Case description
A 28-year-old man without a familiar history of cardiomyopathies or ethanol consumption, only with a medical history of HIV category A1 diagnosed two years ago, who has been receiving antiretroviral therapy (ART) with tenofovir disoproxil fumarate plus emtricitabine and efavirenz since HIV diagnosis, was consulted due to cough, hemoptysis, fatigue, palpitations, and dyspnoea in the previous four weeks. At admission, he was tachycardic; on auscultation, rales were detected in all lung fields, and gallop was also auscultated. Also found were augmented jugular venous pressure with hepatojugular reflux, hepatomegaly, and lower limb edoema.
The hemogram was normal. The transthoracic echocardiogram showed a decreased left ventricular ejection fraction (LVEF) of 10%, and a left ventricular thrombus (LVT) of 40 by 38 mm. The chest computed tomography (CT) showed bilateral pleural effusion, multilobar alveolar occupancy, diffuse alveolar haemorrhage (DAH), pericardial effusion and the LVT. The contrasted enhanced cardiac magnetic resonance (CMR) revealed a normal right atrium with an area of 22 cubic centimetres (cm2); however, the left atrium was dilated with an area of 39 cm2. Also, the right and left ventricles were dilated with augmented diastolic diameters of 45 mm and 75 mm, respectively, in the setting of dilated cardiomyopathy (DCM). It also revealed a LVEF of 18%, subendocardial fibrotic areas in the LV apex and with a diffuse pattern in the right ventricle (RV) with a 20% myocardial fibrosis burden. The contrasted enhanced CMR also revealed a 40 by 38 mm apical mass that does not present perfusion at rest or late enhancement in relation to the LVT previously seen. (Figure 1).
Tuberculosis, histoplasmosis, cryptococcosis, aspergillosis, and acute infections due to aerobic bacteria, toxoplasmosis, cytomegalovirus, hepatitis B, hepatitis C, and Epstein-Barr virus were ruled out. Also ruled out were lupus, rheumatoid arthritis, cryoglobulinemia, and positive ANCA-associated vasculitis. Besides, a pleural and pericardial biopsy plus lobectomy from the subsegmental anterior segment of the right lower lobe was performed, which revealed DAH and pulmonary capillaritis (Figure 2). The pericardial and pleural biopsies only showed tissue congestion. In addition, a cardiac biopsy was performed, the result of which was normal.
The medical staff diagnosed ANCA-negative vasculitis. A course of intravenous methylprednisolone was administered for 3 days, followed by oral administration. Also, cyclophosphamide was administered due to the progression of DAH in a new CT. Warfarin was started for the LVT.
During follow-up, after one month, the patient experienced symptom resolution. Besides, the control echocardiogram showed an improvement of the LVEF with a new value of 25%. It also showed improvement in LVT, evidencing a decrease in their measurements to 29 by 33 mm. Furthermore, the new chest CT showed improvement in the lung involvement previously evidenced.