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.
