Three Thoughts for CPR Instructors and Students

Every person that attends CPR training represents a potential life giving resource, like a bottle of water in the desert, their attendance cannot be squandered. The next time you teach or take a certification level CPR course it may be helpful to keep these three thoughts in mind.

1. Doing something is NOT better than doing nothing - Instructors give their students a free pass when they utter the dreaded "doing something......" line. In most of the classes that I teach some student knowingly expresses the "doing something" sentiment. I gently correct them and point out that CPR is only effective when it is performed properly. Poor CPR produces results that are exactly equal to, not better than, doing nothing.

The reason that people take four or five hours out of their busy schedule to take a CPR course is to acquire a toolkit for protecting the life of a person experiencing a significant cardiovascular event. The excellent science published over the past decade gives us a clear indication of what works and what doesn't work in cardiac resuscitation. CPR instructors must teach the methods that work and CPR students must endeavour to learn and perform those techniques to the best of their ability. Teaching and learning effective CPR is the only way to improve survival rates for cardiac arrest in our communities. We trivialize and demean the process when we tell students that "doing something is better than doing nothing"

2. You DO NOT have to break ribs to perform effective CPR - I cringe every time I hear this. It seems there is a legion of CPR instructors out there that hammer on the idea that if you're not breaking ribs you're not doing it properly, including professional responders that brag to their students "I've done CPR over a dozen times and broken ribs every time." I don't know where to begin with this one, it is just wrong on so many levels.
First of all the science is clear and unequivocal, the majority of survivors do not have broken ribs. More importantly our job as CPR instructors is to bring down the barriers to the public getting involved with a patient when they witness a cardiac arrest. Telling lay people that in order to help that person they will have to break their ribs has precisely the opposite effect. Effective CPR does not require breaking ribs, it is okay to speak to the possibility of broken ribs, but it should be put in proper perspective and the whole discussion should only take up a few seconds of class time. I ask all instructors to please stop with the broken rib bull, and I implore all students the next time your Instructor plays the broken rib card, call his bluff.

3. Count using numbers, 1 and 2 and 3 and 4 ....  NOT old Bee Gee's songs - The Stayin' Alive gimmick is an excellent tool for promoting Bystander CPR in 60 second Public Service Announcements, it is not an appropriate technique to teach to a student that has signed up for certification level CPR training. I've met hundreds of people that can't remember the name of "that song you're supposed to sing" but I've never had a student that can't remember how to count to 5.
When you count properly, 1 and 2 and 3 and 4 and 5 and 1 and 2 and 3 and 4 and 10 ...... many excellent things happen for the patient. With very little practice students can develop a cadence that will produce a rate of 100 bpm, plus or minus 5, virtually every time. Proper rate makes a huge contribution to survival. When you push down on "One" and come up on "And" work is distributed equally between the two critical functions of chest compressions, pushing blood out to the brain and internal organs and allowing the heart to refill with blood on the upstroke. This drives a nice even circulation pattern that helps keep vital organs oxygenated. Finally when you count out loud using numbers, not Bee Gee's songs, all of the people assisting you with the resuscitation know exactly where you are in the CPR cycle and can perform their tasks accordingly.

The four hours that you spend teaching and  learning CPR and other Basic Life Support skills represent an opportunity to give life to another human being, in all likelihood a co-worker, a family member or a friend. It is a kind of sacred trust and should be treated with the appropriate respect.

Defibrillators versus Tasers

The front page of my hometown newspaper, The Hamilton Spectator, carried a story today on a police services request for $1million for the purchase of Tasers. I was struck be the similarity between the price of an X26 Taser and a top of the line Automated External Defibrillator (AED), a virtual dead heat at $1,455. The 687 AED’s that could be purchased with $1 million would allow for an AED to be placed in every Hamilton Police Services vehicle with many left over to be placed strategically throughout the community. In the first eight years of carrying AED’s in their police cruisers and responding to cardiac arrest calls Ottawa Police saved 20 lives. In Rochester, Minnesota a small city of 100,000 people police have been carrying AED’s since 1990 and have been credited with 127 lives saved.

In the Fall of 2012 every Ambulance Service in the Ontario submitted their new response time commitments to the Ministry of Health and Long-term Care. Hamilton EMS agreed to “endeavour” to achieve the Ministry established benchmark of having a trained responder equipped with an AED arriving on scene at 75% of all cardiac arrest calls within 6 minutes of EMS receiving the 911 call. The 6 minute benchmark is a worthy target and taking any longer to arrive at the side of a cardiac arrest victim will not likely result in a life saved. However several Ambulance Services have indicated to the Ministry that, while it is desirable, it is unlikely that responders will be arriving on scene in 6 minutes at or near the 75^th percentile. Historically this target has been difficult to achieve and increasing demand and scarce resources suggest it is unlikely to improve significantly as long as EMS is the sole responder to cardiac arrest events.

