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Posted by on Aug 7, 2015 in 2015, Cardiac Risk in the Young, CRY screenings

Back to the future – no UK screening so it’s still down to @CRY_UK

Zadok england2

Tom Clabburn: “…so rare.” But not in the sense the UK NSC meant.

The announcement by the UK National Screening Committee (UK NSC) that it will not be recommending a national cardiac screening programme for young people aged 12-39 left me, as with much in life, with more questions than answers, a healthy dose of bewilderment, no little anger and much sadness.

Sadness because the UK NSC’s next review will not start until 2018/19. So we know from the outset that, based on CRY’s 12-a-week figure, just short of 2,000 more UK citizens, all of them young, will likely have died by the time anyone takes another look. Many could have lived.

That’s shameful.

Can you imagine if 12 cyclists were killed each week on London’s roads? That would be carnage. There would, quite rightly, be outrage, a huge public outcry. So why not in this case, too?

There was an impassioned response here to the UK NSC’s decision by CRY’s Dr Steve Cox. He writes that the UK NSC has “… let down thousands of bereaved families and above all it has let down our young people and our future generations …” and that policy makers should “stop pushing this issue ‘under the carpet’”. Strong words.

But there are, of course, usually at least two sides to any argument. Members of the UK NSC didn’t just pluck their recommendation out of thin air. They won’t have a particular agenda against cardiac screening. So why and how did the UK NSC arrive at the decision it did?

As a layman, trying to find that out meant relying on whatever the UK NSC chose to make public. Fortunately, that’s a lot – a good thing. The difficulty for the non-expert, perhaps, lies in interpreting that information.

The UK NSC website, here, was the starting point. It says the committee’s 13 members, mainly medical professionals but also including those representing broader interests, have a clear role: The UK NSC advises ministers and the NHS in the 4 UK countries about all aspects of screening and supports implementation of screening programmes”.

To do so it “will call on sound evidence …” Note that phrase. It also must have “regard to the broad balance of benefits and costs, and the effective use of available resources.” The NHS is driven by priorities. There’s no infinite cash pot.

Sudden Cardiac Death

A link from the home page leads to all UKNSC screening recommendations here. The Sudden Cardiac Death (SCD) entry reads “Systematic population screening programme not recommended”. Next review, 2018/19.

This, in turn, links to a page here summarising the recommendation and which additionally includes three “key downloads” and one “related document”.

First up is the language used on this summary page. It’s plainly not written for punters like me, neither as an aid to understanding nor as a model of sensitivity. CRY’s Dr Cox was rightly exercised by the line “Sudden cardiac death in young people is always shocking and very sad. This is in part because it is so rare”. There’s more than a whiff of the Stalinist about it: “A single death is a tragedy; the deaths of 600 young people a year are a statistic.”

Reaction to the use of language is, however, subjective. It strikes me as a poor choice of words, a casual dismissal of a ‘mere’ 600, but I’ve dealt with and heard worse. What’s wanted is an understanding of the substance behind the UK NSC decision. Perhaps one of the key downloads, entitled “Recommendation Statement” will help but it adds little to the first summary page. It has a link to find out more about the sudden cardiac death recommendation but unfortunately at time of writing this mistakenly led to a page about bladder cancer.

Ok, forget that, back to the summary page with its two remaining key downloads and one related document. Here are the reasons its gives for the committee’s recommendation (my numbering):

1: “Currently there are a number of uncertainties over the test, the conditions that can cause SCD, and the overall benefit of identifying those at risk when weighed against the potential harms. 

2: There is very little research into the reliability of the tests for identifying those at risk of SCD, therefore the UK NSC cannot recommend its use in a screening programme. 

3: There is no agreed treatment or care pathway for supporting those who have been identified as at risk of SCD. Someone who is identified as having a high risk of SCD may become anxious about their physical activity and stop regularly exercising which can be detrimental to their overall health.”

It was, as it turned out, not a huge step from this summary to wondering quite how we’ve reached a point where nothing will be done for at least three years.

The 6 out of 13 committee members who are draft minuted as having attended the latest UK NSC meeting in Edinburgh on June 18, 2015, had the benefit of expert opinion and discussion. I, obviously, don’t (UK NSC minutes are available via its homepage).

