Classic-Pattern Dyssynchrony Is Associated with Outcome in Patients with Fontan Circulation

Background: Morbidity and mortality increase as Fontan patients age into adulthood. Limited studies have examined cardiac magnetic resonance and echocardiographic parameters to predict death and transplantation in children after the Fontan operation. The aim of this study was to investigate echocardiographic parameters in adolescents and adults after Fontan operation, including myocardial mechanics such as classic-pattern dyssynchrony (CPD), as predictors of transplantation or death. Methods: In a cross-sectional retrospective study, strain analysis was performed on echocardiographic studies performed between 2001 and 2015 on 110 patients with single-ventricle physiology after the Fontan procedure. Strain curves were measured and visually assessed for the presence of CPD. The primary end point was death or transplantation after a follow-up period of 85 6 35 months after echocardiography. Results: The median age at the date of echocardiography was 20 years (range, 3-45 years). Of 110 patients, 28 had undergone transplantation. During the study period, three patients died after transplantation and seven patients died without undergoing transplantation. CPD was seen in 16 and protein-losing enteropathy in 21 of 110 patients. On multivariate analysis, CPD (hazard ratio [HR], 9.4; 95% confidence interval [CI], 2.6-34.6), protein-losing enteropathy (HR, 10.6; 95% CI, 3.4-33.2), systolic blood pressure (HR, 0.954; 95% CI, 0.913-0.996), systolic/diastolic duration ratio (HR, 6.83; 95% CI, 1.33-35.0), and E-wave deceleration time (HR, 0.98; 95% CI, 0.97-0.99) were independently associated with the primary end point. Conclusions: CPD, protein-losing enteropathy, and systolic and diastolic ventricular dysfunction are significantly associated with transplantation or death in Fontan-operated patients. In selected patients, the presence of CPD may be a basis to investigate cardiac resynchronization therapy as a treatment strategy.

Automated summary

This study investigated echocardiographic parameters in adolescents and adults after the Fontan operation and found that classic-pattern dyssynchrony (CPD), protein-losing enteropathy, and ventricular dysfunction are significantly associated with transplantation or death.