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Chronic thromboembolic pulmonary hypertension

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https://www.readbyqxmd.com/read/29738821/discovery-and-development-of-sgc-stimulators-for-the-treatment-of-pulmonary-hypertension-and-rare-diseases
#1
REVIEW
Peter Sandner, Eva-Maria Becker-Pelster, Johannes Peter Stasch
The NO/sGC/cGMP signaling cascade plays a pivotal role in regulation of cardiovascular, cardiopulmonary and cardiorenal diseases and impairment of this cascade results in severe pathologies. Therefore, pharmacological interventions, targeting this pathway are promising strategies for treating a variety of diseases. Nitrates, supplementing NO and, PDE5 inhibitors preventing cGMP degradation, are used for angina pectoris treatment and the treatment of pulmonary arterial hypertension (PAH), respectively. More recently, a new class of drugs which directly stimulate the sGC enzyme and trigger NO-independent cGMP production was introduced and termed sGC stimulators...
May 5, 2018: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/29730328/poor-subpleural-perfusion-predicts-failure-after-balloon-pulmonary-angioplasty-for-non-operable-chronic-thromboembolic-pulmonary-hypertension
#2
Yu Taniguchi, Philippe Brenot, Xavier Jais, Carlos Garcia, Jason Weatherald, Olivier Planche, Elie Fadel, Marc Humbert, Gérald Simonneau
BACKGROUND: Poor subpleural perfusion (PSP) in the capillary phase of pulmonary angiography predicts worse outcomes after pulmonary endarterectomy in operable chronic thromboembolic pulmonary hypertension (CTEPH). Balloon pulmonary angioplasty (BPA) has emerged for non-operable CTEPH. We assessed the association between PSP and BPA failure. METHOD: Subpleural perfusion was classified as poor (defined as subpleural spaces either not or minimally perfused in all segments) or normal...
May 3, 2018: Chest
https://www.readbyqxmd.com/read/29730148/mri-derived-regional-biventricular-function-in-patients-with-chronic-thromboembolic-pulmonary-hypertension-before-and-after-pulmonary-endarterectomy
#3
Sabine K Maschke, Christian O Schoenfeld, Till F Kaireit, Serghei Cebotari, Karen Olsson, Marius Hoeper, Frank Wacker, Jens Vogel-Claussen
RATIONALE AND OBJECTIVES: The aim of this study was to assess regional myocardial function in patients with chronic thromboembolic hypertension (CTEPH) before and after successful pulmonary endarterectomy (PEA) using magnetic resonance imaging. METHODS: Twenty-two patients with CTEPH underwent cardiac magnetic resonance imaging before and 12 (11, 17) days after PEA. Mean pulmonary artery pressure was evaluated preoperatively by right heart catheterization and during post-PEA intensive care unit-stay using a Swan-Ganz catheter...
May 2, 2018: Academic Radiology
https://www.readbyqxmd.com/read/29704099/usefulness-of-scoring-right-ventricular-function-for-assessment-of-prognostic-factors-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#4
Yoshihiro Kamimura, Naoki Okumura, Shiro Adachi, Shigetake Shimokata, Fumitaka Tajima, Yoshihisa Nakano, Akihiro Hirashiki, Toyoaki Murohara, Takahisa Kondo
Right ventricular (RV) function is associated with prognosis in chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to establish an RV dysfunction score using RV echocardiographic parameters to clarify the clinical characteristics in patients with CTEPH and to compare RV dysfunction score with parameters such as World Health Organization (WHO) functional class, hemodynamics, exercise capacity, and plasma BNP level. We enrolled 35 inpatients with CTEPH (mean age, 62 ± 15 years, 15 males)...
April 27, 2018: Heart and Vessels
https://www.readbyqxmd.com/read/29688945/quantitation-of-perfused-lung-volume-using-hybrid-spect-ct-allows-refining-the-assessment-of-lung-perfusion-and-estimating-disease-extent-in-chronic-thromboembolic-pulmonary-hypertension
#5
Thorsten Derlin, Catharina Kelting, Katja Hueper, Desiree Weiberg, Katrin Meyer, Karen M Olsson, James T Thackeray, Tobias Welte, Frank M Bengel, Marius M Hoeper
BACKGROUND: We evaluated the feasibility of perfusion SPECT/CT for providing quantitative data for estimation of perfusion defect extent in chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: Thirty patients with CTEPH underwent Tc-human serum albumin lung perfusion SPECT/CT. Perfusion defects were quantified using 3 different methods: (1) visual, semiquantitative scoring of perfusion defect extent in each lung segment, (2) threshold-based segmentation of perfused lung volumes, and (3) threshold-based segmentation of perfused lung volumes divided by segmented lung volumes at CT (perfusion index)...
