Jennifer K Peterson1, Lazaros K Kochilas2, Jessica Knight3, Courtney McCracken4, Amanda S Thomas4, James H Moller5, Shaun P Setty6. 1. Johns Hopkins University School of Nursing, Baltimore, MD. 2. Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta, Atlanta, GA. 3. Department of Epidemiology and Biostatistics, University of Georgia College of Public Health, Athens, GA. 4. Department of Pediatrics, Emory University School of Medicine, Atlanta, GA. 5. Departments of Pediatrics and Medicine, University of Minnesota, Minneapolis, MN. 6. Long Beach Memorial Heart and Vascular Institute, Long Beach, CA; Children's Heart Institute, MemorialCare Miller Children's and Women's Hospital, Long Beach, CA. Electronic address: ssetty@memorialcare.org.
Abstract
OBJECTIVE: To evaluate long-term transplant-free survival and causes of death in the trisomy 21 (T21) population after surgery for congenital heart disease (CHD) in comparison with patients who are euploidic. STUDY DESIGN: This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, enriched with prospectively collected data from the National Death Index and the Organ Procurement and Transplantation Network for patients with sufficient direct identifiers. Kaplan-Meier survival plots were generated and multivariable Cox proportional hazards models were used to examine risk factors for mortality between patients with T21 and 1:1 matched patients with comparable CHD who are euploidic. RESULTS: A long-term survival analysis was completed for 3376 patients with T21 (75 155 person-years) who met inclusion criteria. The 30-year survival rate for patients with T21 ranged from 92.1% for ventricular septal defect to 65.3% for complex common atrioventricular canal. Of these, 2185 patients with T21 were successfully matched with a patient who was euploidic. After a median follow-up of 22.86 years (IQR, 19.45-27.14 years), 213 deaths occurred in the T21 group (9.7%) compared with 123 (5.6%) in the euploidic comparators. After adjustment for age, sex, era, CHD complexity, and initial palliation, the hazard ratio of CHD-related mortality was 1.34 times higher in patients with T21 (95% CI, 0.92-1.97; P = .127). CONCLUSIONS: CHD-related mortality for patients with T21 after cardiac surgical intervention is comparable with euploidic comparators. Children with T21 require lifelong surveillance for co-occurring conditions associated with their chromosomal abnormality.
OBJECTIVE: To evaluate long-term transplant-free survival and causes of death in the trisomy 21 (T21) population after surgery for congenital heart disease (CHD) in comparison with patients who are euploidic. STUDY DESIGN: This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, enriched with prospectively collected data from the National Death Index and the Organ Procurement and Transplantation Network for patients with sufficient direct identifiers. Kaplan-Meier survival plots were generated and multivariable Cox proportional hazards models were used to examine risk factors for mortality between patients with T21 and 1:1 matched patients with comparable CHD who are euploidic. RESULTS: A long-term survival analysis was completed for 3376 patients with T21 (75 155 person-years) who met inclusion criteria. The 30-year survival rate for patients with T21 ranged from 92.1% for ventricular septal defect to 65.3% for complex common atrioventricular canal. Of these, 2185 patients with T21 were successfully matched with a patient who was euploidic. After a median follow-up of 22.86 years (IQR, 19.45-27.14 years), 213 deaths occurred in the T21 group (9.7%) compared with 123 (5.6%) in the euploidic comparators. After adjustment for age, sex, era, CHD complexity, and initial palliation, the hazard ratio of CHD-related mortality was 1.34 times higher in patients with T21 (95% CI, 0.92-1.97; P = .127). CONCLUSIONS: CHD-related mortality for patients with T21 after cardiac surgical intervention is comparable with euploidic comparators. Children with T21 require lifelong surveillance for co-occurring conditions associated with their chromosomal abnormality.
Authors: Salil Ginde; Janna Lam; Garick D Hill; Scott Cohen; Ronald K Woods; Michael E Mitchell; James S Tweddell; Michael G Earing Journal: J Thorac Cardiovasc Surg Date: 2015-05-07 Impact factor: 5.209
Authors: James D St Louis; Upinder Jodhka; Jeffrey P Jacobs; Xia He; Kevin D Hill; Sara K Pasquali; Marshall L Jacobs Journal: J Thorac Cardiovasc Surg Date: 2014-07-21 Impact factor: 5.209
Authors: Alireza Raissadati; Heta Nieminen; Jari Haukka; Heikki Sairanen; Eero Jokinen Journal: J Am Coll Cardiol Date: 2016-08-02 Impact factor: 24.094
Authors: Andrew M Atz; John A Hawkins; Minmin Lu; Meryl S Cohen; Steven D Colan; James Jaggers; Ronald V Lacro; Brian W McCrindle; Renee Margossian; Ralph S Mosca; Lynn A Sleeper; L LuAnn Minich Journal: J Thorac Cardiovasc Surg Date: 2010-12-15 Impact factor: 5.209
Authors: Jennifer K Peterson; Shaun P Setty; Jessica H Knight; Amanda S Thomas; James H Moller; Lazaros K Kochilas Journal: Congenit Heart Dis Date: 2019-07-22 Impact factor: 2.007
Authors: Brett R Anderson; Kacie Dragan; Sarah Crook; Joyce L Woo; Stephen Cook; Edward L Hannan; Jane W Newburger; Marshall Jacobs; Emile A Bacha; Robert Vincent; Khanh Nguyen; Kathleen Walsh-Spoonhower; Ralph Mosca; Neil Devejian; Steven A Kamenir; George M Alfieris; Michael F Swartz; David Meyer; Erin A Paul; John Billings Journal: J Am Coll Cardiol Date: 2021-10-26 Impact factor: 24.094