Cardiac Magnetic Resonance Parameters Predict Transplant-Free Survival in Fontan 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 Fontan patients who had a CMR study from 1/2002 to 1/2011 were retrospectively reviewed. The endpoint 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]. Over a median post-CMR follow-up period of 4.1 years [2.6, 6.2], 24 patients (11%) reached the endpoint: 20 deaths, 3 transplants, and 1 transplant listing. In a multivariable Cox regression model with clinical parameters only, protein losing enteropathy (PLE) was associated with transplant-free survival. A multivariable model including clinical and CMR parameters showed that in addition to PLE, ventricular end-diastolic volume (EDVi) >125 mL/BSA1.3 was associated with transplant-free survival. A likelihood-ratio test comparing the 2 models showed that the addition of EDVi resulted in a significantly improved endpoint prediction (P<0.001) — C-index increased from 0.63 to 0.79.
Conclusions—CMR-derived ventricular EDVi is an independent predictor of transplant-free survival late after the Fontan operation and adds incremental value over clinical symptoms alone for risk stratification.
- Received October 2, 2013.
- Accepted March 4, 2014.