, and vessel density to diffusion capacity). More than 250 lung specimens were inflated and fixed with hot formalin vapor with use of an apparatus resembling a simplified Engstrom respirator. Radiographs were obtained of 1-cm sections of the fixed lungs in mammography technique. Pulmonary segmental arteries were filled before fixation with barium sulfate. Computer-assisted morphometry was performed on healthy and diseased lungs

  1. Protein losing enteropathy secondary to a pulmonary artery stent

    Directory of Open Access Journals (Sweden)

    Narayanswami Sreeram

    2012-01-01

    Full Text Available A 2-year-old patient with hypoplastic left heart syndrome presented 6 months following Fontan completion with protein-losing enteropathy (PLE. He had undergone stent implantation in the left pulmonary artery after the Norwood procedure, followed by redilation of the stent prior to Fontan completion. Combined bronchoscopic and catheterization studies during spontaneous breathing confirmed left bronchial stenosis behind the stent, and diastolic systemic ventricular pressure during expiration of 25 mm Hg. We postulate that the stent acts as a valve, against which the patient generates high expiratory pressures, which are reflected in the ventricular diastolic pressure. This may be the cause of PLE.

  2. 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.

  3. Changes in cardiopulmonary values after heartworm removal from pulmonary artery using flexible alligator forceps

    International Nuclear Information System (INIS)

    Radiograph, arteriograph, echocardiogram, electrocardiogram and cardiopulmonary values were obtained in dogs with dirofilariasis before and after heartworm removal with a flexible alligator forceps. After heartworm removal, the following results were obtained: 1) Dilations of the pulmonary artery, the right ventricle and the right atrium reduced on radiograph and ultrasonic echocardiogram; 2) tortuousness of the pulmonary artery was reduced, and pruning of the peripheral arteries improved on arteriogram: 3) electrocardiographic findings indicated the correction of right axis deviation in mean electric axis in frontal plane and decreases of voltages; 4) pulmonary arterial and right ventricular pressures fell (improvement of pulmonary hypertension); 5) right cardiac output decreased; 6) heart rate tended to decrease; and 7) total pulmonary arterial resistance decreased. These results indicated that pulmonary arterial resistance and blood flow volume might be reduced, thus decreasing the burden on the heart. It was considered that not only lesions of the pulmonary arteries but also heartworms themselves in the pulmonary artery play an important role in affecting right heart hemodynamics

  4. 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.

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

    Directory of Open Access Journals (Sweden)

    Ali Kemal Gür

    2013-12-01

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

  7. 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

  8. Effects of different pulmonary vasodilators on arterial saturation in a model of pulmonary hypertension.

    Directory of Open Access Journals (Sweden)

    Eva Maria Becker

    Full Text Available BACKGROUND: Approved therapies for pulmonary arterial hypertension can induce oxygen desaturation when administered to patients with secondary forms of pulmonary hypertension (PH, probably due to an increase in ventilation/perfusion mismatch. Thus, so far these treatments have largely failed in secondary forms of PH. METHODS: We established an animal model of heterogeneous lung ventilation to evaluate the desaturation potential of mechanistically distinct vasoactive drugs launched or currently in clinical development for the treatment of PH. Single-lung ventilation was induced in five groups (N = 6 of anesthetized minipigs (7 weeks, 4 to 5 kg BW, and their hemodynamic parameters were monitored before and after intravenous injection of control (vehicle only, endothelin antagonist (bosentan; 0.3, 1, 3, 10 mg/kg, phosphodiesterase type 5 inhibitor (sildenafil; 3, 10, 30, 100 µg/kg, and soluble guanylate cyclase stimulators (BAY 41-8543 and riociguat; 1, 3, 10, 30 µg/kg. Cumulative doses were administered before successive unilateral ventilation cycles. The doses were chosen to achieve equal effect on blood pressure by the different pharmacologic principles. RESULTS: Single-lung ventilation resulted in transient increases in mean pulmonary artery pressure (mPAP and desaturation. In contrast to control, all drugs dose-dependently decreased hypoxic mPAP (a positive treatment effect and increased area under the arterial hemoglobin saturation curve (unwanted desaturation effect. Riociguat and bosentan reduced hypoxic mPAP to the greatest extent, while the soluble guanylate cyclase stimulators riociguat and BAY 41-8543 lowered arterial oxygen saturation of hemoglobin the least. CONCLUSIONS: Future investigations will be required to confirm these findings in clinical settings.

  9. 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

  10. 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.

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

    OpenAIRE

    Bohn Olga L; de León Eric; Lezama Oscar; Rios-Luna Nina P; Sánchez-Sosa Sergio; Llombart-Bosch Antonio

    2012-01-01

    Abstract Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall or by histologic type. Angiosarcomas are malignant neoplasms that arise from the endothelial lining of the blood vessels; those arising in the intimal compartment of pulmonary artery are rare. We report a case of pulmonary artery angiosarcoma in a 36-year old female with pulmonary masses. The patient had no other primary malignant neoplasm, thus excluding a metastatic lesion. G...

  12. Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger’s syndrome: a clinical dilemma

    OpenAIRE

    Broberg, C.; Ujita, M.; Babu-Narayan, S.; Rubens, M; S.K. Prasad; GIBBS J.S.R.; Gatzoulis, M. A.

    2004-01-01

    Although the frequency of haemoptysis in Eisenmenger’s syndrome is well recognised, the high prevalence of pulmonary artery thrombus has been newly appreciated through the growing use of non-invasive imaging. Three patients with Eisenmenger’s syndrome with haemoptysis are reported who underwent computed tomography pulmonary angiography and cardiovascular magnetic resonance. Each patient was found to have aneurysmal dilatation of the right pulmonary artery with large laminar thrombus. These ca...

  13. Ultrasonic Estimation of Mechanical Properties of Pulmonary Arterial Wall Under Normoxic and Hypoxic Conditions

    Science.gov (United States)

    Waters, Kendall R.; Mukdadi, Osama M.

    2005-04-01

    Secondary pediatric pulmonary hypertension is a disease that could benefit from improved ultrasonic diagnostic techniques. We perform high-frequency in vitro ultrasound measurements (25 MHz to 100 MHz) on fresh and fixed pulmonary arterial walls excised from normoxic and hypoxic Long-Evans rat models. Estimates of the elastic stiffness coefficients are determined from measurements of the speed of sound. Preliminary results indicate that hypoxia leads to up to increase of 20 % in stiffening of the pulmonary arterial wall.

  14. Role of Hypoxia-Induced Brain Derived Neurotrophic Factor in Human Pulmonary Artery Smooth Muscle

    OpenAIRE

    Hartman, William; Helan, Martin; Smelter, Dan; Sathish, Venkatachalem; Thompson, Michael; Pabelick, Christina M.; Johnson, Bruce; Y S Prakash

    2015-01-01

    Background Hypoxia effects on pulmonary artery structure and function are key to diseases such as pulmonary hypertension. Recent studies suggest that growth factors called neurotrophins, particularly brain-derived neurotrophic factor (BDNF), can influence lung structure and function, and their role in the pulmonary artery warrants further investigation. In this study, we examined the effect of hypoxia on BDNF in humans, and the influence of hypoxia-enhanced BDNF expression and signaling in hu...

  15. Fenfluramine-induced gene dysregulation in human pulmonary artery smooth muscle and endothelial cells

    OpenAIRE

    Yao, Weijuan; Mu, Wenbo; Zeifman, Amy; Lofti, Michelle; Remillard, Carmelle V.; Makino, Ayako; Perkins, David L.; Garcia, Joe G.; Yuan, Jason X. J.; Zhang, Wei

    2011-01-01

    Fenfluramine is prescribed either alone or in combination with phentermine as part of Fen-Phen, an anti-obesity medication. Fenfluramine was withdrawn from the US market in 1997 due to reports of heart valvular disease, pulmonary arterial hypertension, and cardiac fibrosis. Particularly, idiopathic pulmonary arterial hypertension (IPAH), previously referred to as primary pulmonary hypertension (PPH), was found to be associated with the use of Fen-Phen, fenfluramine, and fenfluramine derivativ...

  16. 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.

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

    International Nuclear Information System (INIS)

    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.

  18. 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.)

  19. 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.

  20. 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.

  1. 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.

  2. 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 .

  3. 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

  4. 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.

  5. Effects of bosentan on peripheral endothelial function in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension

    OpenAIRE

    Hirashiki, Akihiro; Adachi, Shiro; Nakano, Yoshihisa; Kamimura, Yoshihiro; Shimokata, Shigetake; Takeshita, Kyosuke; Murohara, Toyoaki; Kondo, Takahisa

    2016-01-01

    Endothelin receptor antagonists (ERAs) have been shown to improve the prognosis of patients with pulmonary arterial hypertension (PAH). However, the effect of the oral dual ERA bosentan on peripheral endothelial dysfunction (PED), as assessed by flow-mediated vasodilation (FMD), in patients with pulmonary hypertension is not well characterized. We investigated the effect of bosentan on PED in patients with PAH or inoperable chronic thromboembolic pulmonary hypertension (CTEPH). A total of 18 ...

  6. The relationship between the pathologic changes of lung tissue in experimental pulmonary artery hypertension and pulmonary perfusion imaging

    International Nuclear Information System (INIS)

    Objective: To study the relationship between the pathologic changes of lung tissue in experimental pulmonary artery hypertension and pulmonary perfusion imaging. Methods: Twenty-nine japan big-eared white rabbits were used as the animal models. Among them, 13 rabbits underwent pulmonary perfusion imaging, 5 rabbits underwent cardiac catheterization and 2 rabbits underwent lung tissue biopsy before the experiment. Models of pulmonary artery hypertension (PAH) were established in 27 rabbits in different degrees by means of drug and shortage of oxygen. The rabbits were sacrificed after the pulmonary perfusion imaging and the cardiac catheterization to observe the pathological changes of lung tissue. Results: In cases of normal lung tissues, the alveoli and alveolar capsules were uniform in size and there were no dilatation of the arterioles and venulae; In pulmonary perfusion imaging, the radioactivity distribution count ratio of the dorsal side to the ventral side (as apex to bottom in human) was less than 1; The pressure detected by cardiac catheterization was normal. In cases of mild PAH, the lung tissue showed compensatory pulmonary emphysema and dilatation of the venulae; In pulmonary perfusion imaging, the radioactivity distribution count ratio of the dorsal side to the ventral side was more than or equal to 1, and there was difference compared with the normal control (P0.05). In cases of moderate PAH, endothelial cells of the arteriole proliferated and dropped, and the alveoli expanded and fused. In pulmonary perfusion imaging, the radioactivity distribution count ratio of the dorsal side to the ventral side was more than 1. There was significant difference compared with the normal control (P<0.01). The pressure detected by the cardiac catheterization increased significantly too (P<0.05, vs normal control). In severe PAH, hypertrophy was found in muscular layer of arteriole, and stenosis and distortion were found in endomembrane and mid membrane of the

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

    Science.gov (United States)

    Papathanasiou, Athanasios; Nakos, George

    2015-11-01

    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

  8. Combined use of non-invasive techniques to predict pulmonary arterial pressure in chronic respiratory disease.

    OpenAIRE

    Bishop, J M; Csukas, M

    1989-01-01

    The value of non-invasive procedures for predicting pulmonary arterial pressure was investigated in 370 patients with chronic obstructive lung diseases and in 73 with fibrosing alveolitis in a combined study at nine centres in six European countries. Measurements included forced expiratory volume in one second, arterial blood gas tensions, standard electrocardiogram, radiographic dimensions of pulmonary artery, right ventricle dimensions by M mode echocardiography, and myocardial scintigraphy...

  9. Veno-arterial ECMO for rescue of severe airway hemorrhage with rigid bronchoscopy after pulmonary artery thromboendarterectomy.

    Science.gov (United States)

    Chacón-Alves, Silvia; Pérez-Vela, Jose Luis; Grau-Carmona, Teodoro; Domínguez-Aguado, Helena; Marín-Mateos, Helena; Renes-Carreño, Emilio

    2016-07-01

    Pulmonary endarterectomy (PEA) is the treatment of choice to relieve pulmonary artery obstruction in patients with chronic thromboembolic pulmonary hypertension (CTEPH). We present a patient with airway obstruction and acute respiratory failure due to large blood clots obstructing the trachea and main left bronchus. This condition was accompanied by right ventricle failure and cardiogenic shock. A venoarterial ECMO system was used for cardiopulmonary support before extracting the clots and clearing the airway by rigid bronchoscopy. PMID:27229321

  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. The Effect of Respiratory Functions and Pulmonary Artery Pressure on Right and Left Ventricular Diastolic Function

    OpenAIRE

    Alpsoy, Şeref; Akyüz, Aydın; Akkoyun, Dursun Çayan; Oran, Mustafa; Mutlu, Levent Cem; TOPÇU, Birol; Değirmenci, Hasan; Yalçın, Banu Çiçek

    2012-01-01

    Introduction: The aim of the study was to evaluate of biventricular diastolic function and to investigate the effect of pulmonary function and pulmonary artery pressure on diastolic functions in people with chronic obstructive pulmonary disease (COPD). Patients and Methods: Sixty patients with COPD and 40 healthy individuals were assessed by echocardiography and pulmonary function tests (PFTs). Forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) were determined. Co...

  12. Blood flow redistribution and ventilation-perfusion mismatch during embolic pulmonary arterial occlusion

    OpenAIRE

    Burrowes, K. S.; Clark, A. R.; Tawhai, M.H

    2011-01-01

    Acute pulmonary embolism causes redistribution of blood in the lung, which impairs ventilation/perfusion matching and gas exchange and can elevate pulmonary arterial pressure (PAP) by increasing pulmonary vascular resistance (PVR). An anatomically-based multi-scale model of the human pulmonary circulation was used to simulate pre- and post-occlusion flow, to study blood flow redistribution in the presence of an embolus, and to evaluate whether reduction in perfused vascular bed is sufficient ...

  13. 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

  14. Isorhynchophylline protects against pulmonary arterial hypertension and suppresses PASMCs proliferation

    International Nuclear Information System (INIS)

    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. Anomalous origin of the left coronary artery from the pulmonary artery presenting as dilated cardiomyopathy: a case report

    OpenAIRE

    Gribaa, Rym; Slim, Mehdi; Ben Salem, Helmi; Neffati, Elyes; Boughzela, Essia

    2014-01-01

    Introduction Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly and one of the causes of myocardial ischemia. The usual clinical course is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. Case presentation We report the case of a 6-month-old Tunisian girl who presented with dilated cardiomyopathy. Echocardiography suspected anomalous origin of the left coronary artery. The definitive dia...

  16. 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.

  17. 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.

  18. 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.

  19. 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.

  20. Lung effect on the hemodynamics in pulmonary artery

    Science.gov (United States)

    Tsai, S. F.; Sheu, Tony W. H.; Chang, T. M.

    2001-06-01

    The present study investigates blood flow in a pulmonary artery. The aim is to gain a better understanding of offset value in vascular circulation through a two-dimensional analysis of the Navier-Stokes equations. In this study, the hemodynamics in a blood vessel with truncated outlets at which constant pressure is specified is examined. To simplify the analysis, the vessel walls are regarded as being rigid. In quadratic elements, the streamline upwind Petrov-Galerkin finite element model is employed to simulate the incompressible Newtonian blood flow. The adopted finite element model introduces artificial damping terms solely in the streamline direction. With these terms added to the formulation, the discrete system is enhanced while solution accuracy is maintained without deterioration due to numerical diffusion errors. Copyright

  1. 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.

  2. Macitentan (Opsumit) for the treatment of pulmonary arterial hypertension.

    Science.gov (United States)

    Clarke, Megan; Walter, Claire; Agarwal, Richa; Kanwar, Manreet; Benza, Raymond L

    2014-07-01

    The endothelin pathway is a key pathway for the pathogenesis of pulmonary arterial hypertension (PAH). Antagonism of this pathway is recommended as initial therapy in low-risk patient with PAH to inhibit fibrosis, cell proliferation, and inflammation caused by endothelin. Prior to October 2013, ambrisentan, a selective ETA receptor antagonist and bosentan, a dual ETA/ETB antagonist, were the only currently available agents for PAH targeting the endothelin pathway. Based on the results of the SERAPHIN trial, macitentan (brand name Opsumit®), a new ETA/ETB antagonist, has been US FDA approved to delay disease progression and reduce hospitalizations for PAH. SERAPHIN is the first ERA trial to use an event-driven strategy with a composite primary end point of morbidity or mortality. Previous trials have focused on short-term outcomes, such as improved 6-min walk distance and WHO functional class. PMID:24851934

  3. 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.

  4. 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.

  5. 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

  6. 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

  7. Effects of cigarette smoke on endothelial function of pulmonary arteries in the guinea pig

    Directory of Open Access Journals (Sweden)

    Martínez Anna

    2009-08-01

    Full Text Available Abstract Background Cigarette smoking may contribute to pulmonary hypertension in chronic obstructive pulmonary disease by altering the structure and function of pulmonary vessels at early disease stages. The objectives of this study were to evaluate the effects of long-term exposure to cigarette smoke on endothelial function and smooth muscle-cell proliferation in pulmonary arteries of guinea pigs. Methods 19 male Hartley guinea pigs were exposed to the smoke of 7 cigarettes/day, 5 days/week, for 3 and 6 months. 17 control guinea pigs were sham-exposed for the same periods. Endothelial function was evaluated in rings of pulmonary artery and aorta as the relaxation induced by ADP. The proliferation of smooth muscle cells and their phenotype in small pulmonary vessels were evaluated by immunohistochemical expression of α-actin and desmin. Vessel wall thickness, arteriolar muscularization and emphysema were assessed morphometrically. The expression of endothelial nitric oxide synthase (eNOS was evaluated by Real Time-PCR. Results Exposure to cigarette smoke reduced endothelium-dependent vasodilatation in pulmonary arteries (ANOVA p Conclusion In the guinea pig, exposure to cigarette smoke induces selective endothelial dysfunction in pulmonary arteries, smooth muscle cell proliferation in small pulmonary vessels and reduced lung expression of eNOS. These changes appear after 3 months of exposure and precede the development of pulmonary emphysema.

  8. An international physician survey of pulmonary arterial hypertension management.

    Science.gov (United States)

    Preston, Ioana R; Hinzmann, Barbara; Heinz, Sabina; Gall, Henning; Jenkins, David; Kim, Nick H; Lang, Irene

    2016-09-01

    We conducted an international study to evaluate practices in the diagnosis and management of pulmonary arterial hypertension (PAH) globally across different geographic regions. Between July and October 2012, PAH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 3 or 5 most recent patients with PAH. Overall, 560 physicians (Europe: 278; United States: 160; Argentina: 53; Japan: 69) completed the questionnaire and provided data for 2,618 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension center ranged from 13% in Argentina to 74% in the United States. At the time of diagnosis, patients' New York Heart Association functional class differed significantly between regions. At the time of last assessment, functional class had improved overall, and differences between regions had largely disappeared. A large proportion of patients did not undergo right heart catheterization for the diagnosis of PAH (Europe: 7%-21%; United States: 21%; Japan: 19%; Argentina: 51%). Variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of triple or greater combination therapy in Japan than in other regions. Results from this study, which includes a global aspect of PAH care, demonstrate that there are significant differences in PAH management between regions and low adherence to guidelines recommending right heart catheterization for the diagnosis of PAH. PMID:27683611

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

    International Nuclear Information System (INIS)

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

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

    International Nuclear Information System (INIS)

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

  11. 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.

  12. KCNQ Modulators Reveal a Key Role for KCNQ Potassium Channels in Regulating the Tone of Rat Pulmonary Artery Smooth Muscle

    OpenAIRE

    Joshi, Shreena; Sedivy, Vojtech; Hodyc, Daniel; Herget, Jan; Gurney, Alison M

    2009-01-01

    Potassium channels are central to the regulation of pulmonary vascular tone. The smooth muscle cells of pulmonary artery display a background K+ conductance with biophysical properties resembling those of KCNQ (KV7) potassium channels. Therefore, we investigated the expression and functional role of KCNQ channels in pulmonary artery. The effects of selective KCNQ channel modulators were investigated on K+ current and membrane potential in isolated pulmonary artery smoo...

  13. Non-arterial assessment of blood gas status in patients with chronic pulmonary disease.

    OpenAIRE

    Elborn, J. S.; Finch, M B; Stanford, C. F.

    1991-01-01

    Assessment of blood gas status is important in the management of patients with chronic pulmonary disease. Arterial puncture is often painful and may damage the arterial wall. Measurement of oxygen saturation by transcutaneous oximetry offers a non-invasive alternative to arterial methods but does not allow assessment of partial pressure of carbon dioxide. We have examined the value of oximetry and dorsal hand venous carbon dioxide as an alternative to arterial puncture. Transcutaneous oxygen ...

  14. Endothelium-dependent relaxations in sheep pulmonary arteries and veins: resistance to block by NG-nitro-L-arginine in pulmonary hypertension.

    OpenAIRE

    Kemp, B K; Smolich, J J; Ritchie, B C; Cocks, T. M.

    1995-01-01

    1. The effect of the nitric oxide synthase inhibitor, NG-nitro-L-arginine (L-NOARG), on endothelium-dependent relaxation to a receptor-independent agent, ionomycin, was examined in isolated pulmonary arteries and veins from control, short-term and chronic pulmonary hypertensive sheep. All vessel segments were contracted to optimal levels of active force with endothelin-1 to record endothelium-dependent relaxation. 2. Pulmonary hypertension was induced by continuous pulmonary artery air emboli...

  15. 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.

  16. 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.

  17. Dual left anterior descending artery with anomalous origin of long LAD from pulmonary artery - rare coronary anomaly detected on computed tomography coronary angiography

    Science.gov (United States)

    Vohra, Aditi; Narula, Harneet

    2016-01-01

    Dual left anterior descending artery is a rare coronary artery anomaly showing two left anterior descending arteries. Short anterior descending artery usually arises from the left coronary artery, while long anterior descending artery has anomalous origin and course. Dual left anterior descending artery with origin of long anterior descending artery from the pulmonary artery (ALCAPA) is a very rare coronary artery anomaly which has not been reported previously in the literature. We present the computed tomography coronary angiographic findings of this rare case in a young female patient who presented with atypical chest pain.

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

    Directory of Open Access Journals (Sweden)

    Antonio Lopes

    2014-01-01

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

  19. 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.

  20. Transcatheter closure of large right pulmonary artery-to-left atrial fistula

    Directory of Open Access Journals (Sweden)

    Karunakar Vadlamudi

    2013-01-01

    Full Text Available We report the successful transcatheter closure of right pulmonary artery fistula to left atrium in a six-year-old boy, who had presented with cyanosis and shortness of breath. The two-dimensional echocardiogram with bubble contrast study demonstrated the communication between right pulmonary artery and left atrium. Computerized tomography confirmed the diagnosis and delineated the anatomy. The fistula was closed successfully by a transcatheter trans-septal approach using an 18/20 duct occluder.

  1. Epigenetic mechanisms in pulmonary arterial hypertension: the need for global perspectives

    OpenAIRE

    Prakash Chelladurai; Werner Seeger; Soni Savai Pullamsetti

    2016-01-01

    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. Th...

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

    OpenAIRE

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

    2007-01-01

    Background Controlled exposures of animals and humans to particulate matter (PM) or ozone air pollution cause an increase in plasma levels of endothelin-1, a potent vasoconstrictor that regulates pulmonary arterial pressure. Objectives The primary objective of this field study was to determine whether Mexico City children, who are chronically exposed to levels of PM and O3 that exceed the United States air quality standards, have elevated plasma endothelin-1 levels and pulmonary arterial pres...

  3. Factors Affecting the Response to Exercise in Patients with Severe Pulmonary Arterial Hypertension

    OpenAIRE

    Flox-Camacho, Ángela; Escribano Subías, Pilar; Jiménez-Lépez Guarch, Carmen; Fernández Vaquero, Almudena; Martín Ríos, María Dolores; Saenz de la Calzada-Campo, Carlos

    2011-01-01

    Introduction: Ergospirometry objectively quantifies exercise capacity. Up until now, the response to exercise evaluated by ergospirometry in patients with pulmonary arterial hypertension has only been described in recently diagnosed.patients. Our aim is to describe the response to exercise in patients with severe pulmonary arterial hypertension under specific treatment and define which parameters determine their exercise capacity. Patients and method: A cross-sectional study was performed on ...

  4. 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....

  5. Pulse pressure variation and volume responsiveness during acutely increased pulmonary artery pressure: an experimental study

    OpenAIRE

    Daudel, Fritz; Tüller, David; Krähenbühl, Stefanie; Jakob, Stephan M; Takala, Jukka

    2010-01-01

    Introduction We found that pulse pressure variation (PPV) did not predict volume responsiveness in patients with increased pulmonary artery pressure. This study tests the hypothesis that PPV does not predict fluid responsiveness during an endotoxin-induced acute increase in pulmonary artery pressure and right ventricular loading. Methods Pigs were subjected to endotoxemia (0.4 μg/kg/hour lipopolysaccharide), followed by volume expansion, subsequent hemorrhage (20% of estimated blood volume), ...

