Provides a general overview of disease mechanisms and clinical challenges in adults with congenital heart disease who develop pulmonary arterial hypertension.
Underscores persistent clinical challenges in managing PAH in adults with congenital heart disease; leaves open gaps for targeted interventions and prospective research.
Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.
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Schulze‐Neick et al. (2007) studied this question.
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