Chronic pulmonary embolism. Often misdiagnosed, difficult to treat

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 cause, a ventilation-perfusion scan, echocardiography, and (if indicated) pulmonary angiography should be done. Prevention is especially important because 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 cava filter. The only effective treatment is pulmonary endarterectomy, which is being performed at an increasing number of specialty centers across the United States.