The genetic basis of pulmonary arterial hypertension

  title={The genetic basis of pulmonary arterial hypertension},
  author={Lijiang Ma and Wendy K Chung},
  journal={Human Genetics},
Pulmonary arterial hypertension (PAH) is a rare disease characterized by distinctive changes in pulmonary arterioles that lead to progressive elevation of pulmonary artery pressure, pulmonary vascular resistance, right ventricular failure, and a high mortality rate. The etiology of PAH is heterogeneous and incompletely understood. Based on clinical classification, WHO Group 1 PAH includes sporadic disease (idiopathic PAH), inherited PAH (heritable PAH), and association with certain medical… CONTINUE READING