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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
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    • 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
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    • Research, Life Expectancy & Prognosis for PH
  • Diagnosing & Monitoring PAH
    • Pulmonary Arterial Hypertension Diagnosis
    • How Am I Doing? Assessing Your PAH.
    • Blood Tests
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    • Six Minute Walk
    • Ventilation Perfusion Scan (VQ Scan)
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      • CTEPH: WHO Group 4 Pulmonary Hypertension
      • PTE (Pulmonary Thromboendarterectomy) Surgery
      • Adempas used to Treat PAH and CTEPH
  • PAH Treatment
    • Pulmonary Hypertension Centers
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    • 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

Update on Medical Management of Pulmonary Hypertension in Congenital Heart Disease (PAH-CHD)

February 29, 2016 By Dr. Jeremy Feldman

Update on PAH TreatmentsIn 1995 epoprostenol, a continuously infused prostanoid medication, was approved for the treatment of PAH. Although this medicine worked to decrease pulmonary artery pressures, there were many risks with this therapy in congenital heart disease (CHD) patients.  Since an intravenous catheter was required, there was a small but real risk of blood stream infection.  There was also a risk of small air bubbles from the catheter passing to the arteries in the brain and leading to strokes.  In 2002 a related medicine, treprostinil, was approved.  This medicine is given in a continuous form with a small plastic catheter placed under the skin.  By delivering the medicine under the skin, the risks of blood stream infection and stroke were removed.

Over the next 13 years, two inhaled therapies and two oral medications that are in the prostanoid family were approved by the Food and Drug Administration (FDA).  Despite the approved therapies, there are still no randomized trials with prostanoid medications in PAH due to congenital heart disease (PAH-CHD), but small studies hint that there is an improved quality of life and improved ability to work, and live independently with fewer symptoms.

In 2007 the only large trial specific to patients with PAH-CHD called BREATHE-5 showed that bosentan, an endothelin receptor antagonist, improved symptoms and exercise capacity. There are now 3 medicines like bosentan in use to treat PAH-CHD today.  Ambrisentan and macitentan are improved versions of bosentan in that they are both once daily and don’t have an associated increased risk of liver problems.  They also have fewer drug interactions than bosentan.

Several small studies using medicines like sildenafil and tadalafil have also shown an improvement in symptoms in PAH-CHD.  Patients with complex PAH-CHD are usually not eligible to take part in the largest studies with medications and as a result there is less data about the best treatment regimens.  The good news is that patients with PAH-CHD generally have a better prognosis than patients with other kinds of PAH.   Talk to your congenital cardiologist or pulmonary hypertension specialist to see if one of these medicines might work for you.

Filed Under: Pulmonary Hypertension Treatments

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