The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy
Aims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype o...
| Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
|---|---|
| Formato: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2022 |
| País: | España |
| Recursos: | Fundació Sant Joan de Déu |
| Repositorio: | r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
| OAI Identifier: | oai:fsjd.fundanetsuite.com:p19329 |
| Acesso em linha: | https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19329 |
| Access Level: | acceso abierto |
| Palavra-chave: | Children Hypertrophic Cardiomyopathy Sudden death Electrocardiogram |
| id |
ES_5081067b1f2dcabdfd8de2b0c539343d |
|---|---|
| oai_identifier_str |
oai:fsjd.fundanetsuite.com:p19329 |
| network_acronym_str |
ES |
| network_name_str |
España |
| repository_id_str |
|
| spelling |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathyNorrish GTopriceanu CQu CField EWalsh HZiólkowska LOlivotto IPassantino SFavilli SAnastasakis AVlagkouli VWeintraub RKing IBiagini ERagni LPrendiville TDuignan SMcLeod KIlina MFernández ABökenkamp RBaban ADrago FKubuš PDaubeney PEFChivers SSarquella-Brugada GCesar SMarrone CMedrano CAlvarez Garcia-Roves RUzun OGran FCastro FJGimeno JRBarriales-Villa RRueda FAdwani SSearle JBharucha TSiles AUsano ARasmussen TBJones CBKubo TMogensen JReinhardt ZCervi EElliott PMOmar RZKaski JPChildrenHypertrophicCardiomyopathySudden deathElectrocardiogramAims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods and results Data from 356 childhood HCM patients with a mean age of 10.1 years (+/- 4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484-0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7% Conclusion In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited.OXFORD UNIV PRESS2022info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19329European Journal of Preventive CardiologyISSN: 20474873ISSNe: 20474881reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déuinstname:Fundació Sant Joan de DéuInglésinfo:eu-repo/semantics/openAccessoai:fsjd.fundanetsuite.com:p193292026-05-27T12:37:41Z |
| dc.title.none.fl_str_mv |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy |
| title |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy |
| spellingShingle |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy Norrish G Children Hypertrophic Cardiomyopathy Sudden death Electrocardiogram |
| title_short |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy |
| title_full |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy |
| title_fullStr |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy |
| title_full_unstemmed |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy |
| title_sort |
The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy |
| dc.creator.none.fl_str_mv |
Norrish G Topriceanu C Qu C Field E Walsh H Ziólkowska L Olivotto I Passantino S Favilli S Anastasakis A Vlagkouli V Weintraub R King I Biagini E Ragni L Prendiville T Duignan S McLeod K Ilina M Fernández A Bökenkamp R Baban A Drago F Kubuš P Daubeney PEF Chivers S Sarquella-Brugada G Cesar S Marrone C Medrano C Alvarez Garcia-Roves R Uzun O Gran F Castro FJ Gimeno JR Barriales-Villa R Rueda F Adwani S Searle J Bharucha T Siles A Usano A Rasmussen TB Jones CB Kubo T Mogensen J Reinhardt Z Cervi E Elliott PM Omar RZ Kaski JP |
| author |
Norrish G |
| author_facet |
Norrish G Topriceanu C Qu C Field E Walsh H Ziólkowska L Olivotto I Passantino S Favilli S Anastasakis A Vlagkouli V Weintraub R King I Biagini E Ragni L Prendiville T Duignan S McLeod K Ilina M Fernández A Bökenkamp R Baban A Drago F Kubuš P Daubeney PEF Chivers S Sarquella-Brugada G Cesar S Marrone C Medrano C Alvarez Garcia-Roves R Uzun O Gran F Castro FJ Gimeno JR Barriales-Villa R Rueda F Adwani S Searle J Bharucha T Siles A Usano A Rasmussen TB Jones CB Kubo T Mogensen J Reinhardt Z Cervi E Elliott PM Omar RZ Kaski JP |
| author_role |
author |
| author2 |
Topriceanu C Qu C Field E Walsh H Ziólkowska L Olivotto I Passantino S Favilli S Anastasakis A Vlagkouli V Weintraub R King I Biagini E Ragni L Prendiville T Duignan S McLeod K Ilina M Fernández A Bökenkamp R Baban A Drago F Kubuš P Daubeney PEF Chivers S Sarquella-Brugada G Cesar S Marrone C Medrano C Alvarez Garcia-Roves R Uzun O Gran F Castro FJ Gimeno JR Barriales-Villa R Rueda F Adwani S Searle J Bharucha T Siles A Usano A Rasmussen TB Jones CB Kubo T Mogensen J Reinhardt Z Cervi E Elliott PM Omar RZ Kaski JP |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Children Hypertrophic Cardiomyopathy Sudden death Electrocardiogram |
| topic |
Children Hypertrophic Cardiomyopathy Sudden death Electrocardiogram |
| description |
Aims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods and results Data from 356 childhood HCM patients with a mean age of 10.1 years (+/- 4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484-0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7% Conclusion In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited. |
| publishDate |
2022 |
| dc.date.none.fl_str_mv |
2022 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
| format |
article |
| status_str |
publishedVersion |
| dc.identifier.none.fl_str_mv |
https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19329 |
| url |
https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=19329 |
| dc.language.none.fl_str_mv |
Inglés |
| language_invalid_str_mv |
Inglés |
| dc.rights.none.fl_str_mv |
info:eu-repo/semantics/openAccess |
| eu_rights_str_mv |
openAccess |
| dc.publisher.none.fl_str_mv |
OXFORD UNIV PRESS |
| publisher.none.fl_str_mv |
OXFORD UNIV PRESS |
| dc.source.none.fl_str_mv |
European Journal of Preventive Cardiology ISSN: 20474873 ISSNe: 20474881 reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu instname:Fundació Sant Joan de Déu |
| instname_str |
Fundació Sant Joan de Déu |
| reponame_str |
r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
| collection |
r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
| repository.name.fl_str_mv |
|
| repository.mail.fl_str_mv |
|
| _version_ |
1869407894889299968 |
| score |
15,81155 |