June 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29678648/determinants-of-health-related-quality-of-life-1-year-after-pulmonary-thromboendarterectomy
#6
Oksana Kamenskaya, Asya Klinkova, Irina Loginova, Alexander Chernyavskiy, Alexander Edemskiy, Vladimir V Lomivorotov, Alexander Karaskov
AIM: Pulmonary thromboendarterectomy (PTE) is a recognized treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH), with good long-term haemodynamic results. The aim of this study was to test the hypothesis that the dynamics of health-related quality of life (QoL), 1 year after PTE, depends both on the clinical determinants associated with the underlying disease and the adverse events occurring in the early postoperative period. METHODS: This prospective cohort study included 136 patients with CTEPH...
April 17, 2018: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/29678310/grossly-abnormal-ventilation-perfusion-spect-study-in-idiopathic-pulmonary-arterial-hypertension-without-thromboembolism
#7
Kenneth Chan, John G Coghlan, Margaret Hall, Jamanda Haddock, Alan Bates, Benjamin E Schreiber
Pulmonary hypertension is a serious condition with multiple underlying aetiologies which require different treatment strategies. We present a case of severe idiopathic pulmonary arterial hypertension in a 20-year-old patient with ongoing breathlessness. She was initially diagnosed with asthma and panic attacks in community care. As the symptoms became progressively worse, she was referred for pulmonary hypertension clinic assessment. Ventilation/perfusion single-photon emission computed tomography (V/Q SPECT) showed grossly abnormal perfusion defects which were mismatched to the ventilation scan, suggestive of chronic thromboembolic disease...
January 31, 2018: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29674186/pulmonary-hypertension-real-world-data-from-a-portuguese-expert-referral-centre
#8
A Gomes, C Cruz, J Rocha, M Ricardo, M Vicente, A Melo, M Santos, L Carvalho, F Gonçalves, A Reis
BACKGROUND: Pulmonary hypertension (PH) is a heterogeneous, debilitating condition with highly relevant impact on functional capacity, quality of life, and life-expectancy. OBJECTIVES: This study aims to provide long-term data on the Portuguese PH population, by characterising the clinical presentation, evolution, and outcomes of PH patients in a specialised referral centre. METHODS: Retrospective analysis of a cohort of 101 patients with pre-capillary PH (pcPH) referenced to an expert tertiary care referral centre in northern Portugal from 2002 to 2013...
April 16, 2018: Pulmonology
https://www.readbyqxmd.com/read/29669971/after-the-dawn-balloon-pulmonary-angioplasty-for-patients-with-chronic-thromboembolic-pulmonary-hypertension
#9
Aiko Ogawa, Hiromi Matsubara
In the past 5 years, balloon pulmonary angioplasty (BPA) for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are deemed inoperable has undergone significant refinement. As a result, the procedure is now used worldwide and has become a promising therapeutic option for those patients. However, pulmonary endarterectomy remains the gold standard treatment for patients with CTEPH because the techniques and strategies for BPA are not yet unified. The best therapeutic option for each patient should be determined based on discussion among a multidisciplinary team of experts...
April 17, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29659150/chronic-obstructive-pulmonary-disease-in-patients-with-chronic-thromboembolic-pulmonary-hypertension-prevalence-and-implications-for-surgical-treatment-outcome
#10
Oksana Kamenskaya, Irina Loginova, Alexander Chernyavskiy, Aleksander Edemskiy, Vladimir V Lomivorotov, Aleksander Karaskov
OBJECTIVE: The aim of our study was to investigate the prevalence of chronic obstructive pulmonary disease (COPD) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and examine their impact on the results of pulmonary thrombendarterectomy (PEA). METHODS: We enrolled 136 patients with CTEPH who scheduled for elective PEA. Pulmonary function tests (PFTs) including full-body plethysmography with bronchodilation test and lung diffusion capacity assessment were performed in all patients prior to surgery treatment...
April 16, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29649782/balloon-pulmonary-angioplasty-in-a-patient-with-prostate-cancer-and-chronic-thromboembolic-pulmonary-hypertension
#11
Yolanda Bryce, Ernesto Santos
Chronic thromboembolic pulmonary hypertension (CTEPH) is a subtype of pulmonary hypertension characterized by a mean pulmonary artery pressure ≥25 mm Hg due to obstructive fibrotic thromboembolic material in the pulmonary arteries. Malignancy is a significant risk for developing CTEPH. The option for inoperable CTEPH is largely medical with variable outcomes. Balloon pulmonary angioplasty (BPA) is a promising option for inoperable patients given distal-type CTEPH, co-morbidities, or residual pulmonary hypertension after surgery...
April 6, 2018: Clinical Imaging
https://www.readbyqxmd.com/read/29643129/vessel-stretching-is-a-cause-of-lumen-enlargement-immediately-after-balloon-pulmonary-angioplasty-intravascular-ultrasound-analysis-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#12
Hiroto Shimokawahara, Aiko Ogawa, Hiroki Mizoguchi, Hiroki Yagi, Hidekazu Ikemiyagi, Hiromi Matsubara
BACKGROUND: Balloon pulmonary angioplasty (BPA) has become an alternative therapy for patients with chronic thromboembolic pulmonary hypertension who are ineligible for pulmonary endarterectomy. We retrospectively investigated intravascular ultrasound measurements to elucidate how the lumen enlarges immediately after BPA. METHODS AND RESULTS: A total of 326 lesions in 71 patients with chronic thromboembolic pulmonary hypertension were analyzed. The cross-sectional areas (CSAs) of the external elastic membrane and lumen measured by intravascular ultrasound before and after BPA were assessed for each lesion type (type A, ring-like stenosis lesions; type B, web lesions; type C, subtotal occlusion lesions)...
