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Pulmonary arterial hypertension

Christina Binder, Caroline Zotter-Tufaro, Diana Bonderman
The development of pulmonary hypertension (PH) has multifactorial underlying pathophysiological causes and can be classified into five groups. While three different classes of therapeutic drugs are licensed for the treatment of pulmonary arterial hypertension (PAH, WHO group 1), specific medical therapies are lacking for other forms of PH, such as PH due to left heart disease. In 2013 riociguat, a first-in class soluble guanylate cyclase stimulator, has also become available for the treatment of PAH. Riociguat was further introduced as the first approved pharmacotherapy for the treatment of patients with chronic thromboembolic PH (WHO group 4, CTEPH)...
October 18, 2016: Expert Opinion on Drug Safety
Ying Li, Bo Shi, Liping Huang, Xin Wang, Xiaona Yu, Baosheng Guo, Weidong Ren
Hypoxia-inducible factor-1α (HIF-1α) has been implicated in the pathogenesis of hypoxic pulmonary hypertension (PH). However, the potential clinical value of HIF-1α as a therapeutic target in the treatment of PH has not yet been evaluated. In this study, an animal model of hypoxia-induced PH was established by exposing adult rats to 10% O2 for 3 weeks, and the effects of the lentivirus-mediated delivery of HIF-1α short hairpin RNA (shRNA) by intratracheal instillation prior to exposure to hypoxia on the manifestations of hypoxia-induced PH were assessed...
October 14, 2016: International Journal of Molecular Medicine
Anikó Ilona Nagy, Ashwin Venkateshvaran, Béla Merkely, Lars H Lund, Aristomenis Manouras
AIMS: The diastolic pulmonary pressure gradient (DPG) has recently been introduced as a specific marker of combined pre-capillary pulmonary hypertension (Cpc-PH) in left heart disease (LHD). However, its diagnostic and prognostic superiority compared with traditional haemodynamic indices has been challenged lately. Current recommendations explicitly denote that in the normal heart, DPG values are greater than zero, with DPG ≥7 mmHg indicating Cpc-PH. However, clinicians are perplexed by the frequent observation of DPG <0 mmHg (DPGNEG ), as its physiological explanation and clinical impact are unclear to date...
October 17, 2016: European Journal of Heart Failure
E Mazzotta, C Guglielmini, G Menciotti, B Contiero, M BaronToaldo, M Berlanda, H Poser
BACKGROUND: Red blood cell distribution width (RDW) is a quantitative measurement of anisocytosis. RDW has prognostic value in humans with different cardiovascular and systemic disorders, but few studies have investigated this biomarker in dogs. OBJECTIVES: To compare the RDW in dogs with precapillary and postcapillary pulmonary hypertension (PH) and a control population of dogs and to correlate RDW with demographic, echocardiographic, and laboratory variables. ANIMALS: One hundred and twenty-seven client-owned dogs including 19 healthy dogs, 82 dogs with myxomatous mitral valve disease (50 dogs without PH and 32 dogs with postcapillary PH), and 26 dogs with precapillary PH...
October 17, 2016: Journal of Veterinary Internal Medicine
Zhongmin Wang, Yuhao Liu, Yu Xu, Chuanyu Gao, Yan Chen, Hongxing Luo
BACKGROUND: WE AIMED TO PROSPECTIVELY EVALUATE THE: use of 3-dimensional printing (3DP) for the percutaneous transcatheter closure of a secundum atrial septal defect (ASD) with rim deficiency less than 5 mm. METHODS: Patients with ASD were scanned using multi-slice computed tomography to acquire raw data for virtual 3DP reconstruction models. Different ASD occluders were tried on the 3DP models to select the optimal size for intraoperative use. The patients were followed up 1 month postoperatively, and 3DP models were again manufactured to observe the operative effects...
October 17, 2016: Cardiology Journal
Ioana R Preston, Harrison W Farber
No abstract text is available yet for this article.
September 2016: Journal of Thoracic Disease
Mina Akizuki, Naoki Serizawa, Atsuko Ueno, Taku Adachi, Nobuhisa Hagiwara
RATIONALE: Balloon pulmonary angioplasty (BPA) in chronic thromboembolic pulmonary hypertension (CTEPH) improves hemodynamics and exercise capacity. However, its effect on respiratory function is unclear. OBJECTIVES: To investigate the effect of BPA on respiratory function. METHODS: We enrolled inoperable CTEPH who underwent BPA primarily in lower lobe arteries (first series) and upper and middle lobe arteries (second series). We compared changes in hemodynamics and respiratory function between different BPA fields...
October 13, 2016: Chest
Ahmed M Selim, Nitasha Sarswat, Iosif Kelesidis, Muhammad Iqbal, Ramesh Chandra, Ronald Zolty
BACKGROUND: The relationship between heart failure (HF) and the serotonergic system has been established in animal studies. However, data on human plasma serotonin level in HF and its significance over the course of the disease is lacking. METHODS: Serotonin levels were measured in 173 patients (108 males, 65 females), 116 were stable HF and 40 were acute decompensated HF patients. The normal control group included 17 healthy volunteers with no known medical or psychiatric conditions...
