Patients with obstructive hypertrophic cardiomyopathy (oHCM) have increased risk of arrhythmia, stroke, heart failure, and sudden death. Contemporary management of oHCM has decreased annual hospitalization and mortality rates, yet patients have worsening health-related quality of life due to impaired exercise capacity and persistent residual symptoms. Here we consider the design of clinical trials evaluating potential oHCM therapies in the context of SEQUOIA-HCM (Safety, Efficacy, and Quantitative Understanding of Obstruction Impact of Aficamten in HCM). This large, phase 3 trial is now fully enrolled (N = 282). Baseline characteristics reflect an ethnically diverse population with characteristics typical of patients encountered clinically with substantial functional and symptom burden. The study will assess the effect of aficamten vs placebo, in addition to standard-of-care medications, on functional capacity and symptoms over 24 weeks. Future clinical trials could model the approach in SEQUOIA-HCM to evaluate the effect of potential therapies on the burden of oHCM. (Safety, Efficacy, and Quantitative Understanding of Obstruction Impact of Aficamten in HCM [SEQUOIA-HCM]; NCT05186818).
- MeSH
- hypertrofická kardiomyopatie * komplikace MeSH
- kvalita života MeSH
- lidé MeSH
- sekvoj * MeSH
- srdeční selhání * farmakoterapie MeSH
- tolerance zátěže MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
BACKGROUND: Sudden deaths in competitive athletes are highly visible but potentially preventable events that generate great interest amongst the cardiovascular community and general public. METHODS: Internet searches were performed using a combination of keywords and operators to produce search results for sudden death or cardiac arrest on the field in professional soccer players. RESULTS: We identified 35 male professional soccer players (mean age 26 ± 5 years) who experienced collapse and cardiac arrest on the field (most during matches) in Europe from December 2002 to February 2022 with 63% in the last 6 years. Twenty-five have died on the field or later in a hospital despite cardiopulmonary resuscitation. Of the 10 survivors, eight were implanted with cardioverter-defibrillators for secondary (n = 6) or primary (n = 2) prevention and returned to full competition; five of the 8 required successful device therapy during matches or training. CONCLUSIONS: Cardiac arrest and sudden death can occur not uncommonly in professional athletes highly trained over decades and participating at an elite sports level. Our observations also underscore the importance of targeted preparticipation cardiovascular screening, and availability of external defibrillators on the playing field.
- MeSH
- dospělí MeSH
- fotbal * MeSH
- kardiopulmonální resuscitace * metody MeSH
- lidé MeSH
- mladý dospělý MeSH
- náhlá srdeční smrt etiologie prevence a kontrola MeSH
- sportovci MeSH
- sporty * MeSH
- srdeční zástava * etiologie terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Hypertrophic cardiomyopathy (HCM) has been considered the most common cause of sudden death (SD) in the young. However, introduction of implantable cardioverter-defibrillators (ICDs) in HCM has proved highly effective and the mainstay of preventing SD in children, adolescents, and adults by terminating malignant ventricular tachyarrhythmias. Nevertheless, ICD decision making is generally regarded as more difficult in pediatrics, and the strategy for selecting ICD patients from this population remains without consensus. Prospective studies in HCM children and adolescents have shown the American Heart Association/American College of Cardiology traditional major risk marker strategy to be reliable with >90% sensitivity in selecting patients for SD prevention. International data in >2000 young HCM patients assembled over 20 years who were stratified by major risk markers showed ICDs effectively prevented SD in 20%. Alternatively, novel quantitative risk scoring initiatives provide 5-year risk estimates that are potentially useful as adjunctive tools to facilitate discussion of prophylactic ICD risks vs benefit but are as yet unsupported by prospective outcome studies. Risk scoring strategies are characterized by reasonable discriminatory statistical power (C-statistic 0.69-0.76) for identifying patients with SD events but with relatively low sensitivity, albeit with specificity comparable with the risk marker strategy. While some reticence for obligating healthy-appearing young patients to lifelong device implants is understandable, underutilization of the ICD in high-risk children and adolescents can represent a lost opportunity for fulfilling the long-standing aspiration of SD prevention. This review provides a critical assessment of the current strengths and weaknesses of SD risk stratification strategies in young HCM patients in an effort to clarify clinical decision making in this challenging subpopulation.
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