  6. 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....

  7. 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.

  8. 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.

  9. Quantification of pulmonary arterial wall distensibility using parameters extracted from volumetric micro-CT images

    Science.gov (United States)

    Johnson, Roger H.; Karau, Kelly L.; Molthen, Robert C.; Dawson, Christopher A.

    1999-09-01

    Stiffening, or loss of distensibility, of arterial vessel walls is among the manifestations of a number of vascular diseases including pulmonary arterial hypertension. We are attempting to quantify the mechanical properties of vessel walls of the pulmonary arterial tree using parameters derived from high-resolution volumetric x-ray CT images of rat lungs. The pulmonary arterial trees of the excised lungs are filled with a contrast agent. The lungs are imaged with arterial pressures spanning the physiological range. Vessel segment diameters are measured from the inlet to the periphery, and distensibilities calculated from diameters as a function of pressure. The method shows promise as an adjunct to other morphometric techniques such as histology and corrosion casting. It possesses the advantages of being nondestructive, characterizing the vascular structures while the lungs are imaged rapidly and in a near-physiological state, and providing the ability to associate mechanical properties with vessel location in the intact tree hierarchy.

  10. 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)

  11. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with connective tissue diseases

    OpenAIRE

    Adel Boueiz; Hassoun, Paul M.

    2014-01-01

    The explosive growth of medical literature on pulmonary hypertension (PH) has led to a steady increase in awareness of this disease within the medical community during the past decade. The recent revision of the classification of PH is presented in in the main guidelines. Group 1 PH or pulmonary arterial hypertension (PAH) is a heterogeneous group and includes PH due to inheritable, drug-induced, and toxin-induced causes and to such underlying systemic causes as connective tissue diseases,...

  12. Molecular mechanisms of hypoxia-inducible factor-induced pulmonary arterial smooth muscle cell alterations in pulmonary hypertension.

    Science.gov (United States)

    Veith, Christine; Schermuly, Ralph T; Brandes, Ralf P; Weissmann, Norbert

    2016-03-01

    Oxygen (O2) is essential for the viability and function of most metazoan organisms and thus is closely monitored at both the organismal and the cellular levels. However, alveoli often encounter decreased O2 levels (hypoxia), leading to activation of physiological or pathophysiological responses in the pulmonary arteries. Such changes are achieved by activation of transcription factors. The hypoxia-inducible factors (HIFs) are the most prominent hypoxia-regulated transcription factors in this regard. HIFs bind to hypoxia-response elements (HREs) in the promoter region of target genes, whose expression and translation allows the organism, amongst other factors, to cope with decreased environmental O2 partial pressure (pO2). However, prolonged HIF activation can contribute to major structural alterations, especially in the lung, resulting in the development of pulmonary hypertension (PH). PH is characterized by a rise in pulmonary arterial pressure associated with pulmonary arterial remodelling, concomitant with a reduced intravascular lumen area. Patients with PH develop right heart hypertrophy and eventually die from right heart failure. Thus, understanding the molecular mechanisms of HIF regulation in PH is critical for the identification of novel therapeutic strategies. This review addresses the relationship of hypoxia and the HIF system with pulmonary arterial dysfunction in PH. We particularly focus on the cellular and molecular mechanisms underlying the HIF-driven pathophysiological processes. PMID:26228924

  13. 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

  14. Pulmonary Artery Pseudoaneurysm: A Rare Cause of Hemoptysis in a Child.

    Science.gov (United States)

    Vaideeswar, Pradeep; Karande, Sunil; Yadav, Subhash; Pardeshi, Kirti

    2016-01-01

    Aneurysms and pseudoaneurysms of pulmonary vasculature are uncommon occurrences that contribute to mortality and morbidity, without timely diagnosis and intervention. We report a fatal massive hemoptysis in a child due to a consolidation-related pulmonary arterial pseudoaneurysm, an extremely rare phenomenon. PMID:26366670

  15. Inspiratory and expiratory pulmonary CT: change of bronchial and accompanying pulmonary arterial diameter during respiration in healthy adult men

    International Nuclear Information System (INIS)

    To evaluate changes in the diameter of bronchi and pulmonary arteries during respiration. The ratio of the diameter of matched inner bronchi and accompanying pulmonary arteries was calculated from full inspiration and expiration HRCT of healthy men. In 106 pairs of matched bronchi and pulmonary arteries, the bronhchial-arterial ratios were0.61 ±0.18(upper lung), 0.72±0.21(lower lung), and 0.65±0.20(total) at full inspiration and 0.51±0.32(upper lung), 0.52±0.15(lower lung), and 0.51±0.26(total) at full expiration HRCT, denotiong a statistically significant increase in bronchial diameter of the entire lung at full inspiration. The inner diameter of the bronchus was larger than the diameter of the accompanying pulmonary artery by 0.1% at full expiration and 8.5% at full inspiration. The result of this study would be helpful in understanding the physiology of the lung during respiration

  16. Pulmonary intimal sarcoma: a rare differential diagnosis for arterial filling defects on a chest CT

    International Nuclear Information System (INIS)

    We present a rare case of pulmonary intimal sarcoma mimicking pulmonary embolism in a 40-year-old woman. Although extremely rare, these tumors must be considered in patients who present inappropriate imaging findings that suggest embolism. Chest computed tomography is the modality of choice to determine the extent of the tumor. We present a female patient with suspected embolism that was in fact found to be an endothelial sarcoma of the pulmonary arteries

  17. Pulmonary artery pressure responses to increased cardiac output in chickens with raised metabolic rate

    OpenAIRE

    West, John B.; Fu, Zhenxing; Gu, Yusu; Wagner, Harrieth E.; Carr, J. Austin; Peterson, Kirk L.

    2010-01-01

    Previous work has shown remarkable differences in the pressure-flow relations of the pulmonary circulation between birds and mammals. For example several studies suggest that the avian pulmonary blood vessels behave like rigid tubes, very different from the situation in mammalian lung. We therefore speculated that birds would develop high pulmonary artery pressures when the cardiac output was substantially increased during heavy exercise, for example during flight. However because of the tech...

  18. In vivo and in vitro measurements of pulmonary arterial stiffness: A brief review

    OpenAIRE

    Tian, Lian; Chesler, Naomi C.

    2012-01-01

    During the progression of pulmonary hypertension (PH), proximal pulmonary arteries (PAs) undergo remodeling such that they become thicker and the elastic modulus increases. Both of these changes increase the vascular stiffness. The increase in pulmonary vascular stiffness contributes to increased right ventricular (RV) afterload, which causes RV hypertrophy and eventually failure. Studies have found that proximal PA stiffness or its inverse, compliance, is strongly related to morbidity and mo...

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

    International Nuclear Information System (INIS)

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

  20. Hemodynamic and Histologic Characterization of a Swine (Sus scrofa domestica) Model of Chronic Pulmonary Arterial Hypertension

    OpenAIRE

    Rothman, Abraham; Wiencek, Robert G; Davidson, Stephanie; William N. Evans; Restrepo, Humberto; Sarukhanov, Valeri; Rivera-Begeman, Amanda; Mann, David

    2011-01-01

    The purpose of this work was to develop and characterize an aortopulmonary shunt model of chronic pulmonary hypertension in swine and provide sequential hemodynamic, angiographic, and histologic data by using an experimental endoarterial biopsy catheter. Nine Yucatan female microswine (Sus scrofa domestica) underwent surgical anastomosis of the left pulmonary artery to the descending aorta. Sequential hemodynamic, angiographic, and pulmonary vascular samples were obtained. Six pigs (mean weig...

  1. 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.

  2. Gene Therapy by Targeted Adenovirus-mediated Knockdown of Pulmonary Endothelial Tph1 Attenuates Hypoxia-induced Pulmonary Hypertension

    OpenAIRE

    Morecroft, Ian; White, Katie; Caruso, Paola; Nilsen, Margaret; Loughlin, Lynn; Alba, Raul; Reynolds, Paul N; Danilov, Sergei M.; Andrew H. Baker; MacLean, Margaret R.

    2012-01-01

    Serotonin is produced by pulmonary arterial endothelial cells (PAEC) via tryptophan hydroxylase-1 (Tph1). Pathologically, serotonin acts on underlying pulmonary arterial cells, contributing to vascular remodeling associated with pulmonary arterial hypertension (PAH). The effects of hypoxia on PAEC-Tph1 activity are unknown. We investigated the potential of a gene therapy approach to PAH using selective inhibition of PAEC-Tph1 in vivo in a hypoxic model of PAH. We exposed cultured bovine pulmo...

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    Farha Samar

    2013-01-01

    Full Text Available Abstract 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 CO of PAH patients did not change over time, 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.

  5. 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

  6. Updated Evidence-Based Treatment Algorithm in Pulmonary Arterial Hypertension

    Science.gov (United States)

    Barst, Robyn J.; Gibbs, J. Simon; Ghofrani, Hossein A.; Hoeper, Marius M.; McLaughlin, Vallerie V.; Rubin, Lewis J.; Sitbon, Olivier; Tapson, Victor; Galiè, Nazzareno

    2009-01-01

    Uncontrolled and controlled clinical trials with different compounds and procedures are reviewed to define the risk-benefit profiles for therapeutic options in pulmonary arterial hypertension (PAH). A grading system for the level of evidence of treatments based on the controlled clinical trials performed with each compound is used to propose an evidence-based treatment algorithm. The algorithm includes drugs approved by regulatory agencies for the treatment of PAH and/or drugs available for other indications. The different treatments have been evaluated mainly in idiopathic PAH, heritable PAH, and in PAH associated with the scleroderma spectrum of diseases or with anorexigen use. Extrapolation of these recommendations to other PAH subgroups should be done with caution. Oral anticoagulation is proposed for most patients; diuretic treatment and supplemental oxygen are indicated in cases of fluid retention and hypoxemia, respectively. High doses of calcium channel blockers are indicated only in the minority of patients who respond to acute vasoreactivity testing. Nonresponders to acute vasoreactivity testing, or responders who remain in World Health Organization (WHO) functional class III, should be considered candidates for treatment with either an oral phosphodiesterase-5 inhibitor or an oral endothelin-receptor antagonist. Continuous intravenous administration of epoprostenol remains the treatment of choice in WHO functional class IV patients. Combination therapy is recommended for patients treated with PAH monotherapy who remain in New York Heart Association functional class III. Atrial septostomy and lung transplantation are indicated for refractory patients or where medical treatment is unavailable. PMID:19555861

  7. 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.

  8. 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.

  9. 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.)

  10. 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.

  11. 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.

  12. MRI-based flow measurements in the main pulmonary artery to detect pulmonary arterial hypertension in patients with cystic fibrosis

    International Nuclear Information System (INIS)

    Development of pulmonary arterial hypertension (PH) is a common problem in the course of patients suffering from cystic fibrosis (CF). This study was performed to evaluate MRI based flow measurements (MRvenc; Velocity ENCoding) to detect signs of an evolving PH in patients suffering from CF. 48 patients (median age: 16 years, range: 10 - 40 years, 25 female) suffering from CF of different severity (mean FEV1: 74 % ± 23, mean Shwachman-score: 63 ± 10) were examined using MRI based flow measurements of the main pulmonary artery (MPA). Phase-contrast flash sequences (TR: 9.6 ms, TE: 2.5 ms, bandwidth: 1395 Hertz/Pixel) were utilized. Results were compared to an age- and sex-matched group of 48 healthy subjects. Analyzed flow data where: heart frequency (HF), cardiac output (HZV), acceleration time (AT), proportional acceleration time related to heart rate (ATr), mean systolic blood velocity (MFG), peak velocity (Peak), maximum flow (Flussmax), mean flow (Flussmitt) and distensibility (Dist). The comparison of means revealed significant differences only for MFG, Flussmax and Dist, but overlap was marked. However, using a scatter-plot of AT versus MFG, it was possible to identify five CF-patients demonstrating definite signs of PH: AT = 81 ms ± 14, MFG = 46 ± 11 cm/s, Dist = 41 % ± 7. These CF-patients where the most severely affected in the investigated group, two of them were listed for complete heart and lung transplantation. The comparison of this subgroup and the remaining CF-patients revealed a highly significant difference for the AT (p = 0.000001) without overlap. Screening of CF-patients for the development of PH using MRvenc of the MPA is not possible. In later stages of disease, the quantification of AT, MFG and Dist in the MPA may be useful for the detection, follow-up and control of therapy of PH. MRvenc of the MPA completes the MRI-based follow-up of lung parenchyma damage in patients suffering from CF. (orig.)

  13. 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

  14. 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.

  15. 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

  16. Changes in pulmonary arterial wall mechanical properties and lumenal architecture with induced vascular remodeling

    Science.gov (United States)

    Molthen, Robert C.; Heinrich, Amy E.; Haworth, Steven T.; Dawson, Christopher A.

    2004-04-01

    To explore and quantify pulmonary arterial remodeling we used various methods including micro-CT, high-resolution 3-dimensional x-ray imaging, to examine the structure and function of intact pulmonary vessels in isolated rat lungs. The rat is commonly used as an animal model for studies of pulmonary hypertension (PH) and the accompanying vascular remodeling, where vascular remodeling has been defined primarily by changes in the vessel wall composition in response to hypertension inducing stimuli such as chronic hypoxic exposure (CHE) or monocrotaline (MCT) injection. Little information has been provided as to how such changes affect the vessel wall mechanical properties or the lumenal architecture of the pulmonary arterial system that actually account for the hemodynamic consequences of the remodeling. In addition, although the link between primary forms of pulmonary hypertension and inherited genetics is well established, the role that genetic coding plays in hemodynamics and vascular remodeling is not. Therefore, we are utilizing Fawn-Hooded (FH), Sprague-Dawley (SD) and Brown Norway (BN)rat strains along with unique imaging methods to parameterize both vessel distensibility and lumenal morphometry using a principal pulmonary arterial pathway analysis based on self-consistency. We have found for the hypoxia model, in addition to decreased body weight, increased hematocrit, increased right ventricular hypertrophy, the distensibility of the pulmonary arteries is shown to decrease significantly in the presence of remodeling.

  17. Influence of thyroid status on responses of rat isolated pulmonary artery, vas deferens and trachea to smooth muscle relaxant drugs.

    OpenAIRE

    O'Donnell, S R; Wanstall, J. C.; Mustafa, M. B.

    1987-01-01

    1 Responses to relaxant drugs have been examined on isolated KCl-contracted smooth muscle preparations from rats in which thyroid status was changed by prior treatment with either thyroxine (T4) for 1 week (preparations of pulmonary artery, trachea and vas deferens) or methimazole for 10-12 weeks (pulmonary artery preparations). 2 On pulmonary artery preparations, T4 treatment caused a significant increase in the magnitude of the relaxant responses to noradrenaline and isoprenaline but not th...

  18. Differential effects of formoterol on thrombin- and PDGF-induced proliferation of human pulmonary arterial vascular smooth muscle cells

    OpenAIRE

    Goncharova Elena A; Khavin Irene S; Goncharov Dmitry A; Krymskaya Vera P

    2012-01-01

    Abstract Background Increased pulmonary arterial vascular smooth muscle (PAVSM) cell proliferation is a key pathophysiological component of pulmonary vascular remodeling in pulmonary arterial hypertension (PH). The long-acting β2-adrenergic receptor (β2AR) agonist formoterol, a racemate comprised of (R,R)- and (S,S)-enantiomers, is commonly used as a vasodilator in chronic obstructive pulmonary disease (COPD). PH, a common complication of COPD, increases patients’ morbidity and reduces surviv...

  19. Differential effects of formoterol on thrombin- and PDGF-induced proliferation of human pulmonary arterial vascular smooth muscle cells

    OpenAIRE

    Goncharova, Elena A.; Khavin, Irene S; Goncharov, Dmitry A; Vera P Krymskaya

    2012-01-01

    Background Increased pulmonary arterial vascular smooth muscle (PAVSM) cell proliferation is a key pathophysiological component of pulmonary vascular remodeling in pulmonary arterial hypertension (PH). The long-acting β2-adrenergic receptor (β2AR) agonist formoterol, a racemate comprised of (R,R)- and (S,S)-enantiomers, is commonly used as a vasodilator in chronic obstructive pulmonary disease (COPD). PH, a common complication of COPD, increases patients’ morbidity and reduces survival. Recen...

  20. 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.

  1. Scintigraphic and echocardiographic evaluation of pulmonary flow after percutaneous closure of patent arterial duct

    International Nuclear Information System (INIS)

    Introduction: During the evolution of patients submitted to percutaneous closure of a patent arterial duct (PAD), some degree of protrusion of the occlusive device into the left pulmonary arterial branch has been described. Aim: We tested the hypothesis that patients (pts) with device protrusion have also decreased pulmonary blood perfusion. Material and Methods: We studied 70 pts submitted to percutaneous closure of the PAD with Gianturco coils (mean age: 8.6, median: 7.5 years), with a mean time follow-up of 55 months. Comprehensive Doppler echocardiographic studies were performed. We calculated the percentage of difference of blood flow velocities between the left and right pulmonary arterial branches. Pulmonary scintigraphy was performed using an intravenous injection of 99mTc labeled human albumin macroaggregates to evaluate bilateral lung perfusion ( relative quantification ). The same determinations were performed in 22 controls. Results: 66 pts (94,3%) showed some degree of protrusion of the device into the left pulmonary artery. Patients were grouped according to the difference in the percentage of flow velocity between arterial branches as detected by Doppler: ≥ 30% (16 pts, group A); < 30% (54 pts, group B) and controls (22 pts, group C). The mean value of left lung perfusion was significantly lower in group A (41.9% ± 3,2%), compared to group B (46.8% ± 1,93 %) and to group C (46,8% ± 2,4%) (Kruskal-Wallis, p<0.001). There was a significant negative linear correlation between the values of left lung perfusion and the percentage difference of blood flow velocities. Conclusion: Protrusion of the occlusive device in pts with PAD is not always associated to pulmonary artery obstruction. However, pts showing blood flow acceleration ≥30% at the left pulmonary arterial branch by Doppler have significantly lower values of lung perfusion

  2. Pulmonary inflammatory response: cellular events in experimental pulmonary arterial hypersensitivity disease

    International Nuclear Information System (INIS)

    Horseradish peroxidase (HRP) or bovine serum albumin (BSA) was covalently linked to polyacrylamide or agarose beads and was injected into control Syrian hamsters and hamsters previously immunized with either HRP or BSA. Animals sensitized to soluble antigen and subsequently challenged intravenously with the same antigen immobilized on beads developed an acute focal inflammatory response within 2 to 6 hours after injection. The acute response involved local deposition of IgG and complement (β1A/β1C globulin), polymorphonuclear leukocyte exudation, and variable amounts of hemorrhage. A focal vasculitis was occasionally present. Within 72 hours, the reaction had become largely mononuclear or granulomatous in nature, and giant cell formation was seen within 4 days after immobilized antigen injection. Severe reactions developed only upon recognition of specific antigenic determinants; thus hamsters immunized against soluble HRP developed characteristic lesions only upon intravenous challenge with HRP-coated beads but not with beads coated with unrelated antigen (BSA). The beads elicited only a mild foreign body reaction in the control hamsters at 5 to 7 days after injection, a reaction that was temporally and histopathologically distinct from the lesions in immunized hamsters. Thus, the state of existing immunity can influence the character and severity of the local pulmonary inflammatory response. (U.S.)

  3. Giant pulmonary artery aneurysm in a patient with rheumatic mitral stenosis

    Directory of Open Access Journals (Sweden)

    Vikas Singh

    2014-01-01

    Full Text Available Pulmonary artery (PA aneurysm is a rare condition, frequently associated with pulmonary hypertension. However, the evolution and treatment of this pathology is still not clear. We report a case of a 45-year-old female patient with giant PA aneurysm associated with rheumatic mitral stenosis and severe pulmonary arterial hypertension. The patient had undergone balloon mitral valvotomy around 7 years back; aneurysm was first identified 3 years back during routine follow-up. The PA aneurysm size, however, had remained almost unchanged with associated severe pulmonary regurgitation. Surgical correction was advised but denied by the patient. To our knowledge, this is the first case report of such a large PA aneurysm in association with rheumatic heart disease. Although medical therapy for pulmonary hypertension was started, surgical correction of the aneurysm was advised in order to prevent the future complications.

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

    International Nuclear Information System (INIS)

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

  5. 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.

  6. Pulmonary Artery Access Embolization in Patients with Massive Hemoptysis in Whom Bronchial and/or Nonbronchial Systemic Artery Embolization Is Contraindicated

    International Nuclear Information System (INIS)

    The objective of this paper is to present an alternative therapeutic approach for the treatment of patients with massive hemoptysis in whom bronchial and/or nonbronchial systemic arterial embolization is not possible. We describe a percutaneous procedure for pulmonary segmental artery embolization. Between May 2000 and July 2006, 27 adult patients with hemoptysis underwent percutaneous treatment at our department; 20 of 27 patients were embolized via bronchial and or nonbronchial systemic arteries and 7 patients were embolized via pulmonary artery. Femoral arterial access for systemic artery catheterization and femoral vein access for pulmonary arterial catheterization were used. Gelfoam particles and coils were used for embolization. In this study, we report on three cases of massive hemoptysis from a systemic arterial source in whom bronchial and/or nonbronchial arteries embolization was not possible. Percutaneous embolization via the pulmonary artery access was successful in all three patients. In conclusion, embolization via pulmonary artery is presented as an alternative approach for the management of hemoptysis in patients in whom bronchial arterial embolization is not possible

  7. Right Ventricular Myocardial Stiffness in Experimental Pulmonary Arterial Hypertension

    Science.gov (United States)

    Rain, Silvia; Andersen, Stine; Najafi, Aref; Gammelgaard Schultz, Jacob; da Silva Gonçalves Bós, Denielli; Handoko, M. Louis; Bogaard, Harm-Jan; Vonk-Noordegraaf, Anton; Andersen, Asger; van der Velden, Jolanda; Ottenheijm, Coen A.C.

    2016-01-01

    Background— The purpose of this study was to determine the relative contribution of fibrosis-mediated and myofibril-mediated stiffness in rats with mild and severe right ventricular (RV) dysfunction. Methods and Results— By performing pulmonary artery banding of different diameters for 7 weeks, mild RV dysfunction (Ø=0.6 mm) and severe RV dysfunction (Ø=0.5 mm) were induced in rats. The relative contribution of fibrosis- and myofibril-mediated RV stiffness was determined in RV trabecular strips. Total myocardial stiffness was increased in trabeculae from both mild and severe RV dysfunction in comparison to controls. In severe RV dysfunction, increased RV myocardial stiffness was explained by both increased fibrosis-mediated stiffness and increased myofibril-mediated stiffness, whereas in mild RV dysfunction, only myofibril-mediated stiffness was increased in comparison to control. Histological analyses revealed that RV fibrosis gradually increased with severity of RV dysfunction, whereas the ratio of collagen I/III expression was only elevated in severe RV dysfunction. Stiffness measurements in single membrane-permeabilized RV cardiomyocytes demonstrated a gradual increase in RV myofibril stiffness, which was partially restored by protein kinase A in both mild and severe RV dysfunction. Increased expression of compliant titin isoforms was observed only in mild RV dysfunction, whereas titin phosphorylation was reduced in both mild and severe RV dysfunction. Conclusions— RV myocardial stiffness is increased in rats with mild and severe RV dysfunction. In mild RV dysfunction, stiffness is mainly determined by increased myofibril stiffness. In severe RV dysfunction, both myofibril- and fibrosis-mediated stiffness contribute to increased RV myocardial stiffness. PMID:27370069

  8. Risk factors for hemoptysis in idiopathic and hereditary pulmonary arterial hypertension.

    Directory of Open Access Journals (Sweden)

    Darryl Tio

    Full Text Available INTRODUCTION: When hemoptysis complicates pulmonary arterial hypertension (PAH, it is assumed to result from bronchial artery hypertrophy. In heritable PAH, the most common mutation is in the BMPR2 gene, which regulates growth, differentiation and apoptosis of mesenchymal cells. The aim of this study is to determine the relationship in PAH between the occurrence of hemoptysis, and disease progression, bronchial artery hypertrophy, pulmonary artery dilation and BMPR2 mutations. METHODS: 129 IPAH patients underwent baseline pulmonary imaging (CT angio or MRI and repeated right-sided heart catheterization. Gene mutations were assessed in a subset of patients. RESULTS: Hemoptysis was associated with a greater presence of hypertrophic bronchial arteries and more rapid hemodynamic deterioration. The presence of a BMPR2 mutation did not predispose to the development of hemoptysis, but was associated with a greater number of hypertrophic bronchial arteries and a worse baseline hemodynamic profile. CONCLUSION: Hemoptysis in PAH is associated with bronchial artery hypertrophy and faster disease progression. Although the presence of a BMPR2 mutation did not correlate with a greater incidence of hemoptysis in our patient cohort, its association with worse hemodynamics and a trend of greater bronchial arterial hypertrophy may increase the risk of hemoptysis.

