What is Endocardial Cushion Defect?

The structure of a human heart shows that it has four valves; namely the right atrium (on the upper right side of the heart), the left atrium (on the upper left side of the heart), the right ventricle (on the lower right side of the heart) and the left ventricle. Each of these chambers is divided by walls. The impure blood enters the right atrium through the superior and inferior vena cava and then the deoxygenated blood is oxygenated inside the heart and finally flows out of the left ventricle through the aorta and is distributed throughout the body.

When a person has an endocardial cushion defect in his or her heart, then the valves of the heart are not built properly. Due to underdevelopment of the valves, the person will find difficulty in breathing or even surviving. If a person has endocardial cushion defect then the walls that each one valve from the other is not built or have not grown at all. This makes it difficult for the heart to spate out the deoxygenated blood from the oxygenated blood inside the heart. As the wall separating the right and left atrium and the right and left ventricles are not there, the process of oxygenating the deoxygenated blood cannot be carried out well. Due to lack of oxygen in our body, our body will get fatigued; the carbon dioxide content in the blood keeps increasing and therefore, troubles in breathing is noticed and survival becomes difficult.

The endocardial cushion defect is mostly found in new born babies. The disease is congenital that is the baby is born with endocardial cushion defect. When the baby develops within the womb, the growth of the walls does not take place making the baby being born with endocardial cushion defect. This defect is soon analyzed in the new born child and if that is done, then the treatment can begin soon enough to save the child.

What is Endocardial Cushion Defect?

Types of Endocardial Cushion Defect

There are two types of endocardial cushion defect:

  • Complete endocardial cushion defect where both the (ASD) atrial septal defect (an abnormal opening in the wall in between the 2 atria) and the VSD or ventricular septal defect (an abnormal opening in the wall in between the 2 atria) have developed and instead of a tricuspid and the mitral valve, there is a singular valve.
  • Incomplete endocardial cushion defect where either the ASD or the VSD has developed and both the valves are there, but one of them is not working properly.

Causes of Endocardial Cushion Defect

The walls that separate the auricles and the ventricles are made of endocardial cushions. It is the endocardial cushions which give rise to the septum creating the divisions within the auricles and the ventricles and separate the top chambers of the hearts (the right and left atrium) that collect the deoxygenated blood from the chambers at the bottom (the right and left ventricle) that pumps the oxygenated blood out of the heart. The endocardial cushions also help in developing the tricuspid and mitral valves that opens and closes to the chambers of the heart.

When the child is born with endocardial cushion defect, the above septum and the tricuspid and the mitral valves are missing. This makes the heart difficult to separate the deoxygenated blood from the oxygenated blood. Then, a large amount of blood flows directly from the heart to the lungs developing a pressure in the lungs. The blood from the heart flows through any kind of abnormal opening either on the left or right side or from both sides that has developed in the heart to the lungs and rise the blood pressure of the lungs.

This defect in the formation of the septum during the formation of the child leads to the endocardial cushion defect in the child.

Symptoms of Endocardial Cushion Defect

The symptoms of endocardial cushion defect are common once noticed the child should be taken to the doctors immediately. The symptoms are:

  • Breathlessness or very fast breathing is the primary symptom of endocardial cushion defect.
  • The baby does not grow properly neither does it gains weight.
  • Loss of appetite and difficulty in feeding the baby, also breathing problems may be noticed during feeding the baby.
  • Pale bluish tone of the skin is seen in the new born with endocardial cushion defect.
  • Sweating.
  • Increase in the rate of heartbeat.
  • Swelling of the knees and legs and other joints (also in the abdominal section).
  • The baby will seem to the tiring very easily.
  • Developing various infections and illnesses like pneumonia quite often.

Tests to Diagnose Endocardial Cushion Defect

Various tests are done by the doctors to find out if the baby is suffering from endocardial cushion defect:

  • ECG to blow up the picture of the structure of the heart.
  • X-Ray of the chest.
  • The procedure named Cardiac Catheterization where a very lean tube is passed to the heart to check the blood flow of the heart.

