Рекомендации ESC по спортивной кардиологии и физическим тренировкам у пациентов с сердечно-сосудистыми заболеваниями 2020Рабочая группа Европейского кардиологического общества (ESC) по спортивной кардиологии и физическим тренировкам у пациентов с сердечно-сосудистыми заболеваниями Авторы/члены Рабочей группы: Antonio Pelliccia* (Председатель) (Италия), Sanjay Sharma* (Председатель) (Соединенное Королевство), Sabiha Gati (Соединенное Королевство), Maria Bäck (Швеция), Mats Börjesson (Швеция),

Diseases that are associated with sudden cardiac death were identified in 0.38% of adolescent soccer players in a cohort that underwent cardiovascular screening. The incidence of sudden cardiac death was 1 per 14,794 person-years, or 6.8 per 100,000 athletes; most of these deaths were due to cardiomyopathies that had not been detected on screening. (Funded by the English Football Association and others.).

AimsUnderstanding the spectrum of disease, symptom burden and natural history are essential for the management of children with hypertrophic cardiomyopathy (HCM). The effect of changing screening practices over time has not previously been studied. This study describes the clinical characteristics and outcomes of childhood HCM over four decades in a well-characterized United Kingdom cohort.Methods and resultsSix hundred and eighty-seven patients with HCM presented at a median age of 5.2 years (range 0–16). Aetiology was: non-syndromic (n = 433, 63%), RASopathy (n = 126, 18.3%), Friedreich’s ataxia (n = 59, 8.6%) or inborn errors of metabolism (IEM) (n = 64, 9%). In infants (n = 159, 23%) underlying aetiology was more commonly a RASopathy (42% vs. 11.2%, P < 0.0001) or IEM (18.9% vs. 6.4% P < 0.0001). In those with familial disease, median age of presentation was higher (11 years vs. 6 years, P < 0.0001), 141 (58%) presented <12 years. Freedom from death or transplantation was 90.6% (87.9–92.7%) at 5 years (1.5 per 100 patient years) with no era effect. Mortality was most frequently sudden cardiac death (SCD) (n = 20, 2.9%). Children diagnosed during infancy or with an IEM had a worse prognosis (5-year survival 80.5% or 66.4%). Arrhythmic events occurred at a rate of 1.2 per 100 patient years and were more likely in non-syndromic patients (n = 51, 88%).ConclusionThis national study describes a heterogeneous disease whose outcomes depend on the age of presentation and aetiology. Overall mortality and SCD rates have not changed over time, but they remain higher than in adults with HCM, with events occurring in syndromic and non-syndromic patients.

Medication errors occurred commonly in this study, but adverse consequences were rare. The non-punitive, multidisciplinary approach to medication errors utilised in this study increased staff vigilance, highlighted sources of recurrent error, and led to changes in drug policies and staff training, which resulted in improved patient safety and quality of care.

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