Disclosure Information: Dr. Kazamel has the following financial relationships with commercial interests:
Consulting Fee – Akcea Therapeutics; Bio Stealth Therapeutics
Dr. Kazamel does not intend to discuss the off-label use of a product. No other speakers, planners or content reviewers have any relevant financial relationships to disclose. There is no commercial support for this activity.
TranscriptionMelanie Cole (Host): Modern autonomic function tests can noninvasively evaluate the severity and distribution of autonomic failure. My guest today is Dr. Mohamed Kazamel. He is an Assistant Professor in the Division of Neuromuscular Disease in the Department of Neurology at UAB Medicine. Welcome to the show, Dr. Kazamel. Let's start with the grouping of the autonomic disorders that we are discussing here today.
Dr. Mohamed Kazamel (Host): Thanks, Melanie, for having me. In fact, the autonomic disorders that we are able to test for are divided into different categories, which include disorders of maintenance of the blood pressure like orthostatic hypertension and the famous postural orthostatic cardio syndrome, which is referred to as POTS. We also try to test for growing disorders of the peripheral nervous system called “small fiber neuropathy”. And, small fiber neuropathy happens when a patient comes to our office complaining of tingling or burning sensation in their feet and we perform the regular routine nerve conduction studies on them and these studies come back normal. So, there is no effective way of evaluating the small fiber neuropathies other than performing autonomic function testing on them and also performing skin biopsy. The third category of the autonomic dysfunction diseases include the central neuro degenerative diseases like multiple system atrophy and the different Parkinson's plus syndromes. These disorders manifest with the movement disorders; the tremors, the dementia and all of this. They also present with dysfunction in the autonomic functions. Oftentimes the patients with multiple system atrophy, they develop significant hypotension and orthostatic intolerance when they stand up. And, they cannot be distinguished that much from advanced Parkinson's disease patient, because in advanced Parkinson’s you have the same hypotension and also the treatments that are used for Parkinson’s to lower the blood pressure. So, we need different autonomic function testing to differentiate severe neuro degenerative disease, multiple system atrophy from just a Parkinson’s disease case.
Melanie: What other symptoms would present with a patient that would indicate the autonomic testing?
Dr. Kazamel: So, symptoms that indicate autonomic testing, again, if the patient has painful burning sensation in their feet or they have tingling sensation while we perform the nerve conduction study and find them normal, then they have to referred for autonomic function testing. Also symptoms of orthostatism in general, like inability to maintain the blood pressure after standing. These patients start developing symptoms of cerebral hypo-perfusion like headaches, dizziness, giddiness, blackouts, pain between the shoulder blades and some of them also develop symptoms of adrenergic activation like feeling of heart beat racing, chest pain, tremors, increased anxiety, all of these.
Melanie: And, if they present with some of these symptoms you have discussed, what are some of the tests that you are using now, Dr. Kazamel?
