Deciding Return to Play in the Grey Area of Guidelines

Authors: Neal M. Dixit MD1, Ali Nsair MD1,2, Jeffrey J. Hsu MD, PhD1,2 1Department of Medicine, 2Division of Cardiology David Geffen School of Medicine at UCLA Los Angeles, California 90095

ACM is an intrinsic disorder of the myocardium which classically involves the right ventricle (known as arrhythmogenic right ventricular cardiomyopathy, or ARVC) (5). More recently ALVC and mixed variants have been identified. Most cases involve genetic defects in desmosomes, ion channels and related proteins in cardiac myocytes. The diagnosis is typically made with ECG and non-invasive imaging (2). T-wave inversions are often seen in the distribution of the pathological tissue. The classic imaging findings include fibro-fatty infiltration of the ventricle, ventricular dysfunction and mid-myocardial late gadolinium enhancement on cardiac MRI (6). Multiple gene mutations are associated with ACM but approximately 37% of cases do not have an identifiable mutation (7). The incidence of sudden cardiac death (SCD) in ACM is unclear and most recommendations are based on the better studied RV variant. ARVC accounts for nearly 20% of SCD cases (2) and half of patients with ICDs for primary prevention of SCD can expect a lifesaving shock in the first 5 years (8). 

Establishing the diagnosis of ACM can be challenging. The modified Task Force Criteria published in 2010 incorporates clinical (i.e., ventricular arrhythmias, family history), electrocardiographic, imaging and histological assessments for the diagnosis of ARVC (9). These features are categorized into major and minor criteria, helping to identify a definite, borderline, or possible diagnosis of ACM. The 2020 Padua criteria provides additional clinical features to aid in diagnosis of the ALVC (4).

The 2019 Heart Rhythm Society Expert Consensus statement (2) provides detailed guidance on the management of ACM. Beta blockers, rhythm control agents, and heart failure medications are used in appropriate situations. The presence of certain risk factors and disease characteristics can necessitate ICD placement. The Society also recommends that patients with ARVC do not participate in high intensity exercise (>6 METs) due to the risk of SCD. Intense exercise may also worsen the underlying cardiomyopathy.

The decision to preclude an athlete from competitive sports is often difficult, and a shared-decision making approach is a critical element of management in these cases (10). In cases of understudied pathology without clear guidelines, clinicians should look to expert recommendations for similar disease processes. Given our patient’s presentation with precordial T-wave inversions on ECG, documented NSVT episodes on ambulatory ECG monitoring, and abnormal cardiac MRI findings concerning for ALVC, he met criteria for at least a borderline diagnosis for ACM (8). However, without abnormal RV findings or genetic mutations associated with ACM he did not satisfy the Padua criteria for diagnosis of ALVC. The AHA/ACC Scientific Statement published 2015 recommends that athletes with a possible, borderline, or definite diagnosis of ARVC not participate in competitive sports (Class III, Level of Evidence: C) (3). After discussion of the uncertain diagnosis of ACM but likely risk of sudden death and ventricular arrhythmia based on his phenotypic features, our patient opted to stop playing competitive sports and continue moderate intensity physical activity with close follow-up.  

References

  1.   Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm. 2011;8(8):1308-1339. doi:10.1016/j.hrthm.2011.05.020
  2.   Towbin JA, McKenna WJ, Abrams DJ, et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm. 2019;16(11):e301-e372. doi:10.1016/j.hrthm.2019.05.007
  3.   Maron BJ, Udelson JE, Bonow RO, et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 3: Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Other Cardiomyopathies, and Myocarditis: A Scientific Statement From the American Heart Association and American College of Cardiology. Circulation. 2015;132(22):e273-e280. doi:10.1161/CIR.000000000000023
  4. Corrado D, Perazzolo Marra M, Zorzi A, et al. Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria. Int J Cardiol. 2020;319:106-114. doi:10.1016/j.ijcard.2020.06.005
  5.   Sen-Chowdhry S, Morgan RD, Chambers JC, McKenna WJ. Arrhythmogenic cardiomyopathy: etiology, diagnosis, and treatment. Annu Rev Med. 2010;61:233-253. doi:10.1146/annurev.med.052208.130419
  6.   Tandri H, Calkins H. MR and CT imaging of Arrhythmogenic Cardiomyopathy. Card Electrophysiol Clin. 2011;3(2):269-280. doi:10.1016/j.ccep.2011.02.002
  7.   Edgar T. Hoorntje, Wouter P. te Rijdt, Cynthia A. James, Kalliopi Pilichou, Cristina Basso, Daniel P. Judge, Connie R. Bezzina, J. Peter van Tintelen, Arrhythmogenic cardiomyopathy: pathology, genetics, and concepts in pathogenesis, Cardiovascular Research, Volume 113, Issue 12, October 2017, Pages 1521–1531, https://doi.org/10.1093/cvr/cvx150
  8.   Bhonsale A, James CA, Tichnell C, et al. Incidence and predictors of implantable cardioverter-defibrillator therapy in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy undergoing implantable cardioverter-defibrillator implantation for primary prevention. J Am Coll Cardiol. 2011;58(14):1485-1496. doi:10.1016/j.jacc.2011.06.043
  9.   Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121(13):1533-1541. doi:10.1161/CIRCULATIONAHA.108.840827
  10. Baggish AL, Ackerman MJ, Putukian M, Lampert R. Shared Decision Making for Athletes with Cardiovascular Disease: Practical Considerations. Curr Sports Med Rep. 2019;18(3):76-81. doi:10.1249/JSR.0000000000000575