ABC | Volume 110, Nº2, February 2018

Anatomopathological Correlation Favarato & Benvenuti Cardiomyopathy with arrhythmia and syncope Arq Bras Cardiol. 2018; 110(2):195-200 During the study, poorly-tolerated sustained ventricular tachycardia of epicardial origin was observed and reversed with electrical cardioversion. The procedure was successful. In March 2014, the patient sought the emergency unit reporting three shocks of the implantable cardioverter defibrillator in the morning while walking on the beach, the first being preceded by tachycardia. The patient had bradycardia (40 bpm) while receiving intravenous amiodarone. He remained asymptomatic during hospitalization (March 3 to 14, 2014). The patient was discharged with the following prescription: 50 mg of losartan, 200 mg of amiodarone, 25 mg of spironolactone and 75 mg of metoprolol succinate, in addition to programming of the implantable cardioverter defibrillator to pacemaker capture threshold of 40 bpm. His catheterization (September 29, 2015) revealed: mean right atrial pressure, 14 mm Hg; right ventricular pressures (systolic/initial diastolic/final diastolic), 28/06/14 mmHg; pulmonary artery pressures (systolic/diastolic/mean), 28/18/21 mmHg; pulmonary occlusion pressure, 18 mmHg; aortic pressures (systolic/diastolic/mean), 93/60/71 mmHg; cardiac output, 3.78 mL/min; pulmonary vascular resistance, 0.79 woods; arterial O 2 saturation, 99.1%; venous O 2 saturation, 70.4%. A new electrophysiological study was performed (September 29, 2015). At the beginning of the procedure, the patient was in sinus rhythm, and had periods of atrioventricular block 2:1 and total atrioventricular block during the procedure. To desfibrillitor was summed a pacemaker elecatrode programmed to VVI pacing for 40 bpm. Electrophysiological mapping and voltage mapping of the right ventricle (endocardial) were performed, evidencing a scar area in the lateral region of the right ventricular outflow tract, extending to the tricuspid annulus. The extra stimuli induced type I ventricular tachycardia (VT1) with positive complexes in I and aVL leads, with negative superior axis in V 1 and no transition. The activation mapping during tachycardia evidenced mesodiastolic potential in the scar area of the lateral region of the right ventricular outflow tract. Radiofrequency application in that site terminated the VT1. In addition, the scar area was homogenized from the lesion to the tricuspid annulus. New tests with extra stimuli failed to induce arrhythmias. The patient had no arrhythmia and only a few symptoms of dyspnea. During a medical visit in January, 2016, the patient reported worsening of symptoms, with dyspnea occurring while taking a bath or walking less than two blocks, in addition to weight loss, although his appetite was preserved. His daily prescription was as follows: amiodarone 400 mg, spironolactone 25 mg, metoprolol succinate 50 mg, losartan 50 mg, levothyroxine 75 mcg, magnesium 400 mg, and furosemide 20 mg. His physical examination revealed blood pressure of 100/80 mm Hg, heart rate of 60 bpm, regular perfusion, and signs of neither hypervolemia nor pulmonary congestion. His daily dose of losartan was increased to 75 mg. The patient continued very limited regarding his daily activities, being placed on the waiting list for cardiac transplantation, which was performed on April 12th, 2016. Clinical aspects The patient had syncope episodes since the age of 20 years, and heart failure since the age of 30 years, undergoing cardiac transplantation at the age of 36 years. Since symptom onset, heart disease with marked left ventricular dilatation and moderate dysfunction was detected. The exercise test revealed frequent ventricular arrhythmia. His syncope episodes were attributed to malignant ventricular arrhythmias, and a cardioverter defibrillator was implanted. The patient remained stable and with no syncope episode for 11 years, when marked decrease in ventricular ejection fraction was detected. At the age of 34 years, the cardioverter defibrillator delivered an appropriate shock during an episode of ventricular tachycardia, and the patient was submitted to an electrophysiological study, which triggered atrial fibrillation and the cavotricuspid isthmus was blocked. During the same procedure, areas compatible with scars and low-voltage late potentials in the basal posterior and lateral walls of the right ventricle were observed, undergoing ablation, which eliminated the potentials. However, during the procedure, poorly-tolerated sustained ventricular tachycardia of epicardial origin was observed and reversed with electrical cardioversion. One year later, the patient had a new episode of tachycardia and appropriate cardioverter defibrillator discharge, undergoing then a new electrophysiological study, which evidenced a scar in the right ventricular outflow tract, extending to the tricuspid annulus. The extra stimuli triggered VT1 arising in the right ventricle, which was terminated with ablation with radiofrequency application. A few months later, the patient was hospitalized due to NYHA functional class III heart failure, being placed on the waiting list for cardiac transplantation. This is a case of heart disease, presenting as episodes of syncope due to ventricular arrhythmias and mild left ventricular dysfunction, despite left ventricular dilatation. Some of the heart diseases that progress mainly with ventricular arrhythmias are as follows: Chagas heart disease, sarcoidosis, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy. Regarding Chagas disease, it is known to cause frequent arrhythmias, heart failure and sudden death, but the patient had neither a typical ECG nor a typical echocardiogram. His ECG showed neither right bundle branch block nor left anterior hemiblock, and his echocardiogram revealed neither diffusemarked hypokinesia nor an apical aneurysm. In addition, apparently there was neither positive epidemiology for that disease, nor predominance of right heart failure signs. 1,2 In sarcoidosis, heart impairment is mainly characterized by atrioventricular blocks, malignant arrhythmias and sudden death, all caused by infiltration of the conduction system and myocardium by noncaseous granuloma. Some studies on pulmonary or systemic sarcoidosis have reported cardiac impairment in 5% of the patients and in up to 25% of postmortem examinations. However, imaging tests have shown impairment in up to 50% of the patients. Individuals with extracardiac sarcoidosis diagnosis confirmed on biopsy should 196