Treatment for Endocardial Cushion Defect

If the report of the ECG is abnormal, where one can see an enlarged heart of the Cardiac Catheterization can record a murmur in the heart, the doctors diagnose it as endocardial cushion defect. The treatment procedure then begins. Surgery is the only step to treat this defect. The surgery is very delicate and can be done if the baby is 3 to 6 months old. The surgery then builds all separate walls and the valves that are missing in the heart. More than one surgery may be required to create a complete endocardial cushion. The surgery is done only after checking the condition of the baby. Usually, water pills are given to the baby to increase its weight before the surgery for endocardial cushion defect. Pills like digoxin are given so that the contractions of the heart occur in time.

Home Remedies for Endocardial Cushion Defect

  • One needs to look after the child all the time.
  • Frequent visits to the doctor should be done to keep the medicine on-going.
  • Feeding only very light food is a good home remedy for endocardial cushion defect.

Recovery Period/Healing Time for Endocardial Cushion Defect

The time that endocardial cushion defect will take to recover depends on the surgery and the healing time. The doctor treating the baby will be able to give information regarding this. The physician will also be able to tell if there would be any further complications after the surgery or not.

Prevention of Endocardial Cushion Defect

If the parents have a history of endocardial cushion defect, then child can tend to develop it, hence medical consultations should be done in time to prevent endocardial cushion defect to pass on to the children.

Risk Factors for Endocardial Cushion Defect

The greatest risk in endocardial cushion defect is that the child will have a life risk. Also lack in the growth of the child will lead to further complications and diseases. Lack of oxygen in the blood will affect in the growth of the kid. Loss of appetite and pale bluish color of the skin will degrade the health of the kid.

Complications of Endocardial Cushion Defect

Both types of endocardial cushion defect leads to risk the life of the child like the heart failure, Eisenmenger syndrome, huge blood pressure in the lungs causing irreversible damage to the lungs. Various symptoms of endocardial cushion defect are not noticed until the child grows of age. Also after the surgery, infections of the heart may occur.

Endocardial cushion defect can give rise to several complications like:

  • Failure of the heart due to the missing wall, heart gives extra effort in pumping out the blood. This makes the heart work harder with larger pressure. The process weakens the muscles of the heart. The muscles of the heart may even enlarge and get loose. This defect may lead to the swelling in the inside of the baby, cause difficulty in breathing and affects the growth of the infant.
  • Development of cyanosis is a complication of endocardial cushion defect as the pressure in the lungs increase and this disrupts the flow of the blood from one side to the other. The disruption in the blood flow from the right side to the left side of the heart mixes the deoxygenated and the oxygenated blood. Hence, a large amount of blood containing less oxygen flows back to the body causing cyanosis or bluish skin.

Prognosis/Outlook for Endocardial Cushion Defect

The prognosis or outlook for endocardial cushion defect is excellent if it is detected immediately after birth and the surgery is done within the first year of birth. The condition of your infant can be understood with the stage of endocardial cushion defect the infant has, if he or she has already developed a lung disease and how well the surgery went. Often children live a fine life after the surgery. The surgery requires to be done in the first year of the baby to prevent any long term disease or illness. If not, the complications may increase. Lung diseases easily develop in children with Down’s syndrome.

Lifestyle Changes for Endocardial Cushion Defect

The child needs to be taken to the doctor immediately after the symptoms are noticed. Only dilute food that is easy to be taken should be given to the child and it is a good lifestyle change. The child should not be allowed to get exhausted soon. After the surgery, if infection like endocarditis occurs, the doctors should be consulted immediately. Inform the doctors before the child goes under any kind of surgery like dental later to avoid infection.

Coping with Endocardial Cushion Defect

It is very difficult for the family to know that their child is suffering from endocardial cushion defect. The parents need to be strong during this time. Losing hope will not help. One should always be upbeat and should keep the child happy for better coping.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: April 27, 2017

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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