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Chronic thromboembolic pulmonary hypertension

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https://www.readbyqxmd.com/read/28919201/macitentan-for-the-treatment-of-inoperable-chronic-thromboembolic-pulmonary-hypertension-merit-1-results-from-the-multicentre-phase-2-randomised-double-blind-placebo-controlled-study
#1
Hossein-Ardeschir Ghofrani, Gérald Simonneau, Andrea M D'Armini, Peter Fedullo, Luke S Howard, Xavier Jaïs, David P Jenkins, Zhi-Cheng Jing, Michael M Madani, Nicolas Martin, Eckhard Mayer, Kelly Papadakis, Dominik Richard, Nick H Kim
BACKGROUND: Macitentan is beneficial for long-term treatment of pulmonary arterial hypertension. The microvasculopathy of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension are similar. METHODS: The phase 2, double-blind, randomised, placebo-controlled MERIT-1 trial assessed macitentan in 80 patients with CTEPH adjudicated as inoperable. Patients identified as WHO functional class II-IV with a pulmonary vascular resistance (PVR) of at least 400 dyn·s/cm(5) and a walk distance of 150-450 m in 6 min were randomly assigned (1:1), via an interactive voice/web response system, to receive oral macitentan (10 mg once a day) or placebo...
September 8, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28918335/modern-diagnosis-of-chronic-thromboembolic-pulmonary-hypertension
#2
Aileen Kharat, Anne-Lise Hachulla, Stéphane Noble, Frédéric Lador
Chronic thromboembolic pulmonary hypertension (CTEPH) should be suspected in patients presenting persistent dyspnea three months after a pulmonary embolism or in patients presenting with acute pulmonary embolism and suggestive images on the CT-scan. For these patients, a specific diagnostic work-up should be performed. First step consists of the ventilation/perfusion (V/Q) scan which is a good screening test due to its high sensitivity and high negative predictive value. Pulmonary angiography remains the gold standard approach for the confirmation of the diagnosis and pre-surgical evaluation of CTEPH...
September 9, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28904974/microarray-analysis-and-detection-of-micrornas-associated-with-chronic-thromboembolic-pulmonary-hypertension
#3
Ran Miao, Ying Wang, Jun Wan, Dong Leng, Juanni Gong, Jifeng Li, Yunxia Zhang, Wenyi Pang, Zhenguo Zhai, Yuanhua Yang
The aim of this study was to understand the importance of chronic thromboembolic pulmonary hypertension- (CTEPH-) associated microRNAs (miRNAs). miRNAs differentially expressed in CTEPH samples compared with control samples were identified, and the target genes were predicted. The target genes of the key differentially expressed miRNAs were analyzed, and functional enrichment analyses were carried out. Finally, the miRNAs were detected using RT-PCR. Among the downregulated miRNAs, MiR-3148 regulated the most target genes and was significantly enriched in pathways in cancer, glioma, and ErbB signaling pathway...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28892403/plexiform-vasculopathy-in-chronic-thromboembolic-pulmonary-hypertension
#4
Maximilian Ackermann, Andreas Gaumann, Steven J Mentzer, Jan B Hinrichs, Gregor Warnecke, Marius M Hoeper, Peter Braubach, Mark Kuehnel, Lavinia Maegel, Danny Jonigk
No abstract text is available yet for this article.
September 11, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28875371/the-effective-systematic-heparin-pre-treatment-on-thrombus-formation-on-pulmonary-artery-catheter-tips-during-pulmonary-endarterectomy-for-chronic-thromboembolic-pulmonary-hypertension-a-randomized-double-blind-study
#5
Christoph B Wiedenroth, Christoph Liebetrau, Henning Gall, Thomas Risch, Matthias Arlt, Eckhard Mayer, Stefan Guth
Pulmonary artery (PA) catheters are routinely used for hemodynamic management in patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA). Tip-associated thrombi are frequently detected and might increase the peri-operative risk in these patients. The aim of the study was to investigate the effects of low-dose heparinization before the insertion of the PA catheter on thrombus formation and thrombus weight during PEA surgery. From September 2013 to February 2015, 60 CTEPH patients undergoing PEA were included in the study and randomized into two groups of 30 patients each, including a heparin group (heparin bolus (70 IU per kg body weight) administration before PA catheter insertion) and a control group (pretreatment with placebo)...
