Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest

Aarti Dalal, Richard J. Czosek, Joshua Kovach, Johannes C. von Alvensleben, Santiago Valdes, Susan P. Etheridge, Michael John Ackerman, Debbie Auld, Jeryl Huckaby, Courtney McCracken, Robert Campbell

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)191-196
Number of pages6
JournalJournal of Pediatrics
Volume177
DOIs
StatePublished - 2016

Fingerprint

Sudden Cardiac Death
Pediatrics
Long QT Syndrome
Hypertrophic Cardiomyopathy
Syncope
Exercise
Sinus of Valsalva
Inborn Genetic Diseases
Incidence
Chest Pain
Signs and Symptoms
Physical Examination
Coronary Vessels
Polymorphic catecholergic ventricular tachycardia

Keywords

  • catecholaminergic polymorphic ventricular tachycardia
  • congenital coronary artery anomalies
  • family history
  • hypertrophic cardiomyopathy
  • long QT syndrome
  • patient history
  • sudden cardiac arrest

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Dalal, A., Czosek, R. J., Kovach, J., von Alvensleben, J. C., Valdes, S., Etheridge, S. P., ... Campbell, R. (2016). Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest. Journal of Pediatrics, 177, 191-196. https://doi.org/10.1016/j.jpeds.2016.06.088

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest. / Dalal, Aarti; Czosek, Richard J.; Kovach, Joshua; von Alvensleben, Johannes C.; Valdes, Santiago; Etheridge, Susan P.; Ackerman, Michael John; Auld, Debbie; Huckaby, Jeryl; McCracken, Courtney; Campbell, Robert.

In: Journal of Pediatrics, Vol. 177, 2016, p. 191-196.

Research output: Contribution to journalArticle

Dalal, A, Czosek, RJ, Kovach, J, von Alvensleben, JC, Valdes, S, Etheridge, SP, Ackerman, MJ, Auld, D, Huckaby, J, McCracken, C & Campbell, R 2016, 'Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest', Journal of Pediatrics, vol. 177, pp. 191-196. https://doi.org/10.1016/j.jpeds.2016.06.088
Dalal A, Czosek RJ, Kovach J, von Alvensleben JC, Valdes S, Etheridge SP et al. Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest. Journal of Pediatrics. 2016;177:191-196. https://doi.org/10.1016/j.jpeds.2016.06.088
Dalal, Aarti ; Czosek, Richard J. ; Kovach, Joshua ; von Alvensleben, Johannes C. ; Valdes, Santiago ; Etheridge, Susan P. ; Ackerman, Michael John ; Auld, Debbie ; Huckaby, Jeryl ; McCracken, Courtney ; Campbell, Robert. / Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest. In: Journal of Pediatrics. 2016 ; Vol. 177. pp. 191-196.
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abstract = "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.",
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AU - Valdes, Santiago

AU - Etheridge, Susan P.

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N2 - 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.

AB - 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.

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KW - congenital coronary artery anomalies

KW - family history

KW - hypertrophic cardiomyopathy

KW - long QT syndrome

KW - patient history

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