Valve Replacement in Children with Single Ventricle Physiology

  • Noor Alshami
  • Amber Leila Sarvestani
  • Amanda S. Thomas
  • James St. Louis
  • Lazaros Kochilas
  • Geetha RaghuveerEmail author
Original Article


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.


Atrioventricular valve replacement Semilunar valve replacement Single ventricle physiology Single ventricle palliation 



The authors thank the PCCC centers, program directors, and data coordinators. In particular, the authors wish to acknowledge the contributions of Ms. Jessica Knight, Ms. Elizabeth Turk, and Dr. James Moller. The study was supported by the National Heart, Lung, and Blood Institute (R0l-HL-122392).

Author Contributions

NA: study conception and design, acquisition of data, analysis and interpretation of data, drafting of manuscript, critical revision. AS: study conception and design, acquisition of data, analysis and interpretation of data, drafting of manuscript, critical revision. AT: analysis and interpretation of data, critical revision. JSL: interpretation of data, critical revision. LK: analysis and interpretation of data, critical revision. GR: study conception and design, acquisition of data, analysis and interpretation of data, drafting of manuscript, critical revision, and project supervision. All authors reviewed, discussed, and approved the final manuscript.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Supplementary material

246_2019_2234_MOESM1_ESM.docx (19 kb)
Supplementary file1 (DOCX 18 kb)


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of PediatricsUniversity of Missouri-Kansas City School of MedicineKansas CityUSA
  2. 2.Department of Pediatrics, Ward Family Heart CenterChildren’s Mercy HospitalKansas CityUSA
  3. 3.Department of PediatricsEmory University School of MedicineAtlantaUSA

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