Long QT syndrome type 2 – can alarm clock really kill? (RCD code: V-1A.2)

Marek Czajkowski

Full Text:



This paper’s aim was to critically analyze existing research supporting statement found in many review articles that auditory stimuli may specifically and exclusively trigger cardiac events in patients with type 2 of congenital long QT syndrome (LQTS2). By the use of thorough systematic literature search relevant papers were gathered and analyzed. As a result, 8 publications were included in this analysis: 5 case-series studies, 2 single case reports and 1 expert opinion. 5 of the studies proved that auditory stimuli often precede cardiac events in patients with many types of congenital long QT syndrome (LQTS). None of the included studies concerned auditory stimulus as an exclusive trigger of cardiac events in LQTS2 patients (it was most often combined with exercise and/or sleep, or performed on groups with small part of LQTS2 patients). There is lack of strong evidence that auditory stimuli specifically and exclusively trigger cardiac events in patients with LQTS2. However, high occurrence of cardiac events shortly after being awoken from sleep by auditory stimuli and nocturnal deaths in families of patients with LQTS2 may provide indirect support. Due to high risk associated with auditory stimuli for patients with all types of LQTS it is still advisable for them to remove loud and noisy equipment from their close environment. JRCD 2014; 1 (7): 4–6


LQTS2, auditory stimuli, cardiac events


Curran ME, Splawski I, Timothy KW, et al. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell 1995; 80: 795–803.

Roden DM, Lazzara R, Rosen M, et al. Multiple mechanisms in the long‑QT syndrome. Current knowledge, gaps, and future directions. The SADS Foundation Task Force on LQTS. Circulation 1996; 94: 1996–2012.

Goldenberg I, Moss AJ, Zareba W. QT interval: how to measure it and what is “normal”. Journal of cardiovascular electrophysiology 2006; 17: 333–336.

Topaz O, Castellanos A, Grobman LR, et al. The role of arrhythmogenic auditory stimuli in sudden cardiac death. American heart journal 1988; 116: 222–226.

Garson A, Jr. Dick M 2nd, Fournier A, et al. The long QT syndrome in children. An international study of 287 patients. Circulation 1993; 87: 1866–1872.

Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype‑phenotype correlation in the long‑QT syndrome: gene‑specific triggers for life‑threatening arrhythmias. Circulation 2001; 103: 89–95.

Moss AJ, Robinson JL, Gessman L, et al. Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming

among subjects with the long QT syndrome. The American journal of cardiology 1999; 84: 876–879.

Wilde AA, Jongbloed RJ, Doevendans PA, et al. Auditory stimuli as a trigger for arrhythmic events differentiate HERG‑related (LQTS2) patients

from KVLQT1‑related patients (LQTS1). Journal of the American College of Cardiology 1999; 33: 327–332.

Jongbloed RJ, Wilde AA, Geelen JL, et al. Novel KCNQ1 and HERG missense mutations in Dutch long‑QT families. Human mutation 1999; 13: 301-310.

Wellens HJ, Vermeulen A, Durrer D. Ventricular fibrillation occurring on arousal from sleep by auditory stimuli. Circulation 1972; 46: 661–665.

Nakajima T, Misu K, Iwasawa K, et al. Auditory stimuli as a major cause of syncope in a patient with idiopathic long QT syndrome. Japanese circulation journal 1995; 59: 241–246.

DOI: http://dx.doi.org/10.20418%2Fjrcd.vol1no7.112


  • There are currently no refbacks.
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659
e-mail: softq@softq.pl