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Pulmonary Hypertension RN

  • What is Pulmonary Hypertension?
    • What is Pulmonary Hypertension?
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    • Causes of Pulmonary Hypertension
    • PH vs. PAH
    • Diastolic Heart Failure vs. PAH
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      • 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
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  • 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)
    • 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
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    • Getting the Most Out of PAH Therapy
      • Letairis
      • Opsumit
      • Sildenafil (Revatio)
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  • 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

Living with Complex Congenital Heart Disease

June 20, 2016 By Dr. Jeremy Feldman

By Tabitha Moe and Jeremy Feldman

Internal anatomy of the pulmonary arteriesA normal heart has 4 chambers: 2 top chambers and 2 bottom chambers.  In some patients, only a single bottom chamber (ventricle) develops.  These patients with a single ventricle are the most complex form of congenital heart disease.

The first surgery to repair this defect occurred in 1968 and was published by Dr. Fontan in 1971.  The repair is named after this creative and courageous surgeon. Today there are thousands of adults worldwide who received this life-saving surgical procedure in childhood.  Patients who underwent the Fontan surgery do not have a heart chamber to pump blue deoxygenated blood from the body to the lungs.  The return of blood to the lungs relies on lung movement within the chest. As the lungs move through cycles filling with air, and emptying a negative pressure creates a vacuum within the chest pulling some blood flow up from the body into the lungs.  The lungs also have very low pressure.  This allows blood flow to flow downstream towards the lungs.  This surgical repair, the Fontan, increases the pressure in the veins of the legs and arms.

There is mounting evidence that suggests when we lower the pressure in the pulmonary arteries there is improved blood flow to the lungs as the resistance to blood flow decreases. There has been great interest in using Pulmonary Arterial Hypertension medications to lower the pressure within the pulmonary arteries to improve lung blood flow.  There is a growing experience using PAH medications successfully.  The most studied are the PDE5 inhibitors.  There is also limited experience with using endothelin receptor antagonists and even prostanoid medications.  It is a very exciting time to be able to offer medications to help patients with complex congenital heart disease.  Talk to your adult congenital cardiologist or pulmonary hypertension specialist to see if you might benefit.

Filed Under: Congenital Heart Disease

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