What are the differences in treatment approaches for pulmonary hypertension (PH) versus hypertension (HTN)?

Treatment Approaches for Pulmonary Hypertension vs. Hypertension

Pulmonary hypertension (PH) and systemic hypertension (HTN) require fundamentally different treatment approaches, with PH demanding specialized care at expert centers and targeted therapies based on disease classification, while HTN typically requires standard antihypertensive medications.

Fundamental Differences

• Pulmonary hypertension is characterized by progressive increases in pulmonary vascular resistance leading to right ventricular failure and death if untreated 1
• PH requires referral to specialized centers due to the complexity of diagnostic evaluation and treatment options available 1
• Systemic hypertension (HTN) is typically managed in primary care settings with standard antihypertensive medications 2

Diagnostic Approach Differences

• PH diagnosis requires right heart catheterization showing mean pulmonary artery pressure ≥25 mmHg and normal pulmonary capillary wedge pressure ≤15 mmHg 1
• Acute vasoreactivity testing is essential in PH to identify patients who may respond to calcium channel blockers 1
• HTN diagnosis is based on office and/or ambulatory blood pressure measurements 2

Treatment Algorithm for Pulmonary Hypertension

Initial Assessment and Classification

• Risk stratification using WHO functional class, exercise capacity (6-minute walk test), echocardiographic findings, BNP/NT-proBNP levels, and hemodynamic parameters 1, 2
• Vasoreactivity testing during right heart catheterization to determine eligibility for calcium channel blocker therapy 1

Treatment Based on Vasoreactivity Status

• For vasoreactive patients with IPAH: High-dose calcium channel blockers (amlodipine, diltiazem, nifedipine) 1, 2
• For non-vasoreactive patients or those who fail CCB therapy: 1
  • WHO FC II-III with low/intermediate risk: Initial monotherapy or combination therapy with:
    • Phosphodiesterase-5 inhibitors (sildenafil, tadalafil) 3, 4
    • Endothelin receptor antagonists (bosentan, ambrisentan) 1, 2
    • Soluble guanylate cyclase stimulators (riociguat) 5
  • WHO FC IV or high risk: Initial combination therapy including IV prostacyclin analogs 1

Sequential Therapy for PH

• If inadequate response to initial therapy, add sequential combination therapy targeting different pathways 1
• Triple combination therapy should be attempted if inadequate response to dual therapy 1
• Consider lung transplantation after inadequate response to maximal combination therapy 1

Treatment Approach for Systemic Hypertension

• Standard antihypertensive medications including:
  • Diuretics
  • ACE inhibitors/ARBs
  • Calcium channel blockers
  • Beta-blockers
• Treatment based on comorbidities, age, and race 2
• No specialized testing required before initiating therapy 2

Supportive Care Differences

For Pulmonary Hypertension

• Supplemental oxygen for hypoxemia 1, 6
• Diuretics for fluid retention and right heart failure 6
• Anticoagulation should be considered for IPAH patients 2
• Avoidance of pregnancy, high altitude, and excessive physical activity 2, 6
• Vaccination against influenza and pneumococcal pneumonia 2, 7

For Systemic Hypertension

• Lifestyle modifications (weight loss, sodium restriction, physical activity) 2
• No specific activity restrictions unless uncontrolled 2

Monitoring and Follow-up

For Pulmonary Hypertension

• Regular assessment of exercise capacity (6-minute walk test) 1
• Echocardiographic evaluation of right ventricular function 1
• BNP/NT-proBNP monitoring 1
• Assessment for clinical worsening requiring therapy escalation 1, 6

For Systemic Hypertension

• Regular blood pressure monitoring 2
• Assessment of target organ damage 2

Special Considerations

For Pulmonary Hypertension

• Different PH classifications (Groups 1-5) require different treatment approaches 1, 5
• Group 1 (PAH) has specific approved therapies 1, 5
• Groups 2 (left heart disease) and 3 (lung disease) generally should not receive PAH-specific vasodilator therapy 5
• Group 4 (chronic thromboembolic PH) may benefit from pulmonary endarterectomy or riociguat 5

For Systemic Hypertension

• Treatment approach varies based on comorbidities (diabetes, chronic kidney disease, etc.) 2
• No classification-specific therapies as in PH 2

Prognosis and Outcomes

• Untreated PH leads to right ventricular failure and death, with poor prognosis indicators including clinical evidence of right ventricular failure, rapid symptom progression, syncope, WHO FC IV, short 6-minute walk distance, elevated BNP/NT-proBNP, pericardial effusion, and poor hemodynamics 1, 6
• Appropriate PH therapy has improved survival, particularly for vasoreactive patients on CCBs who may have 94% 5-year survival 1
• HTN has a more gradual impact on end-organ damage when untreated, with complications developing over years to decades 2

Common Pitfalls to Avoid

• Treating all PH groups with the same medications - PAH-specific therapies may be harmful in PH due to left heart disease or lung disease 5
• Failure to refer PH patients to specialized centers, delaying appropriate diagnosis and treatment 1
• Not performing vasoreactivity testing in PAH patients, missing potential responders to CCB therapy 1
• Confusing pulmonary hypertensive crisis management with standard hypertensive emergency protocols 6