TN 5 (07-11)
DI 23022.540 Aortic Atresia
COMPASSIONATE ALLOWANCE INFORMATION
Aortic Valve Atresia; Aortic Valve Stenosis
Aortic Atresia is a rare congenital heart defect in which there is no opening from the left ventricle of the heart into the aorta. This type of obstruction causes blood flow from the left ventricle of the heart to the body to become interrupted. Because of this blockage, the only other way for blood to flow to the rest of the body is through another structure in the heart called the ductus ateriosus. Aortic atresia usually occurs in combination with other heart defects, such as hypoplastic left heart syndrome. This combination is the most frequent cause of congestive heart failure and death in the neonatal period (the first 28 days of life).
Infants with aortic valve atresia surviving into adulthood may develop problems with their heart functioning later in life due to worsening of the condition. Over time, the surgical treatments that were used at infancy to repair the aortic heart valve may leave scar tissue behind, increasing the chances of abnormal heart rhythm (arrhythmia) and an area for infection called SBE (subacute bacterial endocarditis).
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
The physical findings associated with aortic atresia includes features of cyanosis (blue-tinged skin), dyspnea (shortness of breath), rapidly progressive heart failure with hepatomegaly (enlarged liver), and a gallop rhythm. Chest x-ray, electrocardiogram, echocardiogram, cardiac catheterization, and cardiac magnetic resonance imaging are used to diagnose this condition.
ONSET AND PROGRESSION
A diagnosis of aortic atresia is usually made shortly after birth. Disease progression is variable based on the severity of the congenital heart defects and the response to medication and surgical interventions.
The treatment of aortic atresia is based on the severity of the condition. Infants are usually treated with medications to keep the ductus arteriosis open and staged surgical intervention. Adults should be monitored by a cardiologist to assess the need for medication, surgery, and for heart infections (endocarditis) throughout their lifetime.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Clinical description of findings, cardiology consultation reports, imaging studies of the heart, and blood laboratory testing
Suggested Listings for Evaluation:
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.