Long‐term Outcomes after Truncus Arteriosus Repair: A Single‐center Experience for More than 40 Years. (29th April 2016)

• Record Type: Journal Article
• Authors: Asagai, Seiji; Inai, Kei; Shinohara, Tokuko; Tomimatsu, Hirofumi; Ishii, Tetsuko; Sugiyama, Hisashi; Park, In‐Sam; Nagashima, Mitsugi; Nakanishi, Toshio
• Is Part Of: Congenital heart disease. Volume 11:Number 6(2016)
• Page Start: 672
• Page End: 677
• Publication Date: 2016-04-29
• Subjects: Truncus Arteriosus -- Long‐term Outcome -- Functional Outcome -- RV Outflow Stenosis -- Truncal Valve Regurgitation; Congenital heart disease -- Periodicals; 616.1204305
• DOI: 10.1111/chd.12359
• Languages: English
• ISSNs: 1747-079X

Abstract

Abstract: Objectives: This study aimed to analyze long‐term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow‐up. Methods: Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty‐five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1–123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg). Results: The median age at follow‐up was 23.6 years (range, 12.4 to 44.5 years). The median follow‐up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow‐up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty‐six percent of patients had full‐time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation. Conclusions: In this single‐center retrospective study, with long‐term follow‐up after repair of truncus arteriosus, long‐term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.