Aortic Arch Interruption

Definition

Definition of Aortic Arch Interruption
Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births) in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. Almost all patients also have other cardiac anomalies. Interrupted aortic arch is often associated with DiGeorge Syndrome.

Symptoms

Symptoms of Aortic Arch Interruption
Signs and symptoms of poor perfusion or congestive heart failure may develop when the ductus arteriosus begins to close, usually within the first day or two of life.

The infant may develop weakness, fatigue, poor feeding, rapid breathing, fast heart rate, or low oxygen levels, particularly when measured in the legs and feet.

This condition can worsen and lead to shock. The infant will then be pale, mottled, and cool. The infant will likely have decreased urine output and poor pulses, especially in the lower extremities.

Causes

Causes of Aortic Arch Interruption
Interrupted Aortic Arch (IAA) is the absence or discontinuation of a portion of the aortic arch. There are three types of Interrupted Aortic Arch, and they are classified according to the site of the interruption.

1. Type A: the interruption occurs just beyond the left subclavian artery. Approximately 30 percent to 40 percent of the infants with Interrupted Aortic Arch have Type A.
2. Type B: (shown in diagram): the interruption occurs between the left carotid artery and the left subclavian artery. Type B is the most common form of Interrupted Aortic Arch. It accounts for about 53 percent of reported cases.
3. Type C: the interruption occurs between the innominate artery and the left carotid artery. Type C is the least common form of Interrupted Aortic Arch, accounting for about 4 percent of reported cases.

Diagnosis

Diagnosis of Aortic Arch Interruption
It can be diagnosed with an echocardiogram. Patients will have a loss of appetite, turn pale, may feel cold in the lower half of the body due to not enough blood flow.

Treatment

Treatment of Aortic Arch Interruption
Treatment consists of open heart surgery soon after birth. Awaiting surgery, prostaglandin can be administered to keep the ductus arteriosus open, thereby allowing blood flow to the lower body. Failure to treat the condition yields a mortality rate of 90% at a median age of 4 days.

Prognosis

Prognosis of Aortic Arch Interruption
Complications after Interrupted Aortic Arch repair may include residual obstruction or stenosis (narrowing) at the aortic repair site.

The aortic valve or the area below the valve are often small and may not grow, which can result in stenosis (narrowing) months or years following surgery.

Prevention

Prevention of Aortic Arch Interruption
Consult with your doctor.