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Published on: August 2, 2024
Cardiac magnetic resonance parameters predict transplantation-free survival in patients with fontan circulation
Rahul H Rathod1, Ashwin Prakash2, Yuli Y Kim2
1From the Department of Cardiology, Boston Children's Hospital, Boston, MA; and Department of Pediatrics, Harvard Medical School, Boston, MA. rahul.rathod@childrens.harvard.edu.
Insights
Cardiac magnetic resonance (CMR) can predict death or heart transplant in Fontan circulation patients. Ventricular indexed end-diastolic volume measured by CMR improves risk assessment beyond clinical factors alone.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiovascular Imaging
Background:
- Fontan circulation patients face risks of death and heart transplantation.
- Clinical factors are known predictors, but the role of cardiac imaging is unclear.
Purpose of the Study:
- To determine if cardiac magnetic resonance (CMR) measurements of ventricular size and function independently predict outcomes in Fontan patients.
- To assess if CMR improves risk stratification beyond clinical factors.
Main Methods:
- Retrospective review of 215 Fontan patients who underwent CMR between 2002-2011.
- Endpoint defined as death or heart transplantation listing.
- Multivariable Cox regression analysis incorporating clinical and CMR parameters.
Main Results:
- Protein-losing enteropathy was a clinical predictor of adverse outcomes.
- Ventricular indexed end-diastolic volume >125 mL/m^1.3 was an independent predictor.
- Adding CMR data significantly improved risk prediction (C-index from 0.63 to 0.79).
Conclusions:
- Ventricular indexed end-diastolic volume from CMR is an independent predictor of death or transplant in late Fontan survivors.
- CMR measurements provide incremental value for risk stratification in these patients.
Background:
Several clinical risk factors for death and heart transplantation have been identified in patients with Fontan circulation. It is unknown whether cardiac magnetic resonance (CMR) measurements of ventricular size and function are independently associated with these outcomes and further improve risk stratification.
Methods And Results:
Data on patients with Fontan circulation who had a CMR study from January 2002 to January 2011 were retrospectively reviewed. The end point was time to death or listing for heart transplantation after the CMR study. The median age of the 215 patients was 18.3 years (25th, 75th percentiles: 14, 26) with a median age at Fontan of 3.6 years (2.3, 7.1). During a median post-CMR follow-up period of 4.1 years (2.6, 6.2), 24 patients (11%) reached the end point: 20 deaths, 3 transplantations, and 1 transplantation listing. In a multivariable Cox regression model with clinical parameters only, protein-losing enteropathy was associated with death or listing for transplant. A multivariable model, including clinical and CMR parameters, showed that in addition to protein-losing enteropathy, ventricular indexed end-diastolic volume >125 mL/body surface area raised to the 1.3 power was associated with the endpoint. A likelihood-ratio test comparing the 2 models showed that the addition of indexed end-diastolic volume resulted in a significantly improved end point prediction (P<0.001)-C-index increased from 0.63 to 0.79.
Conclusions:
CMR-derived ventricular indexed end-diastolic volume is an independent predictor of death or transplant in patients late after the Fontan operation and adds incremental value over clinical symptoms alone for risk stratification.

