Outcomes of Biventricular Surgical Treatment for Interrupted Aortic Arch with/without Associated Complex Cardiac Anomalies: A Single-Center Experience in 16 Years

Objectives: This study aims to analyze operative results of patients with interrupted aortic arch (IAA) especially with additional complex cardiac anomalies (CCAs) and their effect on mortality and morbidity.

Methods: Between 2002 and 2018, 41 patients were operated with IAA; 15 patients (36.6%) had type A, 24 patients (58.5%) had type B, and 2 patients had type C (4.9%). Associated CCA was seen in 23 patients (56%); 11 patients had truncus arteriosus communis (TAC), 6 patients hypoplastic left heart complex (HLHC), 2 patients Taussig–Bing anomaly, one double outlet right ventricle (DORV), 2 patients aortopulmonary window, and 1 patient transposition of great arteries. Twenty-six patients (63.4%) were corrected primarily with a median age of 11 days (2–200) and weight of 3.3 kg (2.5–4.3). Thirteen of these patients had additional CCA. Fifteen patients (36.5%) received initially a palliative therapy. Fourteen patients had bilateral pulmonary arterial banding and ductal stenting and one had central pulmonary arterial banding in the neonatal period. Ten of these patients had additional CCA. All 15 patients could be further biventricular repaired in the infancy period. Two patients with TAC and restrictive cardiomyopathy underwent heart transplantation (Htx). Arch reconstruction was always performed using antegrade selective cerebral perfusion under 28°C body temperature. End-to-side posterior wall anastomosis combined with anterior wall patchplasty was used in 24 patients (59%). In 10 patients (24.3%), autologous pulmonary artery tissue was used as posterior wall interposition combined with anterior wall patchplasty.

Results: Hospital mortality was seen in two patients (4.9%) in early primary repair group. Both patients had additional CCA (HLHC and TAC) with low birth weight. No operative mortality was observed in patients without additional CCA and in secondary repair group. Median follow-up of survivors was 78.5 months (0.2–186 months). Late mortality was seen in one patient after 6 years due to lymphoma. Two patients with Htx are still alive. During follow-up, four patients (10.2%) needed reoperation and two (5.1%) catheter interventions for aortic arch. Thirteen patients (33.3%) who had additional CCA received another reinterventions (nine surgical and four catheter).

Conclusion: IAA repair without additional CCA can be performed with good early and late results. Associated CCA and low birth can be a risk factor for mortality and morbidity. Initial palliative surgery in neonatal period can reduce mortality in these patients.