Ebstein’s Anomaly

Ebstein’s anomaly or Ebstein’s malformation is a rare congenital heart defect, which accounts for less than 1% of all congenital heart disease. With Ebstein’s, the opening of the tricuspid valve (septal and posterior leaflets) is displaced towards the apex of the right ventricle of the heart. This defect is characterized by remarkable morphologic variability and a broad spectrum of clinical presentations. Consequently, diagnosis may be made in symptomatic newborn infants, young adults, or middle-aged adults, depending on the severity of the defect and clinical manifestations.  Often there’s also a hole in the wall between the atria, the heart’s two upper chambers. This hole is called an atrial septal defect or ASD. Because the tricuspid valve is malformed in Ebstein’s anomaly, it often doesn’t work properly and may leak. If the valve leaks, some of the blood pumped by the right ventricle goes backwards through the valve with each heartbeat.
Surgical therapy should be considered in any patient with progressive symptoms, exercise intolerance, or frequent arrhythmia. Goals of surgical intervention are as follows: to increase pulmonary blood flow, to minimize tricuspid insufficiency, to reduce or eliminate right-to-left shunting, to optimize right ventricular function, and to reduce or eliminate arrhythmia. Ideally, the tricuspid valve can be repaired, which may allow the patient to avoid valve replacement with a bioprosthetic valve and the need for future valve replacement. Different types of procedures can be used to surgically treat Ebstein’s anomaly and associated defects, including:
Tricuspid valve repair. This involves reducing the size of the valve opening and allowing the existing valve leaflets to come together to work properly. This procedure is usually done when there’s enough valve tissue to allow for repair.
Tricuspid valve replacement. If the existing valve can’t be repaired, it may be replaced by removing the deformed valve and inserting either a mechanical valve or a specially treated biological tissue (bioprosthetic) valve. If a mechanical valve is used, you’ll need to take a blood-thinning medication for the rest of your life.
Closure of the atrial septal defect (ASD). If an ASD is present, your surgeon can close the hole during surgery to repair or replace the defective valve.
Maze procedure. Another procedure that doctors may perform during valve repair or replacement surgery is called a maze procedure. It’s used to help correct fast heart rhythms in the atrium (atrial tachyarrhythmias). To do the procedure, your surgeon makes a series of incisions in the right atrium with a scalpel or a device that destroys tissue by freezing it. When these incisions heal, the scars create a barrier of tissue in your heart that causes it to beat at a normal rate. The Maze procedure is an open-heart surgery.
Modified Danielson Technique by Dr. Vijit Koshy Cherlan
To provide a place for all those affected-by or involved-in ebsteins anomaly to share information and exciting developments in research, and learn from the experiences of others.
To raise awareness and provide up to date information about ebsteins anomaly.
To raise money to fund research into ebsteins anomaly that will not otherwise be carried out.
In the News:
Durham Bulls manager, Charlie Montoyo—
his son Alex Montoyo, was born with Ebstein’s anomaly.
Follow the Durham Bulls on Twitter: @DurhamBulls
Notable Tweets:
@CheckOrphan EBSTEIN’S MALFORMATION: ‘A heart that’s too big: Merrimac native’s rare disease leads to fourth open heart …..’, http://tinyurl.com/mstvhz
[Dawn Frost prepares for her fourth heart surgery…]
@mizzlizwhizz: I had to have ECG, 24hr tape recording of my heartbeat – turned out to have very rare and harmless (for me) Ebstein’s anomaly

Ebstein’s anomaly or Ebstein’s malformation is a rare congenital heart defect, which accounts for less than 1% of all congenital heart disease. With Ebstein’s, the opening of the tricuspid valve (septal and posterior leaflets) is displaced towards the apex of the right ventricle of the heart. This defect is characterized by remarkable morphologic variability and a broad spectrum of clinical presentations. Consequently, diagnosis may be made in symptomatic newborn infants, young adults, or middle-aged adults, depending on the severity of the defect and clinical manifestations.  Often there’s also a hole in the wall between the atria, the heart’s two upper chambers. This hole is called an atrial septal defect or ASD. Because the tricuspid valve is malformed in Ebstein’s anomaly, it often doesn’t work properly and may leak. If the valve leaks, some of the blood pumped by the right ventricle goes backwards through the valve with each heartbeat.

Surgical therapy should be considered in any patient with progressive symptoms, exercise intolerance, or frequent arrhythmia. Goals of surgical intervention are as follows: to increase pulmonary blood flow, to minimize tricuspid insufficiency, to reduce or eliminate right-to-left shunting, to optimize right ventricular function, and to reduce or eliminate arrhythmia. Ideally, the tricuspid valve can be repaired, which may allow the patient to avoid valve replacement with a bioprosthetic valve and the need for future valve replacement. Different types of procedures can be used to surgically treat Ebstein’s anomaly and associated defects, including:

  • Tricuspid valve repair. This involves reducing the size of the valve opening and allowing the existing valve leaflets to come together to work properly. This procedure is usually done when there’s enough valve tissue to allow for repair.
  • Tricuspid valve replacement. If the existing valve can’t be repaired, it may be replaced by removing the deformed valve and inserting either a mechanical valve or a specially treated biological tissue (bioprosthetic) valve. If a mechanical valve is used, you’ll need to take a blood-thinning medication for the rest of your life.
  • Closure of the atrial septal defect (ASD). If an ASD is present, your surgeon can close the hole during surgery to repair or replace the defective valve.
  • Maze procedure. Another procedure that doctors may perform during valve repair or replacement surgery is called a maze procedure. It’s used to help correct fast heart rhythms in the atrium (atrial tachyarrhythmias). To do the procedure, your surgeon makes a series of incisions in the right atrium with a scalpel or a device that destroys tissue by freezing it. When these incisions heal, the scars create a barrier of tissue in your heart that causes it to beat at a normal rate. The Maze procedure is an open-heart surgery.

Modified Danielson Technique by Dr. Vijit Koshy Cherlan

http://www.ebsteins.org

  • To provide a place for all those affected-by or involved-in ebsteins anomaly to share information and exciting developments in research, and learn from the experiences of others.
  • To raise awareness and provide up to date information about ebsteins anomaly.
  • To raise money to fund research into ebsteins anomaly that will not otherwise be carried out.

In the News:

Durham Bulls manager, Charlie Montoyo—
his son Alex Montoyo, was born with Ebstein’s anomaly.
http://www.newsobserver.com/sports/story/1600793.html
Follow the Durham Bulls on Twitter: @DurhamBulls

Notable Tweets:

@CheckOrphan: EBSTEIN’S MALFORMATION: ‘A heart that’s too big: Merrimac native’s rare disease leads to fourth open heart …..’, http://tinyurl.com/mstvhz
[Dawn Frost prepares for her fourth heart surgery…]

@mizzlizwhizz: I had to have ECG, 24hr tape recording of my heartbeat – turned out to have very rare and harmless (for me) Ebstein’s anomaly

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~ by marbleroad on July 10, 2009.

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