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Born with AVM: A Silent Killer of Young People – In-Depth Doctor’s Interview

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Neurosurgeon at Washington University in St. Louis, Dr. Gregory Zipfel, talks about a surgery that fixed one woman’s AVM problem.

Interview conducted by Ivanhoe Broadcast News in 2023.

What’s an AVM?

ZIPFEL: AVM is short for Arteriovenous malformations. It’s a condition that you’re born with, and it happens when blood vessels in your body tangle. Most people have it for many years, often for a few decades before they find out about it. An AVM can come to attention in a couple of different ways. The most dramatic is when it bleeds. This can cause rupturing and bleeding – leading to a type of stroke in the brain. In situations like that, the patient needs to go to the emergency department and be cared for urgently to take care of the bleeding; ultimately treating that AVM so it doesn’t bleed again. AVMs can also appear in a couple other less dramatic ways. One being it can cause a seizure. In this case, it hasn’t ruptured yet, but it did cause a seizure. Once we find out about the AVM, oftentimes, it needs to be managed — but it’s because of the seizure we learn of this additional treatment needed. The second common way is just by chance. Someone may get frequent headaches or perhaps was involved in a car accident and decides to get a CAT scan or brain imaging. Through such testing, an AVM incidentally is identified.

For your patient Krysta, the AVM ruptured like a stroke, but her symptoms weren’t typical stroke symptoms, right?

ZIPFEL: Right. Depending on where the AVM is located or where the stroke occurs, the symptoms may or may not be clear-cut stroke type symptoms. Someone who has an abrupt onset of weakness or numbness in an arm or leg or suddenly loses eyesight, speech, or motor function is an individual exhibiting clear cut stroke symptoms. That’s an emergency that needs to be attended to very quickly in the emergency department to figure out why those symptoms occurred, what type of stroke you had, and what the best treatment would be. But sometimes, when the stroke is smaller, or it’s in a less eloquent part of the brain (meaning a part of the brain that’s a little bit more silent) — you can have a stroke, but it’s not as evident that it’s a stroke. This can sometimes delay their arrival to the emergency department or to medical attention. For that reason, location and size of where the event occurs can impact the types of symptoms patients have.

Was it a relatively rare type of stroke? Or is it more common?

ZIPFEL: Yes, AVMs are rare. The most common type of stroke is usually found in older people. They often have underlying blood vessel problems like diabetes, high blood pressure, or high cholesterol. This makes them a greater target for certain types of strokes.

Do those health issues innately come with AVM? Are you born with them too?

ZIPFEL: You’re not born with them. But those kinds of illnesses contribute to atherosclerosis and other conditions that can lead to the most common form of stroke –an ischemic stroke. This is where there’s a blood clot, or some other blockage of blood vessels, stopping blood from getting to a portion of the brain. The brain needs that blood to stay alive but also for nutrition, therefore if you have a blockage of blood flow getting to a part of the brain, it can die and that would be one form of stroke, an ischemic stroke. A less common form of stroke is a bleeding type of stroke, where you generally have a rupture for one reason or another, leading to blood leaving the blood vessel and entering the brain. That’s a less common form of stroke. However, the most common form of stroke that includes bleeding is still in older patients. Patients with high blood pressure is the most common because it makes the blood vessel fragile. It ruptures and causes a bleeding type of stroke. An AVM is something you’re born with, and the bleeding happens often in younger patients — those in their 20s, 30s, and 40s. Now, a young patient with a bleeding type of stroke is unusual and often means there’s something underlying like an AVM, aneurysm, or something of that nature.

When an AVM happens and it ruptures, is the end result a lot like a stroke where you lose function on one side, in speech, and everything?

ZIPFEL: Correct. That’s most common.

When you saw Krysta for the first time, where was she in this whole process?

ZIPFEL: When Krysta first had her symptoms and realized she needed medical attention, she went to a local community hospital and was first evaluated and given a CAT scan of her brain. It was then they discovered she had bleeding, and that the cause of her bleeding type of stroke was an AVM. They decided pretty early on through some family connections in the medical world here in St. Louis that they wanted to come to Barnes Hospital in Washington University. They were familiar with our expertise in strokes, AVMs, and the management of those particular problems. They transferred from the community hospital to our hospital and that’s when I first met her and her family.

Where was her AVM? Was it in a common area of the brain?

