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.
Aortopulmonary septal defect; Aortopulmonary fenestration; Congenital heart defect - aortopulmonary window; Birth defect heart - 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 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 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 patients usually have no other heart defects.
If the defect is small, it may not cause any symptoms. However, most defects are large.
Symptoms can include:
- Delayed growth
- Heart failure
- Poor eating and lack of weight gain
- Rapid breathing
- Rapid heartbeat
- Respiratory infections
Exams and Tests
The health care provider will usually hear an abnormal heart sound (murmur) when listening to the child's heart with a stethoscope.
The provider 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
- MRI of the heart
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.
Surgery to correct aortopulmonary window is successful in most cases. If the defect is treated quickly, the child should not have any lasting effects.
Delaying treatment can lead to complications such as:
When to Contact a Medical Professional
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.
There is no known way to prevent aortopulmonary window.
Hirsch-Romano JC, Ohye RG, Si M-S, Bove EL. Truncus arteriosus and aortopulmonary window. In: Sellke FW, del Nido PJ, Swanson SJ, eds. Sabiston and Spencer Surgery of the Chest. 9th ed. Philadelphia, PA: Elsevier; 2016:chap 121.
Park MK. Miscellaneous congenital cardiac conditions. In: Park MK, ed. Park's Pediatric Cardiology for Practitioners. 6th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 15.
Reviewed By: Scott I. Aydin, MD, Assistant Professor of Pediatrics, Albert Einstein College of Medicine, Division of Pediatric Cardiology and Critical Care Medicine, The Children's Hospital at Montefiore, Bronx, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.