CHILD HEART TRANSPLANT WAIT LIST - STATUS LEVELS 1A/1B

ALTERNATE NAMES

Childhood Heart Transplant Wait List 1A; Childhood Heart Transplant Wait List 1B; Pediatric Cardiac Transplant Wait List 1A; Pediatric Cardiac Transplant Wait List 1B; Pediatric Heart Transplant Wait List 1A; Pediatric Heart Transplant Wait List 1B

DESCRIPTION

A heart transplant is a life-saving surgical procedure to replace a diseased heart with a healthy heart from a deceased donor. Transplant is considered when a child has “end-stage” heart failure due to congenital heart defect or acquired heart disease. This means that the condition has become so severe that all treatments, other than heart transplant, have failed.

Congenital heart defects such as hypoplastic left heart syndrome (HLHS) are the most common causes of end-stage heart failure in children. Acquired heart disease leading to heart transplant in children are less common and includes chronic lung disease, viral infection, and adverse side effect from medications.

Children in need of a heart transplant go through a careful selection process at a heart transplant center. The United Network for Organ Sharing (UNOS) manages the heart transplant waiting list. To be assigned a status level on the waiting list, a heart transplant status justification form must be submitted. After the submission of this form, UNOS assigns a status that reflects the medical urgency for transplant. UNOS will not assign a status without submission of the form.

The status levels for children 17 years old or younger are:

Status 1A: The child must be admitted to the hospital that registered the child for the waiting list and the child must meet at least one of the following criteria:

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  Requires continuous mechanical ventilation;

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  Requires assistance of an intra-aortic balloon pump (IABP);

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  Has ductal dependent pulmonary or systemic circulation, with ductal patency maintained by stent or prostaglandin infusion;

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  Has a hemodynamically significant congenital heart disease diagnosis, requires infusion of multiple intravenous inotropes or a high dose of a single intravenous inotrope. The Organ Procurement and Transplantation Network (OPTN) maintains a list of approved congenital heart disease diagnoses and qualifying inotropes and doses that qualify a candidate for pediatric status 1A; or

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  Requires assistance of a mechanical circulatory support device.

These children have the highest priority on the transplant list.

Status 1B: There is no hospital admission requirement. The child must meet one of the following:

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  Requires infusion of one or more inotropic agents but does not qualify for pediatric status level 1A. The OPTN maintains a list of the approved status level 1B inotropic agents and doses; or

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  Is less than one year old at the time of the candidate’s initial registration and has a diagnosis of hypertrophic or restrictive cardiomyopathy.

Status 2: Children classified as pediatric status level 2 do not meet the criteria for pediatric status levels 1A or 1B but are suitable transplant.

NOTE: Pediatric status levels 1A and 1B are the most severe status levels for children and are considered CAL level conditions. Although severe status level 2 is not considered a CAL level condition.

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

Diagnostic testing: Testing to determine if a child has end-stage heart disease may include:

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  Complete history and physical examination;

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  Blood tests;

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  Viral studies;

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  Urine tests;

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  Chest x-ray;

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  Echocardiogram (echo);

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  Electrocardiogram (ECG/EKG);

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  Cardiac catheterization; and

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  Cardiac magnetic resonance imaging (MRI).

Signs and Symptoms: Some signs and symptoms a child with end-stage heart disease may experience are:

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  Cyanosis (bluish color in the skin, lips, and nail beds);

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  Tachypnea (abnormally rapid breathing);

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  Dysrhythmias (abnormal or irregular heartbeat);

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  Poor perfusion (inadequate circulation of blood through organs and tissues);

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  Feeding intolerance;

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  Chronic cough or wheezing;

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  Fatigue, weakness, faintness;

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  Need to urinate at night;

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  Palpitations (fast or irregular pulse or a sensation of feeling the heartbeat);

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  Swollen liver or abdomen;

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  Weight gain;

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  Edema (swelling caused by too much fluid trapped in the body's tissues);

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  Nausea or lack of appetite; and

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  Tachycardia (a high heart rate).

ICD-9: V42.1; V49.83; 428.0

TREATMENT

Children on the waiting list for a donor heart receive ongoing treatment for heart failure. Depending on the severity of their condition, they may receive a mechanical assist device to help the heart pump blood before the actual heart transplant surgery.

PROGRESSION

Time spent on the heart transplant waiting list is a key factor in determining who receives a donor heart. The wait for a heart transplant could be days to months. Some children may wait years. The amount of time a child waits for a heart transplant depends on several factors including urgency of need, antibody levels, blood type, body size, and the number of donors in the area the child lives in.

A child can be taken off the waiting list due to a serious medical event such as a stroke, infection, or kidney failure.

Donor hearts are in short supply. Some children die while waiting for a suitable donor heart.

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 and procedure reports;

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  Cardiology consultation reports;

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  Imaging studies (ECG/EKG, chest x-ray, MRI) showing heart failure; and

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  Echocardiogram showing indications of the need for mechanical assist devices such as VADs.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

104.02

104.05

104.06

Equals

104.02

104.05

104.06