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Pulmonary endarterectomy, chronic thromboembolic pulmonary hypertension

H Wilkens, S Konstantinides, I Lang, A C Bunck, M Gerges, F Gerhardt, A Grgic, C Grohé, S Guth, M Held, J Hinrichs, M M Hoeper, W Klepetko, T Kramm, U Krüger, M Lankeit, B C Meyer, K M Olsson, H-J Schäfers, M Schmidt, H J Seyfarth, S Ulrich, C B Wiedenroth, E Mayer
The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH), but also other significant subgroups of pulmonary hypertension (PH). In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany to discuss open and controversial issues surrounding the practical implementation of the European Guidelines...
October 2016: Deutsche Medizinische Wochenschrift
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
Takashi Kawakami, Aiko Ogawa, Katsumasa Miyaji, Hiroki Mizoguchi, Hiroto Shimokawahara, Takanori Naito, Takashi Oka, Kei Yunoki, Mitsuru Munemasa, Hiromi Matsubara
BACKGROUND: Balloon pulmonary angioplasty (BPA) is an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for standard therapy, pulmonary endarterectomy. Although there are several classifications of vascular lesions, these classifications are based on the features of the specimen removed during pulmonary endarterectomy. Because organized thrombi are not removed during balloon pulmonary angioplasty, we attempted to establish a new classification of vascular lesions based on pulmonary angiographic images...
October 2016: Circulation. Cardiovascular Interventions
Erika B Rosenzweig, Mauer Biscotti, Kirsten Cleary, Richard Smiley, Matthew D Bacchetta
It is well described that patients with group 1 forms of pulmonary arterial hypertension have a high risk of mortality during pregnancy and in the early postpartum period. However, to the authors' knowledge, the diagnosis and management of group 4 pulmonary hypertension due to chronic thromboembolic pulmonary hypertension (CTEPH) during pregnancy with early postpartum pulmonary endarterectomy (PEA) has not been previously reported. We report the case of a 28-year-old woman who received a diagnosis of CTEPH during her pregnancy, was managed as an inpatient by a multidisciplinary team throughout the pregnancy and early postpartum period, and underwent PEA 6 weeks after delivery...
September 2016: Pulmonary Circulation
Rika Suda, Nobuhiro Tanabe, Keiichi Ishida, Fumiaki Kato, Takashi Urushibara, Ayumi Sekine, Rintaro Nishimura, Takayuki Jujo, Toshihiko Sugiura, Ayako Shigeta, Seiichiro Sakao, Koichiro Tatsumi
BACKGROUND AND OBJECTIVE: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease in some patients, despite improved treatments. Microvasculopathy has been implicated in the poor outcomes of patients with CTEPH. A reduction in the diffusing capacity for carbon monoxide (DLCO ) was previously suggested to indicate microvasculopathy in CTEPH patients; therefore, we assessed DLCO /alveolar ventilation (DLCO /VA ) as a prognostic and pathophysiological marker in CTEPH...
September 11, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
F Vanden Eynden, M Bol Alima, J Racape, B El Oumeiri, J-L Vachiéry, G Van Nooten
No abstract text is available yet for this article.
June 2016: Acta Cardiologica
Masahiko Oka, Ivan F McMurtry, Kaori Oshima
Chronic thromboembolic pulmonary hypertension (CTEPH) is a hot topic in the field of pulmonary hypertension, because many CTEPH patients are now curable by surgical pulmonary endarterectomy and more recently possibly by pulmonary balloon angioplasty. However, there are still uncertainties regarding the pathogenesis of CTEPH, specifically how and where the small vessel arteriopathy that is indistinguishable from that in pulmonary arterial hypertension (plexogenic arteriopathy) develops, and how pulmonary endarterectomy improves hemodynamics and possibly cures CTEPH...
September 2, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
Takayuki Jujo, Nobuhiro Tanabe, Seiichiro Sakao, Hatsue Ishibashi-Ueda, Keiichi Ishida, Akira Naito, Fumiaki Kato, Takao Takeuchi, Ayumi Sekine, Rintaro Nishimura, Toshihiko Sugiura, Ayako Shigeta, Masahisa Masuda, Koichiro Tatsumi
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by occlusion of pulmonary arteries by organized chronic thrombi. Persistent hypoxemia and residual pulmonary hypertension (PH) following successful pulmonary endarterectomy (PEA) are clinically important problems; however, the underlying mechanisms remain unclear. We have previously reported that residual PH is closely related to severe pulmonary vascular remodeling and hypothesize that this arteriopathy might also be involved in impaired gas exchange...
