Ultrasound diagnostics of Bland-White-Garland syndrome, difficulties and errors

Anomalous left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome (BWGS) occur in 0.45-0.24% of all congenital heart disease (CHD) cases. 80-90% patients die without operation during their first year of life. There are two types of ALCAPA syndrome: the infant type and the adult type. Aim. Objective of the study was to evaluate the ultrasound diagnostic signs of BWGS in children and causes of errors. Methods. Total database comprised 6912 cases; 19 BWGS patients aged 12 days to 10 years were examined. Echocardiography, ECG, and coronarography were performed. Results and discussion. Isolated ALCAPA was diagnosed in 16 patients (0.23%); combination of ALCAPA with other CHD was diagnosed in 3 patients (0.04%). The ultrasound signs of BWGS were releases of the left coronary artery (LCA) from the pulmonary trunk, reverse flow from the lateral wall of PA, collateral flows between the RCA and LCA, LV remodeling with myocardial dysfunction, and mitral regurgitation. Diagnostic difficulties were due to the fact that BWGS “wears masks” of other diseases such as: myocarditis, dilated cardiomyopathy, mitral insufficiency, and myocardial infarction. Left ventricular remodeling can be associated with endocardial fibroelastosis. Direct visualization of the LCA arising from the pulmonary artery is not 100%-specific diagnostic sign which remain undetected by sonographer. The presence of reverse blood flow in the pulmonary trunk depends on pathophysiological phase, pulmonary pressure, and the development of collateral circulation. Therefore, it cannot be detected in some patients. In some patients, only collateral flows were present. Sometimes, mitral regurgitation due to ischemic genesis is underestimated and its causes are not determined. Thus, differential diagnosis is required for children with signs of LV remodeling and heart failure in the presence of mitral regurgitation and BWGS; the evaluation of coronary arteries should be included in the Echo algorithm.