Interrupted Aortic Arch : Procedures, Side effects, Recovery time, Costs and alternatives

Interrupted Aortic Arch (IAA) is heart defect that affects children at birth. It is a rare congenital heart condition, that is said to affect about 3 cases per million live births. In this condition, children are born with an incompletely developed aorta, due to which oxygenated blood cannot be transported to different parts of the body. It is detected within a few days of birth, and in some cases, even before birth. Treatment requires immediate surgery, and delay in treatment may cause the infant to go into shock and may even lead to death.

The aorta is the largest blood vessel in humans which delivers oxygen rich blood from the heart to the rest of the body. It arises upwards from the left side of the heart, then curves downwards and leads to blood vessels distributed throughout the lower body. The initial part of aorta that arises from the heart is called ascending aorta. The curved section is called aortic arch and the part of aorta that heads downwards is called descending aorta. The ascending and descending aorta are completely separated at the aortic arch in infants suffering from this defect. Depending on the site where the interruption or blockage is present, this condition can be categorised as:

• Type A: When the aortic arch is blocked at the left subclavian artery (leftmost branch on top of aortic arch)
• Type B: When the aortic arch is interrupted at the left common carotid artery (middle branch on top of aortic arch)
• Type C: When the aortic arch is interrupted at the brachiocephalic trunk (rightmost branch on top of aortic arch)

There are no known causes for this disease, other than genetics.Though it is rare, it can affect any new-born baby. As such, there aren’t any preventive measures for this disease either. Children affected by this condition may have one or more of the following symptoms such as:

These symptoms are often detected within a few days of birth. This condition completely blocks the flow of blood in aorta and prevents oxygenated blood from reaching parts of the body. So surgery will have to be performed within a week after detecting the condition, otherwise lack of blood in the child’s body can prove to be fatal. Interrupted aortic arch is almost always accompanied by some other heart defect like ventricular septal defect or truncus arteriosus.

Corrective surgery is the only method of treatment for children diagnosed with this condition. If the symptoms of interrupted aortic arch are observed in new-born babies, the doctor will perform a diagnosis with the help of tests like chest x-ray, electrocardiogram and echocardiogram.The condition may also be detected before birth, but it only happens in rare cases. Once the diagnosis is confirmed, the doctor will prepare for the treatment.

First, the child’s ductus arteriosus will be observed. Ductus arteriosus is a blood vessel that connects the pulmonary artery to descending aorta in prenatal stage but closes a few days after birth. Before proceeding for surgery, it is important to keep the ductus arteriosus open as it allows some blood to reach the different part of the body. This helps doctors to buy more time to prepare for surgery. The ductus arteriosus is kept open after birth using a medication called Prostaglandin E1. But in some children, the ductus arteriosus may remain open naturally, which is a rare condition called patent ductus arteriosus (PDA).

After that, the child will be able to stay in proper condition for a few days while preparations are done for the surgery. The surgical procedure involves many complicated steps. A simple and general concept of the surgical process is explained:

• First, a small incision is made on the chest depending on the site of interruption.
• The skin and muscles are fixed in place around the incision using sutures, which leaves the heart open and visible.
• The ductus arteriosus remains connected to the descending aorta. This connection is removed or cut, and the opening of the ductus arteriosus is closed.
• Then the blockage in the ascending and descending aortas or also cut or removed, and the two portions of aorta are sewn together using special sutures.
• With this, the aortic arch becomes complete and functional so blood can flow through the aorta.
• Once the process is completed, the incision is closed with stitches.
• Some steps in the surgery may vary depending on what type of interrupted aortic arch it is (type A,B,C)

The process mentioned above is only for correcting the interrupted aortic arch. But this condition mostly occurs along with some other heart defect such as ventricular septal defect. So the overall surgery will also include necessary procedures for treating the other defects present.

Any infant affected by an interrupted aortic arch is eligible for the treatment. In fact, surgery is a must for the permanent cure of this condition. The surgery is done within a week at most after birth.

Infants that are not affected by interrupted aortic arch do not require this treatment and can be considered as not eligible for it. This defect doesn’t occur in adults so they too do not need any treatment for it.

The condition or the surgery don’t have any significant side effects. Children affected by interrupted aortic arch can certainly go on to live a healthy life. But there are a few complications that may affect such children in the later stage of their lives, such as,

• Higher risk of Subacute Bacterial Endocarditis – a bacterial infection in the endocardium, which is the inner lining of the heart. Some children may also have to take antibiotics for a long term to avoid endocarditis.
• Higher risk of SubacuSome children may develop a defect called Subaortic Stenosis in the future. It is a condition that leads to obstruction in the flow of blood at the left ventricle.
• Higher risk of SubacuNot able to participate in physical activities that require over exertion of one’s limits, such as powerlifting or intensive sprinting.
• Higher risk of SubacuNeed to avoid activities that can affect the functioning of heart and circulation. In case any of these side effects persists or there any other health issues arising during or after treatment, it is advised to kindly consult the health specialist immediately.

Children who have undergone surgical repair for an interrupted aortic arch need to maintain some simple post treatment guidelines:

• Maintain a proper diet and healthy lifestyle
• Indulge in moderate exercise and physical activity, but do not exert extra pressure on the body.
• Keep the body well-rested at all times, to avoid stress and fatigue.
• May have to take antibiotics for some years, or even for a long term, to avoid risk of bacterial infection in the heart.
• Should not take part in competitive sports and physical activities at a professional level, as such activities require rigorous exercise regimes and pushing one’s limits.

Post-surgery, the child will be kept in the hospital under medical supervision for about two to four weeks. This period may be extended if any complications arise, which doesn’t happen often. Even after being discharged, the child must be kept in optimal conditions and under regular supervision of a doctor.

Since this is a rare heart condition, there is no clear details about the cost of surgery for interrupted aortic arch. Considering how critical and complicated the surgical process is, total expense of the treatment will certainly amount to six figures or greater. The cost of consulation may vary from Rs: 1000 to 3000 INR and the surgery may cost around Rs: 7 lakhs to Rs: 50 lakhs. Though in some cases the cost of the surgey may go even higher.

85 to 90 % patients who have undergone surgery for interrupted aortic arch have reported positive results post treatment. So majority of children suffering from this condition are able to live a normal life. However, they will also have to undergo occasional check-ups throughout their lives to ensure they stay healthy.

There are no alternative treatments for interrupted aortic arch other than the surgical repair. Since this condition often occurs together with other heart defects, the surgical procedure will vary in different cases. But no other treatment other than surgery is available at the moment for interrupted aortic arch.