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Chronic thromboembolic pulmonary hypertension

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https://www.readbyqxmd.com/read/28316030/a-new-ct-score-as-index-of-hemodynamic-changes-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#1
Maria Barbara Leone, Marica Giannotta, Massimiliano Palazzini, Mariano Cefarelli, Sofia Martìn Suàrez, Enrico Gotti, Maria Letizia Bacchi Reggiani, Maurizio Zompatori, Nazzareno Galiè
PURPOSE: The aim of this study was to retrospectively assess the relationship between radiological and hemodynamic parameters in patients with chronic thromboembolic pulmonary hypertension (CTEPH). We introduced a new CT-score to evaluate hemodynamic changes, only employing CT-pulmonary angiography (CTPA). MATERIALS AND METHODS: 145 patients affected by CTEPH underwent hemodynamic and CTPA evaluation. Among these 145 patients, 69 underwent pulmonary endarterectomy (PEA) and performed a CTPA evaluation even after surgery...
March 18, 2017: La Radiologia Medica
https://www.readbyqxmd.com/read/28302503/the-giessen-pulmonary-hypertension-registry-survival-in-pulmonary-hypertension-subgroups
#2
Henning Gall, Janine F Felix, Franziska K Schneck, Katrin Milger, Natascha Sommer, Robert Voswinckel, Oscar H Franco, Albert Hofman, Ralph T Schermuly, Norbert Weissmann, Friedrich Grimminger, Werner Seeger, Hossein A Ghofrani
BACKGROUND: Pulmonary hypertension (PH) is a severe, progressive disease. Although 5 PH subgroups are recognized, reports on survival have focused mainly on pulmonary arterial hypertension (PAH). METHODS: Long-term transplant-free survival and its determinants were investigated in patients with PH (diagnosed by right heart catheterization) within a prospective registry at a single referral center in Giessen, Germany. RESULTS: In total, 2,067 patients were enrolled (PAH, 685 patients [33...
February 17, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28298388/pulmonary-endarterectomy-in-the-management-of-chronic-thromboembolic-pulmonary-hypertension
#3
REVIEW
David Jenkins, Michael Madani, Elie Fadel, Andrea Maria D'Armini, Eckhard Mayer
Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature.Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are <5% and survival is >90% at 1 year and >70% at 10 years...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28298387/diagnosis-of-chronic-thromboembolic-pulmonary-hypertension
#4
REVIEW
Deepa Gopalan, Marion Delcroix, Matthias Held
Chronic thromboembolic pulmonary hypertension (CTEPH) is the only potentially curable form of pulmonary hypertension. Rapid and accurate diagnosis is pivotal for successful treatment. Clinical signs and symptoms can be nonspecific and risk factors such as history of venous thromboembolism may not always be present. Echocardiography is the recommended first diagnostic step. Cardiopulmonary exercise testing is a complementary tool that can help to identify patients with milder abnormalities and chronic thromboembolic disease, triggering the need for further investigation...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28294875/-efficiency-of-operation-trombendarterektomy-from-pulmonary-artery-branches-depending-on-the-degree-of-pulmonary-hypertension-and-duration-of-disease
#5
A M Chernyavskiy, A G Edemskiy, M A Chernyavskiy, V G Efimenko, A R Tarkova, S N Ivanov
AIM: to assess effect of thromboendarterectomy from pulmonary artery branches on clinical functional state of patients with chronic thromboembolic pulmonary hypertension (CTEPH) in dependence on degree of pulmonary hypertension and disease duration. MATERIAL: Patients (n=152) were divided into 3 groups: (1) with systolic pulmonary artery pressure (SPAP) less or equal 50 mm Hg (n=20), (2) with SPAP>50- less or equal 80mm Hg (n=46), (3) with SPAP >80mm Hg (n=86)...
