IJCS | Volume 33, Nº3, May / June 2020

301 Table 1 - Patient laboratory data Admission D14 Hemoglobin (g/dL) 10.8 9.8 Hematocrit (%) 34.2 30.9 Leukocytes (mm 3 ) 10,700 10,600 Bands (%) 1 2 CRP (mg/dL) 8.91 13.61 INR 1.15 Urea (mg/dL) 73 65 Creatinine (mg/dL) 1.06 0.86 Sodium (mEq/L) 133 130 Potassium (mEq/dL) 4.2 4.4 Nt-proBNP (pg/mL) 40,643 101,821 Viegas et al. CAVB and systemic sclerosis Int J Cardiovasc Sci. 2020; 33(3):299-302 Case Report the presence of anemia, increased CRP and increased NT-proBNP levels (Table 1). Despite correction of the conduction disorder, there was clinical worsening of HF and of the underlying disease activity with the ongoing treatment, thus a decision was made to start rituximab as rescue therapy 8 of SSc. Therewas progressiveworsening of HF symptoms despite optimal medical therapy and the patient evolved to death due to refractory cardiac shock. Discussion Cardiac involvement in SSc includes pericarditis, myocardial disease and conduction abnormalities. Be tween 25% and 75% of the pa t i ent s have electrocardiographic abnormalities, such as ST segment changes, ventricular or supraventricular arrhythmias and conduction disorders. 2 The most frequent conduction abnormality in SSc patients are left branch block, first- degree AV block, left anterior fascicular block and right branch block. CAVB is a rare complication that affects less than 2 percent of patients. 4-7,9 In a large international series of 3656 SSc patients, conduction disorders were observed in 12.7% of them. 10 Lung disease is seen in 61% of SSc patients, 11 and the most prevalent clinical manifestations are pulmonary fibrosis and pulmonary vascular disease, which cause arterial pulmonary hypertension (APH). 1-4,8 The prevalence of APH varies according with the diagnostic method used: between 8 and 12%, by right cardiac catheterization, and about 38% by TTE. 1,2,4 APH is often diagnosed late in the evolution of the disease, as observed in our patient. Patients who have clinically evident cardiopulmonary involvement evolve with a worse prognosis; 1-4,8 therefore screening of subclinical involvement in patients with SSc should be considered. Nevertheless, data on the best screening method are not well-defined yet. Initial investigation of cardiac involvement must include ECG, TTE and cardiac biomarker testing, such as brain natriuretic peptide (BNP) or its inactive form NT- proBNP. 2 In asymptomatic patients, without confirmed diagnosis by initial examination, the 24h Holter and cardiac imaging exams, such as magnetic resonance imaging, may be indicated. 8,11 The rapid progression of the underlying disease, refractoriness to HF treatment and the significant presence of APH contributed to the patient’s death. Conclusion The reported case reinforces the importance of early diagnosis of cardiac and pulmonary involvement in SSc, aiming at better therapeutic approaches and the reduction of morbidity and mortality. Author contributions Conception and design of the research: Nani ES, Mocarzel LOC, Gismondi RA. Acquisition of data: Viegas EC, ÁvilaDX. Writing of themanuscript: Viegas EC, Ávila DX. Critical revision of the manuscript for intellectual content: Nani ES, Mocarzel LOC, Gismondi RA. Potential Conflict of Interest No potential conflict of interest relevant to this article was reported. Sources of Funding There were no external funding sources for this study. Study Association This article is part of the thesis of graduation work submitted by Eduarda Cal Viegas, from Universidade Federal Fluminense .