Warning Symptoms and Family History in Children and Young Adults With Sudden Cardiac Arrest

Jonathan A. Drezner, MD; Jessie Fudge, MD; Kimberly G. Harmon, MD; Stuart Berger, MD; Robert M. Campbell, MD; Victoria L. Vetter, MD

Disclosures

J Am Board Fam Med. 2012;25(4):408-415. 

In This Article

Abstract and Introduction

Abstract

Background: Children and young adults with undiagnosed cardiovascular disorders at risk for sudden death may have warning symptoms or significant family history that is detectable through screening. The objective of this study was to determine the prevalence of warning symptoms and family history in a cohort of children and young adults who suffered sudden cardiac arrest (SCA).
Methods: A retrospective survey investigating warning symptoms and family history of cardiovascular disease was completed by families with a child or young adult who suffered SCA.
Results: Eighty-seven of 146 families (60%) returned a completed survey. The SCA victims were an average age of 16 years (range, <5–29 years), 69% male, and 68% white. Seventy-two percent of SCA victims were reported by their parents to have at least one cardiovascular symptom before SCA, with fatigue (44%) and near-syncope/lightheadedness (30%) the two most common. Twenty-four percent of SCA victims had one or more (average 2.6; range, 1 to 10) events of syncope or unexplained seizure that remained undiagnosed as a cardiac disorder before SCA. Parents reported that cardiovascular symptoms first occurred, on average, 30 months (range, 19 to 71 months) before SCA; a symptom was brought to the attention of the child's physician in 41% of cases. Twenty-seven percent of families reported a family member had suffered sudden death before age 50 because of a heart condition.
Conclusions: Many children and young adults who suffered SCA are reported to have cardiac symptoms or a family history of premature cardiac death. Syncope and unexplained seizure activity are distinct events but often go unrecognized as ominous signs of underlying cardiovascular disease. Physician education and increased public awareness regarding cardiovascular warning signs in the young may improve early detection of those at risk and prevent tragedies.

Introduction

Sudden cardiac arrest (SCA) is the leading cause of nontraumatic sudden death in children and young adults and usually is the result of an undiagnosed cardiovascular disorder.[1,2] The causes of pediatric and young adult SCA involve a heterogeneous group of cardiac diseases including structural cardiovascular abnormalities, such as cardiomyopathies, and primary cardiac electrical diseases, such as ion channel disorders. The prevalence of warning signs or symptoms in children and young adults who later suffer SCA is highly variable and poses a challenge to identifying through screening persons at elevated risk of sudden cardiac death (SCD).[3] Sudden death is the first clinical manifestation of underlying cardiovascular disease in up to 50% to 80% of young athletes with SCD.[4–7]

Not much is known about the potential to identify children and young adults at elevated risk of SCD through a comprehensive symptom and family history. During routine well-child evaluations, little attention may be given to a focused cardiovascular risk assessment. In young athletes, preparticipation cardiovascular screening is routinely recommended by most major sporting and medical associations including the American Heart Association (AHA).[8–11] However, universal endorsement of a single screening strategy to identify athletes at risk for SCD remains elusive and a topic of tremendous debate within the sports medicine and cardiology communities. In addition, recommendations for use of a comprehensive medical and family history questionnaire to guide the preparticipation evaluation have not been widely adopted in practice, and screening protocols often are implemented inadequately.[12–14] The management and subsequent evaluation of children with warning symptoms also may be incomplete, with the potential to overlook underlying cardiovascular pathology. The purpose of this study was to characterize the prevalence of warning symptoms and significant family history in children and young adults who suffered SCA.

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