Diagnosis and Management of Group 1 Pulmonary Arterial Hypertension in the UAE

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Group 1 pulmonary artery hypertension (PAH) is a debilitating disease characterized by progressively increased pulmonary vascular resistance
(PVR) and mean pulmonary arterial pressure (mPAP) due to obstructions in the pulmonary vasculature. Group 1 PAH is further subdivided into
idiopathic PAH, PAH associated with congenital heart disease (PAH-CHD), PAH associated with connective tissue disease (CTD-PAH),
portopulmonary hypertension (PoPH), and paediatric PAH, out of which the first 3 contribute to substantial disease burden. Evidence from
registries suggests that survival rates are better in PAH-CHD, followed by idiopathic PAH and PAH-CTD. The cause of idiopathic PAH is not
evident, but one-third of the patients belong to this group. CTD-PAH is the second most common cause of PAH, following idiopathic PAH. In all
cases, lung transplantation is generally considered the last option when medical therapies are not working.


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The current article discusses the various unmet needs in the UAE, including less effective monotherapy (exception considered in specific subsets of
PAH), late referral to the PH expert center, no systemic follow-up (in patients with PAH-CHD), no routine assessment, and underutilization of
screening and treatment algorithms.


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This article highlights the current approach to classification, screening, diagnosis, and management of each type of Group 1 PAH. This also
emphasizes the need for tailored therapy for each patient. Screening and treatment algorithms can help healthcare professionals in the early
detection and effective management of PAH, respectively.