Coarctation of the aorta, is a congenital defect and occurs when the aorta narrows or becomes pinched. Coarctation can occur anywhere in the aorta, but is most likely to happen in the segment just after the Coarctation of Aorta Repairaortic arch. This narrowing restricts the amount of oxygenated blood that can travel to the lower part of the body. Varying degrees of narrowing can occur.

The more severe the narrowing, the more symptoms a child will experience, and the earlier the problem will be noticed.

Coarctation of the aorta occurs in about 6 to 8 percent of all children with congenital heart disease. Boys have the defect twice as often as girls do.

Causes of aortic coarctation

Coarctation of the aorta occurs due to improper development of the aorta in the first eight weeks of fetal growth.Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality or environmental exposure, causing heart problems to occur more often in certain families. Most of the time this heart defect occurs by chance, with no clear reason for its development.

Coarctation of the aorta causes several problems, including the following:

• The left ventricle has to work harder to move blood through the narrowing in the aorta. Eventually, the left ventricle is no longer able to handle the extra workload, and it fails to pump blood to the body efficiently. This could lead to a serious condition called congestive heart failure
• Blood pressure is higher above the narrowing, and lower below the narrowing. Older children may have headaches from too much pressure in the vessels in the head, or cramps in the legs or abdomen from too little blood flow in that region. Also, the kidneys may not make enough urine since they require a certain amount of blood flow and a certain blood pressure to perform this task
• The walls of the ascending aorta, the aortic arch, or any of the arteries in the head and arms may become weakened by high pressure. Spontaneous tears in any of these arteries can occur, which can cause a stroke or uncontrollable bleeding
• There is a higher than average chance of developing an infection in the lining of the heart or aorta known as bacterial endocarditis
• The coronary arteries, which supply oxygen-rich (red) blood to the heart muscle, may narrow in response to elevated pressure
• The blood pressure in the pulmonary artery may rise above normal levels, a condition called pulmonary hypertension

Symptoms

Symptoms noted in early infancy are caused by moderate to severe aortic narrowing. The following are the most common symptoms of coarctation of the aorta. Each child may experience symptoms differently. Symptoms may include:

• Irritability
• Pale skin
• Sweating
• Heavy and/or rapid breathing
• Poor feeding
• Poor weight gain

High blood pressure or a heart murmur is noted on a physical examination. Some may complain of headaches or cramps in the lower sections of the body. The symptoms of coarctation of the aorta may resemble other medical conditions or heart problems.

Diagnosis

A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. A physical examination wil be made, listening to the child's heart and lungs, and other observations that help in the diagnosis. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur, will give the cardiologist an initial idea of which heart problem your child may have. Other tests are needed may include the following:

• Chest X-ray
• Echocardiogram (echo) — A procedure that evaluates the structure and function of the heart by using sound waves, recorded on an electronic sensor, that produce a moving picture of the heart and heart valves.
• Electrocardiogram (ECG or EKG) — A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias) and detects heart muscle damage.
• Magnetic Resonance Imaging (MRI)

Treatments

Treatment for coarctation of the aorta will be decided based on:

• The child's age, overall health and medical history
• Extent of the disease
• Child's tolerance for specific medications & procedures
• Coarctation of the aorta is treated with repair of the narrowed vessel. It can be done through a catheterization procedure or through surgery in an operating room:
• Cardiac Catheterization — During the procedure, the child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist will pass an inflated balloon through the narrowed section of the aorta to stretch the area open. A small device, called a stent, may also be placed in the narrowed area after the balloon dilation to keep the aorta open
• Surgical Repair — The surgical repair of aortic coarctation is done through an incision on the left chest below the armpit. The ribs are spread, the lung pushed aside, and the aorta exposed near the heart. The aorta is clamped on either side of the narrowing and the narrow segment with the adjacent ductus arteriosus is cut out. The two ends of aorta are then sewn together, clamps are removed, and flow through the aorta is reestablished

Most children who have had a coarctation of the aorta surgical repair will live healthy lives. Activity levels, appetite, and growth should eventually return to normal. As the child grows, the aorta may once again become narrow on occasion. If this happens, a balloon procedure or operation may be necessary to repair the coarctation.

After surgery, infants will return to the intensive care unit (ICU) to be closely monitored during recovery.

While your child is in the ICU, special equipment will be used to help him/her recover, and may include the following:

• Ventilator - a machine that helps your child breathe while he/she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he/she is too sleepy to breathe effectively on his/her own. Many children remain on the ventilator for a while after surgery so they can rest
• Intravenous (IV) catheters - small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medicines that help your child recover from the operation
• Arterial line - a specialized IV placed in the wrist, or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU
• Nasogastric (NG) tube - a small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery
• Urinary catheter - a small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys to remove excess fluid from the body.
• Chest tube - a drainage tube will be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.
• Heart monitor - a machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values

Other equipment not mentioned here may be needed to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.

Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.

After discharged from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.

The other major cardiac procedures are:

• Heart Valve Replacement
• Coronary Angioplasty
• VSD (Ventricular Septal Defect) Closures etc

Few popular hospitals for Coarctation of the Aorta Repair are:

• Narayana Hrudayalaya
• Bangkok Hospital Medical Center
• Florence Nightingale Hospital

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