Vol.:(0123456789) 1 3 Pediatric Cardiology https://doi.org/10.1007/s00246-020-02347-6 ORIGINAL ARTICLE Right Ventricular Systolic Function After the Cone Procedure for Ebstein’s Anomaly: Comparison Between Echocardiography and Cardiac Magnetic Resonance Alessandro Cavalcanti Lianza 1  · Ana Clara T. Rodrigues 1  · Laura Mercer‑Rosa 2  · Marcelo L. C. Vieira 1  · Wercules A. A. de Oliveira 1  · Tania Regina Afonso 1  · Cesar H. Nomura 3  · Jose Pedro da Silva 4  · Luciana da Fonseca da Silva 5  · Gilberto Szarf 6  · Glaucia M. P. Tavares 1  · Claudio H. Fischer 1  · Samira S. Morhy 1 Received: 3 February 2020 / Accepted: 8 April 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020 Abstract Although the Cone procedure has improved outcomes for patients with Ebstein´s anomaly (EA), neither RV systolic func- tion 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). Echocardiog- raphy 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. Keywords Ebstein · Cone procedure · Three-dimensional echocardiography · Two-dimensional echocardiography · Cardiac magnetic resonance Abbreviations EA Ebstein’s anomaly RV Right ventricle 2DEcho Two-dimensional echocardiography 3DEcho Three-dimensional echocardiography RVEF Right ventricle ejection fraction CMR Cardiac magnetic resonance TR Tricuspid regurgitation RV-GLS Right ventricular global longitudinal strain RV-FLS Right ventricular free wall longitudinal strain FAC Fractional area change TAPSE Tricuspid annular plane systolic excursion MPI Myocardial performance index RV-EDV Right ventricle end-diastolic volume RV-EDVi Indexed right ventricle end-diastolic volume RV-ESV Right ventricle end-systolic volume RV-ESVi Indexed right ventricle end-systolic volume NYHA New York Heart Association Introduction Ebstein’s anomaly (EA) is characterized by a failure in the delamination of the tricuspid valve leafets from the adjacent myocardium, resulting in various degrees of tri- cuspid valve abnormalities. The myocardium is invariably abnormal in EA, characterized by the so-called myopa- thy of the right ventricle (RV) [1–3]. There is evidence to support that RV dysfunction is present in EA patients * Alessandro Cavalcanti Lianza alessandro.lianza@einstein.br Extended author information available on the last page of the article