U. Kreutzer et al., SUCCESSFUL RESUSCITATION FROM VENTRICULAR-FIBRILLATION IN BLAND-WHITE-GARLAND SYNDROME IN ADULTHOOD - A CASE-REPORT, Zeitschrift fur Kardiologie, 87(7), 1998, pp. 560-565
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.