Mandatory ECG for Paediatric Syncope - MEPS

ECG’s acquired when a child faints provide lifesaving data

Every Paramedic in Ontario, and probably where you live, is trained and equipped to take an ECG (Electrocardigram) and do basic heart rhythm interpretation. When Paramedics respond to a call for a child that has fainted it should be mandatory to get an ECG and to provide the strip to the Doctors in the ER, even if upon arrival at the scene the child has regained consciousness and “seems” fine.

Of the 700 young people that die suddenly of cardiac arrest each year in Canada about half had a fainting episode in the days, weeks, months or years prior to their death. These sentinel events provide the best opportunity to identify an underlying heart rhythm disorder and initiate treatment and prevention therapies.

Important information about what was happening to the child’s heart at the time of collapse is lost as time passes. The sooner the first or "presenting" ECG is taken the greater the understanding that Doctor’s will have of what triggered the event.  As time passes the heart returns to baseline and important clues may be lost.  

It is not essential for Paramedics to interpret these early ECG’s, they simply have to acquire the strip. Once acquired the ECG strip can be shared with the physicians in the ER or the Paediatric Cardiologists for analysis. Once acquired the ECG becomes part of the electronic call record and can be accessed weeks, months or even years later perhaps making an important contribution to a future investigation.

Studies estimate that roughly 1 in 20, about 5%, of all childhood faints are a warning sign for an underlying heart rhythm disorder. For faints occurring during or shortly after physical activity, while swimming or as a result of a loud unexpected noise (auditory startle) that number may go as high as 1 in 3, or 33%. Building communities that are more sensitive and responsive to childhood fainting can only result in young lives being saved.

There are some questions around the ability of Paramedics and the equipment they work with to collect accurate ECG’s from prepubescent patients. Two things occur to me - first, get the strip and let the physicians decide if it contains useful information – second, perform more ECG’s on children and get better at it which may also require additional training and equipment software upgrades with better paediatric programs.  As taxpayers we are paying for our Paramedics to be equipped and trained to gather ECG data, this toolkit has its greatest potential value when it is applied to our children.

Currently at least 14 states and provinces have legislation passed or pending designed to increase sensitivity to the warning signs of paediatric heart rhythm disorders in our communities. If Teachers and Coaches call 9-1-1 every time a child faints and Paramedics acquire an ECG for every child that faints and ER Doctors do a thorough work-up including event history, patient history and family history (and consults with appropriate specialists when required) for every child that faints, many young lives will be saved.

When a youngster has one or more fainting episodes and then dies an opportunity to save that child’s life has likely been missed. I have listened to the stories from dozens of families that have lost children to sudden cardiac arrest and far too many of those stories include anecdotes of fainting episodes that educators, sport officials and most concerning healthcare professionals have overlooked, brushed off or misdiagnosed.  Paramedics and the EMS system can play a vital role in ensuring that potentially lifesaving information is recognized and acted upon whenever our children experience a loss of consciousness faint.

Watching our Children Die on the Ice

Somebody do Something … just don’t ask me to perform CPR

For nine years I was the Public Access Defibrillation (PAD) Program Co-ordinator for a municipality with a population of just under 500,000. It was an exciting time on the frontier of AED placement in Canadian communities. In five years the number of cardiac arrest survivors in the community more than quadrupled, from 7 in 2005 to 31 in 2010, with many of those additional lives being saved by members of the public performing effective CPR and deploying an AED within minutes of patient collapse.

One life that was not saved was that of a hockey referee. He collapsed during a game that was well attended in an arena equipped with an AED. No one that witnessed the collapse knew where the AED was located or how to use it. No one was able to locate any of the arena staff that were trained to use the AED. CPR was performed but by the time Paramedics arrived it was too late for a defibrillator to be effective. In this earlier blog I share my thoughts on why this should have never happened.  

http://leadingcause.blogspot.ca/2012/12/cancel-one-game.html

A few weeks after the event the EMS Chief and me met with Executives from the Hockey and the Referee’s Associations. There were lots of emotions and lots of suggestions coming from all sides. The consensus was to move forward in a positive manner that would help ensure that the next time resuscitation needed to be performed the hockey community would be better prepared.

