Abstract

Pulmonary hypertension (PH) is a progressive disease characterized by elevation of pulmonary arterial pressure over 25mmHg. PH has become a global issue associated with substantial morbidity and mortality despite the emergence of several treatment options in the last two decades.

The Emirates Thoracic Society, Emirates Rheumatology Society, and Emirates Cardiac Society took up the task of coming up with regional practical recommendations. The recommendations were formulated based on a careful consideration of scientific and medical knowledge and the evidence available at the time of publication. All endeavors were directed towards making the updated guideline handy for practicing clinicians in the region involved in the management of patients with PH.

This article highlights the updated guidelines in a reader-friendly format and highlights the recent updates made by the 6th World Symposium on Pulmonary Hypertension and the updated ESC ERS 2022 guidelines on PH with regards to classification. Group 1 sub-group has been further classified into “definitive” and “possible” with the inclusion of sub-group “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers” and “PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/ pulmonary capillary haemangiomatosis) involvement”. Group 2.1 was re-named “PH due to heart failure with preserved left ventricular ejection fraction” and Group 2.2 was re-named “PH due to heart failure with reduced left ventricular ejection fraction”. Group 5 has two major updates: (i) removing splenectomy and thyroid disorders, and (ii) the classification of LAM-associated PH together with other parenchymal lung diseases in group 3.

This article highlights the clinical classification updates and the gaps and challenges associated with genetic testing in the region.

Keywords: Pulmonary hypertension, Clinical classification, Mean pulmonary arterial pressure, Risk factors, Pulmonary arterial hypertension, Post-capillary, Pre-capillary, Genetics.