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

Abstract

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 cm2 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 cm2 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.

Percutaneous Mitral Balloon Valvuloplasty in a Patient with Situs Inversus with Dextrocardia: A Case Report

“…"Situs inversus totalis refers to the heart being a mirror image situated on the right side with all visceral organs to be mirrored with incidence of approximately 1 in 12,000 people" [1]. "Distorted cardiac anatomy makes fluoroscopy guided transcatheter procedures difficult which become technically more challenging in the cases with percutaneous mitral balloon valvuloplasty (PMBV), where the cardiac malpositions substantially increase the complications beginning from interatrial septal puncture to left ventricular entry" [11][12][13].…”