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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