Two adult cases of Bland–White–Garland syndrome with lethal arrhythmia due to coronary steal phenomenon during physical or mental stress

Abstract

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. <Learning objective: We experienced two rare cases of patients who were admitted with ventricular tachycardia and ventricular fibrillation who had adult type Bland-White-Garland syndrome during exertion in daily life. It is important to be aware of congenital heart disease in ordina...

What caused this patient's cardiac arrest?

“…Over time, myocardial fibrosis and scarring develop because of chronic ischemic changes related to this coronary steal phenomenon, or rerouting of coronary blood flow from high-resistance coronary vessels to low-pressure pulmonary circulation. 7 A few histologic studies have reported higher vascular resistance and greater arteriolar wall thickening in patients with alcapa, which causes the coronary slow flow phenomenon identified during coronary angiography. 8 Adults with alcapa may present with dyspnea, chest pain, palpitations, dysrhythmia, myocardial infarction, cyanosis, syncope, mitral regurgitation, cardiac arrest, or sudden cardiac death.…”

“…These adaptations allow perfusion of the LV in a retrograde fashion. Over time, myocardial fibrosis and scarring develop because of chronic ischemic changes related to this coronary steal phenomenon, or rerouting of coronary blood flow from high-resistance coronary vessels to low-pressure pulmonary circulation 7. A few histologic studies have reported higher vascular resistance and greater arteriolar wall thickening in patients with ALCAPA, which causes the coronary slow flow phenomenon identified during coronary angiography 8…”

Adult ALCAPA: from histological picture to clinical features

“…A modified-Takeuchi procedure was performed, and an ICD was implanted. The patient's postoperative course was uneventful, and as of 11.5 years after the surgical correction, he is alive and well with no complications and no ICD activations [10)].…”