Elsevier

The Journal of Pediatrics

Volume 177, October 2016, Pages 191-196
The Journal of Pediatrics

Original Articles
Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest

https://doi.org/10.1016/j.jpeds.2016.06.088Get rights and content

Objectives

To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R).

Study design

This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014.

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. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT).

Conclusion

Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to 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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    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.

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