Sunday, December 17, 2017

Syncope in a young female

Syncope in a young female

18 y/o female presented with syncope. Parents also state that she has seizure like moments when the phone rings. The following EKG was obtained. What is the reason for syncope and "seizures"?

From the post author

Thank you for the discussion. This patient has a congenital long QT syndrome. She has R-on-T phenomenon that induces VT. Her "seizures" are VT episodes that are induced by high level of epinephrine from a startle response. She is undergoing genetic testing and will get a secondary prevention ICD[implantable cardioverter defibrillator].

A contributor

Short PR interval with delta wave and brief runs of anti-dromic tachycardia through an inferior and somewhat lateral accessory bypass tract. She needs an EP study. I find the telephone ringing precipitation of arrhythmia fascinating. It must be disturbing her sympathetic-parasympathetic balance and initiating antidromic tachycardia. The "seizures" are global cerebral ischemia from tachycardia.

Another contributor

Based on ECG is is definitely long QT syndrome, non corrected QT 600 ms plus. High risk for sudden cardiac death. Syncope with audible stimulation more typical for LQT 2. Assuming she is not on any QT prolonging meds, recommend ICD , genetical testing for LQT, screening family members with 12 lead ECG. Consider aldactone therapy if not hypotensive Her seizures are due to TdP [torsade de pointes].

Another contributor

This ECG shows an ectopic atrial rhythm, a long QT interval, and characteristic R-on-T ventricular ectopy, including a 4-beat run of polymorphic VT. The QT is extraordinarily long at > 700 msec. Assuming there is no exogenous reason for her long QT (a drug, for example), this is characteristic of congenital Long QT syndrome. The startle trigger (telephone) is most commonly a manifestation of Long QT2. Torsade de pointes VT in this syndrome can cause syncope so abrupt that the syncope appears convulsive. A great example of why a young person who is believed to have a seizure disorder needs an ECG.

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