Clinical review for general practice

Bland–White–Garland syndrome (BWGS) is a relatively rare inborn pathology. The defect is detected in about 1 out of 300 000 a live newborns, which is 0.24–0.46% of all alive newborns. Anomalies in the arising of the coronary arteries may occur more often, causing coronary pathology, including myocardial infarction, and heart failure and sudden cardiac death. At the same time, the world literature describes sporadic cases of BWGS in the elderly. There are four variants of this pathology: anomalous origin of left coronary artery (LCA), right coronary artery (RCA), both coronary arteries or the accessory pulmonary artery. The most common variant is abnormal origin of the LCA – 90%, which, as a rule, branches from the left or posterior sinus of the pulmonary trunk. Abnormal origin of the RCA or both coronary arteries from the pulmonary trunk is less common, in only 10% of cases. With the infantile version of BWGS, counting for 10% of observations, there was an almost 100% mortality rate in children under one year of age. Other types of BWGS can have masks of coronary heart diseases – angina pectoris, myocardial infarction, post myocardial cardiosclerosis, dilated cardiomyopathy, mitral valve prolapse or insufficiency. These patients are characterized by sudden cardiac death, considering a variety of arrhythmias which they may have. The article describes the case of a 60-year-old patient with abnormal anomalous origin of the RCA from the pulmonary trunk, having a mask of coronary artery disease, equivalent to angina pectoris. 

1. Ishihata T, Takeda H, Katohno E et al. An adult case of Bland–White–Garland syndrome with collaterals from the bronchial artery. Heart Vessels 1994;9:218-22.