The communities that will meet or exceed the standard when the 2013 reports are submitted to the Ministry in March of next year will be those with deep and wide Public Access Defibrillation (PAD) programs and excellent Police and Fire tiered response protocols for cardiac arrest. Any event where an AED arrives at the patient’s side within 6 minutes whether it is manned by a teacher, a hockey coach, a co-worker, a family member, a firefighter, a paramedic or a police officer contributes to achieving the benchmark.

Why is the 6 minute standard so critical? The key statistical factor for improving outcomes is time, specifically the time from when the patient collapses to the time when they receive the first shock from a defibrillator. The places with the highest survival rates for Out-of-Hospital Cardiac Arrest (OOHCA) have the shortest time to first shock. Each year in Ontario over 7,000 people experience OOHCA and overall survival is less than 8%.  In Hamilton that translates to roughly 400 events per year with less than 32 survivors.

Across North America there are a growing number of communities committed to improving cardiac arrest response protocols that are achieving survival rates in excess of 20%. Large urban areas such as Seattle WA and Tucson AZ are reporting at 20%, Halton Region reported a 30% survival rate in 2010 and Rochester Minnesota reports a 42% survival rate. If survival rates for cardiac arrest in Hamilton doubled to 16% that would represent 32 additional lives saved each year and if they tripled to 24%, a lofty but realistic target, it would represent more than one additional life saved every week of the year.

Even $500,000 would purchase over 300 AED’s, more than enough to put one in every frontline police vehicle. Police officers are currently required to be trained in Basic Life Support/CPR so there is no additional training burden. The ability of police to be first on scene at most serious medical calls has been proven in dozens of urban centres including Ottawa. Equipping police with AED’s would go a long way to assisting Hamilton EMS achieve the benchmark of 6 minutes at the 75^th percentile.

It’s popular and easy (although not always productive) to suggest alternative uses for budget lines. In this instance one can’t help but see the irony. Hamilton Police are requesting $1 million to purchase hundreds of high energy devices that are used to debilitate and even "accidentally" kill people. That same dollar amount, or less, could just as easily be applied to the purchase of  high energy devices that police forces around the world are using daily to save lives.  

A Five Point Cardiac Arrest Prevention Strategy for Canadian Schools

Here is an excerpt from an article written on research presented by Dr. Andrew Krahn at the 2012 Canadian Cardiovascular Congress.

“Our research gives us an idea of the scope of the problem – there are almost 200 young people who die suddenly every year in Ontario. A good proportion of them have unrecognized heart disease. So the question is: How can we catch this before it happens,” says Krahn.

He suggests that more attention be paid to possible warning signs such as fainting. He believes that teachers, coaches and an aware public may be key to detecting risk, ensuring prevention and formal medical evaluation and therapy.

“I would advocate for careful screening of people who faint, using questionnaires and education of healthcare professionals so that when warning signs present themselves, they recognize them and this information gets passed on to the right people,” he says.

http://news.bioscholar.com/2012/10/hidden-disease-sports-sudden-cardiac-arrest.html

With virtually no hard cost every School Board could implement Dr. Krahn’s recommendations for preventing sudden death in children. The five key elements of a Cardiac Arrest Prevention Strategy are: 

A 20 Minute Arrhythmia Awareness Training Program that provides some background on Inherited Heart Rhythm Disorders (IHRD) and explains the warning signs and how to respond appropriately to those warnings. It should be available on-line to all staff and families but should be mandatory for all staff taking AED/CPR certification training and for all Physical Education Teachers and Coaches

A Pre-Participation Screening Questionnaire that would be completed by all parents/guardians at the time of enrolment at a new school. When there are positive answers in both the patient history and family history sections of the questionnaire the family should consult a physician immediately.
http://leadingcause.blogspot.ca/2013/05/inherited-heart-rhythm-disorder-pre.html

Mandatory 9-1-1 Calling  for Loss-of-Consciousness Fainting (syncope) It may be mandatory 9-1-1 for all syncope or at the very least mandatory 9-1-1 for all syncope occurring during or shortly after physical activity. In most communities ambulance are equipped with ECG monitoring equipment and paramedics are trained in heart rhythm recognition. Our tax dollars paid for this equipment and training it only makes sense to use it. 

Mandatory Notification of Parents/Guardians of all Syncope including providing them with information about IHRD’s. Parent's must be informed on the day of the event and must be provided with information that will help them understand the significance of fainting and other warning signs and the importance of physician follow-up. 

Mandatory Medical Clearance for Return to Play Post Syncope Perhaps the most tragic teen deaths are those where there is one or more fainting episodes in the weeks or months prior and the child is allowed to continue to participate in physical activity and dies because of it.

