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Dr. Tara Karamlou provides and overview of the AAOCA registry and present controversies at the Treatment Options and Interventions for Anomalies of the Coronary Arteries Tall Rounds® Session.

Enjoy the full Tall Rounds® & earn free CME

  • Case Presentations: Calvin Sheng, MD
  • Embryology and Incidence: Jennifer McBride, PhD
  • Advanced Imaging Evaluation: Paul Cremer, MD
  • Invasive Functional Assessment: Joanna Ghobrial, MD
  • AAOCA Registry and Present Controversies: Tara Karamlou, MD
  • When to Operate, When Not to Operate –Unroofing Procedure: Gosta Pettersson, MD
  • Innovative Surgical Options: Hani Najm, MD

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Talking Tall Rounds®: Anomalies of the Coronary Arteries

Podcast Transcript

Announcer:
Welcome to the Talking Tall Rounds Series brought to you by the Sydell and Arnold Miller Family Heart, Vascular & Thoracic Institute at Cleveland Clinic.

Gosta Pettersson, MD:
Good morning, everybody, and welcome to this Tall Rounds about the treatment options and interventions for anomalies of the coronary arteries, which has actually become a pretty big business for us. And the increased awareness about these issues is resulting in increasing number of referrals, and we have some exciting new options to offer these patients. Old ones have been simplified and new ones have come so it will be an interesting session. I think the first presentation will be a case presentation by Calvin Sheng.

Calvin Sheng, MD:
Hi. Good morning, everyone. My name's Calvin. I'm one of the cardiology fellows here, and I'm going to be just presenting some of the cases.

Calvin Sheng, MD:
One of the first cases is an 18-year-old gentleman who had an incidental finding of anomalous right coronary artery rising from the left sinus of Valsalva with an internal arterial course during just routine screening for a family history of HOCM. That was back in about 2015 and in 2016 had a negative ammonia PET study for ischemia and unfortunately earlier this year suffered a sudden cardiac arrest, V-fib with three shocks during a basketball game, underwent some invasive hemodynamic evaluations, which will be discussed later, and ultimately had successful division and re-implantation of that right coronary artery.

Calvin Sheng, MD:
These are just some representative images from his pre-op cath. On the left there is an LAO view of his anomalous coronary artery, as well as his IVUS findings, and he had a positive iFR of 0.84 during stress with IV dobutamine and atropine. On the right is his pre-op and on the bottom, his postop CT is showing the takeoff is RCA with that narrow compression there.

Calvin Sheng, MD:
In the second case, this is a 20-year-old gentleman with chronic exertional dyspnea and fatigue since childhood. He was also found to have anomalous left coronary artery that's arising from the right sinus of Valsalva, also with an inter-arterial course and an intramural segment. A couple years ago, underwent his invasive hemodynamic testing and ultimately had successful unroofing of about 8 to 9 millimeter segment of intramural segment. And during his follow-up, he's been doing very well without any new complaints.

Calvin Sheng, MD:
These are his representative images. On the left you have an LAO view of his coronary anatomy and then on the right is his IVUS finding. You can see when you get into that intramural segment a slit-like compression. He also indeed had positive iFR findings of 0.75 during stress with IV dobutamine and postop actually.

Calvin Sheng, MD:
Here again, you know, on the cath, it's hard to really visualize well the compression, but on IVUS you really get the sense pre and post that you don't have nearly as much of that intramural slit-like compression, and he actually had negative iFR testing in his postop of 0.9 during stress.

Calvin Sheng, MD:
These are his representative CTs from preop on the left and postop on the right, where on the left, you see that narrow slit-like compression again with a very angulated takeoff and on the right, it just is a little bit better.

Calvin Sheng, MD:
Then, in the third case it's a 62-year-old female initially presented for exertional angina, that was back in 2009, had both LAD and circumflex ischemia. That was ultimately how they found she had an anomalous left coronary arising from the right sinus of Valsalva with a transeptal course. Then she underwent a two-vessel CABG with two vein grafts. Unfortunately about eight years later developed recurrent symptoms and they found that she had interval occlusions of both of the vein grafts. Then a year later came to us, had invasive testing and ultimately had successful transconal unroofing of that transeptal segment, as well as RVOT reconstruction with autologous pericardial patch.

Calvin Sheng, MD:
These are her representative findings. Her pre-op cath on the left with her IVUS, and I apologize if the IVUS is not playing here due to technical reasons, but again, she had very positive iFR finding with 0.32 during stress. Then after her procedure, as you can tell, was very successful with an iFR now of 0.92, and the IVUS shows that she does not have any of the dynamic compressions, that we saw pre-op. Again, these are her representative CTs from pre-op and postop and imagings will be discussed later.

Calvin Sheng, MD:
Thank you for this opportunity to present the cases.

Tara Karamlou, MD:
My topic was controversies. Obviously, that's a massive topic that we're not going to cover in great detail today. So let's cone it down to something that we actually can speak objectively about.

Tara Karamlou, MD:
How does the adult population with anomalous aortic origin differ compared to a pediatric population? How do these adults present? What is their morphology? How do we deal with them surgically and what are those outcomes? Most importantly, what is the impact of concomitant cardiac pathology that is not present in pediatric populations, which arguably have been better characterized at this point, and specifically with regard to coronary artery disease and aortic valve pathology?

