Pediatric Track Session
SCMR 22nd Annual Scientific Sessions
Background: The prognostic value of diastolic dysfunction remains unclear in patients with Fontan circulation. This study aimed to elucidate the relationship between diastolic dysfunction and cardiovascular events in such patients, and if a certain relationship was observed, to clarify whether the predictive ability of diastolic dysfunction is independent of systolic function.
Methods: We enrolled consecutive patients older than 15 years with Fontan circulation from 2003 to 2016, and performed cardiac MRI as per protocol. To assess diastolic function, we measured longitudinal early diastolic strain rate (GLSRe) in the long axis of the ventricle(s) using a feature tracking method. When present, both the left and right ventricles were traced. Catheter-related and other clinical data were obtained from medical records. Patients were followed up until the occurrence of a major adverse cardiovascular event. Patients with ejection fraction (EF) more than 0.50 were divided into “preserved GLSRe with preserved EF (pGLSRe / pEF)” and “reduced GLSRe with pEF (rGLSRe / pEF) groups,” and the outcomes between the groups were compared.
Results: In total, 82 patients (median age 24 [range, 15–45] years, 40 male) were enrolled; the median GLSRe was 0.80/s (range: 0.12–2.39/s). The median GLSRe was significantly lower in patients with New York Heart Association (NYHA) class ≥2 (≥2: 0.63 vs. 1: 0.87, p < 0.01). GLSRe was negatively correlated with log-transformed brain natriuretic peptide levels (r 0.35, p < 0.01) and ventricular mass index (r 0.32, p 0.50), 22 (64.7%) were categorized into the pGLSRe (>0.78 /s)/pEF group and 12 (35.3%), into the rGLSRe (<0.78/s)/pEF group. Cox regression analysis revealed that patients in the rGLSRe/pEF group had higher risks of future cardiac events than those in the pGLSRe/pEF group did (HR 4.65, p = 0.024).
Conclusion: About 35% of patients with Fontan circulation and preserved EF were affected by reduced GLSRe or diastolic dysfunction. GLSRe predicts cardiovascular events independent of systolic function.