Redo mitral valve replacement in an adult atrioventricular septal defect heart with severe pulmonary hypertension: A case study

Abstract Background Pulmonary hypertension associated with left heart disease (Nice PH classification group-II) improves when left heart disease is treated, but optimal medication for group-II PH is still controversial. Case presentation A 37-year-old female who had undergone patch closure for a partial atrioventricular septal defect (AVSD), and mitral valve replacement with a rather large bioprosthesis at juxtaannular position for mitral regurgitation was referred to our institute because of severe PH. Echocardiography revealed severe calcification of the bioprosthesis, and its stent post protrusion into the left ventricular outflow tract, therefore, redo mitral valve replacement at the supraannular position using a mechanical valve was performed successfully. Postoperatively PH gradually improved with meticulous medical management. Conclusions Narrowing of the left ventricular outflow tract in AVSD is well-known; however, potentially occurred left ventricular outflow tract obstruction (LVOTO) due to its stent post and extremely severe group-II PH due to severe calcification in bioprosthetic mitral valve should be minded.