Rapid Fire Abstracts
Lars Grosse-Wortmann, MD
Professor of Pediatrics
Oregon Health & Science University
Lars Grosse-Wortmann, MD
Professor of Pediatrics
Oregon Health & Science University
Paul Kantor, MD
pediatric cardiologist
Children's Hospital of Los Angeles
Reza Nezafat, PhD
Professor
Harvard Medical School
Seshadri Balaji, MD, PhD
pediatric cardiologist
Oregon Health and Science University
Hypertrophic cardiomyopathy (HCM) is a leading cause of sudden cardiac death (SCD) in children and adolescents. Prevention of SCD in pediatric HCM is challenging as risk prediction is imprecise, despite several algorithms and calculators that have been developed to assist. Late gadolinium enhancement (LGE) imaging by cardiac magnetic resonance (CMR) has been proposed to aid in risk prediction, but is currently included in only one of these risk calculators and in none of the two tools designed for use in children and adolescents. The objective of this study was to obtain an overview of how SCD risk is assessed in pediatric HCM patients and how CMR is utilized in implantable cardioverter-defibrillator (ICD) decision making.
Methods:
A survey was mailed in July 2024 to pediatric electrophysiologists, cardiomyopathy experts, and other cardiologists who were the primary decision makers regarding placement of ICDs at their institutions.
Results: Responses were received from 113 physicians (out of 365, response rate of 31%). Of them, 66% were electrophysiologists and 21% were cardiomyopathy specialists. Respondents were based in the USA (55%), Canada (8%), Europe (21%), and other countries (15%) and were predominantly (88%) practicing at academic medical centers. Most cardiologists (97%) use one of more of the four currently available online risk calculators (AHA/ACC, ESC, HCM-RiskKids, and PRIMaCY). However, the average ‘confidence level in ability to reliably predict SCD’ was only 6.6 out of 10 and 22% recalled at least one patient who was deemed to be at low risk who later had a SCD event. 96% of respondents obtained a CMR as part of their patients’ risk stratification and the presence or absence of LGE was considered either “very important” or “critically important” for 73% of respondents. 31% routinely obtained quantitative LGE and 48% semiquantitative LGE. 30% of respondents stated that they would strongly consider an ICD in a patient with severe LGE ( >20% of total myocardium) even if the risk calculators indicated a low SCD risk. Conversely, most respondents (88%) would proceed with an ICD in the absence of LGE if the risk calculator yielded a significant SCD risk ( >6% over 5 years).
Conclusion:
Experts in clinical HCM care rate their ability to assess SCD risk in pediatric patients as ‘average’ (6.6 out of 10 confidence points), despite widespread adoption of risk stratification techniques. A substantial number recalled children and adolescents who experienced SCD despite a reassuring risk assessment, highlighting a need for better risk stratification. Nearly all expert respondents include LGE in their prognostic work-up, although less than one third use quantitative LGE. One third of respondents would consider an ICD on the basis of severe LGE alone, but almost nobody would ignore other concerning findings in the presence of a CMR with negative LGE. In synopsis, this survey indicates that clinicians have adopted LGE into their ICD decision-making as part of a comprehensive work-up for their pediatric HCM patients and that there is a need to incorporate it into multi-modality risk-assessment tools.
