SUCCESSFUL RESUSCITATION FROM VENTRICULAR-FIBRILLATION IN BLAND-WHITE-GARLAND SYNDROME IN ADULTHOOD - A CASE-REPORT

The Bland-White-Garland Syndrome represents the anomalous origin of th e left coronary artery of pulmonary trunk. Only 10 % of the patients r each adulthood. Clinical manifestations of the syndrome are angina, dy spnoe, ECG signs of ischemia, myocardial infarction, and death in chil dhood. We present the case of a 47 year old woman with Bland-White-Gar land Syndrome, who was resuscitated from ventricular fibrillation. The only symptom shown in her personal history was progressive dyspnoea i n the last 6 months, though mitral insufficiency was known since child hood. On echocardiographic examination, she showed an anterolateral in farction and a mitral insufficiency II. As operation procedure, the li gation of the left main coronary artery and bypass surgery with a left internal mammarian graft to the left descending branch of the left co ronary artery was chosen. The mechanism of onset of ventricular tachyc ardia in our patient is not known. Three pathophysiological mechanisms may be possible: (1) local ischemia caused by the shunt, (2) a reentr y circuit in the border zone of myocardial infarction, (3) electrical instability caused by endocardial fibrosis. As local ischemia and reen try circuit were widely excluded, only endocardial fibrosis could indu ce further ventricular arrhythmia. We therefore intended to implant an AICD to have the most possible safety for our patient. But this, post operatively was refused by the patient. In analogy to Coronary Artery Disease, the risk for sudden cardiac death postoperatively may be due to three factors: (1) presence of a reentrant circuit, (2) LV-function below 40 %, and (3) presence of endocardial fibrosis. Our patient sho wed a low risk for sudden cardiac death. On electrophysiological study , no ventricular tachycardia could be induced in our patient, indicati ng the absence of a reentry circuit. LV function exceeded more than 40 %. In Holter EGG, only few ventricular premature beats could be regis trated, indicating a low risk for sudden cardiac death in the presence of endocardial fibrosis. In the follow-up of fourteen months, the pat ient remained free from arrhythmic events.