Right Ventricular Systolic Function After the Cone Procedure for Ebstein’s Anomaly: Comparison Between Echocardiography and Cardiac Magnetic Resonance

Lianza AC, Rodrigues ACT, Mercer-Rosa L, Vieira MLC, de Oliveira WAA, Afonso TR, Nomura CH, da Silva JP, da Silva LDF, Szarf G, Tavares GMP, Fischer CH, Morhy SS.
Pediatr Cardiol. 2020 Apr 25. doi: 10.1007/s00246-020-02347-6. [Epub ahead of print] PMID: 32335735
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Although the Cone procedure has improved outcomes for patients with Ebstein´s anomaly (EA), neither RV systolic function recovery in long-term follow-up nor the best echocardiographic parameters to assess RV function are well established. Thus, we evaluated RV performance after the Cone procedure comparing two-dimensional (2DEcho) and three-dimensional (3DEcho) echocardiography to cardiac magnetic resonance (CMR). We assessed 27 EA patients after the Cone procedure (53% female, median age of 20 years at the procedure, median post-operative follow-up duration of 8 years). Echocardiography was performed 4 h apart from the CMR. RV global longitudinal strain (GLS), fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), myocardial performance index and tissue Doppler S’ velocity were assessed using 2DEcho, whereas 3DEcho was used to evaluate RV volumes and ejection fraction (RVEF). Echocardiographic variables were compared to CMR-RVEF. All patients were in the NYHA functional class I. Median TAPSE was 15.9 mm, FAC 30.2%, and RV-GLS -15%; median RVEF by 3DEcho was 31.9% and 43% by CMR. Among 2DEcho parameters, RV-GLS and FAC had a substantial correlation with CMR-RVEF (r = – 0.63 and r = 0.55, respectively); from 3DEcho, the indexed RV volumes and RVEF were closely correlated with CMR (RV-EDVi, r = 0.60, RV-ESVi, r = 0.72; and RVEF r = 0.60). RV systolic function is impaired years after the Cone procedure, despite a good clinical status. FAC and RV-GLS are useful 2DEcho tools to assess RV function in these patients; however, 3DEcho measurements appear to provide a better RV assessment.