Anomalous Origin of the Left or Right Coronary Artery from the Pulmonary Artery (ALCAPA/ARCAPA): A 10-year single center experience

Introduction: Patients with ALCAPA/ARCAPA often present with severely impaired ventricular function and mitral regurgitation. Nowadays coronary transfer is regarded as first line surgical treatment. Aim of the study: To present our single center ten year experience with treatment of patients with ALCAPA/ARCAPA. Methods: Retrospective analysis. All patients with a diagnosis of ALCAPA (n=18)/ARCAPA (n=3) admitted to our hospital from 1999–2009 were included.

Results: Median patient age was 6 months (range 13 days to 17,5 years). Clinical status and left ventricular function (LVFx) at time of admission was severly impaired in 9 (43%), moderately impaired in 4 (19%), and normal or mildly reduced in 8 patients (38%). In 10 patients (48%) mitral regurgitation was grade 2 or greater. All patients underwent surgical treatment (direct reimplantation, n=20, 95%, bypass procedure n=1, 5%). One patient had simultaneous mitral annuloplasty. Mean follow up was 3,5 years (range 4 month to 9,7 years) No early or late mortality was seen. Left ventricular assist devices (LVAD) were used in five patients (24%). At last follow up all patients were in NYHA class I. 20 patients (95%) had a mildly or normalized LVFx. In 4 patients (19%) a mitral regurgitation grade 2 or 3 was evaluated. Coronary stenosis occurred in three patients, one was treated with coronary stent implantation. Conclusions: Coronary reimplantation in patients with ALCAPA or ARCAPA is a surgical procedure with low mortality. In patients with severe impaired ventricular function implantation of LVAD is a usefull postoperative cardiac support technique. Left ventricular function usually normalizes over time. Mitral valve regurgitation often improves along with ventricular function. Coronary stenosis may occur as early or late postoperative complication.