What is the treatment for pulmonary hypertension?

Treatment for Pulmonary Hypertension

The treatment of pulmonary arterial hypertension (PAH) requires a comprehensive, multi-pathway pharmacological approach with combination therapy tailored to the patient's risk assessment, ideally managed at specialized pulmonary hypertension centers by a multidisciplinary team. 1

Risk Assessment and Classification

Before initiating treatment, it's crucial to:

1. Confirm diagnosis with right heart catheterization 1
2. Perform vasoreactivity testing in idiopathic, heritable, and drug-induced PAH 1
3. Assess disease severity using:
   • WHO functional class
   • Exercise capacity (6-minute walk distance)
   • Echocardiographic parameters
   • Biochemical markers
   • Hemodynamic evaluation 1

Treatment Algorithm

Step 1: General and Supportive Measures

• Avoid pregnancy (Class I recommendation) 1
• Immunization against influenza and pneumococcal infection (Class I) 1
• Psychosocial support (Class I) 1
• Supervised exercise training for deconditioned patients (Class IIa) 1
• Supplemental oxygen for patients with WHO-FC III/IV and arterial O₂ < 60 mmHg 1
• Diuretics for fluid overload
• Avoid excessive physical activity that causes distressing symptoms 1

Step 2: Initial Pharmacological Therapy

Based on risk assessment:

For Vasoreactive Patients

• High-dose calcium channel blockers (for the small subset who demonstrate vasoreactivity) 1

For Non-vasoreactive Patients with Low/Intermediate Risk

• Initial monotherapy with one of the following drug classes 1:

  • Endothelin receptor antagonists (bosentan, ambrisentan, macitentan)
  • Phosphodiesterase-5 inhibitors (sildenafil, tadalafil)
  • Soluble guanylate cyclase stimulator (riociguat)
  • Prostacyclin pathway agents (epoprostenol, treprostinil, selexipag)

• Initial oral combination therapy is recommended for treatment-naïve patients 1, particularly:

  • Ambrisentan plus tadalafil (shown to improve 6MWD) 1

Step 3: Sequential Combination Therapy

For patients with inadequate response to initial therapy:

• Add a second class of PAH medication if treatment goals are not met 1
• Consider adding a third class if response remains inadequate 1
• Prostacyclin analogs (epoprostenol, treprostinil) are typically added for severe disease or rapid progression 2, 3

Step 4: Advanced Therapies

For patients with progressive disease despite optimal medical therapy:

• Referral for lung transplantation evaluation
• Atrial septostomy in select cases

Special Considerations

Route of Administration

• Prostacyclin analogs: Available as IV (epoprostenol, treprostinil), subcutaneous (treprostinil), inhaled, or oral formulations 2, 3
• IV prostacyclins are indicated for severe disease (WHO FC III-IV) 3

Specific Patient Groups

• Chronic thromboembolic pulmonary hypertension (CTEPH):

  • Surgical pulmonary endarterectomy is the treatment of choice
  • Riociguat for inoperable cases 4

• PH due to left heart disease or lung disease:

  • PAH-specific therapies are not recommended 1, 4

Monitoring and Follow-up

• Regular assessments every 3-6 months in stable patients 1
• Treatment goal: Achieve and maintain low-risk status (WHO-FC II, 6MWD >440m) 1

Common Pitfalls to Avoid

1. Delayed referral to specialized PH centers
2. Misclassification of PH group (treatment differs significantly between groups)
3. Inappropriate use of PAH therapies in PH due to left heart or lung disease
4. Abrupt discontinuation of PAH therapies (can lead to clinical deterioration)
5. Monotherapy approach when combination therapy has shown superior outcomes 5, 6

The field of PAH treatment has evolved significantly, with combination therapy targeting multiple pathways now showing improved survival compared to monotherapy approaches 5. Early diagnosis, prompt initiation of appropriate therapy, and regular follow-up at specialized centers are essential for optimizing outcomes in this progressive disease.