Pulmonary Hypertension (PHT)

PHT defined as PA mean pressure ≥25 mmHg at rest (in future ? ≥20 mmHg based on emerging data [ Lancet Respir Med 2018;6:168])
PA mean = CO × PVR + PA wedge pressure. Trans pulm gradient = PA mean – PA wedge.
Etiologies (Revised WHO Classification) (JACC 2013;62:D34)
Primary pulmonary arterial HTN (PAH) (group 1)
Precapillary PHT
PCWP ≤15 mmHg
↑ transpulm grad
↑ PVR
  • Idiopathic (IPAH): yearly incidence 1–2 per million; mean age of onset 36 y (♂ older than ♀); ♂:♀ = ~2:1, usually mild ↑ n PAP