MITRAL VALVE ATRESIA

Mitral Valve Atresia is a rare congenital heart defect in which the mitral valve of the heart does not develop correctly. The mitral valve normally consists of two leaflets and associated supporting structures. The job of the mitral valve is to allow passage of oxygenated blood from the left atrium to the left ventricle during the relaxation phase of the heart cycle and to keep blood from flowing back from the left ventricle to the left atrium during the contraction phase of the heart cycle. In mitral valve atresia, there is severe reduction or no flow of blood across the mitral valve. When atresia occurs, blood from the left atrium of the heart does not flow to the left ventricle, causing the left ventricle to become small and underdeveloped. Infants surviving into adulthood may develop problems with their heart functioning later in life due to worsening of the condition.

Diagnostic testing:

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  Chest x-ray;

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  Electrocardiogram;

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  Echocardiogram;

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  Cardiac catheterization; and

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  Cardiac magnetic resonance imaging (MRI).

Physical findings:

Infants with mitral valve atresia have the following signs:

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  Difficult or rapid breathing;

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  Shortness of breath;

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  Cool and clammy skin;

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  Blue tinted skin, lips, and nail beds (cyanosis); and

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  Difficulties feeding.   

ICD-9: 746.89

A diagnosis of mitral valve atresia is usually made shortly after birth. Disease progression is variable based on the severity of the congenital heart disease and the response to medication and surgical interventions.

The treatment of mitral valve atresia is based on the severity of the condition. Infants are usually treated with staged surgical interventions and medications. Adults with congenital heart conditions should be monitored by a cardiologist to assess the need for medication and surgery and check for infection throughout their lifetime.

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Operative reports;

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  Cardiology consultation reports; and

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  Electrocardiogram.