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Parameters for Risk Stratification After Fontan Operation
Quick Takes
- Lower total indexed volume (<156 ml/BSA1.3) was the strongest predictor of transplant-free survival in patients with Fontan circulation.
- For patients with larger ventricular volumes, worse global circumferential strain was the next most important predictor.
Study Questions:
What are the relative importance and interactions of clinical and cardiac magnetic resonance imaging (CMR)-derived parameters for risk stratification after the Fontan operation?
Methods:
A retrospective cohort study was performed at a single center. Clinical and CMR parameters were analyzed using univariable Cox regression. The primary endpoint was time to death or listing for transplant. Classification and regression tree survival analysis was performed to identify patients at the highest risk for the endpoint.
Results:
A total of 416 patients were studied (62% male) with a median age of 16 years (25th, 75th percentiles: 11, 23 years). The median follow-up period was 5.4 years (25th, 75th percentiles: 2.4, 10.0 years) after CMR. During the follow-up period, 57 patients (14%) reached the endpoint (46 deaths, seven heart transplants, four heart transplant listings). Lower total indexed end-diastolic volume (EDVi) was the strongest predictor of transplant-free survival. Among patients with dilated ventricles (EDVi ≥156 ml/BSA1.3), worse global circumferential strain (GCS) was the next most important predictor, with 73% of patients with GCS ≥ -6.6% meeting the endpoint as compared with 44% with GCS < -6.6%. In patients with smaller ventricles (EDVi <156 ml/ BSA1.3), New York Heart Association (NYHA) functional class ≥II was the next most important predictor (30% vs. 4%).
Conclusions:
The authors concluded that increased ventricular dilatation was the strongest independent risk factor for death or need for transplant. Patients with both ventricular dilatation and worse GCS were at the highest risk.
Perspective:
With increasing number of adolescents and adults with Fontan circulation, the ability to accurately prognosticate for individual patients is becoming increasingly important. This study confirmed previous studies demonstrating the prognostic value of EDVi. Additional risk factors in the study were worse global circumferential strain in patients with large ventricles, and NYHA class II or worse symptomatology for patients with smaller ventricles. One of the most difficult decisions in congenital cardiology is determining the timing for moving forward for consideration for transplantation and other advanced therapies. Studies such as this one contribute to our knowledge of risk factors for poor outcome, which may prompt earlier consideration for such therapies.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Heart Transplant, Interventions and Imaging, Interventions and Structural Heart Disease, Magnetic Resonance Imaging
Keywords: Dilatation, Diagnostic Imaging, Fontan Procedure, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Magnetic Resonance Imaging, Risk Assessment, Risk Factors
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