Pulmonary atresia
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| ICD-10 | Q25.5 | |
|---|---|---|
| ICD-9 | 747.3 | |
| MedlinePlus | 001091 | |
| eMedicine | ped/2526 ped/2898 | |
| MeSH | C14.240.670 | |
Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs.
[edit] Prognosis
Babies with this type of cyanotic congenital heart disease survive only for the first few days of life while the normal fetal shunts between left and right circulations remain patent.
Without an operation in that period to open the pulmonary valve or to make a shunt between the aorta and the pulmonary arteries, the condition is fatal.
Many children with Pulmonary Atresia will go on to lead 'normal' lives.
[edit] Treatment
The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery.
If they are normal in size and the right ventricle is able to pump blood, open heart surgery can be performed to make blood flow through the heart in a normal pattern.
If the right ventricle is small and unable to act as a pump, doctors may perform operations called the Fontan procedure. In this two-stage procedure, the right atrium is disconnected from the pulmonary circulation. The systemic venous return goes directly to the lungs, by-passing the heart.
[edit] External links
- Pulmonary Atresia information from Seattle Children's Hospital Heart Center
- americanheart.org
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