Evolution and Predictors of Right Ventricular Failure in Fontan Patients: A Case-Control Study.

Area of Science:

• Cardiology
• Pediatric Cardiology
• Cardiac Surgery

Background:

• Single right ventricular (SRV) morphology can lead to progressive heart failure post-Fontan procedure, increasing morbidity, mortality, and transplant need.
• Understanding the longitudinal cardiac mechanics in SRV failure is crucial for improving patient outcomes.

Purpose of the Study:

• To longitudinally characterize cardiac mechanics and disease progression in Fontan patients with SRV.
• To identify predictors of SRV failure and the need for heart transplantation.

Main Methods:

• A case-controlled longitudinal study compared 52 patients with SRV who underwent extracardiac Fontan palliation.
• Echocardiographic measures of right ventricular (RV) function, RV-systemic vascular coupling, and ventricular remodeling were assessed at four time points.
• Patients were divided into a heart transplant group (n=26) and a control group (n=26).

Main Results:

• RV circumferential shortening function declined over time, significantly lower in the transplant group.
• RV-systemic vascular coupling was preserved in controls but significantly altered in the transplant group.
• The transplant group showed decreased relative wall thickness and increased minor/major-axis ratio, indicating adverse ventricular remodeling.

Conclusions:

• Positive ventricular remodeling and preserved RV-vascular coupling appear protective against RV failure in Fontan patients with SRV.
• These cardiac mechanics indices may serve as quantifiable markers for disease evolution and early therapeutic intervention targets.