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Pulmonary hypertension , CTEPH

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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/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
#4
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
#5
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
#6
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/28832374/chronic-thromboembolic-pulmonary-hypertension-epidemiology-diagnosis-and-management
#7
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
#8
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/28804055/extensive-revascularization-by-balloon-pulmonary-angioplasty-for-chronic-thromboembolic-pulmonary-hypertension-beyond-hemodynamic-normalization
#9
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/28795615/international-survey-on-the-perioperative-management-of-pulmonary-endarterectomy-the-perfusion-perspective
#10
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
#11
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
#12
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
#13
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/28750932/pulmonary-endarterectomy-in-chronic-thromboembolic-pulmonary-hypertension
#14
Mareike Lankeit, Valentin Krieg, Lukas Hobohm, Sebastian Kölmel, Christoph Liebetrau, Stavros Konstantinides, Christian W Hamm, Eckhard Mayer, Christoph B Wiedenroth, Stefan Guth
BACKGROUND: Management and outcome of patients with operable chronic thromboembolic pulmonary hypertension (CTEPH) who underwent pulmonary endarterectomy (PEA) at a large German referral center were investigated. METHODS: In Germany, 394 PEAs were performed in 2014 and 2015 with an in-hospital mortality rate of 5.8%. Of these, 253 patients (64.2%) were treated at the Kerckhoff Clinic, Bad Nauheim, and 237 (93.7%; median age, 62 years [interquartile range [IQR], 52-72 years]; 46...
July 1, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28705306/riociguat-for-patients-with-chronic-thromboembolic-pulmonary-hypertension-usefulness-of-transitioning-from-phosphodiesterase-type-5-inhibitor
#15
Keiko Yamamoto, Nobuhiro Tanabe, Rika Suda, Akane Sasaki, Akane Matsumura, Ryogo Ema, Hajime Kasai, Fumiaki Kato, Ayumi Sekine, Rintaro Nishimura, Takayuki Jujo, Toshihiko Sugiura, Ayako Shigeta, Seiichiro Sakao, Koichiro Tatsumi
BACKGROUND: Riociguat, the first approved drug for patients with chronic thromboembolic pulmonary hypertension (CTEPH), is a soluble guanylate cyclase (sGC) Stimulator. It directly stimulates sGC independently of nitric oxide (NO) and increases sGC sensitivity for NO. The safety and efficacy of transitioning from a phosphodiesterase 5 inhibitor (PDE5i) to riociguat is unknown. METHODS AND RESULTS: Twenty-three patients were prospectively enrolled: 8 symptomatic patients with inadequate clinical responses to PDE5i were changed to riociguat (transitioned group); 15 started riociguat anew (new or add-on group)...
July 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28701673/pulmonary-artery-diameter-predicts-lung-injury-after-balloon-pulmonary-angioplasty-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#16
Koichi Sugimoto, Kazuhiko Nakazato, Nobuo Sakamoto, Takayoshi Yamaki, Hiroyuki Kunii, Akiomi Yoshihisa, Hitoshi Suzuki, Shu-Ichi Saitoh, Yasuchika Takeishi
Balloon pulmonary angioplasty (BPA) has been an attractive strategy for chronic thromboembolic pulmonary hypertension (CTEPH), even though it occasionally causes lung injury. However, predictive factors of lung injury after BPA have not been established. Pulmonary artery (PA) dilatation is often observed in patients with pulmonary hypertension. We investigated the association between PA diameter and complications after BPA.The subjects were 19 CTEPH patients who underwent BPA. Patients were divided into two groups: patients with lung injury including asymptomatic lung infiltration on computed tomography (CT) images or mild hemoptysis (group L, n = 9) and no complications (group N, n = 10)...
August 3, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28682884/microarray-expression-profile-of-circular-rnas-in-chronic-thromboembolic-pulmonary-hypertension
#17
Ran Miao, Ying Wang, Jun Wan, Dong Leng, Juanni Gong, Jifeng Li, Yan Liang, Zhenguo Zhai, Yuanhua Yang
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but debilitating and life-threatening complication of acute pulmonary embolism. Circular RNAs (circRNAs), presenting as covalently closed continuous loops, are RNA molecules with covalently joined 3'- and 5'-ends formed by back-splicing events. circRNAs may be significant biological molecules to understand disease mechanisms and to identify biomarkers for disease diagnosis and therapy. The aim of this study was to investigate the potential roles of circRNAs in CTEPH...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28680585/pulmonary-thromboendarterectomy-in-the-setting-of-a-mediastinal-venous-malformation-with-a-congenitally-absent-left-subclavian-vein
#18
Vickram Tejwani, Karunakaravel Karuppasamy, Marcelo P Gomes, Abraham Levitin, James M Luethke, Christopher J Morin, Nicholas G Smedira, Gustavo A Heresi, Wayne F Yakes
Venous malformations have static venous lakes that predispose to spontaneous venous thrombosis within the malformation due to its low-flow static state. Thrombi of varying sizes can then embolize continually into the pulmonary arterial circulation, and occlude and narrow elastic pulmonary arteries causing chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary thromboendarterectomy (PTE) is potentially curative in CTEPH, but has not been previously reported in the setting of mediastinal and chest wall venous malformations...
March 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28671932/associations-of-exercise-tolerance-with-hemodynamic-parameters-for-pulmonary-arterial-hypertension-and-for-chronic-thromboembolic-pulmonary-hypertension
#19
Yasunori Tsuboi, Hidekazu Tanaka, Ryo Nishio, Takuma Sawa, Daisuke Terashita, Kazuhiko Nakayama, Seimi Satomi-Kobayashi, Yoshitada Sakai, Noriaki Emoto, Ken-Ichi Hirata
PURPOSE: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are the main subgroups of pulmonary hypertension (PH). Despite differences in their etiologies, both diseases are characterized by vascular remodeling, resulting in progressive right heart failure. Noninvasive periodic evaluation of exercise tolerance has become increasingly important. Cardiopulmonary exercise testing (CPET) and a 6-minute walk test (6MWT) are now both recommended for evaluating exercise tolerance, but there is insufficient knowledge about possible differences in the associations of exercise tolerance with right heart catheterization (RHC) data for patients with PAH and CTEPH...
September 2017: Journal of Cardiopulmonary Rehabilitation and Prevention
https://www.readbyqxmd.com/read/28666570/dead-space-ventilation-is-linked-to-exercise-capacity-and-survival-in-distal-chronic-thromboembolic-pulmonary-hypertension
#20
Laurent Godinas, Caroline Sattler, Edmund M Lau, Xavier Jaïs, Yu Taniguchi, Mitja Jevnikar, Jason Weatherald, Olivier Sitbon, Laurent Savale, David Montani, Gérald Simonneau, Marc Humbert, Pierantonio Laveneziana, Gilles Garcia
BACKGROUND: Cardiopulmonary exercise testing (CPET) is frequently used for the evaluation of patients with pulmonary hypertension (PH). Non-operable distal chronic thromboembolic pulmonary hypertension (CTEPH) represents a unique subgroup of PH where microvascular disease resembling pulmonary arterial hypertension (PAH) may predominate and efficacious medical therapy is now available. However, little is known regarding the detailed CPET profile of patients with distal CTEPH, and whether ventilation and gas exchange responses are different from PAH...
May 22, 2017: Journal of Heart and Lung Transplantation
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