CARDIOVASCULAR MEDICINE

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

N Sumitomo¹, K Harada¹, M Nagashima², T Yasuda², Y Nakamura³, Y Aragaki⁴, A Saito⁵, K Kurosaki⁵, K Jouo⁶, M Koujiro⁷, S Konishi⁸, S Matsuoka⁹, T Oono¹⁰, S Hayakawa¹¹, M Miura¹², H Ushinohama¹³, T Shibata¹⁴, I Niimura¹⁴

¹ Department of Paediatrics, Nihon University School of Medicine, Tokyo, Japan
² Department of Paediatrics, Nagoya University, Japan
³ Department of Paediatric Cardiology, Kinki University, Japan
⁴ Department of Paediatrics, National Cardiovascular Centre, Tokyo, Japan
⁵ Department of Cardiology, Shizuoka Children’s Hospital, Japan
⁶ Department of Paediatrics, Kyushu Koseinenkin Hospital, Japan
⁷ Department of Paediatrics, University of Occupational and Environmental Health, Japan
⁸ Department of Paediatrics, Hiroshima University School of Medicine, Japan
⁹ Department of Paediatrics, School of Medicine, University of Tokushima, Japan
¹⁰ Department of Paediatrics, Kyushu University, Japan
¹¹ Department of Paediatrics, Seirei Mikatahara General Hospital, Japan
¹² Department of Cardiology, Metropolitan Kiyose Children’s Hospital, Japan
¹³ Department of Cardiology, Fukuoka Children’s Hospital, Japan
¹⁴ Department of Paediatrics, Yokohama City University, School of Medicine, Japan

Correspondence to:
Correspondence to:
Dr Naokata Sumitomo, Department of Paediatrics, Nihon University School of Medicine, 30-1 Oyaguchi Kamimachi, Itabashi, Tokyo 173-8610, Japan;
jt9n-smtm{at}asahi-net.or.jp

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. ß 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.

Keywords: ventricular tachycardia; calcium channel blocker; ventricular fibrillation; sudden death

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