Ebstein's Anomaly

[SUMAN-SAJAN]

EBSTEIN'S ANOMALY
Ebstein's anomaly also known as tricuspid regurgitation is a rare cardiac anomaly and constitute about less than 0.1% of all cardiac anomalies.
The disease is named after Wilhelm Ebstein- a German physician who, in 1886, first describes this disease.
Causes:
congenital (idiopathic)
maternal ingestion of lithium during first trimester
maternal benzodiazepine use
Pathophysiology
The basic abnormality in ebstein's anomaly is abnormal tricuspid valve development.
Tricuspid valve develops from two sources:
1. endocardial cushion
2. muscles of ventricle
If the leaflets of tricuspid valve develop aberrantly then ebstein's anomaly ensues.
The posterior and septal leaflets of valve do not ascent (anterior leaflet being normal) to normal position, so that there is aberrant closure of the tricuspid valve. The result of which is tricuspid valve regurgitation i.e. blood goes back to venous circulation and causes peripheral pooling of blood- edema.
Symptoms
shortness of breath
fatigue
palpitation
hiccoughs
heart races
cyanosis(rare finding)
clubbing(obliteration of nail bed angle due to deposition of glycosaminoglycans
holosystolic murmur
Less common symptoms are:
brain abscess(due to right and left shunt)
embolism
stroke
ischemic attack
essenmenger's complex
Besides leakage another clinical course of people with ebstein's anomaly is whether other abnormalities are present such as ASD or VSD or transposition of great arteries.
it is associated with ELECTRICAL SHORT CIRCUIT OF THE HEART- WOLF PARKINSON WHITE SYNDROME.
there is problem of electrical signal that travel from top to bottom of heart and is characterized by:
supraventricular tachycardia
palpitation
fainting
Complications:
congestive heart failure
infective endocarditis
clot formation
Diagnosis
cachexia
Cardiomegaly- in chest x-ray (apex beat is displaced.)
Normal cardiac shadow: 0.5
In cardiomegaly> 1
Echocardiography-sonogram of heart in which sound wave provide abnormal position of valve
ECG- supraventricular tachycardia
cardiac catheterization in severe case
[U]Treatment
Medical[/u]
penicillin "B" against infective endocarditis
digoxin to control heart rate
diuretics to relieve edema
let the heart rest
Surgical
The ultimate therapy is surgical. There are two ways of surgical correction
replacing or reconstructing the tricuspid valve and correction of other abnormalities
Inserting a conduit made of synthetic material to bypass tricuspid valve. if the valve is replaced by a synthetic valve CARPENTIER EDWARDS PIG VALVE is used

COMPILED BY:SUMAN PRASAD ADHIKARI
BISHNU SIGDEL
8 TH BATCH ,MBBS

[Probin]

Interesting post

[basant]

u did an excellent job suman! hope all people will now know what ebstien anamoly exactly is & its causes and treatment.......
anyhow keep it on
basant

[basant]

here is a sad news for us. the ebstiens's anamoly sufferer(i had mentioned in my thread before)who had ben admitted to sahid gangalal hospital, has been dischaarged from hospital because he has renal problem. the boy says he had haematuria since birth, so he has been sent for kidney biopsy, andd the reasult will come on 7th of december. (hope he will have good reasult )after the reasult of biopsy, he will be admitted again.i am a tensed.
anyhow hoping for good.

[SPRimal]

hey great job mr suman and mr basant,,,,,keep it up

[GUNNER]

nice post.....