Little Hearts Matter - Half a Heart ... Not Half a Life
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Medical Information
Antenatal Diagnosis
Normal Heart
Circulation before birth
Conditions and Treatments
Tricuspid Atresia
Pulmonary Atresia
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Hypoplastic Left Heart Syndrome
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Life with a Single Functioning
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Complex Pulmonary Atresia

External Fontan Procedure


Pulmonary Atresia is a Congenital Heart Condition (a problem that a baby is born with). As the heart forms the Pulmonary Valve, at the base of the lung artery (Pulmonary Artery) does not develop and the Right Ventricle does not develop normally and is often small (Hypoplastic).

These defects mean that little or no blue (deoxygenated) blood passes through the lung artery to the lungs. If blood is unable to pass to the lungs through the right side of the heart it will pass through a hole (Atrial Septal Defect) between the right and left collecting chambers (Atria) into the left side of the heart where it mixes with red (oxygenated) blood and passes into the body artery (Aorta). Some blood will then flow to the body and some will pass to the lungs via the connection (Patent Ductus Arteriosus) between the body artery and the lung artery.

The children may be blue at birth, due to a lack of body oxygen and they usually need surgery within the first few days or months of life. It is important to keep the blood vessel between the lungs and the body (Patent Ductus Arteriosus) open after birth as this gives the medical team and parents the opportunity to plan the baby’s treatment path before the child’s condition deteriorates. See Fetal Circulation.



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