Pathophysiologie und Diagnostik komplexer angeborener Herzfehler

Pathophysiology and Diagnostics in Complex Congenital Heart Disease. The advances in pediatric cardiology and pediatric cardiac surgery have led to a long term survival of children with congenital heart defects of > 90%. A new patient population has developed, the adults with congenital heart defects (ACHD). Due to the mostly reparative and not corrective surgery of patients with complex congenital heart defects long term complications often occur. These complications have to be recognized in time and treated correctly. Thus the knowledge of the heterogenous anatomy, pathophysiology and hemodynamics of these heart defects are necessary. The optimal care of ACHD should be multidisciplinary and take place in special ACHD-centers in cooperation with the treating specialist in internal medicine or cardiologist. The whole spectrum of diagnostic modalities has to be provided in these centers. Exemplarily the most frequent cyanotic heart defect, tetralogy of Fallot, the different forms of transposition of the great arteries as well as the features of the univentricular circulation are presented with special emphasis on the anatomy, pathophysiology and diagnostic options for longterm follow-up. J Kardiol 2014; 21 (Pre-Publishing Online).