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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