Sudden Cardiac Death and ICD Use in Rasopathy-Associated Hypertrophic Cardiomyopathy
| Autor: | Joseph W. Rossano, Stephanie J. Nakano, Paul F. Kantor, Katey R. Armstrong, Jennifer Conway, Pete F. Aziz, Ann M. Lynch, B. Seshadri, Robert Whitehill, R. Weintraub, Heather T. Henderson, L. Gardin, S. Ahuja, A. Miron, Marc E. Richmond, Seema Mital, Beth D. Kaufman, Ashwin K. Lal, Taylor S. Howard, John J. Parent, Steve Colan, Chet R. Villa, Justin Godown, Ryan J. Butts, Mark W. Russell |
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| Rok vydání: | 2021 |
| Předmět: |
Pulmonary and Respiratory Medicine
Transplantation medicine.medical_specialty business.industry Incidence (epidemiology) Hypertrophic cardiomyopathy Retrospective cohort study Sudden cardiac arrest macromolecular substances RASopathy medicine.disease Sudden death Sudden cardiac death Internal medicine cardiovascular system Cardiology Medicine Surgery Cumulative incidence cardiovascular diseases medicine.symptom Cardiology and Cardiovascular Medicine business |
| Zdroj: | The Journal of Heart and Lung Transplantation. 40:S33-S34 |
| ISSN: | 1053-2498 |
| DOI: | 10.1016/j.healun.2021.01.1821 |
| Popis: | Purpose The incidence and risk factors for sudden cardiac death in Rasopathy patients with hypertrophic cardiomyopathy (Ras-HCM) are not well understood. We compared the cumulative incidence of sudden death and ICD therapy in Ras-HCM compared to primary HCM. Methods Phenotype-positive pediatric patients with Ras-HCM (n=157) and primary HCM (n=720) were included in this 22-center retrospective cohort study. The primary outcome was 5-year freedom from sudden death events, defined as a composite of sudden death, resuscitated sudden cardiac arrest, and aborted events i.e. appropriate shock from a primary prevention ICD, estimated using the Kaplan Meier method. Results There were 17 deaths in Ras-HCM patients - 8 sudden death, 9 from noncardiac causes. The 5-year freedom from sudden death events was higher in Ras-HCM than in primary HCM (97.2% vs 93.8% respectively, p=0.039) (Figure 1A). The median age at diagnosis was lower in Ras-HCM (1.1 yr) versus primary HCM (10 yrs) (p Conclusion The incidence of sudden cardiac death is lower in pediatric Rasopathy patients with HCM compared to primary HCM with most events occurring in infancy. It is less likely for Rasopathy patients to receive an ICD than a primary HCM patient even for secondary prevention, possibly due to younger patient age at the time of the event. Further research on risk factors for and mechanisms of sudden death in Ras-HCM is warranted. |
| Databáze: | OpenAIRE |
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