  9. Risk Factors for Hemoptysis in Idiopathic and Hereditary Pulmonary Arterial Hypertension

    Science.gov (United States)

    Tio, Darryl; Leter, Edward; Boerrigter, Bart; Boonstra, Anco; Vonk-Noordegraaf, Anton; Bogaard, Harm Jan

    2013-01-01

    Introduction When hemoptysis complicates pulmonary arterial hypertension (PAH), it is assumed to result from bronchial artery hypertrophy. In heritable PAH, the most common mutation is in the BMPR2 gene, which regulates growth, differentiation and apoptosis of mesenchymal cells. The aim of this study is to determine the relationship in PAH between the occurrence of hemoptysis, and disease progression, bronchial artery hypertrophy, pulmonary artery dilation and BMPR2 mutations. Methods 129 IPAH patients underwent baseline pulmonary imaging (CT angio or MRI) and repeated right-sided heart catheterization. Gene mutations were assessed in a subset of patients. Results Hemoptysis was associated with a greater presence of hypertrophic bronchial arteries and more rapid hemodynamic deterioration. The presence of a BMPR2 mutation did not predispose to the development of hemoptysis, but was associated with a greater number of hypertrophic bronchial arteries and a worse baseline hemodynamic profile. Conclusion Hemoptysis in PAH is associated with bronchial artery hypertrophy and faster disease progression. Although the presence of a BMPR2 mutation did not correlate with a greater incidence of hemoptysis in our patient cohort, its association with worse hemodynamics and a trend of greater bronchial arterial hypertrophy may increase the risk of hemoptysis. PMID:24194909

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

    International Nuclear Information System (INIS)

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

  11. 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.

  12. 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.

  13. 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.

  14. Reconciling paradigms of abnormal pulmonary blood flow and quasi-malignant cellular alterations in pulmonary arterial hypertension.

    Science.gov (United States)

    Happé, C M; Szulcek, R; Voelkel, N F; Bogaard, H J

    2016-08-01

    In pulmonary arterial hypertension (PAH) structural and functional abnormalities of the small lung vessels interact and lead to a progressive increase in pulmonary vascular resistance and right heart failure. A current pathobiological concept characterizes PAH as a 'quasi-malignant' disease focusing on cancer-like alterations in endothelial cells (EC) and the importance of their acquired apoptosis-resistant, hyper-proliferative phenotype in the process of vascular remodeling. While changes in pulmonary blood flow (PBF) have been long-since recognized and linked to the development of PAH, little is known about a possible relationship between an altered PBF and the quasi-malignant cell phenotype in the pulmonary vascular wall. This review summarizes recognized and hypothetical effects of an abnormal PBF on the pulmonary vascular bed and links these to quasi-malignant changes found in the pulmonary endothelium. Here we describe that abnormal PBF does not only trigger a pulmonary vascular cell growth program, but may also maintain the cancer-like phenotype of the endothelium. Consequently, normalization of PBF and EC response to abnormal PBF may represent a treatment strategy in patients with established PAH. PMID:26804008

  15. 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

  16. Anomalous origin of the left circumflex coronary artery from the pulmonary artery. A very rare congenital anomaly in an adult patient diagnosed by cardiovascular magnetic resonance

    Directory of Open Access Journals (Sweden)

    Giannitsis Evangelos

    2008-01-01

    Full Text Available Abstract Here we report for the first time on the diagnostic potential of cardiovascular magnetic resonance (CMR to delineate the proximal course of an anomalous left circumflex coronary artery (LCX originating from the right pulmonary artery in an adult patient with no other form of congenital heart disease. The patient was referred to our institution due to exertional chest discomfort. X-Ray coronary angiography showed a normal left anterior descending coronary artery (LAD and right coronary artery (RCA, while the LCX was filled retrograde by collateral flow through the LAD and the RCA. The origin of the LCX was postulated to be the pulmonary artery, but the exact origin of the anomalous artery could not be depicted on conventional angiograms. CMR provided the unambiguous depiction of the origin of the anomalous LCX from the right pulmonary artery and the delineation of its proximal course in this case of a very rare coronary anomaly in adults.

  17. Pulmonary artery flow pattern in normal babies and those with persistence of fetal circulation

    International Nuclear Information System (INIS)

    This paper discusses how, in normal babies, spectral and color Doppler flow patterns in the main pulmonary artery (MPA) and its branches (the left and right pulmonary arteries [LPA and RPA]) are vastly different from those encountered in babies with persistence of fetal circulation (PFC). Flow velocity from the MPA to the LPA or RPA accelerates by as much as 240% in normal subjects, whereas in babies with PFC, velocity in the MPA, LPA, and RPA are identical. Because of fetal circulation, all babies are born with physiologic peripheral pulmonary artery stenosis. A right-to-left shunt across the patent dictus arteriosus in PFC minimizes the flow through the LPA and RPA, disallowing physiologic acceleration before treatment

  18. 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.

  19. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Schistosomiasis and pulmonary arterial hypertension.

    Science.gov (United States)

    Butrous, Ghazwan

    2014-07-01

    Schistosomiasis is caused by infection with the parasite Schistosoma, which is a flat-worm or fluke. The dominant species are Schistosoma mansoni, Schistosoma japonicum, and Schistosoma haematobium. Schistosomiasis is the third most common parasitic disease in the world after malaria and amoebiasis. It is endemic in more than 70 countries affecting about 200 million people worldwide, of whom 80% are in sub-Saharan Africa. There are pockets of infection in north-eastern Brazil, near the Yangtze River in China, and some pockets in south East Asia. In the East Mediterranean regions, the Schistosoma have been reported in Iraq and Egypt as well as in Sudan. The latter has the highest infection rate nowadays, particularly in the Al Jazeera area, due to the poor Schistosoma control program. In the Arabian peninsula, schistosomiasis has been reported in southwest part of Saudi Arabia, mainly in the Asir province and Jizan province, which lay in the southwest corner of Saudi Arabia and directly north of the border with Yemen. The efforts to control schistosomiasis have been very successful in Saudi Arabia due to the irrigation system control. However, the infection is prone in Yemen, where the schistosomiasis control is much less strict. Thus as a result, the problem still exists due to transmigration of the populations from both countries. As a cause of pulmonary arterial hypertension (PAH), schistosomiasis is still under diagnosed and undertreated. This article with give a highlight about the pathophysiology of the disease and both diagnostic and therapeutic strategies. PMID:25076995

  20. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Schistosomiasis and pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Ghazwan Butrous

    2014-01-01

    Full Text Available Schistosomiasis is caused by infection with the parasite Schistosoma, which is a flat-worm or fluke. The dominant species are Schistosoma mansoni, Schistosoma japonicum, and Schistosoma haematobium. Schistosomiasis is the third most common parasitic disease in the world after malaria and amoebiasis. It is endemic in more than 70 countries affecting about 200 million people worldwide, of whom 80% are in sub-Saharan Africa. There are pockets of infection in north-eastern Brazil, near the Yangtze River in China, and some pockets in south East Asia. In the East Mediterranean regions, the Schistosoma have been reported in Iraq and Egypt as well as in Sudan. The latter has the highest infection rate nowadays, particularly in the Al Jazeera area, due to the poor Schistosoma control program. In the Arabian peninsula, schistosomiasis has been reported in southwest part of Saudi Arabia, mainly in the Asir province and Jizan province, which lay in the southwest corner of Saudi Arabia and directly north of the border with Yemen. The efforts to control schistosomiasis have been very successful in Saudi Arabia due to the irrigation system control. However, the infection is prone in Yemen, where the schistosomiasis control is much less strict. Thus as a result, the problem still exists due to transmigration of the populations from both countries. As a cause of pulmonary arterial hypertension (PAH, schistosomiasis is still under diagnosed and undertreated. This article with give a highlight about the pathophysiology of the disease and both diagnostic and therapeutic strategies.

  1. Compression of the Right Pulmonary Artery by a Massive Descending Aortic Aneurysm Causing Bilateral Perfusion Defects on Pulmonary Scintigraphy

    OpenAIRE

    Makis, William; Derbekyan, Vilma

    2011-01-01

    A 67-year-old woman, who presented with a 2 month history of dyspnea, had a ventilation and perfusion lung scan that showed absent perfusion of the entire right lung with normal ventilation, as well as a rounded matched defect in the left lower lung adjacent to midline, suspicious for an aortic aneurysm or dissection. CT pulmonary angiography revealed a massive descending aortic aneurysm compressing the right pulmonary artery as well as the left lung parenchyma, accounting for the bilateral p...

  2. Endovascular treatment of a ruptured pulmonary artery aneurysm in a patient with Behcet's disease using the amplatzer vascular plug 4

    International Nuclear Information System (INIS)

    A pulmonary artery aneurysm is a common manifestation and the leading cause of mortality in Behcet's disease. We describe a case of spontaneous rupture of a pulmonary artery aneurysm that, due to the inadequacy of medical therapy and the disadvantages of surgery, became the ideal candidate for endovascular management and was successfully performed by using the Amplatzer Vascular Plug 4.

  3. Mediation of endogenous peroxynitrite in the injury of cultured pulmonary artery endothelial cells induced by lipopolysaccharide

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    This experiment, using cultured bovine pulmonary artery endothelial cells (BPAEC), was undertaken to investigate roles of endogenous ONOO- in lipopolysaccharide (LPS)-caused injury to endothelial cells. The fluorescent intensity of nitrotyrosine (NT), a marker of ONOO- generation, in BPAEC represented content of endogenous ONOO- generation. The fluorescent intensity of NT and number of NT positive cells were detected with flow cytometry, and the percentage of NT positive cells was calculated. Results were as follows. (1) LPS (1 mg/L, 5 mg/L and 10 mg/L) caused marked increase in fluorescent intensity of NT in a dose dependent manner. The number and percentage of NT positive cells were markedly increased (P<0.05). Aminoguanidine (AG), a selective inhibitor of inducible nitric oxide synthase, inhibited the increase in fluorescent intensity of NT in BPAEC induced by LPS. However, the number and percentage of NT positive cells had tendency to reduce. (2) LPS caused the enhancement of MDA content and activity of LDH in cultured supernatant (P<0.01). AG reversed the enhancement of MDA content induced by LPS (P<0.01). In contrast, AG had marginal effect on activity of LDH. (3) LPS induced the increase in apoptotic rate in BPAEC in a dose dependent manner. Some BPAEC stained with fluorescent probe ethidium bromide showed morphological features of apoptosis with chromatin condensation and nuclear fragmentation. AG reduced the apoptotic rate and number of apoptotic cells, both of which were still higher than those of vehicle group (P<0.05). (4) LPS inhibited mitochondrial respiration. Effect of LPS on mitochondrial membrane potential (ΔΨ) depended on the doses of LPS. 1 mg/L LPS led to a little increase in ΔΨ, while 5 mg/L and 10 mg/L LPS significantly reduced ΔΨ. In conclusion, LPS caused injury to cultured BPAEC and increased production of ONOO-. Cytotoxicity of LPS may be mediated by endogenous ONOO-.

  4. The Surgical Outcome of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

    Directory of Open Access Journals (Sweden)

    Tasneem Muzaffar

    2014-06-01

    Full Text Available Background:: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA is a rare congenital anomaly which represents one of the most common causes of myocardial ischemia and infarction in children. This anomaly, if left untreated, results in a very high mortality rate within the first year of life. Yet, immediate surgical correction can lead to excellent results.. Objectives:: The present study aimed to determine the surgical outcome of ALCAPA.. Methods:: This study was conducted on 53 patients with ALCAPA operated from January 2005 to December 2012. Surgical repair was carried out as soon as the diagnosis was made. Surgery was thus undertaken on an urgent basis (within 48 hours in the patients with congestive heart failure or critical clinical status and on a semi- elective basis (within a few days in the remaining children. Operations for all the patients were performed through a median sternotomy using established standard cardiopulmonary bypass technique. Grouped variables were compared using chi-square test with Yates’ correction. Besides, McNemar’s test was used to assess the relationship between preoperative ejection fraction and mitral incompetence. All the analyses were performed using the SPSS statistical software, version 11.5 (SPSS Inc., Chicago, IL.. Results:: The patients’ median age at presentation was 4 months. The mean preoperative ejection fraction was 36.5%. The results showed a significant relationship between age at presentation and impairment of ejection fraction (P < 0.001. At first, 23% of our patients presented with ejection fraction < 35%. However, 6 months after the operation, the ejection fraction improved to a mean of 53.07% (SD = 8.5 ranging from 38 - 66%. There were 5 postoperative hospital deaths with an overall mortality rate of 9.6%.. Conclusions:: Excellent results with desirable long-term outcomes can be achieved in the infants with ALCAPA using coronary artery implantation

  5. CT pulmonary angiography during veno-arterial extracorporeal membrane oxygenation in an adult

    International Nuclear Information System (INIS)

    Veno-arterial extracorporeal membrane oxygenation (VA ECMO) causes changes in the filling and blood flow of the cardiac chambers and pulmonary vessels as well as alterations in the path of intravenous contrast injected during CT. We present a patient with a potentially misleading CT pulmonary angiogram while on full VA ECMO. We demonstrate circulatory changes as well as alterations in contrast flow when ECMO flows are reduced.

  6. Pulmonary arterial hypertension in children: Diagnostic work-up and challenges

    OpenAIRE

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

    2009-01-01

    The diagnostic evaluation of a pediatric patient with suspected pulmonary arterial hypertension (PAH) is extensive but essential, given the rapid progression of the disease if left undiagnosed and untreated. The major goals of performing a complete diagnostic work-up are to confirm the diagnosis of PAH, assess disease severity, rule out associated diseases, and begin to formulate an individualized treatment plan for the pediatric patient with pulmonary hypertension. This article will provide ...

  7. Numerical simulation of blood flow and pressure drop in the pulmonary arterial and venous circulation.

    Science.gov (United States)

    Qureshi, M Umar; Vaughan, Gareth D A; Sainsbury, Christopher; Johnson, Martin; Peskin, Charles S; Olufsen, Mette S; Hill, N A

    2014-10-01

    A novel multiscale mathematical and computational model of the pulmonary circulation is presented and used to analyse both arterial and venous pressure and flow. This work is a major advance over previous studies by Olufsen et al. (Ann Biomed Eng 28:1281-1299, 2012) which only considered the arterial circulation. For the first three generations of vessels within the pulmonary circulation, geometry is specified from patient-specific measurements obtained using magnetic resonance imaging (MRI). Blood flow and pressure in the larger arteries and veins are predicted using a nonlinear, cross-sectional-area-averaged system of equations for a Newtonian fluid in an elastic tube. Inflow into the main pulmonary artery is obtained from MRI measurements, while pressure entering the left atrium from the main pulmonary vein is kept constant at the normal mean value of 2 mmHg. Each terminal vessel in the network of 'large' arteries is connected to its corresponding terminal vein via a network of vessels representing the vascular bed of smaller arteries and veins. We develop and implement an algorithm to calculate the admittance of each vascular bed, using bifurcating structured trees and recursion. The structured-tree models take into account the geometry and material properties of the 'smaller' arteries and veins of radii ≥ 50 μm. We study the effects on flow and pressure associated with three classes of pulmonary hypertension expressed via stiffening of larger and smaller vessels, and vascular rarefaction. The results of simulating these pathological conditions are in agreement with clinical observations, showing that the model has potential for assisting with diagnosis and treatment for circulatory diseases within the lung. PMID:24610385

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

    Directory of Open Access Journals (Sweden)

    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.

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

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    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.

  10. 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.

  11. An evaluation of vardenafil as a calcium channel blocker in pulmonary artery in rats

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    Edibe Minareci

    2014-01-01

    Full Text Available Objective: Vardenafil was reported to relax rat pulmonary artery through endothelium-dependent mechanisms. The aim of this in vitro study was to investigate other related mechanisms for this effect. Materials and Methods: Endothelium-intact and denuded artery rings were suspended in order to record isometric tension. In the rings with or without endothelium, the concentration-response curves for vardenafil were generated. In the rings without endothelium the contractile response induced by phenylephrine (Phe or KCl was assessed in the presence or absence of vardenafil. In the last set of experiments, pulmonary artery rings were exposed to calcium-free isotonic depolarizing solution and the contractile response induced by the addition of calcium was evaluated in the presence or absence of vardenafil, nifedipine, verapamil or 1H-[1,2,4] oxadiazolo[4,3-a] quinoxalin-1-one (ODQ. Results: Vardenafil attenuated pulmonary artery contraction induced by phenylephrine in the presence and absence of endothelium. In addition, vardenafil attenuated both Phe or KCl-induced contraction but, it′s effect on the KCl dose-response curve was more significant. Vardenafil also inhibited the contractile response induced by calcium in a dose-dependent manner. Addition of nifedipine or verapamil did not significantly alter this effect while ODQ incubation significantly inhibited vardenafil-induced relaxation. Conclusion: From these findings, it was proposed that vardenafil relaxed rat pulmonary artery through inhibiting calcium influx.

  12. Tissue remodeling of rat pulmonary arteries in recovery from hypoxic hypertension

    OpenAIRE

    Li, Zhuangjie; Huang, Wei; Jiang, Zong Lai; Gregersen, Hans; Fung, Yuan-Cheng

    2004-01-01

    The reversibility of tissue remodeling is of general interest to medicine. Pulmonary arterial tissue remodeling during hypertension induced by hypoxic breathing is well known, but little has been said about the recovery of the arterial wall when the blood pressure is lowered again. We hypothesize that tissue recovery is a function of the oxygen concentration, blood pressure, location on the vascular tree, and time. We measured the changes of blood pressure, vessel lumen, vessel wall thickness...

  13. Krüppel-like Factor 5 contributes to pulmonary artery smooth muscle proliferation and resistance to apoptosis in human pulmonary arterial hypertension

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    Paulin Roxane

    2011-09-01

    Full Text Available Background Pulmonary arterial hypertension (PAH is a vascular remodeling disease characterized by enhanced proliferation of pulmonary artery smooth muscle cell (PASMC and suppressed apoptosis. This phenotype has been associated with the upregulation of the oncoprotein survivin promoting mitochondrial membrane potential hyperpolarization (decreasing apoptosis and the upregulation of growth factor and cytokines like PDGF, IL-6 and vasoactive agent like endothelin-1 (ET-1 promoting PASMC proliferation. Krüppel-like factor 5 (KLF5, is a zinc-finger-type transcription factor implicated in the regulation of cell differentiation, proliferation, migration and apoptosis. Recent studies have demonstrated the implication of KLF5 in tissue remodeling in cardiovascular diseases, such as atherosclerosis, restenosis, and cardiac hypertrophy. Nonetheless, the implication of KLF5 in pulmonary arterial hypertension (PAH remains unknown. We hypothesized that KLF5 up-regulation in PAH triggers PASMC proliferation and resistance to apoptosis. Methods and results We showed that KFL5 is upregulated in both human lung biopsies and cultured human PASMC isolated from distal pulmonary arteries from PAH patients compared to controls. Using stimulation experiments, we demonstrated that PDGF, ET-1 and IL-6 trigger KLF-5 activation in control PASMC to a level similar to the one seen in PAH-PASMC. Inhibition of the STAT3 pathway abrogates KLF5 activation in PAH-PASMC. Once activated, KLF5 promotes cyclin B1 upregulation and promotes PASMC proliferation and triggers survivin expression hyperpolarizing mitochondria membrane potential decreasing PASMC ability to undergo apoptosis. Conclusion We demonstrated for the first time that KLF5 is activated in human PAH and implicated in the pro-proliferative and anti-apoptotic phenotype that characterize PAH-PASMC. We believe that our findings will open new avenues of investigation on the role of KLF5 in PAH and might lead to the

  14. Detection of experimentally produced acute pulmonary arterial occlusion by methyl iodide-131 inhalation imaging

    International Nuclear Information System (INIS)

    Methyl iodide-131 (CH3I-131) is described as an agent for detection of acute experimentally produced pulmonary arterial occlusion in dogs. When gaseous CH3I-131 is inhaled, radioactivity passes instantaneously from the alveoli to the lung capillary bed. Where pulmonary blood flow exists, activity is washed out into the systemic circulation, but in areas of blood stasis, a transient pulmonary hot spot remains. CH3I-131 is easily produced and inexpensive, but administration is awkward and strict radiation safety precautions are mandatory

  15. Oral Therapies for the Treatment of Pulmonary Arterial Hypertension: A Population-Based Cost-Minimization Analysis

    OpenAIRE

    George Dranitsaris; Sanjay Mehta

    2009-01-01

    Background and objective:Background and objective: Pulmonary arterial hypertension (PAH) is a rare but life-threatening condition that is characterized by progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, leading to right-sided heart failure and frequently death. Orally administered agents used for the treatment of symptomatic, moderate-to-severe PAH include sildenafil and the endothelin (ET) receptor antagonists (ERAs), bosentan and sitaxentan (sitaxsentan...

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

    Energy Technology Data Exchange (ETDEWEB)

    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

  17. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. Pulmonary artery pressure limits exercise capacity at high altitude.

    OpenAIRE

    Naeije, Robert; Huez, Sandrine; Lamotte, Michel; Retailleau, Kathleen; Neupane, S; Abramowicz, Daniel; Faoro, Vitalie

    2010-01-01

    Altitude exposure is associated with decreased exercise capacity and increased pulmonary vascular resistance (PVR). Echocardiographic measurements of pulmonary haemodynamics and a cardiopulmonary exercise test were performed in 13 healthy subjects at sea level, in normoxia and during acute hypoxic breathing (1 h, 12% oxygen in nitrogen), and in 22 healthy subjects after acclimatisation to an altitude of 5,050 m. The measurements were obtained after randomisation, double-blinded to the intake ...

  20. 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

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

    DEFF Research Database (Denmark)

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

    2013-01-01

    BACKGROUND: Pulmonary vascular findings are largely unreported in end-stage chronic obstructive pulmonary disease (COPD). METHODS: Pulmonary vascular lesions in explanted lungs from 70 patients with COPD/emphysema or α-1-antitrypsin deficiency were analyzed retrospectively. Patients were stratified...

  2. Hemodynamics and right-ventricle functional characteristics of a swine carotid artery-jugular vein shunt model of pulmonary arterial hypertension: An 18-month experimental study.

    Science.gov (United States)

    Wu, Ji; Luo, Xiaoju; Huang, Yuanyuan; He, Yun; Li, Zhixian

    2015-10-01

    The continuous changes in pulmonary hemodynamic properties and right ventricular (RV) function in pulmonary arterial hypertension (PAH) have not been fully characterized in large animal model of PAH induced by a carotid artery-jugular vein shunt. A minipig model of PAH was induced by a surgical anastomosis between the left common carotid artery and the left jugular vein. The model was validated by catheter examination and pathologic analyses, and the hemodynamic features and right-ventricle functional characteristics of the model were continuously observed by Doppler echocardiography. Of the 45 minipigs who received the surgery, 27 survived and were validated as models of PAH, reflected by mean pulmonary artery pressure ≥25 mmHg, and typical pathologic changes of pulmonary arterial remodeling and RV fibrosis. Non-invasive indices of pulmonary hemodynamics (pulmonary artery accelerating time and its ratio to RV ventricular ejection time) were temporarily increased, then reduced later, similar to changes in tricuspid annular displacement. The Tei index of the RV was elevated, indicating a progressive impairment in RV function. Surgical anastomosis between carotid artery and jugular vein in a minipig is effective to establish PAH, and non-invasive hemodynamic and right-ventricle functional indices measured by Doppler echocardiography may be used as early indicators of PAH. PMID:25595189

  3. 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.

  4. Proximal interruption of a main pulmonary artery with transpleural collateral vessels: CT and MR appearances.

    Science.gov (United States)

    Morgan, P W; Foley, D W; Erickson, S J

    1991-01-01

    The plain chest radiographic, CT, and MR findings in a 31-year-old woman with proximal interruption of the right main pulmonary artery and transpleural collaterals are presented. The diagnosis can be established by both dynamic CT and MR. Intercostal collaterals and their transpleural connections are best demonstrated with dynamic CT.

  5. Unilateral absence of lung perfusion resulting from endarteritis of the pulmonary artery

    International Nuclear Information System (INIS)

    This case shows endarteritis of left pulmonary artery in the angiographic study with absent perfusion of the left lung in the MAA 131I scintigraphy. Considerations are made conderning the presumable diagnosis of infection by 'toxocara canis' larval form. The literature review don't point out a similar case. (author)

  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. 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.

  8. 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

  9. Survival in an incident cohort of patients with pulmonary arterial hypertension in Denmark

    DEFF Research Database (Denmark)

    Korsholm, Kasper; Andersen, Asger; Kirkfeldt, Rikke E;

    2015-01-01

    We aimed to characterize and estimate survival rates in patients diagnosed with pulmonary arterial hypertension (PAH) in western Denmark in the modern management era. All incident cases of PAH were consecutively enrolled in our single-center prospective cohort study between January 2000 and March...

