The most influential international guidelines on treating aortic disease have just been updated and published online.
My printer has been busy: the main text is 177 pages, published in the Journal of the American College of Cardiology (JACC) as Isselbacher EM et al, 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease.
There are three important changes highlighted, since the previous guidelines in 2010 (Hiratzka et al).
Although the co-sponsors are the American Heart Association and the writing group is very North American, these guidelines will be strongly influential in the UK and across Europe, where new European guidelines - last issued in 2014 but with an update process just beginning - are likely to follow these changes.
The first important change is to strengthen guidelines for monitoring aortic disease with CT, MRI and echocardiography, before and after any surgery, to standardise the quality and timing of images to guide and assess treatment. In the UK, this might imply that anyone monitored for (ascending) aortic disease should be referred to specialists who can follow such guidelines.
The second important change is a shift from 5.5cm to 5.0cm for preventive surgical treatment of unexplained ('sporadic') aneurysms of the aortic root and ascending aorta, in centres with expert surgeons working with multidisciplinary aortic teams. Realistically, such centres are the only ones that should be doing this kind of surgery, so this recommendation should logically become standard in the- UK as much as in the USA. Lower thresholds are advised where there are high risk genetic markers or family history.
The third change is a strong recommendation that first-degree relatives of anyone with an aneurysm of the aortic root or ascending aorta, or with an aortic dissection, should be screened with aortic imaging. This is already the basis of a substantial project at the University of Leicester, which could also support wider screening of families affected by genetically-based aortic disease.
There is an online hub which gives short summaries as well as full text and background material, for anyone interested.
I think that patient information about ascending aortic aneurysms in the UK is still really inadequate, and would like to use these guidelines to lobby for better provision, signposting to services and support.
I'd love to hear from anyone who is interested in discussing what that might look like ...
i would be very interested to hear about service, support in fact anything and to be kept in the loop about what happening ..
I just found out this year I have a 4.4 cm ascending aortic aneurysm and my mother had one and died of it too.. My cousin told me that I had an uncle also who died before I was born that had one too
Do you have a specialist cardiologist, and have you already talked to a surgeon?
I was diagnosed at 4.5/4.6, now more like 4.8/4.9, am being monitored (echos and MRI) and treated (with irbesartan) by a specialist cardiologist, have discussed surgery, with 5.0cm already agreed as the threshold for offering it to me.
In your circumstances, the surgical threshold could well be 4.5cm rather than 5.0cm, but only if you are being seen at one of the leading centres, and then only if you and your family are ready for it and really want to go down that path.
I haven’t had a face to face appointment with a cardiologist yet.. I’ve had lots of tests and he has spoken on the phone .. my last one was 3 weeks ago ..was a stress test (I would need to look up the correct name) where they put your heart under stress and radioactive dye and scan your heart.. Anyway I rang the cardiac Dept and spoke to the secretary and ask for a face to face appointment to get my results has I didn’t want it over the phone like last time and I have lots of questions I would like to ask…
I’m waiting to hear
How do you get to see a specialist? Is there a specialist hospital near me or I can get to London?
I was the one that found my moyher so I know how quickly it can happen without warning
There's a major centre in Southampton but London is just as close to Eastbourne and has more options.
St Thomas's, where I am, has an Adult Congenital Heart Disease team where the aortic specialist is Dr Yaso Emmanuel who runs a joint clinic with a cardiac geneticist.
The Royal Brompton hosts the regional genetics lab that runs aortic tests; they also have a multidisciplinary aortic team and if you prefer, you could be referred there.
St George's, Tooting, where I was diagnosed by sports cardiologists, has a specialist aortopathy cardiologist in their team. Those are, I think, the main South/Central London ones ...
I was referred to St Thomas's from another cardiologist at a different hospital, in a busy general cardiology department ... my GP asked me at the outset where I wanted to be referred, and I have lived and learned: patient choice is a real thing, but you do need to know just how specialist these things actually are. Congenital Heart Disease clinics have a very different atmosphere to crisis-oriented ones: they see the same patients, many of them younger, for years, and I think they're great. Good luck!
See my response below! I was told by a previous lady GP at least 10 years ago this can be inherited. Insist on seeing a specialist, your aneurysm IS needing attention.
Both my mother and elder sister died of an aortic aneurysm. My sister because ‘they’ were watching it🤷🏼♀️ waiting for it to burst it seems. Where does this measurement differ for women? Women are not the stature of men. Both mum and sister were tiny women. My brother in law pointed out that she was tiny and not the same stature as most probably other examples. I have had two aortic scans in 10 year period to check mine, luckily I’m ok. I have a younger sister who’s 8 years younger, her dr sent her for a scan without question. It seems this is inherited in our family.
You are quite right, the precise threshold for surgery can and should be adjusted for height, and for a shorter female patient would be lower. And for an exceptionally tall male patient, higher. This is well explained in the guidelines - I've only given a few of the main messages.
