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Mitral Valve Insufficiency Does Not Predict Need for a Mitral Repair or Replacement in Patients with Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery
J. W. Brown, M. Ruzmetov, M. D. Rodefeld*, M. W. Turrentine. Indiana University School of Medicine, Indianapolis, IN,
BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) causes severe myocardial ischemia, which can produce papillary muscle dysfunction, annular dilatation secondary to global left ventricular (LV) dysfunction, and frank myocardial infarction with papillary muscle rupture. Mitral regurgitation (MR) secondary to the LV and/or papillary muscle dysfunction will usually improve in the absence of continuing ischemia. The aim of this study is to determine the influence of mitral valve (MV) insufficiency on early and late outcomes of patients with ALCAPA who underwent surgical repair.
METHODS: Twenty-five patients underwent ALCAPA repair by left coronary reimplantation over a 30-year period (median age, 4 months; range, 1 month-16 years) with a median follow-up of 7 years (range, 2 months-30 years). Before repair, 11 infants (44%) presented in extremis requiring ventilatory and inotropic support, and 16 infants (64%) presented with heart failure. MR was present in 18 patients (72%); mild in 10, moderate in 5, and severe in 3. No patient underwent MV repair at the time of ALCAPA repair.
RESULTS: Hospital survival was 96%. Three patients underwent MV repair at the mean time of 3.5 years (all with preoperative severe MR). The degree of MR gradually improved in all patients with preoperative mild and moderate MR. Echocardiographic follow-up demonstrated improved LV function in all children. No patient showed supravalvar pulmonary stenosis after repair.
CONCLUSIONS: Long-term clinical outcome and LV function are good, despite severe LV dysfunction at presentation. Mitral valve repair or replacement is generally not necessary at the time of ALCAPA repair. Persistent and significant MR is present in a small percentage of patients and can usually be managed by repair at a later date.
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