What is Ebstein’s Anomaly?
‘Ebstein’s anomaly: In this heart condition, the attachments of the tricuspid valve to the heart are misplaced so that this valve sits more into the right ventricle than at the connection point of right atrium and right ventricle. Babies with this heart condition are blue, may have fast breathing, difficulty in feeding and poor weight gain.’
Ebsteins anomaly is a birth defect of the Tricuspid valve (the valve between the Right atrium and Right Ventricle). The attachments of the tricuspid valve to the heart are misplaced so that this valve sits more into the right ventricle than at the connection point of right atrium and right ventricle (right upper chamber of the heart and right lower-pumping chamber of the heart).
And so, the wrongly positioned tricuspid valve leaks a lot of blood into the right upper chamber every time the heart beats.
Unfortunately, this leakage in the valve increases the load on the heart, makes the right ventricle weaker, the heart gets bigger and bigger in size, there can be shunting of blue blood to the left side and ultimately if this heart condition is not treated in time, very often it will cause heart failure and untimely death.
Treatment of this heart condition is an open-heart surgery- Surgical Repair of the Tricuspid Valve (Cone Repair) but sometimes replacing the valve with an implant may be necessary.
Some children with this heart condition have problems with the electrical circuits of the heart and this may cause occasional fast heartbeat (and this is dangerous), these children will also need catheter based ablation of these circuits in the cardiac catheterization lab.
Why do the surgery?
Ebsteins anomaly is a life-threatening heart condition and untreated – most babies/children with this birth defect will have heart failure and untimely death. On the other hand- most children that undergo surgical repair will go on to live a ‘Near Normal’ life.
The Surgical Procedure
Surgical repair is recommended if the child is blue or if the heart size is bigger than normal or if the child is having shortness of breath. Some children will be very blue and very sick after birth and these babies will need immediate medical help followed by surgery. Others may tolerate the heart condition for a few months to a few years when they start having symptoms that limit their day-to-day activity. Since these children usually do not become inoperable, they should undergo surgical repair even if the heart condition is identified late.
The surgeon makes an incision down the front of the chest (called a median sternotomy) and divides the breastbone (sternum) in half to get access to the heart. The heart is placed on cardiopulmonary bypass, meaning that a machine takes over the heart’s work of pumping of the blood so the surgeon can enter the heart to repair it.
The repair will consist of the following steps:
- Closing the hole in the heart (ASD).
- Repairing the Tricuspid Valve (Cone Repair or other forms of tricuspid valve repair) but sometimes replacing the valve with an implant may be necessary.
- Making the Right Atrium smaller.
- Sometimes a BD Glenn Operation is needed in addition to the tricuspid valve repair to help the weak right ventricle.
The breastbone is put back together, stainless steel wires are usually wrapped around the bone to fix it. The surgery will take an average of six to seven hours from start to finish.
Risks and benefits of the surgery
Risks of any heart surgery include bleeding, infection, stroke, organ damage, requirement for a temporary or permanent pacemaker, or possibly death. However, the risk of these occurring is about 5%.
The benefits surgery for the child (once recovered from the surgery) is that he/she will have a near normal life expectancy and quality of life. This would mean that the child will not be blue anymore and will not have heart failure symptoms and his/her growth and development will be better. In addition, the risk of death and heart failure are also avoided.
What to expect during the hospital stay
The length of the hospital stay usually varies from 7 to 9 days, depending on how quickly the heart recovers. For a few days following surgery, we will take care of your child in the ICU. In the Intensive Care Unit, we will keep a watch on the child’s Heart Rate, Blood Pressure, Respiration and other organ functions. To avoid frequent interruptions in the care of your child, there is a restriction to visitors, however, parents can visit the child.
The child may need to be in the hospital for longer if they experience any of the following:
- Low heart function
- Fluid in the chest (pleural effusions), requiring a chest tube that is put in place at the end of surgery to stay in longer or additional chest tubes to be added
- Needing longer than usual help from a machine (ventilator) to breathe.
- A fast heartbeat (supraventricular tachycardia or SVT), requiring medications to slow down the heart
These challenges are part of the normal recovery after surgery in a child with Ebsteins anomaly.
For a few days after surgery, the child will be on heart medications to improve heart function and will be on in sedation and on a ventilator- to help the heart get stronger.
After the ventilator is removed and the child is breathing on its own- he/she will be sleepy while recovering from and will be under the influence of strong pain medications. It is normal for a child to be confused, thirsty, and irritable.
Going home
The hospital will supply specific discharge instructions, and all instructions from the hospital, cardiac surgeon, and cardiologist should be strictly followed. The child should get back to his or her normal routine upon arrival at home. For the first week, the child may wish to take more frequent naps or wake up occasionally at night. The child should be seen by his or her cardiac surgeon/ cardiologist within the first 3-5 days after discharge from the hospital. The incision should be kept clean until it is healed. It should also be protected from the sun for a full year.
The child should not be picked up under the arms for four weeks following surgery. Any of the following symptoms should be reported immediately to the cardiologist:
- fever greater than 101,
- redness, puffiness, discharge from the incision, or
- unusual nausea or flu-like symptoms.
In only about 5-8% children, with time there can be recurrence of leak in the tricuspid valve. And so, in the initial phase of follow up we see the child frequently, but after a few months- the follow up is usually limited to once a year.
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