High Blood Pressure in the lungs, medically termed as primary pulmonary hypertension (PPH), is a disease characterized by progressive blockage or obliteration of small and medium-sized pulmonary arteries. Pulmonary hypertension happens when the pressure in the blood vessels leading from the heart to the lungs is too high. As a result of pulmonary hypertension, the blood vessels to the lungs develop an increased amount of muscle in the wall of the blood vessels. (1)
A person with pulmonary hypertension can generally live for up to around five years, but life expectancy seems to be improving. It is attributed to novel ways to manage the disease to live even longer after being diagnosed.
Symptoms of Hypertension
Patients with pulmonary hypertension are often treated by cardiologists, pulmonologists or a combination of both. Non-specific symptoms of pulmonary hypertension include shortness of breath, exercise intolerance, fainting, palpitations, feeling lightheaded and chest discomfort, which can be fatal. (2)
Treatment of PAH
PAH (Pulmonary arterial hypertension) is treated with medicines belonging to one of the three classes of drugs: endothelin receptor antagonists, nitric oxide pathway drugs and prostanoid therapies. Bosentan is a generic name drug belonging to the drug class of endothelin receptor antagonists. Research trials have demonstrated that bosentan improves exercise capacity in patients with PPH and PAH associated with systemic sclerosis.
Bosentan was the first oral drug approved by the US Food and Drug Administration and the European Agency for the Evaluation of Medicinal Products to treat PPH and PAH associated with collagen vascular disease. It is available under the brand name of Tracleer, the FDA approval of which was received by the company Actelion Pharmeceuticals US, Inc. – one of the Janssen Pharmaceutical Companies of Johnson & Johnson. (3)
Bosentan controls the symptoms of PAH but does not cure it. It may take 1 to 2 months or longer to get the benefits of bosentan. The starting dose of Tracleer is 62.5 mg twice daily for four weeks and then increased to the maintenance dose of 125 mg twice daily. It is not recommended for pregnant or breastfeeding women. Some side effects of Tracleer may be flushing, stomach upset, fatigue, dizziness, headache, and swelling up of the peripheries. (4)
To conclude, clinical research studies show that a treatment regime that includes bosentan is safe and productive in the long-term management of PAH. It may be a slightly costly front line therapy compared with epoprostenol and conventional or palliative therapy. However, bosentan still ranks high in its efficacy as an orally active therapy for PAH.
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Shubham Pharmachem’s blog posts have been written with the information gathered from approved medical journals and websites online. Our research and technical teams strive to provide relevant information through such articles. We strongly advise readers to not consume or administer any medication without prior consultation with their doctor.