Abstract A 27-year-old female with a history of glomerulonephritis,with diagnosed systemic lupus and retroperitoneal fibrosis was admitted due to fast worsening dyspnoea(NYHA class IV) over the prior four weeks with suspicion for PA hypertension. In 2015, retroperitoneal fibrosis was diagnosed based on MR examination. With the methylprednisolone treatment a regression of changes was obtained. The patient was chronically treated with prednisone 7.5mg/day and mycophenolate mofetil 2g/d. When admitted to the ward, the patient was in a severe condition, with symptoms of resting dyspnea and blood oxygen saturation up to 80%. A loud systolic murmur was audible over the entire chest. In laboratory tests, the NT-proBNP values (11,000 pg/ml) were significantly elevated, arterial blood gases showed tendency to alkalosis, hypoxemia (PO2 45mmHg)with hypocapnia. Parameters of inflammation were moderately elevated (CRP 25mg/l, WBC 12,000/ul), with normal serum IgG4 concentrations. In TTE, significant dilatation of the right ventricle with left chamber compression were observed. Tricuspid ring dilation with no leaflets coaptation and a severe tricuspid regurgitation (Vmax 5m/s, RVSP 100mmHg) were found. In addition, the critical proximal pulmonary artery (PA) stenosis was revealed (left PA Vmax 3.3m/s, Pmax 42mmHg, right PA Vmax 5.2 m/s, Pmax 110mmHg). Angio-CT confirmed critical proximal constriction of both PAs with a flow channelup to 4 mm. Based on angio-CT and cardiac MR study the inflammatory etiology of stenoses was suspected and an empiric diagnosis of the Takayahu disease of pulmonary arteries was made. The patient was qualified for conservative treatment using prednisone and cyclophosphamide. Over the course of two months,2.8 g of cyclophosphamide were administered intravenously in three cycles with good response to the treatment. It resulted in regression of dyspnea and a improvement in exercise tolerance - to the NYHA class II, normalization of saturation. Laboratory tests showed reduction of NT-proBNP (1300 pg/ml). Control TTE revealed reduction in the size of the right ventricle and regression of PAs stenoses (left PA 11mm, right PA 7mm), also reduction of systolic flow gradients (left PA Vmax 3.1 m / s, Pmax 39 mmHg, right PA 4.1 m/s, Pmax 68 mmHg). Because of the significant improvement in patient’s condition,it was decided to continue the treatment. Legend for the Fig.1 A angio-CT, critical proximal constriction of both PA B TTE, parasternal short axis view, imflammatory infiltration C TTE, continuous wave doppler, flow velocity pattern in the PA stenosis D TTE PLAX, dilatation of the right ventricle with left chamber compression fig. 1 Abstract P1323 Figure. Fig.1 artety pulmonary stenosis
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