What is Coarctation of the Aorta?
Coarctation of Aorta is a birth defect in which there is narrowing in the great artery (Aorta) that takes blood from the heart and distributes it to the body. Babies with this heart condition may have fast breathing, heart failure, renal failure, upper body hypertension etc
After oxygen from the blood has been used up by the body- oxygen poor blood turns blue. This Blue blood from the body returns to the Right upper chamber of the heart (Right Atrium) with the help of two veins (SVC and IVC).
From the right atrium (right upper chamber of the heart that collects blood) blood will go to the Right Pumping chamber (Right Ventricle). The Right ventricle then pumps this blue- oxygen poor blood to the lungs (through a tube-like Great Artery-Pulmonary artery).
The Lungs will put Oxygen in this blue blood, making it Oxygen rich and red again. This Red blood returns (with the help of small tube-like pulmonary veins) to the left upper (blood collecting) chamber of the heart- Left Atrium.
From the left atrium this red blood will go to the Left Pumping chamber (Left Ventricle). The Left ventricle then pumps this red- oxygen rich blood to the body (through a tube-like Great Artery- AORTA).
The aorta will first supply blood to the upper body (head, hands and brain) and then go down to supply blood to the lower body that is-organs (Kidney, Liver, Gut etc.) in the belly and legs.
In children that have coarctation of aorta- there is narrowing in the aorta after it has supplied branches to upper body and so blood supply to the lower body is compromised. The child’s condition will depend on how severe this narrowing is.
If the narrowing is severe then the child will have problems after birth or within a few days after birth, this happens because there will be little or no blood going to the lower body and because the narrowing puts a lot of load on the heart. These babies may have heart failure, shock, kidney failure and respiratory failure. For these babies, treatment of this heart condition is an emergency and may include- medication (prostaglandins) to temporarily open blood supply to the lower body, ventilator and medication to increase heart function and this is rapidly followed with a repair by surgery.
In some children the narrowing is very narrow but not will still permit some blood flow to the lower body. These children may not become very sick immediately after birth but will have a very high risk of heart failure and death during the first year of life.
In children that survive the first year of life with this heart condition, there will be high blood pressure in the upper body (and brain) and so if they are not treated, they will have a risk of life-threatening bleeding in the brain from this uncontrolled hypertension.
Treatment of this heart condition is a surgery- Repair of Coarctation of Aorta, in which the narrow part of the artery is cut and removed, and the ends are sutured back together.
Why do the surgery?
Coarctation of Aorta is a life-threatening heart condition and untreated – many babies with this birth defect will not survive.
On the other hand- most children that undergo a surgical repair will go on to live a near normal life. In a small proportion of children this repaired part of the aorta does not grow as the child gets bigger, and so follow up with a pediatric cardiologist is strongly recommended. In the small percentage of children in which this repeat narrowing happens, most can simply be treated by a Balloon Dilation in the Cath lab (without surgery)
The surgical procedure
Surgery is usually done immediately after birth or within a few weeks of birth. The surgeon makes an incision down the left side of the chest (called a left thoracotomy) to get access to the narrow part of the Aorta. The blood supply in this narrow part is stopped for a little while to do the operation.
The repair will consist of the following steps:
- Cutting and removing the narrow part of Aorta.
- Sewing the normal sized ends together
The surgery will take an average of three to four hours from start to finish.
Risks and benefits of the surgery
Risks of any surgery include bleeding, infection, renal failure, paraplegia (leg weakness), or possibly death. However, the risk of these occurring is 1 to 2%.
The benefit of the surgery is for the child (once recovered from the surgery) will have a near normal life expectancy and quality of life. This would mean that the child will not have heart failure symptoms and his/her growth and development will be better. In addition, the risk of death, heart failure are also avoided.
What to expect during the hospital stay
The length of the hospital stay usually varies from 4 to 7 days, depending on how quickly the child 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.
These challenges are part of the normal recovery after surgery in a child with coarctation of aorta.
For a day of two after surgery, the child may 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 and dry until it is healed.
It should also be protected from the sun for a full year. 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.
