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HYPOPLASTIC LEFT HEART SYNDROME (HLHS)

Hypoplastic Left Heart Syndrome (HLHS) is a rare congenital heart defect. When a baby is born with HLHS, the left side of the heart is severely under-developed. The affected structures of the heart can include the mitral valve, left ventricle, aortic valve, and the aorta. Because the left side of the heart is unable to send enough blood to the body, the right side of the heart must do all the work and maintain the circulation for both the lungs and the body. This extra work eventually causes the right side of the heart to fail. 

There are a number of surgeries that a person with HLHS can have to prolong their life. As the Compassionate Allowance Rule for HLHS states, “palliative surgical intervention is often in the first years of life in a series of three staged surgical procedures. The first surgical procedure called the Norwood operation is done within the first few days of life. A second surgical procedure, called the Glenn shunt or hemi-Fontan procedure, is often done when the child is 4 to 6 months of age. The final surgical stage, stage III, is called the Fontan procedure. Additional surgeries, including possible heart transplantation in surviving children and adults.” See POMS DI 23022.565. Most adults with HLHS do not survive past their thirties.

MEETING LISTING 4.06 FOR HYPOPLASTIC LEFT HEART SYNDROME (HLHS)

This is the criteria the SSA uses to determine if you have a disability under HLHS. A claimant with HLHS meets listing 4.06:

4.06 Symptomatic congenital heart disease (cyanotic or acyanotic), documented by appropriate medically acceptable imaging (see 4.00A3d) or cardiac catheterization, with one of the following:

  1. Cyanosis at rest, and:
  2. Hematocrit of 55 percent or greater; or
  3. Arterial O2 saturation of less than 90 percent in room air, or resting arterial PO2 of 60 Torr or less. 

OR

  1. Intermittent right-to-left shunting resulting in cyanosis on exertion (e.g., Eisenmenger’s physiology) and with arterial PO2 of 60 Torr or less at a workload equivalent to 5 METs or less. 

OR

  1. Secondary pulmonary vascular obstructive disease with pulmonary arterial systolic pressure elevated to at least 70 percent of the systemic arterial systolic pressure.heart disability

HYPOPLASTIC LEFT HEART SYNDROME IS A COMPASSIONATE ALLOWANCE

In August of 2020, the SSA made HLHS a Compassionate Allowance. DI 23022.565 Hypoplastic Left Heart Syndrome

HLHS is a fatal congenital disease, if not surgically treated during the first weeks of life. A newborn with this defect appears normal at birth. Symptoms usually occur in the first few hours of life and include bluish or pale skin color (cyanosis), cold hands and feet, lethargy, and poor feeding. Surgical intervention is usually a series of three staged surgical procedures, as noted above. The child may need a heart transplant as time goes on. Congenital heart defects can be chronic conditions with life-time health implications. Children with HLHS who survive into adulthood may require additional surgical intervention, such as a heart transplant. Lifelong medical follow-up by a cardiologist is necessary.

DIAGNOSTIC TESTING FOR HYPOPLASTIC LEFT HEART SYNDROME (HLHS)

Diagnostic testing: A physical exam showing signs of heart failure (faster than normal heart rate, lethargy, liver enlargement, and rapid breathing) weak pulses at various locations (wrist, groin, and others); and abnormal heart sounds when listening to the chest. Testing may include: cardiac catheterization; electrocardiogram (ECG); echocardiogram; and other imaging studies.

Symptoms and physical findings may include:  bluish (cyanosis) or poor skin color;  cold hands and feet (extremities); lethargy; poor pulse; poor suckling and feeding; pounding heart; rapid breathing; and shortness of breath. 

CANNON DISABILITY CAN HELP YOU WIN YOUR HLHS CASE

The attorneys at CDL have the experience you need to win your HLHS case. Contact us today, whether you live in Utah, Nevada, Idaho, or California, we can help. HLHS is a serious disease, but it is also rare. Therefore, the SSA may not properly evaluate your case. You need the help of an attorney in order to proceed. Contact us today.

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