In adults with congenital heart disease and pulmonary arterial hypertension, disease-targeting therapies offer symptomatic improvement, though survival benefits are not yet established.
Supports symptomatic use in congenital heart disease-PAH; leaves open survival benefit pending higher-level evidence.
A significant number of adult patients with congenital heart disease suffer from pulmonary arterial hypertension leading to a markedly increased morbidity and mortality. Some defects may be eligible for operative or interventional repair in adulthood but careful selection of candidates is crucial. With the emergence of disease-targeting therapies, symptomatic improvement and stabilization have become possible while the impact on survival currently remains unclear.
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Radke et al. (2013) studied this question.
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