US Pharm. 2006;7:10-13.

Characterized by severe elevation in the mean pulmonary arterial pressure and pulmonary vascular resistance, pulmonary hypertension is a devastating chronic disease with a poor long-term prognosis. The early symptoms (e.g., difficulty in breathing, fatigue) and later manifestations (e.g., palpitations, fainting) are shared with other diseases. Hence, treatment may be delayed while physicians exclude other causes for these symptoms. Results from a national registry of patients with primary pulmonary hypertension indicated that the duration from onset of symptoms to death averaged 2.8 years.

In recent years, new treatments have become available for pulmonary hypertension, particularly for pulmonary arterial hypertension. The treatments, which include anticoagulants, calcium channel blockers, and prostacyclins, prolong survival and provide clinical improvement, but they can be expensive. Although effective management might be possible with early detection, most people are diagnosed with pulmonary hypertension in later stages of the disease, making treatment more difficult and less successful. Pulmonary hypertension is reported on hospital records and death certificates either as primary pulmonary hypertension or as pulmonary hypertension secondary to another underlying condition or disease. Pulmonary hypertension might be secondary to congenital heart disease, valvular heart disease, chronic thromboembolic disease, lung diseases, liver diseases, sleep-disordered breathing, hypoxemia, lupus, scleroderma, rheumatoid arthritis, vasculitis, or HIV infection. Limited national statistics are available regarding pulmonary hypertensive diseases. Although a rare condition, primary pulmonary hypertension as the reported cause of death has increased since 1979, and the number of all cases is likely to be higher than reported numbers because of difficulties in diagnosis.




Hospitalizations
During 1980 to 2002, the estimated number of hospitalized patients with pulmonary hypertension as the diagnosis tripled for the total U.S. population. Compared with estimated numbers of hospitalizations in 1980, the numbers in 2002 were two times higher among men and three times higher among women. Rates of hospitalization for pulmonary hypertension among women were higher than those in men, but only after 1995. In 2002, the hospitalization rate (per 100,000 population) for pulmonary hypertension as the diagnosis was 95.3 for women and 82.3 for men. The number of hospitalizations and hospitalization rates increased among all age-groups in the U.S. population. The greatest increase in hospitalization rates was among adults 75 years and older.

During 1980 to 2002, the greatest increase in hospitalizations for pulmonary hypertension occurred in men 85 years and older and in women 65 years and older. Because of major increases in hospitalizations among women 85 years and older during 2000 to 2002, women had higher hospitalization rates than men did in this older age-group.

During 1980 to 2002, trends in the most commonly reported principal diagnoses changed. From 1980 to 1984, the most commonly reported principal diagnoses among hospitalized patients with a diagnosis of pulmonary hypertension were chronic lower respiratory diseases (42%), followed by pulmonary hypertension (12.8%) and cardiovascular diseases (6.8%). By 2000 to 2002, heart failure (18.7%) was the most commonly reported principal diagnosis among hospitalized patients with a diagnosis of pulmonary hypertension; this was followed by chronic lower respiratory diseases (12.9%). During 2000 to 2002, pulmonary hypertension was the principal diagnosis in only 4.2% of hospitalizations involving a diagnosis of pulmonary hypertension. Declines in reporting pulmonary hypertension and chronic lower respiratory disease and the increase in reporting heart failure as the principal diagnosis in these cases were observed among all groups defined by sex and age.

Among those younger than 45 years who were hospitalized, the principal diagnosis was more likely to be pulmonary hypertension (26%) and congenital malformations (22.9%) during 1980 to 1984; however, by 2000 to 2002, these conditions had declined as the principal diagnoses among hospitalized patients with pulmonary hypertension as the listed diagnosis. Other cardiovascular diseases (29.8%), other respiratory diseases (9.8%), pulmonary hypertension (9.5%), and chronic lower respiratory diseases (7.1%) were the major principal diagnoses from 2000 to 2002. Among patients younger than 45 years who were hospitalized for pulmonary hypertension, influenza and pneumonia (4.6%), congenital malformations (4.3%), complications related to specific procedures (3.9%), cellulitis and abscesses (3.6%), and complications related to pregnancy and childbirth (3.2%) were principal diagnoses.

Medicare Claims
During 1990 to 2002, the annual number of hospitalizations among Medicare enrollees 65 years and older who had pulmonary hypertension as a diagnosis tripled from 55,516 to 187,205. Until 1999, men were more likely than women to be hospitalized for pulmonary hypertension. Increases in numbers of Medicare hospitalizations for pulmonary hypertension were observed among all groups defined by race and age. Hospitalization rates were higher for blacks than for whites. Hospitalization rates increased for all age-groups, but the rates were not the highest among adults ages 85 years and older until after 1995. By 2000 to 2002, hospitalization rates were higher among women ages 65 to 74 years and 75 to 84 years, and whites ages 85 years and older had higher hospitalization rates than did blacks in the same age-group.

Conclusion
The high proportion of pulmonary hypertension-related deaths and hospitalizations that occurred among adults 65 years and older suggests that as the proportion of older adults in the U.S. population increases, pulmonary hypertension might continue to be a more frequent diagnosis, particularly with concomitant chronic heart failure. Because the majority of patients with pulmonary hypertension are older adults, the burden of chronic disability and morbidity on the Medicare system and families will increase.

Prevention efforts, including broad-based public health efforts to increase awareness of pulmonary hypertension and foster appropriate diagnostic evaluation and timely treatment, should be considered. Although multiple predisposing factors and associated conditions have been identified for pulmonary hypertension, the CDC believes that the causal roles and strengths of association have not been well established. Thus, it is not possible to establish preventive measures regarding risk factor reduction.

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