I lobbied hard with management to be allowed to offer “free” certification level CPR/AED training to any adult associated with the local minor hockey association – coaches, trainers, referees, officials and parents. In the end I was given the go ahead to offer six free sessions for up to 20 people per session over six consecutive Wednesday evenings in February and March. I would provide the training but the hockey association had to provide the people. The marketing and promotion, the filling of the seats, was entirely their responsibility, a condition which they readily agreed to.  The first week five adults with two teenagers in tow showed up – I had prepared 20 manikins and brought in another trainer to assist. I called the association and they promised more people the next week, four is not more than five.  After three people showed up in week three I spoke to the association and we mutually agreed to end the program. Out of the 120 seats that we had made available to the association 12 were filled.

The following winter good friends of mine that had lost a child on the ice to cardiac arrest (the AED was deployed but could not convert the heart back into a blood pumping rhythm) partnered with a local charity to donate an AED to an arena in their community. After a very nice on ice presentation ceremony that included a long and heartfelt standing ovation in memory of their beautiful son we announced that the following morning in the arena’s community room we would be offering free CPR/AED training to any and all interested parties, the session was also promoted through other means. Three people, plus the three of us attended.

The next time a coach, referee or player collapses to the ice and no one in attendance knows what to do where will the blame lie? In a public sports venue equipped with an AED it is the responsibility of every adult that regularly attends that venue as a coach, trainer, official or spectator, paid or volunteer, to acquire basic resuscitation skills. It is not the sole responsibility of the Zamboni driver to safeguard everyone’s life or to be highly visible whenever someone collapses.  

Again this week a child collapsed after taking a puck in the chest (a phenomenon called “commotio cordis” that claims the lives of about twenty young teens each year in North America) at an arena in Toronto. Thankfully arena staff and trained spectators were able to resuscitate the child. Nice job by Doug Jamieson and team. According to the CBC News story no hockey people – coaches, trainers, referees - helped with the resuscitation effort; if this is not true please let me know. 

The CBC interviewed several parents that witnessed the event and they put forward suggestions on how cardiac emergency response could be improved at Toronto arenas. The suggestions ranged from somewhat plausible to downright silly. However not one parent stepped up and suggested that they themselves and in general more people from the minor hockey community should be trained in CPR and AED use. There was lots of half-baked, buck-passing, ill-informed recommendations but not one person stepping up to take a little personal ownership for resuscitating friends or family members in cardiac arrest.

http://www.cbc.ca/news/canada/toronto/aed-defibrillator-helps-save-teen-hockey-player-s-life-1.2837497

In a related CBC story from June 2014 a senior executive from Hockey Canada explained that despite at least 8 on ice deaths in 9 years Hockey Canada is satisfied with its cardiac emergency response protocols which do not require CPR/AED training for coaches and trainers. In fact the recommendation is that if a coach or trainer suspects a player is in cardiac arrest they should seek out someone in the arena that is trained to manage the situation. This is the stated policy of the governing body of the sport that our entire nation loves so much.

http://www.cbc.ca/news/canada/nova-scotia/cardiac-arrests-kill-8-young-hockey-players-in-9-years-1.2669393

Long before the hockey referee died and every day since (it has been more than 6 years now) I have shouted from the mountaintops the importance of universal user group CPR/AED training. I believe that both minor and adult sport organizations should not be allowed to rent municipal sports venues unless the majority of their members are trained in CPR/AED and in general anyone that lives, works or plays in a building equipped with an AED should be trained.

My final two thoughts on this story:

1. When interviewed  by CBC Ali's sister used the word lucky.  A lot of forethought, planning and training on the part of The City of Toronto and the arena staff went into 13 year old Ali being "lucky" that day.
2. When you buy a Ford Escape everywhere you go you see Ford Escapes. When you take AED training everywhere you go you will notice the AED hanging on the wall. Take the training.

The Role of the Death Investigator in Preventing Sudden Cardiac Death

When a child dies of a cardiac cause seize the opportunity to protect siblings and cousins

In some jurisdictions they are called Coroners in others Medical Examiners. Regardless of job title they have the potential to significantly reduce the toll – 7,700 per year - that Inherited Heart Rhythm Disorders take on young people in provinces and states across Canada and the US.

