Written by Stuart Rich, Sheila Glennis Haworth, Magdi Yacoub and Paul Hassoun
Pulmonary hypertension: the unaddressed global health burden
The history of pulmonary hypertension is unique among non-communicable diseases (NCDs). Although recognised for centuries, only the development of cardiac catheterisation in the 1950s allowed the diagnosis to be made clinically. The initial case series characterised it as a rare disease of young women that was progressive and fatal. After the European epidemic of pulmonary hypertension in 1965, attributed to the diet pill aminorex, WHO held a symposium on pulmonary hypertension in 1973, which created international awareness. The National Institutes of Health sponsored the first registry on primary pulmonary hypertension in 1980, providing information about sex (1-7 times more frequent in women), onset (mean age 36 years), and survival (mean 2.8 years from diagnosis). In 1992, another epidemic of pulmonary hypertension attributed to diet pills, this time fenfluramines, brought pulmonary hypertension to world attention again. A second WHO symposium on pulmonary hypertension in 1998, established the clinical classification of five groups of pulmonary hypertension, but it was largely designed to serve the needs of North America and Europe. Pulmonary hypertension as a disease of low-income and middle-income countries was never addressed.
Heightened awareness among physicians in North America and Europe has resulted in a dramatic rise in pulmonary hypertension diagnosis and the establishment of centres of excellence. A cohort study from Canada reported a 28% increase in prevalence from 1993–2012 to 127 cases per 100,000 population. A population-based study from the USA found that 20% of cohorts of older participants (72-96 years) had echocardiographic evidence of pulmonary hypertension, and that increasing pulmonary artery pressure was associated with increased all-cause mortality independent of both age and the presence of cardiopulmonary disease. The diagnosis of pulmonary hypertension carries a 7-times increase in mortality, regardless of cause.
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