What are the guidelines for managing pulmonary hypertension?

Guidelines for Management of Pulmonary Hypertension

The management of pulmonary hypertension requires a comprehensive, risk-stratified approach with treatment tailored to the specific PH classification group, with referral to specialized centers being essential for optimal outcomes. 1

Classification and Diagnosis

• PH is defined as mean pulmonary artery pressure >20 mmHg measured by right heart catheterization 1

• Classification into 5 groups is essential for treatment planning:

  • Group 1: Pulmonary Arterial Hypertension (PAH)
  • Group 2: PH due to Left Heart Disease
  • Group 3: PH due to Lung Diseases
  • Group 4: Chronic Thromboembolic PH (CTEPH)
  • Group 5: PH with unclear/multifactorial mechanisms

• Right heart catheterization is mandatory to confirm diagnosis before initiating treatment 1

Risk Assessment and Follow-up

• Evaluate severity using multiple parameters 2:

  • Clinical assessment (WHO functional class)
  • Exercise capacity (6-minute walk distance)
  • Biochemical markers (BNP/NT-proBNP)
  • Echocardiographic and hemodynamic evaluation

• Regular follow-up every 3-6 months for stable patients 2

• Risk stratification into low, intermediate, or high-risk categories guides therapy decisions 2

Treatment Approach by PH Group

Group 1 (PAH) Treatment

1. Vasoreactivity testing is mandatory to identify patients who may respond to calcium channel blockers 2

2. For vasoreactive patients:

   • High-dose calcium channel blockers (nifedipine, diltiazem) 1
   • Close monitoring for sustained response

3. For non-vasoreactive patients:

   • Treatment based on risk assessment:
     • Low/intermediate risk: Initial monotherapy or upfront combination therapy
     • High risk: Upfront combination therapy including parenteral prostanoids

4. Approved PAH-specific therapies:

   • Endothelin receptor antagonists (bosentan, ambrisentan, macitentan)
   • Phosphodiesterase-5 inhibitors (sildenafil, tadalafil) 3
   • Soluble guanylate cyclase stimulators (riociguat)
   • Prostacyclin pathway agents (epoprostenol, treprostinil, iloprost, selexipag)

5. Combination therapy:

   • Sequential add-on therapy for inadequate response 4, 5
   • Upfront combination therapy for high-risk patients

Group 2 (PH due to Left Heart Disease)

• Focus on treating the underlying heart condition 1
• PAH-specific therapies are not recommended

Group 3 (PH due to Lung Diseases)

• Optimize treatment of the underlying lung disease 1
• Long-term oxygen therapy for hypoxemic patients
• PAH-specific drugs are generally not recommended

Group 4 (CTEPH)

• Pulmonary endarterectomy is the treatment of choice 1
• Balloon pulmonary angioplasty for inoperable cases
• Medical therapy with riociguat for inoperable CTEPH

General Measures for All PH Patients

• Avoid pregnancy (high mortality risk) 2
• Immunization against influenza and pneumococcal infection 2
• Psychosocial support 2
• Supervised exercise training for deconditioned patients 2
• In-flight oxygen for WHO FC III/IV patients 2
• Epidural rather than general anesthesia for elective surgery 2
• Avoid excessive physical activity that causes distressing symptoms 2

Supportive Therapy

• Diuretics for fluid overload and right heart failure
• Oxygen therapy for patients with hypoxemia
• Oral anticoagulation (primarily for IPAH, HPAH, and CTEPH)
• Iron supplementation for iron deficiency

Treatment Goals

• Achievement/maintenance of low-risk profile 2
• WHO FC I-II status
• 6MWD >440m
• Normal/near-normal RV function
• Low BNP/NT-proBNP levels

Common Pitfalls and Caveats

• Delayed diagnosis is common due to non-specific symptoms
• Misclassification of PH group can lead to inappropriate treatment
• Using PAH-specific therapies in Group 2 or 3 PH may worsen outcomes
• Failure to refer to specialized centers with expertise in PH management
• Inadequate follow-up and risk assessment
• Underutilization of combination therapy in appropriate patients
• Not recognizing treatment failure early enough to escalate therapy

The management of pulmonary hypertension has evolved significantly with improved understanding of pathophysiology and development of targeted therapies. Early diagnosis, proper classification, and referral to specialized centers remain crucial for optimal outcomes.