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

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Abstract

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.

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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

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Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Dr. Mercer-Rosa is supported by grant NIH K01HL125521 and by Pulmonary Hypertension Association Supplement to K01HL125521.

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Authors and Affiliations

  1. Echocardiography Laboratory, Hospital Israelita Albert Einstein, Av. Albert Einstein, 627/701, Morumbi, São Paulo, SP, 05652-900, Brazil

    Alessandro Cavalcanti Lianza, Ana Clara T. Rodrigues, Marcelo L. C. Vieira, Wercules A. A. de Oliveira, Tania Regina Afonso, Glaucia M. P. Tavares, Claudio H. Fischer & Samira S. Morhy

  2. Division of Cardiology, Pediatrics Perelman School of Medicine, Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, USA

    Laura Mercer-Rosa

  3. Radiology Unit, Hospital Sirio Libanês, São Paulo, Brazil

    Cesar H. Nomura

  4. Center for Valve Therapy, Children’s Hospital of Pittsburg, Pittsburg, USA

    Jose Pedro da Silva

  5. Cardiothoracic Surgery, Children’s Hospital of Pittsburg, Pittsburg, USA

    Luciana da Fonseca da Silva

  6. Radiology Unit, Hospital Israelita Albert Einstein, São Paulo, Brazil

    Gilberto Szarf

Authors
  1. Alessandro Cavalcanti Lianza
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  2. Ana Clara T. Rodrigues
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  3. Laura Mercer-Rosa
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  4. Marcelo L. C. Vieira
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  5. Wercules A. A. de Oliveira
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  8. Jose Pedro da Silva
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  9. Luciana da Fonseca da Silva
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  11. Glaucia M. P. Tavares
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Correspondence to Alessandro Cavalcanti Lianza.

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Lianza, A.C., Rodrigues, A.C.T., Mercer-Rosa, L. et al. Right Ventricular Systolic Function After the Cone Procedure for Ebstein's Anomaly: Comparison Between Echocardiography and Cardiac Magnetic Resonance. Pediatr Cardiol 41, 985–995 (2020). https://doi.org/10.1007/s00246-020-02347-6

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  • DOI: https://doi.org/10.1007/s00246-020-02347-6

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