C85. Right heart failure due to chronic thromboembolic pulmonary hypertension in systemic lupus erythematosus patient: a case report

Abstract

Abstract Background Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with varying clinical manifestations and high risk of thrombosis. Chronic Thromboembolic Pulmonary Hypertension (CTEPH) as late complication of acute pulmonary embolic sometimes occured in SLE patients and in advanced condition it can manifest as right heart failure. Case Summary A female, 29 years old with complaints of bilateral ankle oedema and bloated stomach, as well as dyspneu on effort occurred progressively since approximately 4 months. These complaints were also accompanied by hair loss and redness on both cheeks. Physical examination revealed increased jugular venous pressure, systolic murmur in left lower sternal border, ascites, hepatomegaly, and bilateral ankle oedema. Echocardiography revealed dilated right atrium and right ventricle (RV), decreased RV systolic function, severe tricuspid regurgitation, and distended inferior vena cava. Computed tomography (CT) pulmonary angiography showed filling defect in the left pulmonary artery. Lab examination showed an increase in D-dimer, ANA titer >1: 1000, and lupus anticoagulant ratio 1.59. Diuretics therapy, anticoagulant, corticosteroid, and immunosuppressant were given to this patient. Right heart function was improved following the treatment. Discussion The increased risk of thrombosis in this case is related to several factors, including: antiphospholipid antibodies, inflammation and disease activity. CTEPH is formed as a result of poor resolution of the thrombus in the pulmonary arteries, resulting in fibrosis of residual thrombotic material that can impede blood flow. Increased intrapulmonary pressure due to pulmonary vascular obstruction causes RV remodeling and dysfunction.