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Long- und Short-QT-Syndrome

Notfalltherapie und Sekundärprophylaxe

Long and short QT syndromes

Emergency treatment and secondary prophylaxis

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Zusammenfassung

Das Long-QT-Syndrom (LQTS) ist eine seltene Ionenkanalerkrankung, die kongenital oder erworben auftritt und unbehandelt mit einer signifikanten Mortalität einhergeht. Unter Therapie kann diese stark gesenkt werden. Es existieren 17 Subtypen, von denen LQTS 1–3 am häufigsten auftreten. Das Spektrum reicht von asymptomatischen Anlagenträgern bis zu Torsade de Pointes (TdP) mit plötzlichem Herztod („sudden cardiac death“, SCD). Die Notfalltherapie besteht neben Defibrillation aus Magnesiumgabe, Betablockade und ggf. passagerem Herzschrittmacher sowie Sedierung. Die Sekundärprophylaxe beruht auf einer Betablockertherapie und ggf. ICD-Implantation. Sowohl im Notfall als auch zur Dauertherapie stellt die kardiale Denervation eine probate Therapie dar. Das Short-QT-Syndrom (SQTS) eine sehr seltene Ionenkanalerkrankung, die sich in 34 % als SCD mainifestiert. Bislang wurden ca. 250 Fälle mit Mutationen in 8 Genen beschrieben. Der ICD ist der einzig verlässliche Schutz vor SCD durch polymorphe ventrikuläre Tachykardien/Kammerflimmern (VT/VF). Die medikamentöse Therapie beruht auf Hydrochinidin. Für einzelne Subtypen beider Erkrankungen bestehen teils weitere Therapieoptionen. Die Behandlung beider Erkrankungen sollte in spezialisierten Zentren erfolgen.

Abstract

Long QT syndrome (LQTS) is a rare inherited or acquired channelopathy associated with a relevant mortality if left untreated. Therapy can reduce the sudden cardiac death (SCD) rate significantly. Of 17 subtypes, LQTS1–3 are the most common. Clinical presentation ranges from asymptomatic patients to torsade de pointes (TdP) and SCD. Emergency therapy includes defibrillation, administration of magnesium, betablockers and temporary pacing and sedation. Secondary prevention is based on betablocker therapy and implantation of an implantable cardioverter–defibrillator (ICD), if appropriate. Short QT syndrome (SQTS) is a rare channelopathy that manifests as SCD in 34%. So far 250 cases with mutations in 8 genes have been reported. ICDs are the only reliable protection against SCD. Drug therapy is based on hydroquinidine. Further therapeutic options exist for certain subtypes of both diseases. Patients should be referred to specialized centers.

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Authors and Affiliations

  1. Klinik für Innere Medizin, Kardiologie, Nephrologie und internistische Intensivmedizin, Klinikum Ludwigsburg, Posilipostraße 4, 71640, Ludwigsburg, Deutschland

    N. Horn, N. Rüb & C. Wolpert

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N. Horn, N. Rüb und C. Wolpert geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Horn, N., Rüb, N. & Wolpert, C. Long- und Short-QT-Syndrome. Herzschr Elektrophys 31, 48–54 (2020). https://doi.org/10.1007/s00399-020-00666-y

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