Congenital mitral valve regurgitation in adult patients

Congenital mitral valve regurgitation (MVR) is a rare disease found in adults. We report on our 5-year surgical experience with congenital MVR in adults.

We reviewed the data for 48 consecutive patients (26 men), aged >18 years (median, 42 years; range, 18-78 years) who underwent operations for severe congenital MVR between June 2005 and May 2010. Patients with atrioventricular septal defect were excluded.

Congenital MVR was preoperatively diagnosed in 28 cases (58%). The lesions consisted of annular dilation (100%), valvular cleft (58%), prolapsed leaflet (40%), papillary muscle abnormality (5%), commissure fusion (2%), and leaflet deficiency (2%). Mitral valve repair was performed in 42 cases (88%) by means of Carpentier techniques. The other 6 patients underwent mitral valve replacement; one of these patients died of ventricular fibrillation 2 days after surgery. There were no other hospital deaths or late mortality. At the last follow-up (median, 38 months; range, 2-50 months), all 47 patients were in New York Heart Association functional class I or II. Echocardiography evaluations for the 42 patients who underwent the repairs revealed that 32 (76%) of the patients had no or trivial MVR and 10 patients (24%) had mild MVR. No patient underwent reoperation.

Congenital MVR is rare and often misdiagnosed in adults. Mitral valve repair is feasible in the majority of patients, with excellent immediate and medium-term results.