Thanks for your excellent summary, Julian. I saw this was out but so far had only seen the infographic on surgical intervention diameters.I was, frankly, disappointed, which, when you consider I dissected at 4.5 cm after 20 years of monitoring after my enlargement was spotted on a cardiac echo, no CTDs or familial history, is not surprising. My emergency operation was nothing short of miraculous but the road back is still very hard, and I have now been diagnosed with ICU-Acquired Weakness / Critical Illness Neuropathy as a result of my 6 week ICU stay. This is permanent nerve and tissue damage and I struggle with all sorts of physical challenges, and sincerely wish I had been operated on prophylactically.
The problem is, whilst diameter is pretty much all they've got to assess an aneurysm on, it is an extremely complex situation and diameter is an extremely crude part of that.
Ever since IRAD pointed out in 2007 that 5.5 cm was inadequate as a guide (60% dissected below 5.5) there have been numerous studies confirming this concern, with a number of reasons for this (such as: the risk balance improves in a high volume centre, the aorta bloats at the point of dissection around 10-15%, so post-dissection diameters are misleading, etc etc). There have been attempts to explain what Elefteriades calls the aortic paradox around his work on 5.5 and all the other studies. Part of the problem is that the original 5.5 cm papers presented their conclusions on an incremental risk graph (as diameter increased) without the corresponding absolute risk figures. This is likely to have led to a false sense of security. The cohort used in the original papers was also not representative of a wide-enough populace. One London centre terms this whole area "The Aortic Myth".
Now, when you consider most incidental findings will come about through a cardiac route (echo), then the folk who need this complex problem distilled are the Cardiologists, and to date 5.5 cm and/or a rapid rate of growth have become engrained as the simple criteria. With the challenges that any discipline has, the cardiologists will be no different, and are unlikely, in my view, to read through the guidelines fully, and would rely on the summaries and infographics alone - unless they have the aorta as a special interest. Would they pick up the need to refer straight to a specialist centre? Would they know how crude a parameter diameter alone is? What would really shift awareness in the non-specialist from the old simple 5.5 cm criterion?
I can't offer a solution, as one doesn't exist, but we need to really press home that diameter is a crude measure and only one part of a complex whole, and those with aneurysms need always to be seen in expert centres.
One final point, the height-related adjustments to aortic diameter criteria imply an accuracy that doesn't exist. I am 2m tall and should have been "safe" until some margin above 5.5 cm. I believe these adjustments are invalid in tall people, since we tend to be "long" rather than "big" - the BMI index is unfair to tall people, as one example. It may be that for smaller people a reduction in diameter is valid, in which case an earlier operation becomes the safe option, unlike for tall people, where pushing the criterion above 5.5 increases their risk unduly.
Hi Cliff - it's great to hear from you, though I'm really sorry about the ongoing neurology challenges, and hope there's relief to be had.
Part of me thinks that the time to offer prophylactic surgery - if it's to be offered at all - is ideally at the same time that lifestyle restrictions kick in. The challenge is that about 1 in 500 of the population could then be due for major surgery, and there's not the provision for it. So looking for cheaper, safer, medical interventions - like the daily irbesartan I'm now taking - but with painfully little evidence to back it up - would be the top priority.
Screening relatives is definitely, in my view, a step in the right direction, and it was good to catch up with Gavin Murphy at the recent Aortic Dissection Awareness Day event in London; his group are definitely thinking about what next after finding more people at risk.
The new guidelines, on page 60, discuss a paper by Parachuri et al [CORRECTION: Paruchuri ...] which found that, compared to a control group whose aortas were less than or equal to 3.4cm, those with an aorta 4.0 to 4.4 cm were 89 times more likely to dissect, and those greater than or equal to 4.5cm were 6,300 times more likely to dissect.
They make no attempt to convert this into absolute risks, but 6,300 times an annual background risk of (say) 1 in 100,000 would give a 6.3% annual risk and completely justify prophylactic surgery. If the background risk is 1 in 40,000, then it's 15% plus. But that last group would include some people with much larger aortas, and the reality is that no-one can tell me what my 4.8cm aorta really means in terms of dissection risk.
I think the question of whether taller people should have higher thresholds is complicated by the fact that connective tissue disorders are not fully understood and our height may sometimes be linked to genetic risk.
Speaking personally, I'm not all that tall - but in family photos, I'm the taller one. I don't meet Marfan criteria, but my Ghent score is not zero. When my consultant looked for arterial tortuosity, she found it in my neck. Like the majority of John Elefteriades' patients at Yale, I'm positive for arachnodactyly on the thumb/palm and wrist tests. My arm span is definitely longer than my height, too. I have a strong suspicion that many 'non-syndromic' aneurysms are just cases where no-one has asked the right questions ...
