Pulmonary Atresia (PA) also known as Intact Ventricular Septum, PA/IVS: Pulmonary Atresia means the pulmonary valve hasn’t formed correctly or is closed. The Pulmonary Valve allows blood to flow from the lower right chamber (right ventricle) of the heart to the lungs. Children with PA may also have an underdeveloped tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart. Pulmonary atresia may occur with or without a ventricular septal defect (VSD). Symptoms of PA are similar to other heart defects: bluish skin, shortness of breath, trouble eating, and fatigue.
Causes: Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD.
Possible complications: Children with PA are at greater risk for delayed growth and development, seizures, stroke, heart failure and death.
Treatment: Treatment would typically include medication to help blood move through the lungs. Surgery may also be necessary. There are several surgical options: a thin, flexible tube (heart catheterization) to repair the problem, open heart surgery to repair or replace the valve or to place a tube between the right ventricle and the pulmonary arteries, reconstructing the heart as a single ventricle, or, in extreme cases, a heart transplant. Many children adopted internationally will not have early intervention, which puts them at greater risk for additional complications.
Prognosis: The prognosis for PA is generally very good. However, how well a child does depends on the severity of the defect and whether or not they have additional heart defects.
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