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Pulmonary veno-occlusive disease.

U Thadani, C Burrow, W Whitaker

    The Quarterly Journal of Medicine
    |January 1, 1975
    PubMed
    Summary
    This summary is machine-generated.

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    Pulmonary veno-occlusive disease (PVOD) is a fatal cause of pulmonary arterial hypertension. Diagnosis can be challenging, often requiring histological confirmation due to overlapping symptoms with other conditions.

    Area of Science:

    • Cardiology
    • Pulmonary Medicine
    • Pathology

    Background:

    • Pulmonary veno-occlusive disease (PVOD) is a rare condition causing pulmonary arterial hypertension.
    • It is characterized by insidious onset, progressive nature, and a poor prognosis due to lack of effective treatment.
    • Early symptoms like breathlessness can be misdiagnosed as anxiety states.

    Purpose of the Study:

    • To review reported cases and a new patient with pulmonary veno-occlusive disease.
    • To highlight diagnostic challenges and differentiate PVOD from other pulmonary hypertension causes.
    • To describe the pathological features of PVOD.

    Main Methods:

    • Review of twenty previously reported cases and one new patient.
    • Clinical assessment including symptoms, signs, and diagnostic imaging (pulmonary angiography, lung scanning).

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  • Histopathological examination of lung tissue to identify characteristic venous lesions.
  • Main Results:

    • PVOD presents with breathlessness and signs of pulmonary arterial hypertension.
    • Some patients exhibit signs of pulmonary venous and capillary hypertension, aiding diagnosis when left atrial pressure is normal.
    • Histology reveals widespread occlusion of pulmonary veins by intimal fibrosis, often with recanalization.

    Conclusions:

    • Pulmonary veno-occlusive disease is a distinct pathological entity causing pulmonary arterial hypertension.
    • Clinical recognition is possible in some cases, but histological confirmation is often necessary.
    • The disease is invariably fatal, with a median survival of approximately two years post-diagnosis.