Pulmonary veno-occlusive disease: diagnosis during life in four patients.

Abstract:

:Pulmonary veno-occlusive disease is a rare form of primary pulmonary hypertension of unknown aetiology. Four cases were diagnosed in young patients. The diagnosis was suspected on the basis of clinical, radiological, echocardiographic, and catheter evidence and confirmed by taking a lung biopsy sample. In all patients the histology showed obstruction of the pulmonary veins by intimal fibrosis. The clinical course of all patients has been one of progressive deterioration. Although there is no specific treatment for this disease, to establish the diagnosis during life is of great importance in overall clinical management, including counselling the patient and family.

journal_name

Arch Dis Child

authors

Justo RN,Dare AJ,Whight CM,Radford DJ

doi

10.1136/adc.68.1.97

subject

Has Abstract

pub_date

1993-01-01 00:00:00

pages

97-100

issue

1

eissn

0003-9888

issn

1468-2044

journal_volume

68

pub_type

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