Andersen-Tawil syndrome electrocardiogram

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

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:

• A long QTc (LQT) interval
• T-U patterns which are very characteristic to the syndrome
• 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)
  • 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

“U on P” sign (black arrow). “U on P” sign (U-wave masquerading P-wave) ; the P-wave during sinus tachycardia is inscribed on the U-wave of the preceding beat. Case courtesy by Piotr Kukla et al

Post- extrasystolic “pseudo – LQTS pattern” (arrows). This is detected in a sinus beat following a PVC. The fusion of T+U-waves can mimic LQTS. This pattern is not observed in the subsequent sinus beats. Case courtesy by Piotr Kukla et al

Pseudo “Tee - Pee sign” (black arrow). Pseudo “Tee-pee sign” during a PVC, there is a prolongation of the descending limb of the T+U-wave. In ATS1, the QT interval can appeared prolonged and difficult to quantify because of a prominent U-wave. In order to properly determine the QT interval, the tangent technique should be carefully applied.Case courtesy by Piotr Kukla et al

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