Intracoronary shunt use in an adult patient with Bland-White-Garland syndrome

Abstract

Bland-White-Garland sendromlu yetişkin hastada intrakoroner şant kullanımıThe anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA or Bland-White-Garland syndrome) is a rare congenital heart defect with an incidence of 1 in 300.000 live births (1). Although it is the most common congenital coronary artery anomaly comprising 0.24%-0.46% of all congenital cardiac anomalies, only 10% of patients survive to adulthood (1, 2).A 44 -year old man was referred to our clinic because of chest pain on exertion. Left main coronary artery (LMCA) could not be visualized during conventional coronary angiography. Computed tomography angiography revealed abnormal origin of the left coronary artery from the pulmonary artery (PA) and muscular bridging of the left anterior descending (LAD) coronary artery (Fig. 1). During the operation, aortic arterial and bicaval venous cannulation, intermittent antegrade and retrograde blood cardioplegia were used. Right coronary artery was aneurysmatic due to collateral flow ( Fig. 2A, B). Through the pulmonary arteriotomy, LMCA orifice was seen to be originating from the left posterior sinus of PA and it was closed primarily by Teflon pledgeted 4/0 polypropylene sutures. Excessive systemic collateral flow was impeding exposure despite a properly placed aortic cross-clamp and a fully arrested and vented heart. Intracoronary shunts (ClearView ® Medtronic Inc. Minneapolis, MN, USA) were used to divert flow away from the site of anastomosis. Left internal mammary artery and a saphenous vein graft were anastomosed to LAD and circumflex artery respectively. The patient was discharged on the sixth postoperative day after an uneventful recovery. He is well and free of ischemic symptoms in his second year following the operation.In untreated adult ALCAPA patients, sudden death secondary to myocardial ischemia and cardiomyopathy is seen with an incidence of 80-90% at a mean age of 35 years (1, 2). Although establishing dual coronary circulation in childhood is a common practice, treatment of this anomaly in adult patients is controversial. Among various treatment options, establishing dual coronary circulation seems to provide better survival and significant recovery in ventricular functions (1, 3, 4). We performed combined simple closure and coronary artery bypass procedure to establish dual coronary circulation.About 10% of ALCAPA patients can survive to adulthood by welldeveloped intercoronary and extra coronary systemic collaterals (mainly from the bronchial arteries) (2, 4). Excessive collateral flow is a challenge during administration and maintenance of cardioplegia and coronary anastomoses in these patients (3)(4)(5). In order to facilitate anastomoses, several techniques have been suggested such as gentle compression of atrioventricular groove (3) but to our knowledge, this is the first report suggesting use of an intracoronary shunt during such an on-pump procedure. Contrary to the common knowledge that intracoronary shunts are used in off-pump surgery, we used them in ...