• Home
  • About Us
  • Blog
  • Contact Us

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
    • Pediatric Pulmonary Arterial Hypertension
    • Sickle Cell and Pulmonary Hypertension
    • Stimulant Associated Pulmonary Arterial Hypertension
    • 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
    • Uptravi is Approved for PAH Treatment
    • 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)
  • 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
  • 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

Eisenmenger’s Syndrome

February 9, 2016 By Dr. Tabitha Moe

Congenital Heart Disease & Eisenmenger’s Syndrome

Pulmonary hypertension and pregnancyFebruary 4th-7th is Congenital Heart Disease Awareness week.  The incidence of congenital heart disease today is 1:100 live births.  With today’s post , I wanted to discuss a complicated problem that adults who are born with congenital heart disease may suffer from for their entire lives: Eisenmenger’s Syndrome.

A normally structured heart has four chambers, two receiving chambers, one which receives deoxygenated blue blood from the veins and one which receives red oxygenated blood from the lungs. There is a wall which separates these two chambers that usually closes after birth.  Sometimes this wall does not completely form, leaving a hole. This hole can have different variations, and even different names, the most common is an atrial septal defect.  When the hole doesn’t close, and isn’t closed surgically, there are a number of changes that occur over the lifetime of a patient. The same kind of hole may also exist between the pumping chambers and is called a ventricular septal defect.

Initially, the hole allows an increased volume of oxygenated red blood to pass into the adjacent catching chamber or atrium.  Over time, that increased volume of blood slowly stretches the atrium larger and larger.  As the child ages, the atrium eventually reaches the limits it can stretch and blue blood begins to mix through the hole.  This process occurs slowly over time, and is called bidirectional as part of each heartbeat allows alternately red and then blue blood to mix between the chambers.

As time passes, the increased volume in the pulmonary arteries changes the lining of the arteries. They become stiff and hard and, as this process occurs, the pressure in the pulmonary arteries increases.  As this pressure increases, more and more of each heartbeat results in blue blood going into the red oxygenated catching chamber. This process may take decades, and patients may present much later in life in their 20’s, 30’s, 40’s or even 50’s.

The end result of this hole is constant blue deoxygenated blood passing into the red oxygenated chamber and then out to the body. On the outside these patients may have blue tinged-lips, blue fingernail beds, and oddly shaped fingernails called clubbing.  On the inside these patients may have other changes like increased blood volume or a high hemoglobin and hematocrit.  They are also at risk for iron deficiency and often need iron supplementation to make more red blood cells.  Gout is also a related process due to a build up of break down products from all the extra blood cells.

The good news is that there are now many medications that have been studied and found to be beneficial in patients with congenital heart disease related PAH.

Filed Under: Pulmonary Hypertension Diagnosis

Advertisement

PAH Life Expectancy

PAH Research

Research, Life Expectancy & Prognosis for PH

Major Developments in Pulmonary Hypertension Affecting Prognosis 1.     Approval of

Advertisement

Where to Get Treatment?

CCCs for PAH

Pulmonary Hypertension Centers

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

Popular Posts

PAH Expert talking with PH patient

Second Opinions

By Dr. Jeremy Feldman

I can’t tell you how frequently my patients ask if their doctor will be offended if the patient goes to a different

PAH treatment team

Who’s Who at your Pulmonary Hypertension Center

By Dr. Jeremy Feldman

The first visit at a PH center can be very overwhelming. Many patients receive the news that they have a serious life

The Doctor is In

  • Facebook
  • Google+
  • RSS
  • Twitter

Get Our Newsletter!

good news in pah

Recent Blog Posts

  • Pulmonary Hypertension Due To Valvular Heart Disease October 29, 2019
  • A New Approach to Treating Pulmonary Arterial Hypertension October 23, 2019
  • Scleroderma: Screening for Pulmonary Hypertension October 10, 2019

Categories

Archives

Copyright © 2019 Inspire Pulmonary Media, LLC