Introduction: Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation. Immediate surgical correction on diagnosis with the aim of restoring a two-coronary system circulation is the current standard of treatment. However, the management of the mitral valve regurgitation in these patients is controversial.

Methods: Fifty one patients of ALCAPA underwent coronary reimplantation over a 10-year period. The mitral valve was not addressed (except one patient with an organic basis) at the time of coronary implantation even in patients with moderate to severe mitral regurgitation. These patients were followed postoperatively with with serial echocardiography at six months and at two years thereafter to assess the improvement in ventricular and mitral valvular function.

Result: A total of 53 cases were studied. Mitral valve regurgitation of varying severity was seen in 51 (96.5%) of the 53 patients preoperatively. There were 5 postoperative deaths (9.6%). Postoperatively, after 6 months, mitral regurgitation was none to mild in 38 patients (84.4%, n= 45). Moderate to severe mitral regurgitation was seen in 7 patients (15.6%). At 2 years follow up, 43 patients (95.5%, n= 45) had none to mild mitral regurgitation. Only two patients had moderate to severe mitral regurgitation.

Conclusion: Reimplantation of anomalous coronary artery to the aorta must be the procedure of choice in patients with ALCAPA. Mitral valve repair is not required even in severe regurgitation, except when there is an organic basis of regurgitation.

Keywords: ALCAPA, mitral regurgitation, coronary reimplantation.

1. Brooks SJ. Two cases of abnormal coronary artery of the heart arising from the pulmonary artery: with some remarks upon the effect of this anomaly in producing cirsoid dilation of the vessels. J AnatPhysiol 1886; 20:26–32.
2. Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J 1959;21:149–61.
3. Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J ThoracCardiovascSurg 1992;103:1049–58.
4. Kirklin JW, Barratt-Boyes BG. Congenital anomalies of the coronary arteries. In: Cardiac surgery. New York: John Wiley, 1986:945–69.
5. Kreutzer C, Schlichter AJ, Roman MI, Kreutzer GO. Emergency ligation of anomalous left coronary artery arising from the pulmonary artery. Ann Thorac Surg 2000;69:1591–2.
6. Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403–25.
7. Laks H, Ardehali A, Grant PW, Allada V. Aortic implantation of anomalous left coronary artery. An improved surgical approach. J Thorac Cardiovasc Surg 1995;109:519–23.
8. Del Nido PJ, Duncan BW, Mayer JE Jr, Wessel DL, LaPierre RA, Jonas RA. Left ventricular assist device improves survival in children with left ventricular dysfunction after repair of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 1999;67:169–72.
9. Neirotti, ANijveld, M Ithuralde, M. Quaglin, C. Seara, L. Lubbers, J Schuller, R. Mollen. Anomalous origin of the left coronary artery from pulmonary artery: repair buy aortic implantation. Eur J Cardio-thorac Surg (1991) 5:368-72.

1. Mary Jane Barth, Bradley S. Allen, Mehmet Gulecyuz, Pipit Chiemmongkoltip, BettinaCuneo and Michel N. Ilbawi. Experience with an alternative technique for the management of anomalous left coronary artery from the pulmonary artery Ann Thorac Surg 2003;76:1429-1434.

11. Sanjay Theodore, Sameet Pathak, Soman Rema Krishna Manohar, Kumarlingan Gopalkrishnan Shyamkrishnan and Kurur Sankaran Neelkandan. Anomalous left coronary artery from pulmonary artery- Clinical profile, operative techniques and patient outcomes. Ind J Thorac Cardiovascular Surgery, 2005; 21: 148- 152.
12. Jin Z, Berger F, Uhlemann F, et al. Improvement in left ventricular dysfunction after aortic reimplantation in 11 consecutive paediatric patients with anomalous origin of the left coronary artery from the pulmonary artery. Early results of a serial echocardiographic follow-up. Eur Heart J 1994;15:1044–9.
13. Walid Ben Ali, Olivier Metton, Franc¸ois Roubertie, Philippe Pouard, Daniel Sidi, Olivier Raisky, Pascal R. Vouhe´. Anomalous origin of the left coronary artery from the pulmonary artery: late results with special attention to the mitral valve. European Journal of Cardio-thoracic Surgery 36 (2009) 244—249.
14. Anthony Azakie, Jennifer L. Russell, Brian W. McCrindle, Glen S. Van Arsdell, Lee N, Benson, John G. Coles and William G. Williams. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome. Ann ThoracSurg2003;75:1535-1541.
15. Ando M, Mee RB, Duncan BW, Drummond-Webb JJ, Seshadri SG, Igor Mesia CI. Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method. Eur J Cardiothorac Surg. 2002 Oct; 22(4):576-81.Vladimir Alexi-Meskishvili and Roland Hetzer Surgical intervention for anomalous origin of the left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 2001;122:635-636.

Dr Tasneem Muzaffar

Consultant CVTS, GMC, Srinagar