What is the treatment for pulmonary hypertension?

Treatment of Pulmonary Hypertension with PAP 39mmHg

The treatment of pulmonary hypertension with a PAP of 39mmHg should follow a risk-stratified approach with combination therapy targeting multiple pathways as initial treatment for most patients, while reserving IV epoprostenol for high-risk patients with WHO Functional Class IV symptoms.

Risk Assessment and Classification

Before initiating treatment, comprehensive risk assessment is essential to guide therapy:

• Risk categories based on estimated 1-year mortality 1:

  • Low risk (<5%): WHO FC I-II, 6MWD >440m, No RV dysfunction
  • Intermediate risk (5-10%): WHO FC III, 6MWD 165-440m, Moderate RV dysfunction
  • High risk (>10%): WHO FC IV, 6MWD <165m, Severe RV dysfunction

• A PAP of 39mmHg confirms pulmonary hypertension (defined as mean PAP ≥25 mmHg) 2

• Further hemodynamic assessment is required to determine:

  • Pre-capillary vs. post-capillary PH (PAWP ≤15 mmHg vs. >15 mmHg)
  • Pulmonary vascular resistance (PVR >3 Wood units for PAH)

Treatment Algorithm Based on Risk Category

For Low to Intermediate Risk Patients (WHO FC I-III):

1. Initial Combination Therapy 2, 1:

   • Oral combination of endothelin receptor antagonist (ERA) plus PDE-5 inhibitor
   • Preferred combinations:
     • Ambrisentan + Tadalafil
     • Bosentan + Sildenafil

2. Monotherapy options when combination therapy is not appropriate:

   • PDE-5 inhibitors (Sildenafil) 3
   • Endothelin receptor antagonists (Bosentan, Ambrisentan)
   • Soluble guanylate cyclase stimulators (Riociguat) 2

For High-Risk Patients (WHO FC IV):

1. IV Epoprostenol as first-line therapy 1, 4

   • Improves exercise capacity and delays clinical worsening
   • Consider adding ERA and/or PDE-5 inhibitor

2. Alternative parenteral prostanoids:

   • IV treprostinil
   • Subcutaneous treprostinil

Supportive Therapies for All Patients

• Oxygen therapy for patients with arterial O₂ <60 mmHg 1
• Diuretics for right ventricular failure with fluid retention 1
• Anticoagulation for idiopathic PAH (consider risk-benefit in other forms) 1
• Supervised exercise rehabilitation 1
• Immunization against influenza and pneumococcal infection 1

Specific Considerations for Different PH Groups

For CTEPH (Group 4):

1. Pulmonary endarterectomy (PEA) is the treatment of choice 2, 1

   • Potentially curative with hemodynamic normalization
   • Patient selection based on:
     • Extent and location of organized thrombi
     • Degree of pulmonary hypertension
     • Comorbidities

2. For inoperable CTEPH or persistent/recurrent PH after PEA:

   • Riociguat (sGC stimulator) 2
   • Consider balloon pulmonary angioplasty (BPA) in expert centers 2

For PH due to Left Heart Disease (Group 2) or Lung Disease (Group 3):

• Focus on treating the underlying condition
• Avoid conventional vasodilators as they may impair gas exchange 1
• Oxygen therapy and diuretics as needed

Advanced Options for Refractory Cases

• Lung transplantation for patients with inadequate response to maximal medical therapy 1
• Balloon atrial septostomy as a palliative procedure or bridge to transplantation in select cases 1

Common Pitfalls to Avoid

• Using calcium channel blockers without vasoreactivity testing 1
• Excessive fluid administration worsening right heart failure 1
• Delayed escalation of therapy leading to poor outcomes 1
• Monotherapy for high-risk patients instead of combination therapy 1
• Failure to refer to specialized PH centers 1

Referral to Specialized Centers

All patients with PAH should be managed at specialized centers with expertise in pulmonary hypertension, with a multidisciplinary team including:

• Consultant physicians with dedicated PH clinical sessions
• Clinical nurse specialists with PH expertise
• Radiologists with expertise in PH imaging
• Cardiologists or PH physicians with expertise in echocardiography and right heart catheterization 1

Regular follow-up assessments every 3-6 months are essential to evaluate treatment response and consider therapy escalation if treatment goals are not met.