Archives of disease in childhood · 2025
Catecholaminergic polymorphic ventricular tachycardia in children-incidence and trends in detection, presentation and management.
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Fitzgerald Nicholas, Lawley Claire, Morrish Ansley, Tarca Adrian, Marcondes Luciana, Asakai Hiroko, Turner Christian, Skinner Jonathan
Аннотация
OBJECTIVE: To establish a birth rate for catecholaminergic polymorphic ventricular tachycardia (CPVT) diagnosed in childhood and observe trends in presentation and management.
DESIGN: Retrospective cohort study.
SETTING: The Inherited Arrhythmia Clinic at The Sydney Children's Hospitals Network, a paediatric tertiary referral network, New South Wales (NSW), Australia (2002-2021), where there are 86 000-97 000 live births/year.
PATIENTS: Children diagnosed with CPVT aged 0-16 years.
INTERVENTIONS: Clinical data were extracted and evaluated for trends. Using birth year data, the birth rate of CPVT detected in childhood was calculated.
MAIN OUTCOME MEASURES: Birth rate of CPVT detected in childhood in NSW (with post hoc comparison to New Zealand), trends in diagnosis and management, and outcome at last follow-up.
RESULTS: 32 children in NSW were diagnosed with CPVT between 2002 and 2021 (0-16 years, median 9 years, 14 (54%) female). Of these, 28 (88%) presented with symptoms (cardiac arrest 20/32, 62.5%) and four (12%) were identified through family screening. Relevant genetic variants were identified in 25/31 (78%). During follow-up (median 4.5 years), symptomatic cardiac events (death n=1) occurred in 10 (33%), largely related to suboptimal adherence or monotherapy beta blocker. In NSW, CPVT was diagnosed during childhood following 1 in 65 000 live births (95% CI 1 in 91 000 to 1 in 46 000). In New Zealand, the corresponding figure was 1 in 84 000 live births (95% CI 1 in 138 000 to 1 in 52 000).
CONCLUSIONS: The rate of infants born who are later diagnosed with CPVT in childhood is approximately 1 in 65 000 live births. Suboptimal adherence and beta blocker therapy without flecainide appeared related to recurrent cardiac events.
DESIGN: Retrospective cohort study.
SETTING: The Inherited Arrhythmia Clinic at The Sydney Children's Hospitals Network, a paediatric tertiary referral network, New South Wales (NSW), Australia (2002-2021), where there are 86 000-97 000 live births/year.
PATIENTS: Children diagnosed with CPVT aged 0-16 years.
INTERVENTIONS: Clinical data were extracted and evaluated for trends. Using birth year data, the birth rate of CPVT detected in childhood was calculated.
MAIN OUTCOME MEASURES: Birth rate of CPVT detected in childhood in NSW (with post hoc comparison to New Zealand), trends in diagnosis and management, and outcome at last follow-up.
RESULTS: 32 children in NSW were diagnosed with CPVT between 2002 and 2021 (0-16 years, median 9 years, 14 (54%) female). Of these, 28 (88%) presented with symptoms (cardiac arrest 20/32, 62.5%) and four (12%) were identified through family screening. Relevant genetic variants were identified in 25/31 (78%). During follow-up (median 4.5 years), symptomatic cardiac events (death n=1) occurred in 10 (33%), largely related to suboptimal adherence or monotherapy beta blocker. In NSW, CPVT was diagnosed during childhood following 1 in 65 000 live births (95% CI 1 in 91 000 to 1 in 46 000). In New Zealand, the corresponding figure was 1 in 84 000 live births (95% CI 1 in 138 000 to 1 in 52 000).
CONCLUSIONS: The rate of infants born who are later diagnosed with CPVT in childhood is approximately 1 in 65 000 live births. Suboptimal adherence and beta blocker therapy without flecainide appeared related to recurrent cardiac events.