Percutaneous Mitral Valvotomy in a Case of Situs Inversus Totalis and Juvenile Rheumatic Critical Mitral Stenosis: Case Report

Situs inversus totalis is a rare congenital disorder where the heart being a mirror image is situated on the right side of the body. Distorted cardiac anatomy makes fluoroscopy-guided percutaneous mitral valvotomy (PMV) technically challenging and there are only few reports of PMV in situs inversus totalis. Here we report a case where PMV was successfully done for situs inversus totalis with rare coincidence of juvenile rheumatic severe mitral stenosis in a 12-year-old boy with a few modifications of standard Inoue technique. He had exertional dyspnea of NYHA class III with initial mitral valve area (MVA) of 0.6 cm(2) and severe pulmonary arterial hypertension with features suitable for PMV. Femoral vein was accessed from the left side to align the septal puncture needle and balloon to facilitate left ventricular entry. Septal descent and puncture by Brockenbrough needle was performed in the right anterior oblique view with the needle facing 5 o'clock position. Accura balloon was negotiated across mitral valve in left anterior oblique and procedure was successfully executed. Echocardiography showed a well-divided anterior commissure with an MVA of 2.0 cm(2) and mild mitral regurgitation. In summary, PMV is safe and feasible in the rare patient with situs inversus totalis with few modifications of the Inoue technique.