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Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study

journal contribution
posted on 2023-08-08, 05:56 authored by Gabrielle Norrish, Henry Chubb, Ella Field, Karen McLeod, Maria Ilina, Georgia Spentzou, Jan Till, Piers EF Daubeney, Alan Graham Stuart, Jane MatthewsJane Matthews, Dominic Hares, Elspeth Brown, Katie Linter, Vinay Bhole, Krishnakumar Pillai, Michael Bowes, Caroline B Jones, Orhan Uzun, Amos Wong, Arthur Yue, Shankar Sadagopan, Tara Bharucha, Norah Yap, Eric Rosenthal, Sujeev Mathur, Satish Adwani, Zdenka Reinhardt, Jasveer Mangat, Juan Pablo Kaski
Abstract Aims Sudden cardiac death (SCD) is the most common mode of death in paediatric hypertrophic cardiomyopathy (HCM). This study describes the implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion in a well-characterized national paediatric HCM cohort. Methods and results Data from 90 patients undergoing ICD insertion at a median age 13 (±3.5) for primary (n = 67, 74%) or secondary prevention (n = 23, 26%) were collected from a retrospective, longitudinal multi-centre cohort of children (<16 years) with HCM from the UK. Seventy-six (84%) had an endovascular system [14 (18%) dual coil], 3 (3%) epicardial, and 11 (12%) subcutaneous system. Defibrillation threshold (DFT) testing was performed at implant in 68 (76%). Inadequate DFT in four led to implant adjustment in three patients. Over a median follow-up of 54 months (interquartile range 28–111), 25 (28%) patients had 53 appropriate therapies [ICD shock n = 45, anti-tachycardia pacing (ATP) n = 8], incidence rate 4.7 per 100 patient years (95% CI 2.9–7.6). Eight inappropriate therapies occurred in 7 (8%) patients (ICD shock n = 4, ATP n = 4), incidence rate 1.1/100 patient years (95% CI 0.4–2.5). Three patients (3%) died following arrhythmic events, despite a functioning device. Other device complications were seen in 28 patients (31%), including lead-related complications (n = 15) and infection (n = 10). No clinical, device, or programming characteristics predicted time to inappropriate therapy or lead complication. Conclusion In a large national cohort of paediatric HCM patients with an ICD, device and programming strategies varied widely. No particular strategy was associated with inappropriate therapies, missed/delayed therapies, or lead complications.

History

Journal

EUROPACE

Volume

23

Pagination

400-408

Location

England

ISSN

1099-5129

eISSN

1532-2092

Language

English

Publication classification

C1.1 Refereed article in a scholarly journal

Issue

3

Publisher

OXFORD UNIV PRESS