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Pulmonary Atresia (PA)
A congenital defect present in one in 10'000 live births, due to abnormal cardiac development in the first trimester or pregnancy, where the pulmonary valve is between the R) ventricle and the pulmonary artery. This late has development issues preventing it from opening, restricting forward flow from the R) ventricle to the lungs.
This is not a threat inutero as the placenta plays the role of the lungs in oxygenation and the actual fetal lungs are not being used. Blood in the right side of the fetal heart shunts through the foramen ovale, allowing oxygen rich blood from the placenta to be go to the left side of the heart and through to the body.
Additionally, these babies may have a ventricular septal defect (see VSD chap for more) info). If this is not present however, the R) ventricle does not develop fully from little blood flow.
After birth when the lungs begin to replace the role of the placenta, with no pulmonary valve opening in the heart, the blood has to take an alternative route to reach the lungs to be oxygenated. The foramen ovale however usually closed in the few first months of life, except in this situation when it is forced to stay open to allow oxygen-poor blood to shunt from the right atrium to the left atrium, into the left ventricle and into the body. This however cannot sustain life long-term, as oxygen poor blood cannot meet body demands.
These babies also have aductus arteriosus (see PDA for more info) to allow some oxygen-poor blood to flow to them units. This however usually closes a few hours or days after birth if Prostaglandin E is not administered.
Signs and Symptoms
Due to the severity of low-oxygenated blood in circulation, these r babies are appropriately labelled with "blue-baby syndrome," and present with severe cyanosis.Symptoms relate to insufficient oxygenation.
☞ Tachypnea/dyspnea
☞ Irritability
☞ Lethargy
☞ Pale, cool or clammy skin
☞ CyanosisDiagnosis
Cyanosis is the initial noted symptom and there will be physical examination and presence of murmur from the turbulence of blood mixing in the heart.Chest X-ray, ECG, Echocardiogram and/or Cardiac catheterisation may be used to look at the anatomy of the heart and assess blood flow. however often this defect is detected prenatally by ultrasound, which will then be followed up with a fetal echocardiogram to learn about the detect.
Treatment
Treatment depends on the baby's age, severity of the defect, tolerance of medications and therapies, and surgical intervention is usually necessary after birth to replace the valve and enlarge the passageway to the pulmonary artery.Lifelong follow up with cardiology is necessary.
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