Long-Term Outcomes of Cardiovascular Operations in Children With Connective Tissue Disorders

• Type of Material: Article
• Authors: Amanda A. Thomas, Emory University; Erin E. Falk, New York-Presbyterian Hospital; Sarah Mahoney, Emory University; Jessica H. Knight, University of Georgia; Lazaros K. Kochilas, Emory University
• Language: English
• Date: 2022-09-19
• Publisher: Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2022 Elsevier Inc. All rights reserved.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): https://doi.org/10.1016/j.amjcard.2022.03.054
• Title of Journal or Parent Work: The American Journal of Cardiology
• Volume: 183
• Start Page: 143
• End Page: 149
• Grant/Funding Information: This study was supported by the National Heart, Lung, and Blood Institute (Bethesda, Maryland), R01 HL122392, and the Department of Defense (Arlington, Virginia) PR180683.
• Keywords: Pediatric Cardiac Care Consortium; Connective tissue disorders; cardiovascular disorder
• Research Categories: Health Sciences, Epidemiology; Health Sciences, Medicine and Surgery

Abstract

Connective tissue disorders can be associated with significant cardiovascular morbidity needing cardiac surgery during childhood. In this retrospective study, we used the Pediatric Cardiac Care Consortium, a multicenter United States-based registry of pediatric cardiac interventions, to describe the long-term outcomes of patients who underwent their first surgery for connective tissue-related cardiovascular conditions aged <21 years. Between 1982 and 2003, a total of 103 patients were enrolled who underwent cardiac surgery for a connective tissue-related cardiovascular disorder, including 3 severe infantile cases operated on within the first year of life. Most patients underwent aortic site surgery (n = 85) as a composite graft (n = 50), valve-sparing (n = 33), or other aortic surgery (n = 2). The remaining patients underwent atrioventricular valve surgery (mitral 17, tricuspid 1). Of the 99 patients surviving to discharge, 80 (including the 3 infantile) had adequate identifiers for tracking long-term outcomes through 2019 through linkage with the National Death Index and the Organ Procurement. Over a median period of 19.5 years (interquartile range 16.0 to 23.1), 29 deaths and 1 transplant occurred in the noninfantile group, whereas all 3 infantile patients died before the age of 4 years. The postdischarge survival for the noninfantile group was 92.2%, 68.2%, and 56.7% at 10, 20, and 25 years, respectively. Cardiovascular-related pathology contributed to all deaths in the infantile and 89% (n = 27) of deaths for the noninfantile cases after hospital discharge. The significant late attrition from cardiovascular causes emphasizes the need for close monitoring and ongoing management in this population.