September 17, 2016: Heart, Lung & Circulation
Marius M Hoeper, Vallerie V McLaughlin, Joan Albert Barberá, Adaani E Frost, Hossein-Ardeschir Ghofrani, Andrew J Peacock, Gérald Simonneau, Stephan Rosenkranz, Ronald J Oudiz, R James White, Karen L Miller, Jonathan Langley, Julia H N Harris, Christiana Blair, Lewis J Rubin, Jean-Luc Vachiery
BACKGROUND: In treatment-naive patients with pulmonary arterial hypertension, initial combination therapy with ambrisentan and tadalafil reduces the risk of clinical failure events compared with monotherapy. We did this secondary analysis to further investigate the effect of combination therapy on survival. METHODS: We analysed survival data from the modified intention-to-treat population of the Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension (AMBITION) trial...
October 10, 2016: Lancet Respiratory Medicine
Martin A Alpert, Jad Omran, Brian P Bostick
Obesity produces a variety of hemodynamic alterations that may cause changes in cardiac morphology which predispose to left and right ventricular dysfunction. Various neurohormonal and metabolic alterations commonly associated with obesity may contribute to these abnormalities of cardiac structure and function. These changes in cardiovascular hemodynamics, cardiac morphology, and ventricular function may, in severely obese patients, predispose to heart failure, even in the absence of other forms of heart disease (obesity cardiomyopathy)...
October 15, 2016: Current Obesity Reports
Amelia Chiara Trombetta, Carmen Pizzorni, Barbara Ruaro, Sabrina Paolino, Alberto Sulli, Vanessa Smith, Maurizio Cutolo
OBJECTIVE: To quantify in patients with systemic sclerosis (SSc) the absolute nailfold capillary number/mm (the absolute number of capillaries, observable in the first row, in 1 mm per field) and fingertip blood perfusion (FBP) during longterm therapy with the endothelin receptor antagonist bosentan (BOSE) and the synthetic analog of prostacyclin PGI2 iloprost (ILO) by multiple diagnostic tools. Observed values were correlated with clinical outcomes. METHODS: Thirty patients with SSc already receiving intravenous ILO (80 μg/day) for 5 continuous days (every 3 mos) were recruited in the clinic...
October 1, 2016: Journal of Rheumatology
Natasha M Rogers, Maryam Sharifi-Sanjani, Mingyi Yao, Kedar Ghimire, Raquel Bienes-Martinez, Stephanie M Mutchler, Heather E Knupp, Jeffrey Baust, Enrico M Novelli, Mark Ross, Claudette St Croix, Johannes C Kutten, Caitlin A Czajka, John C Sembrat, Mauricio Rojas, David Labrousse-Arias, Timothy N Bachman, Rebecca R Vanderpool, Brian S Zuckerbraun, Hunter C Champion, Ana L Mora, Adam C Straub, Richard A Bilonick, Maria J Calzada, Jeffrey S Isenberg
AIMS: Thrombospondin-1 (TSP1) is a ligand for CD47 and TSP1(-/-) mice are protected from pulmonary hypertension (PH). We hypothesized the TSP1-CD47 axis is upregulated in human PH and promotes pulmonary arterial vasculopathy. METHODS AND RESULTS: We analyzed the molecular signature and functional response of lung tissue and distal pulmonary arteries (PAs) from individuals with (n=23) and without (n=16) PH. Compared to controls, lungs and distal PAs from PH patients showed induction of TSP1-CD47 and endothelin-1/endothelin A receptor (ET-1/ETA) protein and mRNA...
October 13, 2016: Cardiovascular Research
Marlieke L A Haeck, Gijsbert F L Kapel, Roderick W C Scherptong, Cees A Swenne, Arie C Maan, Jeroen J Bax, Martin J Schalij, Hubert W Vliegen
BACKGROUND: The aim was to assess the diagnostic value of the Inverse Dower (INVD)-derived vectorcardiogram (VCG) and the Kors-derived VCG to detect elevated systolic pulmonary artery pressure (SPAP) in suspected pulmonary hypertension (PH). METHODS: In 132 patients, morphologic variables were evaluated by comparing the VCG parameters synthesized by INVD and Kors matrix. Comparison of the diagnostic accuracy of detecting SPAP ≥50mmHg between the matrices was performed by ROC curve analysis and logistic regression analysis...
September 8, 2016: Journal of Electrocardiology
Luigi Gabrielli, María Paz Ocaranza, Marta Sitges, Andrés Kanacri, Rodrigo Saavedra, Pablo Sepulveda, Luis Sepulveda, Victor Rossel, Monica Zagolin, Hugo E Verdejo, Fernando Baraona, Ricardo Zalaquett, Mario Chiong, Sergio Lavandero, Pablo F Castro
BACKGROUND: Right atrium function and ventricular function have significant prognostic value in pulmonary arterial hypertension patients. Acute changes in right ventricular synchrony and right atrium function postiloprost inhalation have not been evaluated. METHODS: Cross-sectional study. Consecutive pulmonary arterial hypertension patients (group I from Nice classification) were included. Echocardiographic right atrium and right ventricular function pre- and postiloprost inhalation, including a right ventricular dyssynchrony index and right atrium function using speckle tracking, were performed in all patients...
