David B Badesch, University of Colorado Health Sciences Centre, Denver, Colorado, USA
Published online: April 2001
DOI: 10.1038/npg.els.0002115
Pulmonary hypertension is an abnormal increase in blood pressure within the lung circulation. This potentially fatal disorder was previously associated with a poor prognosis, but long-term survival is now possible with aggressive medical therapy and/or lung transplantation.
Keywords: calcium channel blockers; prostacyclin; epoprostenol; lung transplantation
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Figure 1. Plexiform lesion associated with focal medial disruption (arrowhead) and aneurysmal dilatation of small pulmonary arteries, with formation of a complex proliferative tuft of intimal cells (large arrow) and channels (small arrows).
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Figure 2. General diagnostic and treatment algorithm. This overview of the author's general approach to the patient with suspected pulmonary hypertension requires significant individualization. In addition, it should be noted that diagnostic and therapeutic techniques are changing rapidly. ACE, angiotensin-converting enzyme; ANA, antinuclear antibodies; CBC, complete blood count; CT, computed tomography; ECG, electrocardiogram; ECHO, echocardiography; ESR, erythrocyte sedimentation rate; PFT, pulmonary function test; PT, prothrombin time; PTT, partial thromboplastin time; RF, rheumatoid factor; SMA-19, sequential multiple analyser-19, complete chemistry panel.
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Figure 3. Chest radiograph of patient with severe primary pulmonary hypertension. The posterior–anterior and lateral chest radiographs of this patient demonstrate marked enlargement of the cardiac silhouette, prominence of the main pulmonary artery outflow tract, filling in of the retrosternal air space, and a paucity of peripheral pulmonary vascular markings.
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