ZIPFEL: Yes. Hers was in the frontal lobe, one of the front portions of the brain, but it wasn’t in the most critical parts of the frontal lobe. The most critical part of the frontal lobe affects either speech or your ability to move an arm or leg. Her AVM luckily wasn’t in either of the two critical portions of the frontal lobe, but it was in the frontal lobe. She’d bled there and we quickly realized that she had an AVM. That began a discussion about how to treat the AVM so it couldn’t rupture again. 10% of patients who have an AVM rupture will die because of the initial rupture. Of those who survive, about 20 or 30% will have long-term neurologic deficits because of the damage an AVM rupture causes. The key is once we know about an AVM, especially when it’s ruptured, we want to get it treated and treated pretty quickly so it can’t rupture again and cause those potential problems.

With typical strokes, timing seems to be everything. There’s a 6–8-hour window, I believe, in which help should be sought. Is that the same with AVM?

ZIPFEL: Yes, it is. With ischemic strokes, every hour matters. You need to get to the hospital very quickly to be evaluated. Oftentimes, treatment will be started within minutes or certainly within an hour or so of arrival to the emergency department. Now, from a patient perspective, you don’t know if you have an ischemic stroke or a different type of stroke, so you just need to go and get evaluated. But if it turns out it’s an ischemic stroke, the timing of treatment is within minutes to an hour of presentation, generally. With a bleeding type of stroke, it’s a little bit different. If it’s ongoing bleeding it needs to be treated within minutes to an hour, often with emergency surgery. But a lot of bleeds will start and stop, so when we see patients in the emergency department that’s had a bleed but it’s since stopped, there’s an urgency to it. We need to treat it, but it’s not usually in the order of minutes to an hour. We usually have hours — sometimes 24 hours or something like that to make the treatment decisions, and that’s the case that we had with Krysta.

How did you treat her?

ZIPFEL: Ultimately, for her, she had surgery. For AVMs that are in an accessible area, meaning an area of the brain that we can surgically remove the AVM without causing harm, we like to do surgery as the first line of treatment because we can immediately remove the problem and remove the risk of bleeding. That way we eliminate any chance of the AVM rupturing again. For Krysta, based on where it was located, her young age, and how well she was doing neurologically, we thought that surgery was the right thing to do. Within, I believe about 24 hours of her presentation to us, we took her to surgery to remove the AVM. That surgery went very well. She had a recovery period due to the initial bleed, but ultimately, she was getting back to all her activities including one of her favorite things — riding horses. Thankfully, she eventually got back to all of that after her surgery.

Do a lot of the patients recover fully in that way because they are so young and probably healthy?

ZIPFEL: Yes. Patients who are younger have a better capacity for recovery than patients who are a little bit older. They have more reserve and they’re able to recover more quickly. Krysta’s young age served in her favor. Her bleed was a smaller bleed and in an area of the brain that hadn’t caused a lot of damage. This helped her in terms of her overall recovery and those factors were in our favor. I think getting to the right facility that has a lot of expertise in treating these kinds of patients is also very important. You need to get to a center that sees a lot of this. The neuro critical care team, nurses, physicians, interventional doctors, and whoever else is supporting the patient need to all work well together to get the best outcome.

Is it harder having to see young people suffer from a stroke? You see older people go through that, and that’s hard, but is it more taxing to witness younger people experience it?

ZIPFEL: Yes, it is. I’m of an age now that when I see someone young like that, I think of my daughter. That makes it a little bit more difficult. It certainly impacts you. You consider the decades that young patient, and their family, has in front of them and that impacts you as well. Those things make it difficult, but you’ve got to step back. You need to decouple your emotions and your connection to that family or that situation. You shouldn’t be comparing any medical relationship you have to that of your family. The goal is to do the right thing and help them through that process. But at the same time, treating younger patients in such a condition, and watching them come out on the other side is amazing. They’re able to get back to their lives and to what they’re passionate about and that makes this type of work so ratifying.

END OF INTERVIEW

This information is intended for additional research purposes only. It is not to be used as a prescription or advice from Ivanhoe Broadcast News, Inc. or any medical professional interviewed. Ivanhoe Broadcast News, Inc. assumes no responsibility for the depth or accuracy of physician statements. Procedures or medicines apply to different people and medical factors; always consult your physician on medical matters.

If you would like more information, please contact:

Judy Martin Finch

martinju@wustl.edu

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