A very rare association of grown up congenital heart disease with severe rheumatic mitral stenosis: A case report

Abstract

Ebstein anomaly is a congenital abnormality of the tricuspid valve where is apical displacement of the septal leaflet as well as tethering and also in some cases sail like appearance of the lateral leaflet. This results in non coaptation of the tricuspid leaflets and produces tricuspid regurgitation, most of the cases. The size of the right ventricle depends on degree of the apical displacement of septal cusp of tricuspid valve. The apical displacement of the tricuspid leaflet produces the arterialized portion of the right ventricle and results in rudimentary functional right ventricle. For the diagnosis of Ebstein anomaly apical displacement of the septal or posterior leaflet in adults≥ 0.8 cm/m2 body surface area. A variety of cardiac abnormalities are associated with Ebstein’s anomaly, including atrial septal defect, patent foramen ovale, pulmonary stenosis or atresia and ventricular septal defect, conduction system abnormalities. Ebstein’s anomaly with mild & asymptomatic cases surgery usually not required in most of the cases. When patients of all ages are taken together, the predicted mortality is approximately 50% by the fourth or fifth decade. We present here an uncommon association and presentation of ebstein anomaly with severe rheumatic mitral stenosis with atrial fibrillation with congestive heart failure of a patient of 38 years which is mother of 6 children.