IntroductionAnomalous origin of the left coronary artery from the pulmonary artery (ACLAPA) is widely called BWG syndrome, and was first reported by Bland, White, and Garland in 1933 [1]. This is a rare congenital heart disease with prevalence of one in 300,000 births or 0.25-0.5% of all congenital heart disease [2]. The left main coronary artery (LMCA) originates from the main pulmonary artery in more than 90% of cases of BWG syndrome. We herein report two notable adult BWG syndrome cases that presented with ventricular tachycardia (VT) during exertion in daily life, and recovered well with cardiopulmonary resuscitation (CPR) by appropriate use of an automated external defibrillator (AED).
Case 1A 42-year-old man suddenly collapsed while jogging. Consciousness level was Glasgow Coma Scale (GCS) 1-1-1 at the time the rescue team arrived, and bystander CPR had been performed until then. The electrocardiographic (ECG) monitor showed VT and ventricular fibrillation (VF) when the AED was attached. The AED was operated 7 min after the patient collapsed. ECG showed normal sinus rhythm with ST depression in the chest leads on arrival at our hospital.After administration of amiodarone, VT did not reoccur during the course of admission. Multi-detector computed tomography coronary angiography (CTA) was performed because of suspected ischemic arrhythmia, and showed that the left coronary artery (LCA) originated from the pulmonary artery with marked collateral flow from the right coronary artery (RCA) to the LCA, consistent with BWG syndrome (Fig. 1a).Invasive coronary angiography (CAG) showed that the RCA and conus branch were well developed and provided good collateral flow to the left anterior descending artery and left circumflex Journal of Cardiology Cases xxx (2016) xxx-xxx Anomalous origin of left coronary artery from pulmonary artery Ventricular tachycardia Congenital
A B S T R A C TWe experienced two adult cases of anomalous origin of the left coronary artery from the pulmonary artery, so-called Bland-White-Garland (BWG) syndrome, that presented with ventricular tachycardia (VT) and ventricular fibrillation during exertion in daily life. They presented to our hospital with syncope due to VT, and recovered following application of an automated external defibrillator with cardiopulmonary resuscitation. We diagnosed BWG syndrome by multi-detector computed tomography angiography and coronary angiography. We analyzed the mechanisms of lethal arrhythmias in relation to myocardial ischemia on exertion. Coronary flow modification and implantable cardioverter defibrillator implantation were performed in order to prevent future lethal arrhythmia due to myocardial ischemia. It is important to be aware of congenital heart disease in ordinary cases.