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Pulmonary Hypertension RN

  • What is Pulmonary Hypertension?
    • What is Pulmonary Hypertension?
    • Pulmonary Hypertension Symptoms
    • Types of Pulmonary Hypertension
    • Causes of Pulmonary Hypertension
    • PH vs. PAH
    • Diastolic Heart Failure vs. PAH
    • WHO Group 3 Pulmonary Hypertension, IPF & COPD
    • Pulmonary Veno-Occlusive Disease
    • Hereditary Hemorrhagic Telangiectasia (HHT)
      • Hereditary Hemorrhagic Telangiectasia (HHT) Treatment
    • Functional Classification of Pulmonary Hypertension
    • Congenital Heart Disease
      • Eisenmenger’s Syndrome
      • Update on Management of PAH-CHD
      • Congenital Heart Disease & Sleep Apnea
      • Complex Congenital Heart Disease
      • Atrial Septal Defect and Pulmonary Arterial Hypertension
    • Pediatric Pulmonary Arterial Hypertension
    • Sickle Cell and Pulmonary Hypertension
    • Stimulant Associated Pulmonary Arterial Hypertension
    • Porto-Pulmonary Syndrome
    • Research, Life Expectancy & Prognosis for PH
  • Diagnosing & Monitoring PAH
    • Pulmonary Arterial Hypertension Diagnosis
    • How Am I Doing? Assessing Your PAH.
    • Blood Tests
    • Cardiopulmonary Exercise Test (CPET)
    • CT (Computed Tomography) Scan
    • Echocardiogram: An Overview
    • Echocardiogram: A Detailed Look
    • Pulmonary Function Tests (PFTs)
    • Right Heart Catheterization
    • Six Minute Walk
    • Ventilation Perfusion Scan (VQ Scan)
    • CTEPH
      • CTEPH: WHO Group 4 Pulmonary Hypertension
      • PTE (Pulmonary Thromboendarterectomy) Surgery
      • Adempas used to Treat PAH and CTEPH
  • PAH Treatment
    • Pulmonary Hypertension Centers
    • Pulmonary Hypertension Treatments
    • Continuously Infused Therapies Used to Treat PH
    • Prostanoids for Treatment of PH
    • Adcirca and Revatio Used to Treat Pulmonary Arterial Hypertension (PAH)
    • Adempas used to Treat PAH and CTEPH
    • Tracleer, Letairis, and Opsumit Treatments
    • Oxygen Therapy for Pulmonary Hypertension
    • Salt and Water Restrictions
    • Exercise & PAH
    • Diuretics
    • Blood Thinners
    • Getting the Most Out of PAH Therapy
      • Letairis
      • Opsumit
      • Sildenafil (Revatio)
  • Research
  • FAQ’s
    • Implanted Remodulin Pump, Right Heart Catheterization, PAH & Pregnancy
    • Oral Treatments, Care Centers & Hole in Heart
    • FAQ’s: Altitude, Cold & Allergy Medications
    • FAQ’s: Psoriasis, Idiopathic PAH, Contraceptives & PAH
    • FAQ’s: Causes of Pulmonary Hypertension, Symptom Frequency & Care Centers
    • FAQ: PAH Experts, Pregnancy, Mild PAH & Vacationing
    • FAQ’s: CTEPH, Blood Clots, Corrective Surgery & PAH Care Centers
    • FAQ’s: VQ Scans & PAH Curable?
    • FAQ’s: Mild Pulmonary Hypertension?
    • FAQ’s: Boating, Swimming & Eating with PAH
    • FAQ’s: Borderline Pulmonary Hypertension, Hand Swelling, Children with PAH, Costs

Tracleer, Letairis, and Opsumit Treatments

Tracleer, Letairis, and Opsumit ERAs used to treat pulmonary arterial hypertension

Endothelin Receptor Antagonists (ERAs) are a class of drugs that block the receptors in the blood vessels from responding to endothelin (a vasoconstrictor). There are currently three ERAs approved by the FDA for treatment of pulmonary arterial hypertension, Tracleer (Bosentan), Letairis (Ambresentan), and Opsumit (Macitentan).

Tracleer (Bosentan) an oral treatment for PH

Tracleer was the first ERA approved by the FDA for the treatment of pulmonary arterial hypertension. It is available in tablet form and is taken twice a day. A reduced dose is taken for the first month of treatment and if tolerated the patient is then stepped up to the effective dose. Monthly blood tests are required to check for abnormalities in liver function and pregnancy. Women of child bearing potential should not become pregnant while taking this medication as it can cause birth defects. All patients must complete the Tracleer Enrollment Form.  Tracleer is available only through specialty pharmacies and must be ordered monthly.

Tracleer, like all medications used to treat pulmonary hypertension, has side effects. It is important to communicate any side effects being experienced to the physician as there are medications available to alleviate many of the symptoms. Fluid retention or edema is the most common side effect reported while taking Tracleer. Tracleer is an expensive medication but there are assistance programs available so don’t be shy to call the nurse coordinator and let them know your copay is high. You should not stop taking this medication without speaking to your health care professional. Each patient should speak with their physician about the risk, benefits, and alternatives before initiating this therapy.

Letairis (Ambrisentan) an oral treatment for PH

Letairis is an ERA approved by the FDA in June 2007. It is available in pill form and in two strengths, a 5mg tablet and a10mg tablet. It is taken once a day. Monthly blood tests were required by the FDA to check for liver function abnormalities and pregnancy when the drug first came to market. The FDA removed the requirement for monthly liver function tests in 2011. Monthly pregnancy tests are still required for all women of child bearing potential and two forms of birth control should be used while on this therapy. All patients must complete the Letairis Enrollment Form.  Letairis is only available through specialty pharmacies and must be ordered monthly.

Letairis, like all medications used to treat pulmonary hypertension, has side effects. The most common side effects reported while taking Letairis are nasal congestion and edema. It is important for the patient to communicate any side effects being experienced to their physician as there are medications available to alleviate many of these symptoms. Letairis is an expensive medication but there are assistance programs available so don’t be shy to call the physician and let them know your copay is high. You should not stop taking this medication without speaking to your health care professional. Each patient should speak with their physician about the risk, benefits, and alternatives before initiating this therapy.

Opsumit (Macitentan) an oral treatment for PH

Opsumit is an ERA approved by the FDA in October 2013.  It is available in pill form at a dose of 10mg.  It should be taken once daily.  It is a teratogen and women must use two methods of reliable birth control and complete monthly pregnancy tests while taking this medication. All patients must complete the Opsumit Enrollment Form prior to receiving the medication. Opsumit is available through specialty pharmacies.

The most common side effect reported while studying Opsumit was upper respiratory symptoms.  An increased incidence of anemia(decreased number of red blood cells) was also found.  Blood tests are required prior to starting this medication.  It is important for the patient to communicate any side effects being experienced to their physician as there are medications available to alleviate many of these symptoms. You should not stop taking this medication without speaking to your health care professional. Each patient should speak with their physician about the risk, benefits, and alternatives before initiating this therapy.

PAH Life Expectancy

PAH Research

Research, Life Expectancy & Prognosis for PH

Major Developments in Pulmonary Hypertension Affecting Prognosis 1.     Approval of

Where to Get Treatment?

CCCs for PAH

Pulmonary Hypertension Centers

Where to get treatment for pulmonary hypertension? Pulmonary arterial hypertension is a

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