Andersen-Tawil syndrome electrocardiogram
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Charmaine Patel, M.D. [2]; Raviteja Guddeti, M.B.B.S. [3]
Overview
An ECG may be very helpful in the diagnosis of Andersen-Tawil Syndrome. Findings on an ECG diagnostic of Andersen-Tawil Syndrome include a long QTc (LQT) interval, U waves, wide T-U junction and T-waves.
Electrocardiogram
An ECG may be helpful in the diagnosis of Andersen-Tawil Syndrome. Findings on an ECG diagnostic of Andersen-Tawil Syndrome include:[1]
- A long QTc (LQT) interval
- T-U patterns which are very characteristic to the syndrome[2]
- Prominent U-wave enlargement which are biphasic in limb leads
- A wide T-U junction
- Prolonged terminal T-wave
- Premature ventricular contractions (PVC) especially at "rest" in Andersen-Tawil syndrome (ATS) patients
- PVC's at rest helps in differentiating ATS from catecholaminergic polymorphic ventricular tachycardia (CPVT)
- Polymorphic ventricular tachycardia (PMVT) which is called bidirectional ventricular tachycardia (BiVT)[3]
- PMVT is defined as when the beats are more than 100/min with variation in QRS axis.
- In ATS when the patient deteriorates we can see VF which can lead to sudden death
Electrocardiogram
Although polymorphic ventricular tachycardia is a common arrhythmia in patients with Anderson syndrome, decompensation into a hemodynamically compromising rhythm, or ventricular fibrillation, is rare. The following EKG findings are seen in patients with Andersen-Tawil syndrome:
- Biphasic U waves in limb leads
- Widened T-U junction
- Large U waves
- Prolonged terminal T wave downslope
- Prolonged Q-U intervals
References
- ↑ Kukla P, Biernacka EK, Baranchuk A, Jastrzebski M, Jagodzinska M (2014). "Electrocardiogram in Andersen-Tawil syndrome. New electrocardiographic criteria for diagnosis of type-1 Andersen-Tawil syndrome". Curr Cardiol Rev. 10 (3): 222–8. doi:10.2174/1573403x10666140514102528. PMC 4040873. PMID 24827800.
- ↑ Zhang L, Benson DW, Tristani-Firouzi M, Ptacek LJ, Tawil R, Schwartz PJ; et al. (2005). "Electrocardiographic features in Andersen-Tawil syndrome patients with KCNJ2 mutations: characteristic T-U-wave patterns predict the KCNJ2 genotype". Circulation. 111 (21): 2720–6. doi:10.1161/CIRCULATIONAHA.104.472498. PMID 15911703.
- ↑ Khan IA (2002). "Clinical and therapeutic aspects of congenital and acquired long QT syndrome". Am J Med. 112 (1): 58–66. doi:10.1016/s0002-9343(01)01011-7. PMID 11812408.