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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
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
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
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
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
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
Yuzo Yamasaki, Michinobu Nagao, Kohtaro Abe, Kazuya Hosokawa, Satoshi Kawanami, Takeshi Kamitani, Torahiko Yamanouchi, Koshin Horimoto, Hidetake Yabuuchi, Hiroshi Honda
To use cardiac magnetic resonance imaging (MRI) to investigate the effect of balloon pulmonary angioplasty (BPA) on interventricular dyssynchrony and its associations with ventricular interaction, which impairs LV function in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). This prospective observational study was approved by our institutional review board. Cardiac MRI and right heart catheterization were conducted before BPA sessions and at the follow up after BPA in 20 patients with CTEPH...
September 26, 2016: International Journal of Cardiovascular Imaging
Fritz C Roller, Christoph Wiedenroth, Andreas Breithecker, Christoph Liebetrau, Eckhard Mayer, Christian Schneider, Andreas Rolf, Christian Hamm, Gabriele A Krombach
OBJECTIVES: The aim of this study was to assess septal and right ventricular insertion point (RVIP) fibrosis in patients with chronic thromboembolic pulmonary hypertension (CTEPH) via native T1 mapping and extracellular volume fraction (ECV) determination and to analyze correlations with functional parameters. METHODS: Imaging was performed at 1.5 Tesla in 24 patients diagnosed with CTEPH and 24 controls. T1 values were measured within the septal myocardium, the upper and lower RVIP, and the lateral wall at basal short axis section...
September 20, 2016: European Radiology
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
Darrin J Wong, Unnati Sampat, Michael A Gibson, William R Auger, Michael M Madani, Lori B Daniels, Ajit B Raisinghani, Anthony N DeMaria, Daniel G Blanchard
BACKGROUND: Right ventricular function is impaired in chronic thromboembolic pulmonary hypertension (CTEPH). Tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) have been shown to help assess right ventricular function in pulmonary hypertension. Our goal was to (1) assess TAPSE and RVFAC before and after PTE, and (2) assess correlation of these variables with right heart catheterization data and PVR. METHODS: We evaluated 67 consecutive patients with CTEPH for pulmonary thromboendarterectomy (PTE)...
September 7, 2016: Echocardiography
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
Tatsuo Aoki, Koichiro Sugimura, Kotaro Nochioka, Masanobu Miura, Shunsuke Tatebe, Saori Yamamoto, Nobuhiro Yaoita, Hideaki Suzuki, Haruka Sato, Katsuya Kozu, Satoshi Miyata, Kimio Satoh, Hiroaki Shimokawa
BACKGROUND: Although balloon pulmonary angioplasty (BPA) improves the hemodynamics and prognosis of patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), the mechanisms of improvement in oxygenation remain to be elucidated. METHODS AND RESULTS: From August 2013 to May 2015, we performed a total of 113 BPA procedures in 24 patients with inoperable CTEPH (mean 4.7 procedures per patient). Median age was 70 [60, 74] years and 18 were female (75%)...
September 23, 2016: Circulation Journal: Official Journal of the Japanese Circulation Society
Stavros V Konstantinides, Stefano Barco, Stephan Rosenkranz, Mareike Lankeit, Matthias Held, Felix Gerhardt, Leonard Bruch, Ralf Ewert, Martin Faehling, Julia Freise, Hossein-Ardeschir Ghofrani, Ekkehard Grünig, Michael Halank, Nadine Heydenreich, Marius M Hoeper, Hanno H Leuchte, Eckhard Mayer, F Joachim Meyer, Claus Neurohr, Christian Opitz, Antonio Pinto, Hans-Jürgen Seyfarth, Rolf Wachter, Bianca Zäpf, Heinrike Wilkens, Harald Binder, Philipp S Wild
Acute pulmonary embolism (PE) is a frequent cause of death and serious disability. The risk of PE-associated mortality and morbidity extends far beyond the acute phase of the disease. In earlier follow-up studies, as many as 30 % of the patients died during a follow-up period of up to 3 years, and up to 50 % of patients continued to complain of dyspnea and/or poor physical performance 6 months to 3 years after the index event. The most feared 'late sequela' of PE is chronic thromboembolic pulmonary hypertension (CTEPH), the true incidence of which remains obscure due to the large margin of error in the rates reported by mostly small, single-center studies...
November 2016: Journal of Thrombosis and Thrombolysis
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
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
Irene M Lang, Peter Dorfmüller, Anton Vonk Noordegraaf
Chronic thromboembolic pulmonary hypertension (CTEPH) is a late sequel of venous thromboembolism that cannot be completely reproduced in animal models. The prevalence of CTEPH in humans is estimated at roughly 17-20 per million; however, partly because up to 50% of patients with CTEPH never experience symptomatic pulmonary embolism, precise numbers on the incidence and prevalence are not known. Because CTEPH is diagnosed at a median age of 63 years in patients who often have other concomitant cardiovascular disease or lung disease, assessment of pathophysiology in patients can be challenging, We do know that CTEPH is a dual vascular disorder...
July 2016: Annals of the American Thoracic Society
Timothy Fernandes, Benjamin Planquette, Olivier Sanchez, Timothy Morris
After achievement of adequate anticoagulation, the natural history of acute pulmonary emboli ranges from near total resolution of vascular perfusion to long-term persistence of hemodynamically consequential residual perfusion defects. The persistence of perfusion defects is necessary, but not sufficient, for the development of chronic thromboembolic pulmonary hypertension (CTEPH). Approximately 30% of patients have persistent defects after 6 months of anticoagulation, but only 10% of those with persistent defects subsequently develop CTEPH...
July 2016: Annals of the American Thoracic Society
Marion Delcroix, Kim Kerr, Peter Fedullo
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism. As for most rare diseases, epidemiologic data are scarce, but recent registries suggest an incidence of at least 5 per million inhabitants per year. A history of massive or recurrent acute pulmonary embolism is observed in most patients with CTEPH, but the proportion of patients who develop CTEPH after acute pulmonary embolism is a matter of debate, further complicated by the possible misdiagnosis of CTEPH as acute pulmonary embolism...
July 2016: Annals of the American Thoracic Society
Chaosheng Deng, Dawen Wu, Minxia Yang, Yunfei Chen, Caiyun Wang, Zhanghua Zhong, Ningfang Lian, Hua Chen, Shuang Wu
Few reports have examined tissue factor (TF) and forkhead box transcription factor O-1 (FoxO1) expression in chronic thromboembolic pulmonary hypertension (CTEPH) animal models. To investigate the role of TF and FoxO1 and their interactions during CTEPH pathogenesis in a rat model. Autologous blood clots were repeatedly injected into the pulmonary arteries through right jugular vein to induce a rat model of CTEPH. Hemodynamic parameters, histopathology, and TF and FoxO1expression levels were detected. The mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance and vessel wall area/total area (WA/TA) ratio in the experiment group increased significantly than sham group (P < 0...
November 2016: Journal of Thrombosis and Thrombolysis
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