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Pulmonary Hypertension

Sean Gaine, MD, PhD
JAMA. 2000;284(24):3160-3168. doi:10.1001/jama.284.24.3160.
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A clinically useful, treatment-based classification of pulmonary hypertension divides the disease into 5 distinct categories: (1) pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia; (2) pulmonary venous hypertension; (3) chronic thromboembolic disease; (4) pulmonary arterial hypertension; and (5) pulmonary hypertension due to disorders directly affecting the pulmonary vasculature. Pulmonary arterial hypertension includes individuals with primary pulmonary hypertension, congenital heart disease, connective tissue disease, and liver disease. These heterogeneous diseases have similar characteristic pathological changes, including in situ thrombosis, smooth muscle hypertrophy, and intimal proliferation. Right heart catheterization is essential to confirm diagnosis, determine prognosis, and assign therapy. A minority of patients have a favorable response to an acute vasodilator trial and long-term benefit with calcium channel blocker therapy. Continuous intravenous epoprostenol improves symptoms and survival in patients with advanced primary pulmonary hypertension and has potential benefit in other forms of pulmonary arterial hypertension. Lung transplantation remains an important option for individuals in whom maximal medical therapy fails. The recent discovery of the gene for familial primary pulmonary hypertension and the increase in new drugs undergoing clinical trials are encouraging developments.

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Figure 1. Characteristic Pathological Features Seen in Pulmonary Arterial Hypertension
Graphic Jump Location
A pulmonary artery revealing marked narrowing of the lumen (arrow) from smooth muscle hypertrophy and intimal proliferation. A characteristic plexiform lesion is also present (arrowhead). A pathologist will not be able to determine whether the specimen was from a patient with scleroderma, Eisenmenger syndrome, or whether, like Mrs JL, they took anorexigens and developed primary pulmonary hypertension.
Figure 2. Chest Radiograph in Primary Pulmonary Hypertension
Graphic Jump Location
A, Posteroanterior radiograph revealing the prominent pulmonary arteries and clear lung fields characteristic of primary pulmonary hypertension. B, The lateral radiograph demonstrates enlargement of the right ventricle with encroachment of the retrosternal space.
Figure 3. Pathogenesis of Pulmonary Arterial Hypertension
Graphic Jump Location
Pulmonary arterial hypertension occurs in susceptible patients as a result of an insult to the pulmonary vascular bed resulting in an injury that progresses to produce the characteristic pathological features. HIV indicates human immunodeficency virus; BMPR2, bone morphogenetic protein receptor II gene.
Figure 4. An Echocardiogram From a Patient With Severe Pulmonary Hypertension
Graphic Jump Location
The left side of the heart is compressed by the enlarged right atrium (RA) and right ventricle (RV). LV indicates left ventricle and LA, left atrium.
Figure 5. Treatment Algorithm for Pulmonary Arterial Hypertension
Graphic Jump Location
A right heart catheterization and vasodilator trial are performed to determine the approach to therapy. Patients who have a favorable acute response are treated long term with calcium channel blocker therapy. Nonresponders or those who did not respond to calcium channel blockers are considered for continuous intravenous epoprostenol therapy either as a bridge to transplantation or as definitive long-term therapy. NYHA indicates New York Heart Association.



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