HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
58-year-old male, ex-smoker, ex-drinker and overweight, with a history of ischaemic stroke of unidentified aetiology in 2015 in the middle cerebral artery with motor aphasia as the main sequelae. The echocardiogram performed on admission showed slight dilatation of the right ventricle with hypertrabeculation and left ventricular ejection fraction at the lower limit of normality as the only relevant findings. The patient was awaiting outpatient cardiac magnetic resonance imaging to complete the study.

Usual treatment: ASA 100 mg, atorvastatin 40 mg and ranitidine 300 mg every 24 hours. The patient presented at home with sudden dyspnoea and general malaise, so he called the Emergency Services who performed an electrocardiogram that showed regular wide QRS tachycardia at 205 beats per minute with an image of left bundle branch block and right superior axis. Given that the patient presented acceptable clinical tolerance, iv amiodarone was started and subsequently, given the lack of response, electrical cardioversion was performed and the patient was transferred to our centre in sinus rhythm, maintaining haemodynamic stability and with symptomatic improvement. At our centre, he was admitted to the Coronary Unit, where amiodarone perfusion and low-dose bisoprolol treatment were started, despite which he presented another episode of tachycardia similar to the one described, which required electrical cardioversion. He did not report chest pain or syncope or any other symptoms in the anamnesis.
Physical examination showed blood pressure 95/60 mmHg, 80 beats/minute, BMI 28 kg/m2, baseline oxygen saturation 93 %, and he was afebrile. There was no evidence of jugular ingurgitation. Cardiopulmonary auscultation showed no heart murmurs and preserved vesicular murmur with no added sounds.
Abdomen without masses or megaliths and lower limbs without oedema.
Chest X-ray, blood tests showed no anaemia, normal renal function and ions, and mild elevation of cardiac biomarkers. Transthoracic echocardiogram showed mild left ventricular and severe right ventricular dilatation, severely depressed LVEF and severe right ventricular systolic dysfunction.

COMPLEMENTARY TESTS
Electrocardiogram 1: regular wide QRS tachycardia at 205 bpm with atrioventricular dissociation, left bundle branch block image morphology and right superior axis.
Electrocardiogram 2: sinus bradycardia at 55 bpm, PR 220 ms (first degree BAV), small voltage r wave up to V6 and positive deflection at the end of QRS in V1 compatible with epsilon wave, negative T waves from V1 to V5 and flattened in V6 and frontal leads.
Portable chest X-ray: cardiomegaly and vascular redistribution, without pleural effusion.
CBC: leukocytes 7500 / mm3, haemoglobin 14.5 g/dl, MCV 85 mm3 , MCH 33 / cell, platelets 225,000 / mm3, normal coagulation, creatinine 1.05 mg/dl, urea 30 mg/dl, glomerular filtration rate 74 ml/min/1.73 m2, Na 138 mEq/L, K 4.6 mEq/L, CPK 210 IU/L, troponin I 0.7 ng/ml (peak).
Transthoracic echocardiogram: poor acoustic window in apical window.
Left ventricle slightly dilated (DDVI 59 mm). Severely depressed LVEF (25% by Teicholz) with decreased global contractility. Prolonged diastolic relaxation pattern and mean E/e" of 11. Normofunctioning mitral valve and aortic valve. Slightly dilated left atrium. Severely dilated right chambers. Parasternal long axis RVOT diameter 52 mm, parasternal short axis 52 mm. RV free wall hypertrabeculation and depressed systolic function with TAPSE of 12.9 mm. No adequate recording of tricuspid registry for PSAP estimation. Absence of pericardial effusion, inferior vena cava 21 mm with inspiratory collapse less than 50%.
Coronary angiography: angiographically normal coronary arteries.
Cardio MRI: RA 67 x 55 mm (36 cm2), RV 100 x 70 x 60 mm, LA: 66 x 38 mm, LV: 58 x 58 x 66 mm, IVS thickness 11 mm, RVOT 55 mm. Left ventricular function study: LVEF 44 %, Beat volume 36.5 ml/m2, cardiac output 4.8 l/m, TVD 83.8 ml/ m2, STV 47.3 ml/m2. Right ventricular function study: LVEF 15 %, stroke volume 24.6 ml/m2, cardiac output 3.3 l/min, VTD 165.5 ml/m2, VTS 141.9 ml/m2. Great dilatation of the right ventricle with areas of anteroinferior hypokinesia of 2 cm close to the valvular plane. At the level of the left ventricle there is a thinned, irregular lateral face, with probable fatty infiltration, presenting a small aneurysmal dilatation of 15 mm in the middle inferolateral segment. Middle inferolateral akinesia and apicolateral dyskinesia. Lateral fat replacement. After contrast iv enhancement is seen throughout the subepicardial lateral wall of the thinned LV and inferolateral segment in addition to anterior, inferior basal and right ventricular outflow tract areas.

EVOLUTION
The patient presented two more episodes of ventricular tachycardia under treatment with amiodarone requiring electrical cardioversion. He was switched to treatment with sotalol, achieving electrical and clinical stability. Based on clinical, electrocardiographic and cardiac imaging findings, a diagnosis of arrhythmogenic cardiomyopathy of the right and left ventricle was established. A bicameral bibovine ICD was implanted as secondary prevention with an electrode terminal placed in the middle septum without complications. Subsequent evolution was favourable, with no new episodes or defibrillator shocks. Treatment was started with angiotensin-converting enzyme inhibitor together with low-dose bisoprolol and sotalol and, given the previous cerebrovascular event, it was decided, in conjunction with neurology, to start treatment with apixaban due to the likelihood of cardioembolic aetiology. There were no known cases of this condition in the family, so genetic testing was requested (results pending).

DIAGNOSIS
Sustained ventricular tachycardia in a patient with arrhythmogenic cardiomyopathy of the right and left ventricle.
Severe right and moderate left ventricular systolic dysfunction. First degree AVB, arterial hypertension.
