Details

Autor(en) / Beteiligte

• Norrish, Gabrielle
• Topriceanu, Cristian
• Qu, Chen
• Field, Ella
• Walsh, Helen
• Ziółkowska, Lidia
• Olivotto, Iacopo
• Passantino, Silvia
• Favilli, Silvia
• Anastasakis, Aris
• Vlagkouli, Vasiliki
• Weintraub, Robert
• King, Ingrid
• Biagini, Elena
• Ragni, Luca
• Prendiville, Terrence
• Duignan, Sophie
• McLeod, Karen
• Ilina, Maria
• Fernández, Adrian
• Bökenkamp, Regina
• Baban, Anwar
• Drago, Fabrizio
• Kubuš, Peter
• Daubeney, Piers E F
• Chivers, Sian
• Sarquella-Brugada, Georgia
• Cesar, Sergi
• Marrone, Chiara
• Medrano, Constancio
• Alvarez Garcia-Roves, Reyes
• Uzun, Orhan
• Gran, Ferran
• Castro, Fernandez J
• Gimeno, Juan R
• Barriales-Villa, Roberto
• Rueda, Fernando
• Adwani, Satish
• Searle, Jonathan
• Bharucha, Tara
• Siles, Ana
• Usano, Ana
• Rasmussen, Torsten B
• Jones, Caroline B
• Kubo, Toru
• Mogensen, Jens
• Reinhardt, Zdenka
• Cervi, Elena
• Elliott, Perry M
• Omar, Rumana Z
• Kaski, Juan P

Titel

The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy

Ist Teil von

• European journal of preventive cardiology, 2022-03, Vol.29 (4), p.645-653

Ort / Verlag

England

Erscheinungsjahr

2022

Quelle

MEDLINE

Beschreibungen/Notizen

• 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. 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. 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.