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Aortopulmonary window

Definition

Aortopulmonary window is a rare heart defect in which there is a hole connecting the major artery taking blood from the heart to the body (the aorta) and the one taking blood from the heart to the lungs (pulmonary artery). The condition is congenital, which means it is present from birth.

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Alternative Names

Aortopulmonary septal defect; Aortopulmonary fenestration

Causes, incidence, and risk factors

Normally, blood flows through the pulmonary artery into the lungs, where it picks up oxygen. Then the blood travels back to the heart and is pumped to the aorta and the rest of the body.

Babies with an aortopulmonary window have a hole in between the aorta and pulmonary artery. Because of this hole, blood from the aorta flows into the pulmonary artery, and too much blood flows to the lungs. This causes high blood pressure in the lungs (a condition called pulmonary hypertension) and heart failure. The bigger the defect, the more blood can enter the pulmonary artery.

The condition occurs when the aorta and pulmonary artery do not divide normally as the baby develops in the womb.

Aortopulmonary window is very rare. It accounts for only 0.1% of all congenital heart defects.

This condition can occur on its own or with other heart defects such as patent ductus arteriosus, tetralogy of Fallot, or pulmonary atresia. Fifty percent of patients usually have no other heart defects.

Symptoms

If the defect is small, it may not cause any symptoms. However, most defects are large.

Symptoms can include:

Delayed growth
Heart failure
Irritability
Poor eating and lack of weight gain
Rapid breathing
Rapid heartbeat
Respiratory infections

Signs and tests

The pediatrician will usually hear an abnormal heart sound (murmur) when listening to the child's heart with a stethoscope.

The doctor may order tests such as:

Cardiac catheterization - a thin tube inserted into the arteries around the heart to view the heart and blood vessels and directly measure pressure in the heart and lungs
Chest x-ray
Echocardiogram
MRI of the heart

Treatment

The condition usually requires open heart surgery to repair the defect. Surgery should be done as soon as possible after the diagnosis is made, usually when the child is still a newborn.

During the procedure, a heart-lung machine takes over for the child's heart. The surgeon opens the aorta and closes the defect with a patch made either from a piece of the sac that encloses the heart (the pericardium) or a man-made material.

Expectations (prognosis)

Surgery to correct aortopulmonary window is successful in most cases. If the defect is treated quickly, the child should not have any lasting effects.

Complications

Delaying treatment can lead to complications such as:

Congestive heart failure
Pulmonary hypertension
Death

Calling your health care provider

Call your health care provider if your child has symptoms of aortopulmonary window. The sooner this condition is diagnosed and treated, the better the child's prognosis.

Prevention

There is no known way to prevent aortopulmonary window.

References

Miscellaneous congenital cardiac conditions. In: Park MK. Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa:Mosby Elsevier; 2008:chap 17.

Jansen C. Surgical repair of aortopulmonary window: thirty-seven years of experience. Pediatr Cardiol, 2006;27:552-556.

Koh Su-Mei A, Ju-Le T. Large unrepaired aortopulmonary window--survival into the seventh decade. Echocardiography, 2007;24:71-73.

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