Billie-Jean Martin1, David B Ross2, Gwen Y Alton3, Ari R Joffe4, Charlene M T Robertson5, Ivan M Rebeyka6, Joseph Atallah7. 1. Department of Surgery, University of Alberta, Edmonton, Alberta, Canada. Electronic address: billiejean@ualberta.ca. 2. Department of Surgery, University of Alberta, Edmonton, Alberta, Canada. 3. Pediatric Rehabilitation Outcomes Unit, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada; Pediatric Intensive Care, Stollery Children's Hospital, Edmonton, Alberta, Canada. 4. Pediatric Intensive Care, Stollery Children's Hospital, Edmonton, Alberta, Canada; Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. 5. Pediatric Rehabilitation Outcomes Unit, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada; Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. 6. Department of Surgery, University of Alberta, Edmonton, Alberta, Canada; Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada. 7. Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
Abstract
BACKGROUND: Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair. METHODS: All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis. RESULTS: The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5 patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p = 0.0016). CONCLUSIONS: Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.
BACKGROUND:Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair. METHODS: All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis. RESULTS: The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5 patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p = 0.0016). CONCLUSIONS:Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.
Authors: Amber E L van Nisselrooij; Lotta Herling; Sally-Ann Clur; Ingeborg H Linskens; Eva Pajkrt; Lukas A Rammeloo; Arend D J Ten Harkel; Mark G Hazekamp; Nico A Blom; Monique C Haak Journal: Prenat Diagn Date: 2021-02-26 Impact factor: 3.050
Authors: Darlene Huisenga; Sacha La Bastide-Van Gemert; Andrew Van Bergen; Jane Sweeney; Mijna Hadders-Algra Journal: Dev Med Child Neurol Date: 2020-03-09 Impact factor: 5.449