BACKGROUND: Immediate results after surgical repair of the anomalous origin of the coronary artery are well known, but few studies reported midterm results in a relatively large population. METHODS AND RESULTS: Between 1980 and 1995, 39 consecutive patients with anomalous origin of the coronaries artery from the pulmonary artery had restoration of a two-coronary-artery system. Median age at surgery was 18.5 months (range, 2.7 months to 38 years). Left ventricular (LV) function was normal in 13 patients. Direct aortic implantation was performed in 34 patients (87%), associated with mitral valvuloplasty in 3 patients. Hospital death occurred in 5 patients (13%; 70% confidence limits [CL], 8 to 20). Two patients were lost to follow-up. Mean follow-up was 40 +/- 42 months. There was no late death after 1 month, and the survival rate was 84% (70% CL, 67 to 93). One patient had a mitral valvuloplasty 18 months after reimplantation. At the last follow-up, LV shortening fraction (SF) was normal in 86% (70% CL, 61 to 96), but LV dilation persisted in 73% of patients, and 12 of the survivors (39%) had abnormal regional wall motion of the left ventricle. A perfusion defect with incomplete redistribution was observed in 8 patients on thallium-201 imaging performed in 45% of survivors. Total mortality was related only to the preoperative SF: 12% versus 24.8% (P < .05). No factor was related to SF recovery. CONCLUSIONS: Despite no late deaths and SF recovery, LV dilation and ischemic segments of the left ventricle persisted at the long-term follow-up.
BACKGROUND: Immediate results after surgical repair of the anomalous origin of the coronary artery are well known, but few studies reported midterm results in a relatively large population. METHODS AND RESULTS: Between 1980 and 1995, 39 consecutive patients with anomalous origin of the coronaries artery from the pulmonary artery had restoration of a two-coronary-artery system. Median age at surgery was 18.5 months (range, 2.7 months to 38 years). Left ventricular (LV) function was normal in 13 patients. Direct aortic implantation was performed in 34 patients (87%), associated with mitral valvuloplasty in 3 patients. Hospital death occurred in 5 patients (13%; 70% confidence limits [CL], 8 to 20). Two patients were lost to follow-up. Mean follow-up was 40 +/- 42 months. There was no late death after 1 month, and the survival rate was 84% (70% CL, 67 to 93). One patient had a mitral valvuloplasty 18 months after reimplantation. At the last follow-up, LV shortening fraction (SF) was normal in 86% (70% CL, 61 to 96), but LV dilation persisted in 73% of patients, and 12 of the survivors (39%) had abnormal regional wall motion of the left ventricle. A perfusion defect with incomplete redistribution was observed in 8 patients on thallium-201 imaging performed in 45% of survivors. Total mortality was related only to the preoperative SF: 12% versus 24.8% (P < .05). No factor was related to SF recovery. CONCLUSIONS: Despite no late deaths and SF recovery, LV dilation and ischemic segments of the left ventricle persisted at the long-term follow-up.