Abstract

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 Fontan operation. The aim of the study was to investigate echocardiographic parameters in adolescents and adults after Fontan operation including myocardial mechanics including classic pattern dyssynchrony (CPD) as predictors of transplantation or death.METHODS: In a cross-sectional retrospective study, strain analysis was performed on echocardiography studies performed between 2001 and 2015 of 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±35 months after echocardiography.RESULTS: Median age at date of echocardiography was 20, range 3 to 45 years. Of 110 patients 28 patients were transplanted. During the study-period 3 patients died after transplantation and 7 patients died without being transplanted. CPD was seen in 16 and protein losing enteropathy (PLE) in 21 of 110 patients. By multivariate-analysis, CPD (HR 9.4 CI 2.6-34.6), PLE (HR 10.6 CI 3.4-33.2); systolic blood pressure (HR 0.954 CI 0.913-0.996), systolic/diastolic duration ratio (HR 6.83 CI 1.33-35.0) and E wave deceleration time (HR 0.98 CI 0.97 - 0.99) were independently associated with the primary end point.CONCLUSION: CPD, PLE, 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.

View details for DOI 10.1016/j.echo.2022.01.012

View details for PubMedID 35121052