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.