Tuesday, November 27, 2012

Eisen-what??

The combination of my PH and congenital heart disease is known as Eisenmenger's syndrome. A phriend's mother posted all about Eisenmenger's today on Facebook, because her daughter also has Eisenmenger's syndrome. Since Jane let me, I'm just copying and pasting her post here!

Eisenmenger's is also considered a rare disease by NORD. I couldn't find out approximately how many people have the disorder though but it has to be under 200,000. 

Eisenmenger syndrome progresses over time as a result of a complication of a heart defect and the effects of high bl
ood pressure in the lungs. This hole in your heart causes blood to flow or circulate abnormally in your heart. Oxygen-carrying blood (red blood) from the left ventricle mixes with oxygen-poor blood (blue blood) from the right ventricle. This mixed blood then returns to your lungs instead of going to the rest of your body, causing high blood pressure in your lungs. High blood pressure in your lungs (pulmonary hypertension), when significant, can cause serious medical conditions.

Congenital heart defects of this type include:

patent ductus arteriosus (PDA) - a connection between the aorta and the pulmonary artery which allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs.

atrial septal defect (ASD) - an opening in the atrial septum, or dividing wall between the two upper chambers of the heart known as the right and left atria.

ventricular septal defect (VSD) - an opening in the ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles.

atrioventricular canal defect (AV canal) - a complex heart problem that involves several abnormalities of structures inside the heart, including an ASD, VSD, and improperly formed mitral and/or tricuspid valves.

Eisenmenger syndrome is a life-threatening condition requiring careful medical monitoring. Medications can improve symptoms and prognosis.

Symptoms of Eisenmenger's include:

cyanosis (pale blue or grayish skin due to decreased oxygen in the blood)
dyspnea on exertion (shortness of breath with activity)
shortness of breath at rest
fatigue
chest pain or chest tightness
heart palpitations ("skipping beats" or "racing")
headache
dizziness or syncope (fainting)
paresthesias (numbness and/or tingling of fingers and toes)
blurred vision

Complications of Eisenmenger's can be:

blood clots (e.g., deep vein thrombosis in extremities)
hemorrhage (bleeding)
stroke
brain abscesses
gout
kidney failure

Some individuals with Eisenmenger syndrome who have no other health complications may live into middle adulthood, and a few may survive into the fifth or sixth decade of life.

Some considerations related to Eisenmenger syndrome include, but are not limited to, the following:

Pregnancy is not recommended for females of child-bearing age with Eisenmenger syndrome. Pregnancy may pose a high risk of death for the mother as well as complications for the fetus due to effects of low oxygen levels in the blood.

Any anesthesia and surgery is considered high risk and should be carefully planned. Collaboration with a cardiac specialist is recommended.

Air travel and high altitude exposure requires adequate hydration (fluid intake) and supplemental oxygen to prevent complications.

Severe headache, dizziness, syncope (fainting), or changes in level of consciousness should be taken seriously and evaluated on an emergency basis.

Smoking and alcohol intake are not recommended.

Coughing should be controlled or prevented with a strong suppressant medication to prevent risk of pulmonary hemorrhage (bleeding from the lungs).

Flu shots are recommended annually, and pneumococcal vaccine should be received according to the physician’s recommendation.

Eisenmenger syndrome treatment is aimed at controlling the symptoms and managing the condition. Although there's no cure, medications may help you feel better, improve your quality of life and prevent serious complications. Doctors don't recommend surgery to repair the hole in your heart once Eisenmenger syndrome has developed, and any surgery may be life-threatening. It's important that you're treated by a doctor who has expertise in Eisenmenger syndrome.

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