From the Guidelines
The workup for pulmonary hypertension involves a systematic approach to confirm the diagnosis and determine its underlying cause, with right heart catheterization being the gold standard for diagnosis, as recommended by the most recent guidelines 1.
Initial Evaluation
Initial evaluation should include a thorough history and physical examination, focusing on symptoms like dyspnea, fatigue, chest pain, and syncope.
- Basic testing includes:
- An electrocardiogram to detect right heart strain
- Chest X-ray to evaluate cardiac silhouette and lung fields
- Pulmonary function tests to assess for underlying lung disease
Diagnostic Testing
Echocardiography is essential as it estimates pulmonary artery pressure and evaluates right ventricular function.
- Laboratory tests should include:
- Complete blood count
- Comprehensive metabolic panel
- Thyroid function tests
- HIV testing
- Autoimmune markers
- BNP/NT-proBNP levels
Additional Testing
If pulmonary hypertension is suspected, additional testing may include:
- Ventilation-perfusion scanning to rule out chronic thromboembolic disease
- Chest CT to evaluate lung parenchyma
- Sleep studies to assess for sleep-disordered breathing
- Exercise testing to evaluate functional capacity This comprehensive approach helps classify pulmonary hypertension into one of five groups (WHO classification), which guides appropriate treatment strategies targeting the underlying cause and specific pathophysiologic mechanisms, as outlined in the latest guidelines 1.
Classification and Treatment
The classification of pulmonary hypertension into different groups (e.g., PAH, PH due to left heart disease, PH due to lung diseases and/or hypoxia) is crucial for guiding treatment decisions, with the goal of improving morbidity, mortality, and quality of life for patients with this condition 1.
From the Research
Diagnostic Approach
The workup for pulmonary hypertension involves a range of diagnostic tests to confirm the diagnosis and determine the underlying cause. The following steps are typically involved:
- Clinical history and physical examination to identify symptoms and signs suggestive of pulmonary hypertension 2, 3, 4
- Routine tests such as electrocardiography, chest radiography, and pulmonary function tests to rule out other conditions 2, 4
- Transthoracic echocardiography to estimate pulmonary artery pressure and assess right ventricular function 2, 5, 6
- Ventilation-perfusion scan to exclude chronic thromboembolic pulmonary hypertension (CTEPH) 2
- Right-sided heart catheterization to confirm the diagnosis and determine the severity of pulmonary hypertension 2, 3, 4
Imaging Techniques
Imaging techniques play a crucial role in the diagnosis and management of pulmonary hypertension. The following imaging modalities are commonly used:
- Transthoracic echocardiography to estimate pulmonary artery pressure, assess right ventricular function, and rule out secondary causes of pulmonary hypertension 5, 6
- High-resolution computed tomography of the lungs to assess lung morphology and rule out other conditions 4
- Contrast-enhanced spiral computed tomography of the lungs to assess pulmonary vasculature 4
- Pulmonary angiography to assess pulmonary vasculature and rule out CTEPH 4
Laboratory Tests
Laboratory tests are used to support the diagnosis and monitor the progression of pulmonary hypertension. The following laboratory tests are commonly used:
- Blood tests to assess liver function, renal function, and electrolyte levels 4
- Immunology tests to assess for underlying autoimmune disorders 4
- Serum markers such as brain natriuretic peptide (BNP) to assess disease severity and prognosis 4
Specialized Tests
Specialized tests are used to assess disease severity and monitor treatment response. The following specialized tests are commonly used: