The prevalence of pulmonary arterial hypertension (PAH) in congenital heart anomalies is rising, because an increasing proportion of these patients now reach adulthood. However, morbidity and mortality rates in these patients are modified by the existing cardiac anomaly and thus differ from idiopathic pulmonary hypertension (IPAH). There are, in addition to Eisenmenger's syndrome, special forms such as local PAH or hemodynamically relevant increased pulmonary vascular resistance associated with a dysfunctional or absent right ventricle. In all these forms of PAH it is the therapeutic aim to achieve pulmonary vascular dilatation to reduce symptoms of right ventricular stress and to increase pulmonary blood flow and raise systemic oxygen supply. Just as in IPAH, intravenous, inhaled or oral medications--prostanoids, endothelin-receptor inhibitors, phosphodiesterase-5 inhibitors--are being used with increasing success.