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

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.