Ask Dr. H: Peril of a bicuspid aortic valve

Question:

I was disappointed in your response to a recent question pertaining to the death of Ambassador Richard Holbrooke from a ruptured thoracic (chest) aortic artery dissection. You failed to mention that a bicuspid aortic valve, found in 1 to 2 percent of the population, is found in 7 to 14 percent of all thoracic aortic dissections. Could you please let your readers know about the seriousness of bicuspid aortic valves?

Answer: While there is an increased risk of rupture to the aorta in a person with a bicuspid aortic valve, I do not have any information that indicates Holbrooke had the anomaly of a bicuspid valve.

A normal aortic valve has three leaflets that open and close with every heartbeat to control the flow of oxygen-rich blood leaving the left ventricle - the main pumping chamber of the heart.

A bicuspid aortic valve is a defective heart valve present at birth in 1 to 2 percent of the population. It has a two-leaflet valve opening instead of the normal three. This affects the way the valve opens and shuts and can result in either a leaky aortic heart valve or a narrowed opening that impedes the flow of blood. They often need to be outfitted at some point with a replacement valve.

It so happens that the same congenital defect that created a two-leaflet valve may be associated with weak, abnormal connective tissue in the walls of the aorta. Since the aorta is an artery normally subjected to high blood pressures, weak vessel walls seen in association with a bicuspid valve defect can bulge and develop an eventual aneurysm.

Folks who have a bicuspid aortic valve should receive regular imaging of the heart and the aorta by ultrasound, MRI, or CT every 6, 12 or 24 months - depending upon the age of the patient and stability of the aorta's diameter. Excellent blood pressure control is critical to reduce the risk of an aneurysm. If the aorta is enlarged, I'd avoid weight lifting and frequent straining.

Surgical repair of a ruptured aorta has a 25 to 50 percent risk of death. If there's an aneurysm of the thoracic aorta present at the time surgery is planned to replace the defective aortic valve, it'll usually be repaired as well.

To learn more, check out this Cleveland Clinic link: http://my.clevelandclinic.org/heart/disorders/bicuspid-aortic -valve-disease.aspx

nolead begins

Q:

I have high cholesterol but cannot tolerate any statin medications, including Zocor, Lipitor, and Pravastatin, due to severe muscle pain and cramps. I've also tried taking them with CoQ10 supplements, but that didn't work either. I can't tolerate niacin. My doctor is worried that I'm going to have a heart attack if I don't take something. What can I do?

A: It sounds as if any of the statins taken daily is not going to be tolerated. You've already tried adding the over-the-counter supplement Coenzyme Q10, which does help some folks offset muscle aches and pains that statins may cause. It works by giving your mitochondria a bit of "octane boosting" effect. In cases like yours, I'd suggest 400mg per day of CoQ10.

Since statins are the most effective evidence-based treatment for the prevention and treatment of heart disease and stroke, rather than abandoning them altogether, I would suggest that you see if you can tolerate a long-acting statin drug like Lipitor or Crestor taken only once a week at its starting dose.

There is evidence (American Journal of Cardiology; Feb. 1, 2009) that once-a-week Crestor is safe, generally tolerated and effective in previously statin-intolerant folks like you.

While not as effective as daily dosing, the study showed a 17 percent drop in total cholesterol, a 23 percent reduction in LDL "bad" cholesterol, and a 5 percent increase in HDL "good" cholesterol.

If that's tolerated and your LDL cholesterol is still too high, I'd then try to increase the statin to twice a week and reassess tolerability and lab results. If that's tolerated and your LDL is still not to goal, I'd wait about three months and then try increasing the frequency to three times a week and see how it goes.

I've even had statin-intolerant folks like you do OK on every other day dosing with nearly the same LDL, triglyceride, and HDL results as the daily dosing when using a long-acting statin like Lipitor or Crestor (but not shorter-acting statins like Pravastatin or Zocor).

Weekly, biweekly or otherwise, the bottom line is that anything you can do to improve your cholesterol levels beyond what diet and exercise can do will help reduce your risk of cardiovascular disease.