[A successful surgical repair of total anomalous pulmonary venous connection associated with Williams syndrome].

The surgical correction was performed successfully in a 14-year-old boy with total anomalous pulmonary venous connection (type Ia) associated with Williams syndrome. The diagnosis was made at the age of 3 and he had been well until the age of 13 when sudden chordal rupture caused severe mitral regurgitation and supraventricular tachyarrhythmia. The operational procedure includes common pulmonary vein and left atrium anastomosis through Gersony-Malm approach, chordal reconstruction of mitral valve with 4-0 Core Tex sutures. His postoperative course has been well without the sign of venous obstruction. As far as we know, this is the first documentation of a case of Williams syndrome associated with total anomalous pulmonary venous connection.