Ebstein's Anomaly Resources
What Is A Cyanotic Heart Defect?
A cyanotic heart defect(also called right-to-left cardiac shunt and right-to-left circulatory shunt)is a group of congenital heart defects(CHD's),or defects prent at birth,that result in low blood oxygen levels.Cyanotic heart dia often caus the skin of the baby to look blue(this is caleld"cyanosis.")and is most noticible upon the lips, fingers,and toes,or upon exertion.
Certain cyanotic heart defects cau major problems immediately after birth,but some cyanotic heart defects may not be diagnod until adulthood.
What is Ebstein's Anomaly?
Ebstein's Anomaly is a rare congenital heart defect in which parts of the tricuspid valve of the heart are abnormal.To understand how Ebstein's Anomaly works,read about the normal role of the tricuspid valve.
Normal Functioning of the Tricuspid Valve:
The tricuspid valve is compod of three parts(leaflets or flaps)which open to allow blood to flow from the right atrium to the right ventricle as the heart relaxes.The tricuspid leaflets clo to prevent blood from eping from the right ventricle to the right atrium while the heart pumps.
Functioning of the Tricuspid Valve in Ebstein's Anomaly:
For people who have Ebstein's Anomaly,the leaflets of the tricuspid valve have developed in an unusually deep part of the right ventricle.The leaflets are often bigger than normal,as well.The defects of the tricuspid valve often cau the valve to work poorly-the blood may circulate improperly back up into the right atrium.
This backup of blood flow may lead to swelling of the heart and the build-up of fluid in the lungs or liver.Occasionally,the blood cannot leave the heart to enter the lungs-which caus the person with Ebstein's Anomaly to appear blue.
What Other Conditions Are Associated With Ebstein's Anomaly?
There are other heart conditions that are associated with Ebstein's anomaly.The most common conditions are:秦时明月结局
Wolff-Parkinson-White(WPW)Syndrome-an abnormal electrical pathway in the heart that can lead to rapid heart-rate and fainting spells.
Cardiac Arrhythmia-some people who have Ebstein's Anomaly have an abnormal heart rhythm called"Tachycardia"or a rapid heart rate.Tachyarrhythmias can lead to the heart working less effectively or cau fainting spells.
Atrial Septal Defect-approximately half of the people who have Ebstein's Anomaly also have a hole between the atria(the two upper chambers of the heart).This hole can allow the deoxygenated blood in the right atrium to mix with the oxygenated blood in the left atrium.This decreas the amount of oxygen available in the blood and can lead to cyanosis-or a bluish discoloration of the nails and lips.Having an ASD as well as Ebstein's Anomaly can increa the risks of blood clots to the brain,causing a stroke.
Read more about strokes.
What Caus Ebstein's Anomaly?
Most cas of Ebstein's Anomaly have no identifiable cau.The congenital heart defects happen early in embryonic development and no risk factors have been properly identified.
Genetic and environmental factors are thought to play a role in the development of Ebstein's Anomaly.
People with a family history of congenital heart defects may be more likely to develop Ebstein's Anomaly.
What Are The Symptoms of Ebstein's Anomaly?
Symptoms of Ebstein's Anomaly can range from very mild to very vere.Generally symptoms of Ebstein's Anomaly develop shortly after birth.
Symptoms of Ebstein's Anomaly in babies can include:
星空照片•Blueish-colored nails
•Bluish-colored lips
•Difficulty breathing
长风破浪会有时直挂云帆济沧海的意思•Heart murmur
鼻烟•Heart swelling
Symptoms of Ebstein's Anomaly in older children can include:
•Rapid heart-rate
•Shortness of breath
•Cough
•Failure to grow
•Fatigue
•Heart murmur
•Heart swelling
How is Ebstein's Anomaly Diagnod?
If an underlying cardiac problem is suspected by a doctor,the following tests may be performed.
Chest X-Ray-Can reveal an enlarged heart.
