From the Guidelines
Evaluation of Chronic Thromboembolic Pulmonary Hypertension (CTEPH) requires a comprehensive diagnostic approach beginning with a ventilation-perfusion (V/Q) scan, which serves as the initial screening test due to its high sensitivity, as recommended by the most recent guidelines 1.
Diagnostic Approach
The diagnostic approach for CTEPH involves several key steps:
- Initial screening with a ventilation-perfusion (V/Q) scan to identify mismatched perfusion defects
- Right heart catheterization to confirm pulmonary hypertension and measure hemodynamic parameters
- Pulmonary angiography, either conventional or CT pulmonary angiography (CTPA), to visualize the location and extent of thromboembolic obstruction
- Additional imaging with cardiac MRI to assess right ventricular function, and echocardiography to evaluate cardiac structure and function
- Pulmonary function tests and cardiopulmonary exercise testing to provide information about functional capacity and gas exchange
- Laboratory testing, including thrombophilia screening, particularly in younger patients without obvious risk factors
Importance of Multidisciplinary Team
A multidisciplinary CTEPH team should review all diagnostic information to determine operability for pulmonary endarterectomy, which remains the definitive treatment for eligible patients, as emphasized in the guidelines 1. This systematic evaluation is crucial because CTEPH is potentially curable with appropriate surgical intervention, unlike other forms of pulmonary hypertension, making accurate diagnosis vital for optimal patient outcomes.
Recent Guidelines
The most recent guidelines from 2020 1 highlight the importance of early diagnosis and the challenges associated with diagnosing CTEPH, with a median time of 14 months between symptom onset and diagnosis in expert centers. The guidelines also emphasize the need for a comprehensive diagnostic approach, including V/Q scan, right heart catheterization, and pulmonary angiography, to confirm the diagnosis of CTEPH and determine operability for pulmonary endarterectomy.
From the Research
Evaluation of Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
To evaluate CTEPH, the following steps can be taken:
- Screening with ventilation/perfusion scan is recommended for patients with suspicion of pulmonary hypertension, especially if risk factors for CTEPH are present 2, 3, 4
- Transthoracic echocardiography can be used as an initial investigation, followed by right heart catheterization and pulmonary angiography as the gold standard for diagnosis 5
- Conventional pulmonary angiography with right heart catheterization is important in the final diagnosis of CTEPH 3
- CT scan imaging and other advanced imaging methods can also play a larger role in disease detection and confirmation 2
- Perfusion defects on V˙/Q˙ scintigraphy in the setting of pulmonary hypertension are suggestive of CTEPH, but pulmonary angiography and right heart catheterization are required for confirmation and treatment planning 2
Diagnostic Criteria
The diagnosis of CTEPH should be considered in all patients with suspicion of pulmonary hypertension, and the following criteria can be used:
- Presence of persistent organized thromboembolic obstruction of the pulmonary arteries from incompletely resolved acute pulmonary embolism 2
- Characteristic chronic thromboembolic lesions on imaging examinations (CT pulmonary angiography and/or conventional pulmonary angiography) 4
- Presence of PH and imaging examinations that reveal characteristic chronic thromboembolic lesions 4
Treatment Options
Treatment options for CTEPH include:
- Pulmonary endarterectomy, which remains the guideline recommended treatment and has the best chance of cure 3
- Medical therapy, which can be used for inoperable patients or those with residual disease post-PEA 3, 5
- Balloon pulmonary angioplasty, which is a viable option for inoperable patients or those with residual disease post-PEA 2, 3, 5, 4