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Pulmonary Arterial Hypertension as a Systemic Disease

James West


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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 01.04.2013

Presentation Type: Scientific Sessions

PH as Systemic Disease

Learning Objectives:

  • Identify systemic involvement in PAH patients
  • Recognize mechanisms by which BMPR2 mutation regulates steroid hormone trafficking
  • Describe alterations in energy metabolism caused by BMPR2 mutation
  • Discuss evidence that metabolic defects are causative in PAH

While pulmonary arterial hypertension (PAH) is usually thought of as a disease whose primary manifestations are only in the lung, recent research on both the heritable and idiopathic forms of group I PAH suggest that it has molecular and physiologic manifestations throughout the body. These include alterations in energy metabolism found in every tissue on a cellular level, alterations in how the right heart adapts to stress, and alterations in intracellular protein trafficking found in the systemic as well as pulmonary vasculature. In animal models, these changes predate development of elevated pulmonary pressures, and penetrance and severity of disease can be modulated by interventions against them, suggesting that they are cause, not effect.