The often relentless progression of idiopathic pulmonary hypertension (IPH) in patients who are usually young and otherwise healthy pressures the physician to do something. The primary clinical mandate to do no harm, however, must supersede the urge to act.
There are two positive steps that follow the "no harm" mandate in the patient with suspected IPH. The first is to exclude the multiple entities mimicking IPH. These include intracardiac shunts, radiographically silent interstitial lung diseases, left atrial myxoma and thrombi, clinically occult left ventricular or mitral valvular diseases, pulmonary veno-obstructive disorders, and chronic thromboembolic obstruction. Alternative therapies exist for most of these disorders. Therefore, detailed cardiopulmonary evaluation to exclude those diseases is mandatory before diagnosing IPH.
The second step entails trying—under carefully controlled conditions—to define a practical drug regimen that will reduce right ventricular afterload. Several drugs known to have the ability to relax pulmonary arterial smooth muscle have been
Moser KM. Idiopathic Pulmonary Hypertension: Therapeutic Opportunities and Pitfalls. JAMA. 1982;247(22):3119. doi:10.1001/jama.1982.03320470065037
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