  10. 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

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

    Directory of Open Access Journals (Sweden)

    Butrous Ghazwan S

    2011-10-01

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

  12. 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

  13. Pulmonary artery--bronchial fistula: a new complication of Swan-Ganz catheterization.

    Science.gov (United States)

    Rubin, S A; Puckett, R P

    1979-04-01

    A patient with a Swan-Ganz catheter developed massive hemoptysis. Injection of radiographic contrast media through the catheter revealed rapid filling of the tracheo-bronchial tree, consistent with direct pulmonary artery-bronchial communication. Development of hemoptysis in a patient with a Swan-Ganz catheter should alert the clinician to this possibility. PMID:446146

  14. 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.

  15. 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

  16. Mediastinal lymphadenopathy and pulmonary arterial hypertension in mixed connective tissue disease

    International Nuclear Information System (INIS)

    A case of mixed connective tissue disease (MCTD) is presented in which mediastinal lymphadenopathy was the most prominent radiological finding detected by plain chest radiographs and computed tomography. Pulmonary arterial hypertension, which is a rare and often fatal complication of MCTD, also developed in this patient

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

    OpenAIRE

    Vishal Kumar Jain; Swarna G. Jain; Pratibha Jain Shah; Satya Bhuvan Singh Netam; Sanjay Kumar; Rajesh Singh

    2015-01-01

    We report a case of direct communication between the right pulmonary artery and the left atrium; a rare cause of central cyanosis in 10 year old boy, emphasizing the role of multislice computed tomography scanner in delineating the complex vascular abnormality over more invasive conventional angiography.

  18. 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. Sildenafil add-on therapy in paediatric pulmonary arterial hypertension, experiences of a national referral centre

    NARCIS (Netherlands)

    Douwes, Johannes M.; Roofthooft, Marcus T. R.; Van Loon, Rosa L. E.; Ploegstra, Mark-Jan; Bartelds, Beatrijs; Hillege, Hans L.; Berger, Rudolphus

    2014-01-01

    Objective In paediatric pulmonary arterial hypertension (PAH), the effectiveness of add-on combination PAH-therapy has not yet been systematically studied. The purpose of this study was to determine the effect of sildenafil add-on therapy in paediatric PAH patients treated with bosentan. Methods In

  20. 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

  1. Smooth Muscle Proliferation and Role of the Prostacyclin (IP) Receptor in Idiopathic Pulmonary Arterial Hypertension

    OpenAIRE

    Falcetti, Emilia; Hall, Susan M.; Phillips, Peter G.; Patel, Jigisha; Morrell, Nicholas W.; Haworth, Sheila G; Clapp, Lucie H.

    2010-01-01

    Rationale: Prostacyclin analogs, used to treat idiopathic pulmonary arterial hypertension (IPAH), are assumed to work through prostacyclin (IP) receptors linked to cyclic AMP (cAMP) generation, although the potential to signal through peroxisome proliferator-activated receptor-γ (PPARγ) exists.

  2. Chronic obstructive pulmonary disease and lipid core carotid artery plaques in the elderly: The Rotterdam study

    NARCIS (Netherlands)

    L. Lahousse (Lies); Q.J.A. Bouwhuijsen (Quirin); D.W. Loth (Daan); G.F. Joos (Guy); A. Hofman (Albert); J.C.M. Witteman (Jacqueline); A. van der Lugt (Aad); G.G. Brusselle (Guy); B.H.Ch. Stricker (Bruno)

    2013-01-01

    textabstractRationale: Chronic obstructive pulmonary disease (COPD) is an independent risk factor for ischemic stroke and the risk increases with severity of airflow limitation. Even though vulnerable carotid artery plaque components, such as intraplaque hemorrhage and lipid core, place persons at h

  3. Pulmonary arterial hypertension in rats due to age-related arginase activation in intermittent hypoxia.

    Science.gov (United States)

    Nara, Akina; Nagai, Hisashi; Shintani-Ishida, Kaori; Ogura, Sayoko; Shimosawa, Tatsuo; Kuwahira, Ichiro; Shirai, Mikiyasu; Yoshida, Ken-ichi

    2015-08-01

    Pulmonary arterial hypertension (PAH) is prevalent in patients with obstructive sleep apnea syndrome (OSAS). Aging induces arginase activation and reduces nitric oxide (NO) production in the arteries. Intermittent hypoxia (IH), conferred by cycles of brief hypoxia and normoxia, contributes to OSAS pathogenesis. Here, we studied the role of arginase and aging in the pathogenesis of PAH in adult (9-mo-old) and young (2-mo-old) male Sprague-Dawley rats subjected to IH or normoxia for 4 weeks and analyzed them with a pressure-volume catheter inserted into the right ventricle (RV) and by pulsed Doppler echocardiography. Western blot analysis was conducted on arginase, NO synthase isoforms, and nitrotyrosine. IH induced PAH, as shown by increased RV systolic pressure and RV hypertrophy, in adult rats but not in young rats. IH increased expression levels of arginase I and II proteins in the adult rats. IH also increased arginase I expression in the pulmonary artery endothelium and arginase II in the pulmonary artery adventitia. Furthermore, IH reduced pulmonary levels of nitrate and nitrite but increased nitrotyrosine levels in adult rats. An arginase inhibitor (N(ω)-hydroxy-nor-1-arginine) prevented IH-induced PAH and normalized nitrite and nitrate levels in adult rats. IH induced arginase up-regulation and PAH in adult rats, but not in young rats, through reduced NO production. Our findings suggest that arginase inhibition prevents or reverses PAH. PMID:25490411

  4. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Gautam V Ramani

    2010-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Stojković Gabrijela

    2011-01-01

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

  8. 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.

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

    International Nuclear Information System (INIS)

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

  10. Preoperative and postoperative pulmonary function tests in patients with atrial septal defect and their relation to pulmonary artery pressure and pulmonary:systemic flow ratio.

    OpenAIRE

    Schofield, P. M.; Barber, P V; Kingston, T

    1985-01-01

    Pulmonary diffusing properties and lung volumes were investigated in 44 patients with atrial septal defect, and in 30 of them preoperative and postoperative data were obtained. The patients were divided into three groups according to mean pulmonary artery pressure: less than or equal to 15 mm Hg (group 1), 16-29 mm Hg (group 2), and greater than or equal to 30 mm Hg (group 3). Patients in groups 1 and 2 had a high carbon monoxide transfer test which became normal after surgical correction of ...

  11. Adenosine-5'-triphosphate release by Mannheimia haemolytica, lipopolysaccharide, and interleukin-1 stimulated bovine pulmonary epithelial cells.

    Science.gov (United States)

    Craddick, Michael; Patel, Rakhi; Lower, Amanda; Highlander, Sarah; Ackermann, Mark; McClenahan, David

    2012-09-15

    Mannheimia haemolytica, one of the agents associated with bovine respiratory disease complex, can cause severe lung pathology including the leakage of vascular products into the airways and alveoli. Previous work by this laboratory has demonstrated that bovine lung endothelial and epithelial cells undergo dramatic permeability increases when exposed to adenosine-5'-triphosphate (ATP). Therefore, we wanted to determine if ATP levels were elevated in bronchoalveolar lavage (BAL) samples from calves experimentally infected with M. haemolytica. In addition, cultured bovine pulmonary epithelial (BPE) cells were stimulated with heat-killed and live M. haemolytica bacteria, lipopolysaccharide (LPS), lipoteichoic acid (LTA), interleukin-1 (IL-1), and zymosan activated plasma (ZAP) to determine whether they might release extracellular ATP during in vitro infection. Calves experimentally exposed to M. haemolytica had an approximately 2-fold higher level of ATP in their BAL samples compared to control. BPE cells exposed to increasing numbers of heat-killed or live M. haemolytica had significantly increased levels of ATP release as compared to time-matched controls. Finally, BPE cells treated with several concentrations of LPS and IL-1 had increases in ATP release as compared to time-matched controls. This increase appeared to be a result of active ATP secretion by the cells, as cell viability was similar between treated and non-treated cells. Neither ZAP nor LTA induced any ATP release by the cells. In conclusion, ATP levels are elevated in lung secretions from calves infected with M. haemolytica. In addition, lung epithelial cells can actively release ATP when exposed to heat-killed or live M. haemolytica, LPS or IL-1. PMID:22771196

  12. 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.

  13. 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.

  14. Pleural subxyphoid drain confers better pulmonary function and clinical outcomes in chronic obstructive pulmonary disease after off-pump coronary artery bypass grafting: a randomized controlled trial

    OpenAIRE

    Solange Guizilini; Marcela Viceconte; Esperança, Gabriel Tavares da M.; Douglas W. Bolzan; Milena Vidotto; Rita Simone L Moreira; Andréia Azevedo Câncio; Gomes, Walter J.

    2014-01-01

    Objective: To evaluate the lung function and clinical outcome in severe chronic obstructive pulmonary disease in patients undergoing off-pump coronary artery bypass grafting with left internal thoracic artery graft, comparing the pleural drain insertion in the intercostal versus subxyphoid region. Methods: A randomized controlled trial. Chronic obstructive pulmonary disease patients were randomized into two groups according pleural drain site: II group (n=27) - pleural drain in intercostal sp...

  15. Inferior phrenic arteries supply to the pulmonary hemorrhagic lesions: angiographic identification and interventional management

    International Nuclear Information System (INIS)

    Objective: To describe the manifestations of the inferior phrenic arteries (IPA) supply to the pulmonary hemorrhagic lesions and to evaluate the safety and efficacy of transcatheter arterial embolization (TAE) of the IPA. Methods: The clinical data and imaging findings of eighteen patients with the additional blood supply to the pulmonary hemorrhagic lesions from the IPA were evaluated retrospectively. The causes of the bleeding were lung malignancies in 9, bronchiectasis in 7, and chronic inflammation in 2 patients. TAE supplementally was performed in patients with IPA supply to the pulmonary lesions, using polyvinyl alcohol particles, gelatin sponge particles, and microcoils. Results: Selective arteriogram demonstrates an enlarged IPA, with numerous branches and hypervascularity in all 18 cases, with tumor staining in 9, the contrast material extravasation in 6, and non-specific staining in 2 cases. In addition, IPA-to-pulmonary shunting was found in 9 cases. All the lesions supplying by IPA were adjacent to the pleurae, including adjacent to the diaphragmatic pleura in 11, the mediastinal pleura in 5, and the lateral pleura of the lower lobe in 2 cases. Technical success of IPA embolization was achieved in the 18 cases. Embolization of other nonbronchial systemic arteries (the internal thoracic artery in 7 and intercostal artery in 3) was performed at the same session. All bleeding ceased immediately after' supplemental IPA embolization. Follow-up time ranged from 8 months to 4 years. Mild recurrent hemoptysis occurred in 3 patients at 1,2, 6 months respectively, after the embolization. These patients were responsive to conservative management. Recurrent bleeding did not occur in 15 patients during the follow-up. Conclusion: The pulmonary hemorrhagic lesions, especially adjacent to the diaphragmatic and mediastinal pleurae, can be supplied by IPA, and may result in clinical failure following BAE. Supplemental TAE of IPA is a safe and effective adjunct to BAE in

  16. Anomalous origin of the left coronary artery from the main pulmonary artery. First reported case in Uruguay diagnosed by multislice tomography

    International Nuclear Information System (INIS)

    Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital heart disease (1 in 300,000 live births or 0.5% of all congenital heart diseases in children), producing myocardial ischemia with left ventricular dysfunction and mitral insufficiency of varying degree (dilated cardiomyopathy). Diagnosis allows curative treatment most likely to return as early as ad integrum is made.The clinical diagnosis is suspected by the presence of dilated cardiomyopathy in infants with an electrocardiogram (ECG) showing a characteristic image and pathognomonic . The diagnosis is confirmed by demonstration of the anatomical origin of the left coronary artery from the main pulmonary artery. This demonstration is done classically color Doppler echocardiography and cardiac catheterization with coronary angiography showing the right coronary artery (dilated) originating from the aorta and circulation intercoronary displayed the left coronary artery in the main pulmonary artery. Currently you have new imaging techniques such as multislice CT, able to demonstrate satisfactorily that anomaly. We report the case of an infant with anomalous origin of the left coronary artery from the pulmonary artery, suspected clinically, anatomically demonstration by multislice CT and confirmed by coronary angiography

  17. Borderline pulmonary arterial pressure in systemic sclerosis patients: a post-hoc analysis of the DETECT study

    OpenAIRE

    Visovatti, Scott H; Distler, Oliver; Coghlan, J.; Denton, Christopher P; Grünig, Ekkehard; Bonderman, Diana; Müller-Ladner, Ulf; Pope, Janet E; Vonk, Madelon C; Seibold, James R; Torres-Martin, Juan-Vicente; Doelberg, Martin; Chadha-Boreham, Harbajan; Rosenberg, Daniel M; McLaughlin, Vallerie V.

    2014-01-01

    Introduction Patients with mean pulmonary artery pressures (mPAP) of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)—a condition thought to represent early-stage pulmonary arterial vasculopathy. Based on the DETECT study, this post-hoc analysis examined patient characteristics of systemic sclerosis (SSc) patients with normal mPAP, BoPAP and elevated mPAP, fulfilling pulmonary arterial hypertension (PAH) criteria. Methods Adult patients with a duration of SSc more than 3 y...

  18. 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.

  19. 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

  20. 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. PMID:27419193

  1. 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.

  2. 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

  3. 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.

  4. Changes of Protein Kinase Cα and Cyclin D1 Expressions in Pulmonary Arteries from Smokers with and without Chronic Obstructive Pulmonary Disease

    Institute of Scientific and Technical Information of China (English)

    向敏; 刘先胜; 曾大雄; 王苒; 徐永健

    2010-01-01

    The purpose of this study was to investigate the changes of protein kinase Cα(PKCα) and cyclin D1 expressions in pulmonary arteries from smokers with normal lung function and smokers with mild to moderate chronic obstructive pulmonary disease(COPD).The peripheral lung tissues were obtained from 10 non-smokers with normal lung function(non-smoker group),14 smokers with normal lung function(smoker group),11 smokers with mild to moderate COPD(COPD group).The morphological changes of pulmonary arteries were obs...

  5. Sphingosine-1-phosphate is involved in the occlusive arteriopathy of pulmonary arterial hypertension.

    Science.gov (United States)

    Gairhe, Salina; Joshi, Sachindra R; Bastola, Mrigendra M; McLendon, Jared M; Oka, Masahiko; Fagan, Karen A; McMurtry, Ivan F

    2016-09-01

    Despite several advances in the pathobiology of pulmonary arterial hypertension (PAH), its pathogenesis is not completely understood. Current therapy improves symptoms but has disappointing effects on survival. Sphingosine-1-phosphate (S1P) is a lysophospholipid synthesized by sphingosine kinase 1 (SphK1) and SphK2. Considering the regulatory roles of S1P in several tissues leading to vasoconstriction, inflammation, proliferation, and fibrosis, we investigated whether S1P plays a role in the pathogenesis of PAH. To test this hypothesis, we used plasma samples and lung tissue from patients with idiopathic PAH (IPAH) and the Sugen5416/hypoxia/normoxia rat model of occlusive PAH. Our study revealed an increase in the plasma concentration of S1P in patients with IPAH and in early and late stages of PAH in rats. We observed increased expression of both SphK1 and SphK2 in the remodeled pulmonary arteries of patients with IPAH and PAH rats. Exogenous S1P stimulated the proliferation of cultured rat pulmonary arterial endothelial and smooth-muscle cells. We also found that 3 weeks of treatment of late-stage PAH rats with an SphK1 inhibitor reduced the increased plasma levels of S1P and the occlusive pulmonary arteriopathy. Although inhibition of SphK1 improved cardiac index and the total pulmonary artery resistance index, it did not reduce right ventricular systolic pressure or right ventricular hypertrophy. Our study supports that S1P is involved in the pathogenesis of occlusive arteriopathy in PAH and provides further evidence that S1P signaling may be a novel therapeutic target. PMID:27683614

  6. Calcilytics enhance sildenafil-induced antiproliferation in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Yamamura, Aya; Yagi, Satomi; Ohara, Naoki; Tsukamoto, Kikuo

    2016-08-01

    Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and fatal disease of the pulmonary artery resulting from currently unidentified etiology. IPAH is pathologically characterized as sustained vasoconstriction and vascular remodeling of the pulmonary artery. Phosphodiesterase type 5 (PDE5) inhibitors have been clinically used in the treatment of IPAH. Recently, we have shown that Ca(2+)-sensing receptor (CaSR) antagonists, or calcilytics, inhibit excessive cell proliferation of pulmonary arterial smooth muscle cells (PASMCs) from IPAH patients. In this study, the additive or synergistic effect of calcilytics on antiproliferation following PDE5 inhibition was examined in IPAH-PASMCs by MTT assay. Treatment with sildenafil blocked the excessive cell proliferation of IPAH-PASMCs in a concentration-dependent manner with an IC50 value of 16.9μM. However, sildenafil (0.03-100μM) did not affect the cell growth of PASMCs from normal subjects and patients with chronic thromboembolic pulmonary hypertension (CTEPH). Co-treatment with 0.3μM NPS2143, a calcilytic, additively enhanced the antiproliferative effect induced by sildenafil (3 or 30μM) in IPAH-PASMCs. Additionally, the inhibitory effect of calcilytics, NPS2143 or Calhex 231 (1 or 10μM), on excessive cell proliferation of IPAH-PASMCs was synergistic increased in the presence of 1μM sildenafil. Similar results were obtained by BrdU incorporation assay. These findings reveal that calcilytics additively/synergistically enhance the antiproliferative activity mediated by PDE5 inhibition, suggesting that a combination therapy of a PDE5 inhibitor with a calcilytic may be useful as a novel therapeutic approach for IPAH. PMID:27164419

  7. 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, Thomas; Kofoed, Klaus Fuglsang; Helqvist, Steffen;

    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...

  8. 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.

  9. Effects of bosentan on peripheral endothelial function in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension

    Science.gov (United States)

    Adachi, Shiro; Nakano, Yoshihisa; Kamimura, Yoshihiro; Shimokata, Shigetake; Takeshita, Kyosuke; Murohara, Toyoaki; Kondo, Takahisa

    2016-01-01

    Abstract Endothelin receptor antagonists (ERAs) have been shown to improve the prognosis of patients with pulmonary arterial hypertension (PAH). However, the effect of the oral dual ERA bosentan on peripheral endothelial dysfunction (PED), as assessed by flow-mediated vasodilation (FMD), in patients with pulmonary hypertension is not well characterized. We investigated the effect of bosentan on PED in patients with PAH or inoperable chronic thromboembolic pulmonary hypertension (CTEPH). A total of 18 patients with PAH and 8 with CTEPH were treated with bosentan. All patients underwent FMD assessment before and after 3 months of bosentan treatment. Whereas FMD increased from 6.01% ± 2.42% at baseline to 8.07% ± 3.18% after 3 months (P FMD after bosentan therapy. In addition, FMD at baseline showed no correlation with pulmonary vascular resistance (r = 0.09) or plasma brain natriuretic peptide levels (r = −0.23) in patients with PAH. Bosentan treatment ameliorated PED in patients with PAH but not in those with inoperable CTEPH. In addition, FMD did not correlate with PAH severity. PMID:27252842

  10. 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

  11. Oxidative and nitrosative signalling in pulmonary arterial hypertension - Implications for development of novel therapies.

    Science.gov (United States)

    Hansen, Thomas; Galougahi, Keyvan-Karimi; Celermajer, David; Rasko, Nathalie; Tang, Owen; Bubb, Kristen J; Figtree, Gemma

    2016-09-01

    Pulmonary arterial hypertension (PAH) is a syndrome characterised by an increase in pulmonary vascular resistance. This results in elevated resting pulmonary artery pressure and leads to progressive right ventricular (RV) failure, secondary to increased afterload. Although initially thought to be a disease driven primarily by endothelial dysfunction with a resultant vasoconstrictor versus vasodilator imbalance, it has become increasingly apparent that the rise in pulmonary vascular resistance that causes RV failure is also attributable to pulmonary vascular remodelling. This inflammatory, hyper-proliferative and anti-apoptotic phenotype is accompanied by a metabolic switch from physiological mitochondrial oxidative phosphorylation to aerobic glycolysis. The molecular pathways triggering this cellular metabolic shift have been the subject of extensive investigation, as their discovery will inevitably lead to new therapeutic targets. Reactive oxygen/nitrogen species (ROS/RNS) including hydrogen peroxide, superoxide and peroxynitrite are second messenger molecules that are involved in functional oxidative and nitrosative modification of proteins. Dysregulation of oxidative signalling caused by an excess of ROS and RNS relative to antioxidants has been heavily implicated in the underlying pathophysiology of PAH and likely participates in this metabolic reprogramming. This review will focus on the role of oxidative signalling and redox reactions to the molecular pathology of PAH. In addition, promising novel therapeutic agents targeting these pathways will be discussed.

  12. [Towards new targets for the treatment of pulmonary arterial hypertension : Importance of cell-cell communications].

    Science.gov (United States)

    Tu, Ly; Ghigna, Maria-Rosa; Phan, Carole; Bordenave, Jennifer; Le Hiress, Morane; Thuillet, Raphaël; Ricard, Nicolas; Huertas, Alice; Humbert, Marc; Guignabert, Christophe

    2016-01-01

    Pulmonary arterial hypertension (PAH) is a disorder in which mechanical obstruction of the pulmonary vascular bed is largely responsible for the rise in mean pulmonary arterial pressure (mPAP), resulting in a progressive functional decline despite current available therapeutic options. There are multiple mechanisms predisposing to and/or promoting the aberrant pulmonary vascular remodeling in PAH, and these involve not only altered crosstalk between cells within the vascular wall but also sustained inflammation and dysimmunity, cell accumulation in the vascular wall and excessive activation of some growth factor-stimulated signaling pathways, in addition to the interaction of systemic hormones, local growth factors, cytokines, and transcription factors. Heterozygous germline mutations in the bone morphogenetic protein receptor, type-2 (BMPR2) gene, a gene encoding a receptor for the transforming growth factor (TGF)-β superfamily, can predispose to the disease. Although the spectrum of therapeutic options for PAH has expanded in the last 20 years, available therapies remain essentially palliative. Over the past decade, however, a better understanding of key regulators of this irreversible remodeling of the pulmonary vasculature has been obtained. New and more effective approaches are likely to emerge. The present article profiles the innovative research into novel pathways and therapeutic targets that may lead to the development of targeted agents in PAH. PMID:27687598

  13. Transforming growth factor-β inhibition attenuates pulmonary arterial hypertension in rats

    OpenAIRE

    Megalou, Aikaterini J; Glava, Chryssoula; Oikonomidis, Dimitrios L.; Vilaeti, Agapi; Agelaki, Maria G; Baltogiannis, Giannis G.; Papalois, Apostolos; Vlahos, Antonios P.; Kolettis, Theofilos M

    2010-01-01

    The role of transforming growth factor-β in the pathogenesis of pulmonary arterial hypertension is unclear. We examined the effects of T9429, an antibody against transforming growth factor-β receptors, on hemodynamic, histological and functional parameters in the rat model of monocrotaline-induced pulmonary hypertension. One week after monocrotaline injection (60 mg/kg) in 28 Wistar rats, T9429 (0.1mg/kg daily) was administered intraperito-neally in 19 rats (268±10g) via an osmotic mini-pump ...

  14. Pulmonary arterial hypertension combined with a high cardiac output state: Three remarkable cases

    Science.gov (United States)

    Spruijt, Onno A.; Bogaard, Harm-Jan; Vonk-Noordegraaf, Anton

    2013-01-01

    A congenital extrahepatic portosystemic venous shunt (CEPVS), also known as an Abernethy malformation, is a rare cause of pulmonary arterial hypertension (PAH). In this case series, we describe three male patients of 30, 23, and 27 years of age with PAH due to a CEPVS. In all three patients, a right heart catheterization revealed a high cardiac output. The aim of this case series is to make pulmonary hypertension physicians aware of the possibility of a CEPVS when PAH is accompanied with a high cardiac output state. PMID:24015348

  15. 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.

  16. 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. PMID:26046873

  17. Evidence for two P2-purinoceptor subtypes in human small pulmonary arteries.

    OpenAIRE

    Liu, S. F.; McCormack, D. G.; Evans, T. W.; Barnes, P.J.

    1989-01-01

    1. P2-purinoceptors have not been characterized in human pulmonary vessels and we therefore examined the effects of adenosine 5'-triphosphate (ATP) and its analogues on human isolated small pulmonary arteries (SPA) in vitro. 2. Contractile responses were induced by all of the analogues, with the rank order of potency alpha,beta-methylene-ATP (alpha,beta-meATP) = beta,gamma-methylene-ATP (beta,gamma-meATP) greater than ATP greater than 2-methylthio-ATP, indicating the presence of vasoconstrict...

