Cardiovascular FAQ


Frequently Asked Questions - Cardiovascular


  1. Are there special risks for PXE patients who need arterial grafts? 
  2. Are Zocor, Lipitor and Pravachol for high cholesterol safe to take for individuals with PXE? 
  3. What are the symptoms of intermittent claudication? 
  4. Is difficulty finding a pulse, except below the jaw, a symptom of intermittent claudication? 
  5. Is stroke a major concern for individuals with PXE? 
  6. Is warfarin safe to take for someone with PXE who has had small and large strokes?
  7. My doctor wants me to take aspirin to reduce the risk of heart attack or stroke. Is this safe with PXE?

1. Are there special risks for PXE patients who need arterial grafts?


PXE affects middle sized and small arteries, causing mineralization in the vessel walls, which stiffens normally elastic tissues. Although coronary disease is not one of the more common complications of PXE, it is well recognized, and it can lead to sudden death, heart attack, and sometimes chest pain even in young individuals. The effect is somewhat like atherosclerosis, producing narrowing of the coronary vessels, and the treatment is not that different. However, revascularization procedures (grafts using arterial tissue from another part of the body to repair damaged arteries) should probably not use arterial vessels as a source because those vessels can be affected by PXE changes as well. Veins are not usually clinically affected, although ultra-structural changes in elastic tissue can be found even in veins.

PXE International is not aware of any studies of the pathology of PXE venous bypasses that are subject to arterial pressures for years. Such vessels become arterialized, but whether they develop PXE-related occlusive disease is not known. Unfortunately, the medical literature contains no good current reviews or large series. [March 2006]

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2. Are Zocor, Lipitor and Pravachol for high cholesterol safe to take for individuals with PXE?


Yes. Zocor (simvastatin), Lipitor (atorvastatin) and Pravachol (prevastatin) are cholesterol-lowering agents, members of a growing class of HMG-CoA reductase inhibitors. HMG-CoA reductase is an essential enzyme that helps convert HMG-CoA to mevalonic acid, an early step in the biosynthesis of cholesterol. The enzyme HMG-CoA reductase is functional only in the liver. There is no evidence that it functions at all in the eye, and it is not present and has no function in systemic blood vessels and retinal vessels, to the best of our knowledge. There is no reason to be concerned about any PXE-specific risk with this medication. [March 2006]

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3. What are the symptoms of intermittent claudication?


The typical symptoms of intermittent claudication are calf, foot, arm or thigh/buttock cramping or tightness with exertion (such as walking, climbing stairs, and running)—the location of the narrowing of the arteries determines where it happens. The important sign of claudication is that it is reproducible—it tends to occur each time after roughly the same amount of exertion or exercise—and is relieved by rest. Tingling and possibly itching are, most likely, not part of this. But if they are, you should experience the symptoms repeatedly, after you do the same amount of exercise. A physical exam, which might include imaging such as ultrasound, is essential to determine whether you have intermittent claudication. Tightness and leg cramping can have other causes. [March 2006] 

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4. Is difficulty finding a pulse, except below the jaw, a symptom of intermittent claudication?


Difficulty in finding pulses is not unusual for people with PXE. However, if there is concern about its significance, Doppler studies (an ultrasound test) can measure the actual pressures in the arms and legs. These tests are readily available and painless and use sound waves rather than x-rays. [March 2006]

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5. Is stroke a major concern for individuals with PXE?


The arteries to the brain are small and middle-sized arteries (except the carotids which are large) and are subject to the same vascular problems as arteries in the leg and the heart. The risk in patients with PXE, particularly for "mini-strokes", is probably higher than in the general population. It is still a small risk, but one with which we are familiar. People who experience warning signs such as transient numbness, weakness, or slurred speech need prompt neurological evaluation and probably need to be on blood thinners if there are no active bleeding problems elsewhere or serious risk of one. The known risks from stroke should be treated whenever possible. If you have questions about specific symptoms, see your doctor. [March 2006]

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6. Is warfarin safe to take for someone with PXE who has had small and large strokes?


This is the perennial question for individuals with transient ischemic attacks (TIAs) or stroke who also have PXE. The risk of further strokes and neurological deficit and the benefits of anticoagulants and platelet inhibitors (aspirin, warfarin, Plavix) have to be weighed against the risk of GI bleeding or retinal hemorrhage as a result of the medications. In a situation with several small strokes having occurred in the recent past, the risk of further strokes likely outweighs the risk of bleeding. Your neurologist should make the decision based on an assessment of the risk of further neurological deficit and stroke. [March 2006]

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7. My doctor wants me to take aspirin to reduce the risk of heart attack or stroke.  Is this safe with PXE?  


While it is not generally recommended that someone affected by PXE take any nonsteroidal anti-inflammatory because it increases the risk of eye or gastrointestinal bleeding, sometimes you have to weigh the risks and benefits. With your doctor, you need to decide if the risk of heart attack or stroke is high, and if it is more important to reduce that risk, than to worry about potential eye or gastrointestinal bleeding.  Read about how KS from Ohio dealt with this issue.

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 Disclaimer


These are replies to general and specific questions which have been submitted to us in the past. Our responses may not apply to any particular individual´s situation and are not a substitute for medical advice given by a physician who is familiar with the individual´s case and who has examined the patient. In addition, the responses are updated on a periodic basis but may not be current.