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:
Congenital heart defects are abnormalities of the structure of the heart present at birth.
Congenital heart disease (CHD) includes Atrial Septal Defect, Ventricular Septal Defect, Complete Atrioventricular Canal, Aortic Valve Stenosis & Insufficiency, Coarctation of the Aorta, Pulmonary Valve Stenosis, Tetralogy of Fallot, Single-Ventricle Defects, Truncus Arteriosus, Transposition of the Great Arteries, and Ebsteins Anomaly.
With modern surgical interventions, over 90% of babies born with a congenital heart defect survive into adulthood. Uncorrected significant congenital heart defects in adults are not very common because most are surgically corrected in childhood.
Even if a congenital heart defect is repaired, complications can later develop. For example, significant pulmonary regurgitation (weakness of the pulmonic heart valve) often follows surgical repair of Tetralogy of Fallot, which can cause dilatation of the right side of the heart, tricuspid regurgitation (weakness of the tricuspid heart valve), arryhthmias (abnormal heart rhythm), and shortness of breath many years after the operation. Significant mitral regurgitation (weakness of the mitral heart valve) may follow surgical repair of an atrial septal defect.
“Symptomatic” means these conditions cause sensations such as shortness of breath, fatigue, palpitations, and fainting.
Appropriate medically acceptable imaging may include ultrasound studies of the heart, such as transthoracic echocardiography (ECHO) and transesophageal echocardiography (TEE). Cardiac catheterization (angiography) can also show CHD.
Listing level CHD requires low blood oxygen levels and associated cyanosis.
A. Cyanosis at rest, and:
Cyanosis occurs due to low blood oxygen levels resulting in a blue tinge to the skin, especially noticeable in the lips, fingers and toes. Some individuals with CHD develop cyanosis only with activity, but Listing 4.06A calls for cyanosis at rest.
1. Hematocrit of 55 percent or greater; or
When oxygen levels are low in the blood, the body attempts to compensate for this by producing more oxygen carrying red blood cells resulting in an increased hematocrit (percentage of blood volume consisting of red blood cells). Normal hematocrit is approximately 38-52% in men and 37-47% in women.
2. Arterial O2 saturation of less than 90 percent in room air, or resting arterial PO2 of 60 Torr or less.
Normal resting arterial oxygen (O2) saturation is greater than 95% and measured by pulse oximetry (sensor attached to the skin).
Normal arterial pO2 is 80-100 Torr and measured with an arterial blood gas (ABG) test by sticking a needle or catheter in an artery.
Listing 4.06A requires an oxygen saturation of less than 90% or an arterial pO2 of 60 Torr or less while breathing room air at rest.
OR
B. Intermittent right-to-left shunting resulting in cyanosis on exertion (e.g., Eisenmenger's physiology) and with arterial PO 2 of 60 Torr or less at a workload equivalent to 5 METs or less.
The left side of the heart gets oxygenated blood from the lungs and pumps it out to the body. The right side of the heart picks up deoxygenated blood returning from the body and pumps it into the lungs to pick up more oxygen.
Some individuals with congenital heart disease develop Eisenmenger’s syndrome. This condition happens when a congenital heart defect allows blood to inappropriately flow from the left side of the heart to the right side of the heart. This shunting of blood causes an abnormal increase in blood pressure in the right side of the heart and in the blood vessels of the lungs (pulmonary hypertension).
Once the blood pressure in the lungs and right side of the heart increases higher than the blood pressure in the left side of the heart, the blood flow through the heart defect then reverses and begins to flow from the right side of the heart to the left side of the heart. This reversal of the shunt now allows blood without oxygen to flow out to the body causing cyanosis due to low blood oxygen levels.
Keep in mind that 4.06B (Eisenmenge’s physiology) requires cyanosis on exertion (with activity) while 4.06A requires cyanosis at rest.
Individuals with Eisenmenger's syndrome have an 80% survival rate at age 10 years, 77% at age 15 years, and 42% at age 25 years. The most common cause of sudden death in these individuals is due to ventricular arrhythmias.
Individuals with Eisenmenger's physiology have very poor functional capacity due to chronically low blood oxygen levels.
Normal arterial pO2 is 80-100 Torr and measured with an arterial blood gas (ABG) test by sticking a needle or catheter in an artery.
Since this Listing requires proof of a pO2 of 60 Torr or less while exercising at a workload equivalent to 5 METS or less, your medical records may not have this type of detailed documentation unless your records contain an exercise stress test with an associated arterial blood gas study. Without this type of test in your medical records, an SSA medical consultant could still look at the entire medical record for consideration of "equalling" this Listing.
As discussed in the section on Chronic Heart Failure, metabolic equivalents of task (METs) is a measure of exercise capacity. For disability purposes, SSA looks at the level of METs you achieve on an exercise (treadmill) stress test.
Approximately 3 METs are required for walking, sitting, or other such low level activities levels.
5 METS is approximately three minutes of walking on a typical Bruce protocol exercise stress test.