May 2016: Kardiologiia
https://www.readbyqxmd.com/read/28291667/medical-therapy-versus-balloon-angioplasty-for-cteph-a-systematic-review-and-meta-analysis
#6
Kevin Phan, Helen E Jo, Joshua Xu, Edmund M Lau
BACKGROUND: A significant number of chronic thromboembolic pulmonary hypertension (CTEPH) patients will have an inoperable disease. Medical therapy and balloon pulmonary angioplasty (BPA) have provided alternate therapeutic options for patients with inoperable CTEPH, although there are a limited number of published studies examining the outcomes. Thus, our study aims to evaluate and compare the efficacy of medical therapy and BPA in patients with inoperable CTEPH. METHODS: An electronic search of six databases was performed and the search results were screened against established criteria for inclusion into this study...
March 1, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28289496/surgical-treatment-of-chronic-thromboembolic-pulmonary-hypertension-pulmonary-thromboendarterectomy
#7
Michael M Madani
Pulmonary thromboendarterectomy (PTE), also referred to as pulmonary endarterectomy, is the definitive treatment for chronic thromboembolic pulmonary hypertension (CTEPH). The true incidence of CTEPH is unknown and difficult to ascertain; however, most experts agree that approximately 4% to 5% of all patients who have an acute episode of pulmonary embolism (PE) will continue to develop CTEPH. Based on an incidence rate of about 0.1% for acute PE, this translates into a CTEPH incidence of approximately 10,000 to 15,000 annually in the United States alone...
October 2016: Methodist DeBakey Cardiovascular Journal
https://www.readbyqxmd.com/read/28289495/treatment-of-chronic-thromboembolic-pulmonary-hypertension-the-role-of-medical-therapy-and-balloon-pulmonary-angioplasty
#8
Timothy M Fernandes, David S Poch, William R Auger
Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable disease when treated with pulmonary thromboendarterectomy (PTE). However, even at experienced surgical centers, nearly one-third of patients with CTEPH will be deemed inoperable for reasons including distal disease, comorbidities, or out-of-proportion pulmonary hypertension. It is in these patients with inoperable CTEPH that pulmonary hypertension (PH)-targeted medical therapy and balloon pulmonary angioplasty have potential therapeutic value...
October 2016: Methodist DeBakey Cardiovascular Journal
https://www.readbyqxmd.com/read/28289494/chronic-thromboembolic-pulmonary-hypertension-pearls-and-pitfalls-of-diagnosis
#9
Humna Abid Memon, C Huie Lin, Ashrith Guha
Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic obstruction of major pulmonary arteries by organized thromboembolic material. Untreated CTEPH can result in pulmonary hypertension and eventually right heart failure, yet it is the only form of pulmonary hypertension that is potentially curable with surgical or catheter-based intervention. While early diagnosis is key to increasing the likelihood of successful treatment, CTEPH remains largely underdiagnosed. This article reviews the role of echocardiogram, ventilation/perfusion scan, and other available modalities in the diagnosis of CTEPH...
October 2016: Methodist DeBakey Cardiovascular Journal
https://www.readbyqxmd.com/read/28289493/epidemiology-and-pathophysiology-of-chronic-thromboembolic-pulmonary-hypertension-risk-factors-and-mechanisms
#10
Sarah Medrek, Zeenat Safdar
Chronic thromboembolic pulmonary hypertension (CTEPH) occurs when thromboemboli travel to the pulmonary vasculature, fail to resolve, and cause elevated pulmonary arterial pressure. Untreated, this disease leads to progressive right heart failure and death. It develops in approximately 1% to 5% of patients who suffer an acute pulmonary embolism (PE) and has an overall incidence of 3 to 30 per million in the general population. While it is not entirely evident why most but not all people are able to clear this clot burden, there are known risk factors for the development of CTEPH...
October 2016: Methodist DeBakey Cardiovascular Journal
https://www.readbyqxmd.com/read/28289492/diagnostic-and-treatment-advances-for-chronic-thromboembolic-pulmonary-hypertension
#11
EDITORIAL
Alan B Lumsden, Myung Park
No abstract text is available yet for this article.
