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What is Pulmonary Atresia
The lungs are an integral part of making sure our bodies function properly. For life to be sustained in a body, the lungs and heart must work together. The lungs replace carbon dioxide in the blood with oxygen, and the heart pumps oxygen-poor blood out of the organs and muscles, and replaces it with oxygen-rich blood.
Sometimes defects in other areas of the body prevent the lungs from doing their work. One of those defects is a congenital heart defect known as pulmonary atresia.
The heart has two pumping chambers (right and left ventricles). Each ventricle pumps blood through a valve to an artery. The right ventricle pumps blood to the lungs through the pulmonary artery. Where the pulmonary artery joins the heart there is a valve which allows blood to flow in one direction and prevents it from going backwards. This is the pulmonary valve (http://pediatriccardiology.uchicago.edu). Pulmonary atresia means that the pulmonary valve has not developed completely and is unable to open correctly, so blood can not travel from the right ventricle to the lungs to be oxygenated. Since the blood cannot be oxygenated and then circulated through the body, the body - including the heart - will stop functioning very quickly.
Pulmonary atresia is the tenth most common type of heart disease in newborns and occurs in one in every 14,000 births.
Diagnosis and Symptoms
Pulmonary atresia rarely appears on its own. It is usually accompanied by other defects, but symptoms will appear shortly after birth, if not discovered during routine prenatal examinations or ultrasounds. All children appear bluish upon delivery, but grow pinker as oxygenated blood flows through the body. Those babies with pulmonary atresia will grow paler or bluer within a few moments of birth. Diagnosis will be confirmed through a sonogram of the heart (fetal echocardiogram or ECG).
Besides cyanosis (blue tinge to the skin), other symptoms include:
- shortness of breath
- difficulty breathing
- murmur
- irritability
- low energy
- clammy skin
Standard Treatment Protocols
Once pulmonary atresia has been officially diagnosed, the next move is into surgery. Until the surgery takes place, the infant will be kept in intensive care to receive oxygen treatments and medication that will temporarily keep the ductus arteriosus open. This duct usually closes shortly after birth, and can actually lead to heart failure if left open too long, but in the short-term it will allow blood to flow to the lungs to be oxygenated, before being pumped through the rest of the body.
While the ductus ateriosus is open, doctors will usually place a shunt, which will be followed by placement of a Glenn and then a Fontan a little later - each surgery is intended to improve upon the heart's function as the child grows. Valve replacement is also an option in certain cases, but the valves need to be replaced every five years.
Cardiac catheterization may also be done, which is a non-surgical procedure and may involve the use of a balloon (balloon atrial septostomy) to improve the mix of oxygen-rich blood and oxygen-poor blood between the left and right atria.
Whether surgery or catheterization is warranted depends on the severity of the defect.
Most children who undergo the full surgical repair will go on to lead normal, fully active lives. Some children will require a heart transplant 15 to 30 years following the placement of the Fontan.
Sources: http://pediatriccardiology.uchicago.edu; www.aboutkidshealth.ca; www.wisegeek.com; www.americanheart.org
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