Thanks Julian. Interesting about the Parachuri paper. Not one I've come across so will go hunting. If the Guidelines are indeed including such information, this is good, but the original problem still stands, in my view - how to get the straightforward Cardiologist or other specialist finding an aneurysm to refer the patient immediately to a specialist centre. Ana Lopez-Marco recently published a paper on retrograde dissections (Retrograde type A aortic dissection: a different evil? - PMID 36271851) and the diameters there completely confirm that 5.5 (or even 5.0!) is overly simplistic. "Aortic dimensions at presentation were as follows: aortic root 38.5 ± 3.3 mm (34 – 44), mid ascending aorta 42.9 ± 8.4 mm (30 – 70), mid arch 37.5 ± 8.5 mm (31 – 60), proximal DTA 44.2 ± 9.2 mm (35 – 67)."
The Paruchuri paper has been published in different versions which I think you will have seen, Elefteriades was a co-author. Eric Isselbacher, who is the chair of the writing group this time round, has worked on data from IRAD and published on it. Here's the link to the version referenced in the guidelines: karger.com/Article/FullText...
Starting to look at the Guidelines. Page 13 Figure 5 shows the actual risk increase, which is actually (I think) data originally contained in the Elefteriades papers, but is much more indicative of absolute risk than the statistical presentation by Elefteriades of the relative increase in risk as you go to larger diameters, which is where the 5.5 came from.
Ah, I was searching my papers for Parachuri. It's Paruchuri, and yes I had seen that. TBH I found these series of papers a bit defensive (understandably in some ways). The original 1997 paper Coady et al (Elefteriades co-author) jtcvs.org/article/S0022-522... is heavy going and I'm only half way through, but you can see how the statistical manipulation leading to 5.5 cm happened, and I'm not sure I agree with it.
My understanding is that the 5.5 came from early analyses of Elefteriades' Yale/New Haven database, and their more recent 'Is it time for a left shift in guidelines' analysis - Ziganshin et al, which is also referred to in the current guidelines, based on a great deal more data, points to three distinct inflexion points, at 4.5cm, 5.25cm and 5.75cm, which led Yale to shift to 5.0cm as their preferred threshold for offering surgery.
That's correct. But they are still persisting with a derivative measure, Fig 1A, on the Y-axis. "The Y-axis represents the percentage point increase in 'lifetime' probability of complications compared to baseline [between 4 and 4.25 cm]". "Baseline" in some ways suggest low risk, whereas what really counts is the absolute risk at every point from normal up to large aortic sizes, and in particular it is far from zero at 4, 4.25 or 5.5. "Hinge point" suggests there is solid data either side of the hinge point, but it's a pretty unscientific term imho. What should really be done is to plot and regress all data with confidence limits and take it from there against the much lower risks these days of elective surgery.
The guidelines also refer to the currently-recruiting first-ever randomised trial for prophylactic surgery for ascending aortic aneurysms in Canada, where they tend to hold to 5.5cm. As I understand it, this is comparing surgery at 5.0-5.4cm to deferral until current guidelines are met.
If this trial can actually be completed it might start giving convincing evidence about the risk of dissection at this size, but I'm not holding my breath.
In an ideal world, again, I'd like to see randomised early access trials for prophylactic surgery here in the UK, but the challenge of getting the numbers needed for any convincing answers, agreeing what surgery to offer, and funding it all, may make this unrealistic in a system where practice varies quite a bit depending on where you go.
The TITAN: SvS trial is coordinated from Ottawa with 21 centres across Canada and the USA, aiming to recruit 610 participants to be randomised to early surgery or surveillance. It is hoped the study will be completed the by the end of May 2026. The 2021 protocol is at: bmjopen.bmj.com/content/bmj...
On the subject of levels of risk, I have just seen a report from the KP - TAA study, published online on 5 October 2022, which looked at the relationship between the initial size of non-syndromic ascending aortas and the risk of dissection, based on 2050 female and 4322 male patients enrolled in the Kaiser Permanente system in Northern California covering a population of around 5 million, diagnosed between 2000 and 2016 and followed up for an average of more than 3 years:
SIZE 5-year risk (95% confidence intervals) Equivalent risk/1000 patient years
4.0-4.4 0.3% (0.3-0.7) 0.6
4.5-4.9 0.6% (0.4-1.3) 1.2
5.0-5.4 1.5% (1.2-3.9) 3
5.5-5.9 3.6% (1.8-12.8) 7.2
6.0 + 10.5% (2.7-44.3) 21
I haven't seen the full paper, which also reports on the risk of surgery and all-cause mortality; it is possible that these figures underestimate dissection risk because some deaths may not be correctly accounted for, or overestimate it because not everyone with enlarged aortas would be diagnosed, but this is clearly a serious effort to answer the question, of when prophylactic surgery is justified. They go for 5.5cm.
I would be interested, 13 years ago after volunteering for research, I found out that I have a missing gene and bicuspid heart valve. I'm only 4ft 10, apparently I should have been taller, but because of this missing gene I'm not. The gene is called 22Q11. I'm due to have surgery soon. My ascending aortic aneurysm is 5.3 cm.
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