Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era

Amadeo-José Wals-Rodriguez,Maria-Jose Rodriguez-Puras, Rocío Garcia-Orta,Juan Robledo,Eduardo Moreno, Carmen Federero, Rocio Camacho,Begoña Manso, Nuria Hernandez,Jose María Cubero, Raquel Ladrón Abia,Pilar Cejudo Ramos,Antonio Ordoñez,Pastora Gallego

Heart & Lung（2022）

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摘要

•Clinical phenotypes of PAH in adult CHD patients are associated with prognosis.•In this real-world study cohort, PAH-specific therapy has a low impact on mortality.•In advanced NYHA functional class combination therapy significantly improves exercise capacity.•Advanced stage of the disease at initiation of treatment involves a high risk of death.

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关键词

Congenital heart disease,Pulmonary hypertension,Eisenmenger,Pulmonary vasodilators

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