Global Prevalence of Pulmonary Arterial Hypertension
Global Prevalence of Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension Overview
Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterized by vascular proliferation and remodeling of the small pulmonary arteries. In PAH, blood vessels in the lungs are narrowed, blocked, or destroyed. The damage slows blood flow through the lungs. This results in progressively increasing pulmonary vascular resistance, leading to an increase in pulmonary arterial pressure. The heart muscles work harder to pump blood through the lungs, eventually causing the heart muscles to become weak and fail. PAH can be idiopathic, heritable, or associated with different conditions such as connective tissue disease (CTD), congenital heart disease (CHD), human immunodeficiency virus (HIV) infection, and exposure to toxins or drugs.
The most common pathological features in all forms of PH are medial hypertrophy, local dilation, and intimal atheromas. PAH is specifically characterized by the presence of constrictive lesions, which include medial hypertrophy and intimal and adventitial thickening, and by complex lesions that include plexiform and dilation lesions, as well as arthritis. Historically, the pathology of PAH has been attributed mainly to endothelial dysfunction and vascular remodeling, although increasing evidence indicates that the disease is more of an inflammatory proliferative nature.
Over the years, three major biochemical pathways have been implicated in the development of therapeutic treatments for PAH. These approaches to treatment can be classified broadly into two types: those that inhibit vasoconstriction (narrowing of blood vessels) and those that enhance vasodilation (widening of blood vessels). PAH-specific treatments target elements of molecular pathways of the endothelin pathway, nitric oxide (NO) pathway, and prostacyclin pathway NO is a pulmonary vasodilator that acts via the cGMP pathway. Patients with PAH have low levels of circulating endogenous NO, and the resulting reduced levels of cGMP are thought to contribute to some of the pathophysiological processes of the disease.
Diagnosed Prevalent Cases and incident cases of Pulmonary Arterial Hypertension
China and India recorded the largest number of incident and diagnosed prevalent cases for PAH, with China accounting for 173,953 diagnosed prevalent cases and 26,940 incident cases and India accounting for 144,055 diagnosed prevalent cases and 22,245 incident cases in 2020. China and India reported YoY growth of 1.0%, 1.6% of diagnosed prevalent cases and 1.3%, 2.0% of incident cases, between 2019 and 2020.
The 2 countries are followed by Brazil, Japan and Germany having significantly fewer incident and diagnosed prevalent cases, compared to China and India, in 2020.
Key Players in the PAH Segment
United Therapeutics: United Therapeutics is a major player in the PAH disease space, with multiple PAH-specific drugs, and has continued to be committed to the treatment of the disease for a number of years.
GlaxoSmithKline: GlaxoSmithKline has been an experienced player in the PAH field. Flolan was the first therapy approved for PAH treatment across the major countries and due to its price advantage, can still be promoted strongly in price-conscious markets.
Actelion (a subsidiary of Johnson & Johnson): Actelion has championed PAH treatment for many years and is an experienced player in the market. Actelion was acquired by J&J on June 16, 2017 and has continued to grow its PAH portfolio.
COVID-19 Impact on the PAH market
The COVID-19 pandemic has altered routine standard-of-care practice and acute management, particularly for those patients with PAH, where PAH-specific treatments are used. The COVID-19 pandemic presents many challenges when caring for patients with pulmonary hypertension.
In a recently published case series from Washington state, it was suggested that 86% of critically ill COVID-19-infected patients had heart failure and chronic kidney disease as the most common underlying medical conditions. These conditions are commonly seen in PAH patients and increase their risk for developing severe disease. PAH is a chronic, progressively fatal condition and requires complicated medical management. PAH patients are very sensitive to changes in their cardiopulmonary status and the disease course may decline rapidly due to any disruption in treatment or development of additional cardiac or pulmonary pathology. Many PAH patients are on complicated regimens, including oral, inhaled, or continuous intravenous therapies, some of which may be difficult to deliver during an acute illness such as COVID-19.
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