[A successful operative case of congenital mitral stenosis associated with double outlet right ventricle, patent ductus arteriosus, persistent left superior vena cava and severe pulmonary hypertension in adult].
A 29-year-old woman, who was diagnosed as congenital mitral stenosis associated with DORV, PDA, PLSVC and severe pulmonary hypertension, was reported. The mitral valve, consisting of leaflet dysplasia, shortened and thickened chordae tendineae, obliteration of interchordal spaces, under-development of papillary muscle, and stenotic supravalvular membrane, was replaced with 25 SJM prosthesis. Simultaneously, patch closure of VSD and intrapulmonary direct closure of PDA was performed. Her postoperative course was uneventful and systolic pulmonary arterial pressure fell down from 95 mmHg to 56 mmHg. She is doing well after correction. To our knowledge, this is very rare corrective case with congenital mitral stenosis in adult to be reported in the literature in Japan.