Sudden Death in the Young: Information for the Primary Care Provider.

There are multiple conditions that can make children prone to having a sudden cardiac arrest (SCA) or sudden cardiac death (SCD). Efforts have been made by multiple organizations to screen children for cardiac conditions, but the emphasis has been on screening before athletic competition. This article is an update of the previous American Academy of Pediatrics policy statement of 2012 that addresses prevention of SCA and SCD. This update includes a comprehensive review of conditions that should prompt more attention and cardiology evaluation. The role of the primary care provider is of paramount importance in the evaluation of children, particularly as they enter middle school or junior high. There is discussion about whether screening should find any cardiac condition or just those that are associated with SCA and SCD. This update reviews the 4 main screening questions that are recommended, not just for athletes, but for all children. There is also discussion about how to handle post-SCA and SCD situations as well as discussion about genetic testing. It is the goal of this policy statement update to provide the primary care provider more assistance in how to screen for life-threatening conditions, regardless of athletic status.

Performance of the American Heart Association ( AHA ) 14-Point Evaluation Versus Electrocardiography for the Cardiovascular Screening of High School Athletes: A Prospective Study.

Background Preparticipation cardiovascular screening in athletes is fully endorsed by major medical societies, yet the most effective screening protocol remains debated. We prospectively compared the performance of the American Heart Association ( AHA ) 14-point screening evaluation and a resting ECG for cardiovascular screening of high school athletes. Methods and Results Competitive athletes participating in organized high school or premier/select level sports underwent cardiovascular screening using the AHA 14-point history and physical examination, and an ECG interpreted with the Seattle Criteria. A limited echocardiogram was performed for all screening abnormalities. The primary outcome measure was identification of a cardiovascular disorder associated with sudden cardiac death. From October 2014 to June 2017, 3620 high school athletes (median age, 16 years; range 13-19; 46.2% female; 78.6% white, 8.0% black) were screened. One or more positive responses to the AHA 14-point questionnaire were present in 814 (22.5%) athletes. The most common history responses included chest pain (8.1%), family history of inheritable conditions (7.3%), and shortness of breath (6.4%). Abnormal physical examination was present in 356 (9.8%) athletes, and 103 (2.8%) athletes had an abnormal ECG . Sixteen (0.4%) athletes had conditions associated with sudden cardiac death. The sensitivity (18.8%), specificity (68.0%), and positive predictive value (0.3%) of the AHA 14-point evaluation was substantially lower than the sensitivity (87.5%), specificity (97.5%), and positive predictive value (13.6%) of ECG . Conclusions The AHA 14-point evaluation performs poorly compared with ECG for cardiovascular screening of high school athletes. The use of consensus-derived history questionnaires as the primary tool for cardiovascular screening in athletes should be reevaluated.

International criteria for electrocardiographic interpretation in athletes: Consensus statement.

Sudden cardiac death (SCD) is the leading cause of mortality in athletes during sport. A variety of mostly hereditary, structural or electrical cardiac disorders are associated with SCD in young athletes, the majority of which can be identified or suggested by abnormalities on a resting 12-lead electrocardiogram (ECG). Whether used for diagnostic or screening purposes, physicians responsible for the cardiovascular care of athletes should be knowledgeable and competent in ECG interpretation in athletes. However, in most countries a shortage of physician expertise limits wider application of the ECG in the care of the athlete. A critical need exists for physician education in modern ECG interpretation that distinguishes normal physiological adaptations in athletes from distinctly abnormal findings suggestive of underlying pathology. Since the original 2010 European Society of Cardiology recommendations for ECG interpretation in athletes, ECG standards have evolved quickly, advanced by a growing body of scientific data and investigations that both examine proposed criteria sets and establish new evidence to guide refinements. On 26-27 February 2015, an international group of experts in sports cardiology, inherited cardiac disease, and sports medicine convened in Seattle, Washington (USA), to update contemporary standards for ECG interpretation in athletes. The objective of the meeting was to define and revise ECG interpretation standards based on new and emerging research and to develop a clear guide to the proper evaluation of ECG abnormalities in athletes. This statement represents an international consensus for ECG interpretation in athletes and provides expert opinion-based recommendations linking specific ECG abnormalities and the secondary evaluation for conditions associated with SCD.

Incidence and Etiology of Sudden Cardiac Arrest and Death in High School Athletes in the United States.

