The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy. (27th March 2021)

• Record Type: Journal Article
• Authors: 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;
• Is Part Of: European journal of preventive cardiology. Volume 29:Number 4(2022)
• Page Start: 645
• Page End: 653
• Publication Date: 2021-03-27
• Subjects: Children -- Hypertrophic -- Cardiomyopathy -- Sudden death -- Electrocardiogram; Cardiovascular system -- Diseases -- Prevention -- Periodicals; Cardiac patients -- Rehabilitation -- Periodicals; 616.12
• DOI: 10.1093/eurjpc/zwab046
• Languages: English
• ISSNs: 2047-4873

Abstract

Abstract: 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.