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 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:
Fifty percent of people 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:
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.
Richard Mainwaring is a Thoracic Surgeon and a Pediatric Cardiologist in Stanford, California. Dr. Mainwaring has been practicing medicine for over 40 years and is rated as an Elite doctor by MediFind in the treatment of Aortopulmonary Window. He is also highly rated in 17 other conditions, according to our data. His top areas of expertise are Pulmonary Atresia, Aortopulmonary Window, Pulmonary Atresia with Ventricular Septal Defect, and Ventricular Septal Defects. He is licensed to treat patients in California. Dr. Mainwaring is currently accepting new patients.
Robert Anderson is an Occupational Medicine doctor in Guilford, Connecticut. Dr. Anderson is rated as an Elite doctor by MediFind in the treatment of Aortopulmonary Window. He is also highly rated in 47 other conditions, according to our data. His top areas of expertise are Partial Atrioventricular Canal, Heterotaxy Syndrome, Ventricular Septal Defects, and Transposition of the Great Arteries. He is licensed to treat patients in Connecticut.
Sachin Talwar is in New Delhi, India. Talwar is rated as an Elite expert by MediFind in the treatment of Aortopulmonary Window. He is also highly rated in 43 other conditions, according to our data. His top areas of expertise are Transposition of the Great Arteries, Ventricular Septal Defects, Congenital Coronary Artery Malformation, and Tetralogy of Fallot.
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:
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.
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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.