Staged Surgical Repair of Truncus Arteriosus Communis in Critically Ill Neonates with Complex Congenital Malformations

Objective: The presence of complex congenital malformations in patients with truncus arteriosus communis (TAC) increases the mortality and morbidity of the required neonatal repair. This report describes neonates with TAC and other complex malformations who underwent a staged repair.

Method: 3 neonates underwent a staged TAC repair, involving an initial RV-PA valveless conduit and either aortic or pulmonary artery reconstruction followed by an intracardiac repair later in infancy.

Case Nr.	Age (d)	Weight (kg)	Cardiovascular defects	Other cong. malformations	Initial surgery
TAC: Patient demographic and surgery.
1	8	3.1	TAC type A4, IAA type A, preoperative cardiogenic schock	CHARGE syndrome, s/p omphalocele repair	Aortic arch repair, RV-PA-conduit
2	21	3.3	TAC type A1, ARSA (aberrant right subclavian artery)	CHARGE syndrome, cleft lip and palate, hydrocephalus, mult. cerebral dysgenesis	ARSA repair, RV-PA-conduit
3	7	2.8	TAC type A4, IAA type B, dysplastic truncal valve	none	Aortic arch repair, RV-PA-conduit

Results: There was no hospital death. 2 patients (cases 1 and 2) have proceeded to a biventricular repair, which consisted of VSD closure, takedown of the initial RV-PA conduit, and placement of a valved RV-PA conduit. A PA patch plasty was necessary in case 1. After their 2nd surgery, both patients were discharged from the hospital in excellent condition and are currently clinically stable. Patient 3 is awaiting further surgery under a stable condition. At the last follow up (range 3 months to 3 years), no re-do surgeries related to the aortic arch or truncal valve were required among survivors, no progressive truncal valve regurgitation was observed. Late pulmonary artery intervention with balloon dilatation and stent placement was required in case 1.

Conclusion: In patients with TAC, complete primary repair is preferred in our institution. In critically ill neonates and in the presence of complex cardiovascular malformation however, the complexities and the time-consuming surgery could be associated with a higher mortality. In these cases, a staged TAC repair shortens the myocardial ischemic and bypass time at initial surgery. Providing an unobstructed systemic outflow is essential to prevent the development of truncal valve regurgitation and congestive heart failure. Although not seen in this series, leaving a VSD along with a valveless RV-PA-conduit might cause RV volume overload.