Review Articles

Evaluation and Management of Pulmonary Arterial Hypertension in Congenital Heart 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.


pulmonary arterial hypertensionPAH pathwayspulmonary hypertension
  • Year: 2021
  • Volume: 17 Issue: 2
  • Page/Article: 145-151
  • DOI: 10.14797/UFEJ2329
  • Accepted on 28 Apr 2021
  • Published on 1 Jul 2021
  • Peer Reviewed