September 5, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28870062/initial-clinical-and-hemodynamic-results-of-a-regional-pulmonary-thromboendarterectomy-program
#6
Farhan Raza, Anjali Vaidya, Anne-Sophie Lacharite-Roberge, Vladimir Lakhter, Hayan Al-Maluli, Irfan Ahsan, Pamela Boodram, Chandra Dass, Frances Rogers, Martin G Keane, Sheila Weaver, Riyaz Bashir, Yoshiya Toyoda, Paul Forfia
BACKGROUND: Pulmonary thromboendarterectomy (PTE) is the treatment of choice for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, access to CTEPH and PTE care is limited. There is a paucity of published data on PTE efficacy and outcomes from alternative, regional centers of excellence in CTEPH and PTE care in the United States, outside a single national and international referral center. METHODS: We performed a retrospective review of patients undergoing PTE at our institution from June 2013 to December 2016 (42 months), and collected clinical, echocardiographic and hemodynamic data on our patients pre- and post-PTE (n=71)...
September 4, 2017: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28856715/feasibility-of-quantitative-regional-ventilation-and-perfusion-mapping-with-phase-resolved-functional-lung-preful-mri-in-healthy-volunteers-and-copd-cteph-and-cf-patients
#7
Andreas Voskrebenzev, Marcel Gutberlet, Filip Klimeš, Till F Kaireit, Christian Schönfeld, Alexander Rotärmel, Frank Wacker, Jens Vogel-Claussen
PURPOSE: In this feasibility study, a phase-resolved functional lung imaging postprocessing method for extraction of dynamic perfusion and ventilation parameters using a conventional 1H lung MRI Fourier decomposition acquisition is introduced. METHODS: Time series of coronal gradient-echo MR images with a temporal resolution of 288 to 324 ms of two healthy volunteers, one patient with chronic thromboembolic hypertension, one patient with cystic fibrosis, and one patient with chronic obstructive pulmonary disease were acquired at 1...
August 30, 2017: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/28845873/-late-complications-of-fontan-operation
#8
Alessandro Frigiola, Mauro Lo Rito
Complex univentricular heart defects are treated with the Fontan operation that consists in the diversion of the systemic venous return to the pulmonary arteries. Currently, the operation is achieved through a staged approach and with the use of prosthetic extracardiac conduits to connect the inferior vena cava to the pulmonary arteries. The improvement of early and late survival allowed these patients to reach adulthood, although long-term complications remain a relevant problem. Most of the complications are caused by chronic systemic venous hypertension due to the lack of a pumping sub-pulmonary ventricle...
September 2017: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/28843647/-non-surgical-chronic-thromboembolic-pulmonary-hypertension-in-a-subject-with-antiphospholipid-syndrome
#9
Luis Efren Santos-Martínez, Nielzer Armando Rodríguez-Almendros, César Antonio Flores-García, Patricia Soto-Márquez, Moisés Jiménez-Santos, Joel Estrada Gallegos, Moisés Cuttiel Calderón-Abbo
No abstract text is available yet for this article.
August 23, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28832374/chronic-thromboembolic-pulmonary-hypertension-epidemiology-diagnosis-and-management
#10
Srikanth Yandrapalli, Sohaib Tariq, Jessica Kumar, Wilbert S Aronow, Ramin Malekan, William H Frishman, Gregg M Lanier
Chronic thromboembolic pulmonary hypertension (CTEPH), classified as WHO group 4 pulmonary hypertension (PH), is an interesting and rare pulmonary vascular disorder secondary to mechanical obstruction of the pulmonary vasculature from thromboembolism resulting in PH. The pathophysiology is complex, beginning with mechanical obstruction of the pulmonary arteries, which eventually leads to arteriopathic changes and vascular remodeling in the non-occluded arteries and in the distal segments of the occluded arteries mediated by thrombus non-resolution, abnormal angiogenesis, endothelial dysfunction, and various local growth factors...
