From the Guidelines
Pulmonary hypertension is diagnosed through a comprehensive approach that begins with clinical suspicion based on symptoms like shortness of breath, fatigue, chest pain, and syncope, with the gold standard for diagnosis being right heart catheterization, which directly measures pulmonary artery pressure, with pulmonary hypertension defined as a mean pulmonary artery pressure ≥20 mmHg at rest, as recommended by the most recent guidelines 1.
Diagnostic Approach
The diagnostic approach to pulmonary hypertension involves several steps, including:
- Initial evaluation with a thorough history and physical examination, looking for signs such as elevated jugular venous pressure, a loud P2 heart sound, or right ventricular heave
- Non-invasive diagnostic testing, including:
- Echocardiography to estimate pulmonary artery pressure and assess right heart function
- Electrocardiogram
- Chest X-ray
- Pulmonary function tests
- CT scans to evaluate for underlying lung disease
- Blood tests, including:
- BNP levels
- HIV testing
- Liver function tests
- Autoimmune markers to identify potential causes
Classification of Pulmonary Hypertension
Once pulmonary hypertension is confirmed, additional testing may be performed to classify the type of pulmonary hypertension (Groups 1-5) and determine appropriate treatment, as outlined in the 2015 ESC/ERS guidelines 1.
Importance of Early Diagnosis
Early diagnosis is crucial as treatment options vary significantly based on the underlying cause and classification of pulmonary hypertension, with the goal of improving morbidity, mortality, and quality of life for patients with this condition.
From the Research
Diagnostic Tests for Pulmonary Hypertension
- Electrocardiography: used to estimate the probability of pulmonary hypertension 2
- Chest radiography: used to diagnose pulmonary hypertension 2, 3, 4
- Pulmonary function tests: used to diagnose pulmonary hypertension 2, 3, 4, 5
- Transthoracic echocardiography: used to estimate the probability of pulmonary hypertension and to exclude any secondary causes of pulmonary hypertension 2, 3, 4, 5
- Ventilation-perfusion scan: used to exclude chronic thromboembolic pulmonary hypertension (CTEPH) 2, 3, 4
- Right-sided heart catheterization: essential for accurate diagnosis and classification of pulmonary hypertension 2, 3, 4, 5, 6
- Blood tests: used to identify underlying conditions that may be causing pulmonary hypertension 4, 5, 6
- Exercise capacity assessment: used to predict prognosis in patients with pulmonary hypertension 3
Classification of Pulmonary Hypertension
- Pulmonary arterial hypertension (PAH) 2, 3, 4, 5, 6
- PH due to left-sided heart disease 2, 4
- PH due to chronic lung disease 2, 4
- Chronic thromboembolic PH (CTEPH) 2, 3, 4
- PH with an unclear and/or multifactorial mechanisms 2, 4