The Bland-White-Garland syndrome - Analysis of 22 cases

BACKGROUND: The Bland-White-Garland syndrome is a rare congenital anomaly in which left coronary artery (LCA) originates from the pulmonary mink. It may be complicated by myocardial infarction, left ventricular dilatation end mitral insufficiency. Surgery is a treatment of choice. AIM: To assess diagnostic difficulties and long-term results of surgery in the Bland-White-Garland syndrome. METHODS: Retrospective analysis of the course of the disease, symptoms, results of non-invasive tests and surgical treatment in 22 children (13 gifts, 9 boys), aged between 2 months and 12 years, treated in 4 centres. RESULTS: The most common symptoms were anxiety, sweating and frequent respiratory infections. Abnormal ECG findings suggesting ischaemia or old myocardial infarction were present in all patients. Cardiac enlargement on chest X-ray or echocardiography was found in 95% of patients. Using colour Doppler, retrograde flow in the LCA, its anomalous origin from the pulmonary think, and fight coronary artery dilatation were demonstrated in 15 patients. Fifteen patients underwent surgery: in 8 the Takeuchi method was used with mortality of 25%, and in 7 patients the LCA was transplanted to the aorta with mortality of 57% (difference NS). In three patients mitral valve repair or valve replacement were performed. CONCLUSIONS: (1) In children with ECG signs of ischaemia or infarction the Bland-White-Garland syndrome should be suspected. (2) Doppler echocardiography is a useful methods for diagnosing this condition. (3) Surgical treatment is effective in a significant proportion of patients.

Duke Scholars

Author Larry Bruce Goldstein Neurology, Stroke and Vascular Neurology