Persistent truncus arteriosus repaired beyond infancy

Patients with untreated Persistent Truncus Arteriosus (PTA) usually do not survive or develop irreversible pulmonary vascular obstructive disease beyond infancy. The present study reports the anatomic and hemodynamic data, and results of surgery in patients undergoing surgical repair of PTA beyond 1 year of age.

Between January 2000 and March 2012, 9 patients aged 1 year or more underwent complete surgical repair of PTA. The median age was 3 years (range, 1 year to 12 years). Median weight was 9 kg (range, 4.7 kg to 30 kg). Seven patients had type I PTA and two patients had type II PTA. Five patients had mild and two patients had moderate truncal valve regurgitation. The mean pre-operative oxygen saturation was 87 % (SD ± 5.07); mean indexed pulmonary vascular resistance was 9.1 units.m² (range, 4.5 units.m² to 12.3 units.m²). The right ventricular to pulmonary artery continuity was created by aortic homograft (n = 3), pulmonary homograft (n = 2) or valved bovine xenograft (n = 4). In one patient, the ventricular septal defect was closed with unidirectional valved patch. Follow up was complete for all patients.

There were two in-hospital deaths: one each due to sepsis and intractable pulmonary hypertension. Mean follow up duration was 39 months (range, 3 months to 138 months). There were no late deaths. One patient underwent conduit replacement secondary to aneurysmal dilatation of the conduit with clots leading to conduit obstruction 1 month after the initial operation. None of the patients underwent an additional procedure for truncal regurgitation, which at follow up was trivial in 4 and mild in 3 patients. All patients had good biventricular function and pulmonary hypertension subsided in all. All survivors have systemic saturation in excess of 95 %.

A few naturally selected patients with PTA are still suitable candidates for surgical repair after infancy and the early and mid-term outcomes are satisfactory.