title: Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Seiji
authors.initials: S
authors.lastname: Asagai
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kei
authors.initials: K
authors.lastname: Inai
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Tokuko
authors.initials: T
authors.lastname: Shinohara
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Tomimatsu
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Tetsuko
authors.initials: T
authors.lastname: Ishii
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Hisashi
authors.initials: H
authors.lastname: Sugiyama
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: In-Sam
authors.initials: IS
authors.lastname: Park
authors.affiliation: Department of Cardiovascular Surgery, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Mitsugi
authors.initials: M
authors.lastname: Nagashima
authors.affiliation: Department of Pediatric Cardiology, Heart Institute, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Toshio
authors.initials: T
authors.lastname: Nakanishi
mesh_terms: D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D001157:Arterial Occlusive Diseases / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000009:adverse effects* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D018572:Disease-Free Survival; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011651:Pulmonary Artery / Q000503:physiopathology; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D017741:Survivors; D013997:Time Factors; D014041:Tokyo; D016896:Treatment Outcome; D014338:Truncus Arteriosus / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D014654:Vascular Patency; D014694:Ventricular Outflow Obstruction / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D055815:Young Adult
keywords: Functional Outcome; Long-term Outcome; RV Outflow Stenosis; Truncal Valve Regurgitation; Truncus Arteriosus