Klinische Aspekte und Therapieoptionen bei Angeborenen Herzfehlern mit pulmonal-arterieller Hypertonie

Congenital defects of the heart and great vessels (CHD) are the most frequent congenital anomalies. Owing to an improved interdisciplinary management, about 90 % of CHD patients reach adulthood. Up to 10 % maintain or newly develop pulmonary arterial hypertension (PAH) over time, which impairs exercise tolerance and prognosis. According to the 3rd World Conference in Dana Point in 2008, PAH with CHD belongs to Group 1 of the PAH-classification. Several changes to this classification were proposed during the World Conference in Nice in 2013, but have not been implemented yet. Until recently, especially the severe forms of PAH due to CHD were assessed as hardly treatable. This has changed radically since the introduction of endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids. These substances have been shown to improve quality of life and prognosis of patients with PAH due to CHD. Further improvements are expected from drugs that are currently under development or in trials (e.g. macitentan). As the clinical care of these complex patients is challenging and even dangerous, it should be in the hands of proven experts for congenital heart defects (certified congenital cardiologists) and take place in connection to experienced, certified centers.