Characterization and Z-score Calculation of Cardiac MRI parameters in patients after the Fontan operation. A Fontan Outcome Registry using CMR Examinations (FORCE) study

Tarek Alsaied; Runjia Li; Adam Christopher; Mark Fogel; Timothy C. Slesnick; Rajesh Krishnamurthy; Vivek Muthurangu; Adam L. Dorfman; Christopher Z. Lam; Justin Weigand; Jong-Hyeon Jeong; Joshua D. Robinson; Laura J. Olivieri; Rahul H. Rathod; Yue-Hin Loke; Kenan Stern; Jonathan H. Soslow; Thomas P. Graham; Manish Aggarwal; Tiffanie Johnson; Markus Renno; Amee Shah; Michael Quail; Shelby Kutty; Ruchira Garg; Jeremy Steele; Pierangelo Renella; Madhuradhar Chegondi; Aswathy Vaikom House; Alison Marsden; Francesca Raimondi

doi:10.1016/j.jocmr.2024.101113

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Characterization and Z-score Calculation of Cardiac MRI parameters in patients after the Fontan operation. A Fontan Outcome Registry using CMR Examinations (FORCE) study

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Characterization and Z-score Calculation of Cardiac MRI parameters in patients after the Fontan operation. A Fontan Outcome Registry using CMR Examinations (FORCE) study

Tarek Alsaied, Runjia Li, Adam Christopher, Mark Fogel, Timothy C. Slesnick, Rajesh Krishnamurthy, Vivek Muthurangu, Adam L. Dorfman, Christopher Z. Lam, Justin Weigand, …

Journal of cardiovascular magnetic resonance, Vol.26(2), 101113

2024

DOI: 10.1016/j.jocmr.2024.101113

PMCID: PMC11655684

PMID: 39442671

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Abstract

Background Cardiac magnetic resonance (CMR) offers valuable hemodynamic insights post-Fontan, but is limited by the absence of normative single ventricle data. The Fontan Outcomes Registry using CMR Examinations (FORCE) is a large international Fontan-specific CMR registry. This study used FORCE registry data to evaluate expected CMR ventricular size/function and create Fontan-specific z-scores adjusting for ventricular morphology (VM) in healthier Fontan patients. Methods “Healthier” Fontan patients were defined as patients free of adverse outcomes, who are New York Heart Association Class I, have mild or less valve disease, and <30% aortopulmonary collateral burden. General linear modeling was performed on 70% of the dataset to create z-scores for volumes and function. Models were tested using the remainder (30%) of the data. The z-scores were compared between children and adults. The z-scores were also compared between “healthier” Fontan and patients with adverse outcomes (death, listing for transplantation or multiorgan disease). Results The “healthier” Fontan population included 885 patients (15.0 ± 7.6 years) from 18 institutions with 1,156 CMR examinations. Patients with left ventricle morphology had lower volume, mass and higher ejection fraction (EF) compared to right or mixed (two-ventricles) morphology (p<0.001 for all pairwise comparisons). Gender, BSA and VM were used in z-scores. Of the “healthier” Fontan patients, 647 were children <18 years and 238 were adults. Adults had lower ascending aorta flow (2.9 ± 0.7 vs 3.3 ± 0.8 L/min/m2, p<0.001) and ascending aorta flow z-scores (-0.16 ± 1.23 vs 0.05 ± 0.95, 0.02) compared to children. Additionally, there were 1595 patients with adverse outcomes who were older (16.1 ± 9.3 vs 15.0 ± 7.6, p<0.001) and less likely to have LV morphology (35 vs 47%, p<0.001). Patients with adverse outcomes had higher z-scores for ventricular volume and mass and lower z-scores for EF and ascending aorta flow compared to the “healthier” Fontan cohort. Conclusion This is the first study to generate CMR z-scores post-Fontan. Importantly the z-scores were generated and tested in “healthier” Fontan patients and both pediatric and adult Fontan patients. These equations may improve CMR-based risk stratification after the Fontan operation.

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Title: Subtitle: Characterization and Z-score Calculation of Cardiac MRI parameters in patients after the Fontan operation. A Fontan Outcome Registry using CMR Examinations (FORCE) study
Creators: Tarek Alsaied - Children's Hospital of Pittsburgh
Runjia Li - University of Pittsburgh
Adam Christopher - Children's Hospital of Pittsburgh
Mark Fogel - Children's Hospital of Philadelphia
Timothy C. Slesnick - Children's Healthcare of Atlanta
Rajesh Krishnamurthy - Nationwide Children's Hospital
Vivek Muthurangu - University College London
Adam L. Dorfman - University of Michigan
Christopher Z. Lam - University of Toronto
Justin Weigand - Baylor College of Medicine
Jong-Hyeon Jeong
Joshua D. Robinson - Northwestern University
Laura J. Olivieri - Children's Hospital of Pittsburgh
Rahul H. Rathod - Boston Children's Hospital
Yue-Hin Loke
Kenan Stern
Jonathan H. Soslow
Thomas P. Graham
Manish Aggarwal
Tiffanie Johnson
Markus Renno
Amee Shah
Michael Quail
Shelby Kutty
Ruchira Garg
Jeremy Steele
Pierangelo Renella
Madhuradhar Chegondi
Aswathy Vaikom House
Alison Marsden
Francesca Raimondi
Resource Type: Journal article
Publication Details: Journal of cardiovascular magnetic resonance, Vol.26(2), 101113
DOI: 10.1016/j.jocmr.2024.101113
PMID: 39442671
PMCID: PMC11655684
NLM abbreviation: J Cardiovasc Magn Reson
ISSN: 1097-6647
eISSN: 2588-9311
Publisher: Elsevier
Grant note: Evan's Heart FundNational Institutes of Health: UL1 TR001857, KL2 TR001856, TL1 TR001858
The FORCE registry is funded through a grant from Additional Ventures and Evan's Heart Fund. The project described was supported by the National Institutes of Health through Grant Number UL1 TR001857, KL2 TR001856, and/or TL1 TR001858.
Language: English
Electronic publication date: 10/2024
Date published: 2024
Academic Unit: Stead Family Department of Pediatrics
Record Identifier: 9984737130302771

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