Pulmonary Hypertension in Adults with Congenital Heart Disease

Link to article at PubMed

Cardiol Clin. 2022 Feb;40(1):55-67. doi: 10.1016/j.ccl.2021.08.006.


Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis. The primary management strategy for most patients with PAH-CHD is medical therapy, although defect closure is considered in select cases.

PMID:34809917 | DOI:10.1016/j.ccl.2021.08.006

Leave a Reply

Your email address will not be published. Required fields are marked *