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Clinical primary pulmonary hypertension: three pathologic types

W D Edwards, J E Edwards

    Circulation
    |November 1, 1977
    PubMed
    Summary
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    Primary pulmonary hypertension encompasses three conditions: plexogenic pulmonary arteriopathy, recurrent pulmonary thromboembolism, and pulmonary veno-occlusive disease. Definitive diagnosis requires histologic examination of lung tissue.

    Area of Science:

    • Cardiovascular Medicine
    • Pulmonary Medicine
    • Pathology

    Background:

    • Elevated pulmonary arterial pressure without apparent cause is clinically termed primary pulmonary hypertension.
    • Pulmonary arterial wedge pressure is not elevated in these cases.
    • This clinical diagnosis encompasses distinct underlying pathological conditions.

    Purpose of the Study:

    • To differentiate three pathological entities within primary pulmonary hypertension.
    • To describe the characteristic histopathology of each entity.
    • To emphasize the necessity of histological examination for definitive diagnosis.

    Main Methods:

    • Clinical categorization of primary pulmonary hypertension.
    • Histologic examination of lung tissue (biopsy or necropsy).

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  • Description of pathological features for each entity.

    • Main Results:

    • Primary pulmonary hypertension comprises plexogenic pulmonary arteriopathy, recurrent pulmonary thromboembolism, and pulmonary veno-occlusive disease.
    • Plexogenic pulmonary arteriopathy involves vasoconstriction, medial hypertrophy, and plexiform lesions.
    • Recurrent pulmonary thromboembolism is characterized by thrombi in small pulmonary arteries.
    • Pulmonary veno-occlusive disease involves obstruction of pulmonary veins and venules.

    Conclusions:

    • The clinical presentation of these three conditions can be similar.
    • Histologic examination of lung tissue is crucial for accurate diagnosis.
    • Distinguishing these entities is vital for appropriate patient management.