Pediatric Cardiology

, Volume 40, Issue 1, pp 168–176 | Cite as

Immediate Postnatal Ventricular Performance Is Associated with Mortality in Hypoplastic Left Heart Syndrome

  • Gabriel AltitEmail author
  • Shazia Bhombal
  • Valerie Y. Chock
  • Theresa A. Tacy
Original Article


Right ventricular (RV) function as assessed by deformation has been evaluated prenatally and after palliation in hypoplastic left heart syndrome (HLHS). However, limited data exist about the immediate postnatal cardiac adaptation and RV function in HLHS. We compared echocardiographic measures of cardiac performance in HLHS versus controls in their first week of life. As a secondary objective, we evaluated if markers at the first echocardiogram were associated with mid- and long-term outcomes. Clinical and echocardiographic data of patients with HLHS between 2013 and 2016 were reviewed. The study population was matched with controls whose echocardiograms were obtained due to murmur or rule out coarctation. Speckle-tracking echocardiography was used to assess deformation. Thirty-four patients with HLHS and 28 controls were analyzed. Age at echocardiogram was similar between HLHS and controls. The RV of HLHS was compared to both RV and left ventricle (LV) of controls. HLHS deformation parameters [RV peak global longitudinal strain (GLS), global longitudinal strain rate (GLSR)] and tricuspid annular plane systolic excursion (TAPSE) were decreased compared to RV of controls. The LV-fractional area change, peak GLS, GLSR, circumferential strain, and strain rate of controls were higher than the RV of HLHS. Calculated cardiac output (CO) was higher in the HLHS group (592 vs. 183 mL/kg/min, p = 0.0001) but similar to the combined LV and RV output of controls. Later mortality or cardiac transplantation was associated with the RV CO and RV stroke distance at initial echocardiogram. Cox proportional hazard regression determined that restriction at atrial septum, decreased initial RV stroke distance and decreased TAPSE had a higher risk of death or cardiac transplantation. TAPSE and RV stroke distance by velocity time integral had adequate inter-reader variability by Bland–Altman plot and Pearson’s correlation. Our study found that the HLHS RV deformation is decreased in the early postnatal period when compared to both LV and RV of controls, but deformation was not associated with mid- and long-term outcomes. Later mortality or cardiac transplantation was associated with decreased initial stroke distance and cardiac output. Early evaluation of patients with HLHS should include an assessment of stroke distance and future research should evaluate its implication in management strategies.


Hypoplastic left heart syndrome (HLHS) Speckle-tracking echocardiography Ventricular performance Cardiac output Neonatal adaptation Mortality Outcomes 



Aortic atresia


Aortic stenosis


Aortic valve


Cardiac output


Digital imaging and communications in medicine format


Ejection fraction


Extracorporeal membrane oxygenation


Fractional area change


Head ultrasound


Hypoplastic left heart syndrome


Intra-ventricular hemorrhage


Left ventricle


Left ventricular outflow tract


Magnetic resonance imaging


Minute distance


Mitral atresia


Mitral stenosis


Mitral valve


Neonatal intensive care unit


Peak global longitudinal systolic




Pulmonary valve


Right ventricle


Small for gestational age


Strain rate


Superior vena cava


Total anomalous pulmonary venous return


Tricuspid valve


Tricuspid annular plane systolic excursion


Velocity vector imaging


Velocity time integral


Ventricular septal defect


Compliance with Ethical Standards

Conflict of interest

We have no conflict of interest related to the content of this study. Gabriel Altit is the author that wrote the first draft. There was no payment, grant, or honorarium given to anyone to produce the manuscript. All the authors (Gabriel Altit, Shazia Bhombal, Valerie Y. Chock, and Theresa A. Tacy) declare that they have no conflict of interest.

Ethical Approval

This study was approved by the institutional review board of Stanford University (protocol—IRB-39544). All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Supplementary material

246_2018_1974_MOESM1_ESM.docx (13 kb)
Supplementary material 1 (DOCX 14 KB)


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Copyright information

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

Authors and Affiliations

  1. 1.NeonatologyMcGill University - Montreal Children’s HospitalMontrealCanada
  2. 2.Department of Developmental and Neonatal MedicineStanford University – Lucile Packard Children’s HospitalPalo AltoUSA
  3. 3.Pediatric CardiologyStanford University – Lucile Packard Children’s HospitalPalo AltoUSA

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