[An unusual cause of arterial and venous pulmonary hypertension]

A 49 year old woman with syphilis of recent onset was repeatedly admitted to the hospital on account of recurrent dyspnoea and hemoptysis. The symptoms and radiological signs indicated the presence of a "cardiac lung". The ECG disclosed right ventricular overload which was confirmed by echocardiography. The left heart was normal, not only on echocardiography but also on cardiac catheterization, which documented severe pulmonary hypertension, partly precapillary, partly postcapillary. The presence of chronic lung congestion in the absence of pathological findings pertaining to the left heart lead to the diagnosis of pulmonary venous occlusive disease. However, pulmonary angiography showed notable and widespread arterial amputations which are not present in this syndrome. Autopsy provided the correct diagnosis: sclerosing mediastinitis localized at the hilum with prevalent involvement of both arterial and venous pulmonary vessels.