ABC | Volume 112, Nº4, April 2019

Anatomopathological Correlation Case 2/2019 – Man with Arrhythmogenic Cardiopathy Followed by Rapidly Progressive Heart Failure Marcella Abunahman Freitas Pereir a, Wilma Noia Ribeiro , Léa Maria Macruz Ferreira Demarc hi Instituto do Coração (InCor) – Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP – Brazil Mailing Address: Vera Demarchi Aiello • Avenida Dr. Enéas de Carvalho Aguiar, 44, subsolo, bloco I, Cerqueira César. Postal Code 05403-000, São Paulo, SP – Brazil E-mail: demarchi@cardiol.br , anpvera@incor.usp.br Keywords Heart Failure; Stroke Volume; Cardiomyopathy, Dilated; Myocarditis; Heart Transplantation. Section Editor: Alfredo José Mansur (ajmansur@incor.usp.br ) Associated editors: Desidério Favarato (dclfavarato@incor.usp.br ) Vera Demarchi Aiello (anpvera@incor.usp.br ) DOI: 10.5935/abc.20190065 A 36-year-old man was referred to the medical service for surgical treatment of heart failure refractory to drug treatment. At the age of 26, 1 st -degree atrioventricular block and episodes of non-sustained ventricular tachycardia were detected on the electrocardiogram (ECG). After 4 years, he started to have episodes of pre-syncope. Themagnetic resonance performed at that time (09/29/2010) disclosed diastolic diameter of 59 mm; systolic diameter of 49 mm; 10-mm septum; posterior wall of 11 mm; 48% left ventricular ejection fraction and 53% right ventricular ejection fraction, with no contraction abnormalities. The late enhancement imaging showed an infero-septal, medium-basal subepicardial focus, compatible with fibrosis, suggestive of myocarditis or idiopathic dilated cardiomyopathy. An electrophysiological studywas indicated. After extra‑stimuli, sustained ventricular tachycardia with hemodynamic instability was triggered and an implantable-cardioverter defibrillator (ICD) was implanted and the patient received a beta-blocker. However, several episodes of ventricular tachycardia were recorded by the ICD and the use of amiodarone was initiated. He remained asymptomatic for approximately 3 years until he developed heart failure, which rapidly evolved into functional class IV, which resulted in hospitalization for compensation and with acute pulmonary edema at 34 years of age, followed by a new hospitalization a few months later for new heart failure compensation. At that time, hypothyroidism (TSH of 88 um / L) was diagnosed, which was attributed to amiodarone use. The echocardiogram disclosed severe left ventricular systolic dysfunction, with EF = 21%. Myocardial resynchronization was indicated, with pacemaker implantation with electrodes implanted at two points in the left ventricle in March 2015, but he was readmitted due to arterial hypotension, atrial fibrillation and heart failure decompensation in September 2015. Amiodarone, dobutamine, spironolactone, furosemide and rivaroxaban were administered. The patient was transferred to the Heart Institute (InCor) for possible surgical treatment of heart failure (heart transplantation) on October 20, 2015. The patient reported a 20 kg loss over a 4-year period. He denied arterial hypertension and diabetes mellitus. Physical examination showed cachexia, jugular swelling+, hepatojugular reflux, no distension alteration at the Valsalva maneuver or exhalation, vesicular murmurs present in the lungs, slightly reduced on the left pulmonary basis, palpable thrill in the mitral, tricuspid, accessory aortic foci; arrhythmic heart sounds with a more audible holosystolic murmur in the mitral focus, radiating into the posterior axillary line. The abdomen was flat, with a 6-cm hepatomegaly from the right costal border, palpable caudate lobe nearby, without ascites. Lower limbs without edema, with no signs of deep venous thrombosis. He was receiving intravenous dobutamine. The patient was evaluated by the heart transplant team due to the persistent need of high-dose inotropic and vasodilator drugs during ICU observation. The patient was prioritized for cardiac transplantation due to use of vasoactive drug and joined the list on 11/09/15. The ECG showed a pacemaker rhythm operating in VAT mode and chest X-ray showed cardiomegaly with signs of pulmonary congestion. Laboratory tests (10/21/2015) showedhemoglobin11.1 g/dL, hematocrit 34%, leukocytes 7290 (neutrophils 82%, eosinophils 3%, lymphocytes 7%, monocytes 6%), platelets 259,000/mm 3 , urea 28 mg/dL, creatinine 0.92 mg/dL, CRP of 69.34 mg/L, sodium 137 mEq/L, potassium 3.4 mEq/L, PAT (INR) of 2.4; APTT (rel. Times) of 1.31; Urinalysis with proteinuria of 0.67 g/L. The echocardiogram (10/21/2015) disclosed left ventriclewith diffuse hypokinesia, worse in the inferior and inferolateral walls and ejection fraction of 25%; the right ventricle showedmoderate diffuse hypokinesia. There was marked mitral regurgitation; the other valves showed no alterations. The pulmonary artery pressure was estimated at 49 mmHg. He had two bloodstream infections, which were treated with meropenem and vancomycin and tazobactam during the month of November 2015. Serological tests were positive for toxoplasmosis and mononucleosis in the IgG. Abdominal, thyroid and carotid artery ultrasonography results were normal. Right-chamber catheterization disclosed systolic pulmonary artery pressure of 55 mmHg, diastolic pressure of 23 mmHg and a mean pressure of 34 mmHg; the pulmonary occlusion pressure was 24 mmHg, the cardiac output was 5.5 L/min and the transpulmonary gradient was 10 mmHg; pulmonary 466