Open AccessEvaluation and Management of Pulmonary Arterial Hypertension in Congenital Heart Disease
Author(s) -
Hassaan Arshad,
Valeria E. Duarte
Publication year - 2021
Publication title -
methodist debakey cardiovascular journal
Language(s) - English
Resource type - Journals
eISSN - 1947-6094
pISSN - 1947-6108
DOI - 10.14797/ufej2329
Subject(s) - medicine , eisenmenger syndrome , pulmonary hypertension , etiology , heart disease , cardiology , natural history , intensive care medicine , complication , disease
Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts. The choice of interventional, medical, or both types of therapy is an ongoing dilemma that requires further data. This review focuses on the evaluation and management of PAH-CHD in the contemporary era.