Original ArticlesClinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest
Section snippets
Methods
This was a multicenter, retrospective chart review of all newly diagnosed pediatric patients (aged ≤21 years) with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R presenting for their first pediatric cardiac evaluation between January 2008 and December 2014.
Data were collected from 6 different high-volume pediatric centers in the US with large primary and secondary care outpatient networks, including (1) Children's Healthcare of Atlanta, Atlanta, Georgia; (2) Cincinnati Children's Hospital
Results
A total of 450 patients (257 male, 193 female), median age 10.1 years (3.6-13.8 years, 25th-75th percentiles) were enrolled from the participating centers with a fairly even distribution: Children's Healthcare of Atlanta (96/21.3%), Cincinnati Children's Hospital Medical Center (86/19.1%), C.S. Mott Children's Hospital (71/15.8%), Texas Children's Hospital (70/15.6%), Primary Children's Hospital (66/14.7%), and Children's Hospital of Wisconsin (61/13.6%). The final diagnoses were LQTS
Discussion
This study from 6 large US centers describes the clinical presentation of 4 cardiac conditions potentially predisposing to pediatric sudden cardiac arrest, highlighting the opportunity to enhance diagnostics and identify patients affected by these disorders before sudden cardiac arrest. The median age for subjects in this study was 10.1 years (3.6-13.8 years, 25th-75th percentiles). More than 70% of this cohort presented at age ≤13 years, thus before the usual age for preparticipation physical
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Outcomes of Implantable Loop Monitoring in Patients <21 Years of Age
2021, American Journal of CardiologySymptoms and healthcare contact preceding sudden cardiac death in persons aged 1–49 years
2021, Trends in Cardiovascular MedicineCitation Excerpt :Discrepancies in the reported frequency of symptoms are attributable to a broad range of factors. Firstly, studies that report low frequencies of symptoms often do not have registration of symptoms prior to SCD as the main objective of their study [25,40], whereas, studies by Lynge et al. and Wisten and Messner had mapping of symptoms as their main objective [11,14]. Secondly, dissimilar populations may also explain differences in frequency ofsymptoms, as some studies were nationwide, while others were conducted among selected populations (e.g. soldiers or patients at cardiac centers) [11,14,22,24].
The “Great Debate” continues
2020, Heart RhythmECG screening in the United States: Are we there yet?
2020, Heart RhythmGenetic testing and cascade screening in pediatric long QT syndrome and hypertrophic cardiomyopathy
2020, Heart RhythmCitation Excerpt :For 20% of the cohort, mostly HCM patients, the status of genetic testing was unknown. An analysis of this index cohort diagnostic workup is reported elsewhere.11 The rate of positive genetic testing was consistent with expected rates for LQTS (81%) and HCM (60%) patients.12
2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society
2018, Journal of the American College of Cardiology
M.A. serves as a consultant for Boston Scientific, Gilead Sciences, Medtronic, and St. Jude Medical and receives royalties from Transgenomic for Familion-Long QT syndrome and Familion-Catecholaminergic polymorphic ventricular tachycardia genetic tests. The other authors declare no conflicts of interest.