Isolated cleft of the mitral valve: clinical spectrum and course

Abstract

We have reviewed the clinical presentations, courses, and outcomes of 90 patients seen at Texas Children's Hospital from 1983 through 2008 who had an isolated cleft of the mitral valve without some form of endocardial cushion defect. Additional congenital cardiac defects were present in 61 of the 90 patients, 35 of whom had a congenital syndrome. Seven patients had isolated cleft of the mitral valve without other intracardiac defects, and in these 7 there was a progressive increase in the degree of mitral regurgitation during a median time of 26.5 months from diagnosis to surgery. The patients ranged from a gestational age of approximately 32 weeks to 21.9 years of age. No death was observed among the 39 surgical patients, including 32 who had additional cardiac defects. There was a significant reduction in the degree of mitral regurgitation in all patients who underwent surgery. Among the 51 patients who did not have surgery, the degree of regurgitation did not change significantly over the course of 1 to 27 years' observation. Isolated cleft of the mitral valve is an uncommon (but not rare) congenital malformation of the mitral valve that can cause all degrees of mitral regurgitation but can be managed medically or surgically.

Keywords: Adolescent; child; echocardiography, Doppler; heart defects, congenital/surgery/ultrasonography; infant; mitral valve insufficiency; mitral valve/abnormalities/ultrasonography; retrospective studies.

Fig. 2. This short-axis view of the left ventricle in diastole shows an isolated cleft of the septal leaflet of the mitral valve. The cleft “points” towards the left ventricular outflow tract (arrow).

Fig. 1. This short-axis echocardiographic view of the left ventricle in a patient with a complete atrioventricular septal defect shows the “cleft” between the anterior and posterior bridging atrioventricular valve leaflets in systole. The cleft “points” towards the inlet septum (arrow).

Fig. 3. A plot of the mitral regurgitation scores in 39 surgical patients, from the immediate preoperative echocardiogram to the last follow-up echocardiogram.

Fig. 4. Kaplan-Meier plot of the freedom from worsening mitral regurgitation from the 1st echocardiogram to the last follow-up echocardiogram, in 37 nonsurgical patients who had at least 2 echocardiograms. The numbers above the X axis are the numbers of patients available for follow-up at 3, 6, 9, 12, and 15 years.