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Case Reports
. 2011 Dec;100(12):1123-7.
doi: 10.1007/s00392-011-0358-4. Epub 2011 Sep 14.

A rare association of long QT syndrome and syndactyly: Timothy syndrome (LQT 8)

U Krause, V Gravenhorst, T Kriebel, W Ruschewski, T Paul
Case Reports

A rare association of long QT syndrome and syndactyly: Timothy syndrome (LQT 8)

U Krause et al. Clin Res Cardiol. 2011 Dec.
No abstract available

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Figures

Fig. 1
Fig. 1
Characteristic phenotypic features of Timothy syndrome: bald head and lower–set ears, webbing of fingers and toes
Fig. 2
Fig. 2
ECG after birth showed a prolonged QT interval (QTc 600 ms), 2:1 atrioventricular block and significant bradycardia (ventricular rate 60/min)
Fig. 3
Fig. 3
Chest X-ray after implantation of an extracardiac cardioverter defibrillator system at the age of five months
Fig. 4
Fig. 4
At the age of 21 months, patient developed Torsade de pointes ventricular tachycardia which was successfully terminated by ICD discharge (15 J)
See this image and copyright information in PMC

References

    1. Crotti L, Celano G, Dagradi F, Schwartz PJ. Congenital long QT syndrome. Orphanet J Rare Dis. 2008;3:18. doi: 10.1186/1750-1172-3-18. - DOI - PMC - PubMed
    1. Jacobs A, Knight BP, McDonald KT, Burke MC. Verapamil decreases ventricular tachyarrhythmias in a patient with Timothy syndrome (LQT8) Heart Rhythm. 2006;3:967–970. doi: 10.1016/j.hrthm.2006.04.024. - DOI - PubMed
    1. Kriebel T, Ruschewski W, Gonzalez M, et al. ICD Implantation in infants and small children: the extracardiac technique. Pacing Clin Electrophysiol. 2006;29:1319–1325. doi: 10.1111/j.1540-8159.2006.00542.x. - DOI - PubMed
    1. Marks ML, Trippel DL, Keating MT. Long QT syndrome associated with syndactyly identified in females. Am J Cardiol. 1995;76:744–745. doi: 10.1016/S0002-9149(99)80216-1. - DOI - PubMed
    1. Marks ML, Whisler SL, Clericuzio C, Keating M. A new form of long QT syndrome associated with syndactyly. J Am Coll Cardiol. 1995;25:59–64. doi: 10.1016/0735-1097(94)00318-K. - DOI - PubMed

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