Serial magnetic resonance imaging in hypoplastic left heart syndrome gives valuable insight into ventricular and vascular adaptation

Hannah R. Bellsham-Revell, Shane M. Tibby, Aaron J. Bell, Thomas Witter, John Simpson, Philipp Beerbaum, David Anderson, Conal B. Austin, Gerald F. Greil*, Reza Razavi

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

47 Citations (Scopus)


Objectives: This study sought to investigate changes in magnetic resonance imaging (MRI) ventricular volumes and vascular dimensions before hemi-Fontan (HF) and before total cavopulmonary connection (TCPC) in children with hypoplastic left heart syndrome (HLHS). Background: The systemic right ventricle (RV) in HLHS is subject to significant changes in volume loading throughout the surgical stages of palliation, particularly after the HF. Methods: Fifty-eight patients had paired pre-HF and pre-TCPC MRI for assessment of changes of RV volumes, neoaortic flow, and vascular dimensions. Results: Comparison of pre-HF and pre-TCPC MRI results showed a decrease of indexed RV end-diastolic volume and end-systolic volume (98 ml/m2 to 87 ml/m2 and 50 ml/m2 to 36 ml/m2, respectively) with stroke volume remaining constant (49 ml/m2 vs. 51 ml/m2), leading to an increased RV ejection fraction (51% vs. 59%). These findings persisted after excluding the 3 patients who underwent tricuspid valve repair as part of their HF procedure. Indexed RV end-diastolic volume plotted against neoaortic stroke volume demonstrated a Frank-Starling-like curve that shifted upward after HF. The indexed distal left and right cross-sectional pulmonary artery areas were reduced after HF. Conclusions: In HLHS, serial MRI shows the adaptation of the systemic RV after HF with volume reduction in the context of a preserved stroke volume and an increased ejection fraction. The staged palliation in HLHS may be a risk factor particularly for reduced left pulmonary artery growth in itself as no factors investigated in this study were found to significantly impact on this.

Original languageEnglish
Pages (from-to)561-570
Number of pages10
JournalJournal of the American College of Cardiology
Issue number5
Publication statusPublished - 5 Feb 2013
Externally publishedYes


  • Norwood procedure
  • hemi-Fontan operation
  • hypoplastic left heart syndrome
  • magnetic resonance imaging
  • remodeling of systemic right ventricle


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