Tetralogy of Fallot with an abnormal coronary artery: surgical options and prognostic factors.

OBJECTIVES The objectives were to determine in patients with Tetralogy of Fallot (ToF) and abnormal coronary artery (ACA): the long-term outcomes of different surgical strategies; the risk factors for right ventricular outflow tract (RVOT) obstruction, reoperation, heart failure and mortality. To date, the surgical strategies and prognostic factors for repair of ToF with an ACA, crossing the RVOT and avoiding a classic repair, have not been evaluated in a large series using a multivariate analysis. METHODS A retrospective study (1986-2011) included 72 patients. The mean follow-up was 9.6 ± 6.8 years. Median age at surgery was 1.5 years (0.2-11.3). The main surgical techniques were 'tailored' right ventriculotomy and patch of the RVOT (63%; n = 45), implantation of a conduit between the right ventricle (RV) and the pulmonary artery (PA; 25%; n = 18) and a transatrial ± transpulmonary approach (11%; n = 8). Univariate and multivariate logistic regression analyses were performed. RESULTS Intrahospital mortality was 2.7%. Actuarial freedom from reoperation and actuarial survival at 15 years were 77% (confidence interval [CI]: 70-83%) and 94% (CI: 90-97%), respectively. Reoperations occurred more frequently after conduit implantation (50%) than after patch reconstruction (17%) or transatrial ± transpulmonary approach (0%; P = 0.002). The transatrial ± transpulmonary approach was significantly less complicated, with a long-term RVOT obstruction of 0% compared with the other surgical techniques (45.4%; P = 0.03). Implantation of a RV-PA conduit was an independent risk factor for RVOT obstruction (odds ratio [OR]: 31; P < 0.001) and reoperation (OR: 20; P = 0.02). An immediate postoperative right ventricle/left ventricle (RV/LV) pressure ratio >0.5 was independently associated with a long-term RV/LV pressure ratio >0.5 (OR: 14; P = 0.001), but was not a risk factor for reoperation (P = 0.8). Postoperative electric ischaemic signs independently increased the risk of long-term heart failure (OR: 22; P = 0.04). CONCLUSIONS The transatrial ± transpulmonary approach displays the best long-term outcomes, by reducing the risks for RVOT obstruction and reoperation, but does not improve the patient survival. A RV-PA conduit was an independent risk factor for RVOT obstruction and reoperation. An immediate postoperative RV/LV pressure ratio >0.5 was not a risk factor for reoperation. The transatrial ± transpulmonary approach should be preferred to the implantation of a conduit or a tailored right ventriculotomy whenever possible.