What is the treatment for pulmonary hypertension?

Treatment of Pulmonary Hypertension

The treatment of pulmonary hypertension should be guided by classification type, risk assessment, and specialized care at centers with expertise in pulmonary hypertension management. 1, 2, 3

Diagnostic Evaluation and Classification

• Right heart catheterization is essential to confirm diagnosis, establish specific classification, determine severity, and guide therapy 4, 3
• Vasoreactivity testing is mandatory for patients with idiopathic PAH to identify potential responders to calcium channel blockers 4, 2
• Ventilation-perfusion scanning should be performed to rule out chronic thromboembolic pulmonary hypertension (CTEPH) 4
• Risk assessment using clinical parameters, exercise capacity, biomarkers, and imaging is crucial to guide therapy selection 2, 3

Treatment Based on PAH Classification

Group 1: Pulmonary Arterial Hypertension (PAH)

• For vasoreactive patients (approximately 5-10% of IPAH), high-dose calcium channel blockers are the first-line therapy 4, 2
• For non-vasoreactive patients, therapy is guided by risk assessment (low, intermediate, or high risk) 2, 3:
  • Low-risk patients: Initial oral combination therapy targeting multiple pathways 3
  • Intermediate-risk patients: Initial oral combination therapy 3
  • High-risk patients: Intravenous prostacyclin analogues (epoprostenol) as they improve survival 1, 3
• Sequential combination therapy is recommended for patients with inadequate clinical response to initial therapy 1
• Treprostinil is indicated for PAH to diminish symptoms associated with exercise in patients with NYHA Functional Class II-IV symptoms 5

Group 4: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

• Pulmonary endarterectomy is recommended for operable patients 1
• Balloon pulmonary angioplasty should be considered for technically non-operable patients or those with unacceptable surgical risk 1
• Riociguat is recommended for inoperable CTEPH or persistent/recurrent PH after surgery 1
• Lifelong anticoagulation is recommended for all CTEPH patients 1, 3

Treatment Based on Risk Stratification

• Low-risk patients (estimated 1-year mortality <5%): WHO FC I-II, 6MWD >440m, preserved RV function 1, 2
• Intermediate-risk patients (estimated 1-year mortality 5-10%): WHO FC III, moderately impaired exercise capacity, RV dysfunction 1, 2
• High-risk patients (estimated 1-year mortality >10%): WHO FC III-IV, progressive disease, severe RV dysfunction 1, 2

Supportive Care and General Measures

• Diuretics are recommended for managing fluid overload with careful monitoring of electrolytes and renal function 2, 3
• Oxygen supplementation should be used to maintain arterial oxygen saturations >90% 2
• Supervised exercise training should be considered for physically deconditioned PAH patients under medical therapy 2
• Immunization against influenza and pneumococcal infection is recommended for all PAH patients 2
• Pregnancy should be avoided in PAH patients due to 30-50% mortality risk 2, 3

Monitoring and Follow-up

• Regular follow-up assessments every 3-6 months in stable patients are recommended 1, 2
• Treatment goal is to achieve and maintain low-risk status (WHO FC I-II, 6MWD >440m, preserved RV function) 1, 2
• Patients should be evaluated with a panel of data derived from clinical assessment, exercise tests, biochemical markers, and echocardiographic and hemodynamic evaluations 1

Advanced Therapies

• Lung transplantation should be considered for patients with inadequate response to maximal medical therapy 1, 4
• Hospitalization in intensive care unit is recommended for PH patients with high heart rate (>110 beats/min), low blood pressure (systolic BP <90 mmHg), low urine output, and rising lactate levels 1
• Balloon atrial septostomy may be considered after failure of maximal medical therapy 1

Important Considerations and Pitfalls

• Patients with PAH should be managed at specialized centers with expertise in pulmonary hypertension 4, 3
• Avoid abrupt cessation of prostacyclin analogue therapy as this can lead to rebound pulmonary hypertension 5
• In patients with hepatic insufficiency, decrease the initial dose of treprostinil 5
• Angiotensin-converting enzyme inhibitors, angiotensin-2 receptor antagonists, and beta-blockers are not recommended in PAH patients unless required for comorbidities 2