  18. Change of pulmonary arterial and bronchial diameter during respiration : HRCT findings

    International Nuclear Information System (INIS)

    To evaluate the changes and normal ranges of the artery-bronchus ratio(ABR) during respiration We analyzed HRCT of 10 healthy adults. The HRCT findings of ten healthy adults were analysed. CT scanning was performed with 1mm collimation at 3mm intervals during full inspiration and full expiration, with a range during inspiration from 2cm to 4cm above the carina and from 4cm above to 2cm below the right hemidiaphragm. The range during expiration was from 1cm to 3cm above the carina and from 4cm above to 2cm below the right hemidiaphragm. ABiR (defined as the diameter of pulmonary artery divided by the inner diameter of the bronchus), ABoR (defined as the diameter of pulmonary artery divided by the outer diameter of the bronchus) and BLR (defined as the inner diameter of the bronchus divided by the outer diameter of the bronchus) were measured on the display console. The mean inner diameter of the bronchi was 2.04±0.73mm during inspiration and 1.68±0.51mm during expiration, while the mean diameter of the arteries was 3.95±1.03mm during inspiration and 4.37±1.09mm during expiration. The diameters of the bronchi were thus seen to increase during inspiration, and the diameters of the pulmonary arteries, to decrease. The mean thickness of the bronchial wall was 1.07±0.19mm during inspiration and 1.06±0.24mm during expiration;thus, no change in thickness was seen during respiration (p<0.05). Mean ABiR was 2.01±0.60 (range 1.15-4.58) during inspiration and 2.59±0.74(range 1.16-4.9) during expiration, and in all cases the inner diameter of the bronchus was less than that of the accompanying pulmonary artery. Mean ABoR was 0.91±0.19 during inspiration and 1.09±0.22 during expiration. While for BLR, the corresponding fingures were 0.46±0.06, and 0.44±0.09. HRCT is a useful tool for evaluating changes in the pulmonary arteries and bronchi during respiration

  19. Anomalous origin of the left circumflex coronary artery from the pulmonary artery. A very rare congenital anomaly in an adult patient diagnosed by cardiovascular magnetic resonance

    OpenAIRE

    Giannitsis Evangelos; Ringwald Gerd; Korosoglou Grigorios; Katus Hugo A

    2008-01-01

    Abstract Here we report for the first time on the diagnostic potential of cardiovascular magnetic resonance (CMR) to delineate the proximal course of an anomalous left circumflex coronary artery (LCX) originating from the right pulmonary artery in an adult patient with no other form of congenital heart disease. The patient was referred to our institution due to exertional chest discomfort. X-Ray coronary angiography showed a normal left anterior descending coronary artery (LAD) and right coro...

  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. Gadolinium-enhanced magnetic resonance angiography of right ventricle to pulmonary artery shunts following Norwood 1 palliation in infants

    International Nuclear Information System (INIS)

    The classic Norwood I palliation for the treatment of hypoplastic left-heart disease includes an atrial septectomy, neoaorta creation, and a systemic-to-pulmonary artery shunt. The Norwood I palliation has been modified by the use of a right ventricle-to-pulmonary artery shunt instead of a traditional systemic-to-pulmonary artery shunt. Right ventricle-to-pulmonary artery shunts were non-invasively imaged in four infants using gadolinium-enhanced magnetic resonance angiography. Diagnostic MR angiograms were performed in all infants throughout the length of the shunt and were useful in the detection of shunt patency, stenosis, and aneurysm. Associated surgical conduits and shunts were also evaluated. All findings were verified by subsequent conventional angiography or surgery. (orig.)

  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. Main pulmonary artery area limits exercise capacity in patients long-term after arterial switch operation

    NARCIS (Netherlands)

    Baggen, Vivan J M; Driessen, Mieke M P; Meijboom, Folkert J; Sieswerda, Gertjan Tj; Jansen, Nicolaas J G; van Wijk, Sebastiaan W H; Doevendans, Pieter A; Leiner, Tim; Schoof, Paul H; Takken, Tim; Breur, Johannes M P J

    2015-01-01

    OBJECTIVES: Despite excellent survival in patients after the arterial switch operation, reintervention is frequently required and exercise capacity is decreased in a substantial number of patients. This study relates right-sided imaging features in patients long-term after the arterial switch operat

  5. 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....

  6. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: The Role of Multislice Computed Tomography (MSCT).

    Science.gov (United States)

    Al Umairi, Rashid Saif; Al Kindi, Faiza; Al Busaidi, Fadhila

    2016-09-01

    Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality also known as Bland-White-Garland syndrome. The incidence of ALCAPA is about 1 in every 300,000 live births, and constitutes 0.24% and 0.46% of all congenital cardiac disease. It has a high infant mortality rate reaching up to 90% if left untreated. For many years, the diagnosis of ALCAPA was by angiography or autopsy. However, multislice computed tomography (MSCT) is a non-invasive imaging tool that allows accurate, non-invasive diagnosis of ALCAPA. Here we report a case of ALCAPA in a six-month-old girl who presented with a two-week history of cough, fever, tachypnea, and sweating during feeding. During admission, an echocardiogram was performed that revealed ALCAPA, which was confirmed using CT. We discuss the role of MSCT in its diagnosis. PMID:27602196

  7. Utility of intra-operative capnogram to detect branch pulmonary artery obstruction following total correction of tetralogy of fallot

    OpenAIRE

    Garg Rajnish; Murthy Keshava; Rao Shekhar; John Colin

    2011-01-01

    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 c...

  8. Application of A Microstructural Constitutive Model of the Pulmonary Artery to Patient-Specific Studies: Validation and Effect of Orthotropy

    OpenAIRE

    Zhang, Yanhang; Dunn, Martin L.; Hunter, Kendall S.; Lanning, Craig; Ivy, D. Dunbar; Claussen, Lori; Chen, S. James; Shandas, Robin

    2007-01-01

    We applied a statistical mechanics based microstructural model of pulmonary artery mechanics, developed from our previous studies of rats with pulmonary arterial hypertension (PAH), to patient-specific clinical studies of children with PAH. Our previous animal studies provoked the hypothesis that increased cross-linking density of the molecular chains may be one biological remodeling mechanism by which the PA stiffens in PAH. This study appears to further confirm this hypothesis since varying...

  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. 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.

  12. 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.

  13. Pulmonary artery compression by lymphoma: Detection by ventilation and perfusion lung scan

    International Nuclear Information System (INIS)

    Full text: A 47-year-old male with a past history of non-Hodgkin''s Iymphoma presented with a one week history of right sided pleuritic chest pain. His chest X-ray was reported to be normal and subsequently was referred for a ventilation/perfusion (99mTc-technegas/99mTc-macro-aggregated albumin) lung scan to exclude pulmonary embolism. The scan showed normal ventilation to both lungs, normal perfusion to the left lung and absent perfusion to the right lung, findings not typical of the ''classical'' appearance of pulmonary embolism (i.e. multiple mismatched defects). Computed tomography of the chest showed compression of the right pulmonary artery by recurrent mediastinal Iymphoma. He was therefore treated with chemotherapy rather than anticoagulation. A repeat perfusion study performed eight days later showed much improved perfusion to the right lung, confirming good response to chemotherapy. Pulmonary embolism in this case study is not a likely diagnosis since emboli are usually multiple and involve both lungs. It would be unusual to have a massive embolus occluding blood flow to one lung without any perfusion defect in the other lung. The Journal of Nuclear Medicine, March 1991, quoted a recent review of 525 perfusion lung scans. There were 16 cases of massive unilateral mismatched perfusion defects and pulmonary embolism was the cause in only one of these patients. Massive unilateral mismatched perfusion defects should be considered therefore to be in the category of low probability of pulmonary embolism

  14. Scintigraphic prediction of pulmonary arterial systolic pressure by regional right ventricular ejection fraction during the second half of systole

    Energy Technology Data Exchange (ETDEWEB)

    Friedman, B.J.; Holman, B.L.

    1982-11-01

    In 49 patients in whom gated equilibrium ventriculography and cardiac catheterization were performed within a 6 day interval, total and fractional portions of global and regional right ventricular ejection fraction (RVEF) were correlated with pulmonary arterial systolic pressure. Pulmonary arterial systolic pressure was normal (30 mm Hg or less) in 27 patients (Group I) and elevated (31 mm Hg or greater) in 22 patients (Group II). The second-half regional RVEF was 38 +/- 8% (mean +/- standard deviation) with a range of 30 to 54% for Group I and 22 +/- 6% with a range of 13 to 32% for Group II. The difference between the means was statistically significant (p less than 0.001). Use of a second-half regional RVEF of 30% as the criterion of elevated pulmonary arterial systolic pressure resulted in a sensitivity of 0.86 and a specificity of 1.00. A power curve fit in which pulmonary arterial systolic pressure . 10.91 (second-half regional RVEF)-0.87 allowed accurate estimation (r . -0.85) of pulmonary arterial systolic pressure from the second-half regional RVEF. It is concluded that second-half regional RVEF may be used to accurately detect pulmonary arterial hypertension and to estimate its extent.

  15. Peptide-Coated Liposomal Fasudil Enhances Site Specific Vasodilation in Pulmonary Arterial Hypertension

    OpenAIRE

    Nahar, Kamrun; Absar, Shahriar; Gupta, Nilesh; Kotamraju, Venkata Ramana; McMurtry, Ivan F.; Oka, Masahiko; Komatsu, Masanobu; Nozik-Grayck, Eva; Ahsan, Fakhrul

    2014-01-01

    This study sought to develop a liposomal delivery system of fasudil—an investigational drug for the treatment of pulmonary arterial hypertension (PAH)—that will preferentially accumulate in the PAH lungs. Liposomal fasudil was prepared by film-hydration method, and the drug was encapsulated by active loading. The liposome surface was coated with a targeting moiety, CARSKNKDC, a cyclic peptide; the liposomes were characterized for size, polydispersity index, zeta potential, and storage and neb...

  16. Respiratory and limb muscle dysfunction in pulmonary arterial hypertension: a role for exercise training?

    OpenAIRE

    Panagiotou, Marios; Peacock, Andrew J.; Johnson, Martin K.

    2015-01-01

    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 rev...

  17. Cost effectiveness of first-line oral therapies for pulmonary arterial hypertension: A modelling study

    OpenAIRE

    Coyle, K.; Coyle, D.; Blouin, J.; Lee, K; Jabr, MF; Tran, K.; Mielniczuk, L; Swiston, J; Innes, M.

    2016-01-01

    Background: In recent years, a significant number of costly oral therapies have become available for the treatment of pulmonary arterial hypertension (PAH). Funding decisions for these therapies requires weighing up their effectiveness and costs. Objective: The aim of this study was to assess the cost effectiveness of monotherapy with oral PAH-specific therapies versus supportive care as initial therapy for patients with functional class (FC) II and III PAH in Canada. Methods: A cost-utility ...

  18. Two-years therapy with bosentan of pulmonary arterial hypertension related to connective tissue diseases

    Directory of Open Access Journals (Sweden)

    M. Rizzo

    2011-09-01

    Full Text Available Objective: Pulmonary arterial hypertension (PAH is a rare but severe complication of connective tissue diseases (CTD, with a negative impact on patients survival. Bosentan, a receptor antagonist of endothelin, has been proved effective for the treatment of PAH. The aim of this study was to evaluate the effects and the safety of bosentan administered for 2 years in a group of patients with PAH related to CTD. Methods: Twelve patients with PAH related to systemic sclerosis (8 cases, SLE (2 cases, mixed connective tissue disease (1 case and polymyositis (1 case attending the Rheumatology Unit of Padova University were treated with bosentan for two years. Distance walked in 6 minutes, right ventricular systolic pressure and mean pulmonary artery pressure estimated by doppler echocardiography were evaluated at baseline and after 6, 12, 18 and 24 months of treatment. Safety was assessed by laboratory tests performed every two months. Results: During bosentan treatment, a significant decrease of right ventricular systolic pressure was observed after 6, 12, 18 and 24 months in comparison to baseline, whereas pulmonary artery mean pressure remained unchanged. Distance walked in 6 minutes slightly increased after 6 and 12 months, but significantly decreased after 18 and 24 months, mostly because complications of CTD which compromised the ability to walk arose in 4 patients. Adverse events related to bosentan were observed in 2 cases. Conclusions: Bosentan has been demonstrated effective in reducing pulmonary arterial pressure in a two-year period of treatment. Exercise capacity improved only in the first year of therapy and worsened thereafter, suggesting the opportunity of a combination therapy for a long-term treatment of PAH related to CTD.

  19. Do we need a pulmonary artery catheter in cardiac anesthesia? - An Indian perspective

    OpenAIRE

    Kanchi Muralidhar

    2011-01-01

    There has been considerable controversy regarding the use of pulmonary artery catheter (PAC) in clinical practice. Some studies have indicated poor outcome in patients who were monitored with PAC. However, these studies, which have condemned the use of PAC, were conducted on patients in intensive care units, where the clinical scenarios with regard to patients′ status are somewhat different as compared to those of a cardiac operating room. This study was designed to identify the indica...

  20. Endovascular Treatment of an Aneurysmal Aberrant Systemic Artery Supplying a Pulmonary Sequestrum

    DEFF Research Database (Denmark)

    Kristensen, Katrine Lawaetz; Duus, Louise Aarup; Elle, Bo

    2015-01-01

    An aberrant systemic artery originating from the abdominal aorta supplying a pulmonary sequestration is a rare congenital malformation. This causes a left-to-left shunt. Symptoms include recurrent pneumonias, hemoptysis, and, in the long term, heart failure. Aneurysm of the aberrant vessel...... 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....

  1. Phospholipase D signaling in serotonin-induced mitogenesis of pulmonary artery smooth muscle cells

    OpenAIRE

    Liu, Y; Fanburg, B L

    2008-01-01

    We have previously reported the participation of mitogen-activated protein, Rho, and phosphoinositide-3 (PI3) kinases in separate pathways in serotonin (5-HT)-induced proliferation of pulmonary artery smooth muscle cells (SMCs). In this study, we investigated the possible participation of phospholipase D (PLD) and phosphatidic acid (PA) in this growth process. 5-HT stimulated a time-dependent increase in [3H]phosphatidylbutanol and PA generation. Exposure of SMCs to 1-butanol or overexpressio...

  2. Endothelial 5-HT receptors mediate relaxation of porcine pulmonary arteries in response to ergotamine and dihydroergotamine.

    OpenAIRE

    Glusa, E; Roos, A. (Anna)

    1996-01-01

    1. The aim of the present study was to investigate whether antimigraine ergot compounds may act at endothelial 5-hydroxytryptamine (5-HT) receptors which trigger the release of endothelium-derived relaxing factor (EDRF). Changes in tone of porcine isolated pulmonary arteries were measured isometrically. The integrity of the endothelium was assessed by the bradykinin-induced relaxation of prostaglandin F2 alpha (PGF2 alpha, 3 microM)-precontracted vessels. 2. The ergot derivatives ergotamine, ...

  3. Characterization of 5-HT receptors on human pulmonary artery and vein: functional and binding studies

    OpenAIRE

    Cortijo, Julio; Martí-Cabrera, Miguel; Bernabeu, Eva; Domènech, Teresa; Bou, Josep; Fernández, Andrés G; Beleta, Jorge; Palacios, José M; Morcillo, Esteban J

    1997-01-01

    This study aimed to investigate the 5-hydroxytryptamine (5-HT) receptors mediating contraction of ring preparations isolated from human pulmonary arteries and veins. In functional studies, the responses to 5-HT, sumatriptan, ergotamine, serotonin-O-carboxymethyl-glycyl-tyrosinamide (SCMGT), α-methyl 5-HT (α-Me) and 2-methyl 5-HT (2-Me) were studied with WAY100635, GR127935, ritanserin, zacopride and SB204070 as antagonists.All agonists produced concentration-dependent contractions of human pu...

  4. The Impact of Pulmonary Arterial Pressure on Exercise Capacity in Mild-to-Moderate Cystic Fibrosis: A Case Control Study

    OpenAIRE

    Katerina Manika; Pitsiou, Georgia G.; Boutou, Afroditi K.; Vassilis Tsaoussis; Nikolaos Chavouzis; Marina Antoniou; Maria Fotoulaki; Ioannis Stanopoulos; Ioannis Kioumis

    2012-01-01

    Background. Pulmonary hypertension (PH) is an often complication of severe cystic fibrosis (CF); however, data on the presence and impact of pulmonary vasculopathy in adult CF patients with milder disease, is very limited. Aim. To investigate, for the first time, the impact of systolic pulmonary arterial pressure (PASP) on maximal exercise capacity in adults with mild-to-moderate cystic fibrosis, without PH at rest. Methods. This is a Case Control study. Seventeen adults with mild-to-moderate...

  5. Effectiveness of Spironolactone Plus Ambrisentan for Treatment of Pulmonary Arterial Hypertension (from the [ARIES] Study 1 and 2 Trials)

    OpenAIRE

    Maron, Bradley A.; Waxman, Aaron B.; Opotowsky, Alexander R.; Gillies, Hunter; Blair, Christiana; Aghamohammadzadeh, Reza; Loscalzo, Joseph; Jane A. Leopold

    2013-01-01

    In translational models of pulmonary arterial hypertension (PAH), spironolactone improves cardiopulmonary hemodynamics by attenuating the adverse effects of hyperaldosteronism on endothelin type-B receptor function in pulmonary endothelial cells. This observation suggests that coupling spironolactone with inhibition of endothelin type-A receptor—mediated pulmonary vasoconstriction may be a useful treatment strategy for patients with PAH. We examined clinical data from patients randomized to p...

  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

    International Nuclear Information System (INIS)

    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. Correlative magnetic resonance imaging in the evaluation of aortic and pulmonary artery abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Risius, B.; O' Donnell, J.K.; Geisinger, M.A.; Zelch, M.G.; George, C.R.; Graor, R.A.; Moodie, D.S.

    1985-05-01

    Magnetic resonance imaging (MRI) yields excellent quality images of the cardiovascular system utilizing the inherent natural contrast between flowing blood and the surrounding anatomic structures. To evaluate the clinical usefulness of MRI in the noninvasive diagnosis of large vessel disorders, the authors have performed MRI on 40 pts with either aortic or pulmonary artery abnormalities (18 thoracic or abdominal aortic aneurysms, 8 aorto-occlusive disease, 6 dissecting aneurysms, 4 Marfan's syndrome, 2 pulmonary artery aneurysms 1 pulmonary artery occlusion, 1 aortic coarctation). Images were obtained in the transverse, coronal and sagital body planes utilizing a 0.6T superconductive magnet. Cardiac and/or respiratory gating was employed in most cases. Correlation was made for all studies with conventional or digital subtraction angiography, computed tomography, and/or ultrasound. The diagnostic information obtained by MRI equaled or exceeded that obtained by other imaging techniques except for the few cases where cardiac arrhythmias precluded adequate gated acquisition. All aneurysms and their relationships to adjacent structures were readily demonstrated as were the presence or absence of mural thrombi and dissecting intimal flaps. Angiographically demonstrated atherosclerotic plaques and luminal stenoses were seen by MRI in all patients without arrhythmias. The authors concluded that MRI is a powerful noninvasive diagnostic aid in the delineation of large vessel disorders, especially where knowledge of anatomic interrelationships can guide surgical or other interventional planning.

  10. Pulmonary artery stenosis due to embryonal carcinoma with primary mediastinal location.

    Science.gov (United States)

    Grzegorczyk, Franciszek; Dybowska, Małgorzata; Kuca, Paweł; Czajka, Cezary; Burakowski, Janusz; Langfort, Renata; Orłowski, Tadeusz; Tomkowski, Witold

    2015-01-01

    A 29-year old man was admitted to the intensive care unit after losing consciousness. On physical examination, a loud systolic murmur over the heart was found. Echocardiography revealed narrowing of pulmonary artery with high pressure gradient. Computed tomography of the chest revealed the presence of large tumour localised in the upper anterior mediastinum. Due to the risk of total closure of the pulmonary artery, interventional mediastinotomy was performed and diagnosis of carcinoma embryonale was established. Subsequent chemotherapy (BEP regimen) has brought regression of tumour and significant improvement in haemodynamic parameters (relief of pressure gradient in pulmonary artery). During the second surgery, the resection of all accessible tumour mass together with marginal resection of the right upper lobe was performed. No signs of cardiac or great vessels infiltration was found. Histopathologic examination revealed the necrotic masses and neoplastic foci diagnosed as teratoma immaturum. In a four-month follow-up the patient's condition remained good. The patient is still under the care of both oncological and cardiological specialists. Thus far he has not required further chemotherapy. Holter ECG monitoring revealed no arrhythmia, but the patient is still treated with mexiletine. The patient is planning to return to work. PMID:25754058

  11. 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.

  12. [Significance of pulmonary artery diastolic pressure after myocardial infarction for physical training (author's transl)].

    Science.gov (United States)

    Koenig, W; Toth, L; Többicke, K; Linden, G; Kohn, E

    1978-07-01

    Functional disorders of the left ventricle after myocardial infarction at rest and with exercise can be evaluated with right heart floating catheter by measuring pulmonary artery diastolic pressure. 45% of 200 patients with myocardial infarctions did not tolerate bicycle exercise test with a work load of 50 Watt during 6 minutes. A routine digitalisation of these patients without strict indication did not improve the results. The upper borderline to admit a physical training program is a pulmonary artery diastolic pressure of 20 mm Hg; this should be realised otherwise additional complications have to be expected in long term follow up. The effects of digitalis should be controlled with floating catheter observations in these patients too. There are some indications, that it is possible with the same technique to evaluate hemodynamic responses on psychological strain. Measurements of pulmonary artery diastolic pressure after myocardial infarction open a wide field for individual therapy from drugs to physical training and even to behaviour therapy for stressfull situations. PMID:685379

  13. Validation of a brief telephone battery for neurocognitive assessment of patients with pulmonary arterial hypertension

    Directory of Open Access Journals (Sweden)

    Palevsky Harold I

    2005-04-01

    Full Text Available Abstract Background The effects of pulmonary arterial hypertension on brain function are not understood, despite patients' frequent complaints of cognitive difficulties. Using clinical instruments normally administered during standard in-person assessment of neurocognitive function in adults, we assembled a battery of tests designed for administration over the telephone. The purpose was to improve patient participation, facilitate repeated test administration, and reduce the cost of research on the neuropsychological consequences of acute and chronic cardiorespiratory diseases. We undertook this study to validate telephone administration of the tests. Methods 23 adults with pulmonary arterial hypertension underwent neurocognitive assessment using both standard in-person and telephone test administration, and the results of the two methods compared using interclass correlations. Results For most of the tests in the battery, scores from the telephone assessment correlated strongly with those obtained by in-person administration of the same tests. Interclass correlations between 0.5 and 0.8 were observed for tests that assessed attention, memory, concentration/working memory, reasoning, and language/crystallized intelligence (p ≤ 0.05 for each. Interclass correlations for the Hayling Sentence Completion test of executive function approached significance (p = 0.09. All telephone tests were completed within one hour. Conclusion Administration of this neurocognitive test battery by telephone should facilitate assessment of neuropsychological deficits among patients with pulmonary arterial hypertension living across broad geographical areas, and may be useful for monitoring changes in neurocognitive function in response to PAH-specific therapy or disease progression.

  14. 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.

  15. Tyrosine kinase inhibitors in pulmonary arterial hypertension: a double-edge sword?

    Science.gov (United States)

    Godinas, Laurent; Guignabert, Christophe; Seferian, Andrei; Perros, Frederic; Bergot, Emmanuel; Sibille, Yves; Humbert, Marc; Montani, David

    2013-10-01

    New treatments for pulmonary arterial hypertension (PAH) are a crucial need. The increased proliferation, migration, and survival of pulmonary vascular cells within the pulmonary artery wall in PAH have allowed successful transposition of pathophysiological elements from oncologic researches. Next steps will require translation of these biological advances in PAH therapeutic arsenal and guidelines. This review synthesizes recent data concerning the role of receptor tyrosine kinases and their inhibitors in PAH, with implications in animal models and humans. Results of clinical trials are now accumulating to establish beneficial role of tyrosine kinase inhibitors (TKIs) in PAH and further findings are expected in the near future. Beside this curative approach, evidences of a possible TKI-induced cardiotoxicity are emerging. These safety issues raise concern about a potential amplified harmful effect in PAH, a pathology characterized by an underlying cardiac dysfunction. In addition, analyses of PAH registries shed light on a selective pulmonary vascular toxicity triggered by TKIs, especially dasatinib. These possible dual effects of the TKIs in PAH need to be taken in account for future pharmacological development of this therapeutic class in PAH. PMID:24037637

  16. Effects of physical training on pulmonary arterial pressure during exercise under hypobaric hypoxia in rats

    Science.gov (United States)

    Kashimura, Osamu; Sakai, Akio

    1991-12-01

    In this investigation, we assessed the effects of physical training on exercise-induced systemic and pulmonary hemodynamic changes under hypobaric hypoxia in catheter-implanted rats. We made continuous measurements of pulmonary and systemic arterial pressures during progressive treadmill exercises under hypobaric hypoxia (equivalent to altitudes of 2500 and 5500 m) in 46 control and 41 trained rats. Trained rats were exercised on two running schedules: 4 weeks (4-trained) and 6 weeks (6-trained). Both these groups of trained rats were exercised for the same length of running time each day. The increase in resting mean pulmonary arterial pressure(overline {P_{pa} } ) with increasing equivalent altitude was lower in the two trained groups than in the control group. The increase in(overline {P_{pa} } ) with progressive intensity of exercise was lower in the 6-trained than in the 4-trained and control groups at 610 and 2500 m. The 6-trained rats showed higher pH, P a CO 2 and O2 saturation in their blood than did the control group, whereas the P a O 2 was less. Lung tissue cyclic AMP concentration at rest was higher in the 6-trained than in the control group. Finally, it may be noted that exercise-induced lung tissue vasodilator responses seem to be enhanced in well-trained rats under both normobaric normoxia and hypobaric hypoxia. This study indicates that exercise training may be useful in preventing pulmonary hypertension resulting from both hypoxia and exercise.

