Surgical repair for persistent truncus arteriosus in neonates and older children

Abstract

Objectives: Persistent truncus arteriosus represents less than 3% of all congenital heart defects. We aim to analyze mid-term outcomes after primary Truncus arteriosus repair at different ages and to identify the risk factors contributing to mortality and the need for intervention after surgical repair. Methods: This retrospective cohort study included 36 children, underwent repair of Truncus arteriosus in the period from January 2011 to December 2018 in two institutions. We recorded the clinical and echocardiographic data for the patients preoperatively, early postoperative, 6 months postoperative, then every year until their last documented follow-up appointment. Results: Thirty-six patients had truncus arteriosus repair during the study period. Thirty-one patients had open sternum post-repair, and two patients required extracorporeal membrane oxygenation. Bleeding occurred in 15 patients (41.67%), and operative mortality occurred in 5 patients (14.7%). Patients with truncus arteriosus type 2 (p = 0.008) and 3 (p = 0.001) and who were ventilated preoperatively (p < 0.001) had a longer hospital stay. Surgical re-intervention was required in 8 patients (22.86%), and 11 patients (30.56%) had catheter-based reintervention. Freedom from reintervention was 86% at 1 year, 75% at 2 years and 65% at 3 years. Survival at 1 year was 81% and at 3 years was 76%. High postoperative inotropic score predicted mortality (p = 0.013). Conclusion: Repair of the truncus arteriosus can be performed safely with low morbidity and mortality, both in neonates, infants, and older children. Re-intervention is common, preferably through a transcatheter approach.

Case report of rare congenital cardiovascular anomalies associated with truncus arteriosus type 2

“…Our patient therefore received a percutaneous cardiac intervention with implantation of an 8 × 18 mm stainless-steel drug eluting stent into the TA and a Rashkind maneuver for restrictive ASD on the 44th day post-partum, aiming to sustain circulation [ 13 , 14 ], as in interdisciplinary consensus a curative approach was not possible. A curative option for minor cases of cardiac anomalies associated to a TA is a Norwood or Fontane I or II surgery, in which the pulmonary artery is reconnected to the right heart using a graft and VSD is closed [ 13 , 14 ]. As this surgery is very difficult and the outcome often is not optimal, many peri- and postoperative complications occur [ 13 , 14 ].…”

“…A curative option for minor cases of cardiac anomalies associated to a TA is a Norwood or Fontane I or II surgery, in which the pulmonary artery is reconnected to the right heart using a graft and VSD is closed [ 13 , 14 ]. As this surgery is very difficult and the outcome often is not optimal, many peri- and postoperative complications occur [ 13 , 14 ]. Unfortunately, our patient was not stable enough to receive full anaesthesia and surgery, also the anatomical variant with only one common coronary artery made a curative approach impossible.…”

Low-Dose Dual-Source Computed Tomography for Evaluating Persistent Truncus Arteriosus Associated with Cardiovascular Anomalies: Comparison with Transthoracic Echocardiography

“…Accurate preoperative diagnosis of coronary artery anomalies is critical. Firstly, during the surgical repair of PTA, the right ventricle's infundibulum needs to be incised before restoration of right ventricle-pulmonary artery continuity [22]. Therefore, any malformed coronary artery that goes across the right ventricle's infundibulum could be accidentally damaged during the right ventriculotomy.…”

Late follow-up of Persistent Truncus Arteriosus, after one-stage Repair, with Right Ventricular Remodeling.

“…Other published 9 with 36 children, underwent repair of persistent truncus arteriosus, in the period from January 2011 to December 2018, in two institutions. Surgical re-intervention was required in 8 patients (22.86%), and 11 patients (30.56%) had catheter-based reintervention.…”