Pulmonary Arterial Hypertension ( Ph )

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Pulmonary Arterial Hypertension (PAH) belong to group 1 in the classification of Pulmonary Hypertension (PH). It is a chronic progressive disease caused by narrowing of the minor pulmonary arteries due to vascular proliferation and remodeling.

Figure 1: Changes occur in blood vessels in PAH.
The estimated prevalence for PAH is 15-50 cases per million although it is a rare disease. The female to male ratio is 1.7:1 with higher prevalence between 30-40 years old. To date there are 52 cases per million population of Scotland diagnosed with PAH. (1)
The current treatments available for PAH are prostaglandin (PG), calcium channel blocker (CCB), Endothelin Receptor Antagonist (ERA), and Phosphodiesterase-5-inhibitors (PDE-5-I). The need for new drug therapy is due to the drawbacks of the current treatment as shown in the tables below. (3) This has led to the development of Opsumit (Macitentan), a new drug therapy for PAH.

Table 1: Drawbacks of using Calcium Channel Blocker (CCB) in PAH
Calcium Channel Blocker (Nifedipine, Diltiazem) The proportion of patient benefited from this drug is too little.(2)

Table 2: Drawbacks of using Prostaglandins (PGs) in PAH
Epoprostenol I.V Iloprost nebulized Treprostinil S.C
- Inconvenience administration through IV route which requires hospitalization
- Unpresentable body image
- Administration of the drug might be associated with high risk of infection (need to be given through continuous I.V infusion due to short half-life)

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