Case report of a patient with congenital long QT syndrome Type 2 presenting with electrical storm: do not judge a book by its cover!

Figures

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  Figure 1. Extract from the rhythm monitoring of the 47-year-old patient immediately after admission to the emergency department. The rhythm strip shows polymorphous ventricular tachycardias similar to Torsades de Pointes tachycardias. The first two episodes were self-limiting. The third episode converted into ventricular fibrillation and was successfully terminated by the implantable cardioverter-defibrillator (arrow). In the following minutes, the patient developed electric storm and received overall six ICD shocks.

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  Figure 2. Initial 12-channel-electrocardiogram revealed atrially triggered ventricular pacing by the dual-chamber implantable cardioverter-defibrillator (arrow tip). Despite ventricular pacing distinct ST segment deviation concordant with QRS polarity was seen in the inferior Leads II, III, aVF (arrows).

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  Figure 3. Sections from the coronary angiography (anterior–posterior projection with cranial angulation in (A), left anterior oblique projection in (A–D). (A) Angiography of the left coronary artery revealed a short-segment low-grade stenosis (20%, Type A lesion) in the middle third of the left anterior descending artery and an ostial short-segment high-grade stenosis (90%, Type B lesion) of a diagonal branch (white arrow). (B) 10 mm long subtotal occlusion (99%, Type A lesion) of the middle right coronary artery. The white arrowhead points the pace-sense lead fixed in the right-ventricular outflow tract (Membrane E, Pacesetter Systems Inc., Sylmar, CA, USA), the black arrowhead the dual-coil lead in the right-ventricular apex (Endotak C 0125, Cardiac Pacemakers Inc., St. Paul, MN, USA) and the black arrow the atrial lead (Type 4068, Medtronic, Minneapolis, MN, USA). (C) The right coronary artery was treated by pre-dilatation of the lesion and drug-eluting stent implantation (3.5 × 18 mm; white arrow). (D) The culprit lesion was reopened successfully (white arrow).

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  Figure 4. Twelve-channel-electrocardiogram registered immediately after revascularization. Spontaneous atrioventricular conduction resumed and ventricular pacing disappeared. Q-waves and T-wave-negations were observed in the inferior Leads II, III, and aVF and also in V6. The QT interval was substantially prolonged. QTc was calculated 581–628 ms using the Bazett formula.