April 2018: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/29618607/chronic-thromboembolic-pulmonary-hypertension-after-acute-pulmonary-embolism-to-screen-or-not-to-screen
#13
EDITORIAL
Marius M Hoeper
No abstract text is available yet for this article.
April 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29615545/pulmonary-endarterectomy-for-patients-with-chronic-thromboembolic-disease
#14
Şehnaz Olgun Yıldızeli, Alper Kepez, Serpil Taş, Mehmed Yanartaş, Ali Fuad Durusoy, Atakan Erkılınç, Bülent Mutlu, Cihangir Kaymaz, Hasan Sunar, Bedrettin Yıldızeli
OBJECTIVE: Chronic thromboembolic disease (CTED) is characterized by persistent pulmonary thromboembolic occlusions without pulmonary hypertension. Early surgical treatment by performing pulmonary endarterectomy (PEA) may improve symptoms. The goal of the study was to review our experience and early outcome of PEA in patients with CTED. METHODS: Data were prospectively collected on all patients who underwent PEA between 2011 and 2015. Patients with CTED and a mean pulmonary artery pressure (mPAP) of <25 mm Hg were identified...
April 2018: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/29608809/chronic-thromboembolic-pulmonary-hypertension-from-the-perspective-of-patients-with-pulmonary-embolism
#15
REVIEW
Frederikus A Klok, Marion Delcroix, Harm Jan Bogaard
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment due to unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50% of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after very long delay...
April 2, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29593574/the-use-of-biophysical-flow-models-in-the-surgical-management-of-patients-affected-by-chronic-thromboembolic-pulmonary-hypertension
#16
Martina Spazzapan, Priya Sastry, John Dunning, David Nordsletten, Adelaide de Vecchi
Introduction: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) results from progressive thrombotic occlusion of the pulmonary arteries. It is treated by surgical removal of the occlusion, with success rates depending on the degree of microvascular remodeling. Surgical eligibility is influenced by the contributions of both the thrombus occlusion and microvasculature remodeling to the overall vascular resistance. Assessing this is challenging due to the high inter-individual variability in arterial morphology and physiology...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29580746/reveal-risk-score-in-patients-with-chronic-thromboembolic-pulmonary-hypertension-receiving-riociguat
#17
Raymond L Benza, Harrison W Farber, Adaani Frost, Ekkehard Grünig, Marius M Hoeper, Dennis Busse, Christian Meier, Sylvia Nikkho, Hossein-Ardeschir Ghofrani
BACKGROUND: The REVEAL risk score (RRS) was developed to predict survival in patients with pulmonary arterial hypertension (PAH), based on multiple patient characteristics. Herein we calculated RRS for patients in the randomized CHEST-1 study and open-label CHEST-2 extension study of riociguat in inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH). We investigated the effect of riociguat vs placebo on RRS in the CHEST-1 study, and the relationship between RRS and long-term outcomes in the CHEST-2 study...
March 1, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29579597/physiological-insights-of-exercise-hyperventilation-in-arterial-and-chronic-thromboembolic-pulmonary-hypertension
#18
Stefania Farina, Noemi Bruno, Cecilia Agalbato, Mauro Contini, Roberto Cassandro, Davide Elia, Sergio Harari, Piergiuseppe Agostoni
BACKGROUND: Pulmonary hypertension (PH) patients show, during exercise, an excessive increase in ventilation (VE ) compared to carbon dioxide output (VCO2 ), determining a high VE /VCO2 slope. There are several possible causes, including an elevated dead space ventilation (VD ), VE /perfusion (Q) mismatch and/or an enhanced peripheral or central chemoreceptor activity. We evaluated the causes of exercise hyperventilation in PH patients. METHODS: Eighteen group I and IV PH patients underwent cardiopulmonary exercise test with blood gas analysis at every minute...
May 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29572375/klippel-trenaunay-weber-syndrome-as-a-cause-of-chronic-thromboembolic-pulmonary-hypertension
#19
Jirat Chenbhanich, Nophol Leelayuwatanakul, Prasit Phowthongkum
No abstract text is available yet for this article.
March 22, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29563354/adiponectin-in-chronic-thromboembolic-pulmonary-hypertension
#20
Sarasa Isobe, Masaharu Kataoka, Takashi Kawakami, Keiichi Fukuda
BACKGROUND: The correlation between serum adiponectin concentration and hemodynamics or certain metabolic markers in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is unknown.Methods and Results:We enrolled 30 CTEPH patients who underwent interventional therapy of balloon pulmonary angioplasty or pulmonary endarterectomy. Serum adiponectin concentrations positively correlated with B-type natriuretic peptide (BNP) concentrations, pulmonary vascular resistance, and mean pulmonary arterial pressure...
March 21, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
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