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A 40-Year-Old Man With Palpitations

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

Corresponding Author: Eloi Marijon, MD, PhD, Cardiology Department, European Georges Pompidou Hospital, 20-40 Rue Leblanc, 75908 Paris Cedex 15, France (eloi_marijon@yahoo.fr).

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.

References
1.
Marcus  FI, McKenna  WJ, Sherrill  D,  et al.  Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria.  Eur Heart J. 2010;31(7):806-814.PubMedGoogle ScholarCrossref
2.
Marcus  FI, Fontaine  GH, Guiraudon  G,  et al.  Right ventricular dysplasia: a report of 24 adult cases.  Circulation. 1982;65(2):384-398.PubMedGoogle ScholarCrossref
3.
Protonotarios  A, Anastasakis  A, Tsatsopoulou  A,  et al.  Clinical significance of epsilon waves in arrhythmogenic cardiomyopathy.  J Cardiovasc Electrophysiol. 2015;26(11):1204-1210.PubMedGoogle ScholarCrossref
4.
Priori  SG, Blomström-Lundqvist  C, Mazzanti  A,  et al.  2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC).  Eur Heart J. 2015;36(41):2793-2867.PubMedGoogle ScholarCrossref
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Corrado  D, Wichter  T, Link  MS,  et al.  Treatment of arrhythmogenic right ventricular cardiomyopathy/dysplasia: an international task force consensus statement.  Circulation. 2015;132(5):441-453.PubMedGoogle ScholarCrossref
6.
Corrado  D, Leoni  L, Link  MS,  et al.  Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia.  Circulation. 2003;108(25):3084-3091.PubMedGoogle ScholarCrossref
7.
Herman  ARM, Gardner  M, Steinberg  C,  et al.  Long-term right ventricular implantable cardioverter-defibrillator lead performance in arrhythmogenic right ventricular cardiomyopathy.  Heart Rhythm. 2016;13(10):1964-1970.PubMedGoogle ScholarCrossref
8.
Migliore  F, Zorzi  A, Silvano  M,  et al.  Prognostic value of endocardial voltage mapping in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia.  Circ Arrhythm Electrophysiol. 2013;6(1):167-176.PubMedGoogle ScholarCrossref
9.
Dalal  D, Jain  R, Tandri  H,  et al.  Long-term efficacy of catheter ablation of ventricular tachycardia in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.  J Am Coll Cardiol. 2007;50(5):432-440.PubMedGoogle ScholarCrossref
10.
Mast  TP, James  CA, Calkins  H,  et al.  Evaluation of structural progression in arrhythmogenic right ventricular dysplasia/cardiomyopathy.  JAMA Cardiol. 2017;2(3):293-302.PubMedGoogle ScholarCrossref
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