2016: PloS One
Joanna Pepke-Zaba, Xavier Jais, Richard Channick
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but life-threatening condition resulting from unresolved thromboembolic obstructions. Pulmonary endarterectomy surgery is currently the standard of treatment, as it is potentially curative; however, not all cases are amenable to surgical intervention due to distal distribution of the organized thromboembolic material or the presence of comorbidities. Up to one-third of patients have persistent or recurrent pulmonary hypertension after pulmonary endarterectomy...
July 2016: Annals of the American Thoracic Society
Michael Madani, Eckhard Mayer, Elie Fadel, David P Jenkins
In chronic thromboembolic pulmonary hypertension, thromboemboli do not undergo resolution but instead become highly organized and fibrotic, resulting in obstruction of segments of the pulmonary vascular tree. Progressive pulmonary hypertension ensues, with substantial associated morbidity and mortality. Despite advances in medical therapy for some types of pulmonary hypertension, surgical pulmonary endarterectomy, also referred to as pulmonary thromboendarterectomy, remains the only potentially curative option for patients with chronic thromboembolic pulmonary hypertension...
July 2016: Annals of the American Thoracic Society
Deepa Gopalan, Daniel Blanchard, William R Auger
Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 25 mm Hg. Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as pulmonary hypertension in the presence of an organized thrombus within the pulmonary vascular bed that persists at least 3 months after the onset of anticoagulant therapy. Because CTEPH is potentially curable by surgical endarterectomy, correct identification of patients with this form of pulmonary hypertension and an accurate assessment of surgical candidacy are essential to provide optimal care...
July 2016: Annals of the American Thoracic Society
Athanasios Charalampopoulos, J Simon R Gibbs, Rachel J Davies, Wendy Gin-Sing, Kevin Murphy, Karen K Sheares, Joanna Pepke-Zaba, David P Jenkins, Luke S Howard
We tested the hypothesis that patients with chronic thromboembolic pulmonary hypertension (CTEPH) that was deemed to be inoperable were more likely to respond to drugs for treating pulmonary arterial hypertension (PAH) by using cardiopulmonary exercise (CPX) testing than those with CTEPH that was deemed to be operable. We analyzed CPX testing data of all patients with CTEPH who were treated with PAH drugs and had undergone CPX testing before and after treatment at a single pulmonary hypertension center between February 2009 and March 2013...
September 1, 2016: Journal of Applied Physiology
Manuel Jonas Richter, Katrin Milger, Khodr Tello, Philipp Stille, Werner Seeger, Eckhard Mayer, Hossein A Ghofrani, Henning Gall
BACKGROUND: Six-minute walk test (6MWT) is routinely performed in chronic thromboembolic pulmonary hypertension (CTEPH) before pulmonary endarterectomy (PEA). However, the clinical relevance of heart rate response (ΔHR) and exercise-induced oxygen desaturation (EID) during 6MWT is remaining unknown. METHODS: Patients undergoing PEA in our center between 03/2013-04/2014 were assessed prospectively with hemodynamic and exercise parameters prior to and 1 year post-PEA...
2016: BMC Pulmonary Medicine
Coen van Kan, Mart N van der Plas, Herre J Reesink, Reindert P van Steenwijk, Jaap J Kloek, Robert Tepaske, Peter I Bonta, Paul Bresser
BACKGROUND: Patients with chronic thromboembolic disease (CTED) may suffer from exercise intolerance without pulmonary hypertension at rest. Pulmonary endarterectomy (PEA) for symptomatic CTED results in improvement of symptoms and quality of life. Neither the pathophysiology of the exercise limitation nor the underlying mechanisms of the PEA-induced improvement have been studied previously. OBJECTIVES: We studied hemodynamic and ventilatory responses upon exercise in 14 patients with CTED...
