Treatment of Pulmonary Hypertension

TREATMENT OF PULMONARY HYPERTENSION


TREATMENT OF PULMONARY HYPERTENSION

A) TREATMENT OF IDIOPATHIC PULMONARY HYPERTENSION:

Management of specific cases:

1. First-line therapy:

  • If respond to intravenous vasodilators: Oral calcium channel blockers (including amlodipine, diltiazem & nifedipine).
  • If vasodilators are ineffective/irresponsive: Therapy depends on function.

2. Patient with WHO Class 2 symptoms:

  • Phosphodiesterase inhibitors:
    • Sildenafil or tadalafil.
  • Endothelin receptor blockers:
    • Bosentan or ambrisentan

3. Patient with WHO Class 3 symptoms:

  • Prostacyclin analogs:
    • Epoprostenol intravenously
    • Iloprost by inhalation
    • Beraprost or treprostinil subcutaneously.

4. Patients with WHO Class 4 symptoms:

  • Epoprostenol or iloprost – As sole agent therapies.
5. In newborns:
  • Nitric oxide (NO) – Only FDA-approved NO application.

6. Other conjugant therapies:

  • Long-term oral anticoagulation.
  • Supplemental oxygen – Particularly at night, to improve symptoms & helps reduce pulmonary pressure.
  • Diuretics – In right heart edema.
  • Pulmonary transplantation:
    • Viable option in selected centers.
    • Operative mortality is high (around 20–25%).

B) MANAGEMENT OF 1° PULMONARY HYPERTENSION:

1. Endothelin receptor antagonists:

  • Bosentan & sitaxentan – Non-specific antagonist of endothelin receptors, both ETA & ETB.
  • Phosphoramidon – Non-specific inhibitor of endothelin converting enzyme.

2. PGI2 analogs:

  • Particularly with vasodilator action preferred.
  • PGE2 & PGI2 – Vasodilators {Note: PGF2α and TXA2 – Vasoconstrictors}.
  • Drugs:
    • Epoprostenol – PGI2
    • Treprostinil – Longer acting PGI2 analog.

3. Soluble guanylate cyclase (sGC) stimulator:

  • Riociguat
  • Soluble guanylate cyclase (sGC), an enzyme in CVS & nitric oxide (NO) receptor.
  • Helps arteries relax increasing blood flow & decreases BP.
  • Specifically indicated for persistent/recurrent chronic thromboembolic pulmonary hypertension.

General precautions:

  • Women with significant pulmonary hypertension not advised for pregnancy.
  • Permanent birth control measures should be considered.

Future of therapy:

  • Possible use of angiogenesis inhibitors, growth factor inhibitors, endothelial stem cells or progenitor cells.

Exam Important

TREATMENT OF PULMONARY HYPERTENSION

  • First-line therapy for pulmonary hypertension treatment includes oral calcium channel blockers (including amlodipine, diltiazem & nifedipine), only if patients respond to intravenous vasodilators.
  • First-line therapy for pulmonary hypertension treatment mainly depends upon function & patient’s status if patients don’t respond to intravenous vasodilators.
  • Patient with WHO Class 2 symptoms are treated using phosphodiesterase inhibitors (Sildenafil/tadalafil) & Endothelin receptor blockers (Bosentan/ambrisentan).
  • Patient with WHO Class 3 symptoms are treated using prostacyclin analogs particularly epoprostenol intravenously, iloprost by inhalation & beraprost/treprostinil subcutaneously.
  • Patient with WHO Class 4 symptoms are treated with epoprostenol or iloprost, as sole agent therapies.
  • Pulmonary transplantation has high operative mortality (around 20–25%).
  • Bosentan & sitaxentan are useful in treating pulmonary hypertension, which is a non-specific antagonist of endothelin receptors, both ETA & ETB.
  • Phosphoramidon is a non-specific inhibitor of endothelin converting enzyme, which is useful for treating pulmonary hypertension.
  • PGI2 analogs, particularly with vasodilator action is preferred for treating pulmonary hypertension like epoprostenol & longer acting PGI2 analog Treprostinil.
  • Soluble guanylate cyclase (sGC) stimulator like Riociguat is specifically indicated for persistent/recurrent chronic thromboembolic pulmonary hypertension.
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