From the Guidelines
Regular follow-up for pulmonary hypertension should occur every 3-6 months with a pulmonary hypertension specialist, with more frequent visits for severe or unstable disease. These appointments should include clinical assessment of symptoms, functional capacity testing (6-minute walk test), echocardiography to evaluate right ventricular function and pulmonary pressures, and blood tests including BNP/NT-proBNP, complete blood count, and comprehensive metabolic panel 1. Right heart catheterization may be needed periodically to directly measure pulmonary pressures and guide therapy adjustments.
Key Components of Follow-up
- Clinical assessment of symptoms and determination of functional class
- Functional capacity testing, such as 6-minute walk test
- Echocardiography to evaluate right ventricular function and pulmonary pressures
- Blood tests, including BNP/NT-proBNP, complete blood count, and comprehensive metabolic panel
- Right heart catheterization as needed
Medication Management
Medication management should be reviewed at each visit, with common therapies including:
- Phosphodiesterase-5 inhibitors (sildenafil 20mg three times daily or tadalafil 40mg once daily)
- Endothelin receptor antagonists (ambrisentan 5-10mg daily or bosentan 62.5-125mg twice daily)
- Prostacyclin analogs (epoprostenol, treprostinil)
- Soluble guanylate cyclase stimulators (riociguat) 1
Lifestyle Modifications
Patients should also be counseled on lifestyle modifications, including:
- Appropriate exercise
- Sodium restriction
- Fluid management
- Avoiding pregnancy
This comprehensive follow-up approach is essential because pulmonary hypertension is progressive, and early detection of worsening disease allows for timely therapy adjustments that can improve outcomes and quality of life 1.
From the Research
Follow-up Recommendations for Pulmonary Hypertension
- Regular assessment of N-terminal pro-brain natriuretic peptide (NT-proBNP) levels is recommended as it has been shown to be a prognostic parameter in patients with pulmonary hypertension 2, 3, 4, 5.
- NT-proBNP levels can be used to determine the clinical severity of disease and are independently associated with long-term mortality 4.
- Follow-up assessments should include hemodynamic, functional, and biochemical variables, such as 6-min walk distance, right atrial pressure, cardiac index, and mixed-venous oxygen saturation 3.
- Changes in these parameters after initiation of targeted therapy can provide important prognostic information and guide treatment decisions 3.
- The use of NT-proBNP thresholds, such as those outlined in the European Society of Cardiology/European Respiratory Society guidelines, can help identify patients at high risk of morbidity and mortality 5.
- Regular cardio-pulmonary exercise testing (CPET) can also provide valuable information on disease severity and functional capacity 6.
Key Parameters to Monitor
- NT-proBNP levels
- 6-min walk distance
- Right atrial pressure
- Cardiac index
- Mixed-venous oxygen saturation
- Hemodynamic variables
- Functional capacity (e.g. CPET)