August 18, 2017: Cardiology in Review
https://www.readbyqxmd.com/read/28821335/symptoms-are-more-useful-than-echocardiography-in-patient-selection-for%C3%A2-pulmonary-endarterectomy
#11
Laura Donahoe, Rachel Vanderlaan, John Thenganatt, Karen McRae, Anastasia Bykova, Jakov Moric, John Granton, Marc de Perrot
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is still largely underdiagnosed in the general population. Although transthoracic echocardiogram (TTE) is recommended to screen for CTEPH, it may not detect patients with chronic thromboembolic disease (CTED) and mild or exercise-induced pulmonary hypertension (PH) who could also benefit from pulmonary endarterectomy (PEA). METHODS: All patients referred to our CTEPH program with persistent mismatched perfusion defects on ventilation-perfusion (VQ) scan between January 2005 and June 2015 were divided into three groups according to TTE and right heart catheterization (RHC) as follows: (1) typical CTEPH group (PH on RHC and TTE), (2) TTE-negative (neg) CTEPH group (PH on RHC, but not TTE), or (3) CTED group (no PH on RHC and TTE)...
August 16, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28806531/tidal-carbon-dioxide-as-a-prognostic-feature-in-inoperable-chronic-thromboembolic-pulmonary-hypertension
#12
Manuel J Richter, Khodr Tello, Katrin Milger, Hossein A Ghofrani, Henning Gall
No abstract text is available yet for this article.
August 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28806530/reply-tidal-carbon-dioxide-as-a-prognostic-feature-in-inoperable-chronic-thromboembolic-pulmonary-hypertension
#13
Caitlin E Welch, Anna R Hemnes
No abstract text is available yet for this article.
August 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28804055/extensive-revascularization-by-balloon-pulmonary-angioplasty-for-chronic-thromboembolic-pulmonary-hypertension-beyond-hemodynamic-normalization
#14
Yuto Shinkura, Kazuhiko Nakayama, Kenichi Yanaka, Hiroto Kinutani, Naoki Tamada, Yasunori Tsuboi, Seimi Satomi-Kobayashi, Hiromasa Otake, Toshiro Shinke, Noriaki Emoto, Ken-Ichi Hirata
AIMS: Balloon pulmonary angioplasty (BPA) improves hemodynamics and exercise capacity in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, even after BPA many patients still suffered from exertional dyspnea. Our purpose is to clarify the clinical validity of extensive revascularization by BPA (ERBPA) beyond hemodynamic normalization. METHODS AND RESULTS: 35 CTEPH patients with normalized or borderline mean pulmonary arterial pressure (mPAP) after BPA were retrospectively analyzed...
August 15, 2017: EuroIntervention
https://www.readbyqxmd.com/read/28801076/early-development-of-right-ventricular-ischemic-lesions-in-a-novel-large-animal-model-of-acute-right-heart-failure-in-chronic-thromboembolic-pulmonary-hypertension
#15
David Boulate, Jennifer Arthur Ataam, Andrew J Connolly, Genevieve Giraldeau, Myriam Amsallem, Benoit Decante, Lilia Lamrani, Elie Fadel, Peter Dorfmuller, Frederic Perros, Francois Haddad, Olaf Mercier
BACKGROUND: Our aim was to develop a model of acute right heart failure (ARHF) in the setting of pulmonary hypertension and to characterize acute right ventricular lesions that develop early after hemodynamic restoration. METHODS AND RESULTS: We used a described piglet model of chronic pulmonary hypertension (cPH) induced by pulmonary artery occlusions. We induced ARHF in animals with cPH (ARHF-cPH group, n = 9) by volume loading and iterative acute pulmonary embolism until hemodynamic compromise followed by dobutamine infusion for hemodynamic restoration before sacrifice for right ventricular tissue evaluation...
August 8, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28795615/international-survey-on-the-perioperative-management-of-pulmonary-endarterectomy-the-perfusion-perspective
#16
Roger D P Stanzel, Johannes Gehron, Matthias Wolff, Natalie Striegl, Peter Roth, Rolf-Hasso Boedeker, Christine Scheibelhut, Johannes Herrmann, Ingeborg Welters, Eckhard Mayer, Matthias Scheffler
INTRODUCTION: Pulmonary endarterectomy (PEA) is the most effective treatment available for chronic thromboembolic pulmonary hypertension (CTEPH). Patient selection, surgical technique and perioperative management have improved patient outcomes, which are traditionally linked to surgical and center experience. However, optimal perfusion care has not been well defined. The goal of the international survey was to better characterize the contemporary perfusion management of PEA and highlight similarities and controversies...
August 1, 2017: Perfusion
https://www.readbyqxmd.com/read/28790235/the-challenge-in-diagnosis-and-current-treatment-of-chronic-thromboembolic-pulmonary-hypertension
#17
Anggoro Budi Hartopo, Lucida Kris Dinarti, Abdul S Wahab
Chronic thromboembolic pulmonary hypertension (CTEPH) is currently underdiagnosis and consequently undertreatment in the clinical practice. A deficient in diagnostic modality and treatment availability especially in developing countries makes the CTEPH diagnosis unlikely to confirm. However, high index of clinical suspicion of CTEPH will lead to proper diagnosis and correct treatment  with significant reduction in morbidity and mortality. Left untreated, the mean survival time is 6.8 years and the three year mortality rate may be as high as 90 %...
April 2017: Acta Medica Indonesiana
https://www.readbyqxmd.com/read/28776404/angiogenic-and-inflammatory-biomarkers-in-the-differentiation-of-pulmonary-hypertension
#18
Joanna Säleby, Habib Bouzina, Jakob Lundgren, Göran Rådegran
OBJECTIVES: Pulmonary hypertension (PH) is a serious condition where diagnosis often is delayed due to unspecific symptoms. New methods to diagnose and differentiate PH earlier would therefore be of great value. The aim of this study was therefore to evaluate the relationship between circulating angiogenic and inflammatory biomarkers and various hemodynamic variables in relation to different causes of PH. DESIGN: Plasma samples from 63 patients at diagnosis were extracted from Lund Cardio Pulmonary Register, separated into pulmonary arterial hypertension (PAH, n = 22), chronic thromboembolic pulmonary hypertension (CTEPH, n = 15) and left heart disease (LHD) with (n = 21) and without (n = 5) PH...
October 2017: Scandinavian Cardiovascular Journal: SCJ
https://www.readbyqxmd.com/read/28762624/chronic-complications-of-venous-thromboembolism
#19
M-P Winter, G H Schernthaner, I M Lang
Venous thromboembolism (VTE) is a common disease (~700 per 100 000) that is associated with significant risk of recurrence, chronic complications, and substantial mortality, with reported death rates of up to 40% at 10 years. The development of novel anticoagulants has revolutionized the treatment of acute VTE, while strategies for prevention and treatment of chronic complications still seek for such a landmark change. Impaired thrombus resolution is the common denominator behind VTE complications, which are postthrombotic syndrome (PTS) and chronic thromboembolic pulmonary hypertension (CTEPH)...
August 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28751848/anmco-position-paper-long-term-follow-up-of-patients-with-pulmonary-thromboembolism
#20
Carlo D'Agostino, Pietro Zonzin, Iolanda Enea, Michele Massimo Gulizia, Walter Ageno, Piergiuseppe Agostoni, Michele Azzarito, Cecilia Becattini, Amedeo Bongarzoni, Francesca Bux, Franco Casazza, Nicoletta Corrieri, Michele D'Alto, Nicola D'Amato, Andrea Maria D'Armini, Maria Grazia De Natale, Giovanni Di Minno, Giuseppe Favretto, Lucia Filippi, Valentina Grazioli, Gualtiero Palareti, Raffaele Pesavento, Loris Roncon, Laura Scelsi, Antonella Tufano
Venous thromboembolism (VTE), including pulmonary embolism and deep venous thrombosis, is the third most common cause of cardiovascular death. The management of the acute phase of VTE has already been described in several guidelines. However, the management of the follow-up (FU) of these patients has been poorly defined. This consensus document, created by the Italian cardiologists, wants to clarify this issue using the currently available evidence in VTE. Clinical and instrumental data acquired during the acute phase of the disease are the cornerstone for planning the FU...
May 2017: European Heart Journal Supplements: Journal of the European Society of Cardiology
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