Mortality From Primary Pulmonary Hypertension in the United States, 1979-1996

David E. Lilienfeld, MD, MPH, MSEngin and Lewis J. Rubin, MD, FCCP, From the Department of Medicine, School of Medicine (Dr. Rubin), University of California at San Diego, San Diego, CA; and the Drug Safety and Pharmacovigilance (Dr. Lilienfeld), Pharmaceutical Research Institute, Bristol-Myers Squibb Corporation, Princeton, NJ.

Disclosures

CHEST. 2000;117(3) 

In This Article

Results

The average annual age-adjusted primary pulmonary hypertension (ICD-9 rubric 416.0) death rates for white men, white women, black men, and black women are shown in Table 1. These rates are based on > 20,000 deaths. In general, the rates for blacks are the highest for both men and women. Among men, the age-adjusted rate for blacks is more than twice that of whites, and for women, three times.

The temporal trends in age-adjusted primary pulmonary hypertension in the United States from 1979 through 1996 for white men, white women, black men, and black women are shown in Figure 1. The overall mortality trend for all groups is an increase between 1979 and 1996. However, the patterns of that increase are not the same. For white men, the rate tended to rise slightly during the 1980s to two deaths per million persons before returning to zero in 1991, then rising to four deaths per million population in 1996. In contrast, for white women, the death rate remained at zero until 1984, after which it began its ascent to six deaths per million in 1996. For both black men and black women, mortality increased during the study period. Among black men, the rate rose from a low of one death per million persons in 1980 to a peak of nine deaths per million in 1995. Black women experienced a similar increase in mortality, with their death rate increasing from a low of four deaths per million at the beginning of the study to 13 deaths per million in 1996.

Figure 1. Annual age-adjusted primary pulmonary hypertension (ICD-9 rubric 416.0) mortality per one million population in the United States, from 1979 through 1996, by race and gender.

The average annual age-specific rates for white men, white women, black men, and black women in the United States during 1996 are shown in Figure 2. Some patterns are evident in these data. First, there is a sharp drop in the rates observed between birth and the end of infancy, regardless of the race or sex of the child. Among infants, considerable variation in mortality can be seen. The highest rate is observed among black boys, whose rate is almost twice that of the rate for black girls. The latter is almost the same as those for white boys and white girls. With increasing age, mortality fluctuates until the middle of the third decade of life. The mortality rate then begins to ascend with advancing age. The greatest rates at each age, after infancy, are observed for black women, and the lowest, white men. Age-specific mortality for both black men and white women is intermediate, although the rates for these two groups tend to be closer to those for white men than black women. After infancy, for each race, the rates for women are greater than those for men.

Figure 2. Annual age-specific primary pulmonary hypertension (ICD-9 rubric 416.0) mortality per one million population in the United States, 1996, by race and gender.

The changes in the annual age-specific primary pulmonary hypertension mortality rates in the United States between 1979 and 1996, by race and sex, are shown in Table 2. The increases among white boys, white girls, and black boys < 1 year in age were substantial and statistically significant at the p < 0.001 level. For white men, the increases in rates after 35 years of age were statistically significant; the rates themselves are at least doubled. The same observation is true for white women >= 45 years of age. Among black women, a similar pattern of statistically significant increases in rates among those >= 45 years of age is seen. Although some decreases in rates were observed, these changes were not statistically significant.

Our concerns about the possibility of misdiagnosis lead us to investigate the age-adjusted mortality patterns by race and sex within each of the five states with the greatest number of primary pulmonary hypertension deaths from 1992 through 1996. This period is the most recent 5-year period for which mortality data are available. Its use should minimize the effect of temporal changes in the frequency of the disease. The average annual age-adjusted primary pulmonary hypertension death rates from 1992 through 1996 for the United States, California, Florida, Georgia, Illinois, and New York among white men, white women, black men, and black women are shown in Table 3. The pattern of the rates within each of the five states closely parallels that for the United States. Indeed, the magnitude of the rates for the individual states within each demographic group mirrors that of the United States overall.

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