IJCS | Volume 33, Nº3, May / June 2020

DOI: 10.5935/2359-4802.20190069 299 CASE REPORT International Journal of Cardiovascular Sciences. 2020; 33(3):299-302 Mailing Address: Eduarda Cal Viegas Rua Marques de Paraná, 303. Postal Code: 24033-900, Centro, Niterói, RJ – Brazil. E-mail: eduardacviegas@hotmail.com Complete Atrioventricular Block and Cardiopulmonary Involvement in Rapidly Progressive Systemic Sclerosis Eduarda Cal Viega s, D iane Xavier de Ávila , E duardo Nani Silv a, L uís Otávio Cardoso Mocarze l, Ronaldo Altenburg Gismon di Universidade Fluminense Federal (UFF), Rio de Janeiro, RJ - Brazil Manuscript received on March 06, 2018, revised manuscript on May 30, 2018, accepted on November 01, 2018. Heart Failure; Atrioventricular Block; Scleroderma, Systemic; Hypertension, Pulmonary; Pulmonary Fibrosis. Keywords Abstract The heart and lung are target organs in systemic sclerosis (SSc) and similar symptoms (dyspnea and cough) may make the differential diagnosis between the two lesions difcult. In addition, complete atrioventricular block (CAVB) is a rare complication of this disease. This case report is about a patient with SSc and pulmonary fibrosis who was admitted to the emergency room with CAVB, heart failure (HF) and progressive worsening of the underlying disease. Introduction Systemic sclerosis (SSc) is amultifactorial autoimmune connective tissue disease, with high morbidity and mortality rates, whose prevalence in the general population is 5%. 1,2 It is characterized by vascular injury and fibrosis of the skin and internal organs, the heart and lungs being the most frequently involved organs. 3,4 It is divided into 2 main subsets based on the extent of cutaneous involvement, limited and diffuse; the latter is associated with more visceral involvement. 1-3 Cardiac involvement can affect the pericardium, the myocardium, and the conduction system. 4-7 Complete atrioventricular block (CAVB) is the least common conduction abnormality. 4-7 Here, we report a case of rapidly progressive systemic sclerosis complicated by CAVB and heart failure (HF). Case Report We report the case of a 50-year-old black man diagnosed with rapidly progressive diffuse Systemic Sclerosis, confirmed by clinical and serological tests 8 under treatment with methotrexate, folic acid and monthly pulse therapy with methylprednisolone and cyclophosphamide. He sought care in the emergency room presenting with a clinical picture of HF, with progressive worsening in the last three months, in addition to evening fever. On physical examination he presented tachypnea, with no signs of respiratory effort, cold extremities, slow capillary filling, JVP at 45 degrees and cannon “a” wave in JVP and diffuse skin thickness. Blood pressure 110/70 mmHg, heart rate 42 bpm and respiratory rate 26 bpm. The examination of the thorax revealed left deviation of the ictus cordis, regular heart rate, presence of LV third heart sound, a grade 2/6 systolic murmur and a grade 3/6 tricuspid regurgitationmurmur. Lungs with bilateral crackles. Hepatomegaly with pain on palpation. Bilateral lower extremity edema (2+/4+). Electrocardiogram (ECG) showed CAVB (Figure 1). Therapywith intravenous furosemide, spironolactone and enalapril was initiated upon admission, and the patient was referred to the cardiac intensive care unit. On the second day of hospitalization, a permanent dual- chamber, epicardial pacemaker was implanted (Figure 2). The transthoracic echocardiography (TTE) revealed increased left cavities, diffuse hypokinesia, LVEF of 38% (Simpson’s rule), moderate mitral and tricuspid regurgitation and PSAP 65 mmHg. Thoracic computed tomography (CT) displayed ground-glass opacity distributed diffusely through both lungs, bronchiectasis, inter and intralobular septal thickening and paraseptal emphysema in the upper lobes. Laboratory tests showed