But remember the line about “sound evidence”? Well keep that in mind when considering UK NSC summary points one and two (I’ll return to three and care pathways later). Much of the thinking behind these can be found in the remaining two key downloads, dated June 2014 and March 2015, and the related document, dated June 2015.

The June 2014 document, is central as it is the evidence summary of the review commissioned by the UK NSC into “Screening to reduce sudden cardiac death in people aged 12-39 years: an appraisal against UK NSC criteria” by Doctors Wiffen and Clarke.

The UK NSC background document of March 2015, – prepared for the meeting at which cardiac screening was, I presume, originally to have been discussed – states that the review’s remit was to “review the evidence published since 2008” when a UK NSC review focussing solely upon the leading cause of SCD (Sudden Cardiac Death) in young people, hypertrophic cardiomyopathy, also decided against a screening programme.

This document continues “… The scope of this review focuses on screening people between the age of 12 and 39, and explores a broad range of conditions associated with SCD, expanding on the previous review of hypertrophic cardiomyopathy … Interest to explore the broader range of conditions was stimulated by a number of meetings between UKNSC representatives, CRY and Ministers. The UK NSC are aware of the established screening programme for SCD in Italy however the impact of the programme to prevent SCD has not been reported.”

SCD is an important health problem.”

This, then, was the context for the Wiffen/Clarke report. The first sentence of the first “key message“ of Wiffen/Clarke is one nobody disputes: “SCD is an important health problem.” It goes on: “However there is little peer reviewed evidence to enable an accurate assessment of the number of people suffering from SCD. There is no assessment of the test accuracy (sensitivity or specificity) in the literature so it is not possible to recommend its use in a national programme.”

The authors looked at evidence from around the world. From a starting point of many, many thousands of papers dating back to 1982, they worked their way through until they finished with “a total of 216 papers that were checked in full, of which 73 papers were selected for inclusion in this report.”

Most of the included papers appear to concentrate on those taking part in sport, rather than the general population. Can’t be helped because we know where most of the testing is done but that isn’t what the review called for. So, a first layperson question: if CRY has shed-loads of UK screening data from the many tens of thousands of screenings it has conducted both before and after the key date of 2008 – and that these also include the general UK population, not just athletes – was this considered? From what’s online, it’s not obvious.

Oh, and, of course, there’s the already alluded to Italian example where screening of those taking part in sport has been mandatory for more than a quarter of a century. This was scrutinised by the review. One 1995 paper it referenced estimated that “… less than 50% of athletes who were eligible were screened, due to the size of the enterprise and inadequate financial support.” But that’s a 20-year-old paper. The statistics now could be better or worse. And if, say, ‘only’ 40% were screened, how many is that? There may be a clue in the preceding sentence:: “Some 3 million athletes undergo these examinations each year” although it is not clear if this is part of the 1995 paper or published earlier or later. But 3 million? That’s impressive. “These examinations”, by the way, are 12-lead ECG tests. And the Italians say they’ve cut the incidence of SCD by 89%.

Next to revisit the March 2015 download document. It contains submissions from “A three month consultation …hosted on the UK NSC website” which followed the review and which, to my shame, I completely missed. The UK NSC also directly contacted a number of organisations, including CRY. Three members of the public whose families had been affected by SCD responded plus various organisations, including CRY.

The UK NSC’s summary of CRY’s response states immediately that CRY “Acknowledged many of the concerns identified by the review …” and goes on to detail them. Good. CRY has to remain objective. As do we all. Thinking you are the only one with an answer is madness, unless, of course, you happen to be the only individual in the UK trying to come up with one (copyright Alison Cox, 1995).

“Conflated SADS (Sudden Arrhythmic Death Syndrome) and SCD”

The document goes on to note that CRY “also suggested that the review”, had, among other points:

  • Conflated SADS (Sudden Arrhythmic Death Syndrome) and SCD, thereby underestimating the incidence of SCD while missing important published UK data on incidence in a paper which had been submitted
  • Omitted the substantial evidence base underpinning the management of most risk factors for SCD.
  • Did not take into account that ECG appeared to be the more favourable strategy compared to other approaches and that data on key test performance outcomes had been reported in papers which had been submitted

To this layman, these sound serious flaws.

