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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

Adempas used to Treat PAH and CTEPH

Adempas: a soluble guanylate cyclase stimulator used to treat PAH and chronic thromboembolic pulmonary hypertension

Soluble guanylate cyclase stimulators work by stimulating an enzyme inside cells called soluble guanylate cyclace.  By increasing the activity of this enzyme, there is an increase in the production of cyclic GMP, which in turn leads to relaxation of the pulmonary arteries and improvements in pulmonary hypertension.  The pathway by which soluble guanylate cyclase stimulators work is similar to the way in which PDEV inhibitors work but the action is at an earlier point in the cascade.  The FDA (Food and Drug Administration) recently approved the first soluble guanylate cyclase stimulator to treat pulmonary arterial hypertension.

Adempas (Riociguat) an oral treatment for PAH and CTEPH

Adempas is the first in its class of medications (soluble guanylate cyclase stimulators) approved by the FDA to treat PAH.   Two studies have been published recently in the New England Journal of Medicine that showed that Adempas improved exercise capacity and delayed clinical worsening in patients with PAH and chronic thromboembolic pulmonary hypertension.  The most common side effects were head ache, stomach symptoms, swelling of the legs and low blood pressure.  The medication is taken orally and the dose is gradually increased from 05.mg to a maximum of 2.5mg three times daily.  This medicine is a teratogen meaning it can cause harm to a developing fetus.  Women must use two methods of reliable birth control and have monthly pregnancy tests while taking the medication.  All patients must complete the Adempas Enrollment Form prior to receiving the medication.

Adempas is available through a small network of specialty pharmacies.  Nursing visits from a nurse with the specialty pharmacy are required.  It is important not to start this medication until the nurse from the specialty pharmacy has measured the patient’s blood pressure and instructed the patient to do so. It is important to take every dose of this medication.  If a patient misses multiple doses they should contact their physicians office as they may have to restart the drug at a lower dose.

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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