The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension

Pulmonary endarterectomy (PEA) is the gold standard treatment for operable chronic thromboembolic pulmonary hypertension (CTEPH). However, a proportion of patients with operable disease decline surgery. There are currently no published data on this patient group. The aim of this study was to identify outcomes and prognostic factors in a large cohort of consecutive patients with CTEPH. Data were collected for consecutive, treatment-naive CTEPH patients at the Pulmonary Vascular Disease Unit of the Royal Hallamshire Hospital (Sheffield, UK) between 2001 and 2014. Of 550 CTEPH patients (mean±sd age 63±15 years, follow-up 4±3 years), 49% underwent surgery, 32% had technically operable disease and did not undergo surgery (including patient choice n=72 and unfit for surgery n=63), and 19% had inoperable disease due to disease distribution. The 5-year survival was superior in patients undergoing PEA (83%) versus technically operable disease who did not undergo surgery (53%) and inoperable due to disease distribution (59%) (p
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Original Articles: Pulmonary vascular diseases Source Type: research

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CONCLUSIONS: ET-1 overexpression in the pulmonary artery preceded development of PH, but it was clearly and unexpectedly downregulated in the lungs of monocrotaline-treated rats and showed no correlation to disease progression markers. We speculate that endothelin-1 may play opposing roles in the lungs vs pulmonary artery in monocrotaline-induced PH. PMID: 31012341 [PubMed - as supplied by publisher]
Source: Experimental Lung Research - Category: Respiratory Medicine Tags: Exp Lung Res Source Type: research
ConclusionsBPA-induced improvement in hemodynamics was associated with the attenuation of SA in patients with CTEPH and SA. Therefore, close attention should be paid to SA in CTEPH patients, and SA should be re-evaluated after BPA to avoid overestimating its severity.
Source: Heart and Lung: The Journal of Acute and Critical Care - Category: Respiratory Medicine Source Type: research
Publication date: Available online 23 April 2019Source: Blood Cells, Molecules, and DiseasesAuthor(s): Katerina Larissi, Marianna Politou, Alexandra Margeli, Christos Poziopoulos, Pagona Flevari, Evangelos Terpos, Ioannis Papassotiriou, Ersi VoskaridouAbstractThe clinical manifestations of Sickle Cell Disease (SCD) include episodes of vascular occlusion, chronic hemolytic anemia and frequent infections. GDF-15, a multifactorial cytokine, is a member of the transforming growth factor- superfamily. Expression of the GDF-15 gene in cardiomyocytes, vascular smooth muscle cells, and endothelial cells is strongly upregulated in ...
Source: Blood Cells, Molecules, and Diseases - Category: Hematology Source Type: research
Conditions:   Hypertension, Pulmonary;   Biomarkers Intervention:   Other: A biobank will be created from blood samples taken at admission, days 3 and days 7 Sponsor:   Assistance Publique - Hôpitaux de Paris Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Pulmonary hypertension is a progressive and often fatal disease that frequently presents with dyspnoea on exertion and results in increased right ventricular afterload and right ventricular failure. Although cardiac catheterisation is required for a formal diagnosis, transthoracic echocardiography (TTE) has a central role as a screening tool in those with symptoms and those at risk for developing pulmonary vascular disease. Echocardiographic techniques can be employed to estimate pulmonary artery pressure and resistance, right atrial pressure as well as to derive indirect information about right heart structure and function.
Source: Heart, Lung and Circulation - Category: Cardiology Authors: Tags: Review Source Type: research
We have investigated a unique cell type, Blood Outgrowth Endothelial Cells (BOEC), as a cell-based gene therapy approach to pulmonary hypertension. BOEC are bona fide endothelial cells, obtained from peripheral blood, that can be expanded to vast numbers, and are amenable to both cryopreservation and genetic modification. We established primary cultures of rat BOEC and genetically altered them to over-express human eNOS plus green fluorescent protein (rBOEC/eNOS) or to express GFP only (rBOEC/GFP).
Source: Translational Research - Category: Research Authors: Source Type: research
Balloon pulmonary angioplasty (BPA) improves pulmonary hemodynamics in chronic thromboembolic pulmonary hypertension (CTEPH) patients. However, whether it affects the severity of sleep apnea (SA) remains unknown. We investigated the effect of BPA on the severity of SA in CTEPH patients.
Source: Heart and Lung - Category: Intensive Care Authors: Source Type: research
Authors: Paul A, Thomson VS, Refat M, Al-Rawahi B, Taher A, Nadar SK Abstract Despite the advances in the management of thalassaemia major, heart disease remains the leading cause of mortality in patients afflicted with this disorder. Cardiac involvement in thalassaemia encompasses a spectrum of disorders including myocardial dysfunction, arrhythmias, pulmonary hypertension and peripheral vascular disease. Although cardiac siderosis (accumulation of iron in cardiac myocytes) as a consequence of repeated blood transfusions is deemed to be the main aetiologic factor for myocardial dysfunction in transfusion dependent...
Source: Postgraduate Medicine - Category: Internal Medicine Tags: Postgrad Med Source Type: research
The incidence of congenital heart disease (CHD) is about 4 to 10 per 1000 live births. While severe forms of CHD are usually diagnosed in infancy or childhood, milder and more survivable CHD may first manifest in the adult or may be discovered incidentally on imaging studies performed for other reasons. It is important to identify CHD in adults to implement appropriate treatment and prevent complications such as progression of pulmonary hypertension and development of heart failure. The goals of this article are to illustrate the appearance on computed tomography of various forms of uncorrected CHD in adults and describe t...
Source: Journal of Thoracic Imaging - Category: Radiology Tags: Web Exclusive Content-Pictorial Essay Source Type: research
Authors: Yokokawa T, Sugimoto K, Nakazato K, Misaka T, Oikawa M, Kobayashi A, Yoshihisa A, Yamaki T, Kunii H, Ishida T, Takeishi Y Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease that leads to right-sided heart failure with electrocardiographic abnormalities. There are only a few reports about the effects of balloon pulmonary angioplasty for CTEPH on the electrocardiographic criteria of right ventricular hypertrophy. Objective To determine the effect of balloon angioplasty on electrocardiography in patients with CTEPH. Methods We evaluated electrocardiograms in 19 ...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
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