TN 19 (12-18)

DI 23022.595 Tricuspid Atresia

COMPASSIONATE ALLOWANCE INFORMATION

TRICUSPID ATRESIA

ALTERNATE NAMES

Tricuspid Valve Atresia; Tri atresia; Valve disorder – tricuspid atresia; Congenital heart – tricuspid atresia; Cyanotic heart disease – tricuspid atresia; Congenital agenesis of the tricuspid valve

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 an 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.

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 of developing a focus for subacute endocarditis.

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

Diagnostic testing:

  • Echocardiogram;

  • ECG (electrocardiogram);

  • Imaging studies; and

  • Cardiac catheterization

Physical findings:

  • Cyanosis (bluish discoloration of the skin);

  • Tires easily especially during feeding;

  • Difficulty breathing, and

  • Slow growth;

  • 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.

ICD-9: 746.1

TREATMENT

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

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.

SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
  • Clinical history and examination that describes the diagnostic features of the impairment and response to treatment;

  • Operative reports;

  • Cardiology consultation report;

  • Imaging studies; and

  • Laboratory reports.

Suggested Listings for Evaluation:
DETERMINATION

LISTING

REMARKS
Meets 4.02

4.06

104.06

Equals
* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

 


To Link to this section - Use this URL:
http://policy.ssa.gov/poms.nsf/lnx/0423022595
DI 23022.595 - Tricuspid Atresia - 12/03/2018
Batch run: 12/03/2018
Rev:12/03/2018