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Chronic thromboembolic pulmonary hypertension

Benedetta Vanini, Valentina Grazioli, Antonio Sciortino, Maurizio Pin, Vera N Merli, Anna Celentano, Ilaria Parisi, Catherine Klersy, Lucia Petrucci, Maurizio Salati, Pierluigi Politi, Andrea M D'Armini
BACKGROUND: In this prospective, single-center, observational study, we investigated the association between repeated short periods of circulatory arrest with moderate hypothermia during pulmonary endarterectomy (PEA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and different neuropsychological dimensions. METHODS: We examined 70 patients with CTEPH, >18 to 80 years of age, who had been treated with PEA. Neuropsychological testing was performed...
February 19, 2018: Journal of Heart and Lung Transplantation
Julio Sandoval
Despite advances in drug therapy, pulmonary hypertension-particularly arterial hypertension (PAH)-remains a fatal disease. Untreatable right heart failure (RHF) from PAH eventually ensues and remains a significant cause of death in these patients. Lowering pulmonary input impedance with different PAH-specific drugs is the obvious therapeutic target in RHF due to chronically increased afterload. However, potential clinical gain can also be expected from attempts to unload the right heart and increase systemic output...
March 12, 2018: Revista Española de Cardiología
Hideki Miwa, Nobuhiro Tanabe, Takayuki Jujo, Fumiaki Kato, Rie Anazawa, Keiko Yamamoto, Akira Naito, Hajime Kasai, Rintaro Nishimura, Rika Suda, Toshihiko Sugiura, Seiichiro Sakao, Keiichi Ishida, Masahisa Masuda, Koichiro Tatsumi
BACKGROUND: Several new treatments for chronic thromboembolic pulmonary hypertension (CTEPH) have appeared in recent years, which have led to changes in the treatment algorithm. Changes in survival rates and prognostic factors, however, have not been estimated so far.Methods and Results:Two hundred and eighty patients were diagnosed with CTEPH at Chiba University Hospital between June 1986 and June 2016. Survival rate was investigated by date of treatment initiation (group 1, 1986-1998; group 2, 1999-2008; group 3, 2009-2016)...
March 13, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Maria Anna Bazmpani, Georgios Arsos, Paul Zarogoulidis, Argyrios Doumas, Theodoros Dimitroulas, George Sianos, Stavros Hadjimiltiades, Konstantinos Kouskouras, Eckhard Mayer, Haralambos Karvounis, George Giannakoulas
No abstract text is available yet for this article.
January 1, 2018: Pulmonary Circulation
Frederikus A Klok, Stefano Barco
In addition to among others major bleeding from anticoagulant therapy and recurrent venous thromboembolism (VTE), patients who survived acute pulmonary embolism (PE) face an increased risk of chronic functional limitations and decreased quality of life. In recent years, this latter complications have been better framed within the evolving definition of "post-PE syndrome" of which chronic thromboembolic pulmonary hypertension (CTEPH) represents the most extreme presentation. The post-PE syndrome in all its aspects is a frequent and clinically relevant long-term complication of PE but -except for CTEPH- has been largely understudied...
February 2018: Hämostaseologie
Raffaele Pesavento, Paolo Prandoni
Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon and late complication of pulmonary embolism resulting from misguided remodelling of residual pulmonary thromboembolic material and small-vessel arteriopathy. CTEPH is the only form of pulmonary hypertension (PH) potentially curable by pulmonary endarterectomy (PEA). Unfortunately, several patients have either an unacceptable risk-benefit ratio for undergoing the surgical intervention or develop persistent PH after PEA. Novel medical and endovascular therapies can be considered for them...
March 2, 2018: Thrombosis Research
Stavros V Konstantinides
No abstract text is available yet for this article.
