Pediatric Cardiology

, Volume 37, Issue 2, pp 239–247 | Cite as

Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome

  • Emilie Jean-St-MichelEmail author
  • Devin Chetan
  • Steven M. Schwartz
  • Glen S. Van Arsdell
  • Alejandro A. Floh
  • Osami Honjo
  • Jennifer Conway
Original Article


We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.


Heart failure Hypoplastic left heart syndrome Interstage outcomes Norwood procedure Hybrid procedure 


Compliance with Ethical Standards

Conflict of interest



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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Division of Cardiology, The Labatt Family Heart CentreThe Hospital for Sick ChildrenTorontoCanada
  2. 2.Department of PaediatricsUniversity of TorontoTorontoCanada
  3. 3.Division of Cardiovascular Surgery, The Labatt Family Heart CentreThe Hospital for Sick ChildrenTorontoCanada
  4. 4.Department of SurgeryUniversity of TorontoTorontoCanada
  5. 5.Department of Critical Care Medicine, The Labatt Family Heart CentreThe Hospital for Sick ChildrenTorontoCanada
  6. 6.Division of Pediatric CardiologyStollery Children’s HospitalEdmontonCanada
  7. 7.Department of PediatricsUniversity of AlbertaEdmontonCanada

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