Northwestern’s Bicuspid Aortic Valve Program Highlights Trend To Sub-Specialization

Now that my bicuspid aortic valve has been replaced with a mechanical valve do I have to be monitored for future aortic problems? I seem to get conflicting information as to whether I am “fixed” or not.

As for my children, what is the best test to use to evaluate their valve and aorta and how often should they be tested?

Thanks,
Debbie

I was born with BAV, have already had valve replacement surgery. My question is, do my children need to be worried about inheriting this? Have you had Ethan checked?

Debbie,
Great question.
If you’ve had an Aortic valve replaced with a mechanical valve, you’ll need continued monitoring of your proximal ascending aorta ( which is mostly done with an Echo, CT scan or an MRI) , since a good percentage of patients with Bicuspid Aortic valve are at a risk of deveoping Aortic Aneurysms during thier life time.
The frequency of such screening is based on the guidelines published by the AHA/ACC with annual screening recommended once the Ascending Aorta reaches close to 45 mm. Guidelines for screening at dimensions smaller than that are also recommended by the AHA and your Cardiologist would be best able to determine the frequency needed.
As for your children, the best test would be to perform an Echocardiogram which will let you know if they’ve inherited a bicuspid valve or not.
If they do not have a bicuspid valve(BAV), they do not need screening for the valve anymore.However, the risk of family members of patients with BAV developing aneurysms is currently unknown and hence a screening routine has not yet been suggested by any of the relevant Cardiology/Cardiac Surgery societies.

Jyothy Puthumana MD
Medical Director, BAV Program
Northwestern, Chicago

Cindy,
Thanks for the question.
Yes, your children are at a higher risk of inheriting Bicuspid Aortic valve disease since you had a Bicuspid Aortic valve. The literature on the likelihood of transmission seems to suggest that the risk of a child inheriting it may be in the 10 % range.

The AHA/ACC Adult Congenital Heart Disease Guidelines recommend screening of all children of patients who have been identified with a BAV.

Jyothy Puthumana MD
Medical Director, BAV program
Northwestern, Chicago

I am 38 yrs old . Yesterday, I have an echo done and found that my aorta is so dilated and that I also have a bicuspid aortic valve. Now I am to undergo an aortic root repair/ graft and aortic valve replacement…. I am confused of whether I will choose mechanical vs. pig / bovine valve. Please help me decide……also, with the surgery that I am gonna have, do I have risk of redeveloping another aortic aneurysm?

Thank you for your response.

Erwin,
The decision of what kind of valve to choose is personal and best made in collaboration with your surgeon and your support team. The main differences between the valves involve need for medication to anti coagulate (thin the blood) and durability. Mechanical valves are quite durable because they are made of plastic, metal or carbon they last for a long time, however they require the patient to be on a blood thinner, usually Coumadin, for the rest of their lives. Bioprosetic valves (pig/bovine) are made of tissues and do not require anticoagulation, they have been reported to last 10-15 years, however as research is evolving, the lifespan estimates are increasing with one study indicating a 75% lack of valve failure after 15 years. Also if your surgeon determines your valve may be able to be repaired there is the option of a valve sparing root replacement. Adam has an excellent chapter in the book that goes into great detail about the different types of valves and different patient’s experiences. As for your aorta, it will be important for you to continue your care with a cardiologist after surgery as they will ensure appropriate follow up for your aorta over time. BAV is associated with changes in the aorta, so continuing care with you cardiologist will be very important.

Colleen Clennon, RN, BSN, CCRN
Bicuspid Aortic Valve Nurse Clinician
Northwestern’s Bluhm Cardiovascular Institute

Hi there! My husband was born with a BAV, and has had the Ross Procedure done. He also had an aneurysm in his aorta. I was just curious, what are the chances of our children inheriting it? Also, if they are checked and do not have the BAV, should they be monitored for aneurysms? I am hearing a lot of different things, and I am very concerned. Thank you so much for any answers you can give me!

Carlie,

As Dr. Puthumana stated above, “The literature on the likelihood of transmission seems to suggest that the risk of a child inheriting it may be in the 10 % range.”

There aren’t any official guidelines for children on aorta screening other than the initial screening echo. I did check with my colleague at Lurie Children’s Hospital and their standard is if the initial screening echo is normal and they are not yet adult sized they would repeat the echo at regular intervals to check for aortic dilatation. This may be a conservative approach, but it’s their practice so as not to miss anything. If you have other questions you can contact me directly at bav_rn@nmh.org and I can get you in touch with Lurie Children’s.

THANK YOU SO MUCH FOR THE INFORMATION! I truly appreciate your help!