Truncus arteriosus repair: outcomes, risk factors, reoperation and management

Objectives: Truncus arteriosus (TA) continues to be associated with signifi cant morbidity and mortality, but there have been clinically significant im provements with early repair. Methods: Sixty patients underwent physiologic al correction of TA between November 1978 and January 2000. The average age was 76 days (range, 3 days-20 months). Associated cardiac anomalies were f requently encountered, the most common being severe truncal valve regurgita tion (n = 7), interrupted aortic arch (n = 6), coronary artery anomalies (n = 6), non-confluent pulmonary arteries (n = 4), and total anomalous pulmon ary venous return (n = 1). Truncal valve replacement was performed initiall y or subsequently in seven patients with severe regurgitation (mechanical p rostheses in six patients and a cryopreserved aortic homograft in one patie nt). Right ventricle-pulmonary artery continuity was established with an ao rtic (n = 16) or pulmonary homograft (n = 32) in 48 patients, a Dacron poly ester porcine valved conduit in five, a non-valved polytetrafluoroethylene (PTFE) tube in three, direct anastomosis to the right ventricle with anteri or patch arterioplasty in three, and a bovine jugular venous valve conduit in one patient. Results: There were ten hospital deaths (17%; 70% confidenc e limit, 7-25%). Multivariate and univariate analyses demonstrated a relati onship between hospital mortality and associated cardiac anomalies. In the 43 patients without these associated cardiac anomalies, the early survival was 91% (group I). In the 17 patients with one or more of these risk factor s, the survival was 71% (group II, P = 0.002). There was one late death. Tw enty-three patients (46%) required reoperation for right ventricular outflo w tract (RVOT) obstruction at a mean follow-up time of 59.1 months. In 23 p atients, the RVOT reconstruction was performed with a PTFE monocusp, and si x patients had of a variety of replacement conduits inserted. Postoperative ly, there were 34 (68%) patients in New York Heart Association functional c lass I and 16 (32%) in class II. Twenty-eight surviving patients are report ed as doing well without any medication. The freedom of reoperation in the 39 hospital survivors (group 1) without risk factors was 64% at 7 years; an d 36% at 10 years in the I I patients (group II) surviving with risk factor s. Conclusions: Associated cardiac anomalies were risk factors for death af ter the repair of TA. In the absence of these associated lesions, TA can be repaired with an excellent surgical outcome in the neonatal and early infa ncy period. (C) 2001 Elsevier Science B.V. All rights reserved.