  17. 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

  18. Coronary artery fistula draining into pulmonary artery and optimal management: a review

    OpenAIRE

    Rippel, Radoslaw Adam; Kolvekar, Shyam

    2013-01-01

    Coronary artery fistula is a rare congenital malformation of high variability. The disease is illustrated with a description of a case example. The management of patients with coronary artery fistulas remains controversial. Both spontaneous regression and life threatening complications have been described. The fistula can be ligated or embolised; however, there are no long term outcome data regarding management. Intraoperative risk of myocardial infarction is less than 5% and death rate varie...

  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. 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

  1. Bronchial artery embolization for therapy of pulmonary bleeding in patients with cystic fibrosis

    International Nuclear Information System (INIS)

    Introduction: Acute pulmonary emergencies in patient with cystic fibrosis (CF) can be found in cases of pneumothorax as well as hemoptysis. If the bleeding cannot be stopped by conservative methods, an embolization of the bronchial arteries should be done. Materials and Method: 11 patients were embolized using a combination of PVA particles and microcoils. Results: From January 1996 to June 2001 17 bronchial arteries in 11 patients were embolized. 7 patients suffered from chronical hemoptysis, 4 patients had an acute hemoptysis. In 4 patients both sides were embolized, in 3 patients only one side. The remaining 4 patients needed a second intervention, embolizing the other side. The primary embolizated bronchial artery was still closed in all 4 patients. In 1 patient the selective catheterization of a bronchial artery was not successful, thus the embolization could not be carried out. 1 patient died 5 days after the intervention due to a fulminant pneumonia (Pseudomonas aeruginosa) without recurrent bleeding. In two patients atypical branches from intercostal arteries feeding the bronchial arteries were detected and successfully embolized. All patients profited from the therapy, as bleeding could be stopped or at least be reduced. 3 patients suffered from back pain during or after intervention. There were no severe complications like neurological deficiencies or necroses. (orig.)

  2. SNARE-mediated rapid lysosome fusion in membrane raft clustering and dysfunction of bovine coronary arterial endothelium

    OpenAIRE

    Han, Wei-Qing; Xia, Min; Zhang, Chun; Zhang, Fan; Xu, Ming; Li, Ning-Jun; Li, Pin-Lan

    2011-01-01

    The present study attempted to evaluate whether soluble N-ethylmaleimide-sensitive factor attachment protein receptors (SNAREs) mediate lysosome fusion in response to death receptor activation and contribute to membrane raft (MR) clustering and consequent endothelial dysfunction in coronary arterial endothelial cells. By immunohistochemical analysis, vesicle-associated membrane proteins 2 (VAMP-2, vesicle-SNAREs) were found to be abundantly expressed in the endothelium of bovine coronary arte...

  3. Distribution of Pulmonary Perfusion After Myocardial Infarction and its Relationship to Arterial Hypoxaemia

    International Nuclear Information System (INIS)

    Regional distribution of pulmonary blood flow and ventilation was determined at the bedside in the sitting position in patients within five days after myocardial infarction, following hospitalization because of severe angina and myocardial ischaemia and three months after recovery from infarction. A number of patients had several studies in the acute phase of their infarction. Regional distribution studies were initially performed with 133Xe and more recently with 15O-labelled O2 and CO2. Activity distributions in three to five regions of the lung were recorded with a variety of Nal scintillation detection arrays. Positron annihilation coincidence detection as well as gamma-ray collimation was employed in the measurements with 15O-labelled gases. In addition measurements of cardiac output, arterial blood gases and pulmonary gas exchange were carried out in some of the subjects. All subjects were free of cardiorespiratory symptoms at the time of study and none showed any evidence of congestive failure on clinical examination and on chest X-ray. Marked hypoperfusion of the lung base was found in patients by the isotope techniques following acute myocardial infarction. This abnormality, reverted towards normal in the follow-up period, although it never became entirely normal. The patients with myocardial ischaemia showed only very minimal basal hypoperfusion. Cardiac output was normal in all subjects when measured. Arterial hypoxaemia was present in all subjects. The alveolar-arterial O2 difference, while breathing pure O2, was increased after infarction, indicating increased venous admixture (right to left shunting). The hypoxaemia and increased gradient were more marked in those subjects who had greater hypoperfusion of lung base and improved with redistribution of pulmonary perfusion towards normal. The results of these studies suggest that after uncomplicated myocardial infarction there is some element of occult left ventricular failure, which can be detected by

  4. 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

  5. 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.

  6. 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.

  7. 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.

  8. Pulmonary Arterial Hypertension: Use of Delayed Contrast-Enhanced Cardiovascular Magnetic Resonance in Risk Assessment

    International Nuclear Information System (INIS)

    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.m2, 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.m2, 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

  9. 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.

  10. Arterial embolization for management of hemoptysis in pulmonary tuberculosis : factors of rebleeding

    International Nuclear Information System (INIS)

    To evaluate the effectiveness of arterial embolization in reducing hemoptysis in pulmonary tuberculosis, and rebleeding factors after embolization. Fifty-nine patients with massive or recurrent hemoptysis from pulmonary tuberculosis were underwent percutaneous transcatheter embolotherapy and thirteen were subsequently operated on. In 46 patients, we retrospectively analyzed on plain chest PA the extent of pulmonary tuberculosis lesions, the period from initial diagnosis to embolization, and angiographic findings. The extent of lesions shown on plain chest PA were classified into minimal, moderately advanced, and far advanced. If there was no evidence of rebleeding after the first embolization,this was regarded as initial success in the control of hemoptysis. Angiographic findings were classified into hypervascularity, shunt, aneurysmal dilatation, and extravasation. Using the chi-square test, differences in these findings between rebleedig and non-rebleeding cases were anlysed. Immediate control of hemoptysis was achieved in 27 (58.7%) of 46 patients. Hemoptysis recurred in 19 (41.3%) of 46 patients followed up. Rebleeding cases showed more nonbronchial systemic collateral vessels and shunt than non-rebleeding cases (p<0.05). More advanced lesions of pulmonary tuberculosis on plain chest PA showed an increased rebleeding rate after embolization, but this was not statistically significant. There was no correlation between the period from initial diagnosis of pulmonary tuberculosis to embolization and the rate of rebleeding. But the longer the period, the greater the number of nonbronchial systemic collateral vessels. In cases with more advanced lesions of pulmonary tuberculosis on plain chese PA and a long period from initial diagnosis of pulmonary tuberculosis to embolization, angiographic findings showed numerous nonbronchial systemic collateral vessels but increases in the rebleeding rate were statistically not significant. The greater the number of nonbronchial

  11. 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.

  12. Involvement of calcium-sensing receptors in hypoxia-induced vascular remodeling and pulmonary hypertension by promoting phenotypic modulation of small pulmonary arteries.

    Science.gov (United States)

    Peng, Xue; Li, Hong-Xia; Shao, Hong-Jiang; Li, Guang-Wei; Sun, Jian; Xi, Yu-Hui; Li, Hong-Zhu; Wang, Xin-Yan; Wang, Li-Na; Bai, Shu-Zhi; Zhang, Wei-Hua; Zhang, Li; Yang, Guang-Dong; Wu, Ling-Yun; Wang, Rui; Xu, Chang-Qing

    2014-11-01

    Phenotype modulation of pulmonary artery smooth muscle cells (PASMCs) plays an important role during hypoxia-induced vascular remodeling and pulmonary hypertension (PAH). We had previously shown that calcium-sensing receptor (CaSR) is expressed in rat PASMCs. However, little is known about the role of CaSR in phenotypic modulation of PASMCs in hypoxia-induced PAH as well as the underlying mechanisms. In this study, we investigated whether CaSR induces the proliferation of PASMCs in small pulmonary arteries from both rats and human with PAH. PAH was induced by exposing rats to hypoxia for 7-21 days. The mean pulmonary arterial pressure (mPAP), right ventricular hypertrophy index (RVI), the percentage of medial wall thickness to the external diameter (WT %), and cross-sectional total vessel wall area to the total area (WA %) of small pulmonary arteries were determined by hematoxylin and eosin (HE), masson trichrome and Weigert's staining. The protein expressions of matrix metalloproteinase (MMP)-2 and MMP-9, the tissue inhibitors of metalloproteinase (TIMP)-3, CaSR, proliferating cell nuclear antigen (PCNA), phosphorylated extracellular signal-regulated kinase (p-ERK), and smooth muscle cell (SMC) phenotype marker proteins in rat small pulmonary arteries, including calponin, SMα-actin (SMAα), and osteopontin (OPN), were analyzed by immunohistochemistry and Western blotting, respectively. In addition, immunohistochemistry was applied to paraffin-embedded human tissues from lungs of normal human and PAH patients with chronic heart failure (PAH/CHF). Compared with the control group, mPAP, RVI, WT % and WA % in PAH rats were gradually increased with the prolonged hypoxia. At the same time, the expressions of CaSR, MMP-2, MMP-9, TIMP-3, PCNA, OPN, and p-ERK were markedly increased, while the expressions of SMAα and calponin were significantly reduced in lung tissues or small pulmonary arteries of PAH rats. Neomycin (an agonist of CaSR) enhanced but NPS2390 (an

  13. 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

  14. Stent Implantation for Malignant Pulmonary Artery Stenosis in a Metastasizing Non-Small Cell Bronchial Carcinoma

    International Nuclear Information System (INIS)

    A 58-year-old patient with recently diagnosed non-small cell bronchial carcinoma was referred to us with increasing shortness of breath and orthopnea by her family practitioner. To exclude the possibility of a pulmonary embolism, contrast medium-enhanced angio-CT of the thorax was performed. This showed a large mediastinal tumor, which, on the one hand, infiltrated and occluded the left upper lobe bronchus and, on the other, constricted the left pulmonary artery over a considerable part of its length. In view of the palliative situation and massively increasing dyspnea, balloon dilatation of the obstructed left pulmonary artery followed by stent placement was performed. This resulted in an immediate improvement of the symptoms. The originally strongly oxygen-dependent and heavily dyspneic patient could be relieved of the external supply of oxygen and was able to sleep normally without additional medication within 24 h. The patient was able ambulate freely within 2 days, with a markedly improved quality of life.

  15. Leptin and regulatory T-lymphocytes in idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Huertas, Alice; Tu, Ly; Gambaryan, Natalia; Girerd, Barbara; Perros, Frédéric; Montani, David; Fabre, Dominique; Fadel, Elie; Eddahibi, Saadia; Cohen-Kaminsky, Sylvia; Guignabert, Christophe; Humbert, Marc

    2012-10-01

    Immune mechanisms and autoimmunity seem to play a significant role in idiopathic pulmonary arterial hypertension (IPAH) pathogenesis and/or progression, but the pathophysiology is still unclear. Recent evidence has demonstrated a detrimental involvement of leptin in promoting various autoimmune diseases by controlling regulatory T-lymphocytes. Despite this knowledge, the role of leptin in IPAH is currently unknown. We hypothesised that leptin, synthesised by dysfunctional pulmonary endothelium, might play a role in the immunopathogenesis of IPAH by regulating circulating regulatory T-lymphocytes function. First, we collected serum and regulatory T-lymphocytes from controls, and IPAH and scleroderma-associated pulmonary arterial hypertension (SSc-PAH) patients; secondly, we recovered tissue samples and cultured endothelial cells after either surgery or transplantation in controls and IPAH patients, respectively. Our findings indicate that serum leptin was higher in IPAH and SSc-PAH patients than controls. Circulating regulatory T-lymphocyte numbers were comparable in all groups, and the percentage of those expressing leptin receptor was higher in IPAH and SSc-PAH compared with controls, whereas their function was reduced in IPAH and SSc-PAH patients compared with controls, in a leptin-dependent manner. Furthermore, endothelial cells from IPAH patients synthesised more leptin than controls. Our data suggest that endothelial-derived leptin may play a role in the immunopathogenesis of IPAH. PMID:22362850

  16. 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.

  17. A Modified Verhoeff-Van Gieson Elastin Histochemical Stain to Enable Pulmonary Arterial Hypertension Model Characterization

    Science.gov (United States)

    Percival, K.R.; Radi, Z.A.

    2016-01-01

    Optimal histochemical staining is critical to ensure excellent quality stained sections to enable light microscopic and histomorphometric image analysis. Verhoeff-van Gieson is the most widely used histochemical stain for the visualization of vascular elastic fibers. However, it is notoriously difficult to differentiate fine elastic fibers of small vasculature to enable histomorphometric image analysis, especially in organs such as the lung. A tissue fixation procedure of 10% neutral buffered formalin with subsequent fixation in 70% ethanol further compounds the problem of small vessel staining and identification. Therefore, a modified Verhoeff’s elastin stain was developed as a reliable method to optimally highlight the internal and external elastic laminae of small arteries (50-100 µm external diameter) and intra-acinar vessels (10-50 µm external diameter) in 3 µm thick lung tissue sections from models of pulmonary arterial hypertension. This modified Verhoeff’s elastin stain demonstrated well-defined staining of fine elastic fibers of pulmonary blood vessels enabling subsequent histomorphometric image analysis of vessel wall thickness in small arteries and intra-acinar vessels. In conclusion, modification of the standard Verhoeff-van Gieson histochemical stain is needed to visualize small caliber vessels’ elastic fibers especially in tissues fixed in 10% neutral buffered formalin followed by additional fixation in 70% ethanol. PMID:26972717

  18. The physiological basis of pulmonary gas exchange: implications for clinical interpretation of arterial blood gases.

    Science.gov (United States)

    Wagner, Peter D

    2015-01-01

    The field of pulmonary gas exchange is mature, with the basic principles developed more than 60 years ago. Arterial blood gas measurements (tensions and concentrations of O₂ and CO₂) constitute a mainstay of clinical care to assess the degree of pulmonary gas exchange abnormality. However, the factors that dictate arterial blood gas values are often multifactorial and complex, with six different causes of hypoxaemia (inspiratory hypoxia, hypoventilation, ventilation/perfusion inequality, diffusion limitation, shunting and reduced mixed venous oxygenation) contributing variably to the arterial O₂ and CO₂ tension in any given patient. Blood gas values are then usually further affected by the body's abilities to compensate for gas exchange disturbances by three tactics (greater O₂ extraction, increasing ventilation and increasing cardiac output). This article explains the basic principles of gas exchange in health, mechanisms of altered gas exchange in disease, how the body compensates for abnormal gas exchange, and based on these principles, the tools available to interpret blood gas data and, quantitatively, to best understand the physiological state of each patient. This understanding is important because therapeutic intervention to improve abnormal gas exchange in any given patient needs to be based on the particular physiological mechanisms affecting gas exchange in that patient. PMID:25323225

  19. 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 ...

  20. “Expiratory holding” approach in measuring end-expiratory pulmonary artery wedge pressure for mechanically ventilated patients

    Directory of Open Access Journals (Sweden)

    Yang W

    2013-10-01

    Full Text Available Wanjie Yang,1 Xuefeng Zhao,1 Qingguo Feng,1 Youzhong An,2 Kai Wei,1 Wei Wang,1 Chang Li,1 Xiuling Cheng1 1Intensive Care Unit, the Fifth Central Hospital of Tianjin, Tianjin, People’s Republic of China; 2Intensive Care Unit, Peking University People’s Hospital, Beijing, People’s Republic of China Objective: To accurately measure the end-expiratory pulmonary artery wedge pressure (PAWP with the “expiration holding” function on the ventilator and the “pulmonary artery wedge pressure review” software on the monitor. Materials and methods: Fifty prospective measurements were made on 12 patients undergoing pulmonary artery catheter and mechanical ventilation. All measurements were divided into <8 mmHg or ≥8 mmHg subgroups according to respiratory variability, and they were then subdivided into either an airway pressure display measurement group (AM group or an expiration holding (EH group for comparison. Results: In all measurements, the two groups showed similar levels of accuracy; however, for the time spent for measurement, the EH group was much faster than the airway pressure display measurement group (P<0.001. Additionally, the EH group was associated with lower medical costs. Conclusion: The expiration holding approach measured the PAWP more accurately, more quickly, and with reduced costs in comparison to the airway pressure display approach. Keywords: expiration holding, pulmonary artery wedge pressure, eePAWP, mechanical ventilation, pulmonary artery catheter

  1. 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.

  2. Noninvasive identification of left-sided heart failure in a population suspected of pulmonary arterial hypertension.

    Science.gov (United States)

    Jacobs, Wouter; Konings, Thelma C; Heymans, Martijn W; Boonstra, Anco; Bogaard, Harm Jan; van Rossum, Albert C; Vonk Noordegraaf, Anton

    2015-08-01

    Exclusion of pulmonary hypertension secondary to left-sided heart disease (left heart failure (LHF)) is pivotal in the diagnosis of pulmonary arterial hypertension (PAH). In case of doubt, invasive measurements are recommended. The aim of the present study was to investigate whether it is possible to diagnose LHF using noninvasive parameters in a population suspected of PAH.300 PAH and 80 LHF patients attended our pulmonary hypertension clinic before August 2010, and were used to build the predictive model. 79 PAH and 55 LHF patients attended our clinic from August 2010, and were used for prospective validation.A medical history of left heart disease, S deflection in V1 plus R deflection in V6 in millimetres on ECG, and left atrial dilation or left valvular heart disease that is worse than mild on echocardiography were independent predictors of LHF. The derived risk score system showed good predictive characteristics: R(2)=0.66 and area under the curve 0.93. In patients with a risk score ≥72, there is 100% certainty that the cause of pulmonary hypertension is LHF. Using this risk score system, the number of right heart catheterisations in LHF may be reduced by 20%.In a population referred under suspicion of PAH, a predictive model incorporating medical history, ECG and echocardiography data can diagnose LHF noninvasively in a substantial percentage of cases. PMID:25837029

  3. 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.

  4. 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

  5. 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

  6. Nicotinamide Adenine Dinucleotide Phosphate Oxidase-Mediated Redox Signaling and Vascular Remodeling by 16α-Hydroxyestrone in Human Pulmonary Artery Cells: Implications in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Hood, Katie Y; Montezano, Augusto C; Harvey, Adam P; Nilsen, Margaret; MacLean, Margaret R; Touyz, Rhian M

    2016-09-01

    Estrogen and oxidative stress have been implicated in pulmonary arterial hypertension (PAH). Mechanisms linking these systems are elusive. We hypothesized that estrogen metabolite, 16α-hydroxyestrone (16αOHE1), stimulates nicotinamide adenine dinucleotide phosphate oxidase (Nox)-induced reactive oxygen species (ROS) generation and proliferative responses in human pulmonary artery smooth muscle cells (hPASMCs) and that in PAH aberrant growth signaling promotes vascular remodeling. The pathophysiological significance of estrogen-Nox-dependent processes was studied in female Nox1(-/-) and Nox4(-/-) mice with PAH. PASMCs from control subjects (control hPASMCs) and PAH patients (PAH-hPASMCs) were exposed to estrogen and 16αOHE1 in the presence/absence of inhibitors of Nox, cytochrome P450 1B1, and estrogen receptors. Estrogen, through estrogen receptor-α, increased Nox-derived ROS and redox-sensitive growth in hPASMCs, with greater effects in PAH-hPASMCs versus control hPASMCs. Estrogen effects were inhibited by cytochrome P450 1B1 blockade. 16αOHE1 stimulated transient ROS production in hPASMCs, with sustained responses in PAH-hPASMCs. Basal expression of Nox1/Nox4 was potentiated in PAH-hPASMCs. In hPASMCs, 16αOHE1 increased Nox1 expression, stimulated irreversible oxidation of protein tyrosine phosphatases, decreased nuclear factor erythroid-related factor 2 activity and expression of nuclear factor erythroid-related factor 2-regulated antioxidant genes, and promoted proliferation. This was further amplified in PAH-hPASMCs. Nox1(-/-) but not Nox4(-/-) mice were protected against PAH and vascular remodeling. Our findings demonstrate that in PAH-hPASMCs, 16αOHE1 stimulates redox-sensitive cell growth primarily through Nox1. Supporting this, in vivo studies exhibited protection against pulmonary hypertension and remodeling in Nox1(-/-) mice. This study provides new insights through Nox1/ROS and nuclear factor erythroid-related factor 2 whereby 16αOHE1 influences

  7. Histone deacetylation contributes to low extracellular superoxide dismutase expression in human idiopathic pulmonary arterial hypertension.

    Science.gov (United States)

    Nozik-Grayck, Eva; Woods, Crystal; Stearman, Robert S; Venkataraman, Sujatha; Ferguson, Bradley S; Swain, Kalin; Bowler, Russell P; Geraci, Mark W; Ihida-Stansbury, Kaori; Stenmark, Kurt R; McKinsey, Timothy A; Domann, Frederick E

    2016-07-01

    Epigenetic mechanisms, including DNA methylation and histone acetylation, regulate gene expression in idiopathic pulmonary arterial hypertension (IPAH). These mechanisms can modulate expression of extracellular superoxide dismutase (SOD3 or EC-SOD), a key vascular antioxidant enzyme, and loss of vascular SOD3 worsens outcomes in animal models of pulmonary arterial hypertension. We hypothesized that SOD3 gene expression is decreased in patients with IPAH due to aberrant DNA methylation and/or histone deacetylation. We used lung tissue and pulmonary artery smooth muscle cells (PASMC) from subjects with IPAH at transplantation and from failed donors (FD). Lung SOD3 mRNA expression and activity was decreased in IPAH vs. FD. In contrast, mitochondrial SOD (Mn-SOD or SOD2) protein expression was unchanged and intracellular SOD activity was unchanged. Using bisulfite sequencing in genomic lung or PASMC DNA, we found the methylation status of the SOD3 promoter was similar between FD and IPAH. Furthermore, treatment with 5-aza-2'-deoxycytidine did not increase PASMC SOD3 mRNA, suggesting DNA methylation was not responsible for PASMC SOD3 expression. Though total histone deacetylase (HDAC) activity, histone acetyltransferase (HAT) activity, acetylated histones, and acetylated SP1 were similar between IPAH and FD, treatment with two selective class I HDAC inhibitors increased SOD3 only in IPAH PASMC. Class I HDAC3 siRNA also increased SOD3 expression. Trichostatin A, a pan-HDAC inhibitor, decreased proliferation in IPAH, but not in FD PASMC. These data indicate that histone deacetylation, specifically via class I HDAC3, decreases SOD3 expression in PASMC and HDAC inhibitors may protect IPAH in part by increasing PASMC SOD3 expression. PMID:27233998

  8. 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

  9. Endothelin-1 Predicts Hemodynamically Assessed Pulmonary Arterial Hypertension in HIV Infection.

    Directory of Open Access Journals (Sweden)

    Rushi V Parikh

    Full Text Available HIV infection is an independent risk factor for PAH, but the underlying pathogenesis remains unclear. ET-1 is a robust vasoconstrictor and key mediator of pulmonary vascular homeostasis. Higher levels of ET-1 predict disease severity and mortality in other forms of PAH, and endothelin receptor antagonists are central to treatment, including in HIV-associated PAH. The direct relationship between ET-1 and PAH in HIV-infected individuals is not well described.We measured ET-1 and estimated pulmonary artery systolic pressure (PASP with transthoracic echocardiography (TTE in 106 HIV-infected individuals. Participants with a PASP ≥ 30 mmHg (n = 65 underwent right heart catheterization (RHC to definitively diagnose PAH. We conducted multivariable analysis to identify factors associated with PAH.Among 106 HIV-infected participants, 80% were male, the median age was 52 years and 77% were on antiretroviral therapy. ET-1 was significantly associated with higher values of PASP [14% per 0.1 pg/mL increase in ET-1, p = 0.05] and PASP ≥ 30 mmHg [PR (prevalence ratio = 1.24, p = 0.012] on TTE after multivariable adjustment for PAH risk factors. Similarly, among the 65 individuals who underwent RHC, ET-1 was significantly associated with higher values of mean pulmonary artery pressure and PAH (34%, p = 0.003 and PR = 2.43, p = 0.032, respectively in the multivariable analyses.Higher levels of ET-1 are independently associated with HIV-associated PAH as hemodynamically assessed by RHC. Our findings suggest that excessive ET-1 production in the setting of HIV infection impairs pulmonary endothelial function and contributes to the development of PAH.

