Aorto-pulmonary Window (APW) is a very rare congenital heart defect (CHD) which is associated with a hole or window between ascending aorta & pulmonary artery (MPA).
Due to this connection, there is shunting of blood from aorta to pulmonary artery with high pressure.
Generally it is associated with other complex cardiac diseases (like coarctation of the aorta, interrupted aortic arch, ventricular septal defect, tetralogy of fallot, atrial septal defect etc), but can occur as an isolated anomaly.
Effects due to AP window
Excess blood flow from aorta to both lungs leads to very high pressures in both lungs. Blood flow to lungs depends on size of AP window.
If size of AP window or hole is large then it can leads to heart failure and child presents with difficulty in breathing, excess sweating during feeding, poor weight gain etc. With small AP window, child is generally not having symptoms but detected on routing examination by a paediatrician.
If Large AP window not treated on time then it can results in bluish discolouration of lips and fingers and later on results in inoperable state known as Eisenmenger syndrome.
Whenever AP window is present, it is large in size in most of the cases.
Sometimes, it is often missed even during assessment by a cardiologist when AP window is associated with other complex cardiac lesions.
Diagnosis of Aortopulmonary Window (APW)
“Echocardiography of child”is essential for making the diagnosis.
Occasionally, “pediatric cardiac catheterization’’needs to be done for determining whether child is operable or not when AP window is not treated on time.
Other tests that are routinely performed in these cases are chest X ray and ECG.
Treatment of aortopulmonary window (APW)
If AP window is large in size, then only option for treatment is by doing open heart surgery as quickly as possible.
If size of AP window is small, then we can follow up for few months and if suitable then we can close AP window by device closure instead of open heart surgery.
In moderate size AP window, then depending on size and location of AP window, we can plan either device closure or open heart surgery.
Pregnant women with repaired AP window with no signs of pulmonary arterial hypertension (PAH) pose no significant problems during pregnancy.
Pregnancy is contraindicated with established Eisenmenger complex like situation as poses risk both to the mother and child.
Outcome after treatment of AP window
Child who either underwent surgical repair or angiographic closure of AP window (early closure) has no significant problems in long term.