Introduction:
An aortopulmonary window, also known as the aortopulmonary septal defect, is one of the rarest forms of congenital heart defects. This condition accounts for less than 0.5 % of all forms of congenital heart disease, which means for every thousand cases of congenital heart disease, only five cases account for aortopulmonary window defect. This condition can occur separately or in combination with other forms of congenital heart diseases like the interruption of the aortic arch, ventricular septal defect, or tetralogy of Fallot.
What Is Aortopulmonary Window Defect?
An aortopulmonary window defect is an extremely rare congenital heart disease wherein there is a hole in between the aorta and the pulmonary artery. The aorta is the main artery that is responsible for transporting the blood from the heart to the body, and the pulmonary artery is responsible for the blood movement from the heart to the lungs. Due to the hole in between these two major arteries, the blood gets mixed and flows from one artery to the other. As said earlier, this is congenital; hence it is present from the time of birth itself.
What Are the Causes of Aortopulmonary Window Defect?
The normal functioning of the heart and the arteries is that blood flows from the heart to the lungs through the pulmonary artery, where it becomes oxidized. Now, this blood goes back to the heart, where it is being pumped to the other parts of the body through the aorta. Since there is an opening that joins these two arteries, the blood mixes, and as a result, the pressure in the lungs becomes high. This leads to pulmonary hypertension and then congenital heart failure.
The hole in the arteries occur when there is no proper development of arteries during baby development inside the womb. For a healthy person, there is a proper division between these two arteries for the smooth operation of the heart. So improper development of the arteries during the baby development is the only cause that leads to this aortopulmonary window defect.
This condition can occur separately for about 50 % of the cases, and the rest occurs concurrently along with other heart-related defects such as:
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Atrial septal defect.
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Pulmonary atresia.
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Interrupted aortic arch.
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Patent ductus arteriosus.
What Are the Symptoms of Aortopulmonary Window Defect?
In very rare cases when the defect is very small, it usually does not exhibit many symptoms. But in cases of large defects, which is the most common condition, it is associated with the following symptoms:
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Recurring respiratory infections.
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Delayed growth.
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Irritability.
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Increased heartbeat.
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Rapid breathing.
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Not eating properly.
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Slow in gaining weight.
What Are the Possible Complications of Aortopulmonary Window Defect?
There are very limited complications to aortopulmonary window defect if it is treated earlier. But in case of delayed treatment, then it might lead to other serious complications such as:
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Pulmonary hypertension.
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In some cases, even death.
If the condition is identified earlier and is properly treated with surgery, then the prognosis is stable. As well as, if the condition is diagnosed and surgery is done during the early stage itself, it does not have any long-lasting effect on the child as well.
How Is Aortopulmonary Window Defect Diagnosed?
If the child has any of the above-mentioned symptoms, then it is important to reach out to your doctor immediately, as early treatment is very crucial in these conditions. The doctor will use a stethoscope to hear the child’s heart and listen for any murmur. This is nothing but an abnormal sound of the heart, and it can be easily found using the stethoscope.
Then if the doctor suspects there is any heart-related condition, then he will request the following tests.
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Chest X-Ray: A chest X-ray is done in order to identify any cardiomegaly and increase in the pulmonary vascular marking.
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Cardiac Catheterization: During this test, a thin tube with a small opening is sent through the blood vessels around the heart in order to directly measure the blood pressure in the heart and the lungs. This helps to know if there is any pressure buildup due to the defect.
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Electrocardiograms: This is done to visualize tachycardia and variations in the voltages of the left and right sides of the heart.
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MRI (Magnetic Resonance Imaging) of the Heart: This test will provide an image of the heart using the magnetic resonance imaging principle so that any defect can be easily visualized.
An echocardiogram is the main test that is required because it helps the doctors to clearly visualize the communication between the arteries through the aortopulmonary window. Also, with the help of a CT scan, it is possible to completely get the entire cardiac structures which will help the doctors to find out whether the defect is present separately or with any other heart defects that are mentioned above.
Cardiac catheterization is used if the condition has developed and has not been diagnosed during early childhood. This is because it is quite invasive, and since the defect has been for a relatively long time, the doctors will need to know about the exact pressure inside the heart and lungs to check for Eisenmenger syndrome or pulmonary hypertension.
How Is Aortopulmonary Window Treated?
The aortopulmonary window does not get spontaneously closed on its own, and it is important to know that surgery is the only course of treatment for the condition. There are certain anti-congestion medicines such as diuretics that can help in the temporary management of the symptoms, but it is only carried out as a short-term management method while waiting for the surgery.
In the case of larger defects, open-heart surgery is the only treatment option. That too, it is important to undergo surgery as soon as possible. Also, the success rate of this surgery is pretty good if it is done when the baby is just born because as the child grows, it could lead to other complications over a period of time, such as irreversible pulmonary hypertension.
The surgery involves the opening of the heart and placing a surgical patch as a closure on the defective areas of the arteries. During the procedure, a device known as the heart-lungs machine will take over the functioning of the baby while the surgeons could operate on the aorta and close the opening. This closure patch can be made out of either a man-made material or from a piece of heart closing sac. If the child has any other congenital heart disease, the doctors will plan their course of treatment in such a way that all the necessary procedures are done during the same surgery.
Conclusion:
There are no possible prevention methods to avoid aortopulmonary window defect, but it is important to have a healthy lifestyle during pregnancy as it can help in the baby’s development inside the womb. Avoid drinking alcohol and smoking during pregnancy as it might also lead to certain congenital diseases. But if a baby is diagnosed with an aortopulmonary window defect, then surgery is the only course of action required to have an active lifestyle.