The Coroner’s responsibility begins with correctly identifying the cause of death.  For the first degree relatives of a child that died of sudden cardiac arrest caused by an underlying genetic heart rhythm disease the words “unknown” or “undetermined” or “possible cardiac” on the death certificate is too often their own death sentence. (this will sound like hyperbole …. until it happens to your family). Inherited structural diseases, the cardiomyopathies such as HCM and ARVC can normally be detected through routine examine due to the extensive remodelling of the hearts structure. Inherited electrical diseases such as Long QT and Brugada Syndromes are more challenging as the telltale electrical signature of the disease is now switched off.  Often molecular genetic testing is required to determine cause. There are currently at least 15 genes and many more mutations associated with the various heart rhythm diseases and with the intensity of research in the field that number grows almost monthly. When on autopsy a clear phenotypical cause of death does not present the coroner must use DNA testing to seek a genotype that suggests a likely cause.

Coroners and ME’s should be collecting and storing genetic material, blood and tissue, for all young person deaths - under the age of 35 would be ideal, 18 is too low. Every province and state should have a DNA Bank where death investigators can safely store and easily access genetic material. Collecting and testing DNA samples from infants where the death was classified as SIDS (Sudden Infant Death Syndrome) is essential. Published research estimates up to 30% of all SIDS death are caused by an arrhythmia gene.

Every time a young person dies of “cardiac” or “presumed cardiac cause” both sides, the ME's Office and the Family (with guidance from the family physician) should be pursuing DNA testing.  Typically if the family does not ask, the ME will not initiate genetic testing.  Typically if healthcare professionals do not present DNA testing as an option the family is unaware of its availability and unaware of its lifesaving potential for gene positive first degree relatives. This is why death investigators and family physicians should be simultaneously informing parents of their options.  If it takes two or three or ten years for the family to recognize the importance of genetic testing the genetic material should be readily available when the call comes.  Note:  The family physician may wish to call upon the services of a genetic counsellor to help the family understand the importance and significance of genetic testing and the implications of all of the possible results – positive, negative, undetermined or any number of shades of grey.

When the testing is complete and the results are positive for a known genetic association with a heart rhythm disease the real work begins, notification and testing of all first degree relatives.  Most primary heart rhythm disease are transmitted by an autosomal dominant gene – if one parent carries the gene on average half of the children will acquire the gene, with no gender bias.  Step one therefore is determine which parent carries the gene.  Note: I have worked with a family where one parent was positive for Long QT and the other for ARVC, rare but possible.  Step two is to test siblings and then aunts, uncles, cousins and grandparents on the affected side of the family. It is common to identify four, five or more affected first degree relatives for every index patient.  This is the silver lining; this is the gift that the deceased has given to his or her family.  From one child’s death comes the ability to identify many at-risk relatives and provide them with the appropriate prophylaxis for sudden death.  
 

Step two is not without challenges. Identifying and locating all of the potentially affected family members may be difficult, convincing each of them that they should get tested for a genetic disease that they have never heard of  may be near impossible.    “Catcallamungowhatapolywhatacardia….. You think I might have this wack-a-doodle disease and should get tested?”    At this point all of the stakeholders – the Parents of the deceased, the Family Physician, the Paediatric Cardiologist/Electrophysiologist, the Genetic Counsellor, the Coroner’s Office must work together to protect the living.

Often this process moves into areas where the moral/ethical issues around personal health information privacy come into play.  Navigating these waters can be tricky, rules must be followed and boundaries respected. On a very personal note I believe that when a child’s life hangs in the balance it is best to err on the side of protecting the child.  I hate it when children die with their parents privacy still intact.

The other critical challenge in this process is speed. The Family and the Coroner’s Office must move quickly to pinpoint the cause of death and then to locate and test the first degree relatives. It is difficult to say exactly what the appropriate time frame is for this often complex process to play out but I would submit that if seventeen and eighteen year old cousins die just over one year apart the system has failed both families. Anecdotally, I hear some version of this story far too often.

In every jurisdiction in North America there is significant room for improvement in the way death investigations of young people dying from cardiac causes are handled and followed-up.  If you are an advocate for cardiac arrest prevention in youth please be sure to include improving protocols for death investigations on your must-do list when speaking with provincial or state bureaucrats and legislators.  If you are a family that has lost a child to sudden cardiac death and are unclear what triggered the event push the healthcare and death investigation systems for answers.