October 2016: Methodist DeBakey Cardiovascular Journal
https://www.readbyqxmd.com/read/28289017/activated-tafi-promotes-the-development-of-chronic-thromboembolic-pulmonary-hypertension-a-possible-novel-therapeutic-target
#12
Taijyu Satoh, Kimio Satoh, Nobuhiro Yaoita, Nobuhiro Kikuchi, Junichi Omura, Ryo Kurosawa, Kazuhiko Numano, Elias Al-Mamun, Mohammad Abdul Hai Siddique, Shinichiro Sunamura, Masamichi Nogi, Kota Suzuki, Satoshi Miyata, John Morser, Hiroaki Shimokawa
Rationale: Pulmonary hypertension (PH) is a fatal disease; however, its pathogenesis still remains to be elucidated. Thrombin-activatable fibrinolysis inhibitor (TAFI) is synthesized by the liver and inhibits fibrinolysis. Plasma TAFI levels are significantly increased in chronic thromboembolic PH (CTEPH) patients. Objective: To determine the role of activated TAFI (TAFIa) in the development of CTEPH. Methods and Results: Immunostaining showed that TAFI and its binding partner thrombomodulin (TM) were highly expressed in the pulmonary arteries (PA) and thrombus in patients with CTEPH...
March 13, 2017: Circulation Research
https://www.readbyqxmd.com/read/28275536/rheumatoid-arthritis-associated-pulmonary-hypertension-clinical-challenges-reflecting-the-diversity-of-pathophysiology
#13
Evangelia Panagiotidou, Evdokia Sourla, Serafim Xrisovalantis Kotoulas, Sofia Akritidou, Vasileios Bikos, Vasileios Bagalas, Ioannis Stanopoulos, Georgia Pitsiou
The present article reports three clinical cases in order to elucidate the diversity of the pathophysiological mechanisms that underlie rheumatoid arthritis associated pulmonary hypertension. The condition's three major causes are: interstitial lung disease, vasculitis, and chronic thromboembolic disease, but it should be noted that the multiple pulmonary manifestations of rheumatoid arthritis, can all contribute to chronic lung disease or hypoxia. The first patient in this report suffered from moderate restriction due to fibrosis and was diagnosed with pulmonary hypertension during an episode of life threatening hypoxia...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28266961/thoracic-manifestations-of-klippel-trenaunay-syndrome
#14
Mark M Hammer, Wallace T Miller
Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by venous malformations and a predisposition to pulmonary embolism. We reviewed the imaging findings of 7 adult patients with KTS who underwent thoracic CT. While the superficial manifestations of KTS predominantly affect the extremities, patients frequently develop abnormalities of the pulmonary arterial system, particularly chronic thromboembolic pulmonary hypertension. Additionally, some patients are seen to develop pulmonary arteriolar aneurysms; the physiologic significance of this finding is unknown at this time...
March 6, 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28266825/cardiovascular-disease-update-pulmonary-hypertension
#15
Johnathan D McDivitt, Craig Barstow
Pulmonary hypertension (PH) is a spectrum disorder with multiple causes of the elevation of pressure in the lungs. It often is difficult to diagnose because it mimics many commonly reported symptoms (eg, dyspnea, exercise intolerance, chest pain). Diagnosis is made via right heart catheterization; however, transthoracic echocardiography may show evidence of elevated pulmonary pressure as the first clue to the diagnosis. Diagnostic tests to consider include a liver panel, complete blood count, and thyroid function test; electrocardiogram; chest x-ray; pulmonary function testing; and possibly lung imaging via computed tomography scan or ventilation-perfusion scan...
