Major Pulmonary Artery Stenosis Causing Pulmonary Hypertension in Sarcoidosis

doi:10.1378/chest.78.6.888

Chest

Volume 78, Issue 6, December 1980, Pages 888-891

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A chest bruit and evidence of pulmonary hypertension developed in a woman with sarcoidosis. A perfusion lung scan revealed decreased perfusion to the right upper lobe and to the entire left lung, while a ventilation scan was normal. Pulmonary angiography demonstrated multiple bilateral concentric pulmonary artery stenoses most consistent with extrinsic compression by mediastinal granulomatous inflammation and fibrosis. The patient was treated with systemic corticosteroids for one year without improvement Major pulmonary artery stenosis should be considered in patients with sarcoidosis who developed pulmonary hypertension and are found to have lung scans showing segmental perfusion defects in normally ventilated areas or who develop an unexplained chest bruit.

Section snippets

CASE REPORT

A 39-year-old woman was well until 1967 when she developed intermittent migratory chest pain. A chest roentgenogram revealed bilateral hilar and paratracheal adenopathy with normal pulmonary parenchyma. A scalene node biopsy specimen revealed noncaseating granulomas. In 1973, the patient was admitted to the University of Michigan with right 6th and 7th cranial nerve paresis. She had no pulmonary symptoms and was receiving no medication. Pulmonary function testing revealed only a mild reduction

COMMENTS

Stenosis of large pulmonary arteries has been previously demonstrated in patients with sarcoidosis,6, 7, 8, 9, 10 but stenoses sufficiently severe to result in pulmonary hypertension have not been well documented. Battesti and colleagues⁹ have reported a patient with sarcoidosis who had stenoses of central pulmonary arteries and clinical evidence of pulmonary hypertension, but pulmonary artery pressures were not measured and the relative contributions of central and peripheral vascular

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