TRICUSPID ATRESIA

ALTERNATE NAMES

Tricuspid Valve Atresia

DESCRIPTION

Tricuspid Atresia is a rare type of congenital heart disease in which the tricuspid valve is missing, abnormally developed or blocked by a solid sheet of tissue. The defect blocks blood flow from the right atrium of the heart to the right ventricle of the heart. As a result, the right ventricle tends to be very small and underdeveloped (hypoplastic). When this type of defect occurs, the right side of the heart cannot properly pump blood to the lungs as it normally would. As a result, the lungs cannot supply the rest of the body with the oxygen that it needs. This type of blockage causes the blood to flow from the upper right chamber of the heart to the upper left chamber of the heart through a hole in the wall between them. This hole is either a heart defect (atrial septal defect) or an enlarged natural opening (foramen ovale) which is supposed to close soon after birth. If the infant does not have a hole through which the blood can flow, surgery may be needed to create and opening.

Some infants with tricuspid atresia also have a hole between the right ventricle and the left ventricle (ventricular septal defect), a condition, which will need to be medically monitored and may require surgical intervention. Infants diagnosed with tricuspid atresia are often cyanotic (bluish discoloration of the skin), tire easily especially during feeding, have difficulty breathing, and exhibit slow growth. These infants may also develop symptoms of heart failure including fatigue, swelling in the legs, ankles, feet and abdomen, sudden weight gain from fluid retention, and irregular or rapid heartbeat.

Adults, who had corrective heart surgeries as infants, may develop problems with their heart functioning later in life. Over time, the surgical treatments that were used at infancy to repair the heart defect may leave scar tissue behind, increasing the chances of abnormal heart rhythm (arrhythmia), and develop a focus for subacute endocarditis.

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

Echocardiogram, ECG (electrocardiogram), imaging studies, and cardiac catheterization

ICD-9: 746.1

ONSET AND PROGRESSION

A diagnosis of tricuspid atresia is usually made shortly after birth. Infants who survive to adulthood may have medical complications requiring multiple follow-up surgeries. Complications such as the formation of blood clots in the arteries of the lungs, strokes, complaints of fatigue, and heart rhythm abnormalities will require life style changes monitoring for infection and life-long follow-up with a cardiologist. If surgical interventions fail, then a heart transplant may be necessary.

TREATMENT

Tricuspid atresia is treated with multiple staged surgical interventions and medications. Multiple surgical procedures will be required during the life of the individual.

Suggested MER for Evaluation:

Clinical description of findings and response to treatment, operative reports, cardiology consultation report, imaging studies, and laboratory reports

Suggested Listings for Evaluation:

DETERMINATION

 LISTING 

REMARKS

Meets Listing

4.02

4.06

104.06

Medical Equals