Interrupted aortic arch is a rare defect of heart. It occurs when the fetus is still in mother’s womb. In this disorder the development of aorta is not normal. This congenital anomaly is very rare and out of 1 million live births only 3 babies are estimated to be born with this condition. Interrupted aortic arch usually develops with other associated heart defects such as ventricular septal defect, aortic stenosis, truncus arteriosus etc.
In interrupted aortic arch a disconnection occurs at the arch of aorta and the descending aorta. Normally aorta is a large artery originating from the left ventricle (left lower chamber of heart).From the ventricle the aorta makes an upward arch and then curves downwards. From the arch three branches of arteries originate which supply blood to head and neck. The descending aorta delivers blood to lower part of body.
The newborn can survive for few days because the patent ductus arteriosus remains open for few days after birth. Blood circulates from patent ductus arteriosus in fetal heart, but it is of no use after birth hence it gets closed. However the infant having interrupted aortic arch becomes extremely ill as soon as the alternative patent ductus gets closed.This causes stoppage of blood to the lower part of body and ultimately the patient dies. Surgery is needed as early as possible to this life threatening condition.
Causes Of Interrupted Aortic Arch
Interrupted aortic arch is a congenital disease. It may also occur as an inherited condition. In this disorder, the abnormality occurs because certain chromosome which carries information of heart and its vessels formation is missing. This causes abnormality in the heart structure and aorta. Most babies born with interrupted aortic arch also have other congenital heart defects.
Babies having this disorder are also born with DiGeorge syndrome. It is the genetic condition wherein the one part of chromosome 22 is damaged during the early fetal development. Besides interrupted aortic arch, the baby with DiGeorge syndrome also has reduced resistance to viral infection, cleft palate, change in facial structure, renal problem etc.
Symptoms And Diagnosis Of Interrupted Aortic Arch
The signs and symptoms of interrupted aortic arch are visible within one or two days of birth when the patent ductus arteriosus closes. The symptoms are those of congestive heart failure. The infant may become weak and tired. He remains sleepy all the time. There is accompanied breathlessness.
Due to profound weakness and fatigue, the child is unable to suck mother’s breast for feeding. The skin becomes grey in certain lower parts of the body. It appears mottled as the blood is not reaching those areas. The foot and legs are cold and clammy sue to interrupted blood supply from the aorta.
Diagnosis: Interrupted aortic arch can be detected during the prenatal period or after birth with the help of echocardiogram. A heart murmur is heard by the doctor when he puts his stethoscope on the chest of the baby during the physical examination. There may be rapid breathing and absence of pulse in the lower extremities. Once the anomaly is detected with echocardiogram and the diagnosis is confirmed, surgical intervention is critical for the survival of the child.
Treatment Of Interrupted Aortic Arch
The treatment of interrupted aortic arch is surgery. During the surgery, the aorta is repaired and the arch is restored as in a normal case. However, in majority of cases a drug called prostaglandin is administered in the child. It is meant to keep the patent ductus arteriosus open. This will at least help the circulation of blood till surgery repair is done.
During the surgery, the surgeon will first close the patent ductus arteriosus and then proceed ahead connect and repair the malformed aorta. A correct arch is formed with surgery. At the same time if ventricular septal defect is present it is closed.
Because of modern day medical technology, most children having heart defect after surgery are able to live long. But a child with interrupted aortic arch after surgery will need to consult regularly to his cardiologist. He will need regular antibiotics to prevent damage to the valves.