Dr. Kazamel: So, the five standardized autonomic function tests that we use here in the lab include, first the QSART or the commercially available option, which is called a Q-sweat. QSART stands for quantitative sudomotor axonal reflex testing and this test measures the sweat response in different parts of the body, including the forearm, the proximal leg, the distal leg and the foot. So, what we do is that we stimulate the skin sweat glands with iontophoresis of acetylcholine and we measure the sweat response from these areas. And depending on which areas in the body that do not produce too much sweat, we defined what is the distribution of the small fiber neuropathy. We also do the heartrate response to deep breathing and this is one of the most sensitive testing for evaluation of the vagus nerve function. What we do is we ask the patient to breath heavily for six or seven cycles and then we evaluate their heart rate while they are breathing heavily. We average the mean difference between the maximal heartrate and the minimal heartrate and if that means difference decrease, that is an early sign of vagal neuropathy. We also do the Valsalva maneuver, where we try to record the heartrate and blood pressure response to patients when they strain. During the Valsalva maneuver what we do principally is have them forcibly exhale through a bugle, generating a 40 millimeter mercury forcing expiratory pressure for 15 seconds, which is very laborious job. We try to generate a curve of how their blood pressure and their heartrate respond to that. We also do the head-up tilt table testing and we measure the blood pressure for the patient while they are supine and then we tilt them over for three seconds to an upright 70 degrees angle, and we leave them in that position for like 10 minutes and we record how their blood pressure and heartrate respond to that. Our final test is a very unique test which is a thermoregulatory sweat test and in the last test, we depend on a certain type of powder called Alizarin S Powder, which has a unique character of changing its color when it gets exposed to sweat. So, what we do is we bring the patient, dust them all over the body with this powder - and the powder while its dry, its yellow in color and when it gets wet with sweat, it changes its color to purple. And then, after we dust the patient with this powder, we insert them into what's called the autonomic chamber and try to raise their core temperature only one degree, from 37 centigrade to 38. And, as they start to sweat, the powder changes its color and that tells us which part of their body does not sweat and certain patterns on that test leads to different diseases. So, these are the five different modalities of testing that we perform in the autonomic function testing lab.
Melanie: Dr. Kazamel, when you are using the tilt table and doing Valsalva, are there certain medications that you'd like your patients to not take that day of the testing that might interfere with sweating before the test?
Dr. Kazamel: Well, that's a very, very important question. So, certain over the counter medications like allergy medications, the anti-histamine medications like the Benadryl, for instance. We try to ask our patients not to take them in the 48 hours before testing, because these medications have anticholinergic properties and they decrease the amount of sweating. So, they interfere with our testing. Other medications that we ask the patients to stop, too, like the alpha agonist including the prostate medications and the beta blockers. However, for the beta blockers that are used in treatment of hypertension or cardiac dysrhythmias, we are sometimes able to leave that to the discretion of the prescribing physician because stopping those medicines, even for 24 hours, can be a problem for the patient. So, these are the three or four different categories of medications that we would like the patient to stop before performing the test. Also, we would like them not to smoke or drink caffeine within the last three hours before testing and we would like them not to drink alcohol on the night before testing.
Melanie: And, for more information about testing results and making a differential diagnosis, you can visit uabmedicine.org/learnneuro. And, then, in just the last few minutes Dr. Kazamel, how can a community physician refer a patient to UAB Medicine?
Dr. Kazamel: So, we are working currently on our website that has a link that the community physician can fill out a form and fax it over to us in the Neuromuscular Diseases Division. Also, they can call our office at UAB Neuromuscular Disease Division for referrals.
Melanie: And, tell us about your team. Why is UAB so great to work with?
Dr. Kazamel: Well, UAB has a lot of potential across. We here have very large department of neurology that includes more than 65 physicians and scientists in different categories in sub-specialties of neurology. And, we all collaborate with each other to provide the excellent standard of care to our patients. On the other side, we also have major collaborations in research and we believe that the autonomic function testing lab will provide excellent service to our patients and to our community physicians in different aspects. We expect referrals to come from neurologists, we expect referrals to come from cardiologists and family physicians and interns.
Melanie: Thank you so much for being with us today, Dr. Kazamel. You are listening to UAB Medcast. For more information on resources available at UAB Medicine, you can go to www.uabmedicine.org/physician. That's www.uabmedicine.org/physician. This is Melanie Cole. Thanks so much for listening.
Post Test URLhttps://cmecourses.som.uab.edu/mod/quiz/view.php?id=6071
Guest BioDr. Brad David Denney is a native of Birmingham and graduated from Mountain Brook High School. Dr. Denney received his bachelor's degree from The University of Georgia, graduating Summa Cum Laude. He then received his medical degree from the University of Alabama School of Medicine in Birmingham.
