Advances in targeted therapy for chronic thromboembolic pulmonary hypertension

AbstractChronic thromboembolic pulmonary hypertension (CTEPH) is characterized by unresolved thrombi in the pulmonary arteries and microvasculopathy in nonoccluded areas. If left untreated, progressive pulmonary hypertension will induce right heart failure and, finally, death. Currently, pulmonary endarterectomy (PEA) remains the only method that has the potential to cure CTEPH. Unfortunately, up to 40% of patients are ineligible for this procedure for various reasons. In recent years, refined balloon pulmonary angioplasty (BPA) has become an alternative option for inoperable CTEPH patients, and it may be another curative treatment in the future, particularly in combination with prior PEA. Nevertheless, 23% of patients still suffer from persistent PH after BPA. Given that CTEPH shares many similarities with idiopathic pulmonary arterial hypertension (PAH), targeted drugs developed for PAH are also attractive options for CTEPH, especially for inoperable or persistent/recurrent CTEPH patients. To date, riociguat, macitentan, and subcutaneous treprostinil are the only drugs proven by randomized control trials to be capable of improving the exercise capacity (6-min walking distance) of CTEPH patients. In this review, we summarize the achievements and unresolved problems of PAH-targeted therapy for CTEPH over the last decade.
Source: Heart Failure Reviews - Category: Cardiology Source Type: research

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AbstractPurpose of reviewChronic thromboembolic pulmonary hypertension (CTEPH) is a major cause of precapillary pulmonary hypertension leading to right heart failure and death if left untreated. In addition to pulmonary endarterectomy, which is considered the standard of care, and specific drug therapy that is not however expected to offer relief from the mechanical component of the disease, the therapeutic options for CTEPH have expanded with the development of balloon pulmonary angioplasty (BPA). The purpose of this review is a better understanding and evaluation of BPA as a treatment option in CTEPH patients.Recent find...
Source: Current Treatment Options in Cardiovascular Medicine - Category: Cardiology Source Type: research
Backgrounds: Chronic thromboembolic disease (CTED) is characterized by similar symptoms and perfusion defect as chronic thromboembolic pulmonary hypertension (CTEPH), but without pulmonary hypertension at rest. Recently, successful pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA) for symptomatic patients with CTED has been reported. However, it remains unclear whether CTED evolves to CTEPH. The aim of this study is to clarify the natural history of CTED.Methods: Consecutive patients diagnosed as CTED between 1986 and 2017 were retrospective enrolled. CTED was defined as follows: a mean pulmonary arterial pressure at rest
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary hypertension Source Type: research
Abstract BACKGROUND: Although cardiac troponin and natriuretic peptide have been shown to decrease after balloon pulmonary angioplasty (BPA) with improved right ventricular afterload in chronic thromboembolic pulmonary hypertension (CTEPH), biomarkers to evaluate the effects of BPA independently of heart failure status remain to be developed. METHODS: In 39 consecutive CTEPH patients including 31 who underwent BPA, we measured plasma levels of cyclophilin A (CyPA), which we demonstrated is secreted from pulmonary vascular smooth muscle cells in response to mechanical stretch and hypoxia. RESULTS: CyPA le...
Source: Journal of Cardiology - Category: Cardiology Authors: Tags: J Cardiol 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
(CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment st...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Original Articles: World Symposium on Pulmonary Hypertension Source Type: research
Publication date: July 2018Source: Revista Española de Cardiología (English Edition), Volume 71, Issue 7Author(s): Julio SandovalAbstractDespite 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 t...
Source: Revista Espanola de Cardiologia - Category: Cardiology Source Type: research
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but severe complication of acute pulmonary embolism leading to right heart failure and premature mortality. Its cumulative incidence ranges from 0.1 –9.1% within the first 2 years after symptomatic acute pulmonary embolism, although probably underestimated due to the lack of systematic follow-up and screening in clinical routine.1–3
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Original Clinical Science Source Type: research
Publication date: Available online 13 March 2018 Source:Revista Española de Cardiología (English Edition) Author(s): 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 un...
Source: Revista Espanola de Cardiologia - Category: Cardiology Source Type: research
AbstractChronic thromboembolic pulmonary hypertension (CTEPH) has a poor prognosis because of the associated progressive right heart failure. Accurate evaluation of right ventricular (RV) function would thus be useful to predict prognosis. However, the significance of RV diastolic function remains unclear. We aimed to identify which echocardiographic measures are most accurate, and potentially useful, in assessing RV diastolic function in patients with CTEPH, and to study the effects of balloon pulmonary angioplasty (BPA) on them. We enrolled 53 CTEPH patients who underwent BPA. Echocardiographic parameters, including two-...
Source: The International Journal of Cardiovascular Imaging - Category: Radiology Source Type: research
Conclusions— This multicenter registry suggested improved hemodynamic results after BPA. Complication rates were high, but overall survival was comparable with pulmonary endarterectomy. BPA may be an important therapeutic option in patients with chronic thromboembolic pulmonary hypertension.
Source: Circulation: Cardiovascular Quality and Outcomes - Category: Cardiology Authors: Tags: Peripheral Vascular Disease Original Articles Source Type: research
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