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

Authors

Tarek Alsaied, The Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. Electronic address: alsaiedt@upmc.edu.
Runjia Li, Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA, USA.
Adam Christopher, The Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Mark Fogel, Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Timothy C. Slesnick, Departments of Pediatrics, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, GA, USA.
Rajesh Krishnamurthy, The Department of Radiology, Nationwide Children's Hospital, Columbus, OH, USA.
Vivek Muthurangu, UCL Centre for Cardiovascular Imaging, Institute of Cardiovascular Science, University College London, London, UK.
Adam L. Dorfman, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI, USA.
Christopher Z. Lam, Department of Diagnostic Imaging, The Hospital for SickKids and Medical Imaging, University of Toronto, Toronto, Canada.
Justin Weigand, Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.
Jong-Hyeon Jeong, Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA, USA.
Joshua D. Robinson, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Laura J. Olivieri, The Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Rahul H. Rathod, Department of Cardiology, Boston Children's Hospital; Department of Pediatrics, Harvard Medical School, Boston, MA, USA.

Document Type

Journal Article

Publication Date

10-21-2024

Journal

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance

DOI

10.1016/j.jocmr.2024.101113

Keywords

Fontan; Z-scores; congenital heart disease; normal values

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 ± 0.8L/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.

APA Citation

Alsaied, Tarek; Li, Runjia; Christopher, Adam; Fogel, Mark; Slesnick, Timothy C.; Krishnamurthy, Rajesh; Muthurangu, Vivek; Dorfman, Adam L.; Lam, Christopher Z.; Weigand, Justin; Jeong, Jong-Hyeon; Robinson, Joshua D.; Olivieri, Laura J.; and Rathod, Rahul H., "Characterization and Z-score Calculation of Cardiac MRI parameters in patients after the Fontan operation. A Fontan Outcome Registry using CMR Examinations (FORCE) study" (2024). GW Authored Works. Paper 5796.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/5796

Department

Pediatrics