Association of somatometrics at time of Fontan evaluation with in-hospital and long-term survival outcomes of patients with single ventricle palliation Open Access
Luong, Jackie (Spring 2023)
Abstract
In the United States (US), it is estimated that approximately 2,000 newborns are born each year with a single-ventricle (SV) type of congenital heart defect. Survival for SV patients can be improved through a series of palliative procedures which culminate in the Fontan procedure. Growth in children is closely associated with their hemodynamic well-being. However, there is little knowledge of the relationship between height and weight on outcomes of patients with single ventricle physiology. We hypothesize that pre-Fontan somatometrics may be a proxy variable for the hemodynamic efficiency of the single ventricle circulation and a predictor of long-term outcomes for Fontan patients. We assessed the distribution of the somatometrics height-for-age, weight-for-age, and weight-for-height in a large multi-center cohort of patients evaluated for the Fontan procedure. We used Kaplan-Meier survival curves and adjusted Cox hazard models with relevant covariates to assess in-hospital and long-term survival outcomes. The study cohort included a total of 1,401 Fontan patients enrolled between 1982 and 2003 in the Pediatric Cardiac Care Consortium, a US-based registry for congenital heart interventions. Of the 761 (54.3%) had a systemic left ventricle (LV) phenotype and 640 (45.7%) had a systemic right ventricle (RV) phenotype. The median height at pre-Fontan catheterization was 92.0 cm (Q1-Q3: 85.0, 102.0) and the median weight was 13.3 kg (Q1-Q3: 11.5, 15.5). A total of 75 patients died in-hospital after the Fontan procedure, and 1,326 survived to discharge. Over a median follow-up period of 18.8 years (IQR: 16.2-22.4) additional 162 deaths occurred. After adjustment for other covariates, in-hospital mortality was associated with the low-weight tertile, but there was no association between the overall post-discharge survival and the weight z-score. However, stratification analysis revealed a beneficial effect only for patients with systemic LV.
Table of Contents
Introduction 1
Methods 2
Results 4
Discussion 15
References 17
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Association of somatometrics at time of Fontan evaluation with in-hospital and long-term survival outcomes of patients with single ventricle palliation () | 2023-04-19 15:04:18 -0400 |
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