Treatment of Pulmonary Hypertension
The treatment of pulmonary hypertension requires a targeted approach based on the specific classification, with pulmonary arterial hypertension (PAH) patients benefiting from specialized vasodilator therapies while other forms require treatment of underlying causes. 1
Classification and Initial Assessment
- Pulmonary hypertension is defined as a mean pulmonary artery pressure >20 mmHg and is classified into 5 groups based on etiology, pathophysiology, and treatment approach 2, 3
- Right heart catheterization is essential to confirm the diagnosis of PAH (Group 1) and guide treatment decisions 1
- Vasoreactivity testing is recommended in patients with idiopathic PAH, heritable PAH, and drug-induced PAH to identify those who may respond to calcium channel blockers 1
- Risk assessment using clinical parameters, exercise capacity, biomarkers, and imaging is crucial to guide therapy selection and monitor treatment response 1
Treatment Based on PH Classification
Group 1: Pulmonary Arterial Hypertension (PAH)
For vasoreactive patients (5-10% of IPAH cases):
- High-dose calcium channel blockers are the first-line therapy 4
For non-vasoreactive patients with low or intermediate risk:
- Initial approved monotherapy is recommended with:
Initial oral combination therapy is recommended for treatment-naïve patients with low or intermediate risk 1, 3
- Combination of endothelin receptor antagonist and PDE-5 inhibitor has shown improved outcomes 3
For high-risk patients:
For patients with inadequate response to initial therapy:
Group 2: PH due to Left Heart Disease
- PAH-specific therapies are not recommended 1
- Treatment should focus on the underlying cardiac condition 4
Group 3: PH due to Lung Disease
Group 4: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
- Surgical pulmonary endarterectomy in deep hypothermia circulatory arrest is recommended for operable patients 1
- Riociguat is recommended for inoperable CTEPH or persistent/recurrent PH after surgery 1
- Balloon pulmonary angioplasty may be considered for technically inoperable patients 1
- Lifelong anticoagulation is recommended for all CTEPH patients 1
Supportive Care and General Measures
- Diuretics are recommended for fluid overload management with careful monitoring of electrolytes and renal function 9
- Oxygen supplementation to maintain saturation >90% 9
- Supervised exercise training is recommended for physically deconditioned patients 4, 9
- Immunization against influenza and pneumococcal infection 4
- Psychosocial support as part of comprehensive care 4
- Pregnancy should be avoided in PAH patients due to 30-50% mortality risk 1, 4
Monitoring and Follow-up
- Regular follow-up assessments every 3-6 months in stable patients 1
- Assessment should include clinical evaluation, exercise capacity testing, biomarkers, echocardiography, and in some cases, right heart catheterization 1
- Treatment goal is to achieve and maintain low-risk status (WHO FC I-II, good exercise capacity, preserved RV function) 1, 4
- Consider lung transplantation for patients with inadequate response to maximal medical therapy 9
Important Considerations and Pitfalls
- Avoid abrupt discontinuation of PAH-specific therapies as this can lead to clinical deterioration and death 10
- Angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and beta-blockers are not recommended in PAH unless required for comorbidities 4
- In acute decompensation, avoid excessive fluid administration as it may worsen right ventricular function 10
- Patients with PAH should be managed at specialized centers with expertise in pulmonary hypertension 1