PATIENT AND METHODS
We present the case of a 35-year-old Asian man who in 2018 had an echocardiographic diagnosis of severe aortic regurgitation due to aortic root dilatation, with normal left and right ventricular functions and no signs of PH. In the same year the patient underwent aortic root and aortic valve replacement with a mechanical composite valve graft at his local hospital. During the following months he suffered frequent oral and genital aphthosis and folliculitis on his back. Based on these symptoms and a remarkable pathergy phenomenon, Behcet’s disease (BD) was diagnosed. Thus, treatment with glucocorticoids and monthly cyclophosphamide intravenous pulses (up to 6) was started. After finishing induction treatment, maintenance treatment with azathioprine was prescribed1-2.
In 2020 the patient was admitted to our hospital because of a 3-month history of progressive dyspnea and dry cough. The echocardiography documented a large intra-mediastinal pseudoaneurysm, severe PH with systolic pulmonary artery pressure (sPAP) of 65 mmHg, preserved left ventricle function, severe tricuspid regurgitation, and an impaired and dilated right ventricle: basal diameter of 49 mm, tricuspid annular plane systolic excursion (TAPSE ) of 14mm. CT scan documented partial dehiscence of the distal anastomosis of the dacron graft to the ascending aorta, and confirmed the presence of a giant aortic pseudoaneurysm (10x7.3x8.4 cm) compressing the pulmonary trunk and both pulmonary arteries (Figures 1 and 2).
Optimization of medical treatment was carried out replacing azathioprine with infliximab 5mg/kg (300mg per dose)2-3. Surgery was carried out under extracorporeal circulation and in moderate hypothermia (25°C). The voluminous aortic pseudoaneurysm was resected and repaired with a new 28mm dacron graft. The immediate postoperative period was uneventful.
At 2 months the echocardiography showed preserved left ventricle function, dilated and normo-contractile right ventricle (TAPSE 19mm, basal diameter 50 mm), mild tricuspid regurgitation and normalization of pulmonary pressures with sPAP of 27 mmHg.