Феномен или синдром Бругада?

Синдром Бругада (СБ) – наследственная сердечная каналопатия, характеризующаяся повышенным риском развития внезапной сердечной смерти (ВСС). Несмотря на редкую встречаемость синдрома СБ в популяции, своевременная диагностика и стратификация риска является актуальной задачей на сегодняшний день.
Наличие обмороков аритмического генеза в сочетании со спонтанным электрокардиографическим паттерном СБ 1-го типа является надежным маркером неблагоприятного прогноза, в то время как в группе с неаритмическими синкопальными состояниями не наблюдается повышенного риска жизнеугрожающих желудочковых тахиаритмий. Тем не менее этиологию синкопальных состояний трудно определить в 30% случаев, что является актуальной проблемой на сегодняшний день. Известно, что данные, полученные с помощью имплантируемых петлевых регистраторов, изменяли терапевтический подход у 20–36% пациентов с СБ, имеющих необъяснимые обмороки, что является важным аспектом стратификации риска ВСС у данной группы пациентов.
В данной статье представлен краткий обзор медицинской литературы и клинический случай диагностики синкопальных состояний у пациента с ВСС. Описаны современные подходы к диагностике, профилактике и лечению, включая катетерную аблацию и генную терапию. Дана клиническая характеристика течения заболевания у пациента, приведены данные его лабораторного и инструментального исследования.

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