Arrhythmogenic right ventricular cardiomyopathy or dysplasia: a case report
DOI:
https://doi.org/10.18203/2349-3933.ijam20232212Keywords:
Arrhythmogenic, Right ventricle, Cardiomyopathy, DysplasiaAbstract
Arrhythmogenic cardiomyopathy (ACM) is a progressive genetic disease of the myocardium characterized by loss of myocardial cells and replacement by fibrofatty tissue in the right and/or left ventricle (RV/LV), clinically manifested by syncope, palpitations, heart failure, or sudden cardiac death (SCD). We presented a case where the patient suffered from palpitations for the previous three to four years, which resolved spontaneously each time and lately presented with persistent palpitations and chest discomfort. ECG showed a widening of the QRS complexes during sinus rhythm and premature complexes (PVCs) of various patterns. After initial evaluation cardiac MRI was done which revealed typical features of arrhythmogenic right ventricular dysplasia/ cardiomyopathy. The patient was continuing amiodarone 100 mg daily and doing well.
References
Thiene G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med. 1988;318:129-33.
Marcus FI, Fontaine GH, Guiraudon G. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65:384-98.
Corrado D, Basso C. Arrhythmogenic left ventricular cardiomyopathy. Heart. 2022;108(9):733-43.
Paul M, Schulze-Bahr E, Günther B, Wichter T. Genetics of arrhythmogenic right ventricular cardiomyopathy-status quo and future perspectives. Clin Res Cardiol. 2003;92(2):128-36.
Syrris P, Ward D, Evans A, Asimaki A, Gandjbakhch E, Sen-Chowdhry S, McKenna WJ. Arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in the desmosomal gene desmocollin-2. Am J Human Genetics. 2006;79(5):978-84.
Tandri H, Calkins H, Nasir K, Bomma C, Castillo E, Rutberg J, Tichnell C, Lima JA, Bluemke DA. Magnetic resonance imaging findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia. J Cardiovascular Electrophysiol. 2003;14(5):476-82.
Te Riele AS, James CA, Philips BI, Rastegar NE, Bhonsale A, Groeneweg JA et al. Mutation‐positive arrhythmogenic right ventricular dysplasia/cardiomyopathy: the triangle of dysplasia displaced. J Cardiovascular Electrophysiol. 2013;24(12):1311-20.
Murphy DT, Shine SC, Cradock A, Galvin JM, Keelan ET, Murray JG. Cardiac MRI in arrhythmogenic right ventricular cardiomyopathy. Am J Roentgenol. 2010;194(4):W299-306.
Corrado D, Basso C, Pilichou K, Thiene G. Molecular biology and clinical management of arrhythmogenic right ventricular cardiomyopathy/dysplasia. Heart. 2011;97(7):530-9.
Ermakov S, Scheinman M. Arrhythmogenic right ventricular cardiomyopathy–antiarrhythmic therapy. Arrhythmia Electrophysiol Rev. 2015;4(2):86.
Bhonsale A, James CA, Tichnell C, Murray B, Gagarin D, Philips B 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 College Cardiol. 2011;58(14):1485-96.
Wlodarska EK, Wozniak O, Konka M, Rydlewska-Sadowska W, Biederman A, Hoffman P. Thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Europace. 2006;8(8):596-600.
Basso C, Corrado D, Marcus FI, Nava A, Thiene G. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009;373(9671):1289-300.
Fontaine G, Fontaliran F, Hebert JL, Chemla D, Zenati O, Lecarpentier Y, Frank R. Arrhythmogenic right ventricular dysplasia. Annual Rev Med. 1999;50(1):17-35.
Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart. 2000;83(5):588-95.
Fontaine G, Fontaliran F, Frank R. Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses. Circulation. 1998;97(16):1532-5.
Sen-Chowdhry S, Lowe MD, Sporton SC, McKenna WJ. Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management. Am J Med. 2004;117(9):685-95.
Steriotis AK, Bauce B, Daliento L, Rigato I, Mazzotti E, Folino AF, Marra MP, Brugnaro L, Nava A. Electrocardiographic pattern in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol. 2009;103(9):1302-8.
Peters S, Trümmel M. Diagnosis of arrhythmogenic right ventricular dysplasia‐cardiomyopathy: value of standard ECG revisited. Ann Noninvasive Electrocardiol. 2003;8(3):238-45.
Peters S, Trümmel M, Koehler B. QRS fragmentation in standard ECG as a diagnostic marker of arrhythmogenic right ventricular dysplasia-cardiomyopathy. Heart Rhythm. 2008;5(10):1417-21.
Nasir K, Bomma C, Tandri H, Roguin A, Dalal D, Prakasa K et al. Electrocardiographic features of arrhythmogenic right ventricular dysplasia/cardiomyopathy according to disease severity: a need to broaden diagnostic criteria. Circulation. 2004;110(12):1527-34.
Kiès P, Bootsma M, Bax J, Schalij MJ, Van der Wall EE. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: screening, diagnosis, and treatment. Heart Rhythm. 2006;3(2):225-34.
Nava A, Bauce B, Basso C, Muriago M, Rampazzo A, Villanova C et al. Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. J Am College Cardiol. 2000;36(7):2226-33.
Marcus FI, Fontaine G. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: a review. Pacing Clin Electrophysiol. 1995;18(6):1298-314.
Gasperetti A, MD, Carrick R.T., MD, Costa S, MD. Paolo Compagnucci P et al programmed ventricular stimulation as an additional primary prevention risk stratification tool in Arrhythmogenic Right Ventricular Cardiomyopathy. A Multinational Study Circulation. 2022;146:1434-43.
Corrado DMD, Link MS, Calkins H. Arrhythmogenic Right Ventricular Cardiomyopathy. N Engl J Med. 2017;367:61-72.