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Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death
  1. N Sumitomo1,
  2. K Harada1,
  3. M Nagashima2,
  4. T Yasuda2,
  5. Y Nakamura3,
  6. Y Aragaki4,
  7. A Saito5,
  8. K Kurosaki5,
  9. K Jouo6,
  10. M Koujiro7,
  11. S Konishi8,
  12. S Matsuoka9,
  13. T Oono10,
  14. S Hayakawa11,
  15. M Miura12,
  16. H Ushinohama13,
  17. T Shibata14,
  18. I Niimura14
  1. 1Department of Paediatrics, Nihon University School of Medicine, Tokyo, Japan
  2. 2Department of Paediatrics, Nagoya University, Japan
  3. 3Department of Paediatric Cardiology, Kinki University, Japan
  4. 4Department of Paediatrics, National Cardiovascular Centre, Tokyo, Japan
  5. 5Department of Cardiology, Shizuoka Children’s Hospital, Japan
  6. 6Department of Paediatrics, Kyushu Koseinenkin Hospital, Japan
  7. 7Department of Paediatrics, University of Occupational and Environmental Health, Japan
  8. 8Department of Paediatrics, Hiroshima University School of Medicine, Japan
  9. 9Department of Paediatrics, School of Medicine, University of Tokushima, Japan
  10. 10Department of Paediatrics, Kyushu University, Japan
  11. 11Department of Paediatrics, Seirei Mikatahara General Hospital, Japan
  12. 12Department of Cardiology, Metropolitan Kiyose Children’s Hospital, Japan
  13. 13Department of Cardiology, Fukuoka Children’s Hospital, Japan
  14. 14Department of Paediatrics, Yokohama City University, School of Medicine, Japan
  1. Correspondence to:
    Dr Naokata Sumitomo, Department of Paediatrics, Nihon University School of Medicine, 30-1 Oyaguchi Kamimachi, Itabashi, Tokyo 173-8610, Japan;


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. β 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.

  • ventricular tachycardia
  • calcium channel blocker
  • ventricular fibrillation
  • sudden death

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