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I read with interest the recently published Dana Point clinical classification of pulmonary arterial hypertension (PAH) in the Journalsupplement (1). Any globally relevant scheme of clinical classification cannot and should not lose sight of the prevalence of the disease state worldwide. Although the classification is not based on prevalence, and causes such as left ventricular systolic and diastolic dysfunction, in which PAH is not of primary importance, are kept in group 2 only, downgrading PAH associated with congenital heart disease (CHD) in their classification scheme to 1.4.4 seems unjustified. Although precise epidemiological data are not available for most causes of PAH, it is easy to understand the importance of PAH due to CHD on a global scale. In 2009, nearly 80% of the world's population lives in the less and least developed regions of the world. These regions also have a younger population profile, higher growth rates despite high infant mortality, and poor facilities for the management of CHD. Because the incidence of CHD is more or less uniform at 6 to 8 per 1,000 live births, these data easily would translate into a very high burden of PAH associated with CHD in the world, more than any cause listed in group 1.4. Most patients with CHD in the Western world undergo surgery early in life, and therefore this facet of CHD is less and less evident there, but the various aspects of the pathogenesis of PAH associated with CHD are also far from resolved. In view of the continued epidemiological, scientific, and pedagogical importance, PAH associated with CHD should be classified as 1.4.1 or even higher.
- American College of Cardiology Foundation