Adult Congenital Heart Disease

1348014 - Evaluation of Diastolic Function in Patients With and Without Fontan Failure using Cardiac MRI

Thursday, January 26, 2023

1:00 PM – 2:00 PM

Location: Pacific Jewel

Presenting Author(s)

EH

Edward H. Hardison, MD

Resident Physician
Vanderbilt University Medical Center
Nashville, Tennessee, United States

Primary Author(s)

EH

Edward H. Hardison, MD

Resident Physician
Vanderbilt University Medical Center
Nashville, Tennessee, United States

Co-Author(s)

CH

Christopher Henderson, MD

Resident Physician
Vanderbilt University Medical Center, United States
JC

Joshua D. Chew, MD

Assistant Professor of Pediatrics
Vanderbilt University Medical Center
Nashville, Tennessee, United States
KG

Kristen George-Durrett, BSc

Research MRI Technologist
Vanderbilt University Medical Center
Nashville, Tennessee, United States
JS

Jonathan H. Soslow, MD, MSc

Associate Professor of Pediatrics, and Director, Pediatric Cardiac Imaging Research Center
Vanderbilt University Medical Center
Nashville, United States
DC

Daniel Clark, MD, MPH

Assistant Professor of Adult and Pediatric Cardiology
Stanford University, United States

Background:
Ventricular filling patterns and diastolic function among patients palliated with a single ventricle are unknown. We hypothesized that patients with Fontan palliation would have diastolic dysfunction based on cardiac magnetic resonance (CMR) filling curves and those with Fontan failure (FF) would have worse filling and ejection than those without FF.

Methods:
Ninety-eight adults with Fontan palliation were age and gender-matched to 96 healthy controls. FF was defined as death (N=6), transplant (N=5), diagnosis of protein losing enteropathy (N=1), peak VO2 < 50% predicted (N=12), or new diuretic requirement (N=28). Charts were reviewed to identify patients with arrhythmias before or after CMR. All short-axis phases were contoured to generate filling curves, and the following variables were included in analysis: peak filling rate (PFR), peak ejection rate (PER), PFR and PER indexed to end diastolic volume (EDV), time to PFR (tPFR), and time to PER (tPER; Figure 1). Fontan-associated liver disease (FALD) was designated based on laboratory, imaging, and occasionally biopsy data and only when confirmed by a Hepatologist. Wilcoxon rank sum and Chi squared were used for statistical analysis.

Results:
For all patients with Fontan palliation (N=98), the median age was 19 years (IQR, 14-27) and the median time from Fontan was 14 years (IQR, 11-21). Late gadolinium enhancement (LGE) was present in 22/65 (34%) of Fontan subjects who underwent contrasted CMR. LGE was more prevalent in FF patients (50% vs. 23%, p=0.025), but LGE burden, when present, was similar (6.4% vs. 5.3%, p=0.67). Compared to healthy controls, the Fontan group had decreased PFR and PFR/EDV and increased tPFR, all representing worse diastolic function (Table 1). Fontan subjects also had increased tPER and decreased PER/EDV. When examining the Fontan cohort, the Fontan failure subjects had similar ventricular systolic function and volumetrics to the Fontan subjects without failure (Table 2). PFR/EDV was significantly reduced among FF patients with the most severe adverse outcomes (death: 2.18 vs. 1.61, p=0.028; death + transplant: 2.19 vs. 1.81, p=0.051). Diastolic parameters did not differ among patients with and without arrhythmias or FALD.

Conclusion: Patients with Fontan palliation had significant diastolic dysfunction compared to healthy controls. Patients with clinically defined FF did not have a significant difference in systolic or diastolic metrics from those in the Fontan group without FF. However, Fontan patients who died or had a combined outcome of death or transplant had evidence of worse diastolic dysfunction compared to Fontan patients without these complications. Future studies are necessary to understand the prognostic implications of abnormal PFR/EDV.