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There are many ways you can help children and their families get the care they need.
Babies with pulmonary atresia need some type of treatment soon after birth. The specific type of treatment your baby needs will depend on the severity of his or her condition. There are a few types of treatments for pulmonary atresia.
The doctor may give your child an IV (intravenous) medication called prostaglandin to keep the ductus arteriosus open. The ductus arteriosis is the connection between the aorta and the pulmonary artery that is present in all babies before birth. This connection usually closes shortly after birth, but when kept open with medication, it can allow blood to continue to flow to the lungs until doctors decide on a long-term solution.
A cardiac catheterization is often performed to determine how blood is supplied to the heart in patients with pulmonary atresia with intact ventricular septum (PA/IVS). Using this test doctors can tell if the blood supply to the heart is dependent upon flow directly from the right ventricle through abnormal coronary connections called fistulae. If the blood supply is dependent upon the right ventricle, the series of surgeries listed below are usually recommended. Sometimes, the first surgery can be replaced with a procedure to stent open the ductus arteriosis in the catheterization laboratory.
If the cardiac catheterization shows that the blood supply is not dependent upon the right ventricle, and the blockage is short, your doctor may recommend that the pulmonary valve is reopened in the catheterization laboratory, which involves crossing the pulmonary valve with a wire or a radiofrequency ablation catheter. A special balloon can then be expanded in the middle of the valve to reestablish blood flow from the right ventricle to the lungs, allowing the right ventricle to grow over time. In many cases it can allow for a biventricular repair and may reduce the number of surgeries. In some cases, it may eliminate the need for surgery altogether. If the pulmonary valve cannot be opened in the catheterization lab, then the connection between the right ventricle and lungs may be reestablished with surgery.
Many children with pulmonary atresia need one or more surgeries to fix the problem. These surgeries may include:
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