TN 5 (07-11)

DI 23022.545 Eisenmenger Syndrome

COMPASSIONATE ALLOWANCE INFORMATION
 

EISENMENGER SYNDROME

ALTERNATE NAMES

Eisenmenger Complex; Eisenmenger Disease; Eisenmenger Reaction; Eisenmenger Physiology

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. Signs of this condition include: abnormal heart rhythm (arrhythmia), bluish lips, fingers, toes, and skin (cyanosis), heart murmurs (an extra sound when listening to the heart), chest pain, episodes of coughing up blood, dizziness, fainting, fatigue, shortness of breath, and stroke. Some children with this disorder have enlarged fingers and toes (clubbing). 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 for Eisenmenger syndrome include complete blood count (CBC), chest x-ray, MRI scan of the heart, cardiac catheterization, electrocardiogram, and echocardiogram.

   

ICD-9: 745.4

ONSET AND 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.

TREATMENT

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

   

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

Clinical examination describing physical findings that describes diagnostic features of the impairment and laboratory studies are needed to confirm the diagnosis

   

Suggested Listings for Evaluation:

DETERMINATION

 LISTING 

REMARKS

Meets Listing

4.06

104.06

 

Medical Equals

  

* Adjudicators may, at their discretion, use the Medical Evidence of Record or 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 - 07/29/2011
Batch run: 07/29/2011
Rev:07/29/2011