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. 2003 Jan;89(1):66-70.
doi: 10.1136/heart.89.1.66.

Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death

N Sumitomo  1 , K Harada, M Nagashima, T Yasuda, Y Nakamura, Y Aragaki, A Saito, K Kurosaki, K Jouo, M Koujiro, S Konishi, S Matsuoka, T Oono, S Hayakawa, M Miura, H Ushinohama, T Shibata, I Niimura
Affiliations

Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death

N Sumitomo et al. Heart. 2003 Jan.

Abstract

Objective: To investigate the clinical outcome, ECG characteristics, and optimal treatment of catecholaminergic polymorphic ventricular tachycardia (CPVT), a malignant and rare ventricular tachycardia.

Patients and methods: Questionnaire responses and ECGs of 29 patients with CPVT were evaluated. Mean (SD) age of onset was 10.3 (6.1) years.

Results: The initial CPVT manifestations were syncope (79%), cardiac arrest (7%), and a family history (14%). ECGs showed sinus bradycardia and a normal QTc. Mean heart rate during CPVT was 192 (30) beats/min. Most cases were non-sustained (72%), but 21% were sustained and 7% were associated with ventricular fibrillation. The morphology of CPVT was polymorphic (62%), polymorphic and bidirectional (21%), bidirectional (10%), or polymorphic with ventricular fibrillation (7%). There was 100% inducement of CPVT by exercise, 75% by catecholamine infusion, and none by programmed stimulation. No late potential was recorded. Onset was in the right ventricular outflow tract in more than half the cases. During a follow up of 6.8 (4.9) years, sudden death occurred in 24% of the patients, 7% of whom had anoxic brain damage. Autosomal dominant inheritance was seen in 8% of the patients' families. beta Blockers completely controlled CPVT in only 31% of cases. Calcium antagonists partially suppressed CPVT in autosomal dominant cases.

Conclusions: CPVT may arise in certain distinct areas but the prognosis is poor. The onset of CPVT may be an indication for an implanted cardioverter-defibrillator.

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Figures

Figure 1
Figure 1
The onset of the foci and the following catecholaminergic polymorphic ventricular tachycardia (CPVT) beats in the study population. LVA, left ventricular apex; LVOT, left ventricular outflow tract; RVA, right ventricular apex; RVOT, right ventricular outflow tract.
Figure 2
Figure 2
Exercise induced ventricular fibrillation. This is an ECG of a 13 year old girl who had syncope while running. During a treadmill exercise test, bigemini involving right bundle branch block and rightward axis (empty circle) and left bundle branch block, inferior axis type, premature ventricular contraction (filled circles) were induced (left panel). Ventricular fibrillation was induced after left bundle branch block type premature ventricular contraction. Several of the premature beat morphologies appear somewhat different because of the fusion of the sinus beat.
Figure 3
Figure 3
Bidirectional ventricular tachycardia. This is the treadmill exercise ECG of a 12 year old boy who had syncope. Note the typical bidirectional ventricular tachycardia recorded in leads V5 and aVR; however, leads aVL and V1 showed polymorphic ventricular tachycardia. See text for further discussion.
Figure 4
Figure 4
Effect of verapamil in familial CPVT. The ECG shows maximal treadmill exercise tests before (top) and after intravenous injection of 10 mg of verapamil (bottom) in a 33 year old woman with familial CPVT. This patient received no drug treatment during the control state. Top: control state, 25 beats of CPVT were induced at 1 min 24 s after the start of the exercise. Bottom: after injection of verapamil, the endurance time was prolonged and five beats of CPVT were induced. Note the left bundle branch block and the inferior axis type premature ventricular conduction which is the trigger of this polymorphic ventricular contraction.
Figure 5
Figure 5
Cumulative survival rates. Most sudden deaths occurred within 10 years of CPVT diagnosis.
See this image and copyright information in PMC

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