Treatment of Pulmonary Hypertension

Diagnostic Evaluation and Classification

The European Respiratory Society recommends that right heart catheterization is essential to confirm diagnosis, establish specific classification, determine severity, and guide therapy 4, 3
The European Respiratory Society suggests that 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, as recommended by the European Respiratory Society 2, 3

For vasoreactive patients, high-dose calcium channel blockers are the first-line therapy, as recommended by the European Respiratory Society 4, 2
For non-vasoreactive patients, therapy is guided by risk assessment, with low-risk patients receiving initial oral combination therapy, intermediate-risk patients receiving initial oral combination therapy, and high-risk patients receiving intravenous prostacyclin analogues, as recommended by the European Respiratory Society 2, 3, 1
Sequential combination therapy is recommended for patients with inadequate clinical response to initial therapy, as suggested by the European Heart Journal and the European Respiratory Journal 1, 5

Low-risk patients should receive treatment aimed at achieving and maintaining low-risk status, with a treatment goal of WHO FC I-II, 6MWD >440m, and preserved RV function, as recommended by the European Heart Journal 6, 2
Intermediate-risk patients should receive treatment aimed at reducing risk, with a focus on improving exercise capacity and reducing RV dysfunction, as suggested by the European Heart Journal 6, 2
High-risk patients should receive aggressive treatment, including intravenous prostacyclin analogues, to improve survival, as recommended by the European Respiratory Journal 1, 3

Diuretics are recommended for managing fluid overload, with careful monitoring of electrolytes and renal function, as suggested by the European Respiratory Society 2, 3
Oxygen supplementation should be used to maintain arterial oxygen saturations >90%, as recommended by the European Respiratory Society 2
Supervised exercise training should be considered for physically deconditioned PAH patients under medical therapy, as suggested by the European Respiratory Society 2

Regular follow-up assessments every 3-6 months in stable patients are recommended, with a focus on achieving and maintaining low-risk status, as suggested by the European Heart Journal and the European Respiratory Society 6, 2
Treatment goal is to achieve and maintain low-risk status, with a focus on improving exercise capacity, reducing RV dysfunction, and improving quality of life, as recommended by the European Heart Journal 6, 2

Lung transplantation should be considered for patients with inadequate response to maximal medical therapy, as recommended by the European Heart Journal 5, 4
Hospitalization in intensive care unit is recommended for PH patients with high heart rate, low blood pressure, low urine output, and rising lactate levels, as suggested by the European Heart Journal 5
Balloon atrial septostomy may be considered after failure of maximal medical therapy, as recommended by the European Heart Journal 5

Patients with PAH should be managed at specialized centers with expertise in pulmonary hypertension, as recommended by the European Respiratory Society 4, 3
Angiotensin-converting enzyme inhibitors, angiotensin-2 receptor antagonists, and beta-blockers are not recommended in PAH patients unless required for comorbidities, as suggested by the European Respiratory Society 2