Isolated congenital mitral valve regurgitation presenting in the first year of life.

AIM: Isolated congenital mitral regurgitation is rare and, when presenting in infancy, reflects severity of the malformation. The natural history is often fatal, and management during the first year of life remains a therapeutic challenge. These infants are poorly understood largely because of an absence of reporting in the medical literature and limited experience in each institution. We reviewed our own experience in order to add to the understanding of this condition.

METHODS

A retrospective review was performed on seven infants with significant isolated congenital mitral regurgitation. An associated patent ductus arteriosus was present in two. Mean age at referral was 17.7 weeks (1 day to 47 weeks) and mean weight was 6.8 kg (3.7-12.5 kg).

RESULTS

Two infants were managed conservatively, and one underwent surgical ligation of a patent ductus arteriosus. Following spontaneous and surgical duct closure, no further intervention was required in two infants. The remaining four infants underwent three valve repairs and three valve replacements. The in-hospital mortality was 29%, occurring in those under 1 year of age undergoing emergency valve surgery. Two reoperations followed mitral valve repair in the first year of life. No significant complications or late deaths occurred.

CONCLUSIONS

Our experience suggests closure of a patent ductus arteriosus should be undertaken prior to mitral valve surgery. There may be a poorer prognosis in those under one year of age requiring emergency mitral valve surgery. Those who can be managed conservatively or undergo mitral valve surgery as an elective procedure tend to have a better outcome.