Ebstein Anomaly is a rare congenital heart disorder in which parts of the tricuspid valve are defective.
The tricuspid valve separates the right lower heart chamber (right ventricle) from the right upper heart chamber (right atrium).
In Ebstein Anomaly, the positioning of the tricuspid valve and how it functions to separate the two chambers is unusual.
Ebstein Anomaly is a malformation of the tricuspid valve and right ventricle typically featured by:
1. Adherence of the septal and posterior leaflets to the underlying myocardium.
2. Downward (apical) displacement of the functional annulus.
3. Dilation of the atrialized portion of the right ventricle, with various degrees of hypertrophy and thinning of the wall.
4. Redundancy, fenestrations and tethering of the anterior leaflet.
5. Dilation of the right atrioventricular junction.
In persons with Ebstein anomaly, the leaflets are positioned deeper into the right ventricle instead of the normal position.
The leaflets are often bigger than normal.
The defect most often induces the valve to work poorly, and blood may go the wrong way.
Instead of flowing out to the lungs, the blood returns back into the right atrium.
The backup of blood flow can result in heart enlargement and fluid buildup in the body.
There may be narrowing of the valve that leads into the lungs (pulmonary valve).
In many instances, patients also have atrial septal defect (a hole in the wall that separates the heart's two upper chambers) and blood flow across the hole cause oxygen-poor blood to go to the body.
This can produce cyanosis, a blue tint to the skin caused by oxygen-poor blood.
Ebstein anomaly happens as a baby forms in the womb.
Studies have shown both genetic and environmental risk factors:
The anomaly is more frequent in twins and in those with a family history of congenital heart disorder.
Environmental factors found in studies are maternal exposure to benzodiazepines (tranquillizers).
Maternal lithium therapy (for depression) can infrequently result in Ebstein anomaly in the baby.
Symptoms vary from mild to very severe.
Symptoms form soon after birth, and are bluish-colored lips and nails due to low blood oxygen levels.
In severe cases, the baby seems to be very sick and has difficulty breathing.
In mild cases, the involved person may be asymptomatic for many years.
Symptoms in older children may be:
2. Failure to grow
4. Rapid breathing
5. Shortness of breath
6. Very fast heartbeat
Other Symptoms are:
1. Cyanosis: frequent in children and often because of linked atrial right-to-left shunt and severe heart failure.
In adult life, cyanosis increasingly become worse.
2. Fatigue and dyspnea: because of right ventricular failure
3. Palpitations and sudden cardiac death: because of paroxysmal supra-ventricular tachycardia or fatal ventricular arrhythmias.
Heart sounds: the first heart sound is widely split with a loud tricuspid part.
The pan-systolic murmur of tricuspid regurgitation is optimally heard at the lower left parasternal area
Signs of right heart failure: ankle edema, hepatomegaly and ascites
1. Fetal life: diagnosed incidentally by echocardiography.
2. Neonatal life and infancy: manifests with cyanosis and severe heart failure
3. Adult life: right heart failure
Echocardiogram permits definitive diagnosis.
Medicines to help with heart failure
Oxygen and breathing support
Treatment of heart failure and arrhythmias
Tricuspid valve repair is favored over valve replacement.
Bioprosthetic valves are favored over mechanical prosthetic valves
Heart transplant is last resort.
TABLE OF CONTENT
Chapter 1 Ebstein Anomaly
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Atrial Septal Defect
Chapter 8 Congenital Heart Diseases
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About the Author
Medical doctor since 1972. Started Kee Clinic in 1974 at 15 Holland Dr #03-102, relocated to 36 Holland Dr #01-10 in 2009. Did my M.Sc (Health Management ) in 1991 and Ph.D (Healthcare Administration) in 1993. Dr Kenneth Kee is still working as a family doctor at the age of 65. However he has reduced his consultation hours to 3 hours in the morning and 2 hours in the afternoon. He first started writing free blogs on medical conditions seen in the clinic in 2007 on http://kennethkee.blogspot.com. His purpose in writing these simple guides was for the health education of his patients which is also his dissertation for his Ph.D (Healthcare Administration). He then wrote an autobiolographical account of his journey as a medical student to family doctor on his other blog afamilydoctorstale.blogspot.com. This autobiolographical account “A Family Doctor’s Tale” was combined with his early “A Simple Guide to Medical Conditions” into a new Wordpress Blog “A Family Doctor’s Tale” on http://ken-med.com. From which many free articles from the blog was taken and put together into 550 amazon kindle books and some into Smashwords.com eBooks. He apologized for typos and spelling mistakes in his earlier books. He will endeavor to improve the writing in futures. Some people have complained that the simple guides are too simple. For their information they are made simple in order to educate the patients. The later books go into more details of medical conditions. The first chapter of all my ebooks is always taken from my blog A Simple Guide to Medical Conditions which was started in 2007 as a simple educational help to my patients on my first blog http://kennethkee.blogspot.com. The medical condition was described simply and direct to the point. Because the simple guide as taken from the blog was described as too simple, I have increased the other chapters to include more detailed description of the illness, symptoms, diagnosis and treatment. As a result there are the complaints by some readers of constant repetitions of the same contents but in detail and fairly up to date. He has published 550 eBooks on various subjects on health, 1 autobiography of his medical journey, another on the autobiography of a Cancer survivor, 2 children stories and one how to study for his nephew and grand-daughter. The purpose of these simple guides is to educate patient on health conditions and not meant as textbooks. He does not do any night duty since 2000 ever since Dr Tan had his second stroke. His clinic is now relocated to the Bouna Vista Community Centre. The 2 units of his original clinic are being demolished to make way for a new Shopping Mall. He is now doing some blogging and internet surfing (bulletin boards since the 1980's) starting with the Apple computer and going to PC. All the PC is upgraded by himself from XT to the present Pentium duo core. The present Intel i7 CPU is out of reach at the moment because the CPU is still expensive. He is also into DIY changing his own toilet cistern and other electric appliance. His hunger for knowledge has not abated and he is a lifelong learner. The children have all grown up and there are 2 grandchildren who are even more technically advanced than the grandfather where mobile phones are concerned. This book is taken from some of the many articles in his blog (now with 740 posts) A Family Doctor’s Tale. Dr Kee is the author of: "A Family Doctor's Tale" "Life Lessons Learned From The Study And Practice Of Medicine" "Case Notes From A Family Doctor"