Validation of the HCM Risk-SCD model in patients with hypertrophic cardiomyopathy and future perspectives.
HCM risk-SCD
ICD
hypertrophic cardiomyopathy
sudden cardiac death
Journal
Pacing and clinical electrophysiology : PACE
ISSN: 1540-8159
Titre abrégé: Pacing Clin Electrophysiol
Pays: United States
ID NLM: 7803944
Informations de publication
Date de publication:
12 2023
12 2023
Historique:
revised:
04
10
2023
received:
02
09
2023
accepted:
05
11
2023
medline:
17
12
2023
pubmed:
21
11
2023
entrez:
21
11
2023
Statut:
ppublish
Résumé
The hypertrophic cardiomyopathy (HCM) risk- sudden cardiac death (SCD) model provides a convenient tool for determining the risk of SCD in patients with HCM even though some patients with low-risk scores still remain at risk of SCD. Hence, the aim of our study was to assess the performance of HCM Risk-SCD in a large series of consecutive patients with HCM who had been followed up in a tertiary center. The study population consisted of 389 consecutive HCM patients who had been followed up between 2004 and 2021. Demographic and clinical characteristics, estimated 5-year risk using the HCM Risk-SCD model, were compiled, and survival data were collected during follow-up. Patients were divided into 2 groups according to their long-term survival, and HCM risk-SCD scores of these two groups were compared. The long-term mortality was observed in 47 patients out of 389 patients in the during a mean follow-up of 55.5 ± 12.7 months. The mean HCM Risk-SCD score of surviving patients was significantly lower than that of non-survivors (1.8% vs. 3.0%, p < .001). The HCM Risk-SCD score was above 6% in nine (2.6%) survivors and nine (19.1%) non-survivors (p < .001). The ROC curve based on the HCM Risk-SCD score had 61% sensitivity and 61% specificity for risk threshold of for 2.0%, 38% sensitivity and 99% specificity a threshold of ≥4%, 17% sensitivity, and 99% specificity for a threshold of ≥6%. A new risk algorithm with higher sensitivity is needed, although the HCM risk-SCD model is still quite useful in identifying patients at a high risk for SCD.
Sections du résumé
BACKGROUND
The hypertrophic cardiomyopathy (HCM) risk- sudden cardiac death (SCD) model provides a convenient tool for determining the risk of SCD in patients with HCM even though some patients with low-risk scores still remain at risk of SCD. Hence, the aim of our study was to assess the performance of HCM Risk-SCD in a large series of consecutive patients with HCM who had been followed up in a tertiary center.
METHODS
The study population consisted of 389 consecutive HCM patients who had been followed up between 2004 and 2021. Demographic and clinical characteristics, estimated 5-year risk using the HCM Risk-SCD model, were compiled, and survival data were collected during follow-up. Patients were divided into 2 groups according to their long-term survival, and HCM risk-SCD scores of these two groups were compared.
RESULTS
The long-term mortality was observed in 47 patients out of 389 patients in the during a mean follow-up of 55.5 ± 12.7 months. The mean HCM Risk-SCD score of surviving patients was significantly lower than that of non-survivors (1.8% vs. 3.0%, p < .001). The HCM Risk-SCD score was above 6% in nine (2.6%) survivors and nine (19.1%) non-survivors (p < .001). The ROC curve based on the HCM Risk-SCD score had 61% sensitivity and 61% specificity for risk threshold of for 2.0%, 38% sensitivity and 99% specificity a threshold of ≥4%, 17% sensitivity, and 99% specificity for a threshold of ≥6%.
CONCLUSION
A new risk algorithm with higher sensitivity is needed, although the HCM risk-SCD model is still quite useful in identifying patients at a high risk for SCD.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1519-1525Informations de copyright
© 2023 Wiley Periodicals LLC.
Références
Maron BJ, Ommen SR, Semsarian C, Spirito P, Olivotto I, Maron MS. Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol. 2014;64(1):83-99.
Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE. Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults. Circulation. 1995;92(4):785-789.
