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Chicago, IL
Dr. Rich is Professor of Medicine at The University of Chicago. He is also a part-time salaried consultant to United Therapeutics Corporation.
Correspondence to: Stuart Rich, MD, FCCP, Department of Medicine, University of Chicago Hospital, 5841 S Maryland Ave, MC 2016, Chicago, IL 60637; e-mail: srich{at}medicine.bsd.uchicago.edu
Idiopathic (or unexplained) pulmonary hypertension (IPAH) has been characterized as an illness that affects mostly young women. In the article by Shapiro et al1 from the Mayo Clinic in this issue of CHEST (see page 94), we now see IPAH as also affecting elderly men and women, with a seemingly worse prognosis. Is this a different illness or perhaps an overlooked subset of IPAH?
Actually, the description of IPAH in the elderly is not new. In the National Institutes of Health Registry on Primary Pulmonary Hypertension,2 which collected data from 1981 to 1986, 9% of the patients were > 60 years old. A recent report3 of a large registry of pulmonary hypertension (PH) in France reported that 9% of the patients were now > 70 years old. Thus, one can conclude that IPAH harbors no age discrimination. It affects people of all ages.
Perhaps more remarkable is the fact that 24% of the IPAH patients seen at the Mayo Clinic were > 65 years old, a much higher percentage than any previous series. In addition, they observed a trend, with 11% elderly from 1987 to 1995, increasing to 27% from 1996 to 2003. This is, however, consistent with the recent reporting of PH in the United States.
In 2005, the Centers for Disease Control and Prevention published a report4 on the prevalence of PH from 1980 until 2002 based on data from the National Vital Statistics System, National Hospital Discharge Survey, and Medicare hospital claims. Although these data can be imprecise, the size of the database makes the observed trends reliable. One of the striking observations was a threefold increase in reported hospitalizations with PH over the past 20 years, with 30% of the patients who died with PH from 2000 to 2002 being > 75 years old. In addition, the most common coexisting illness has shifted from respiratory disease (presumably COPD or interstitial lung disease) to heart failure.
An important finding of the Mayo Clinic experience was that the elderly patients who were referred with unexplained PH had higher pulmonary capillary wedge pressures than the younger patients. This suggests that many of the patients with a diagnosis of IPAH actually have PH secondary to left ventricular diastolic dysfunction. Although one might expect to be able to easily distinguish them from IPAH patients on the basis of an elevated wedge pressure, there are many patients with left ventricular diastolic dysfunction in whom the wedge pressure is normal at the time of cardiac catheterization.5 This could be a result of a low cardiac output state, technical difficulties in measuring an accurate wedge pressure, or improper balancing of the pressure transducer, and can cause uncertainty in making the correct diagnosis.
This is particularly important since the Mayo Clinic series suggested a worse prognosis of IPAH in the elderly. The reasons for that are not clear, but likely influenced in part by comorbid illnesses that are more common in elderly patients, as well as the possibility that the right ventricle does not adapt as well to chronic pressure overload in the elderly as it does in younger patients6. As these patients are too old to be considered for lung transplantation, medical therapies are their only option. In that regard, a word of caution is worth considering.
One cannot exclude the possibility that many of the elderly patients are being hospitalized for PH because they are being treated inappropriately with newer therapies that actually worsen rather than improve their condition. As the Centers for Disease Control and Prevention has documented, coexisting lung or heart disease is extremely common in these patients. There are both theoretical and real concerns that vasodilator therapies used for IPAH can worsen gas exchange and hypoxemia in patients with lung disease,7 and produce pulmonary edema in patients with heart failure8. We have unfortunately had several elderly patients referred to our institution in the recent years in whom this was the case. This then underscores what experts in this field have emphasized for years: all patients with pulmonary hypertension need a thorough evaluation to determine the cause, including cardiac catheterization,9 The drugs that have been approved for IPAH should always be administered with careful follow-up, especially in this age group. Equally important, therapy should be discontinued in patients who worsen, rather than assume that it is their PH that is getting worse. As suggested by Shapiro et al, we need to better understand why elderly patients with IPAH do poorly. After all, we may become these patients one day.
Footnotes
The author has no conflicts of interest to disclose.
References
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