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Open Access Pulmonary Arterial Hypertension Medical Management of the Adult Patient with Congenital Heart Disease

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This article is Open Access under the terms of the Creative Commons CC BY-NC licence.

Congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation. CHD-associated PAH has an estimated prevalence of 5‐10% in adult patients, with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension (PAH)-targeted pharmacotherapy. Although limited data exist, targeted PAH pharmacotherapy has proven to be beneficial in patients with CHD-associated PAH, with observed improvement in functional class, increase in exercise capacity, and improvement in quality of life and cardiopulmonary hemodynamics. Additionally, there has been increasing interest in the “treat-to-close” strategy. PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’ operability in repairing the cardiac defect. Although there have been significant advances in the management of this disease state in the past 2 decades, mortality remains high, and ongoing clinical trials are needed to better understand the treat-to-close strategy.
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Keywords: Eisenmenger syndrome; arterial septal defect; congenital heart disease; pulmonary arterial hypertension; ventricular septal defect

Document Type: Research Article

Publication date: 01 May 2018

This article was made available online on 19 April 2018 as a Fast Track article with title: "Pulmonary Arterial Hypertension Medical Management of the Adult Patient with Congenital Heart Disease".

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  • Cardiovascular Innovations and Applications (CVIA) publishes focused articles and original clinical research that explore novel developments in cardiovascular disease, effective control and rehabilitation in cardiovascular disease, and promote cardiovascular innovations and applications for the betterment of public health globally. The journal publishes basic research that has clinical applicability in order to promote timely communication of the latest insights relating to coronary artery disease, heart failure, hypertension, cardiac arrhythmia, prevention of cardiovascular disease with a heavy emphasis on risk factor modification. Cardiovascular Innovations and Applications is the official journal of the Great Wall International Congress of Cardiology (GW-ICC). It aims to continue the work of the GW-ICC by providing a global scientific communication platform for cardiologists that bridges East and West.

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