OBJECTIVE: To determine the incidence and etiology of sudden cardiac arrest and death (SCA/D) in US high school athletes. PATIENTS AND METHODS: A prospective media database of SCA/D was queried for cases aged 14 to 18 years from 7 states over 6 school years (September 1, 2007, to August 30, 2013). Event details were investigated to determine participation on a high school athletic team, sex, sport, and occurrence during school-sponsored activity or exertion. National sports participation numbers were used and a conversion factor was applied to account for multisport athletes. Autopsy reports were reviewed and cause of death was adjudicated by an expert panel. RESULTS: A total of 16,390,409 million athlete-seasons representing 6,974,640 athlete-years (AY) were examined, encompassing 36% of the total US high school athlete population. A total of 104 cases of SCA/D were identified (35 SCA with survival and 69 sudden cardiac deaths [SCDs]). The rate of SCD was 1:101,082 AY and of SCA/D 1:67,064 AY. Eighty-eight percent (92) of events occurred in male athletes. The rate of SCA/D in male athletes was 1:44,832 AY and in female athletes 1:237,510 AY (incidence rate ratio, 5.3; 95% CI, 2.9-10.6; P<.001). Men's basketball was the highest risk sport with an SCA/D incidence of 1:37,087 AY followed by men's football at 1:86,494 AY. Men's basketball and football athletes accounted for 57% (39) of deaths. Eighty percent of SCDs (55 of 69) were exertional and 55% (38 of 69) occurred while playing for a school-sponsored team. Autopsy reports were obtained in 73% (50) of cases. The most common findings of autopsy were idiopathic left ventricular hypertrophy or possible cardiomyopathy (13 of 50 [26%]), autopsy-negative sudden unexplained death (9 of 50 [18%]), hypertrophic cardiomyopathy (7 of 50 [14%]), and myocarditis (7 of 50 [14%]). CONCLUSION: The rate of SCA/D in male high school athletes was 1:44,832 AY, with almost half due to possible or confirmed cardiomyopathy disease. It is likely that many cases were not identified because of reliance on media reports, and these numbers represent a minimum estimate.

Electrocardiographic Screening in National Collegiate Athletic Association Athletes.

The most effective protocol for cardiovascular screening of competitive athletes remains highly controversial. This study was a prospective, multicenter trial of cardiovascular screening at 35 National Collegiate Athletic Association institutions. Screening included a standardized history and physical examination (PE) as recommended by the American Heart Association and a 12-lead electrocardiogram (ECG) at rest. Centralized electrocardiographic interpretation was provided using the Seattle criteria. Athletes with screening abnormalities underwent additional evaluation directed by the host institution medical team. Primary outcomes included the proportion of total and false-positive screens; the sensitivity, specificity, and positive predictive value of history, PE, and ECG; and the prevalence of serious cardiovascular disorders associated with severe morbidity or sudden cardiac death. From August 2012 to June 2014, 5,258 athletes from 17 intercollegiate sports were screened: 55% men (mean age 20.1 years), 73% Caucasian, 16% African-American, and 11% other/mixed race. At least 1 positive cardiac symptom or family history response was reported by 1,750 athletes (33.3%). PE was abnormal in 108 athletes (2.1%), and electrocardiographic abnormalities were present in 192 athletes (3.7%). Thirteen athletes (0.25%) were identified with serious cardiac conditions including hypertrophic cardiomyopathy (1), large atrial septal defect with right ventricular dilation (1), and ventricular pre-excitation (11). The false-positive rate for history was 33.3%, PE 2.0%, and ECG 3.4%. The sensitivity/specificity/positive predictive value for history was 15.4%/66.9%/0.1%, PE 7.7%/98.2%/0.9%, and ECG 100%/96.6%/6.8%. In conclusion, electrocardiographic screening in National Collegiate Athletic Association athletes has a low false-positive rate and provides superior accuracy compared with a standardized history and PE to detect athletes with potentially dangerous cardiovascular conditions.

Catecholaminergic polymorphic ventricular tachycardia in children: analysis of therapeutic strategies and outcomes from an international multicenter registry.

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation are not well validated. We sought to define treatment outcomes in children with catecholaminergic polymorphic ventricular tachycardia. METHODS AND RESULTS: This is a Pediatric and Congenital Electrophysiology Society multicenter, retrospective cohort study of catecholaminergic polymorphic ventricular tachycardia patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (interquartile range, 6.8-13.2) years with a delay to diagnosis of 0.5 (0-2.6) years. Syncope (P<0.001), cardiac arrest (P<0.001), and treatment failure (P=0.008) occurred more often in probands. ß-Blockers were prescribed in 205 of 211 patients (97%) on medication, and 25% experienced at least 1 treatment failure event. Implantable cardioverter defibrillators were placed in 121 (54%) and was associated with electrical storm in 22 (18%). Flecainide was used in 24% and left cardiac sympathetic denervation in 8%. Six deaths (3%) occurred during a cumulative follow-up of 788 patient-years. CONCLUSIONS: This study demonstrates a malignant phenotype and lengthy delay to diagnosis in catecholaminergic polymorphic ventricular tachycardia. Probands were typically severely affected. ß-Blockers were almost universally initiated; however, treatment failure, noncompliance and subtherapeutic dosing were often reported. Implantable cardioverter defibrillators were common despite numerous device-related complications. Treatment failure was rare in the quarter of patients on flecainide. Left cardiac sympathetic denervation was not uncommon although the indication was variable.

