Pulmonary veno-occlusive disease.

摘要: Pulmonary veno-occlusive disease has recently been recognized as a distinct pathological entity and cause of pulmonary arterial hypertension. Twenty previously reported cases new patient are here reviewed. The majority presented with breathlessness in the early stages disease, when abnormal signs were not striking, some patients wrongly diagnosed suffering from an anxiety state. condition usually insidious onset but is remorselessly progressive since no effective treatment available at present, invariably fatal have died within two years. fully developed clinical picture dominated by symptoms hypertension, similar to those found other diseases causing raised blood pressure. However, show, addition, venous capillary which can lead its recognition associated normal left atrial In this wedge pressure would appear be unreliable record angiography lung scanning will differentiate massive thromboembolic hypertension primary or micro thromboembolism. Although it should now possible recognise life, there others where, even after full investigation, still impossible thromboembolism these diagnosis only made distinctive histological pattern demonstrated. widespread occlusion veins venules loose intimal fibrosis often basophilic. Recanalization occluded common may very striking. These occlusive lesions elevation vessels, also responsible for chronic oedema elveolar walls subsequent development interstitial fibrosis. present case organised thrombi arteries addition lesions.