Abstract
Objective
This study used cardiac magnetic resonance imaging to evaluate flow characteristics
and ventricular hemodynamics for children with single right (hypoplastic left heart
syndrome) and single left (hypoplastic right heart syndrome) systemic ventricle anatomy
after Fontan palliation compared with normal biventricular controls.
Methods
Twenty children with single ventricle anatomy (hypoplastic left heart syndrome, n = 10;
hypoplastic right heart syndrome, n = 10) underwent standardized 4-dimensional flow
cardiac magnetic resonance and were compared with age-matched controls (n = 10). End-diastolic
volume was partitioned into 4 defined components of variable kinetic energy (direct
flow, retained inflow, delayed ejection, and residual volume) and compared between
groups. Further, volumetric and functional parameters as defined by cardiac magnetic
resonance were evaluated.
Results
Children with hypoplastic left heart syndrome had significantly increased indexed
end-diastolic and end-systolic volumes compared with both hypoplastic right heart
syndrome and control groups. Flow component analysis demonstrated diastolic inefficiency
in both hypoplastic left heart syndrome and hypoplastic right heart syndrome groups
compared with controls as defined by decreased direct flow and increased residual
volumes. Decreased direct flow correlated with decreased ejection fraction and increased
end-diastolic and end-systolic volume indices. Increased residual volume correlated
with decreased ejection fraction and increased end-systolic volume index.
Conclusions
Fontan-palliated patients with single ventricle physiology (hypoplastic left heart
syndrome and hypoplastic right heart syndrome) demonstrate altered and inefficient
flow patterns in the systemic ventricle as defined by 4-dimensional flow cardiac magnetic
resonance compared with normal biventricular controls. Decreased direct flow and increased
residual volume indicate that diastolic ventricular dysfunction is prevalent after
Fontan palliation. This study provides a foundation for future predictive modeling
and cardiac magnetic resonance flow diagnostic studies in this high-risk patient population.
Graphical abstract
Graphical Abstract
Key Words
Abbreviations and Acronyms:
CMR (cardiac magnetic resonance), 4D (4-dimensional), HLHS (hypoplastic left heart syndrome), HRHS (hypoplastic right heart syndrome), MRI (magnetic resonance imaging)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: July 16, 2021
Accepted:
June 17,
2021
Received in revised form:
June 17,
2021
Received:
January 6,
2021
Footnotes
Funding source: Rady Family and Jayden DeLuca Foundations.
The Institutional Review Board of the University of Colorado School of Medicine approved the study protocol and publication of data (Institutional Review Board protocol no. 19-1420; date of approval: June 20, 2019). The patients provided informed written consent for the publication of the study data.
Identification
Copyright
© 2021 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery
ScienceDirect
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