Long-term outcomes following repair of truncus arteriosus and interrupted aortic arch

• Published in: European journal of cardio-thoracic surgery Vol. 57; no. 2; pp. 366 - 372
• Main Authors: Naimo, Phillip S, Fricke, Tyson A, Lee, Melissa G Y, d’Udekem, Yves, Weintraub, Robert G, Brizard, Christian P, Konstantinov, Igor E
• Format: Journal Article
• Language: English
• Published: Germany Oxford University Press 01.02.2020
• Subjects: Aorta, Thoracic - surgery; Aortic Coarctation; Humans; Infant, Newborn; Reoperation; Treatment Outcome; Truncus Arteriosus - surgery; Truncus Arteriosus, Persistent - surgery; Surgery; Interrupted aortic arch; Truncus arteriosus
• ISSN: 1010-7940; 1873-734X; 1873-734X
• DOI: 10.1093/ejcts/ezz176

Abstract OBJECTIVES We aim to evaluate the long-term outcomes following repair of truncus arteriosus with an interrupted aortic arch. METHODS We reviewed all children (n = 24) who underwent repair of truncus arteriosus and an interrupted aortic arch between 1979 and 2018 in a single institution. The morphology of the interrupted aortic arch was type A in 5, type B in 18 and type C in 1. RESULTS The median age at repair was 10 days and the median weight was 3.1 kg. Direct end-to-side anastomosis of the ascending and descending aorta was performed in 16 patients (67%, 16/24), patch augmentation in 5 patients (21%, 5/24) and direct anastomosis with the use of an interposition graft to the descending aorta in 2 patients (8%, 2/24). One patient, the first in the series, underwent interrupted aortic arch repair via subclavian flap aortoplasty prior to truncus repair. A period of deep hypothermic circulatory arrest was used in 16 patients, and isolated cerebral perfusion was used in 8 patients. The early mortality rate was 17% (4 out of 24 patients). There were no late deaths and overall survival was 83 ± 8% [95% confidence interval (CI) 61–93] at 20 years. Freedom from any reoperation was 33 ± 11% (95% CI 14–54) at 5 years and 13 ± 9% (95% CI 2–34) at 10 years. Six patients underwent 10 aortic reoperations. Freedom from aortic arch reoperation was 69 ± 11% (95% CI 42–85) at 10 and 20 years. Follow-up was 95% complete (19/20), with a median follow-up time of 20 years. At last follow-up, no clinically significant aortic arch obstruction was identified in any patient, and all patients were in New York Heart Association Class I/II. CONCLUSIONS Repair of truncus arteriosus with an interrupted aortic arch with direct end-to-side anastomosis results in good survival beyond hospital discharge. Although the long-term functional state of patients is good, reoperation rates are high.