Anomalous Origin of the Left Coronary Artery from the Pulmonary Trunk (Bland-White-Garland Syndrome): A Rare Congenital Heart Defect in a 7-Year-Old Child

Abstract

Objective: To demonstrate the capabilities of modern imaging modalities in diagnosis of Bland-White-Garland (BWG) syndrome (anomalous origin of the left coronary artery [AOLCA] from the pulmonary trunk) and surgical planning in a 7-year-old child.Materials and methods: We report a case of 7-year-old patient S. with a newly diagnosed complex congenital heart defect: AOLCA from the pulmonary trunk. To establish a diagnosis and assess the spatial anatomy of great vessels and the coronary bed, the patient underwent transthoracic echocardiography (TTE), coronary computed tomographic angiography (CCTA), and conventional coronary angiography. Cardiac magnetic resonance imaging (MRI) with delayed contrast enhancement was used to assess the morphological and functional parameters of the heart and fibrotic changes in the myocardium of the left ventricle (LV).Results: During TTE, a congenital heart defect was suspected: a coronary artery anomaly with LV systolic dysfunction and coronaryright ventricular fistulas. Preoperative CCTA detected AOLCA from the pulmonary trunk or BWG syndrome, whereas coronary-right ventricular fistulas were not confirmed. Based on the cardiac MRI findings, we diagnosed diffuse LV myocardial fibrosis (increased extracellular volume, 35%-37% in all segments) with areas of delayed intramyocardial accumulation, areas of focal fibrosis without signs of myocardial edema. The CCTA findings were confirmed by conventional coronary angiography and intraoperatively.Discussion: The technical capabilities of modern minimally invasive cardiac imaging modalities make it possible to determine all existing surgical risks at the preoperative stage and further prognosis in patients undergoing reimplantation for coronary artery anomalies.Conclusions: The algorithm for management of patients with suspected BWG syndrome should be determined based on the capabilities and safety of each cardiac imaging modality. Detailed and accurate assessment of coronary anatomy is a key criterion for choosing a diagnostic tool at the preoperative stage in patients undergoing reimplantation of coronary arteries. Preoperative cardiac MRI with assessment of severity of fibrotic changes in the myocardium should be used to plan management of patients with BWG syndrome and further control dynamics of morphological and functional parameters of the heart after reimplantation of coronary arteries.