Echocardiogram-clearly shows the valves and chambers of the heart to asss for Ebstein's Anomaly.
Electrocardiogram(ECG)-helps to determine irregularities in the rhythm and structure of the heart.
Holter Monitoring-a portable ECG unit that can be ud to detect rhythm disturbances that occur at unpredictable times.The monitor is worn under the clothes as normal activities are performed for a couple of days.
Cardiac Catheterization-this is rarely ud to diagno Ebstein's Anomaly,but may be ud if other cardiac problems are suspected.
Treatment for Ebstein's Anomaly:
Treatment for Ebstein's Anomaly depends entirely upon the verity of the heart defect and symptoms.The goal is to reduce the symptoms and avoid future complications like heart failure and arrhythmias.
Regular Monitoring:
If no symptoms of abnormal heart rhythms are prent,a person with Ebstein's Anomoly may simply have to undergo frequent monitoring by a cardiologist.湿婆
Medications:
Medications may be prescribed to control heart rate and rhythm.The medications may include calcium channel blockers,beta blockers,digitalis and anti-arrhythmics.Diuretics and other medications may be needed if a person exhibits signs of heart failure.
Surgery:
If signs and symptoms of Ebstein's Anomaly begin to affect quality of life,heart function decreas,or heart begins to enlarge,surgical heart repair may be required.There are a number of procedures ud surgically to treat Ebstein's Anomaly.The include:
•Tricuspid Valve Repair-done when there's enough valve tissue to allow for repair.
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In this surgery,the size of the tricuspid valve is reduced so the existing valve leaflets can come together and function properly.
•Tricuspid Valve Replacement-if existing valve is unable to be repaired,it may be replaced by removal of the old valve and inrting a mechanical valve or specially-treated biological tissue(bioprosthetic)valve.Blood thinners will be required should a mechanical valve be ud.
•Maze Procedure-During valve repair or replacement,a doctor may perform a "maze procedure"whic
h helps to correct atrial tachyarrhythmias(rapid heart rate in the atria).During a Maze Procedure,a ries of incisions in the right atrium are made.
When the incisions heal,the scars create a barrier of tissues in the heart,causing it to beat at a normal rate.
•Closure of the Atrial Septal Defect-If an ASD is prent,the surgeon can fix the hole during the surgery for the valve.
•Radiofrequency Catheter Ablation-treats a fast heart rate or Wolff-Parkinson-White Syndrome by inrting catheters into the blood vesls of the inner heart.Electrodes at the catheter tips are heated with radio-frequency energy, which destroys a small part of the cardiac tissue while creating an electrical block to stop the arrhythmia.Repeated procedures may be needed as time goes by.
•Heart Transplant-in cas of vere Ebstein's Anomaly that lead to very poor heart function,a heart transplant may be required.
Complications of Ebstein's Anomaly:
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Generally,people with mild Ebstein's Anomaly have few complications.Severe leakage may lead to s
welling of the heart and liver,leading to congestive heart failure.
Read more about congestive heart failure.
Other Complications May Include:
•Heart arrhythmias-tachyarrhythmias,bradyarrhthmias and heart block
•Blood clots from the heart that travel to other parts of the body
•Brain Abscess
Special Situations That May Cau Complications:
•Being active-depending upon signs and symptoms of Ebstein's Anomaly,activity restriction may be a decision of the medical team.
•During pregnancy-pregnancy for someone who has Ebstein's Anomaly carries a certain amount of risks,as pregnancy adds additional strain on the heart and circulatory system.Be sure to discuss becoming pregnant with your doctor or healthcare team.
Prognosis for Ebstein's Anomaly:
自我解嘲什么意思Generally speaking,the earlier the symptoms of Ebstein's Anomaly appear,the more vere the dia.Some people with Ebstein's Anomaly have no symptoms;others worn over time.
Tips for Coping With Ebstein's Anomaly:
Be vigilant about medical care.Follow up with the cardiologist(or pediatric cardiologist) for regular evaluations even if your symptoms are emingly okay.
