Aortopulmonary Window

What is the definition of Aortopulmonary Window?

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 at birth.

What are the alternative names for Aortopulmonary Window?

Aortopulmonary septal defect; Aortopulmonary fenestration; Congenital heart defect - aortopulmonary window; Birth defect heart - aortopulmonary window

What are the causes for Aortopulmonary Window?

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 as a result too much blood flows to the lungs. This causes high blood pressure in the lungs (a condition called pulmonary hypertension) and congestive heart failure. The bigger the defect, the more blood that is able to 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 less than 1% of all congenital heart defects.

This condition can occur on its own or with other heart defects such as:

• Tetralogy of Fallot
• Pulmonary atresia
• Truncus arteriosus
• Atrial septal defect
• Patent ductus arteriosus
• Interrupted aortic arch

Fifty percent of people usually have no other heart defects.

What are the symptoms for Aortopulmonary Window?

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

What are the current treatments for Aortopulmonary Window?

The condition usually requires open heart surgery to repair the defect. Surgery should be done as soon as possible after the diagnosis is made. In most cases, this is 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.

What is the outlook (prognosis) for Aortopulmonary Window?

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

What are the possible complications for Aortopulmonary Window?

Delaying treatment can lead to complications such as:

• Congestive heart failure
• Pulmonary hypertension or Eisenmenger syndrome
• Death

When should I contact a medical professional for Aortopulmonary Window?

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

How do I prevent Aortopulmonary Window?

There is no known way to prevent aortopulmonary window.

REFERENCES

Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 20th ed. Philadelphia, PA: Elsevier; 2017:chap 58.

Qureshi AM, Gowda ST, Justino H, Spicer DE, Anderson RH. Other malformations of the ventricular outflow tracts. In: Wernovsky G, Anderson RH, Kumar K, et al, eds. Anderson's Pediatric Cardiology. 4th ed. Philadelphia, PA: Elsevier; 2020:chap 51.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease in the adult and pediatric patient. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 75.