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Valve Replacement in Children with Single Ventricle Physiology
Noor Alshami1, Amber Leila Sarvestani1, Amanda S Thomas2
1Department of Pediatrics, University of Missouri-Kansas City School of Medicine, 2411 Holmes Street, Kansas City, MO, 64108, USA.
Insights
Valve replacement for severe heart valve regurgitation in single ventricle physiology shows poor outcomes, particularly for atrioventricular valve replacement. Alternative strategies are recommended for these complex pediatric cardiac cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Severe atrioventricular valve (AVV) or semilunar valve (SLV) regurgitation in single ventricle physiology often necessitates valve replacement when repair fails.
- Limited outcome data exists for pediatric patients undergoing these interventions.
Purpose of the Study:
- To evaluate transplant-free survival after atrioventricular valve replacement (AVVR) or semilunar valve replacement (SLVR) in children with single ventricle physiology.
- To identify outcomes associated with these valve replacement procedures in a pediatric population.
Main Methods:
- A multi-institutional case series utilizing the Pediatric Cardiac Care Consortium (PCCC) registry.
- Outcomes data linked with the National Death Index (NDI) and Organ Procurement Transplant Network (OPTN).
- Analysis of 50 children with single ventricle physiology who underwent either AVVR (n=38) or SLVR (n=12).
Main Results:
- In-hospital mortality was high: 42% for AVVR and 8% for SLVR.
- Intraoperative deaths occurred exclusively in the AVVR group (8 deaths).
- Post-discharge, 10 of 33 survivors faced death or cardiac transplant, predominantly among AVVR patients.
Conclusions:
- Valve replacement, especially AVVR, in children with single ventricle physiology is associated with significant morbidity and mortality.
- These findings suggest that alternative palliative strategies should be explored for single ventricle patients with severe AVV or SLV regurgitation.
Abstract:
Severe atrioventricular valve (AVV) or semilunar valve (SLV) regurgitation in the setting of a single ventricle physiology may proceed to valve replacement if repair strategies fail. Outcome data for these children are limited. We present transplant-free survival of a case series of children with single ventricle physiology undergoing either atrioventricular replacement (AVVR) or semilunar valve replacement (SLVR) from a multi-institutional, US-based registry (Pediatric Cardiac Care Consortium-PCCC). Outcomes were derived from PCCC and by linkage with the National Death Index (NDI) and Organ Procurement Transplant Network (OPTN). Fifty children with single ventricle physiology were identified to have received either AVVR (n = 38) or SLVR (n = 12). There were 17 in-hospital deaths including 8 intraoperative deaths (all intraoperative deaths were among children requiring AVVR). The in-hospital mortality was 42% and 8% for AVVR and SLVR, respectively. Among the 33 children surviving to hospital discharge, follow-up was available in 15 (46%). Death or cardiac transplant after hospital discharge occurred in 10-death in 4 (all among those requiring AVVR), cardiac transplant in 6 (2 following AVVR, 4 following SLVR). Valve replacement in children with single ventricle physiology, especially AVVR, is associated with poor outcomes. Alternative palliation strategies should be considered in children with single ventricle physiology with significant AVV or SLV regurgitations.
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