Coarctation of the Aorta with Congenital Mitral Regurgitation

Among 861 infants and children with coarctation of the aorta examined between 1950 and 1973, inclusive, 18 (2.1%) also had congenital mitral regurgitation (MR).Resection of the coarctation of the aorta was performed in 16 of these 18 patients (89%). There were three operative deaths early in the series, giving a total mortality rate of 18% since 1950. Over the past decade, mortality in this group has been reduced to zero. Mitral valve replacement was performed following resection of the coarctation in 2/13 patients (13%), both successfully. The follow-up of the 13 postoperative patients and the two who have not undergone surgery has ranged from 5 to 18 years, with a median of nine years. At the last examination, an appreciable pressure gradient persisted between the right arm and the legs in 4/15 patients (27%). Weight remained below the third percentile in none. The clinical picture of congestive heart failure was present in none, but significant cardiomegaly was found radiologically in 11/15 (73%) and the electrocardiogram remained abnormal in 8/15 (53%). Over the period of follow-up, the MR became clinically worse in 4/15 patients (27%), remained unchanged in 7/15 (47%), and improved following resection of the coarctation in 4/15 patients (27%).The pathologic anatomy of congenital MR associated with coarctation of the aorta was reviewed and classified. Two unique cases were presented — rupture of chordae tendineae and perforation of the posterior leaflet, both apparently congenital. When mitral surgery becomes necessary, an understanding of the pathologic anatomy of congenital MR may well make it possible to avoid mitral valve replacement in selected cases.