[Hypoplastic left heart with transposition of the great arteries].

Hypoplastic left heart syndrome (HLHS) is a rare congenital cardiovascular malformation with a fatal outcome, despite increasing cardiosurgical therapeutic options. Experiences in three cases are reported which were diagnosed as HLHS in the uncommon combination with transposition of the great arteries (TGA). Pathophysiology, clinical features and diagnostic approaches of the "classic" HLHS and the version with TGA are compared. In contrast to the poor clinical outcome, due to impaired systemic and coronary perfusion in patients with classic HLHS, HLHS with TGA leads to reduced pulmonary perfusion, which can be relieved by aortopulmonary shunt operation. After successful operation, two patients showed normal development during the follow-up period of 18 months and 2 years resp. Because of different therapeutic and prognostic consequences in the two lesions, the necessity to determine topographic relations of great arteries in HLHS is stressed.