CME Post Test Information: Release Date: December 5, 2019 Reissue Date: November 14, 2022 Expiration Date: November 13, 2025
Disclosure Information: Planners: Ronan O’Beirne, EdD, MBA Director, UAB Continuing Medical Education Katelyn Hiden Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Faculty: Brad Denney, MD Assistant Clinical Professor in Plastic Surgery
Dr. Denney has no relevant financial relationships with ineligible companies to disclose. There is no commercial support for this activity.
TranscriptionMelanie Cole, MS (Host): UAB Medcast is an ongoing medical education podcast. The UAB Division of Continuing Education designates that each episode of this enduring material is worth a maximum of .25 AMA PRA Category 1 credit. To collect credit, please visit uabmedicine.org/medcast and complete the episodes post-test.
Abdominoplasty is one of the most commonly formed aesthetic procedures and has undergone a significant evolution over the past several decades. My guest is Dr. Brad Denney. He’s an assistant professor and double board certified plastic surgeon at UAB Medicine. Dr. Denney, pleasure to have you with us as always. Tell us a little bit about tummy tucks and liposuction, abdominoplasty. What's different now? What are you doing different than maybe 20 years ago?
Brad Denney, MD (Guest): First of all, I think it’s important to know who to consider for a tummy tuck. That’s one thing—People hear the terms tummy tuck and abdominoplasty and they're basically one in the same. Someone who may benefit from a tummy tuck or abdominoplasty is someone who’s had children and they want some improvement in their abdominal contour and in the aesthetics of their abdomen. Because very commonly after pregnancy ladies develop what’s in the medical terminology as rectus diastasis. That’s where the six pack muscles widen because the baby pushes on the stomach. Then after the baby’s born, things don’t come back together the way they were before pregnancy. So a tummy tuck or an abdominoplasty takes care of actually three things. It takes care of any excess fat deposits and we do that by liposuction. It takes care of the rectus diastasis because we will suture those abdominal muscles back together, and then it takes care of any excess skin that may have occurred from this phenomenon. We do that by excising the excess skin. All together that results in improvement of the abdominal contour and the abdominal aesthetics.
Now the tradeoff for that is, of course, we have to do a scar. The scar is typically placed where the bikini line is. So we try to put it low enough where the scar is not noticeable say in a bikini. In terms of how we have done things differently and how advances that we’ve made, we used to not do this procedure combined with liposuction. However, now it is very common to combine a tummy tuck or an abdominoplasty with liposuction because that allows us to taper or feather down the local fat deposits that are seen on the flanks and therefore improving the aesthetics of the abdominal silhouette.
Host: So do you feel that doing both together is gonna give you the patient, give the patient the best results? How do you determine whether to do them both or just one or the other?
Dr. Denney: Most of the time we do liposuction and abdominoplasty at the same time. However, there are some patients who may not need the liposuction. The liposuction just treats those focal excess fat areas particularly in the flanks and it an be used to decrease the thickness of the layer of skin and fat of the abdominal wall. There are some patients who don’t need this, who don’t need the liposuction because quite frankly they may not have a lot of fat to liposuction, but they may have the rectus diastasis that I mentioned as a result of pregnancy. In that case, those patients often need what is referred to in kind of the general public as a mini or a mini tummy tuck or a mini abdominoplasty. That is where the incision within the bikini line is much shorter than the traditional abdominoplasty. The abdominal skin and fat is elevated off of the rectus muscles, the rectus diastasis is corrected by a suture repair, and then any excess skin—which is usually minimal—is removed. In that situation because the patient does not have excess local fat deposits, liposuction’s not necessary.
Host: Dr. Denney, is this an option for obese patients or previous bariatric patients? Can a high body mass index cause complications you might not otherwise see?
Dr. Denney: That is a great question. I think that is important for everyone, patients, to know that tummy tucks and abdominoplasties and liposuction, these are not weight loss procedures. Weight loss procedures are gastric bypasses. Tummy tucks, liposuction, these body contouring procedures are exactly that. They are body contouring procedures. They are not weight loss procedures. In fact, in order to be a good candidate for these procedures, you need to be at your ideal body weight or have a BMI less than 35. That is for several reasons. Number one, if you are at your idea body weight or if your BMI is less than 35, you will have a better aesthetic result following liposuction or an abdominoplasty. Number two, complications following surgery are much less when you are at your ideal body weight or if you have a BMI of less than 35.