March 2, 2018: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
David Montani, Julien Henry, Caroline O'Connell, Xavier Jaïs, Vincent Cottin, David Launay, Gilbert Habib, Arnaud Bourdin, Mitja Jevnikar, Laurent Savale, Laurence Rottat, Gérald Simonneau, Olivier Sitbon, Marc Humbert, Yannick Allanore
BACKGROUND: Precapillary pulmonary hypertension (PH), and particularly pulmonary arterial hypertension (PAH), is a life-threatening complication of connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease). The relationship between PH and rheumatoid arthritis (RA) has not been clearly established. OBJECTIVES: The aim of the study was to evaluate the relationship between precapillary PH and RA. METHODS: We identified patients with PH and suspected RA included in the French PH Registry between 1 May 2004 and 31 December 2012 and evaluated the prevalence of confirmed RA in this population...
February 28, 2018: Respiration; International Review of Thoracic Diseases
Takayuki Kawashima, Akihiro Yoshitake, Takashi Kawakami, Hideyuki Shimizu
We performed a two-stage procedure combining balloon pulmonary angioplasty and pulmonary endarterectomy for a high-risk chronic thromboembolic pulmonary hypertension patient with high pulmonary vascular resistance. First, balloon pulmonary angioplasty was performed for distal lesions to improve hemodynamics and decrease the surgical risk. Subsequently, pulmonary endarterectomy was performed for proximal lesions, and the hemodynamics and symptoms improved dramatically. Our strategy was therefore found to be useful for treating a high-risk chronic thromboembolic pulmonary hypertension patient...
February 23, 2018: Annals of Vascular Surgery
Panagiotis Karyofyllis, Varvara Papadopoulou, Vassilis Voudris, Hiromi Matsubara
PURPOSE OF REVIEW: Chronic thromboembolic pulmonary hypertension (CTEPH) is the only potentially curable form of precapillary pulmonary hypertension. Although pulmonary endarterectomy (PEA) is the preferred management strategy, a significant number of CTEPH patients will have an inoperable disease. As drug therapy is not expected to offer relief from the mechanical component of the disease, the novel technique of balloon pulmonary angioplasty (BPA) has provided a new therapeutic option for patients with inoperable CTEPH...
February 24, 2018: Current Treatment Options in Cardiovascular Medicine
Ying Wang, Xiaoxi Huang, Dong Leng, Jifeng Li, Lei Wang, Yan Liang, Jun Wang, Ran Miao, Tao Jiang
Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening disease which is often underpinned by vascular remodeling. Although pulmonary arterial smooth muscle cells (PASMCs) are the main participants in vascular remodeling, their biological role in CTEPH is not entirely clear. In this study, we analyzed the whole epigenome-wide DNA methylation profiles of cultured PASMCs from CTEPH and control cell lines using an Illumina HumanMethylation450K BeadChip. A total of 6829 significantly differentially methylated probes (DMPs) were detected between the two groups, of which 4246 DMPs were hypermethylated while 2583 DMPs were hypomethylated...
February 23, 2018: Physiological Genomics
Joan Albert Barberà, Antonio Román, Miguel Ángel Gómez-Sánchez, Isabel Blanco, Remedios Otero, Raquel López-Reyes, Isabel Otero, Gregorio Pérez-Peñate, Ernest Sala, Pilar Escribano
Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. The causes of pulmonary hypertension are classified into 5 major groups: arterial, due to left heart disease, due to lung disease and/or hypoxemia, chronic thromboembolic, with unclear and/or multifactorial mechanisms. This is a brief summary of the Guidelines on the Diagnostic and Treatment of Pulmonary Hypertension of the Spanish Society of Pulmonology and Thoracic Surgery...
February 19, 2018: Archivos de Bronconeumología
Henning Gall, Jean-Luc Vachiéry, Nobuhiro Tanabe, Michael Halank, Mauricio Orozco-Levi, Lisa Mielniczuk, MiKyung Chang, Kai Vogtländer, Ekkehard Grünig
PURPOSE: A proportion of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) do not achieve treatment goals or experience side effects on their current therapy. In such cases, switching patients to a new drug while discontinuing the first may be a viable and appropriate treatment option. CAPTURE was designed to investigate how physicians manage the switching of patients to riociguat in real-world clinical practice. Observations from the study were used to assess whether recommendations in the riociguat prescribing information are reflected in clinical practice...