Trivedi A, Knight BP. ICD Therapy for primary prevention in hypertrophic cardiomyopathy. Arrhythmia Electrophysiol Rev. 2016;5(3):188-196.
Maron BJ, Olivotto I, Spirito P, et al. Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non-referral-based patient population. Circulation. 2000;102(8):858-864.
Hypertrophic Cardiomyopathy Outcomes Investigators. O'Mahony C, Jichi F, Pavlou M, et al. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J. 2014;35(30):2010-2020.
Maron BJ, Rowin EJ, Casey SA, et al. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. J Am Coll Cardiol. 2015;65(18):1915-1928.
Maron BJ, Maron MS, Semsarian C. Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. J Am Coll Cardiol. 2012;60(8):705-715.
Slama M, Tribouilloy C, Maizel J. Left ventricular outflow tract obstruction in ICU patients. Curr Opin Crit Care. 2016;22(3):260-266.
Authors/Task Force members, Elliott PM, Anastasakis A, Borger MA, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35(39):2733-2779.
American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol. 2011;58(25):e212-e260.
Maron MS, Rowin EJ, Wessler BS, et al. Enhanced American College of Cardiology/American Heart Association Strategy for prevention of sudden cardiac death in high-risk patients with hypertrophic cardiomyopathy. JAMA Cardiol. 2019;4(7):644-657.
Doesch C, Tülümen E, Akin I, et al. Incremental benefit of late gadolinium cardiac magnetic resonance imaging for risk stratification in patients with hypertrophic cardiomyopathy. Sci Rep. 2017;7(1):6336.
Maron BJ, Rowin EJ, Maron MS. Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: twenty years with the implantable cardioverter-defibrillator. Heart Rhythm. 2021;18(6):1012-1023.
Oka K, Tsujino T, Nakao S, et al. Symptomatic ventricular tachyarrhythmia is associated with delayed gadolinium enhancement in cardiac magnetic resonance imaging and with elevated plasma brain natriuretic peptide level in hypertrophic cardiomyopathy. J Cardiol. 2008;52(2):146-153.
Vriesendorp PA, Schinkel AF, Van Cleemput J, et al. Implantable cardioverter-defibrillators in hypertrophic cardiomyopathy: patient outcomes, rate of appropriate and inappropriate interventions, and complications. Am Heart J. 2013;166(3):496-502.
Girolami F, Ho CY, Semsarian C, et al. Clinical features and outcome of hypertrophic cardiomyopathy associated with triple sarcomere protein gene mutations. J Am Coll Cardiol. 2010;55(14):1444-1453.
Saumarez R, Silberbauer J, Scannell J, et al. Should lethal arrhythmias in hypertrophic cardiomyopathy be predicted using non-electrophysiological methods?. Europace. 2023;25(5):euad045.
Maron BJ, Casey SA, Chan RH, Garberich RF, Rowin EJ, Maron MS. Independent assessment of the European Society of Cardiology Sudden Death Risk Model for hypertrophic cardiomyopathy. Am J Cardiol. 2015;116(5):757-764.
Tsang TS, Abhayaratna WP, Barnes ME, et al. Prediction of cardiovascular outcomes with left atrial size: is volume superior to area or diameter? J Am Coll Cardiol. 2006;47(5):1018-1023.
Tsang TS, Barnes ME, Gersh BJ, Bailey KR, Seward JB. Left atrial volume as a morphophysiologic expression of left ventricular diastolic dysfunction and relation to cardiovascular risk burden. Am J Cardiol. 2002;90(12):1284-1289.
Wang N, Xie A, Tjahjono R, et al. Implantable cardioverter defibrillator therapy in hypertrophic cardiomyopathy: an updated systematic review and meta-analysis of outcomes and complications. Ann Cardiothorac Surg. 2017;6(4):298-306.
Woo A, Monakier D, Harris L, et al. Determinants of implantable defibrillator discharges in high-risk patients with hypertrophic cardiomyopathy. Heart. 2007;93(9):1044-1045.
Losi MA, Betocchi S, Aversa M, et al. Determinants of atrial fibrillation development in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2004;94(7):895-900.