Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery

Objective: The aim of this study was to review the results of operations fo r anomalous left coronary artery from the pulmonary artery and the late out come for exercise capacity, left ventricular function, and mitral regurgita tion, Methods: Twenty-one patients underwent operations over an 18-year per iod (median age, 9 months; range, 6 weeks-26 Sears) with a median follow-up of 6.5 years (range, 2 months-18 years). In addition to clinical and echoc ardiographic follow-up, patients at our institution were also investigated with radionuclide scans (n = 10) and treadmill exercise testing (n = 8), Re sults: There were no operative or late deaths (0%; 95% confidence interval [CI], 0% and 16%). Five patients required support with a left ventricular a ssist device. Eighteen patients are currently in New York Heart Association class I, and 3 patients are mildly symptomatic. On nuclear gated scan at a mean of 6 years after the operation, the left ventricular ejection fractio n was 64% (SD, 3%) at rest and increased to 74% (SD = 3%) on exercise (95% CI for the difference, 6%, 14%; P =.001). Treadmill endurance was normal fo r age (9.8-14.5 minutes) in those old enough to exercise, On echocardiograp hy (n = 18), the current fractional shortening was 34% (SD, 4%) in the 15 p atients with normal or only mildly abnormal ventricular septal motion. Thre e patients have undergone mitral valve operations. The left ventricular end -diastolic dimension fell from 48 mm (SD 5.8 mm) before surgery to 35.1 mm (SD, 5.2 mm) at 1 year after the operation, and the fractional shortening i ncreased over the first year from 19.6% (SD, 9.3%) to 32.8% (SD, 5.9%; both P <.001). Conclusions: Long-term clinical outcome and left ventricular fun ction are good, despite severe left ventricular dysfunction at presentation .