Tricuspid annular plane systolic excursion as a marker of right ventricular dysfunction in pediatric patients with dilated cardiomyopathy

Background Right ventricular systolic dysfunction (RVSD) is a predictor of outcomes in dilated cardiomyopathy (DCM) in adults, but little is known in children. Tricuspid annular plane systolic excursion (TAPSE) has emerged as a reliable tool to assess RVSD. We sought to determine the prevalence and prognostic significance of RVSD using TAPSE in children with DCM. Methods The first echocardiogram at the time of diagnosis with DCM was analyzed to obtain left ventricular ejection fraction (LVEF) and 2D TAPSE. RVSD was defined as TAPSE age-based z-score ≤−2. The association of a composite primary endpoint including death, mechanical support, or transplantation with RVSD was analyzed using LVEF and TAPSE. Results Of the 96 patients, RVSD was noted in 84 (88%). During a median follow-up of 17.5 months (IQR 1.4, 76.2), the primary endpoints were reached in 59%. There was a lower LVEF in patients with RVSD (P=.016). Moderate or severe RVSD showed a significant association with the composite primary endpoint (unadjusted OR 2.7 [95% CI: 1.15–6.33], P=.023). Kaplan-Meier analysis showed that the event-free survival was significantly lower in patients with LVEF ≤30% and TAPSE z-score ≤−4. Conclusion Children with DCM have a high prevalence of RVSD based on reduced TAPSE, and those with moderate or severe RVSD have worse clinical outcomes.