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

OBJECTIVES: Truncus arteriosus (TA) continues to be associated with significant morbidity and mortality, but there have been clinically significant improvements with early repair.
METHODS: Sixty patients underwent physiological correction of TA between November 1978 and January 2000. The average age was 76 days (range, 3 days--20 months). Associated cardiac anomalies were frequently encountered, the most common being severe truncal valve regurgitation (n=7), interrupted aortic arch (n=6), coronary artery anomalies (n=6), non-confluent pulmonary arteries (n=4), and total anomalous pulmonary venous return (n=1). Truncal valve replacement was performed initially or subsequently in seven patients with severe regurgitation (mechanical prostheses in six patients and a cryopreserved aortic homograft in one patient). Right ventricle--pulmonary artery continuity was established with an aortic (n=16) or pulmonary homograft (n=32) in 48 patients, a Dacron polyester porcine valved conduit in five, a non-valved polytetrafluoroethylene (PTFE) tube in three, direct anastomosis to the right ventricle with anterior patch arterioplasty in three, and a bovine jugular venous valve conduit in one patient.
RESULTS: There were ten hospital deaths (17%; 70% confidence limit, 7--25%). Multivariate and univariate analyses demonstrated a relationship 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 factors, the survival was 71% (group II, P=0.002). There was one late death. Twenty-three patients (46%) required reoperation for right ventricular outflow tract (RVOT) obstruction at a mean follow-up time of 59.1 months. In 23 patients, the RVOT reconstruction was performed with a PTFE monocusp, and six patients had of a variety of replacement conduits inserted. Postoperatively, there were 34 (68%) patients in New York Heart Association functional class I and 16 (32%) in class II. Twenty-eight surviving patients are reported as doing well without any medication. The freedom of reoperation in the 39 hospital survivors (group I) without risk factors was 64% at 7 years; and 36% at 10 years in the 11 patients (group II) surviving with risk factors.
CONCLUSIONS: Associated cardiac anomalies were risk factors for death after 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 infancy period.