Congenital cardiac malformations are often associated with pulmonary hypertension and structural changes of both, the larger and smaller vessels of the pulmonary circulation. Approximately 5 to 10 % of adults with congenital heart disease, surgically treated or untreated, develop pulmonary arterial hypertension of variable severity from mild to severe (Eisenmenger reaction). Until recently, medical treatment options for the affected patients were very limited. Meanwhile, the advent of new pulmonary vasoactive and antiproliferative substances (including endothelin receptor antagonists, phosphodiesterase-5-inhibitors, prostanoids) offer the option to correct abnormalities in pulmonary endothelial function and to improve the outcome of affected patients. Even patients with severe congenital cardiac malformations and Fontan-type circulation or patients with pulmonary atresia and aorto-pulmonary collaterals may benefit from these new treatment strategies. In any case, the complexity of congenital cardiac malformations when associated with abnormalities in the pulmonary circulation and/or pulmonary arterial hypertension, requires medical care and follow-up in specialized centers for (adult) congenital heart disease.
Georg Thieme Verlag KG Stuttgart, New York.