What Is It?

Aortopulmonary Window is a rare congenital heart defect in which there is a connection (window) between the aorta and the main pulmonary artery. This opening allows oxygenated blood to pass, or shunt, from the aorta into the pulmonary artery.

Aortopulmonary Window, which affects males and females equally, can occur as an isolated defect, or with other defects or more complex heart diseases.

What Are the Effects of Aortopulmonary Window?

The movement of blood from the aorta into the pulmonary artery results in excessive blood flow to the lungs, causing high pulmonary blood pressure. The larger the hole, the greater the volume of blood shunted and the more severe the symptoms.

Babies with Aortopulmonary Window generally do not feed well and tire easily and they may develop congestive heart failure or other complications. Therefore, this defect should be corrected as soon as possible once the diagnosis has been made.

If not diagnosed soon enough, some children can develop sustained high pulmonary blood pressure (pulmonary hypertension) secondary to changes in growth of the pulmonary arteries. This severe result of an Aortopulmonary Window can render some children inoperable.

How Is Aortopulmonary Window Treated?

Surgical treatment of this defect is performed as quickly as possible after the diagnosis has been made in order to avoid high pulmonary pressure.

Quite simply, the connection (window) between the aorta and pulmonary artery is closed with a patch made of pericardium (part of the membrane surrounding the heart) or of a synthetic material. This patch (pink oval in the animation) is sutured into place and the incision in either of the two vessels through which the patch was introduced is closed with sutures.

The likelihood of postoperative difficulties depends on how quickly the defect was repaired after diagnosis. The average hospital stay after surgery is 1 week to 10 days.

Aortopulmonary Window and the Adult Patient

An aortopulmonary window may be overlooked in childhood, especially if pulmonary hypertension is present, and because it often occurs with other more common defects, such as PDA, ventricular septal defect (VSD), atrial septal defect (ASD), Tetralogy of Fallot, or aortic stenosis. Most windows are large, so that, if not recognized in early childhood, they will cause a significant left to right shunt, often resulting in pulmonary hypertension. Therefore, they are frequently associated with high pulmonary blood pressures and pulmonary vascular obstructive disease (PVOD) when first diagnosed in the adult patient.

The symptoms that are associated with this defect depend on the size of the window. Small windows often cause enlargement (dilatation) of the left ventricle and/or heart failure and are characterized by a continuous heart murmur. The more common large aortopulmonary windows result in cyanosis (blueness caused by oxygen poor arterial blood) affecting the whole body, pulmonary hypertension, and Eisenmenger Complex .

Echocardiography is used to confirm the diagnosis and to determine the amount of left to right shunting. A cardiac catheterization procedure may be used to measure the degree of pulmonary hypertension and resistance, if present.

Once the window has been recognized and evaluated, it may be closed surgically or through a catheterization procedure. Regular follow-up after the repair will be necessary and lifelong antibiotic therapy is usually prescribed to guard against infection of the heart’s internal lining (bacterial endocarditis).

Exercise with Aortopulmonary Window

People who underwent repair of isolated aortopulmonary window during childhood may expect normal life expectancy and no restrictions in their activities. Safe levels of activity for patients who received treatment later in life will depend on whether pulmonary hypertension, pulmonary vascular obstructive disease (PVOD), or Eisenmenger Complex are present. Safe levels of exercise should be determined by consultation with your cardiologist.

Anyone with congenital heart disease, repaired or non-repaired, should consult with their cardiologist about physical activity to review the risks.

Pregnancy Issues with Aortopulmonary Window

Aortopulmonary windows which were successfully treated in childhood without the development of other symptoms pose no significant problems for pregnancy. However, pregnancy is dangerous for patients with extant aortopulmonary windows, with significant risks for both mothers and their unborn children.

Anyone with congenital heart disease, repaired or non-repaired, should consult with their cardiologist prior to becoming pregnant to review the risks.