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Congenital heart disease
Original research article
Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology
  1. Gunnar Erikssen1,
  2. Jamil Aboulhosn2,
  3. Jeannette Lin2,
  4. Knut Liestøl3,
  5. Mette E Estensen1,
  6. Ola Gjesdal1,
  7. Helge Skulstad4,
  8. Gaute Døhlen5 and
  9. Harald Lauritz Lindberg6
  1. 1 ACHD Unit, Department of Cardiology, Oslo University Hospital Rikshospitalet, Oslo, Norway
  2. 2 Ahmanson/UCLA Adult Congenital Heart Disease Center, UCLA Medical Plaza, Los Angeles, California, USA
  3. 3 Department of Informatics, University of Oslo, Oslo, Norway
  4. 4 Faculty of Medicine, Institute of Clinical Medicine, University of Oslo, Oslo, Norway
  5. 5 Department of Pediatric Cardiology, Oslo University Hospital, Oslo, Norway
  6. 6 Department of Thoracic Surgery, Oslo University Hospital, Oslo, Norway
  1. Correspondence to Dr Gunnar Erikssen; gunnar.erikssen{at}yahoo.com

Abstract

Objective Patients with univentricular hearts (UVH) have high mortality despite modern treatment, and better methods to identify patients at highest risk are needed. We wanted to improve risk stratification in patients with UVH by focusing on the prognostic significance of single right versus single left ventricular morphology (SRV vs SLV).

Methods All 395 patients with UVH operated at our centre were prospectively included from 1972 to 2016 (195 SRV, 166 SLV, 34 mixed or indeterminate ventricular morphology). Diagnoses, UVH morphology, types of all operations and time and causes of death or heart transplantation (HTX) were recorded. The primary endpoint was death or HTX.

Results Among the 111 non-Fontan patients, 88 died (SRV 62 vs SLV 20; p<0.0001), 32 due to heart failure (SRV 23 vs SLV 5; p=0.0012). Twenty-five  years of cumulative SRV versus SLV survival among the 284 Fontan patients (41 deaths/HTX) was 66.9% vs 87.9% (p=0.0027), partly explained by more deaths/HTX due to heart failure among patients with SRV (p=0.0006). Survival in patients with SRV with and without hypoplastic left heart syndrome (HLHS) was similar. SRV versus SLV was a strong predictor of death/HTX in multivariable proportional hazards analyses (RR 3.3, 95% CI 1.6 to 6.6).

Conclusion SRV versus SLV is a strong short-term and long-term predictor of survival among patients with UVH, mainly explained by higher rates of death/HTX due to heart failure in the SRV group. Our findings apply to patients with SRV both with and without HLHS.

  • congenital heart defects
  • heart surgery
  • univentricular hearts
  • ventricular morphology
  • survival

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/openhrt-2018-000902

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    Footnotes

    • Contributors GE wrote the manuscript with input from all authors. JA, JL and HLL participated in conceiving the project and revised it critically for intellectual content. KL was the main responsible for the statistical analyses. GD, GE and HLL participated in data acquisition. MEE, OG and HS revised the paper critically for intellectual content. All authors approved the final version of the manuscript, and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Not required.

    • Ethics approval Data Protection Officer at Oslo University Hospital.

    • Provenance and peer review Not commissioned; internally peer reviewed.

    • Data sharing statement There are no additional data available.