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An athletic 40-year-old man without a significant medical history was referred to the emergency department for episodes of palpitations. He had no cardiovascular risk factors and no family history of sudden death or cardiomyopathy. During the consultation, the patient was comfortable and afebrile, with a heart rate of 45 beats/min, respiratory rate of 12 beats/min, and blood pressure of 135/62 mm Hg. Physical examination revealed only a discrete pansystolic murmur at the left lower sternal border, which increased with inspiration. Routine blood test results were normal. His resting electrocardiogram (ECG) is shown in Figure 1A. He experienced recurrence of palpitations during several minutes while in the hospital, and a second ECG was recorded (Figure 1B). During this episode, the patient was hemodynamically stable, with no dyspnea or faintness. Transthoracic echocardiography revealed a normal left ventricle ejection fraction, no significant valvular heart disease, and apical dyskinesia of the right ventricle.
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Arrhythmogenic right ventricular dysplasia
A. Therapy with β-blocker and ICD implantation
The first ECG shows all the cardinal signs of arrhythmogenic right ventricular dysplasia (ARVD): incomplete right bundle branch block and inverted T waves in right precordial leads, QRS prolongation, and epsilon wave (Figure 2A).1 First-degree atrioventricular block and significant QT prolongation are also present.
Arrhythmogenic right ventricular dysplasia is a progressive cardiomyopathy characterized by replacement of cardiomyocytes by adipose and fibrous tissue.2 Mostly inherited in an autosomal dominant fashion, it involves genes encoding for desmosomal proteins, components of gap junctions. Involved in cell-cell communication, disruption in their structure leads to disordered electrical activity, forming the basis for reentrant ventricular arrhythmias. The disease preferentially affects the right ventricle (RV), in particular, the so-called triangle of dysplasia (inflow tract, outflow tract, and RV apex); however, left ventricular (LV) involvement has been recognized as fairly frequent.
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Corresponding Author: Eloi Marijon, MD, PhD, Cardiology Department, European Georges Pompidou Hospital, 20-40 Rue Leblanc, 75908 Paris Cedex 15, France (firstname.lastname@example.org).
Published Online: March 14, 2018. doi:10.1001/jamacardio.2017.4186
Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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