Pulmonary arterial hypertension

Abstract

Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ⩾25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ⩾3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management.Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup "pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers", due to the specific prognostic and management of these patients, and a subgroup "PAH with overt features of venous/ capillaries ( pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement", due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.

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“…Pulmonary arterial hypertension (PAH) is the result of pulmonary vascular remodeling characterized by vascular smooth muscle cell and endothelial cell increased proliferation [118]. High resistance and low flow in pulmonary vasculature result from vascular stiffening and thickening.…”

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“…Pulmonary arterial hypertension (PAH) is a relatively rare condition, affecting approximately 10–52 people per million population, characterized by the narrowing of the pulmonary arteries [ 60 , 61 ]. Left untreated, pulmonary arterial hypertension leads to the buildup of pressure on the right side of the heart when blood vessels in the lungs are diseased, causing the arteries to become increasingly narrow with time, and right-sided heart failure, often resulting in premature death [ 62 , 63 ]. While the direct cause of pulmonary arterial hypertension is unknown, a set of factors including liver disease, HIV infection, intravenous drug use, autoimmune disorders, and others are suspected to be culpable.…”