[A case of mitral valve re-replacement with radical correction of Bland-White-Garland syndrome].

A case of an 8-year-old girl with Bland-White-Garland syndrome (BWG) who successfully underwent a second mitral valve replacement is reported. The patient had a past history of mitral valve replacement under the diagnosis of mitral regurgitation and pulmonary hypertension at one year and 3 months of age. Cardiac catheterization at 6 years and 3 months of age revealed an anomalous origin of the left coronary artery from the pulmonary artery but she was asymptomatic. One year and 7 months later, she was admitted to our institution with a sudden onset of chest pain and respiratory distress. Right heart pressure study using a Swan-Ganz catheter showed severe pulmonary hypertension and elevated mean pulmonary wedge pressure (38 mmHg). Echocardiogram revealed severe mitral stenosis and moderate regurgitation. She was diagnosed under fluoroscopy as acute left cardiac failure due to stuck valve and suspected having myocardial damage due to BWG, thus, emergency surgery was performed. Under cardiopulmonary bypass, aorto-left coronary arterial continuity was established by intrapulmonary arterial tunnel using Hamilton's technique. After resection of the prosthetic valve, of which one of two leaflets had been fixed in pannus, re-MVR was done with a St. Jude. Medical valve (25 mm). Postoperative angiogram demonstrated satisfactorily reconstructed two coronary artery systems. Postoperatively, she has been doing well without complaint for the last eighteen months.