Specialized Care for Patients and Family Members with Bicuspid Aortic Valve Disease

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In this podcast, Dr. Jyothy Puthumana explains how individuals born with bicuspid aortic valve (BAV) may be at risk for future valve damage and aortic aneurysms. Complications include narrowing of the valves (called aortic stenosis), incomplete valve closure causing leaking of the valves (called aortic regurgitation) and also increased risk of infection of the abnormal valve leaflets. Additionally, he will discuss the association of BAV disease and aortic aneurysms. Dr. Puthumana also highlights the comprehensive program components and goals of Northwestern’s Bluhm Cardiovascular Institute BAV Program for patients and family members.

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Transcript

Please remember the opinions expressed on Patient Power are not necessarily the views of Northwestern Memorial Hospital, its medical staff or Patient Power.   Our discussions are not a substitute for seeking medical advice or care from your own doctor.  That’s how you’ll get care that’s most appropriate for you.

 

Well, when you think about little babies growing in utero you want everything to grow just perfectly, and most of the time, certainly in cultures where there’s good prenatal care, that works out that way.  But sometimes there are abnormalities, and one abnormality can be of the heart, and that can be where the connection between the heart and the aorta, that main artery, isn’t quite right.  Sometimes it’s detected, but sometimes not.  It could be that someone is an adult and then there are problems that are observed. 

We’re going to talk to a specialist at Northwestern Memorial Hospital and the Bluhm Cardiovascular Institute about this so that whether it’s a child [or adult] that has this condition that they get the right care so that they can lead a long, healthy life. 

So to help us understand that is cardiologist Dr. Jyothy Puthumana. Dr. Puthumana is a cardiologist and medical director of the bicuspid aortic valve program at Northwestern’s Bluhm Cardiovascular Institute.  He’s also an assistant professor of cardiology at Northwestern University’s Feinberg School of Medicine.  Doctor, welcome to Patient Power. 

This usually causes problems over a lifetime, so we as adult cardiologists commonly see patients in the 35s to 40s who have had some damage to this valve due to the dynamics of flow across a valve that is restricted. The common problems due to this can be damage to the margins of the leaflets that cause the valve to either develop premature degeneration, causing it to narrow in a condition called aortic stenosis, or less commonly, damage to the margins to the leaflet that prevent the valve from closing very well leading to leaking of blood back into the heart from the aorta in a condition called aortic regurgitation or aortic insufficiency. 

So the more recent research has focused on the mechanisms that may be in play.  Like I had mentioned, the initial thinking was that this was purely a hemodynamic phenomenon, or a flow phenomenon, related to the abnormal valve morphology or appearance; however, it appears that there is more to the story where the patients may have not only an abnormal valve that they’re born with but may also have abnormal tissue of the aorta that predisposes them to a ballooning which we see in quite a few of these patients and warrants very close screening. 

So as a response to some of these newer studies that have shown a high inheritability in this condition the American College of Cardiologist and the American Heart Association in its congenital heart disease guidelines have updated their recommendation and at present recommend doing an echocardiogram in all first-degree relatives of patients with bicuspid aortic valve given how high the prevalence of this is among immediate family members. 


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