Pediatric Cardiology

, Volume 39, Issue 5, pp 955–966 | Cite as

Survival to Stage II with Ventricular Dysfunction: Secondary Analysis of the Single Ventricle Reconstruction Trial

  • Emilie Jean-St-Michel
  • James M. Meza
  • Jonathon Maguire
  • John Coles
  • Brian W. McCrindle
Original Article


Ventricular dysfunction affects survival in patients with single right ventricle (RV), and remains one of the primary indications for heart transplantation. Since it is challenging to predict the capacity of patients with ventricular dysfunction to proceed to the stage II procedure, we sought to identify factors that would be associated with death or heart transplantation without achieving stage II for single RV patients with ventricular dysfunction after Norwood procedure. The Single Ventricle Reconstruction (SVR) trial public-use database was used. Patients with a RV ejection fraction less than 44% or a RV fractional area of change less than 35% on the post-Norwood echocardiogram were included. Parametric risk hazard analysis was used to identify risk factors for death or transplantation without achieving stage II. Of 365 patients with ventricular function measurements on the post-Norwood echocardiogram, 123 (34%) patients had RV dysfunction. The transplantation-free survival was significantly lower for those with ventricular dysfunction compared to those with normal function (log rank Chi-square = 4.23, p = 0.04). Furthermore, having a Blalock–Taussig (BT) shunt, a large RV, a post-Norwood infectious complication, and a surgeon who performs five or less Norwood per year were independent risk factors for death or transplantation without achieving stage II. The predicted 6-month transplantation-free survival for patients with all four identified risk factors was 1% (70% CI 0–13%). Early heart transplantation referral might be considered for post-Norwood patients with BT shunt and RV dysfunction, especially if other high-risk features are present.


Hypoplastic left heart syndrome Norwood Transplantation Parametric Blalock–Taussig shunt 


Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors since we used the dataset provided by the Pediatric Heart Network which has been completely de-identified, and is now publically accessible via the Pediatric Heart Network website.

Supplementary material

246_2018_1845_MOESM1_ESM.tiff (109 kb)
Supplement Fig. 1 Distribution of time in days from the Norwood procedure to the post-Norwood echocardiogram in days. This graph represents the number of days between the Norwood procedure and the post-Norwood echocardiogram for each subject within the ventricular dysfunction cohort (TIFF 108 KB)
246_2018_1845_MOESM2_ESM.tiff (22 kb)
Supplement Fig. 2 Time to death, heart transplant, or stage II. The time to death, heart transplant or stage II was defined as the time from the Norwood procedure to the post-Norwood echocardiogram plus the time from post-Norwood echocardiogram to the event. This graph represent the observation period included in our analysis after adjusting for left censoring (TIFF 22 KB)
246_2018_1845_MOESM3_ESM.docx (33 kb)
Supplementary material 3 (DOCX 33 KB)


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada
  2. 2.Division of Cardiovascular Surgery, The Labatt Family Heart Centre, Department of Surgery, The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada
  3. 3.Li Ka Shing Knowledge Institute of St. Michael’s hospital, Department of Pediatrics, St. Michael’s HospitalUniversity of TorontoTorontoCanada

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