The Arrhythmia Train

The Arrhythmia Train is pulling out of the station.  In my talk at Canadian Cardiovascular Congress (CCC) in Vancouver I spoke about the growing awareness of Inherited Heart Rhythm Disorders that I have been observing in many communities and organizations.  My observation has been confirmed over the past few weeks as there has been a flood of arrhythmia news both in the mainstream media and in the places where only an arrhythmia geek like me would look.

The folks at CHEO (Children’s Hospital of Eastern Ontario, Ottawa) have put genetic heart rhythm disorders, especially Long QT Syndrome, front and centre. CHEO has launched a legal challenge to the concept of patenting a gene or gene sequence.  A positive outcome to the case will benefit all Canadian families seeking diagnosis of a genetic disease.

http://ottawa.ctvnews.ca/cheo-launches-lawsuit-against-owners-of-gene-patent-1.2084430

In conjunction with the CHEO story CTV ran the story of the Dines Family from Ottawa. Their story is similar to many of the stories we hear from families affected by an arrhythmia gene. The elements of family history and misdiagnosis in the 20^th century are common themes.  The fact that a definitive diagnosis was made immediately based on a simple, inexpensive, non-invasive ECG is also noteworthy.

http://www.ctvnews.ca/health/diagnosis-of-genetic-heart-rhythm-disorder-life-changing-for-families-1.2089409

A colleague from Victoria BC sent me information about Sports Cardiology BC and the ECG screening clinics that they are hosting in that province.  At the end of November they will be hosting a clinic in Victoria for competitive athletes between the ages of 12 and 35. To the best of my knowledge, please correct me if I’m wrong, this is the first and only group to hold ECG screening clinics for Canadian youth.  While the merit of ECG screening is debated in the cardiology community PACED sees this as a very good thing.

http://www.sportscardiologybc.org/news/sports-cardiology-b-c-is-coming-to-victoria/

In a related story Dr. Vicotria Vetter the highly respected paediatric cardiologist from Children’s Hospital of Philadelphia (CHOP) came out firmly in support of ECG screening for all youth.

http://www.pharmiweb.com/PressReleases/pressrel.asp?ROW_ID=102909#.VFzsq410zec

More positive news (with a very sad and tragic etiology) out of BC is the $1.7 million out-of-court settlement paid by a Vancouver School Board to the family of child with Long QT. The board failed to prevent the cardiac arrest which left the Grade 5 girl with significant physical and neurological deficits. These preventable tragedies should not happen however lawsuits encourage the type of due diligence that can prevent future tragedies. Ontario law firm Miller Thomson picked up on the story and discusses the implications in their client newsletter.  Our thoughts and prayers are with Bezawit and her family.

http://www.millerthomson.com/en/publications/newsletters/education-law-newsletter/september-2014/keeping-abreast-of-the-standard-of-care

Perhaps the most exciting news to come out of CCC is that the BRIDGE project at Canadian Cardiovascular Society (CCS) has invited a SUDY (Sudden Unexpected Death in Youth) working  group to join the project. This invitation from CCS represents invaluable recognition for Inherited Heart Rhythm Disorders, the Electrophysiologists that treat them and the families affected by them. To learn more about the BRIDGE project visit the website.

http://bridge.ccs.ca/index.php/en/

Closer to home PACED is very pleased to announce that a new and improved version of Bill 81, The Inherited Heart Rhythm Disorder Awareness Act, 2012 is in the works and may be receiving First Reading at Queen’s Park prior to the Christmas Break.  Amongst other things  bill require that 911 be called for all children that faint during physical activity at school or while participating in minor sport and that they be physician cleared for return to play. It will also require educators and coaches to receive regular training on recognizing and responding to the warning signs.

http://www.ontla.on.ca/web/bills/bills_detail.do?locale=en&BillID=2629&isCurrent=false&ParlSessionID=40%3A1

Here are seven links to recent news stories that are directly related to creating greater awareness and heightened sensitivity to the warning signs and prevention strategies for Inherited Heart Rhythm Disorders and sudden cardiac arrest in youth. The Arrhythmia train is picking up passengers and momentum.