The Psychological Impact of Cardiovascular Screening in Young Athletes: Perspectives Across Age, Race, and Gender.

OBJECTIVE: To determine the psychological impact of electrocardiogram (ECG) screening in young competitive athletes based on age, race, and gender. DESIGN: Prospective multisite cross-sectional investigation. SETTING: Young competitive athletes. PATIENTS: One thousand five hundred six high school athletes (59.3% male with a mean age of 15.5 years, 56.2% white, 24.5% African American). INDEPENDENT VARIABLES: Athletes underwent screening with a standardized American Heart Association-based history, physical examination, and ECG. MAIN OUTCOME MEASURES: Prescreen and postscreen validated assessments for health attitudes, anxiety, and impact of screening on sport. RESULTS: Of the athletes participated, 76.3% had normal screens, 22.7% false-positive (FP) results (12.9% history, 4.2% examination, 3.1% ECG), and 0.9% were diagnosed with serious cardiac disorders. There were no differences in anxiety levels among athletes screening normal versus FP (P = 0.69). There was no difference in anxiety based on the reason for a FP result (history, physical examination, or ECG) both during (P = 0.95) and after (P = 0.40) screening. Analysis by age, race, and gender demonstrated that before screening, females were more likely to prefer knowing about underlying cardiac conditions compared with males (P < 0.001), and males were less concerned about having cardiac disease (P < 0.001) compared with females. African Americans were less concerned about underlying disease (P < 0.001) and less interested (P < 0.001) in cardiac screening compared with whites. Athletes diagnosed with a cardiac disease described anxiety after screening, but still believe that all athletes should receive an ECG before competition. CONCLUSIONS: Electrocardiogram screening does not cause excessive anxiety in US high school athletes across spectrums of age, race, and gender. Recognition of age, race, and gender-specific perspectives could improve physician-patient dialogue and support mechanisms for those diagnosed with potentially lethal cardiac disorders. CLINICAL RELEVANCE: This article provides evidence that undue anxiety should not be used as an argument against the implementation of ECG screening during the preparticipation examinaton for young athletes. Although males and African Americans seem to be at highest risk for sudden cardiac death during exercise, they actually report being the least concerned and least worried about potentially harboring an underlying condition that might predispose them to such an event. Emotional support should readily be available for athletes who are diagnosed with cardiovascular conditions as these individuals report emotional distress after their diagnosis.

Evaluation and management of wolff-Parkinson-white in athletes.

CONTEXT: Wolff-Parkinson-White (WPW) is a cardiac conduction system disorder characterized by abnormal accessory conduction pathways between the atria and the ventricles. Symptomatic patients classically present with palpitations, presyncope, or syncope that results from supraventricular tachycardia. While rare, sudden cardiac death may be the first manifestation of underlying disease and occurs more frequently in exercising individuals. EVIDENCE ACQUISITION: Medline and PubMed databases were evaluated through 2012, with the following keywords: WPW, Wolff-Parkinson-White, pre-excitation, sudden cardiac death, risk stratification, and athletes. Selected articles identified through the primary search, along with relevant references from those articles, were reviewed for pertinent clinical information regarding the identification, evaluation, risk stratification, and management of WPW as they pertained to the care of athletes. STUDY DESIGN: Systematic review. LEVEL OF EVIDENCE: Level 1. RESULTS: Diagnosis of WPW is confirmed by characteristic electrocardiogram changes, which include a delta wave, short PR interval, and widened QRS complex. Utilization of the electrocardiogram as part of the preparticipation physical evaluation may allow for early identification of asymptomatic individuals with a WPW pattern. Risk stratification techniques identify individuals at risk for malignant arrhythmias who may be candidates for curative therapy through transcatheter ablation. CONCLUSION: WPW accounts for at least 1% of sudden death in athletes and has a prevalence of at least 1 to 4.5 per 1000 children and adults. The risk of lethal arrhythmia appears to be higher in asymptomatic children than in adults, and sudden cardiac death is often the sentinel event. The athlete with WPW should be evaluated for symptoms and the presence of intermittent or persistent pre-excitation, which dictates further consultation, treatment, and monitoring strategies as well as return to play.