treatments options

My 7 yr old was just dx's with mild bicuspid aortic stenosis and mod to severe insufficiency. His ejection fraction is 43%. What are his treatment options?

[Jeffrey R Boris, M.D.]

Dear Hfinkhouse,

I have very little information about your son to be able to tell you at this point how to proceed.  It appears that he has a bicuspid aortic valve with mild stenosis and moderate to severe insufficiency (leakage).  His ejection fraction, which is a measure of the squeeze of the left ventricle, is low, which means that the heart is already demonstrating that it is not tolerating the extra volume load of the valve leak.  Thus, your son will need to have the valve either repaired or replaced.  There are different types of surgical choices, including attempting to repair the valve (valvuloplasty), replacing the valve with a tissue valve, replacing the valve with a mechanical valve, or performing a Ross procedure.  Unfortunately, none of these are great options.  Since the aortic valve is bicuspid, there is an intrinsic abnormality of the tissue of both the valve and the aorta around it.  So, repair of the valve may not last very long.  Putting in a tissue valve sounds good, but these do not grow with the child and are often damaged by the immune system.  Mechanical valves don’t grow, either, and require lifelong anticoagulation to prevent clots from forming on the valve.  A Ross procedure, in which his pulmonary valve is used as the replacement aortic valve, and then a pulmonary homograft (a cadaveric pulmonary valve and artery) replace the native pulmonary valve, is not a great option, either.  The replacement aortic valve can dilate and the pulmonary homograft can get damaged by the immune system, causing leakage, obstruction, or both.  Unfortunatly, aortic valve disease is something that children and adults have for life—there is always something wrong with the heart.  In the end, the choice of surgery depends on many things, including his size, age, physical activity, the anatomy of the rest of his heart, the comfort and experience level of the surgeon, and your ability to maintain appropriate anticoagulation therapy (if a mechanical prosthetic valve is used).  Realize that there is a very good chance that he will likely need at least one more heart surgery in his life, if not more, as many of these valves require replacement as they become leaky, obstructed, or outgrown.  And, considering that his left ventricular function is already diminished, there is no guarantee at this time that his heart function will improve to normal.