What is the treatment for pulmonary hypertension?

Treatment of Pulmonary Hypertension

The treatment of pulmonary hypertension requires a classification-based approach, with PAH-specific therapies indicated for Group 1 PAH and select Group 4 patients, while other groups require treatment of their underlying conditions. 1

Classification and Diagnosis

Proper treatment begins with accurate classification into one of five WHO groups:

• 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 essential for confirming diagnosis (mean pulmonary artery pressure >20 mmHg) 1, 2.

Treatment Approach by Classification

Group 1 PAH Treatment

Pharmacological Therapy:

1. Initial Therapy:

   • For vasoreactive patients: Calcium channel blockers 1
   • For non-vasoreactive patients: Initial combination therapy with ambrisentan (endothelin receptor antagonist) and tadalafil (phosphodiesterase-5 inhibitor) is recommended for WHO Functional Class II-III patients 3, 1

2. Medication Classes:

   • Endothelin receptor antagonists: Bosentan, ambrisentan, macitentan
   • Phosphodiesterase-5 inhibitors: Sildenafil 4, tadalafil
   • Soluble guanylate cyclase stimulators: Riociguat
   • Prostacyclin pathway agents:
     • Epoprostenol (IV) 5
     • Treprostinil (IV, SC, inhaled, oral)
     • Iloprost (inhaled)
     • Selexipag (oral prostacyclin receptor agonist) 6

3. Escalation of Therapy:

   • If inadequate response to initial dual therapy, add a third class of PAH therapy 3
   • Consider triple combination therapy for patients with progressive disease 1

Group 2 PH (Left Heart Disease)

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

Group 3 PH (Lung Diseases)

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

Group 4 PH (CTEPH)

• Pulmonary endarterectomy is the treatment of choice
• Balloon pulmonary angioplasty for inoperable cases
• Riociguat for inoperable CTEPH 1

Surgical and Interventional Approaches

• Pulmonary thromboendarterectomy: Potential surgical cure for CTEPH 3, 7
• Lung transplantation: Consider after inadequate response to maximal medical therapy 3, 1
• Atrial septostomy: May be considered as a palliative procedure or bridge to transplantation in patients with refractory right heart failure 3, 7

Supportive Care

• Oxygen therapy: Maintain oxygen saturations >91%, especially during altitude exposure or air travel 3, 1
• Diuretics: For management of fluid overload
• Supervised exercise training: For deconditioned patients 1
• Anticoagulation: Consider in selected patients
• Psychosocial support: Essential component of comprehensive care 1

Special Considerations

• Pregnancy: Should be avoided due to high mortality risk 3, 1
• Surgery: Avoid non-essential surgery; when necessary, use epidural rather than general anesthesia 1
• Immunizations: Maintain immunizations against influenza and pneumococcal pneumonia 1
• Drug interactions: Riociguat is contraindicated with PDE-5 inhibitors 1
• Hepatic impairment: Dose adjustment required for some medications (e.g., selexipag) 6

Follow-up and Risk Assessment

• Regular follow-up every 3-6 months for stable patients 1
• Evaluate severity using multiple parameters:
  • Clinical assessment (WHO Functional Class)
  • Exercise capacity (6-minute walk distance)
  • Biochemical markers (BNP/NT-proBNP)
  • Echocardiographic and hemodynamic evaluation 1

Common Pitfalls to Avoid

• Delayed diagnosis due to non-specific symptoms
• Misclassification of PH group leading to inappropriate treatment
• Failure to refer to specialized centers
• Inadequate follow-up and risk assessment
• Underutilization of combination therapy
• Failure to recognize treatment failure early enough to escalate therapy 1

The management of pulmonary hypertension has evolved significantly with the development of multiple therapeutic options. Early referral to specialized centers with multidisciplinary teams is crucial for optimal outcomes 1, 8.