Diagnosis and Management of Group 4 PH (Chronic Thromboembolic Pulmonary Hypertension (CTEPH)) in the UAE

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct and treatable form of pulmonary hypertension (PH). It occurs due to
persistent thromboembolic obstruction of pulmonary arteries, leading to an increase in pulmonary vascular resistance (PVR) and right ventricular
failure. The prognosis of CTEPH patients is poor if left untreated.


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The diagnosis of CTEPH is challenging and usually delayed due to non-specific CTEPH symptoms, asymptomatic patients or patient negligence in
highlighting specific symptoms, and lack of screening tools. A ventilation/perfusion (V/Q) scan can help detect the early stage of CTEPH, i.e.,
chronic thromboembolic disease (CTED). Computed tomography (CT) pulmonary angiography is used for determining vascular abnormalities and
assessing operability in CTEPH. In the absence of a perfusion defect, haemodynamic measurement while exercising during right heart
catheterisation (RHC) serves as an important diagnostic tool in patients with normal haemodynamic at rest.


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Once CTEPH is diagnosed, pulmonary endarterectomy (PEA) becomes the treatment of choice in operable cases. If residual pulmonary artery
hypertension (PAH) post-PEA occurs, medical therapy with or without percutaneous balloon pulmonary angioplasty (BPA) is given.
Anticoagulants (vitamin K antagonists), diuretics, and riociguat (guanylate cyclase–stimulator) are recommended medical therapies for inoperable
patients.


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It can be concluded that, with the correct diagnostic approach, prevention of misdiagnosis and early detection of CTEPH is possible. Proper
diagnosis offers the possibility of a cure for non-operable patients with PEA. The treatment algorithm emphasises the need for a multidisciplinary
team with an individualised approach to ensure an optimal treatment response.