From the Guidelines
Initial echo findings of pulmonary hypertension should be worked up with a comprehensive evaluation to confirm the diagnosis and determine the underlying cause, starting with a thorough history and physical examination, and including confirmatory right heart catheterization, as recommended by the 2015 ESC/ERS guidelines 1. The evaluation should begin with a thorough history and physical examination, focusing on symptoms like dyspnea, fatigue, and signs of right heart failure.
- Essential laboratory tests include:
- Complete blood count
- Comprehensive metabolic panel
- BNP or NT-proBNP
- HIV testing
- Autoimmune serologies (ANA, RF)
- Thyroid function tests
- Imaging studies should include:
- Chest X-ray
- High-resolution CT scan of the chest
- Ventilation-perfusion (V/Q) scan to evaluate for chronic thromboembolic disease
- Pulmonary function tests with DLCO and six-minute walk test help assess functional capacity and severity.
- Consider sleep study to rule out sleep-disordered breathing, and ECG to evaluate for right heart strain. This systematic approach allows classification of pulmonary hypertension into one of five WHO groups (pulmonary arterial hypertension, left heart disease, lung disease/hypoxia, chronic thromboembolic disease, or miscellaneous causes), which is crucial for determining appropriate treatment, as outlined in the 2015 ESC/ERS guidelines 1 and supported by the 2022 ACR Appropriateness Criteria 1. Early referral to a pulmonary hypertension specialist is recommended, as treatment options vary significantly based on the underlying etiology, and the 2017 ACR Appropriateness Criteria 1 and 2009 ACCF/AHA expert consensus document 1 provide additional guidance on the diagnostic approach.
From the Research
Initial Workup for Pulmonary Hypertension
The initial workup for pulmonary hypertension (PHTN) typically involves a combination of non-invasive and invasive tests. According to 2, routine tests in patients with symptoms and physical findings suggestive of PH include:
- Electrocardiography
- Chest radiography
- Pulmonary function tests
- Transthoracic echocardiography to estimate the probability of PH
Role of Echocardiography
Echocardiography plays a crucial role in the diagnosis and prognosis of PHTN. As stated in 3, echocardiography represents an accessible and feasible real-world tool for screening, differential diagnostic, follow-up assessments, and risk stratification in pulmonary hypertension. Additionally, 4 highlights the importance of echocardiographic parameters, such as:
- Calculation of pulmonary arterial systolic pressure (PASP) through the tricuspid regurgitation (TR)
- Right ventricular outflow track acceleration time (RVOT-AT)
- Right atrial area
- Pericardial effusion
- Tricuspid annular plane systolic excursion (TAPSE)
- TAPSE/PASP ratio
- Tricuspid annular systolic velocity (s')
Diagnostic Accuracy of Echocardiography
The diagnostic accuracy of echocardiography for PHTN has been evaluated in several studies. According to 5, the summary sensitivity and specificity for echocardiography for diagnosing pulmonary hypertension was 83% (95% CI 73 to 90) and 72% (95% CI 53 to 85), respectively. However, as noted in 2 and 5, right heart catheterization remains the gold standard for diagnosing and monitoring pulmonary hypertension due to the limitations of echocardiography.
Further Evaluation
In patients with suspected or confirmed PHTN, without confirmed left-sided heart or lung diseases, a ventilation-perfusion scan should be performed to exclude chronic thromboembolic PH (CTEPH) 2. Right-sided heart catheterization is essential for accurate diagnosis and classification of PHTN 2, 6. A simple non-invasive echocardiographic score can be useful in predicting the diagnosis of pulmonary hypertension at right heart catheterization and may be considered for the selection of patients who should undergo or could avoid right heart catheterization 6.