What is Pulmonary arterial hypertension (PAH)?
Pulmonary arterial hypertension (PAH) is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. In PAH, the small arteries in the lungs become narrowed, blocked, or damaged, leading to increased resistance to blood flow through the lungs.
This causes the right side of the heart to work harder to pump blood through the lungs, which can eventually lead to right-sided heart failure. PAH can cause symptoms such as shortness of breath, fatigue, chest pain, dizziness, fainting, and swollen ankles or legs.
Can PAH cause death?
Pulmonary arterial hypertension (PAH) can be a serious and life-threatening condition if left untreated or if not managed effectively. PAH causes high blood pressure in the arteries of the lungs, which can lead to strain on the right side of the heart as it works harder to pump blood through the lungs. Over time, this increased pressure and strain on the heart can result in right-sided heart failure, a condition where the heart is unable to pump effectively.
If PAH is not treated, or if it progresses despite treatment, it can lead to complications such as heart failure, arrhythmias (irregular heartbeats), blood clots in the lungs, and ultimately, death. Early diagnosis, appropriate treatment, and regular monitoring by healthcare providers are crucial in managing PAH and reducing the risk of complications.
Causes of pulmonary arterial hypertension (PAH)
1. Genetic mutations: In some cases, PAH may be linked to genetic mutations that affect the function of the cells lining the pulmonary arteries, leading to increased constriction and resistance to blood flow.
2. Connective tissue disorders: Conditions such as scleroderma, lupus, and rheumatoid arthritis can be associated with PAH.
3. HIV infection: People with HIV are at an increased risk of developing PAH.
4. Liver disease: PAH can occur in individuals with liver disease, particularly cirrhosis.
5. Congenital heart defects: Some individuals may develop PAH because of congenital heart defects that affect blood flow through the lungs.
6. Drug and toxin exposure: Certain drugs and toxins, such as methamphetamines and certain diet medications, can contribute to the development of PAH.
7. Other medical conditions: PAH can also be associated with conditions such as chronic obstructive pulmonary disease (COPD), sleep apnea, and pulmonary embolism.
Understanding how Adempas and Uptravi can treat PAH.
Adempas (generic riociguat)
What is Adempas?
Adempas is the brand name for the medication ingredient riociguat. It is a prescription drug used to treat two types of pulmonary hypertension: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).
Adempas works by relaxing and widening the blood vessels in the lungs, which helps to improve blood flow and reduce blood pressure in the pulmonary arteries. This can help to alleviate symptoms such as shortness of breath, fatigue, and exercise intolerance in individuals with these types of pulmonary hypertension.
Strengths and dosage
Adempas (riociguat) is available in tablet form and comes in different strengths for dosing. The typical starting dose of Adempas for pulmonary arterial hypertension (PAH) is 1 mg taken three times a day. (A lower starting dose of 0.5 mg three times a day may be used to minimize adverse effects). The dosage may be increased gradually based on individual response and tolerability, up to a maximum dose of 2.5 mg three times a day.
For chronic thromboembolic pulmonary hypertension (CTEPH), the recommended initial dose of Adempas is 1 mg taken three times a day, which may be increased gradually up to a maximum dose of 2.5 mg three times a day. (A lower starting dose of 0.5 mg three times a day may be used to minimize adverse effects).
Adempas 0.5mg, 1mg, 1.5mg, and 2mg and 2.5 mg strengths are available in tablet dosage form.
Indications and usage
Adempas (riociguat) is a soluble guanylate cyclase (sGC) stimulator indicated for the treatment of adults with:
• Persistent/recurrent Chronic Thromboembolic Pulmonary Hypertension (CTEPH) (WHO Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class.
• Pulmonary Arterial Hypertension (PAH), (WHO Group 1), to improve exercise capacity, improve WHO functional class and to delay clinical worsening.
Efficacy was shown in patients taking Adempas monotherapy or in combination with endothelin receptor antagonists or prostanoids. Studies establishing effectiveness included predominately patients with WHO functional class II–III and etiologies of idiopathic or heritable PAH (61%) or PAH associated with connective tissue diseases (25%).
Uptravi (Selexipag)
What is Uptravi?
Uptravi is the brand name for the medication ingredient selexipag. It is a prescription drug used to treat pulmonary arterial hypertension (PAH) in adults. Uptravi works by targeting a specific receptor called the prostacyclin receptor, which helps to dilate (widen) the blood vessels in the lungs and reduce the elevated blood pressure in the pulmonary arteries. This can help improve exercise capacity and symptoms in individuals with PAH.
Uptravi is typically used to reduce the risk of disease progression and hospitalization in patients with PAH. It is available in tablet form and is taken orally as directed by a healthcare provider. Uptravi is often prescribed as part of a comprehensive treatment plan for PAH, which may include other medications, lifestyle changes, and regular monitoring.
Strengths and dosage
Uptravi (selexipag) is available in tablet form and comes in different strengths for dosing. The typical starting dose of Uptravi for pulmonary arterial hypertension (PAH) is 200 micrograms (mcg) twice daily. The dosage may be increased gradually based on individual response and tolerability, up to a maximum dose of 1600 mcg twice daily.
Uptravi 200mcg, 400mcg, 600mcg, 800mcg, 1000mcg, 1200mcg, 1400mcg, and 1600mcg tablet dosage forms are available.
Indications and usage
Uptravi is a prostacyclin receptor agonist indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression and reduce the risk of hospitalization for PAH.
The effectiveness of UPTRAVI tablets was established in a long-term study in PAH patients with WHO Functional Class II–III symptoms. Patients had idiopathic and heritable PAH (58%), PAH associated with connective tissue disease (29%), PAH associated with congenital heart disease with repaired shunts (10%).
For inquiries about prescription medications, contact our team at Jason’s CanadaDrugstore.com by calling 1-800-226 3784 (CAN-DRUG) for assistance from our patient representatives or a licensed Canadian pharmacist.
This article provides medical information to help understand a condition or treatment plan but is not a diagnosis or treatment recommendation. Contact your doctor if you have concerns or symptoms. In case of emergency, call 911.
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