February 22, 2018: Lung
Thorsten Kramm, Heinrike Wilkens, Jan Fuge, Hans-Joachim Schäfers, Stefan Guth, Christoph B Wiedenroth, Bettina Weingard, Doerte Huscher, David Pittrow, Serghei Cebotari, Marius M Hoeper, Eckhard Mayer, Karen M Olsson
BACKGROUND: The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH. METHODS: We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a European pulmonary hypertension registry...
February 15, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
(no author information available yet)
No abstract text is available yet for this article.
February 15, 2018: American Journal of Respiratory and Critical Care Medicine
D Thiwanka Wijeratne, Katherine Lajkosz, Susan B Brogly, M Diane Lougheed, Li Jiang, Ahmad Housin, David Barber, Ana Johnson, Katharine M Doliszny, Stephen L Archer
BACKGROUND: The World Health Organization recognizes 5 groups of pulmonary hypertension (PH), categorized by pathogenesis or comorbidity: 1-pulmonary arterial hypertension 2-left-heart disease, 3-lung disease and hypoxia 4-chronic thromboembolic disease, and 5-miscellaneous. The epidemiology of PH, apart from group 1, is largely unknown. METHODS AND RESULTS: We describe incidence, prevalence, comorbidities, mortality and prescribing patterns for groups 1 to 4 PH from 1993 to 2012...
February 2018: Circulation. Cardiovascular Quality and Outcomes
Susana Hoette, Nicolas Creuzé, Sven Günther, David Montani, Laurent Savale, Xavier Jaïs, Florence Parent, Olivier Sitbon, Carlos Eduardo Rochitte, Gerald Simonneau, Marc Humbert, Rogerio Souza, Denis Chemla
BACKGROUND: The right ventricular ejection fraction (RVEF) is a surrogate marker of right ventricular function in pulmonary hypertension (PH), but its measurement is complicated and time consuming. The tricuspid annular plane systolic excursion (TAPSE) measures only the longitudinal component of RV contraction while the right ventricular fractional area change (RVFAC) takes into account both the longitudinal and the transversal components. The aim of our study was to evaluate the relationship between RVEF, RVFAC, and TAPSE according to hemodynamic severity in two groups of patients with PH: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH)...
February 12, 2018: Lung
Gisela M B Meyer, Fernanda B Spilimbergo, Stephan Atmayer, Gabriel S Pacini, Matheus Zanon, Guilherme Watte, Edson Marchiori, Bruno Hochhegger
INTRODUCTION: Our goal was to assess the diagnostic performance of magnetic resonance imaging (MRI) as a single method to diagnose pulmonary hypertension (PH) compared to right heart catheterization (RHC), computed tomography (CT), and ventilation/perfusion (V/Q) scintigraphy. METHODS: We identified 35 patients diagnosed with PH by RHC in our institution who have also undergone a CT, a scintigraphy, and an MRI within a month. All cases were discussed in multidisciplinary meetings...
February 12, 2018: Lung
Paolo Ruggeri, Federica Lo Bello, Francesco Nucera, Michele Gaeta, Francesco Monaco, Gaetano Caramori, Giuseppe Girbino
We present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atypical resection of the left upper pulmonary lobe and the starting of long term anticoagulation the patient was discharged but did not attend the planned follow up visits until one year later when he was seen again for severe dyspnea and exercise intolerance...
December 19, 2017: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
Tian-Xiang Chen, Bigyan Pudasaini, Jian Guo, Su-Gang Gong, Rong Jiang, Lan Wang, Qin-Hua Zhao, Wen-Hui Wu, Ping Yuan, Jin-Ming Liu
Background: Sex differences in chronic thromboembolic pulmonary hypertension (CTEPH) have been revealed in few studies. Although right heart catheterization (RHC) is the gold standard for clinical diagnosis and assessment of prognosis in pulmonary hypertension (PH), cardiopulmonary exercise testing (CPET) has been a more widely used assessment of functional capacity, disease severity, prognosis, and treatment response in PH. We hypothesized that the "sex-specific" CPET indices could estimate the severity of inoperable CTEPH...
2018: International Journal of Chronic Obstructive Pulmonary Disease
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