Role of Exercise as a Modulating Factor in Arrhythmogenic Cardiomyopathy

Fig. 1. Representative case of left-dominant arrhythmogenic cardiomyopathy variant in a patient with a desmoplakin-gene mutation. The left-dominant arrhythmogenic cardiomyopathy variant may show an unremarkable electrocardiogram except for low-QRS voltages in the limb leads (a) and normal dimension and systolic function of both ventricles (end-diastolic frame of cine cardiac magnetic resonance sequence in long-axis 4-chamber view). b Post-contrast cardiac magnetic resonance images disclose myocardial fibrosis in the form of a late gadolinium enhancement stria in the epicardium of the left ventricular lateral wall (arrowheads) and midmural layer of the interventricular septum (arrows) (c) (reproduced with permission from: De Lazzari M et al. J Am Heart Assoc. 2018 Nov 20;7(22):e009855. doi: 10.1161/JAHA.118.009855) [27]

Fig. 2. Role of intense sports activity in the natural history of arrhythmogenic cardiomyopathy, from desmosomal-gene mutation to phenotypic expression and cardiac arrest due to ventricular fibrillation. Sports activity may promote development of phenotypic expression, accelerate disease progression, and trigger life-threatening ventricular arrhythmias (reproduced with permission from: Corrado D, Zorzi A. Eur Heart J 2015 Jul 14;36(27):1708-10. doi: 10.1093/eurheartj/ehv183. Epub 2015 May 12, with permission from Oxford University Press) [32]

Fig. 3. Representative example of the role of endurance exercise in worsening the arrhythmogenic cardiomyopathy phenotype. A 38-year-old runner received a diagnosis of arrhythmogenic cardiomyopathy after investigation of ventricular arrhythmias and ECG abnormalities at preparticipation screening. Genetic testing revealed the presence of a plakophilin-2 gene mutation. At the time of diagnosis, cardiac magnetic resonance 4-chamber view on cine sequences found dilatation of the right ventricle with mild dysfunction (a) and diffuse right ventricular late-enhancement on post-contrast sequences (arrowheads) (b). Although according to the Italian law he was considered not eligible for competitive sports activity, he continued to practice high-intensity endurance training. After 4 years, a new cardiac magnetic resonance showed a more enlarged right ventricle, an apical aneurysm (arrow), and a moderate right ventricular dysfunction (c). Post-contrast sequences confirmed the presence of a diffuse right ventricular late enhancement (arrowheads) (d)

Fig. 4. Potential unfavorable effects of sports activity in patients/athletes with an ICD. Sports in ICD carriers are potentially associated with an increased risk of: adrenergic-dependent ventricular tachyarrhythmias (a); athlete’s injury caused by loss of consciousness due to a syncopal tachyarrhythmia before the ICD intervention (b); development of phenotypic expression and accelerate disease progression of the underlying arrhythmogenic cardiomyopathy (c); inappropriate shocks caused by supraventricular tachycardia during sports activity (d, left) or by lead fracture with ventricular channel oversensing (d, right) (reproduced with permission from: Corrado D et al. Eur J Prev Cardiol. 2019 May;26(7):760-763. doi: 10.1177/2047487318805584. Epub 2018 Oct 18, with permission from Oxford University Press) [39]

Fig. 5. Differential diagnosis between arrhythmogenic cardiomyopathy and athlete’s heart. The differential diagnosis between athlete’s heart and arrhythmogenic right ventricular cardiomyopathy includes imaging features and comprehensive clinical approaches including ECG, ambulatory ECG monitoring, stress testing, and evaluation of family members. CMR, cardiac magnetic resonance; ECG, electrocardiography; LGE, late gadolinium enhancement; LV, left ventricular; RV, right ventricular; RVOT, right ventricular outflow tract; WMA, wall motion abnormality (reproduced with permission from: D’Ascenzi F et al. JACC Cardiovasc Imaging. 2018 Sep;11(9):1327-1339. doi: 10.1016/j.jcmg.2018.04.031, with permission from Elsevier) [53]