Prognosis of mitral valve regurgitation and left ventricular function in dual coronary repair for anomalous origin of left coronary artery from pulmonary artery (ALCAPA)

Abstract

Abstract Introduction Uncorrected Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) leads to a mortality rate of 90% within the 1st year of life due to severe myocardial and sub-endocardial ischemia, fibrosis and ventricular arrhythmia. In addition, mitral valve regurgitation (MR) can occur as a result from valvular annular dilation or papillary muscle ischemia. Surgical re-implantation of the left coronary artery (LCA) to the aorta is the current golden standard. Postoperative complications include persistent MR, unrecovered left ventricular function and arterial stenosis. Importantly, the consensus regarding simultaneous mitral annuloplasty at the time of ALCAPA repair has not been solved. Purpose We reviewed our institutional experience with ALCAPA in which a dual coronary circulation had been surgically established to assess 1) preoperative variables predictive of outcome; 2) postoperative recovery of cardiac function; 3) short- and long-term postoperative complications; 4) prognosis of mitral regurgitation with and without simultaneous mitral annuloplasty Methods A retrospective, longitudinal study was carried out in 26 consecutive patients who underwent coronary reimplantation for the correction of ALCAPA from April 2010 to March 2017. The preoperative diagnosis was established by two-dimensional transthoracic echocardiography (TTE) and catheterization. Follow-up consisted to echocardiography early post-operative and at 6 months intervals. Results The group consisted of 12 boys (46.2%) and 14 girls (53.8%). Age at the time of operation ranged from 66 days to 19.81 years (4.09±5.49). Severe MR was present in 7 children (41.2%). Nine children (34.6%) underwent simultaneous MV valvuloplasty. There were 2 (7.7%) early post-operative mortalities. The follow-up time ranged from 6 to 60 months (25.95±16.8). Severe MR remained in 4 children (25%) who did not undergo valvuloplasty early post-operative as well as through follow-up. One child (5.88%) required re-operation for MV repair. In the group that underwent MV repair 3 children (33.3%) showed severe mitral regurgitation early post-operative. Severe MR remained in one child (11.1%) throughout follow-up, and another child (11.1%) developed MS at 28 months after surgical repair, which required re-operation. One (11.1%) patient presented with total occlusion of LAD. Improvement of LVEDd, LVESd and EF was not significantly related to age at the time of surgery, gender, weight or the performance of mitral valve repair. Mitral valve repair was not related to a significantly longer CPB or aortic cross clamp time, nor a longer duration of the ICU or hospital stay. Conclusions Our series shows excellent early and late outcomes regarding functional recovery after the successful reinstitution of a dual-coronary arterial system. Concomitant mitral valve repair should only be warranted in patients with severe MR as milder forms of MR tend to improve without intervention. Funding Acknowledgement Type of funding sources: None.