Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease often not recognized until it is far advanced. Medical management does not reverse the effects of the disease, nor does it prevent its progression. Pulmonary thromboendarterectomy is the preferred treatment.We present the case of a patient in whom CTEPH was mistaken for an interstitial lung disease with mediastinal lymph node enlargement. The mediastinal lymph node enlargement was due to vascular transformation of the lymph node sinuses (VTS). This is an unusual case of chronic thromboembolic pulmonary hypertension with vascular transformation of the lymph node sinuses. Eur Respir J 1997; 10: 1191-1193 Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but potentially treatable cause of pulmonary hypertension. Unfortunately, there is often a delay in the diagnosis due to the fact that signs and symptoms are nonspecific in the majority of these patients. For this reason, CTEPH is frequently mistaken for other entities, such as chronic obstructive pulmonary disease, coronary artery disease, interstitial lung disease, congestive heart failure, or psychosomatic dyspnoea [1].We present the case of a patient in whom enlarged mediastinal lymph nodes and a mosaic pattern of lung attenuation on chest computed tomography (CT) scan were mistaken for interstitial lung disease. At autopsy, CTEPH and vascular transformation of the lymph node sinuses (VTS) were found.
Case reportA 32 year old male was transferred to our institution because of respiratory distress and haemoptysis after transbronchial biopsy. Since the age of 10 yrs, the patient had been treated with inhaled β 2 -mimetics on demand, for allergic asthma. He had a smoking history of 15 pack-years but had ceased smoking 3 yrs before admission. Varicectomy of the lower limbs had been performed 2 yrs earlier.The patient was examined at another hospital a few months before admission because of worsening exertional dyspnoea and wheezing. Inhaled corticosteroids, budesonide 400 mg·day -1 , were added to his treatment, without any improvement in his symptoms. Spirometry performed at that time showed moderate obstructive pulmonary disease, with significant bronchial hypersensitivity at histamine provocation testing (provocative dose causing a 20% fall in forced expiratory volume in one second (PD20) 0.8 mg·mL -1 ). A chest radiograph was reported to show cardiac enlargement and pulmonary hyperinflation. An electrocardiogram revealed sinus rhythm with inverted T-waves in the precordial leads, and an echocardiogram demonstrated considerable enlargement of the right atrium and ventricle. Mean pulmonary artery pressure by Doppler evaluation of the tricuspid regurgitant envelope was estimated at 42 mmHg. A CT scan of the chest ( fig. 1a and b) revealed mediastinal lymph node enlargement and a mosaic pattern of lung attenuation, suggesting interstitial lung disease.Bronchoscopy and transbronchial biopsy were performed but haemoptysis and respiratory distress developed soon afterwards. The ...