Enhancing Arrhythmogenic Right Ventricular Cardiomyopathy Detection and Risk Stratification: Insights from Advanced Echocardiographic Techniques
Natália Olivetti,
Luciana Sacilotto,
Danilo Bora Moleta,
Lucas Arraes de França,
Lorena Squassante Capeline,
Fanny Wulkan,
Tan Chen Wu,
Gabriele D’Arezzo Pessente,
Mariana Lombardi Peres de Carvalho,
Denise Tessariol Hachul,
Alexandre da Costa Pereira,
José E. Krieger,
Mauricio Ibrahim Scanavacca,
Marcelo Luiz Campos Vieira,
Francisco Darrieux
Affiliations
Natália Olivetti
Arrhythmia Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Luciana Sacilotto
Arrhythmia Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Danilo Bora Moleta
Echocardiogram Imaging Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Lucas Arraes de França
Echocardiogram Imaging Unit, Hospital Israelita Albert Einstein, Sao Paulo 05652-900, Brazil
Lorena Squassante Capeline
Laboratory of Genetics and Molecular Cardiology, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Fanny Wulkan
Laboratory of Genetics and Molecular Cardiology, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Tan Chen Wu
Arrhythmia Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Gabriele D’Arezzo Pessente
Arrhythmia Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Mariana Lombardi Peres de Carvalho
Laboratory of Genetics and Molecular Cardiology, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Denise Tessariol Hachul
Arrhythmia Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Alexandre da Costa Pereira
Laboratory of Genetics and Molecular Cardiology, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
José E. Krieger
Laboratory of Genetics and Molecular Cardiology, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Mauricio Ibrahim Scanavacca
Arrhythmia Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Marcelo Luiz Campos Vieira
Echocardiogram Imaging Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Francisco Darrieux
Arrhythmia Unit, Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo 05403-900, Brazil
Introduction: The echocardiographic diagnosis criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC) are highly specific but sensitivity is low, especially in the early stages of the disease. The role of echocardiographic strain in ARVC has not been fully elucidated, although prior studies suggest that it can improve the detection of subtle functional abnormalities. The purposes of the study were to determine whether these advanced measures of right ventricular (RV) dysfunction on echocardiogram, including RV strain, increase diagnostic value for ARVC disease detection and to evaluate the association of echocardiographic parameters with arrhythmic outcomes. Methods: The study included 28 patients from the Heart Institute of São Paulo ARVC cohort with a definite diagnosis of ARVC established according to the 2010 Task Force Criteria. All patients were submitted to ECHO’s advanced techniques including RV strain, and the parameters were compared to prior conventional visual ECHO and CMR. Results: In total, 28 patients were enrolled in order to perform ECHO’s advanced techniques. A total of 2/28 (7%) patients died due to a cardiovascular cause, 2/28 (7%) underwent heart transplantation, and 14/28 (50%) patients developed sustained ventricular arrhythmic events. Among ECHO’s parameters, RV dilatation, measured by RVDd (p = 0.018) and RVOT PSAX (p = 0.044), was significantly associated with arrhythmic outcomes. RV free wall longitudinal strain p = 0.033). Conclusion: Our data suggest that ECHO’s advanced techniques improve ARVC detection and that abnormal RV strain can be associated with arrhythmic risk stratification. Further studies are necessary to better demonstrate these findings and contribute to risk stratification in ARVC, in addition to other well-known risk markers.
