Pulmonary Hypertension associated with Congenital Heart Disease

Title:Pulmonary Hypertension associated with Congenital Heart Disease

Volume: 20 Issue: 3

Author(s): Prashanth Venkatesh*Erika B. Rosenzweig

Affiliation:

• Guerin Congenital Heart Program, Smidt Heart Institute, Cedars Sinai Medical Center, Los Angeles, CA 90048, USA

Keywords: Pulmonary arterial hypertension, congenital heart disease, shunt, eisenmenger syndrome, pulmonary vasodilator, lung transplantation. 1. INTRODUCTION

Abstract: Pulmonary hypertension in patients with congenital heart disease is associated with significant mortality, morbidity and health services utilization. The predominant subtype of pulmonary hypertension in these patients is pulmonary arterial hypertension (PAH). PAH associated with congenital heart disease (PAH-CHD) comprises up to one-third of all PAH cases globally and is most commonly associated with anatomically simple shunt lesions. A myriad of clinical phenotypes of PAH-CHD are seen across the spectrum of shunt size, location and directionality. A conceptual framework to categorize these patients based on pathophysiology is described. Contemporary data regarding the management of the varied phenotypes are reviewed, and a novel algorithm to guide decision-making with shunt closure in patients with PAH-CHD is provided. Further data spanning the spectrum of basic, translational and clinical science are much needed to further inform the management of this highly complex and heterogeneous population.

Cite this article as:

Venkatesh Prashanth*, Rosenzweig B. Erika, Pulmonary Hypertension associated with Congenital Heart Disease, Current Respiratory Medicine Reviews 2024; 20 (3) . https://dx.doi.org/10.2174/011573398X269590231026103636