Within this document there are also the aforementioned three responses from members of the public. All are worth reading. These are people who have got their acts together and deserve nothing but the highest praise for putting so much effort in.

June 2015 produces two documents both dated the 18th, one the draft minutes of the UK NSC meeting of that date and the other the “related document” providing “information on further work undertaken on screening”. This latter document states: “Screening to reduce sudden cardiac death was considered at the March 2015 UK NSC meeting [my brackets, I assume this is an error as the March 2015 minutes say the matter was to be forwarded to the June 2015 meeting]. An evidence review was received along with the comments submitted during the three month consultation. On the basis of this it was agreed that a screening programme to reduce sudden cardiac death should not be recommended.”

It goes on “The UK NSC review reported that a number of testing modalities had been proposed for use as screening tools” and, later states  “Since the last meeting, and during the period of political sensitivity, a systematic review of test accuracy based on a larger body of evidence has been published. Because of the controversial nature of this issue, the reviewer was asked to critically appraise the systematic review.”


Screening strategies based on personal / family history and physical examination were found to be less accurate than ECG.”


In conclusion: “The critical appraisal highlights some of the limitations of the systematic review which raise concerns about the reliability of the results. In addition the reported test values are comparable to those reported in the UK NSC review. It is unlikely that including this systematic review in the analysis would alter the substance of the UK NSC review.”

Remember “sound evidence”? Read again that the systematic review is “unlikely” to alter the substance of the review. Not “would not” or “will not” or “not a cat’s chance in” but unlikely. Which means that it could have.

Anyway, I get the substance. No national screening programme.

One of the papers referenced in this last UK NSC document is “The effectiveness of screening history, physical exam, and ECG to detect potentially lethal cardiac disorders in athletes. Harmon A, Zigman M, Drezner J, dated February 2015.”

It struck a chord. To quote from its abstract here:

Fifteen articles reporting on 47,137 athletes were reviewed. After meta-analysis the sensitivity and specificity of ECG was 94%/93%, history 20%/94%, and PE 9%/97%. The overall false positive rate of ECG (6%) was less than that of history (8%), or physical exam (10%). Positive likelihood ratios were ECG 14.8, history 3.22 and PE 2.93 and negative likelihood ratios were ECG 0.055, history 0.85, and PE 0.93. There were a total of 160 potentially lethal cardiovascular conditions detected for a rate of 0.3% or 1 in 294.”

Cue bewilderment. The Harmon/Zigman/Drezner paper posits a detection rate of 1 in 294 among an athlete cohort. CRY say 1 in 300 based on all that evidence accumulated from tens of thousands of UK screenings among both the sporty and non-sporty. It seems to correlate. So layman’s question number two: if Harmon/Zigman/Drezner and CRY incidence figures are so close, doesn’t that rather suggest a case for screening? Isn’t that how the NHS is now supposed to operate, proactively, rather than reactively?­­­­

CRY’s 1 in 300 figure is also separate to the 1 in 100 who are identified with a non-lethal condition at a CRY screening. This might require immediate or later in life management. Again, prevention is better than cure, a proactive approach better than a reactive.

The UK NSC document, incidentally, also references a 2011 paper, abstract here, by Elston, J and Stein, K, Public health implications of establishing a national programme to screen young athletes in the UK, 2011. This concluded that “The impact of screening on reducing SCD in young athletes is only modest and would be achieved with significant harms to population health”. The authors’ make the latter point because they suggest that “Of an expected 196 SCDs per year, 40 (6 to 74) would be prevented. For every life saved, 791 athletes would be disqualified.

Layman’s question number three is a rather larger one. If we are to accept 196 SCDs a year among athletes as in the 2011 paper or 600 among the UK’s general population as CRY suggest, what’s the number we have to hit before testing is recommended? How many have to die when they need not? It’s not as though anything new has to be invented or produced to save the majority.

Everything costs. I get that. Everything has a price, even the life of a young person who might have gone on to lead a productive, taxable existence. I can’t quite get my head around the review’s recent economic estimations table but it appears to suggest a cost for one averted SCD of between US $69,000 and US $14.4m.


