Editor's Memo

It is with great excitement that I introduce the Fall 2009 issue of Advances, devoted to pulmonary hypertension in the setting of lung or respiratory disease (or WHO Diagnostic Group III). Dr Omar Minai has done an outstanding job of coordinating the issue and contributing one of the articles.

The topic of pulmonary hypertension in patients with underlying pulmonary disorders is critical. What are the mechanisms involved in the development of PH in these patients? How common is PH in this group? How severe? When can we attribute PH in an individual patient to the underlying pulmonary disease and when is the PH “out of proportion”? And importantly, can we treat any of these patients with PAH-specific therapies?

This last question is, indeed, a charged topic. In reality, PAH-specific drugs are already being used in patients with underlying pulmonary disease. Does this represent inappropriate, off-label use of costly therapies, or thoughtful, albeit empiric, application of targeted treatment to patients who may share many of the histopathologic features with more “typical” PAH patients? The truth, of course, is probably somewhere in the middle, but what are the data?

As someone who lectures widely on all aspects of pulmonary hypertension, I am asked after virtually every talk whether the current PAH therapies are ever indicated in patients with PH and underlying lung or respiratory disease, especially COPD. This is not a surprising question. After all, these are the patients that physicians see. A community-based pulmonologist rarely ever sees a 30-year-old woman with idiopathic pulmonary arterial hypertension. What he or she sees regularly is a 65-year-old overweight patient with some underlying sleep apnea, hypertension, and COPD. Some of these patients develop pulmonary hypertension, sometimes with severe right ventricular failure.

The practitioners want to know from us, the experts, what to do to help these patients. We need to provide a framework by which clinicians can approach and evaluate such a patient when he or she develops PH. I am hopeful that this issue of Advances will provide, to the practicing physician, at least parts of that framework.