Chronic thromboembolic pulmonary hypertension with cor pulmonale is an
extremely debilitating disease that (1) is more common than generally
recognized, (2) is often misdiagnosed, and (3) is difficult to treat.
When a patient has persistent exertional dyspnea with no obvious caus
e, a ventilation-perfusion, echocardiography, and (if indicated) pulmo
nary angiography should be done. Prevention is especially important be
cause by the time a patient is symptomatic, the disease is already far
advanced and hemodynamic reserves are greatly reduced. Prevention of
recurrence mandates lifetime anticoagulation and placement of a vena c
ava filter. The only effective treatment is pulmonary endarterectomy,
which is being performed at an increasing number of specialty centers
across the United States.