RT Journal Article
SR Electronic
T1 Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP 66
OP 70
DO 10.1136/heart.89.1.66
VO 89
IS 1
A1 Sumitomo, N
A1 Harada, K
A1 Nagashima, M
A1 Yasuda, T
A1 Nakamura, Y
A1 Aragaki, Y
A1 Saito, A
A1 Kurosaki, K
A1 Jouo, K
A1 Koujiro, M
A1 Konishi, S
A1 Matsuoka, S
A1 Oono, T
A1 Hayakawa, S
A1 Miura, M
A1 Ushinohama, H
A1 Shibata, T
A1 Niimura, I
YR 2003
UL http://heart.bmj.com/content/89/1/66.abstract
AB 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.