  10. Clinical characterization and survival of patients with borderline elevation in pulmonary artery pressure.

    Science.gov (United States)

    Heresi, Gustavo A; Minai, Omar A; Tonelli, Adriano R; Hammel, Jeffrey P; Farha, Samar; Parambil, Joseph G; Dweik, Raed A

    2013-12-01

    Normal resting mean pulmonary artery pressure (PAP) is 8-20 mmHg. Pulmonary hypertension is defined as mean PAP of ≥25 mmHg. Borderline PAP levels of 21-24 mmHg are of unclear significance. We sought to determine the clinical characteristics and survival of subjects with mean PAP of 21-24 mmHg. We examined 1,491 patients enrolled in the Cleveland Clinic Pulmonary Hypertension Registry between February 1990 and May 2012 with baseline right heart catheterization. The relationship between PAP and all-cause mortality was assessed by Cox models and a tree-based analysis. Sixty-three patients had borderline PAP (underlying conditions: 12 left heart disease, 20 respiratory disease, 17 connective-tissue disease, 4 others, and 10 none). We then compared 3 groups: borderline PAP without heart or lung disease ([Formula: see text]), normal PAP without heart or lung disease ([Formula: see text]), and category 1 pulmonary arterial hypertension (PAH; [Formula: see text]). Borderline-PAP patients had levels of hemodynamic and functional compromise between those for normal-PAP patients and those for patients with PAH. Borderline PAP was associated with increased mortality compared to normal PAP (hazard ratio: 4.03 [95% confidence interval: 0.78-20.80], [Formula: see text]). A tree-based analysis demonstrated almost identical cut points in mean PAP (≤20, 21-26, and ≥27 mmHg) associated with differential survival ([Formula: see text]). Connective-tissue disease and an elevated transpulmonary gradient were predictors of worse survival in the borderline-PAP population. Borderline PAP elevation is associated with decreased survival, particularly in the context of connective-tissue disease and an elevated transpulmonary gradient. PMID:25006408

  11. 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

  12. 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.

  13. Iptakalim rescues human pulmonary artery endothelial cells from hypoxia-induced nitric oxide system dysfunction

    OpenAIRE

    Zong, Feng; Zuo, Xiang-Rong; Wang, Qiang; ZHANG, SHI-JIANG; Xie, Wei-Ping; Wang, Hong

    2011-01-01

    The aim of this study was to assess whether hypoxia inhibits endothelial nitric oxide synthase (eNOS) activity and nitric oxide (NO) production, and whether iptakalim may rescue human pulmonary artery endothelial cells (HPAECs) from hypoxia-induced NO system dysfunction. HPAECs were cultured under hypoxic conditions in the absence or presence of 0.1, 10 and 1,000 μM iptakalim or the combination of 10 μM iptakalim and 1, 10 and 100 μM glibenclamide for 24 h, and the eNOS activity and NO levels...

  14. 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.

  15. The economic burden of pulmonary arterial hypertension (PAH) in the US on payers and patients

    OpenAIRE

    Sikirica, Mirko; Iorga, Serban R; Bancroft, Tim; Potash, Jesse

    2014-01-01

    Background Pulmonary arterial hypertension (PAH) is a rare condition that can ultimately lead to right heart failure and death. In this study we estimated the health care costs and resource utilization associated with PAH in a large US managed care health plan. Methods Subjects with claims-based evidence of PAH from 1/1/2004 to 6/30/2010 (identification period) were selected. To be included in the final PAH study sample, subjects were required to have ≥2 claims with a primary PH diagnosis; ≥2...

  16. Estimated in vivo postnatal surface growth patterns of the ovine main pulmonary artery and ascending aorta.

    Science.gov (United States)

    Fata, Bahar; Gottlieb, Danielle; Mayer, John E; Sacks, Michael S

    2013-07-01

    Delineating the normal postnatal development of the pulmonary artery (PA) and ascending aorta (AA) can inform our understanding of congenital abnormalities, as well as pulmonary and systolic hypertension. We thus conducted the following study to delineate the PA and AA postnatal growth deformation characteristics in an ovine model. MR images were obtained from endoluminal surfaces of 11 animals whose ages ranged from 1.5 months/15.3 kg mass (very young) to 12 months/56.6 kg mass (adult). A bicubic Hermite finite element surface representation was developed for the each artery from each animal. Under the assumption that the relative locations of surface points were retained during growth, the individual animal surface fits were subsequently used to develop a method to estimate the time-evolving local effective surface growth (relative to the youngest measured animal) in the end-diastolic state. Results indicated that the spatial and temporal surface growth deformation patterns of both arteries, especially in the circumferential direction, were heterogeneous, leading to an increase in taper and increase in cross-sectional ellipticity of the PA. The longitudinal PA growth stretch of a large segment on the posterior wall reached 2.57 ± 0.078 (mean ± SD) at the adult stage. In contrast, the longitudinal growth of the AA was smaller and more uniform (1.80 ± 0.047). Interestingly, a region of the medial wall of both arteries where both arteries are in contact showed smaller circumferential growth stretches-specifically 1.12 ± 0.012 in the PA and 1.43 ± 0.071 in the AA at the adult stage. Overall, our results indicated that contact between the PA and AA resulted in increasing spatial heterogeneity in postnatal growth, with the PA demonstrating the greatest changes. Parametric studies using simplified geometric models of curved arteries during growth suggest that heterogeneous effective surface growth deformations must occur to account for the

  17. 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.

  18. 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.

  19. Utility of intra-operative capnogram to detect branch pulmonary artery obstruction following total correction of tetralogy of Fallot.

    Science.gov (United States)

    Garg, Rajnish; Murthy, Keshava; Rao, Shekhar; John, Colin

    2011-01-01

    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. PMID:21196674

  20. Influence of acute pancreatitis on the in vitro responsiveness of rat mesenteric and pulmonary arteries

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    Antunes Edson

    2008-05-01

    Full Text Available Abstract Background Acute pancreatitis is an inflammatory disease characterized by local tissue injury and systemic inflammatory response leading to massive nitric oxide (NO production and haemodynamic disturbances. Therefore, the aim of this work was to evaluate the vascular reactivity of pulmonary and mesenteric artery rings from rats submitted to experimental pancreatitis. Male Wistar rats were divided into three groups: saline (SAL; tauracholate (TAU and phospholipase A2 (PLA2. Pancreatitis was induced by administration of TAU or PLA2 from Naja mocambique mocambique into the common bile duct of rats, and after 4 h of duct injection the animals were sacrificed. Concentration-response curves to acetylcholine (ACh, sodium nitroprusside (SNP and phenylephrine (PHE in isolated mesenteric and pulmonary arteries were obtained. Potency (pEC50 and maximal responses (EMAX were determined. Blood samples were collected for biochemical analysis. Results In mesenteric rings, the potency for ACh was significantly decreased from animals treated with TAU (about 4.2-fold or PLA2 (about 6.9-fold compared to saline group without changes in the maximal responses. Neither pEC50 nor EMAX values for Ach were altered in pulmonary rings in any group. Similarly, the pEC50 and the EMAX values for SNP were not changed in both preparations in any group. The potency for PHE was significantly decreased in rat mesenteric and pulmonary rings from TAU group compared to SAL group (about 2.2- and 2.69-fold, for mesenteric and pulmonary rings, respectively. No changes were seen in the EMAX for PHE. The nitrite/nitrate (NOx- levels were markedly increased in animals submitted to acute pancreatitis as compared to SAL group, approximately 76 and 68% in TAU and PLA2 protocol, respectively. Conclusion Acute pancreatitis provoked deleterious effects in endothelium-dependent relaxing response for ACh in mesenteric rings that were strongly associated with high plasma NOx- levels as

  1. Color Doppler evaluation of the ocular arterial flow changes in chronic obstructive pulmonary disease

    International Nuclear Information System (INIS)

    Purpose: To evaluate the hemodynamic changes in the extraocular orbital vessels of the patients with chronic obstructive pulmonary disease (COPD), using color Doppler ultrasonography (CDU) technique, and to compare the results with those of healthy control subjects. Methods: Forty-five patients with COPD and 17 healthy control subjects were included in this study. Patients with COPD were classified according to the Global Initiative for Chronic Obstructive Lung Disease (GOLD). Fifteen patients of stage I COPD (mild airflow limitation), stage II COPD (worsening airflow limitation) or stage III COPD (severe airflow limitation) were enrolled into Group I, II and III, respectively. End tidal carbon dioxide (EtCO2), peripheral oxygen saturation (SpO2), pulse rate (PR) and respiratory rate (RR) were measured by using capnograph/pulse oximeter in all patients. Measurements were performed in only one randomly chosen eye of each participant. The peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistance index (RI) were measured in the ophthalmic artery (OA), central retinal artery (CRA), lateral short posterior ciliary artery (LPCA) and medial short posterior ciliary artery (MPCA), using CDU technique. Results: The PSV measurements of the OA were significantly higher in Groups II and III compared to control group (p 2 with PSV (r = 0.53, p < 0.01) and EDV (r = 0.51, p < 0.01) of the OA. Statistically significant correlations were also found for the SpO2 with RI (r = -0.34, p < 0.05) in the OA. Conclusion: We concluded that COPD is associated with impaired retrobulbar hemodynamics, especially in the ophthalmic artery. Moreover, central retinal and posterior ciliary arteries with increased resistance are also found to be affected when compared with healthy control eyes

  2. 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.

  3. Enhanced production and action of cyclic ADP-ribose during oxidative stress in small bovine coronary arterial smooth muscle.

    Science.gov (United States)

    Zhang, Andrew Y; Yi, Fan; Teggatz, Eric G; Zou, Ai-Ping; Li, Pin-Lan

    2004-03-01

    Recent studies in our lab and by others have indicated that cyclic ADP-ribose (cADPR) as a novel second messenger is importantly involved in vasomotor response in various vascular beds. However, the mechanism regulating cADPR production and actions remains poorly understood. The present study determined whether changes in redox status influence the production and action of cADPR in coronary arterial smooth muscle cells (CASMCs) and thereby alters vascular tone in these arteries. HPLC analyses demonstrated that xanthine (X, 40 microM)/xanthine oxidase (XO, 0.1 U/ml), a superoxide-generating system, increased the ADP-ribosyl cyclase activity by 59% in freshly isolated bovine CASMCs. However, hydrogen peroxide (H2O2, 1-100 microM) had no significant effect on ADP-ribosyl cyclase activity. In these CASMCs, X/XO produced a rapid increase in [Ca2+]i (Delta[Ca2+]i=201 nM), which was significantly attenuated by a cADPR antagonist, 8-Br-cADPR. Both inhibition of cADPR production by nicotinamide (Nicot) and blockade of Ca2+-induced Ca2+ release (CICR) by tetracaine (TC) and ryanodine (Rya) significantly reduced X/XO-induced rapid Ca2+ responses. In isolated, perfused, and pressurized small bovine coronary arteries, X at 2.5-80 microM with a fixed XO level produced a concentration-dependent vasoconstriction with a maximal decrease in arterial diameter of 45%. This X/XO-induced vasoconstriction was significantly attenuated by 8-Br-cADPR, Nicot, TC, or Rya. We conclude that superoxide activates cADPR production, and thereby mobilizes intracellular Ca2+ from the SR and produces vasoconstriction in coronary arteries.

  4. 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...

  5. KCNA5 gene is not confirmed as a systemic sclerosis-related pulmonary arterial hypertension genetic susceptibility factor

    OpenAIRE

    Bossini-Castillo, L.; Simeón, Carmen P.; Beretta, L; Broen, Jasper C.; Vonk, Madelon C; Callejas-Rubio, J. L.; Carreira, P; Rodríguez-Rodríguez, Luis; García-Portales, Rosa; González-Gay, M. A.; Castellví, I.; Camps, M T; Tolosa, Carlos; Vicente-Rabaneda, Esther; Egurbide, M V

    2012-01-01

    Introduction: Potassium voltage-gated channel shaker-related subfamily member 5 (KCNA5) is implicated in vascular tone regulation, and its inhibition during hypoxia produces pulmonary vasoconstriction. Recently, a protective association of the KCNA5 locus with systemic sclerosis (SSc) patients with pulmonary arterial hypertension (PAH) was reported. Hence, the aim of this study was to replicate these findings in an independent multicenter Caucasian SSc cohort. Methods: The 2,343 SSc cases...

  6. Osthole relaxes pulmonary arteries through endothelial phosphatidylinositol 3-kinase/Akt-eNOS-NO signaling pathway in rats.

    Science.gov (United States)

    Yao, Li; Lu, Ping; Li, Yumei; Yang, Lijing; Feng, Hongxuan; Huang, Yong; Zhang, Dandan; Chen, Jianguo; Zhu, Daling

    2013-01-15

    Pulmonary arterial hypertension is a life-threatening disease lacking effective therapies. Osthole is a natural coumarin compound isolated from Angelica pubescens Maxim., which possesses hypotensive effect. Although its effects on isolated thoracic aorta (systemic circulating system) are clarified, it remains unclear whether Osthole relaxes isolated pulmonary arteries (PAs) (pulmonary circulating system). The aim of this study was to investigate the effects of Osthole on isolated PAs and the underlying mechanisms. We examined PA relaxation induced by Osthole in isolated human and rat PA rings with force-electricity transducers, the expression and activity of endothelial nitric oxide synthase (eNOS) and protein kinase B (Akt) with western blot, and nitric oxide (NO) production using DAF-FM DA fluorescent indicator. The results showed that Osthole elicited a dose-dependent vasorelaxation activity with phenylephrine-precontracted human and rat PA rings, which can be diminished by endothelium denudation and inhibition of eNOS, while having no effect on rat mesenteric arteries. Osthole increased NO release as well as activation of Akt and eNOS, indicated with increased phosphorylations of Akt at Ser-473 and eNOS at Ser-1177 in endothelial cells. PI3K inhibitor LY294002 also blocked Osthole induced vasodilation. In summary, dilative effect of Osthole was dependent on endothelial integrity and NO production, and was mediated by endothelial PI3K/Akt-eNOS-NO pathway. These may provide a new pulmonary vasodilator for the therapy of pulmonary arterial hypertension. PMID:23220709

  7. 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.

  8. 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. PMID:26667539

  9. Unusual Case of Left Bronchial Compression by Aneurysmal Pulmonary Arteries in a Child With Atrial Septal Defect.

    Science.gov (United States)

    Pawar, Ranjit; Kumar, Gaurav; Sharma, Vipul; Dalal, S S

    2016-07-01

    We report an uncommon case of large ostium secundum atrial septal defect (ASD) with severe pulmonary arterial hypertension, with associated aneurysmal dilatation of the pulmonary arteries (PAs) leading to compression of the left main bronchus and collapse of the entire left lung in a 15-month-old female child. The patient was managed by surgical closure of the ASD, translocation of the right PA anterior to the aorta with PA aneurysmorrhaphy. Left bronchial compression was relieved with complete lung expansion on the third postoperative day. PMID:26865068

  10. Interventional therapy of refractory hemoptysis complicated with bronchial artery to pulmonary circulation shunt

    International Nuclear Information System (INIS)

    Objective: The presence of bronchial artery to pulmonary circulation shunt (BPS) is the pathologic reason for refractory hemoptysis. Those who have accepted traditional bronchial arterial embolization (BAE) treatment usually carry high risks of recurrence of hemoptysis. At the same time,because of the limits of BAE treatment, safety can not be ignored when we pay full attention to its therapeutic effect. This research aims to find out an effective method to treat the refractory hemoptysis with BPS. Methods: During the period from Sep. 1996 to Feb. 2010, two hundred and twelve patients of hemoptysis were treated with BAE. Of the total 212 patients, BPS was confirmed by DSA angiography in 99, including 72 males and 27 females with a mean age of 47.6 years. The primary diseases included bronchiectasis (n = 25), tuberculosis (n = 64) and inflammatory disorders(n = 10). All patients were treated with BAE. According to the embolization agent used in the treatment the patients were divided into pure Gelfoam group (n = 52) and permanent embolization group (n = 47, using PVA particles, coils, etc.). All patients were followed up regularly at one day, two weeks, four weeks, six months, one year, two years after the treatment. The data were analyzed by using chi square test and rank sum test. Results: Of 52 cases in permanent embolization group, complete cure was achieved in 49, excellent results in 2 and effective response in one. For patients in pure Gelfoam group, complete cure was obtained in 37, excellent results in 16, effective response in 2 and ineffectiveness in 2. Statistic analysis showed that a significant difference in therapeutic effectiveness existed between two groups (P < 0.05). Conclusion: This study clearly indicates that selective bronchial arterial permanent dual embolization is an effective technique for the treatment of refractory hemoptysis, which is definitely superior to the pure Gelfoam emboliztion in obtaining excellent therapeutic results. Besides

  11. Dynamic pulmonary perfusion imaging in patients with idiopathic pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Objective: To investigate the value of dynamic PPI in patients with IPAH. Methods: Twenty consecutive patients with IPAH underwent dynamic PPI and right ventricular catheterization. Ten healthy volunteers were also recruited in this study as control. Total lung ROI was drawn to calculate time-activity curves from dynamic PPI. Lung equilibrium time (LET), the duration from 99Tcm-MAA entering the lungs to equilibrium, was measured. Comparisons between two groups were carried out using t-test. Correlations between LET and hemodynamic parameters were determined by Pearson correlation analysis. Results: LET was significantly prolonged in IPAH patients over the control group ((33.9 ± 15.5) s vs (14.4 ± 3.7) s, t=5.340, P<0.001). Significant correlations were found between LET and mPAP (r=0.566, P<0.01) and between LET and total pulmonary resistance (TPR) measured by right heart catheterization (r=0.688, P<0.05). LET had a negative linear relationship with CI (r=-0.480, P<0.05). Conclusion: Dynamic PPI is a practical and noninvasive method for evaluating the haemodynamic function of right heart in patients with IPAH. (authors)

  12. [Systemic-pulmonary artery shunt using Golaski graft: trial for measurement of the shunt flow].

    Science.gov (United States)

    Togo, T; Ito, T; Hata, M; Murata, S; Osaka, K; Komatsu, T; Tabayashi, K; Haneda, K; Mohri, T

    1995-03-01

    For the systemic-pulmonary artery shunt operation, the modified Blalock-Taussig shunt was the first choice for procedure in our institution. Since 1990, Golaski knitted Dacron graft (4 or 5 mm in diameter) was used for the prosthesis. Ex-vivo flow calibration of the electromagnetic flow meter (Nihon Koden, MFV-3100) to Golaski graft showed good correlation between the real flow and value measured by the electromagnetic flow meter. Shunt flow was measured in the consecutive clinical fifteen cases. The shunt flow per body surface area of the patient who required additional shunt operation was 721 ml/min/m2 and one patient in whom the congestive heart failure developed after the shunt operation, had the shunt flow of 3,022 ml/min/m2. The adequate shunt flow in these cases was ranged from 745 to 2,820 ml/min/m2 (mean +/- 1 SD, 1,490 +/- 587.8). Therefore we performed the systemic-pulmonary artery shunt operation using Golaski graft to get the shunt flow of 1,000 ml/min/m2 (approximately a third of cardiac index) for the guide of good results. PMID:7897896

  13. 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.

  14. 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

  15. Do we need a pulmonary artery catheter in cardiac anesthesia? - An Indian perspective

    Directory of Open Access Journals (Sweden)

    Kanchi Muralidhar

    2011-01-01

    Full Text Available There has been considerable controversy regarding the use of pulmonary artery catheter (PAC in clinical practice. Some studies have indicated poor outcome in patients who were monitored with PAC. However, these studies, which have condemned the use of PAC, were conducted on patients in intensive care units, where the clinical scenarios with regard to patients′ status are somewhat different as compared to those of a cardiac operating room. This study was designed to identify the indications of PAC use in cardiac operating rooms. A questionnaire was mailed to anasthesiologists in cardiac centers and the response was analyzed.The practicing cardiac anesthesiologists recommended the use of PAC for following indications in cardiac surgery: coronary artery bypass grafting (CABG with poor left ventricular (LV function, LV aneurysmectomy, recent myocardial infarction (MI, pulmonary hypertension, diastolic dysfunction, acute ventricular septal rupture and insertion of left ventricular assist device (LVAD.The analysis of responses from practicing anesthesiologists clearly indicates that use of a PAC cannot be recommended as a matter of routine, but a definite role is suggested in selected groups of patients undergoing cardiac surgery.

  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. 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

  18. 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

  19. 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.

  20. Hypoxia Inducible Factor-1 Alpha Correlates the Expression ofHeme Oxygenase 1 Gene in Pulmonary Arteries of Ratwith Hypoxia-induced Pulmonary Hypertension

    Institute of Scientific and Technical Information of China (English)

    Qi-FangLI; Ai-GuoDAI

    2004-01-01

    AbstractTo test the hypothesis that hypoxia inducible factor-1 alpha (HIF-1α)up-regulated theexpression of heme oxygenase-1 (HO-1) gene in pulmonary arteries of rats with hypoxia-induced pulmonaryhypertension, 8 male Wistar rats in each of 5 groups were exposed to hypoxia for 0, 3, 7, 14 or 21 d, respectively.Mean pulmonary arterial pressure (mPAP), vessel morphometry and right ventricle hypertrophy index weremeasured. Lungs were inflation fixed for immunohistochemistry, in situ hybridization; frozen for latermeasurement of HO-1 enzyme activity, mPAP increased significantly after 7 d of hypoxia [(18.4 ± 0.4)mmHg, P<0.05], reaching its peak after 14 d of hypoxia, then remained stable. Pulmonary artery remodeling became to develop significantly after 14 d of hypoxia. HIF-1αprotein in control was poorly positive (0.05 ±0.01), but was up-regulated in pulmonary arterial tunica intima of all hypoxic rats. In pulmonary arterialtunica media, the levels of HIF-la protein were markedly up-regulated after 3 d and 7 d of hypoxia(0.20±0.02; 0.22 ± 0.02, P<0.05), then declined after 14 d and 21 d of hypoxia. HIF-mRNA stainingwas poorly positive in control, hypoxia for 3 and 7 d, but enhanced significantly after 14 d of hypoxia(0.20±0.02, P<0.05), then remained stable. HO-1 protein increased after 7 d of hypoxia (0.10±0.01,P<0.05), reaching its peak after 14 d of hypoxia (0.21 0.02, P<0.05), then remained stable. HO-1 mRNA increased after 3 d of hypoxia, reaching its peak after 7 d of hypoxia (0.17 ± 0.01, P<0.05), then declined.Linear correlation analysis showed that HIF-lα mRNA, HO-1 protein and mPAP were associatedwith pulmonary remodeling. HIF-1 α protein (tunica intima) was conversely correlated with HIF-1α mRNA(r=0.921, P<0.01), HO-1 protein was conversely correlated with HIF-1α protein (tunica intima)(r=0.821, P<0.01 ). HIF-1αand HO-1 were both involved in the pathogenesis of hypoxia-induced pulmonaryhypertension in rat. Hypoxia inducible factor-1 alpha

  1. Hypoxia Inducible Factor-1 Alpha Correlates the Expression of Heme Oxygenase 1 Gene in Pulmonary Arteries of Rat with Hypoxia-induced Pulmonary Hypertension

    Institute of Scientific and Technical Information of China (English)

    Qi-Fang LI; Ai-Guo DAI

    2004-01-01

    To test the hypothesis that hypoxia inducible factor-1 alph (HIF-la) up-regulated theexpression ofheme oxygenase-1(HO-1) gene in pulmonary arteries of rats with hypoxia-induced pulmonaryhypertension, 8 male Wistar rats in each of 5 groups were exposed to hypoxia for 0, 3, 7, 14 or 21d, respectively.Mean pulmonary arterial pressure(mPAP), vessel morphometry and right ventricle hypertrophy index weremeasured. Lungs were inflation fixed for immunohistochemistry, in situ hybridization; frozen for latermeasurement of HO-1 enzyme activity, mPAP increased significantly after 7d of hypoxia [(18.4±0.4)mmHg, P<0.05], reaching its peak after 14d of hypoxia, then remained stable. Pulmonary artery remodelingbecame to develop significantly after 14d ofhypoxia. H1F-1α protein in control was poorly positive (0.05±0.01), but was up-regulated in pulmonary arterial tunica intima of all hypoxic rats. In pulmonary arterialtunica media, the levels of HIF-lα protein were markedly up-regulated after 3d and 7d of hypoxia(0.20±0.02; 0.22±0.02, P<0.05), then declined after 14d and 21d of hypoxia. HIF-1a mRNA stainingwas poorly positive in control, hypoxia for 3 and 7d, but enhanced significantly after 14d of hypoxia(0.20±0.02, P<0.05), then remained stable. HO-1 protein increased after 7d of hypoxia (0.10±0.01,P<0.05), reaching its peak after 14d ofhypoxia (0.21±0.02, P<0.05), then remained stable. HO-1 mRNAincreased after 3d of hypoxia, reaching its peak after 7d of hypoxia (0.17±0.01, P<0.05), then declined.Linear correlation analysis showed that HIF-la mRNA, HO-1 protein and mPAP were associatedwith pulmonary remodeling. HIF-1α protein (tunica intima) was conversely correlated with HIF-1a mRNA(r=0.921, P<0.01), HO-1 protein was conversely correlated with HIF-lα protein (tunica intima)(r=0.821, P<0.01). HIF-1α and HO-1 were both involved in the pathogenesis of hypoxia-induced pulmonaryhypertension in rat. Hypoxia inducible factor-1 alpha correlated the

  2. Comparative Transcriptome Analysis Identifies CCDC80 as a Novel Gene Associated with Pulmonary Arterial Hypertension

    Science.gov (United States)

    Sasagawa, Shota; Nishimura, Yuhei; Sawada, Hirofumi; Zhang, Erquan; Okabe, Shiko; Murakami, Soichiro; Ashikawa, Yoshifumi; Yuge, Mizuki; Kawaguchi, Koki; Kawase, Reiko; Mitani, Yoshihide; Maruyama, Kazuo; Tanaka, Toshio

    2016-01-01

    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 two genes was significantly increased in the five datasets, whereas expression of SMAD family member six 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 examined Ccdc

  3. Hypoxia activates NADPH oxidase to increase [ROS]i and [Ca2+]i through mitochondrial ROS–PKCε signaling axis in pulmonary artery smooth muscle cells

    OpenAIRE

    Rathore, Rakesh; Zheng, Yun-Min; Niu, Chun-Feng; Liu, Qing-Hua; Korde, Amit; Ho, Ye-Shih; Wang, Yong-Xiao

    2008-01-01

    The importance of NADPH oxidase (Nox) in hypoxic responses in hypoxia-sensing cells including pulmonary artery smooth muscle cells (PASMCs) remains uncertain. In this study, using Western blot analysis we found that the major Nox subunits Nox1, Nox4, p22phox, p47phox, and p67phox were equivalently expressed in mouse pulmonary and systemic (mesenteric) arteries. However, acute hypoxia significantly increased Nox activity and translocation of p47phox protein to the plasma membrane in pulmonary,...

