Page 86 - JSOM Summer 2024

FIGURE 3 General approach to arrhythmogenic right ventricular cardiomyopathy.

Presentation Evaluation Diagnosis Treatment

• Identify symptoms • Ambulatory cardiac • Lifestyle changes
(exertional syncope, monitoring (PVCs, (exercise limitations)
dyspnea, palpitations) evidence of VT) • Apply Padua criteria • Pharmacologic
• 12-lead ECG (epsilon • TTE (RV dilation or • Cardiology referral (AA, BB)
wave, T wave inverions dysfunction) • Genetics referral
in V1-3, QRS terminal • Non-pharmacologic
activation) • Cardiac CT or MRI (ICD)
ECG = electrocardiogram; RV = right ventricular; EMB = endomyocardial biopsy; VT = ventricular tachycardia; RBBB = right bundle branch
block; PVC = premature ventricular contraction; RVOT = right ventricular outflow tract; TTE = transthoracic echocardiography; SCD = sudden
cardiac death; AA = antiarrhythmic; BB = beta-blocker; ICD = implantable cardioverter-defibrillator.

TABLE 1 Major and Minor Criteria for Diagnosing Arrhythmogenic with cardiology consultation in a population at increased risk
Right Ventricular Cardiomyopathy (ARVC) Per the 2020 Padua of early disease progression and arrhythmogenic complica-
Criteria 10 tions owing to the level of fitness in special operations.
>1 Major or Minor functional (I) AND/OR Structural (II) RV
criteria? References
Yes = ARVC 1. Krahn AD, Wilde AAM, Calkins H, et al. Arrhythmogenic right ven-
Definite ARVC = 2 major; or 1 major + 2 minor; or 4 minor tricular cardiomyopathy. JACC Clin Electrophysiol. 2022;8(4):533–
553. doi:10.1016/j.jacep.2021.12.002
Borderline ARVC = 1 major + 2 minor; or 3 minor 2. Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults:
Possible ARVC = 2 minor An autopsy-based series of a population undergoing active surveil-
lance. J Am Coll Cardiol. 2011;58(12):1254–1261. doi:10.1016/j.
jacc.2011.01.049
undergo a Medical Evaluation Board process and subsequent 3. Smallman DP, Webber BJ, Mazuchowski EL, Scher AI, Jones SO,
medical retirement. However, a 2020 study published in Mili- Cantrell JA. Sudden cardiac death associated with physical exertion
13
tary Medicine identified genotype-positive/phenotype- negative in the US military, 2005-2010. Br J Sports Med. 2016;50(2):118–123.
doi:10.1136/bjsports-2015-094900
ARVC patients may not necessarily be precluded from military 4. Jones SO, Smallman DP, Webber BJ, et al. Activities performed during
service and may maintain the relatively high level of physical sudden cardiac death associated with physical exertion in the United
7
fitness required for military retention. Though some Service- States military. J Am Coll Cardiol. 2015;65(10_Supplement):A369.
doi:10.1016/s0735-1097(15)60369-2
members with ARVC will be medically retired, personalized 5. Brady WJ, Glass III GF. Cardiac rhythm disturbances. In: Tintinalli JE,
risk and benefit discussions with these Servicemembers should Ma OJ, Yealy DM, et al. eds. Tintinalli’s Emergency Medicine: A Com-
nonetheless be undertaken. prehensive Study Guide, 9th Edition. McGraw Hill; 2020.
6. Kaddoura R, Al-Tamimi H. Physical Exercise and Arrhythmogenic Right
Ventricular Cardiomyopathy/Dysplasia: An Overview. Heart Views.
This case raises the importance of ARVC and the consider- 2022;23(4):215–220. doi:10.4103/heartviews.heartviews_70_22
ation of this diagnosis in healthy Special Operations Service- 7. Segre EM, Hellwig LD, Turner C, Dobson CP, Haigney MC. Exercise
members presenting with exertional palpitations or syncope. dose associated with military service: implications for the clinical man-
With a disease process that is exacerbated by intense exercise agement of inherited risk for arrhythmogenic right ventricular car-
and tendency to provoke life-threatening arrhythmias, main- diomyopathy. Mil Med. 2020;185(9–10):e1447–e1452. doi:10.1093/
milmed/usaa185
taining a high clinical suspicion is key to identifying this rare 8. Latt H, Tun Aung T, Roongsritong C, Smith D. A classic case of ar-
but nefarious etiology. If identified early, ARVC can be effec- rhythmogenic right ventricular cardiomyopathy (ARVC) and liter-
tively managed with activity modification, medications, and ature review. J Community Hosp Intern Med Perspect. 2017;7(2):
115–121. doi:10.1080/20009666.2017.1302703
placement of an ICD. 9. Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular car-
diomyopathy: Diagnosis, prognosis, and treatment. Heart. 2000;83
(5):588-595. doi:10.1136/heart.83.5.588
Conclusion 10. Graziano F, Zorzi A, Cipriani A, et al. The 2020 “Padua Criteria”
for diagnosis and phenotype characterization of arrhythmogenic car-
ARVC is a rare, hereditary cardiac condition characterized diomyopathy in clinical practice. J Clin Med. 2022;11(1):279. doi:
by the replacement of normal right ventricular myocardium 10.3390/jcm11010279
with fibrofatty tissue, leading to arrhythmias and an increased 11. McNally E, MacLeod H, Dellefave-Castillo L. Arrhythmogenic right
risk of SCD. While patients commonly present with exertional ventricular cardiomyopathy overview In: Adam MP, Feldman J, Mir-
®
palpitations, dizziness, or syncope, history-taking should also zaa GM, et al., eds. GeneReviews . Seattle (WA): University of Wash-
ington, Seattle; April 18, 2005.
focus on provoking factors as well as family history of ARVC 12. Jurlander R, Mills HL, Espersen KI, et al. Screening relatives in ar-
or SCD, prompting a thorough examination and ECG. Any rhythmogenic right ventricular cardiomyopathy: Yield of imaging and
suspicion for ARVC should trigger further diagnostic workup electrical investigations. Eur Heart J Cardiovasc Imaging. 2020;21(2):
175–182. doi:10.1093/ehjci/jez204
with ambulatory cardiac monitoring and a TTE. The 2020 13. Army Regulation 40-501: Standards of Medical Fitness. Department
Padua criteria should be used to aid in diagnosis, though a car- of the Army; 2019.
diac MRI and cardiologist referral is ultimately required. Once 14. de Jong JSSG. Arrhythmogenic Right Ventricular Cardiomyopa-
identified, abstaining from strenuous activity in conjunction thy. ECGpedia.org. Updated May 5, 2013. Accessed May 27, 2024.
with the use of class II or III antiarrhythmics can help to man- https://en.ecgpedia.org/index.php?title=Arrhythmogenic_Right_
Ventricular_Cardiomyopathy.
age ARVC; ICD placement should also be considered to pre-
vent SCD. Ultimately, this case emphasizes timely recognition, PMID: 38830309; DOI: 10.55460/FFIY-8JLW
accurate diagnosis, and tailored management of the disease

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