September 2016: Journal of Thoracic and Cardiovascular Surgery
Toru Satoh, Masaharu Kataoka, Takumi Inami, Haruhisa Ishiguro, Ryoji Yanagisawa, Nobuhiko Shimura, Yohei Shigeta, Hideaki Yoshino
INTRODUCTION: Percutaneous transluminal pulmonary angioplasty (PTPA) was introduced for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in the late 20(th) century, and first attempts in collective patients were made in 2001 with beneficial effects but a moderate amount of complications. It was refined around 2010, and has been recently established as an effective and safe treatment. AREAS COVERED: The indication was originally inoperable CTEPH with peripheral lesions, but has now widened to symptomatic or hypoxic patients...
September 2016: Expert Review of Cardiovascular Therapy
Zachary R Smith, Charles T Makowski, Rana L Awdish
Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease of the pulmonary vascular bed that is characterized by elevations in the mean pulmonary artery pressure in the setting of perfusion defects on ventilation-perfusion scan, and subsequently confirmed by pulmonary angiography. CTEPH, or World Health Organization (WHO) group 4 pulmonary hypertension, is a result of unresolved thromboembolic obstruction in the pulmonary arteries. Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH as it is a potentially curative therapy...
2016: Therapeutics and Clinical Risk Management
Ivan M Robbins, Meredith E Pugh, Anna R Hemnes
Chronic, unresolved thromboemboli are an important cause of pulmonary hypertension (PH) with specific treatment strategies differing from other types of PH. Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group 4 PH by the World Health Organization. It is a rare, but underdiagnosed, complication of acute pulmonary embolism that does not resolve and results in occlusion of large pulmonary arteries with a fibro-thrombotic material. The etiology of CTEPH remains uncertain, and it is unknown why certain patients with acute pulmonary embolism develop this disorder...
May 25, 2016: Trends in Cardiovascular Medicine
Matthias Held, Philipp Kolb, Maria Grün, Berthold Jany, Gudrun Hübner, Aleksandar Grgic, Regina Holl, Hans-Joachim Schaefers, Heinrike Wilkens
BACKGROUND: Patients with chronic thromboembolic pulmonary disease (CTED) have persistent pulmonary vascular obstruction and exercise intolerance without pulmonary hypertension at rest and may benefit from pulmonary endarterectomy. However, up to now, CTED has been poorly characterized. OBJECTIVES: This study aimed to analyze the exercise capacity and limiting factors in CTED. METHODS: We compared right heart catheterization and cardiopulmonary exercise test results of patients with CTED [mean pulmonary artery pressure (mPAP) at rest <25 mm Hg, n = 10], chronic thromboembolic pulmonary hypertension (CTEPH, n = 31) and a control group (n = 41) presenting with dyspnea but normal pulmonary vascular imaging and excluded pulmonary hypertension...
2016: Respiration; International Review of Thoracic Diseases
Alper Kepez, Bulent Mutlu, Ashok Paudel, Cigdem Ileri, Halil Atas, Bedrettin Yildizeli
Background Our aim was to determine the prevalence of coronary artery - pulmonary artery collaterals in patients with chronic thromboembolic pulmonary hypertension (CTEPH) by retrospectively evaluating coronary angiograms of eligible consecutive patients who had undergone pulmonary endarterectomy (PEA). We also aimed to evaluate predictors and potential clinical associates of these collaterals. Methods Coronary angiograms of 83 consecutive CTEPH patients who had undergone coronary angiography before PEA operation between January 1, 2012 and June 1, 2015 were retrospectively evaluated for presence of coronary artery - pulmonary artery collaterals...
June 21, 2016: Thoracic and Cardiovascular Surgeon
Chen Wang, Zhi-Cheng Jing, Yi-Gao Huang, Da-Xin Zhou, Zhi-Hong Liu, Christian Meier, Sylvia Nikkho, John Curram, Peng Zhang, Jian-Guo He
OBJECTIVE: PATENT-1 and CHEST-1 were pivotal, international phase III trials assessing riociguat for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Here we compare Chinese patients from these studies with the overall populations, and report the clinical effect and safety of riociguat in Chinese patients with PAH and CTEPH. METHODS: PATENT-1 was a 12-week, randomised, double-blind, placebo-controlled trial of riociguat (maximum 2...
2016: Heart Asia
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