  4. Influence of Rho Kinase Inhibitor Fasudil on Late Endothelial Progenitor Cells in Peripheral Blood of COPD Patients with Pulmonary Artery Hypertension

    OpenAIRE

    Liu, Pei; Zhang, Hongmei; Tang, Yijun; Sheng, Chunfeng; Liu, Jianxin; Zeng, Yanjun

    2014-01-01

    The objective of our work was to investigate the influence of Fasudil, a Rho inhibitor on the number and function of the late endothelial progenitor cells in peripheral blood of chronic obstructive pulmonary diseases (COPD) patients with pulmonary artery hypertension. Eighty COPD patients with pulmonary artery hypertension were selected and divided into two groups: the treatment group and the control group, which had 40 patients respectively. The control group received routine treatment, incl...

  5. 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.

  6. In vivo and in vitro measurements of pulmonary arterial stiffness: A brief review.

    Science.gov (United States)

    Tian, Lian; Chesler, Naomi C

    2012-10-01

    During the progression of pulmonary hypertension (PH), proximal pulmonary arteries (PAs) undergo remodeling such that they become thicker and the elastic modulus increases. Both of these changes increase the vascular stiffness. The increase in pulmonary vascular stiffness contributes to increased right ventricular (RV) afterload, which causes RV hypertrophy and eventually failure. Studies have found that proximal PA stiffness or its inverse, compliance, is strongly related to morbidity and mortality in patients with PH. Therefore, accurate in vivo measurement of PA stiffness is useful for prognoses in patients with PH. It is also important to understand the structural changes in PAs that occur with PH that are responsible for stiffening. Here, we briefly review the most common parameters used to quantify stiffness and in vivo and in vitro methods for measuring PA stiffness in human and animal models. For in vivo approaches, we review invasive and noninvasive approaches that are based on measurements of pressure and inner or outer diameter or cross-sectional area. For in vitro techniques, we review several different testing methods that mimic one, two or several aspects of physiological loading (e.g., uniaxial and biaxial testing, dynamic inflation-force testing). Many in vivo and in vitro measurement methods exist in the literature, and it is important to carefully choose an appropriate method to measure PA stiffness accurately. Therefore, advantages and disadvantages of each approach are discussed. PMID:23372936

  7. Impaired pulmonary artery contractile responses in a rat model of microgravity: role of nitric oxide

    Science.gov (United States)

    Nyhan, Daniel; Kim, Soonyul; Dunbar, Stacey; Li, Dechun; Shoukas, Artin; Berkowitz, Dan E.

    2002-01-01

    Vascular contractile hyporesponsiveness is an important mechanism underlying orthostatic intolerance after microgravity. Baroreceptor reflexes can modulate both pulmonary resistance and capacitance function and thus cardiac output. We hypothesized, therefore, that pulmonary vasoreactivity is impaired in the hindlimb-unweighted (HLU) rat model of microgravity. Pulmonary artery (PA) contractile responses to phenylephrine (PE) and U-46619 (U4) were significantly decreased in the PAs from HLU vs. control (C) animals. N(G)-nitro-L-arginine methyl ester (10(-5) M) enhanced the contractile responses in the PA rings from both C and HLU animals and completely abolished the differential responses to PE and U4 in HLU vs. C animals. Vasorelaxant responses to ACh were significantly enhanced in PA rings from HLU rats compared with C. Moreover, vasorelaxant responses to sodium nitroprusside were also significantly enhanced. Endothelial nitric oxide synthase (eNOS) and soluble guanlyl cyclase expression were significantly enhanced in PA and lung tissue from HLU rats. In marked contrast, the expression of inducible nitric oxide synthase was unchanged in lung tissue. These data support the hypothesis that vascular contractile responsiveness is attenuated in PAs from HLU rats and that this hyporesponsiveness is due at least in part to increased nitric oxide synthase activity resulting from enhanced eNOS expression. These findings may have important implications for blood volume distribution and attenuated stroke volume responses to orthostatic stress after microgravity exposure.

  8. Exercise testing in the clinical management of patients affected by pulmonary arterial hypertension.

    Science.gov (United States)

    Paolillo, Stefania; Farina, Stefania; Bussotti, Maurizio; Iorio, Annamaria; PerroneFilardi, Pasquale; Piepolil, Massimo F; Agostoni, Piergiuseppe

    2012-10-01

    Patients affected by pulmonary arterial hypertension (PAH) show a reduced exercise tolerance with early occurrence of dyspnoea and fatigue. The origin of functional capacity limitation is multifactorial and several mechanisms have been proposed, including right heart failure, which leads to a limited increase in cardiac output during exercise, and hyperventilation with a reduced perfusion of properly ventilated alveoli. In addition, abnormalities in arterial blood gases are observed, with the occurrence of hypoxemia and hypocapnia, related to an abnormal ventilation/perfusion match, gas diffusion abnormalities, low mixed venous oxygen saturation and to the development of intra- and extra-pulmonary right-to-left shunts. At present, the 6-minute walking test is the most used method to assess exercise tolerance in PAH; it is also useful to monitor the response to therapy and provides prognostic information. However, the assessment of functional capacity by cardiopulmonary exercise test (CPET) seems to be more complete, because CPET allows for discrimination between the metabolic, cardiovascular and pulmonary components of exercise limitation. Moreover, CPET estimates the severity of disease and assesses patients' prognosis and response to therapy. In PAH, a typical CPET-response is observed, characterized by a severe reduction in peak VO2, work rate, O2 pulse and anaerobic threshold and by a marked increase in VE/VCO2 slope and in the dead space to tidal volume ratio. However, the use of CPET should be limited to experienced centres. This review will focus on resting lung function and exercise tolerance tests, showing that CPET can provide the physiological explanation of functional limitation in PAH. PMID:23126000

  9. HMGB1 promotes the development of pulmonary arterial hypertension in rats.

    Directory of Open Access Journals (Sweden)

    Yukari Sadamura-Takenaka

    Full Text Available Pulmonary arterial hypertension (PAH is characterized by increased pulmonary vascular resistance leading to right ventricular failure and death. Recent studies have suggested that chronic inflammatory processes are involved in the pathogenesis of PAH. However, the molecular and cellular mechanisms driving inflammation have not been fully elucidated.To elucidate the roles of high mobility group box 1 protein (HMGB1, a ubiquitous DNA-binding protein with extracellular pro-inflammatory activity, in a rat model of PAH.Male Sprague-Dawley rats were administered monocrotaline (MCT. Concentrations of HMGB1 in bronchoalveolar lavage fluid (BALF and serum, and localization of HMGB1 in the lung were examined over time. The protective effects of anti-HMGB1 neutralizing antibody against MCT-induced PAH were tested.HMGB1 levels in BALF were elevated 1 week after MCT injection, and this elevation preceded increases of other pro-inflammatory cytokines, such as TNF-α, and the development of PAH. In contrast, serum HMGB1 levels were elevated 4 weeks after MCT injection, at which time the rats began to die. Immunohistochemical analyses indicated that HMGB1 was translocated to the extranuclear space in periarterial infiltrating cells, alveolar macrophages, and bronchial epithelial cells of MCT-injected rats. Anti-HMGB1 neutralizing antibody protected rats against MCT-induced lung inflammation, thickening of the pulmonary artery wall, and elevation of right ventricular systolic pressure, and significantly improved the survival of the MCT-induced PAH rats.Our results identify extracellular HMGB1 as a promoting factor for MCT-induced PAH. The blockade of HMGB1 activity improved survival of MCT-induced PAH rats, and thus might be a promising therapy for the treatment of PAH.

  10. Tadalafil Therapy for Pulmonary Arterial Hypertension%他达那非治疗肺动脉高压进展

    Institute of Scientific and Technical Information of China (English)

    温亮; 刘倩倩; 杨庆辉; 韦世鹏; 白晓鹏; 钟丽华; 陈延军

    2012-01-01

    Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease that is characterized by small pulmonary vascular remodeling and elevation of pulmonary vascular resistance, leading right heart failure and death. Treatment options for pulmonary arterial hypertension target the prostacyclin, endothelin, or nitric oxide pathways. Tadalafil, a phosphodiesterase type-5 inhibitor, increases cGMP, the final mediator in the nitric oxide pathway. PHIRST trial concluded that tadalafil 40 mg improved 6 minutes walk distance and reduced clinical worsening in patients with pulmonary arterial hypertension. This article reviews tadalafil therapy for pulmonary arterial hypertension.%肺动脉高压是一类以小肺动脉血管重构为特征的恶性肺血管疾病,肺血管阻力进行性升高最终导致右心衰竭死亡.肺动脉高压的药物治疗主要针对前列环素、内皮素、一氧化氮三个靶点.他达那非(Tadalafil)是一种口服、长效、选择性5-型磷酸二酯酶抑制剂,增加环磷酸鸟苷(cGMP)浓度,通过一氧化氮途径起到治疗作用.PHIRST临床试验证实肺动脉高压患者每日一次口服他达那非40mg,能够提高6分钟步行距离,减少临床恶化.本文就他达那非治疗肺动脉高压研究新进展作一综述.

  11. Hypoxic vasoconstriction of partial muscular intra-acinar pulmonary arteries in murine precision cut lung slices

    Directory of Open Access Journals (Sweden)

    Goldenberg Anna

    2006-06-01

    Full Text Available Abstract Background Acute alveolar hypoxia causes pulmonary vasoconstriction (HPV which serves to match lung perfusion to ventilation. The underlying mechanisms are not fully resolved yet. The major vascular segment contributing to HPV, the intra-acinar artery, is mostly located in that part of the lung that cannot be selectively reached by the presently available techniques, e.g. hemodynamic studies of isolated perfused lungs, recordings from dissected proximal arterial segments or analysis of subpleural vessels. The aim of the present study was to establish a model which allows the investigation of HPV and its underlying mechanisms in small intra-acinar arteries. Methods Intra-acinar arteries of the mouse lung were studied in 200 μm thick precision-cut lung slices (PCLS. The organisation of the muscle coat of these vessels was characterized by α-smooth muscle actin immunohistochemistry. Basic features of intra-acinar HPV were characterized, and then the impact of reactive oxygen species (ROS scavengers, inhibitors of the respiratory chain and Krebs cycle metabolites was analysed. Results Intra-acinar arteries are equipped with a discontinuous spiral of α-smooth muscle actin-immunoreactive cells. They exhibit a monophasic HPV (medium gassed with 1% O2 that started to fade after 40 min and was lost after 80 min. This HPV, but not vasoconstriction induced by the thromboxane analogue U46619, was effectively blocked by nitro blue tetrazolium and diphenyleniodonium, indicating the involvement of ROS and flavoproteins. Inhibition of mitochondrial complexes II (3-nitropropionic acid, thenoyltrifluoroacetone and III (antimycin A specifically interfered with HPV, whereas blockade of complex IV (sodium azide unspecifically inhibited both HPV and U46619-induced constriction. Succinate blocked HPV whereas fumarate had minor effects on vasoconstriction. Conclusion This study establishes the first model for investigation of basic characteristics of HPV

  12. Right Pulmonary Artery Distensibility Index (RPAD Index). A field study of an echocardiographic method to detect early development of pulmonary hypertension and its severity even in the absence of regurgitant jets for Doppler evaluation in heartworm-infected dogs.

    Science.gov (United States)

    Venco, Luigi; Mihaylova, Liliya; Boon, June A

    2014-11-15

    Despite the term "heartworm disease" Dirofilaria immitis infection in dogs should be considered a pulmonary arterial disease that might only involve the right heart structures in its late stage. Chronic infection by adult heartworms in dogs results in proliferative endoarteritis leading to progressively increasing pulmonary artery pressure due to reduced elasticity. Elasticity allows the pulmonary arteries to stretch in response to each pulse and helps maintain a relatively constant pressure in the arteries despite the pulsating nature of the blood flow. Pulmonary artery distensibility for both acute and chronic pulmonary hypertension has been investigated in humans using MRI and has been correlated with the severity of hypertension and its outcome and treatment response. The aim of the present study was to investigate whether echocardiographic measurement of the percentage change in diameter of the right pulmonary artery in systole and diastole (distensibility) may be of value in assessing the presence and severity of pulmonary hypertension in heartworm-infected dogs. The Right Pulmonary Artery Distensibility Index (RPAD Index) (which is calculated as the difference in diameter of the right pulmonary artery in systole and diastole) was calculated in healthy and naturally infected heartworm-positive dogs. The right pulmonary artery was chosen because it is usually affected earlier and to a greater degree. Data were obtained from healthy heartworm-free dogs without any clinical, radiographic, or echocardiographic signs of pulmonary hypertension; naturally infected heartworm-positive dogs in different stages of the disease in which pulmonary pressure could be measured by Doppler echocardiography (using tricuspid and or pulmonary regurgitation velocity and pressure gradient); and naturally infected heartworm-positive dogs in different stages of the disease (with or without tricuspid and or pulmonary regurgitation) in which the pulmonary pressure was measured

  13. Ginsenoside Rb1 Attenuates Agonist-Induced Contractile Response via Inhibition of Store-Operated Calcium Entry in Pulmonary Arteries of Normal and Pulmonary Hypertensive Rats

    Directory of Open Access Journals (Sweden)

    Rui-Xing Wang

    2015-03-01

    Full Text Available Background: Pulmonary hypertension (PH is characterized by sustained vasoconstriction, enhanced vasoreactivity and vascular remodeling, which leads to right heart failure and death. Despite several treatments are available, many forms of PH are still incurable. Ginsenoside Rb1, a principle active ingredient of Panax ginseng, exhibits multiple pharmacological effects on cardiovascular system, and suppresses monocrotaline (MCT-induced right heart hypertrophy. However, its effect on the pulmonary vascular functions related to PH is unknown. Methods: We examined the vasorelaxing effects of ginsenoside Rb1 on endothelin-1 (ET-1 induced contraction of pulmonary arteries (PAs and store-operated Ca2+ entry (SOCE in pulmonary arterial smooth muscle cells (PASMCs from chronic hypoxia (CH and MCT-induced PH. Results: Ginsenoside Rb1 elicited concentration-dependent relaxation of ET-1-induced PA contraction. The vasorelaxing effect was unaffected by nifedipine, but abolished by the SOCE blocker Gd3+. Ginsenoside Rb1 suppressed cyclopiazonic acid (CPA-induced PA contraction, and CPA-activated cation entry and Ca2+ transient in PASMCs. ET-1 and CPA-induced contraction, and CPA-activated cation entry and Ca2+ transients were enhanced in PA and PASMCs of CH and MCT-treated rats; the enhanced responses were abolished by ginsenoside Rb1. Conclusion: Ginsenoside Rb1 attenuates ET-1-induced contractile response via inhibition of SOCE, and it can effectively antagonize the enhanced pulmonary vasoreactivity in PH.

  14. A Behcet’s Disease Patient with Right Ventricular Thrombus, Pulmonary Artery Aneurysms, and Deep Vein Thrombosis Complicating Recurrent Pulmonary Thromboembolism

    Directory of Open Access Journals (Sweden)

    Selvi Aşker

    2013-01-01

    Full Text Available Intracardiac thrombus, pulmonary artery aneurysms, deep vein thrombosis, and pulmonary thromboembolism are rarely seen symptoms of Behcet’s disease. A 20-year-old female patient was admitted for complaints of cough, fever, palpitations, and chest pain. On the dynamic thorax computed tomograms (CT obtained because of significantly enlarged hilar structures seen on chest radiograms, aneurysmal dilatation of the pulmonary artery segments bilaterally, chronic thrombus with collapse, and consolidation substances compatible with pulmonary embolism involving both lower lobes have been observed. It is learned that, four years ago, the patient had been diagnosed with Behcet’s disease and received colchicine treatment but not regularly. The patient was hospitalized. On the transthoracic echocardiogram, a thrombosis with a dimension of 4.2 × 1.6 cm was recognized in the right ventricle. On abdomen CT, aneurysmal iliac veins and deep vein thrombus on Doppler ultrasonograms were diagnosed. At the controls after three months of immunosuppressive and anticoagulant therapies, some clinical and radiological improvements were recognized. The patient suspended the treatment for a month and the thrombus recurred. We present our case in order to show the effectiveness of immunosuppressive and anticoagulant therapies and rarely seen pulmonary thromboembolism in recurrent Behcet’s disease.

  15. Color Doppler evaluation of the ocular arterial flow changes in chronic obstructive pulmonary disease

    Energy Technology Data Exchange (ETDEWEB)

    Ozer, Tulay [Department of Radiology, Karaelmas University, School of Medicine, Tip Fakueltesi, Radyoloji AD, 67600-Kozlu, Zonguldak (Turkey)]. E-mail: ozertulay@yahoo.com; Altin, Remzi [Department of Pulmonary Medicine, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey); Ugurbas, Suat Hayri [Department of Ophthalmology, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey); Ozer, Yetkin [Department of Anesthesiology and Reanimation, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey); Mahmutyazicioglu, Kamran [Department of Radiology, Karaelmas University, School of Medicine, Tip Fakueltesi, Radyoloji AD, 67600-Kozlu, Zonguldak (Turkey); Kart, Levent [Department of Pulmonary Medicine, Karaelmas University, School of Medicine, Kozlu, Zonguldak (Turkey)

    2006-01-15

    Purpose: To evaluate the hemodynamic changes in the extraocular orbital vessels of the patients with chronic obstructive pulmonary disease (COPD), using color Doppler ultrasonography (CDU) technique, and to compare the results with those of healthy control subjects. Methods: Forty-five patients with COPD and 17 healthy control subjects were included in this study. Patients with COPD were classified according to the Global Initiative for Chronic Obstructive Lung Disease (GOLD). Fifteen patients of stage I COPD (mild airflow limitation), stage II COPD (worsening airflow limitation) or stage III COPD (severe airflow limitation) were enrolled into Group I, II and III, respectively. End tidal carbon dioxide (EtCO{sub 2}), peripheral oxygen saturation (SpO{sub 2}), pulse rate (PR) and respiratory rate (RR) were measured by using capnograph/pulse oximeter in all patients. Measurements were performed in only one randomly chosen eye of each participant. The peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistance index (RI) were measured in the ophthalmic artery (OA), central retinal artery (CRA), lateral short posterior ciliary artery (LPCA) and medial short posterior ciliary artery (MPCA), using CDU technique. Results: The PSV measurements of the OA were significantly higher in Groups II and III compared to control group (p < 0.01, p < 0.001, respectively). The RI values from OA and CRA were significantly higher in Group II and III than the control group (p < 0.05). The RI values of LPCA and MPCA were also significantly higher in Group II than the control subjects (p < 0.05). When RI values were compared, mean values of LPCA and MPCA were significantly lower in Group III than in Group II (p < 0.05). There were no significant differences between Group I and control patients about PSVs, EDVs and RI values of all arteries Statistically significant correlations were found for the EtCO{sub 2} with PSV (r = 0.53, p < 0.01) and EDV (r = 0.51, p < 0.01) of the

  16. Cardiopulmonary exercise testing reveals onset of disease and response to treatment in a case of heritable pulmonary arterial hypertension

    Science.gov (United States)

    Trip, Pia; Vonk-Noordegraaf, Anton; Bogaard, Harm Jan

    2012-01-01

    Patients affected by pulmonary arterial hypertension (PAH) show a typical pattern of abnormalities on cardiopulmonary exercise testing (CPET). However, CPET is not routinely used as a screening method. We discuss a patient with hereditary PAH in whom CPET revealed onset of disease. Furthermore, we show that the abnormalities observed can improve in part by PAH-specific treatment. PMID:23130108

  17. Roles of NHE-1 in the proliferation and apoptosis of pulmonary artery smooth muscle cells in rats

    Institute of Scientific and Technical Information of China (English)

    姚伟; 钱桂生; 杨晓静

    2002-01-01

    Objective To evaluate the roles of Na+/H+ exchanger-1 (NHE-1)in the proliferation and apoptosis of pulmonary artery smooth muscle cells in rats. Methods Twenty Wistar rats were randomized into control group and 3-week hypoxic group. Intracellular pH (pHi) of the smooth muscle was determined with fluorescence measurement of the pH-sensitive dye BCECF-AM, and the expression of NHE-1 mRNA was detected by reverse transcription polymerase chain reaction (RT-PCR). Primary culture of pulmonary artery smooth muscle cells in vitro was performed. In situ cell death detection kit (TUNEL) was used for studying the effect of specific NHE-1 inhibitor-dimethyl amiloride (DMA) on the apoptosis of muscle cells which had intracellular acidification. Results pHi value and NHE-1 mRNA expression of pulmonary artery smooth muscle cells were significantly higher in the hypoxic group than in the control group (P<0.01, P<0.001). DMA elevated the apoptotic ratio remarkably. The effect was enhanced when DMA concentration increased and the time prolonged. Conclusions With the function of adjusting pHi, NHE-1 may play an important role in the proliferation and apoptosis of pulmonary artery smooth muscle cells.

  18. Unilateral pulmonary artery stenosis and late-onset cataract in an adult: a case of suspected congenital rubella syndrome

    Institute of Scientific and Technical Information of China (English)

    LIU Yang; GUO Jun; ZHAO Rui-fu; WANG Lin

    2012-01-01

    Congenital rubella syndrome (CRS) is characterized by the triad of deafness,cataract and cardiovascular malformations.1 The great majority of the cases in the literature have been usually diagnosed in infancy and childhood because of various defects at birth.However,we report a rare case of suspected CRS in an adult with unilateral pulmonary artery stenosis and late-onset cataract.

  19. Mediastinal fibrosis with pulmonary artery obstruction; diagnosis and investigation with helical CT imaging including 3-dimensional reconstructions

    International Nuclear Information System (INIS)

    An aggressive mediastinal fibrosis was found in a 42-year-old female, suffering from dysphagia, stabbing pain in the chest, and an unclear weight loss. In this case, the rare combination of esophageal involvement, bronchial narrowing, and pulmonary artery obstruction could easily be demonstrated with a barium study and a helical CT examination including three-dimensional reconstructions. (orig.)

  20. Non heamoptytic massive Rasmussen's pulmonary artery aneurysm caused by aggressive cavitating squamous cell carcinoma metastasis

    International Nuclear Information System (INIS)

    The authors report an extremely rare occurrence of a massive aneurysm of a major pulmonary artery branch vessel caused by adjacent necrotizing aggressive squamous cell carcinoma metastatic mediastinal nodes. Despite the huge size, there was no hemoptysis due to the walling off effect by the necrotic nodes