C15. Biventricular Heart Failure and Pulmonary Hypertension in Systemic Sclerosis: a rare and unusual case report

Abstract

Abstract Backgrounds Systemic sclerosis (SSc), chronic multisystem autoimmune disease, characterized by excessive fibrosis and vascular endothelial dysfunction. Cardiovascular involvement may be induced by various pathways, either primary or secondary to pulmonary hypertension or renal involvement. Case Summary A 46 years old woman presented to emergency department with cold, dark purplish ulcers in fingertips with progressive worsening pain in upper extremities, claudication intermitten, and dyspneu since 4 months ago. She had a mask-like facial, shiny skin, microstomia, restricted mouth opening, calcinosis cutis, Raynauds phenomenon, and sclerodactyly. There were pansystolic murmur grade III/6 in tricuspid area, and rales in basal lungs. ECG showed sinus rhythm with fragmented QRS, poor R-wave progression. Chest X-Ray revealed pancardiomegaly with pleural effusion and HRCT thorax revealed interstitial lungs disease. The laboratory revealed leucocytosis, increase D-dimer (870 ug/L), negative ANA and ds-DNA test, positive anti SCl test. Echocardiography showed biventricular dysfunction with decreased LVEF 37% (Teichz) and TAPSE 10 mm, RA RV dilatation, severe TR with high probability for PH. Extremities Doppler showed normal arteries and veins system. These findings indicates that primary and secondary cardiac involvement in SSc. Heart failure, pulmonary hypertension, and cyclophosphamide medication were administered Discussion Primary cardiac involvement, a direct consequence of SSc, may manifest as fibrosis of the myocardial, conduction system, pericardial and, valvular disease. Furthermore, a secondary phenomenon due to pulmonary arterial hypertension (PAH), and kidney pathology. Though the burden remains low, it may worsen the outcome, consequently, all patients with SSc should undergo screening.