Pennsylvania has passed a law that includes several of these elements. Many other states including Maryland, Ohio and Indiana have pending Sudden Cardiac Arrest Prevention Legislation. Ontario allowed a bill to die on the order paper and it has yet to be revived despite having unanimous support at first and second reading.

If provincial or state legislation is not pending in your jurisdiction go ahead and implement these policies and best practices in your School Board. Work together with local paediatric cardiologists/electrophysiologists to develop the messaging around warning signs. Work with local EMS and ER Physicians to develop policies and practices around 9-1-1 calling for syncope. Trust that mandatory 9-1-1 calling for syncope (especially a child's first faint) occurring on school property will not create an avalanche of unnecessary "nuisance" calls, it won't. Remember that Automated External Defibrillators don't always work, it is better to prevent cardiac arrest than to try and reverse it with an AED.

One in twenty paediatric faints are sinister (the good news is that 19 in 20 are benign) but that number goes up if physical activity was the obvious trigger.  From a risk management standpoint it makes sense to be proactive on fainting and for the well being of our children it is imperative that all fainting be investigated.

Inherited Heart Rhythm Disorder (Pre-participation) Screening Questionnaire

An underlying genetic disorder is often the cause of sudden cardiac death in young people. These diseases claim as many as 700 young lives in Canada every year.  While the exact prevalence of these diseases is unknown most experts would agree that 1 in 500 is not an unreasonable estimate of the number of young people carrying a potentially lethal gene.  A good starting point for determining if your child may be affected is to complete both a Patient and Family History.

At important times in every child’s life take a moment to complete this questionnaire.

·        When starting Kindergarten

·        When starting Grade 5

·        When starting a competitive sport

·        When starting a prescription drug

·        When starting High School

·        When starting University

·        Anytime you are made aware that your child has fainted

Encourage children to self-report symptoms described in this questionnaire including extreme shortness of breath, palpitations, extreme fatigue and brown-outs during physical activity.         Insure that schools, clubs and sport leagues have a mandatory reporting policy for fainting.

Investigate the family history of both parents. When speaking with relatives and long-time family friends ask them if they are aware of any unexplained or presumed cardiac young person deaths in your family tree.

·        Any unexplained death of a person under the age of 50 should be considered. Drowning and motor vehicle deaths, especially unexplained single vehicle accidents should be included.

·        A key piece of the puzzle for one Canadian family was the story  of an 18 year old relative that died from auditory startle – in Italy, in the 1920’s – more than 80 years before the diagnosis

Patient History Questions

1.      Has this child ever fainted during or shortly after physical activity?

2.      Has this child ever experienced extreme shortness of breath, extreme fatigue or “brown outs” during physical activity? More so or different than other children?

3.      Has this child ever fainted as a result of emotional distress or excitement?

4.      Has this child ever fainted from auditory startle such as an alarm clock, a door slamming or any unexpected noise?

5.      Has this child ever fainted from any cause?

6.      Has this child ever sustained an injury as a result of fainting?

7.      Have any of this child’s faints involved seizure like activity?

8.      Has this child ever been diagnosed with a seizure disorder such as epilepsy?

Family History Questions

1.      Is there any history of unexplained early death on either side (maternal/paternal) of this child’s family? Include parents, siblings, grandparents, aunts, uncles, cousins. Go back as many generations as possible.

a.      More than one early death in the family?

b.      Unexplained death of family members under age 50?

c.      Unexplained death of family members under age 35?

d.      Any deaths occurring during or after intense physical activity? Running, swimming, cycling, soccer, hockey.

e.      Deaths of undetermined origin or “presumed” cardiac origin

f.       Are there any SIDS deaths (Sudden Infant Death Syndrome) in the family?

g.      Are there any deaths attributed to seizure disorder or epilepsy?

2.      Is there any member of this child’s family that has a history of unexplained fainting or seizures?

I have answered “Yes” to one or more questions

If the answer to any of these questions is “yes” you will want to consult with the child’s physician. The more “yes” answers you have the more important it becomes to see a doctor. If you have a “yes” answer in each of the Patient History and Family History sections you may wish to inquire about a few simple, non-invasive tests such as ECG and Echocardiogram.

If there is suspicion of a possible cardiac rhythm disorder in any member of your family it is important to seek definitive answers. Most of these diseases are inherited through an autosomal dominant gene which means that they affect males and females equally and if one parent carries the gene on average half of their children will acquire the gene and the disease. Positively identifying one family member should begin a process of finding others. Once identified there are a number of available therapies that provide excellent protection against Sudden Cardiac Death.

