A 17-year-old multi-sport high school athlete and captain of the lacrosse team collapses on the soccer field during active play. The trainer immediately goes to him, identifies him as unresponsive and pulseless, then promptly begins cardiopulmonary resuscitation (CPR) and quickly applies an automated external defibrillator (AED). The tracing (Figure 1) documents ventricular fibrillation. He is shocked three times and has return of circulation. He is admitted to the hospital intubated, undergoes a cooling protocol, and has a full recovery. The hospital electrocardiogram (ECG) and magnetic resonance imaging (MRI) are seen in (Figure 2, Figure 3). The electrophysiologist reports to the family that the diagnosis is commotio cordis, and he is discharged on no medication and without implantable cardioverter-defibrillator (ICD). Two weeks later he undergoes an evaluation with the family requesting cardiac clearance for return to play. His ECG and echocardiogram from the outpatient visit are seen in (Figure 4, Figure 5).
Which of the following is the next step in the management for this patient?
Show Answer
The correct answer is: F. The hospital ECG shows an epsilon wave in V1 and the right ventricle (RV) appears somewhat generous on MRI. The office ECG documents flipped T-waves in V1-V3. One should consider a diagnosis of ARVC/D and restrict from sports; consider ICD placement. Suggest genetic testing for ARVC/D (as part of a comprehensive arrhythmia panel). Start screening of first-degree relatives.
Commotio cordis is a possibility; unfortunately, in order to make the diagnosis, underlying heart disease cannot be present.1,2 This answer is not the best choice.
If the child has commotio cordis, return to play is a possibility; guidelines leave this decision to the discretion of the clinician.1-3 Establishing the diagnosis is very relevant, the presence of an underlying genetically-based condition would warrant evaluation of first-degree relatives. The treatment of chanellopathies, myopathies, Brugada syndrome, commotio cordis, and right ventricular outflow tract tachycardia are very different, and the medical care should be stylized to the underlying diagnosis. This answer is not the best choice.
Currently, playing contact competitive sports with an ICD is not recommended.3 In regard to genetic testing, the cost of testing still remains high, and insurance companies are reluctant to perform even one panel. Consequently, genetic testing should be performed in a stepwise fashion with the panel containing as many of the most relevant diagnoses from your differential. In this case, the panel chosen is a comprehensive arrhythmia panel.
The hospital ECG is post-arrest, and it is common to see prolonged QTc from the cooling, electrolyte abnormalities, or from autonomic changes related to the arrest. One can also see prolongation to QTc in cardiomyopathies.4 The office ECG has a normal QTc (Figure 5). A stress test looking for abnormal QTc at baseline, during exercise, and special attention to the QTc during recovery could support the diagnosis of LQTS. This patient has a stress test that shows a good level of conditioning, a normal QTc at rest and peak exercise, with a prolonged four-minute recovery QTc of 480ms. Such values can be seen in normal patients as well as individuals with long QTc, and "healthy" elite athletes.4-7 He does have ventricular ectopy throughout his stress test and recovery (Figure 6). The observed QTc behavior during stress does not make or rule out the diagnosis of LQTS. For this athlete, enlarged RV on MRI, an epsilon wave in V1, along with T-wave inversion in V1-V3 are more suggestive of ARVC, making answer F the better choice.
In this setting, CPVT is a distinct possibility and a stress test and genetic testing for CPVT could be helpful in securing the diagnosis. For this athlete, an enlarged RV on MRI, an epsilon wave in V1, and flipped T-waves inV1-V3 are more suggestive of ARVC, making answer F the better choice.
ARVC/D is the best choice. The diagnosis is made with the two major criteria for ARVC/D (inverted T-waves in V1-V3 and an epsilon wave in V1). An additional major criterion is met when he is identified as gene positive for ARVC (DSG2 mutation). Screening of first-degree relatives is recommended, and he is restricted from sports in keeping with the current guidelines.2
In summary, the additional findings that are supportive of ARVC/D include cardiac arrest, frequent premature ventricular complex on resting EKG, ventricular ectopy throughout the stress test, blunted blood pressure response to exercise, low ECG voltage and dilated RV.7 One should also consider the potential risk of accelerating disease progression by allowing aggressive endurance training for ARVC/D patients, or even gene positive patients.8-11 Finally, for the outcome see Figure 7.
References
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Maron BJ, Poliac LC, Kaplan JA, Mueller FO. Blunt impact to the chest leading to sudden death from cardiac arrest during sports activities. N Engl J Med 1995;333:337–42.
Maron BJ, Zipes DP. 36th Bethesda Conference: eligibility recommendations for competitive athletes with cardiovascular abnormalities. J Am Coll Cardiol 45 2005:2-64.
Martin AB, Garson A, Perry JC. Prolonged QT interval in hypertrophic and dilated cardiomyopathy in children. Am Heart J. 1994;127:64-70.
Sy RW, van der Werf C, Chattha IS, et al. Derivation and validation of a simple exercise-based algorithm for prediction of genetic testing in relatives of LQTS probands. Circulation 2011;124:2187-94.
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Zaidi A, Sheikh N, Jongman JK, et al. Clinical Differentiation Between Physiological Remodeling and Arrhythmogenic Right Ventricular Cardiomyopathy in Athletes With Marked
Electrocardiographic Repolarization Anomalies. J Am Coll Cardiol 2015;65:2702-11.
La Gerche A, Burns AT, Mooney DJ, et al. Exercise-induced right ventricular dysfunction and structural remodeling in endurance athletes. Eur Heart J 2012; 33,998–1006.
Sharma S, Zaidi A. Exercise-induced arrhythmogenic right ventricular cardiomyopathy: fact or fallacy? Eur Heart J 2012;33,938-40.
Kirchhof P, Fabritz L, Zweiner M, et al. Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice. Circulation 2006;114:1799-1806.
Fabritz L, Hoogendijk MG, Scicluna BP, et al. Load-reducing therapy prevents development of arrhythmogenic right ventricular cardiomyopathy in plakoglobin-deficient mice. J Am Coll Cardiol 2011;57:740-50.
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