SINGLE VENTRICLE

ALTERNATE NAMES

Birth Defect – DILV; Common-Inlet Left Ventricle; Common Ventricle; Congenital Heart Defect – DILV; Cyanotic Heart Defect – DILV; DILV; Double-Inlet Left Ventricle; Double-Inlet Ventricle; Single Ventricle Congenital Heart Defect; Univentricular Heart; Univentricular Heart of the Left Ventricular Type

DESCRIPTION

Single ventricle defect is a rare congenital heart defect in which a child is born with only one ventricle that is capable of pumping blood; it may also be associated with other congenital heart defects such as transposition of the great arteries and aortic obstruction. Staged surgery is usually started in the first week of life. Subsequent surgeries maybe required. As the child with a single ventricle ages, potential health problems that develop include complaints of heart rhythm disturbances such as faster than normal heart (tachycardia), atrial flutter, slow heart rate (bradycardia), congestive heart failure, liver and biliary dysfunction, and fluid retention in the abdomen and lower extremities. These children are of greater risk for weakening and failing heart muscle, and for developing blood clots. They also have greatly diminished exercise tolerance, and do not grow well.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing:

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  Imaging studies;

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  Electrocardiogram (ECG);

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  Echocardiogram (ultrasound of the heart); and

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  Cardiac catheterization.

Physical findings: Physical findings associated with a single ventricle include:

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  Features of cyanosis (blue tint to skin and nail beds);

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  Heart rhythm problems;

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  Fluid retention;

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  Blood clots; and

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  Weakening of the heart muscle.

ICD-9: 746.9

ICD-10: Q20.4

TREATMENT

The surgical treatment of single ventricle is based on the severity of the condition. Infants are usually treated with staged surgical intervention beginning in the first week of life. The first procedure is called a shunt. The shunt helps to increase blood flow to the lungs. Subsequent surgeries may be warranted to adjust blood flow to the lungs and to create connections between the veins and the lung arteries. Blood clotting may be a feature of this disease and may require monitoring and anticoagulation therapy. Other health problems are common and require life-long monitoring by a cardiologist trained in the care of congenital heart disease.

PROGRESSION

A diagnosis of a single ventricle defect is usually made shortly after birth. Most children with a single ventricle who have survived to adulthood have had at least one operation to repair the ventricle. Response to surgery varies depending on many factors, including the specific defect and the age at which the child undergoes surgery.

Suggested MER for Evaluation:

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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;

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  Imaging testing (magnetic resonanced imaging (MRI) and X-ray); and

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  Blood study coagulation reports can be used to assess blood clots.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

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