Phillip S Naimo1, Tyson A Fricke1, Yves d'Udekem1, Andrew D Cochrane2, Andrew Bullock3, Terry Robertson4, Christian P Brizard1, Igor E Konstantinov5. 1. Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. 2. Department of Cardiothoracic Surgery, Monash Medical Centre, Melbourne, Australia. 3. Department of Cardiology, Princess Margaret Hospital for Children, Perth, Australia. 4. Department of Cardiology, Women's and Children's Hospital, Adelaide, Australia. 5. Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Electronic address: igor.konstantinov@rch.org.au.
Abstract
BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with limited data on long-term outcomes after surgical intervention. METHODS: We conducted a retrospective review of all children (N = 42) who underwent surgical repair of ALCAPA between 1980 and 2014 at the Royal Children's Hospital, Melbourne. RESULTS: Twenty-nine (69% [29 of 42]) patients underwent coronary reimplantation, 12 (29% [12 of 42]) had intrapulmonary baffle (Takeuchi) repair, and 1 (2% [1 of 42]) patient had ligation of the anomalous coronary artery. Nine (21%, 9 of 42) patients had concomitant mitral valve (MV) repair at the time of ALCAPA repair. A left ventricular assist device (LVAD) was used in 36% (15 of 42) of patients. Early mortality was 2.4% (1 of 42 patients). Median follow-up was 14 years (mean, 13 years; range, 4 months-31 years). There were no late deaths. Survival was 98% at 20 years. Freedom from reoperation was 81%, 81%, and 76% at 5, 10, and 20 years after operation, respectively. Eight patients underwent late MV repair or replacement at a median of 3 years (mean, 8 years; range, 2 months-25 years) after operation. Freedom from late MV repair or replacement was 86% at 5 and 10 years and 81% at 20 years after operation. Eleven (26% [11 of 42]) patients had severe mitral regurgitation (MR) preoperatively. Of those 11 patients, 5 (45% [5 of 11]) had concomitant MV repair at the time of ALCAPA repair, 3 (27% [3 of 11]) had late MV repair or replacement, and the remaining 3 (27% [3 of 11]) patients had mild MR at last follow-up. Thirty-six (90% [36 of 41]) patients had normal left ventricular function and 4 (10% [4 of 41]) patients had mildly reduced left ventricular (LV) function at last follow-up. CONCLUSIONS: ALCAPA can be operated on with good outcomes. Persistent MR and a moderate rate of late MV repair warrants close follow-up.
BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with limited data on long-term outcomes after surgical intervention. METHODS: We conducted a retrospective review of all children (N = 42) who underwent surgical repair of ALCAPA between 1980 and 2014 at the Royal Children's Hospital, Melbourne. RESULTS: Twenty-nine (69% [29 of 42]) patients underwent coronary reimplantation, 12 (29% [12 of 42]) had intrapulmonary baffle (Takeuchi) repair, and 1 (2% [1 of 42]) patient had ligation of the anomalous coronary artery. Nine (21%, 9 of 42) patients had concomitant mitral valve (MV) repair at the time of ALCAPA repair. A left ventricular assist device (LVAD) was used in 36% (15 of 42) of patients. Early mortality was 2.4% (1 of 42 patients). Median follow-up was 14 years (mean, 13 years; range, 4 months-31 years). There were no late deaths. Survival was 98% at 20 years. Freedom from reoperation was 81%, 81%, and 76% at 5, 10, and 20 years after operation, respectively. Eight patients underwent late MV repair or replacement at a median of 3 years (mean, 8 years; range, 2 months-25 years) after operation. Freedom from late MV repair or replacement was 86% at 5 and 10 years and 81% at 20 years after operation. Eleven (26% [11 of 42]) patients had severe mitral regurgitation (MR) preoperatively. Of those 11 patients, 5 (45% [5 of 11]) had concomitant MV repair at the time of ALCAPA repair, 3 (27% [3 of 11]) had late MV repair or replacement, and the remaining 3 (27% [3 of 11]) patients had mild MR at last follow-up. Thirty-six (90% [36 of 41]) patients had normal left ventricular function and 4 (10% [4 of 41]) patients had mildly reduced left ventricular (LV) function at last follow-up. CONCLUSIONS: ALCAPA can be operated on with good outcomes. Persistent MR and a moderate rate of late MV repair warrants close follow-up.