Tara Karamlou, MD:
Today I'm going to share with you some insights that will address a few of these issues and share some findings that will actually raise additional questions. I want to acknowledge Mikey Jiang, now Dr. Jiang, who is one of our residents and has done a phenomenal amount in this space. So thank you, Mikey, for sharing all of your data with me.

Tara Karamlou, MD:
So, there has been a tremendous amount of focus on understanding AOCA in the young population because of the high risk of sudden cardiac death. However, we know that adults with anomalous aortic origin also comprises significant portion of this cohort. So while the CHSS, the existing cohorts here, have comprised an impressive registry of pediatric patients and those that are restricted to under age 30 years at diagnosis, there really has been very few studies looking at the adult population.

Tara Karamlou, MD:
So this cohort of primarily adults with anomalous aortic origin was assembled here at the Cleveland Clinic to help us understand some of the questions I mentioned earlier. We know that adult patients potentially, as we hypothesized, might be diagnosed at an older age and may differ from their pediatric population. Note on this slide that the pediatric population, the surgical threshold we hypothesized might be higher, and that the adult population, many of them may actually be diagnosed later and lie below the surgical threshold until some inflection point, at which time the development of associated cardiovascular anomaly, specifically coronary artery disease, might lead to an increase in diagnosis and therapy.

Tara Karamlou, MD:
So we turned to the Cleveland Clinic data, and we looked at a slice of this two-year period and really excluded all pediatric patients and those without anomalous aortic origin. That left us with a population in this two-year period of 167 patients and from there, we looked at those patients who underwent surgical intervention, any cardiac surgery, and those who had concomitant repair of their AOCA or isolated repair.

Tara Karamlou, MD:
So one of the fundamental questions I mentioned was how do these patients present? What prompted them to come to medical attention? We found that the majority of these patients actually were symptomatic with either chest pain or shortness of breath. That's here shown in blue. Then the second most common presentation was patients presenting for cardiac evaluation for either concomitant cardiac surgery or non-cardiac surgery.

Tara Karamlou, MD:
The next question was to characterize the morphology of the anomalous coronary artery. We speculated that adults with anomalous aortic origin may benefit from lead time bias, the phenomenon that they may have a less malignant phenotype because they have survived to adult life. These clustered histograms show the age at diagnosis stratified by the anatomy of the coronary. The anomalous rights from the left sinus are shown in blue and the anomalous lefts in origin, and then the denominator here shown in other colors.

Tara Karamlou, MD:
What you will note is that the majority of patients, in fact, 90% of patients were diagnosed after age 30 and, as we anticipated, the anomalous rights predominated in later life. So in other words, anomalous lefts were diagnosed earlier, potentially proving our hypothesis that these represent a less malignant, I don't want to use the term benign, so less malignant phenotype.

Tara Karamlou, MD:
The other question was how did we operate on these patients? Why did we operate? What was the anatomy of the surgical cohort? As we mentioned, the rights were more common overall, as we know, oops, but what we found was that the rights actually had a greater prevalence of both interarterial and intramural course, potentially not just the increased prevalence of anomalous rights in general, but also the phenotype of the rights was different than the anomalous lefts.

Tara Karamlou, MD:
So surgical repair, we felt that this may be significantly different than how we approach this in the pediatric population, specifically with regard to revascularization with CABG and, in fact, that was indeed the case. Eighteen had unroofing, which is pretty typical among the pediatric population, but a not insignificant proportion had coronary artery bypass grafting, and three, in fact, had ligation of their proximal segment, three re-implantation and aortic coronary window.

Tara Karamlou, MD:
Now, how did we deal with patients with concomitant pathology? If you remember from the first slide, one of the perspectives we had was that these patients may present with a burden of cardiovascular disease that complicates both their evaluation and their therapy and this was indeed the case. So there was 59 patients who had any open cardiac surgery and interestingly 17 had surgery, but the anomalous aortic origin was not addressed. The remainder had concomitant cardiac surgery.

Tara Karamlou, MD:
What were these? While the majority were aortic valve operations and then, coronary artery bypass grafting to another coronary with significant atherosclerotic disease.

Tara Karamlou, MD:
So in conclusion from this initial study, there was a significant older age at diagnosis and presentation. Concomitant disease, as I mentioned, was much more prevalent and can actually complicate the evaluation of these patients and the surgeries to deal with these are varied.

Tara Karamlou, MD:
So quickly I want to just highlight a study that is ongoing as a follow up to this.

Tara Karamlou, MD:
As we mentioned that coronary artery disease is more prevalent potentially in adults, we speculated that there may be an increased predilection to the development of atherosclerotic disease in the coronary with an anomalous origin, either from turbulence or whatnot.

Tara Karamlou, MD:
But interestingly, from this initial look at these patients and there's over 1,000 that we still need to go through, but of this initial population, if you look at the bottom here, this dot plot actually shows that they are equivalent. So we defined any coronary artery disease as any stenosis greater than 10%, and they were equivalent among the anomalous and the normal aortic origins. The median stenosis in these vessels was equivalent, whether it be right, left or another coronary distribution.

Tara Karamlou, MD:
Thank you.

Announcer:
Thank you for listening. We hope you enjoyed the podcast. Like what you heard? Visit Tall Rounds online at clevelandclinic.org/tallrounds and subscribe for free access to more education on the go.

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