A high BMI correlates with a higher risk of complications. These complications can be anything from a DVT or pulmonary embolism to wound dehiscence and problem with wound healing. DVT and pulmonary embolism is something very important to consider with this operation. Because we are tightening the abdominal muscles, we are increasing the pressure on venous blood flow return to the heart. This puts the patient at higher risk for blood clots or DVTs which could eventually become pulmonary embolism in which the worst case scenario can be fatal. So on all our patients we risk stratify them for that risk. If they are at a high enough risk based on something what's called the Caprini score then we will actually keep them on Lovenox injectables at home for up to a month at home after surgery. So achieving an ideal BMI or an ideal weight prior to these operations is very important.
In terms of massive weight loss patients, these patients are absolutely candidates for an abdominoplasty. The difference being is several. One, not only do we want them to be at their ideal body weight or a BMI less than 35, but we also want them to be at least 12 to 18 months out from their gastric bypass which usually correlates to having their weight stable for six months. The reason being is if they're still actively losing weight and we put them through surgery they're at a higher risk for wound healing complications. The other reason is they get a better result aesthetically once they're at their stable weight. The major difference in an abdominoplasty and someone who’s had massive weight loss versus someone who has not is the incision may tend to be longer or larger because we have to excise a larger amount of skin due to the excess skin that develops due to the massive weight loss.
Host: Thank you so much for that answer. What a comprehensive answer that was. Tell us a little bit about some of your outcomes, and what can a patient or referring physician expect post-operatively?
Dr. Denney: Outcomes are excellent. Abdominoplasty has one of the highest patient satisfaction scores of plastic surgery. In terms of return to work, not off work. Something that’s hard for some patients is a lot of these patients have just finished having children. That’s one thing. If they want to have a tummy tuck, they should do so knowing that they're done having children because we don’t want to tighten the abdominal muscles and then they decide to have another child. It makes undergoing pregnancy more difficult. After surgery I tell patients to take two weeks off of work. They can't lift anything more than five pounds for four to six weeks. They’ll also be kind of hunched over when they're walking for about a week or so because we’ve tightened the skin at the bikini line. So it’s going to be a little tight there, but eventually after about a week or so they're walking upright again. In terms of referral to our team, we have clinics at the Kirkland clinic and at our Mountain Brook office as well. Those can be generated through 871-4440 or 205-801-8500.
Host: Do you have some final thoughts which you’d like other providers to know about when it’s important to refer if they have patients that are questioning having abdominoplasty, questions about it, what would you like them to know?
Dr. Denney: I think the best timing to refer a patient in terms of weight loss is if the patient has had their weight stabilized for more than six months and if their BMI is less than 35. For those who are not massive weight loss patients but those patients who have completed childbirth and they have expressed interested in obtaining a more aesthetic abdomen and improving their abdominal contour then I think that’d be the best time for referral.
Host: Thank you so much Dr. Denney for joining us. A community physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST. That was another episode of UAB Medcast. For more information on resources available at UAB Medicine, please visit our website at uabmedicine.org/physician. Until next time, this is Melanie Cole.
Disclosure Information: Release Date: January 3, 2017 Reissue Date: April 21, 2023 Expiration Date: April 20, 2026
Planners: Ronan O’Beirne, EdD, MBA Director, UAB Continuing Medical Education Katelyn Hiden Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Faculty: Marc G. Cribbs, MD Director, Alabama Adult Congenital Heart Disease Program Assistant Professor of Cardiology, Congenital Cardiac Disease
Dr. Cribbs has no relevant financial relationships with ineligible companies to disclose. There is no commercial support for this activity.
