[Long and short QT syndromes : Emergency treatment and secondary prophylaxis]. / Long- und Short-QT-Syndrome : Notfalltherapie und Sekundärprophylaxe.

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.