Now I don’t have access to the same material but it strikes me that CRY manage to offer a subsidised test at £35 and a quick Google search shows that South Devon NHS Healthcare here priced a 12-lead ECG for private and overseas visitors at £49 in financial year 2014/15. Layman’s question number four: wouldn’t it have been reasonable to look at current UK, rather than global, costs?

There is no agreed treatment or care pathway…”

Now, back to point number 3 of the UK NSC’s summary of why screening was rejected. This one made my blood boil. Again, maybe it’s the language but it says “There is no agreed treatment or care pathway for supporting those who have been identified as at risk of SCD.” So what? CRY has its MyHeart groups here which support young people diagnosed with life threatening conditions and whose members seem  a determined bunch when it comes to living with heart conditions.

I have no experience of living with a young person who had a heart condition. Tom died. I do have experience of living with an older person with a heart condition, Claire, and I know that it’s not a bowl of cherries. Yet nobody suggested she should not have been given the opportunity to be diagnosed. To make a lack of a treatment or care plan one of three key reasons for rejecting a national screening programme is breath-taking.

It’s wrong.

I assume there was “no agreed treatment or care pathway” originally for those unfortunates suffering from Ebola or MERS and that there won’t be for whatever other awful disease is the next to rear its ugly head. Lay-man’s question number five: Why is having no treatment plan a reason against screening? It’s cart before horse.

Perhaps I’ll add “frustrated” to bewildered, angry and sad. I’m sure it’s there but it’s hard to follow a coherent argument in the UK NSC’s published documents, hard to accept the conclusion that as a country we’re going to sit on our hands and do precisely nothing.

Layman’s question number six: Could it be that while the UK NSC review took a global view across three decades, a perfectly legitimate approach for a condition that does not respect borders, only to find a miasma of different testing regimes and standards, it might instead have been more strictly mandated to concentrate on what we know and have found for ourselves right here in the UK and then to see to what degree this was supported by relevant international findings?

Finally, could it be that the international community doesn’t have as many answers as we might like because we are ahead of the mainstream, in the sense that CRY’s screening programme, led by its world renowned consultant cardiologist, Professor Sanjay Sharma, here, is actually setting new standards for research and in rigour of testing?

Maybe there is the faintest flicker of hope because the recommendation page also says that “The UK NSC regularly reviews its recommendations on screening for different conditions in the light of new research evidence becoming available.” Layman’s question number seven: does this mean that if evidence arises before 2018/19, it will be considered?”

Enough questions.

Because there is also hope if you look at the list of members of CRY’s All Party Parliamentary Group (APPG). I counted more than 80, including the Prime Minister, someone who, like his predecessor, knows what it is to lose a child. Good politicians are duty bound to listen to expert advice but good politicians don’t have to accept such at face value and they can, must and do question it. Perhaps that will happen in this case. In my view, by virtue of their membership of CRY’s APPG, these men and women of all political stripe go a long way towards qualifying as good politicians.

It is also encouraging to see that Sports Minister, Tracey Crouch, a qualified FA coach, told parliament here in July, 2015, that she’ll be looking at screening programmes “in some detail in the forthcoming sports strategy.” Her remarks followed the death of 24-year-old Tonbridge Angels footballer Junior Dian earlier that month.

For the moment, though, we have the UK NSC’s recommendation: no screening programme.

Does that mean all the fund-raising is pointless, that CRY have it all wrong? Well you pays your money and takes your choice … literally, in this case, as so many of you donate or fund-raise for Tom and Claire’s Fund and CRY.

I can only say that there’s one thing I am neither puzzled, confused nor bewildered by and that is the value of cardiac screening. I say that based on personal experience. I know that Tom and Claire’s Fund screenings have led to people being diagnosed with, and treated for, undiagnosed heart conditions.

If it’s only CRY that’s going to try to do something about the potentially horrendous loss of life over the next three years, then that’s how it will have to be. To quote my favourite Churchillian acronym, it’s time to KBO – Keep Buggering On. It’ll have to do for now.

PC 5/8/15

NB: I have neither spoken to nor consulted CRY re the above. Any mistakes or inaccuracies are down to the author.