October 14, 2016: Echocardiography
Yong Wang, Ting-Ting Ma, Na-Na Gao, Xiao-Ling Zhou, Jiang Hong, Rui Guo, Li-Na Jia, Hong Chang, Ying Gao, Zhi-Min Gao, Lei Pan
ETHNOPHARMACOLOGICAL RELEVANCE: Tongxinluo (TXL),which is a Chinese medicine rooted from traditional used herbs, has been used in clinic to treat cardiovascular and cerebrovascular diseases. However, it remains unknown whether TXL alleviates low pressure hypoxic pulmonary hypertension. AIM OF THE STUDY: Here, we aimed to observe the influence of TXL on pulmonary hypertension in a rat model that exposed to high altitude environment characterized by low pressure hypoxia...
October 10, 2016: Journal of Ethnopharmacology
Masaru Obokata, Yasufumi Nagata, Yuichiro Kado, Masahiko Kurabayashi, Yutaka Otsuji, Masaaki Takeuchi
BACKGROUND: Exercise-induced pulmonary hypertension (EIPH) may develop even at low workloads in heart failure (HF) patients. Ventricular-arterial stiffening plays an important role in the pathophysiology of HF with preserved ejection fraction (HFpEF). This study aimed to compare the response of ventricular-arterial coupling and PH during low-level exercise between HFpEF and HF with reduced EF (HFrEF). METHODS AND RESULTS: Echocardiography was performed at rest and during 10 W of bicycle exercise in HFpEF (n=37) and HFrEF (n=43)...
October 10, 2016: Journal of Cardiac Failure
I E Chazova, T V Martynyuk
Chronic thromboembolic pulmonary hypertension (CTEPH) is precapillary pulmonary hypertension, in which chronic obstruction of large and middle branches of pulmonary arteries (PAs) and secondary changes in the lung microcirculatory bed result in a progressive increase in pulmonary vascular resistance and PA pressure with the development of severe right cardiac dysfunction and heart failure. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The diagnostic criteria for CTEPH are a mean PA pressure of ≥25 mm Hg, as evidenced by right heart catheterization; a PA wedge pressure of ≤15 mm Hg; a pulmonary vascular resistance of >2 Wood units; the presence of chronic/organized thrombi/emboli in the elastic PAs (pulmonary trunk, lobular, segmental, subsegmental PAs); effective anticoagulant therapy at therapeutic dosages over at least 3 months...
2016: Terapevticheskiĭ Arkhiv
V A Nevzorova, E A Kochetkova, L G Ugai, Yu V Maistrovskaya, E A Khludeeva
AIM: To define the role of circulating biomarkers for the metabolism of collagen and intercellular substance and vascular remodeling in the development of osteoporosis (OP) in idiopathic pulmonary arterial hypertension (IPAH). MATERIAL AND METHODS: Functional hemodynamic parameters, bone mineral density (BMD) in the lumbar spine and femoral neck and the serum levels of matrix metalloproteinase-9 (MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1), MMP-9/TIMP-1 complex, C-terminal telopeptide of collagen type 1 (CITP), and endothelin-1 (ET-1) were determined in 27 high-risk IPAH patients and 30 healthy volunteers...
2016: Terapevticheskiĭ Arkhiv
Waleed Ahmed Salah Eldeen Hassan, Eman Abd Elaleem Baraka, Basant Mohammed Elnady, Tahany Mahmoud Gouda, Nehad Fouad
OBJECTIVE: Cluster of differentiation 163 (CD163) is a receptor that binds haptoglobin-hemoglobin complexes and is mainly expressed on macrophages and monocytes. As a result of shedding, the extracellular portion of CD163 circulates in the blood as a soluble CD163 (sCD163). This study aimed to measure serum sCD163 levels in patients with systemic sclerosis (SSc) and to assess its association with the clinical, laboratory, and radiological features of the disease. MATERIAL AND METHODS: We measured serum sCD163 levels in 24 patients with SSc and in 30 healthy controls...
September 2016: Eur J Rheumatol
Soo Han Kim, Jae Won Lee, Jung-Min Ahn, Dae-Hee Kim, Jong-Min Song, Sang-Do Lee, Jae Seung Lee
Background/Aims: Pulmonary endarterectomy (PEA) is the gold standard for treating chronic thromboembolic pulmonary hypertension (CTEPH) in Western countries. The aim of this study was to investigate the long-term outcomes of performing PEA on CTEPH patients in comparison with medical therapy at a single Korean center. Methods: This retrospective study included 88 CTEPH patients. These patients were classified into the PEA group (n = 37) or non-PEA group (i.e., medical therapy; n = 51)...
October 13, 2016: Korean Journal of Internal Medicine
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