IJCS | Volume 31, Nº6, November / December 2018

DOI: 10.5935/2359-4802.20180063 667 CASE REPORT International Journal of Cardiovascular Sciences. 2018;31(6)667-671 Mailing Address: João B. Augusto Hospital Professor Doutor Fernando Fonseca, IC19. Postal Code: 2720-276, Lisboa - Portugal E-mail: joao.augusto@hff.min-saude.pt Biventricular Arrhythmogenic Cardiomyopathy: A New Paradigm? João Augusto, 1 João Abecasis, 2 Victor Gil 2 Service of Cardiology, Hospital Professor Doutor Fernando Fonseca, 1 Amadora - Portugal Cardiovascular Unit, Hospital dos Lusíadas, 2 Lisbon - Portugal Manuscript received on January 8, 2018; revised manuscript on January 16, 2018; accepted on March 27, 2018. Cardiomyopathies; Arrhythmogenic Right Ventricular Dysplasia; Arrhythmias, Cardiac; Magnetic Resonance Imaging; Death, Sudden, Cardiac. Keywords Abstract Arrhythmogenic right ventricular dysplasia is a classic form of chronic myocardial disease with a broad phenotypical spectrum. We report an atypical case of a patient with biventricular arrhythmogenic cardiomyopathy. Although the current diagnosis criteria are the most widely accepted ones, they focus solely on the right ventricular phenotype. The use of late gadolinium enhancement in cardiac magnetic resonance in this patient was essential for the diagnosis and assessment of the left ventricular involvement extent. This tool allows a broader use of current diagnosis criteria for this disease. Introduction Arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD) consists of the classic form of a chronic, progressive and hereditarymyocardial disease that has a broad phenotypic spectrum. 1 The use of the broader term “arrhythmogenic cardiomyopathy” (AC) is now accepted, which also encompasses the variants involving either mainly the left ventricle (LV) or the LV and the right ventricle (RV) - the latter, usually understood as a later form of the disease. 1 The incidence of this disease is 1:2,000 to 1:5,000, and the mean age at diagnosis is approximately 30 years old, constituting an important cause of sudden cardiac death. 2,3 We report on a patient with ARVD and concomitant LV involvement. Case report A 57-year-old male patient with irregular follow-up at a cardiology clinic for 15 years due to complaints of palpitations at exertion was assessed. He only had a history of systemic arterial hypertension, medicated and controlled with bisoprolol and lisinopril, with no other significant personal history or family history. The most recent electrocardiographic exams showed sinus rhythm, left anterior hemiblock pattern, premature ventricular contractions with complete left bundle branch block (LBBB) and superior axis, as well as periods of non- sustained ventricular tachycardia. The previous complementary examinations included a 24-hour Holter monitoring with a non-sustained ventricular tachycardia (VT) episode with LBBB configuration, with very frequent polymorphic premature ventricular contractions (136/hr). The patient had been submitted to a transthoracic echocardiogram performed 3 years earlier, showing slight impairment of LV systolic function (ejection fraction of 47% according to the biplane Simpson’s method) and diffuse hypokinesia; dilated RV also with slight systolic function impairment and apparent asymmetries in the segmental contractility of the inferior wall. The patient was referred for cardiacmagnetic resonance imaging (MRI) (Figure 1), which showed a slightly dilated LV (end-diastolic index volume of 107 mL/m 2 ), with ejection fraction of 44%; and a slightly dilated RV (end- diastolic index volume of 109 mL/m 2 ) with an ejection fraction of 39%, with evident dyskinetic areas on the free and diaphragmatic walls (in systole and diastole). There were also extensive areas of late enhancement (fibrosis) on theRV freewall (particularly at the thirdbasal level), aswell as on theLV interventricular septumandanterolateralwall, with a mid-myocardial distribution. No areas of adipocyte infiltrationwere observed in the fat suppression sequences.