TranscriptionMelanie Cole (Host): Treating congenital heart defects in adults is aided by the understanding of the continuum of the disease from its infancy. My guest today is Dr. Mark Cribbs. He’s the director of Alabama Adult Congenital Heart Program at UAB Medicine. Welcome to the show, Dr. Cribbs. The American College of Cardiology and the American Heart Association have developed standards for treating adults with congenital heart disease. What are some of the challenges that you see in treating these adults?
Dr. Mark Cribbs (Guest): Probably one of the biggest challenges that we face is just the patients not coming for their appointments. In other words, there are a significant number of patients who are lost to follow-up well before they even reach their 18th birthday. A recent study has demonstrated that as many 50% of patients stopped seeing their cardiologist by the time they reached the age of 13 and that percentage does continue to climb as they get older in age. So, even by the time they reach 18 years of age, more than 60% of the patients that have mild, maybe even moderate, or even severely complex congenital heart disease have stopped going to see their doctor.
Melanie: In this continuum of care, how often should patients be seen throughout their lives?
Dr. Cribbs: That’s a great question and it’s one that patients ask me all the time. It really does depend on their cardiac history. For example, someone who has a history of a simple VSD that was closed very early in life, they really might not need to be seen but every two or three years. While someone who has a single ventricle may need to be seen as often as every three to six months.
Melanie: Because there are so many different types of congenital heart disease, Dr. Cribbs, the guidelines include general recommendations that apply to most patients but give us some examples of low risk, simple, congenital heart disease.
Dr. Cribbs: So, as I mentioned before, a VSD that’s been closed early in life is one of our more simple defects; however, a simple VSD can very much range in the spectrum. It really just depends on the size. So, if it was a small VSD that was closed early on, the risks that we expect for that person to have later on down the road are really quite low. However, if it was a very large VSD that was closed later on in life, the patient may have already developed significant symptoms such as pulmonary hypertension. Then, further on in the spectrum would be something like Tetralogy of Fallot which is considered a complex congenital heart defect which has a number of issues that can occur down the road including significant pulmonary insufficiency, dilation of the right ventricle, shortness of breath, and very significant arrhythmias.
Melanie: So, when you were talking about single ventricle, so are these along the lines of examples of highly complex congenital heart disease?
Dr. Cribbs: Absolutely. They are, by far, our most complicated patients that we take care of; however, they are doing very, very well and leading productive lives out in the community and oftentimes having families of their own.
Melanie: If cyanosis is present, what are some other health problems that can result from that?
Dr. Cribbs: So, cyanosis can lead to the ability of a clot for example that might develop in the leg after a long car trip to travel up to the heart and then scoot over to the left side of the heart which would then have free reign to either go into the coronary blood vessels themselves or up to the brain and cause a significant stroke. The other things that we see related to cyanosis are an elevated hematocrit and I think one of the long-term misconceptions related to an elevated hematocrit is that it, by itself, can cause a higher viscosity in the blood and increase in someone’s risk for having a thromboembolic phenomenon, such as a stroke. But, what we’ve learned is that having a high hematocrit doesn’t, in and of itself, put that person at risk for that, it’s when their iron levels are low. That’s what can really increase the risk. The reason why I bring that up is that patients that have a high hematocrit are often sent to our office and tell me that they’ve had multiple times when blood has been removed from their body in order to try to lower their hematocrit and that has really been seen to be more detrimental than good, more harmful than good, especially if their hematocrit has not being causing of strokes.
Melanie: What are some other comorbidities that can arise with congenital heart disease in adults?
Dr. Cribbs: Probably the most common comorbidity that we see, and actually one of the more common reasons that patients re-present to care is the issue of arrhythmia. Pretty much every person that has had a congenital heart surgery is going to be at risk for an atrial-type arrhythmia. Most often, it’s an atrial flutter. The fortunate news about that is that it’s easily treatable in the EP lab. In fact, more than nine times out of ten, it can be cured. The other types of arrhythmia that we encounter in patients with different types of defects however are ventricular arrhythmias and those can lead to sudden cardiac death. Perhaps the other very, very common comorbidity that we find in patients as they age is the issue of heart failure and actually heart failure and arrhythmias are the most common reason why adults with congenital heart disease actually succumb to their disease and die.
