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

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

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.

MeSH terms

  • Adolescent
  • Adrenergic beta-Antagonists / therapeutic use
  • Adult
  • Anti-Arrhythmia Agents / therapeutic use
  • Catecholamines / genetics*
  • Catheter Ablation / methods
  • Child
  • Child, Preschool
  • Death, Sudden, Cardiac / prevention & control*
  • Echocardiography
  • Electrocardiography
  • Female
  • Heart Rate
  • Humans
  • Male
  • Polymorphism, Genetic
  • Survival Rate
  • Tachycardia, Ventricular / mortality
  • Tachycardia, Ventricular / physiopathology*
  • Tachycardia, Ventricular / therapy*
  • Treatment Outcome
  • Verapamil / therapeutic use

Substances

  • Adrenergic beta-Antagonists
  • Anti-Arrhythmia Agents
  • Catecholamines
  • Verapamil