TN 19 (12-18)

DI 23022.545 Eisenmenger Syndrome

COMPASSIONATE ALLOWANCE INFORMATION

EISENMENGER SYNDROME

ALTERNATE NAMES

Eisenmenger Complex; Eisenmenger Disease; Eisenmenger Reaction; Eisenmenger Physiology; Congenital heart defect – Eisenmenger; Cyanotic Heart Disease –Eisenmenger; Birth Defect heart – Eisenmenger; ES

DESCRIPTION

Eisenmenger syndrome is a rare progressive heart condition that results from uncorrected congenital (present at birth) heart disease. Babies born with this condition have abnormal communication between the normal pumping chambers of the heart. This communication allows blood that has already received oxygen from the lungs to flow back through the lungs, instead of being circulated to the rest of the body. This abnormal blood flow through the lungs, results in higher pressure within the pulmonary circulation damaging these vessels and causing pulmonary hypertension. As this condition worsens and the pressure increases, the backed up oxygen poor blood is then circulated to the rest of the body. Eisenmenger syndrome usually develops before a child reaches puberty, but may develop in adolescence and early adulthood.

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

Diagnostic testing: Diagnostic testing for Eisenmenger syndrome includes:

  • Complete blood count (CBC);

  • Chest x-ray;

  • MRI scan of the heart;

  • Cardiac catheterization;

  • Electrocardiogram; and

  • Echocardiogram.

Physical findings:

  • Abnormal heart rhythm (arrhythmia);

  • Bluish lips, fingers, toes, and skin (cyanosis);

  • Heart murmurs (extra sounds when listening to the heart);

  • Chest pain;

  • Episodes of coughing up blood;

  • Dizziness;

  • Fainting;

  • Fatigue;

  • Shortness of breath;

  • Stroke; and

  • Enlarged fingers and toes (clubbing).

ICD-9: 745.4

TREATMENT

Treatment is aimed at controlling the child’s symptoms, improving quality of life, and the prevention of serious complications.

PROGRESSION

Generally, the presence of Eisenmenger syndrome signals inoperability of the underlying congenital problem. Some children may benefit from heart and lung transplantation or lung transplant.

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

  • Laboratory studies are needed to confirm the diagnosis.

Suggested Listings for Evaluation:
DETERMINATION

LISTING

REMARKS
Meets 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/0423022545
DI 23022.545 - Eisenmenger Syndrome - 12/03/2018
Batch run: 12/03/2018
Rev:12/03/2018