Melanie: If they were coming to you on a regular basis, are you doing testing on them? Is there exercise testing, stress testing involved?
Dr. Cribbs: Absolutely. So, it really, again, gets back to what their heart history is like. For someone who has a history of a VSD that was repaired early in life, they’re at a small but present risk of atrial arrhythmia and so if they are complaining of palpitations, we would certainly do monitoring such as a Holter monitor or an event monitor, certainly an EKG. Someone who has a history of Tetralogy of Fallot, where the risk of atrial arrhythmia of even ventricular arrhythmia is much higher, we will routinely screen them with monitors to make sure that we can catch something that they might not even feel. And then, when it comes to stress tests, we do that on a number of patients, particularly when they complain of symptoms such as shortness of breath or chest discomfort when they’re trying to be active or exercise.
Melanie: Are patients more at risk for infective endocarditis?
Dr. Cribbs: That’s another good question that patients ask me a lot and what we’ve found is that the risk of endocarditis is actually probably lower than what we believed it to be in the past. Those who are at risk for endocarditis are those who have prosthetic valves, those who have a history of endocarditis are certainly at risk for having it again. Those patients who remain cyanotic and then those who have a small, residual defect where the surgeon tried to close a hole, for example, a VSD that might not have been closed all the way where there’s a residual defect. Those patients are also at risk for endocarditis. But, by and large, the majority of our congenital heart patients are not at an elevated risk of endocarditis.
Melanie: How important is it that these patients see an experienced, adult congenital disease heart center?
Dr. Cribbs: The adult congenital center that’s experienced with these types of patients is going to be able to offer them a full range of things that they would need--everything from an EEG to an echo to advanced imaging such as cardiac MRI or a cardiac CT to stress test to CAT and so forth. It’s really the experience and the knowledge of the congenital heart defect that helps these patients the most. There’s a number of places in Alabama, for example, where a person could get a cardiac MRI but then the person who’s reading the test may not fully understand the congenital heart defect itself. So, they might get good pictures but they may not be able to interpret all the details the way that someone here at UAB could.
Melanie: And, in just the last few minutes, Dr. Cribbs, how could a community physician refer a patient to UAB Medicine ACHD Center?
Dr. Cribbs: Right. So, one of the easiest ways is to go online to our website which is at UABMedicine.org. However, perhaps the easiest way to do it is just to call. The number is (205) 996-9000 and our schedulers work very hard to get patients in quickly and we really do try to “bend over backwards” to get these patients in on a timely basis because, as I mentioned before, a lot of these patients haven’t been seen for years and years and so getting them in quickly and trying to figure out what’s going on, really is important.
Melanie: Tell us about your team at UAB Medicine.
Dr. Cribbs: Our team at UAB most directly involves myself and, then, I have two other pediatric cardiologists, Dr. Colvin and Dr. Johnson, who have been taking care of children and adults with congenital heart disease for years and they’re certainly and very important part of our team. We have a nurse, an adult congenital nurse, but really and truly, the team is really the entire division of cardiology on the adult side as well as the pediatric side. I would add to that, that the congenital CV surgeons that we work with because each of these patients may have something that goes on that involves CV surgery or involves the electrophysiology folks on the adult side, or involves a congenital cath that would be done on the pediatric side. So, it really is a system-wide team effort.
Melanie: Thank you so much for being with us today, Dr. Cribbs. You’re listening to UAB Medcast. For more information on resources available at UAB Medicine, you can go to UABMedicine.org/Physician. That’s UABMedicine.org/Physician. This is Melanie Cole. Thanks so much for listening.
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