JAMA Clinical Reviews

A Review of Pulmonary Arterial Hypertension

Apr 12, 2022
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INSIGHT

Pulmonary Hypertension Definition

  • Pulmonary hypertension is defined as mean pulmonary artery pressure > 20 mmHg.
  • Pulmonary arterial hypertension (PAH) is a subgroup with specific hemodynamic criteria including pulmonary vascular resistance >= 3 Wood units.
INSIGHT

PAH Pathophysiology

  • Vascular remodeling, including plexiform lesions and elastic lamina fragmentation, increases pulmonary vascular resistance.
  • This leads to higher pulmonary artery pressure and right heart strain, causing right heart failure.
INSIGHT

PAH Prevalence

  • PAH is uncommon, with varying prevalence depending on the registry.
  • US registries show an incidence of ~2 cases per million per year and prevalence ~10.6 per million adults.
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