Pharmacotherapy of Pulmonary Hypertension

  • Definition- Pulmonary Hypertension (PH) is defined as an increase in mean pulmonary arterial pressure (PAPm) ≥25 mmHg at rest as assessed by right heart catheterization (RHC).
  • Normal (PAPm) – 14 ± 3 mmHg
  • PAPm of 21 – 24 mmHg – Carefully followed

Classification – Based on Etiology

  • Group 1Pulmonary Arterial Hypertension Idiopathic Heritable à BMPR2 mutationDrugs and toxins induced Associated with: Connective tissue diseaseHIV Infection Portal hypertension Congenital heart disease Schistosomiasis
  • Group 2 PH Due to Left Heart Disease
  • Group 3Pulmonary Hypertension Due to Lung Disease and/or Hypoxia
  • Group 4Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
  • Group 5Pulmonary Hypertension with Unclear Multifactorial Mechanisms
  • PAH
    • Affects 15-50 people/million – Globally
    • Affects all ages; most prevalent in 4th & 5th decades of life
    • Higher prevalence in females than males (2-3:1 ratio)
  • PH due to Left Heart Diseasemost common cause of PH


  • Pulmonary vascular remodelling
  • Sustained pulmonary vasoconstriction
  • In- situ thrombosis
  • Pulmonary vascular wall stiffening

Pathophysiology & Targets

WHO Functional Classification of Pulmonary Hypertension

Four functional classes have been defined for PAH:

(I) no symptoms or functional limitation;

(II) slight limitation of physical activity;

(III) marked limitation of physical activity; and

(IV) symptoms with any activity or at rest.

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