[Bland-White-Garland syndrome. Clinical aspects, diagnosis, therapy]

Abstract

The anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland Syndrome) is a rare congenital malformation reported to occur in 0.25-0.5% of all congenital cardiac anomalies. The clinical and pathomorphological picture can be classified into 2 types: infantile or adult. The infantile type is thought to lack coronary collaterals; this explains the bad prognosis and symptoms of non-operated patients. In the adult type collaterals are present or have developed in time to provide adequate blood supply to the myocardium. No history of cardiac complains and a nearly normal ECG are common findings. Echocardiography and cardiac catheterisation are mandatory diagnostic tools. The value of new methods like NMR, "stop-flow" angiography and color doppler flow mapping is still in discussion. The reimplantation of the coronary artery and Takeuchi-Operation are well established surgical methods. The time for the operation and prognostic outcome are related to the preoperative underlying myocardial damage measured by left ventricular function.