Pulmonary Arterial Hypertension In the Modern Era: The Intersection of Genotype and Phenotype

The Journal of Heart and Lung Transplantation
Pulmonary Arterial Hypertension In the Modern Era: The Intersection of Genotype and Phenotype
Pulmonary arterial hypertension (PAH) is a severe cardiopulmonary disease that is characterized by increased vascular resistance and early progression to right heart failure clinically, which occurs due to plexigenic, hypertrophic, and fibrotic effacement of distal pulmonary arterioles without systemic vascular involvement.1 There are several features that distinguish PAH from many other vascular disorders. First, the prevalence of PAH is ∼20 per 1,000,000 individuals, and, thus, it is classified as rare.
Source: The Journal of Heart and Lung Transplantation

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