March 2017: FP Essentials
https://www.readbyqxmd.com/read/28262286/pulmonary-arterial-strain-and-remodeling-induced-stiffening-are-differentiated-in-a-chronic-model-of-pulmonary-hypertension
#16
Mark J Golob, Diana M Tabima, Gregory D Wolf, James L Johnston, Omid Forouzan, Ashley M Mulchrone, Heidi B Kellihan, Melissa L Bates, Naomi C Chesler
Pulmonary hypertension (PH) is a debilitating vascular disease that leads to pulmonary artery (PA) stiffening, which is a predictor of patient mortality. During PH development, PA stiffening adversely affects right ventricular function. PA stiffening has been investigated through the arterial nonlinear elastic response during mechanical testing using a canine PH model. However, only circumferential properties were reported and in the absence of chronic PH-induced PA remodeling. Remodeling can alter arterial nonlinear elastic properties via chronic changes in extracellular matrix (ECM) content and geometry...
February 21, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/28256427/midterm-effect-of-balloon-pulmonary-angioplasty-on-hemodynamics-and-subclinical-myocardial-damage-in-chronic-thromboembolic-pulmonary-hypertension
#17
Mai Kimura, Takashi Kohno, Takashi Kawakami, Masaharu Kataoka, Toshimitsu Tsugu, Keitaro Akita, Sarasa Isobe, Yuji Itabashi, Yuichiro Maekawa, Mitsushige Murata, Keiichi Fukuda
BACKGROUND: The acute favourable effect of balloon pulmonary angioplasty (BPA) has been proven in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, data on its effect 6 months after therapy (from now on referred to as mid-term) and influence on the right ventricle and myocardial damage are sparse. To evaluate factors that influence improvement in cardiac output (CO) and subclinical myocardial damage, we examined hemodynamics and serum high-sensitivity troponin T (hs-TnT) levels before, 1 week after, and 6 months after BPA...
December 8, 2016: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28241922/pulmonary-hypertension
#18
Marius M Hoeper, Hossein-Ardeschir Ghofrani, Ekkehard Grünig, Hans Klose, Horst Olschewski, Stephan Rosenkranz
BACKGROUND: About 1% of adults suffer from pulmonary hypertension (PH). The various types of PH differ widely with respect to their incidence, clinical significance, and treatment. METHODS: Selective review of the literature in association with a consensus conference. RESULTS: Pulmonary hypertension is divided into five major categories. Those that are of particular clinical relevance are pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension due to left heart and lung diseases...
February 3, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28236024/molecular-imaging-of-the-human-pulmonary-vascular-endothelium-in-pulmonary-hypertension-a-phase-ii-safety-and-proof-of-principle-trial
#19
François Harel, David Langleben, Steve Provencher, Alain Fournier, Vincent Finnerty, Quang T Nguyen, Myriam Letourneau, Xavier Levac, Gad Abikhzer, Jean Guimond, Asmaa Mansour, Marie-Claude Guertin, Jocelyn Dupuis
PURPOSE: The adrenomedullin receptor is densely expressed in the pulmonary vascular endothelium. PulmoBind, an adrenomedullin receptor ligand, was developed for molecular diagnosis of pulmonary vascular disease. We evaluated the safety of PulmoBind SPECT imaging and its capacity to detect pulmonary vascular disease associated with pulmonary hypertension (PH) in a human phase II study. METHODS: Thirty patients with pulmonary arterial hypertension (PAH, n = 23) or chronic thromboembolic PH (CTEPH, n = 7) in WHO functional class II (n = 26) or III (n = 4) were compared to 15 healthy controls...
February 24, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28218209/clinical-study-of-acute-vasoreactivity-testing-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#20
Qi-Xia Xu, Yuan-Hua Yang, Jie Geng, Zhen-Guo Zhai, Juan-Ni Gong, Ji-Feng Li, Xiao Tang, Chen Wang
BACKGROUND: The clinical significance of acute vasoreactivity testing (AVT) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) remains unclear. We analyzed changes in hemodynamics and oxygenation dynamics indices after AVT in patients with CTEPH using patients with pulmonary arterial hypertension (PAH) as controls. METHODS: We analyzed retrospectively the results of AVT in 80 patients with PAH and 175 patients with CTEPH registered in the research database of Beijing Chao-Yang Hospital between October 2005 and August 2014...
2017: Chinese Medical Journal
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