Mid-Term Results of Congenital Supravalvar Mitral Ring Resection

Supravalvar mitral ring is a rare congenital anomaly leading to left ventricle inflow obstruction. We present our surgical experience and mid-term results in patients operated for supravalvar mitral ring and associated congenital heart defects.

A retrospective analysis was performed in order to evaluate the cases surgically treated with the diagnosis of supravalvar mitral ring between 2001 and 2014. Ten patients were identified, seven of whom had accompanying congenital heart defects. Median age at the operation was 4.5 years.

Ventricular septal defects were encountered in half of the cases. Two of the patients had mitral annular hypoplasia, another two had the components of Shone's complex. The mean preoperative gradient across the supravalvar mitral ring decreased from 14.1 ± 4.2 mmHg to 4.6 ± 2.2 mmHg. All patients are alive and remain in a good clinical condition after a mean follow-up of five years.

Supravalvar mitral ring is a surgically treatable cause of left ventricular inflow obstruction. Although residual gradients may be encountered in patients with mitral annular hypoplasia, surgical resection of the ring is encouraged.