Preventing the Leading Medical Cause of Paediatric Mortality

In his paper presented at the 2012 Canadian Cardiovascular Congress Dr. Andrew Krahn showed that in Ontario in 2008 almost 200 young people, under age 40, died suddenly from cardiac arrest resulting from an underlying Inherited Heart Rhythm Disorder (IHRD). These numbers are consistent with the reported incidence of sudden unexplained death amongst otherwise healthy young people from other countries and jurisdictions around the world. These numbers suggest that taken together the group of heart arrhythmias known as IHRD’s may be the leading medical cause of death in the paediatric population in developed countries.

The challenge in reducing the toll that these diseases take is that in most cases the patient is otherwise healthy making identifying at risk patients difficult. For roughly half of the young people that die from an IHRD related cardiac arrest the first indicator of the disease is death. Of the other half many present with warning signs in the weeks or months prior to their death. The most obvious warning sign is fainting (syncope) others include; palpations, racing heart, extreme shortness of breath, brown outs, and dizziness; with any of these episodes being triggered by physical activity, emotional distress, excitement, auditory startle or no obvious trigger.

Two strategies for identifying at risk individuals and protecting them from cardiac arrest are;

1.      Raising awareness of the warning signs of IHRD’s and encouraging parents, educators and minor sport officials to be diligent in following-up on syncope and other warning signs with a knowledgeable physician. This approach can be very effective for diagnosing disease in the half of the affected population that exhibit warning signs.

2.      Screening programs which include a pre-participation screening questionnaire and a resting ECG. This option is the most cost effective method for identifying patients in the half of the affected population that present with no obvious symptoms of disease.

Note: Most IHRD’s are heritable diseases transmitted by an autosomal dominant gene. Therefore the yield from any awareness or screening programs must be far greater than the total number of index patients identified. Dr. Joel Kirsh, Sick Kids, Toronto, suggests that he typically identifies five or six first degree relatives in addition to the index patient.

Once diagnosed, most patients with an IHRD can expect to live a long and productive life. Excellent prophylaxis from lethal tachyarrhythmia can be provided by implantable devices, pharmaceuticals, surgical procedures, lifestyle modification or a combination of the above.

PACED - Parents Advocating for Cardiac Education

PACED (Parents Advocating for Cardiac Education) is loosely structured group of families affected by IHRD’s advocating for greater awareness and identification of these diseases. PACED calls upon a number of cardiologists and electrophysiologists to advise us on how best to direct our efforts.  To date those efforts have been focused in two areas;

1.      Putting on seminars in our communities to educate; primary care physicians, educators, sport officials on understanding these diseases with a focus on recognizing the warning signs and responding to them.

2.      Developing and advocating for Bill 81, The Inherited Heart Rhythm Disorder Awareness Act, 2012. This legislation unanimously passed second reading in the Ontario Legislature and unfortunately died on the order paper when the provincial parliament was prorogued in October 2012. The bill would have been the first in Canada and the second in North America (Pennsylvania) to address awareness of IHRD’s

A Proposal for Finding Children Living with an IHRD

PACED is interested in implementing both an awareness and screening campaign running concurrently within a defined geography. One jurisdiction which we feel would be an ideal incubator for IHRD awareness is the Hamilton/Niagara/Haldimand/Brant LHIN (Local Health Integration Network). This LHIN features a manageable, yet statistically significant population, a respected teaching hospital and Children’s Hospital at McMaster University, a Cardiac Imaging Technicians program at Mohawk College and a number of organizations such as Heart Niagara that may be supportive of the initiative.  Equally important is potential for researchers from McMaster Children’s Hospital and McMaster University to become involved should one of the objectives be publication. By working with stakeholders in a defined region we hope to be able to produce measurable and quantifiable change in the recognition of IHRD’s and the prevention of paediatric sudden cardiac death.

The Awareness Campaign

Over a decade of working to raise awareness of IHRD’s we have identified three key target audiences that need to hear and act on the awareness message

1.      Primary Care Physicians. Much of the current understanding of IHRD’s has come about in this century with the first genes connected to Long QT being identified in 1995-96. Awareness campaigns are designed to have parents take their children to see a Family or ER physician anytime warning signs are observed. It is therefore critical to the success of a campaign that these key partners are equipped with the latest knowledge and tools for diagnosing and managing these patients

2.      Community Leaders in Education and Sport. The efficacy of an awareness campaign is contingent upon post syncope patients being seen by a physician. School Board Directors of Education and Superintendents and Minor Sport Organization Executives and Directors have the authority to mandate medical follow-up for all syncope and other warning signs. They also have the authority to implement and enforce return to play policies for post syncopal children. In our experience most are willing to do so once they understand what is at stake.

3.      Parents, Teachers and Coaches. Everyone that spends time with young people needs to know the warning sign and be prepared to either get the patient to a physician or advise a parent or guardian of the importance of doing so.

There is a vast array of communication options available to assist in spreading the IHRD awareness message to the target audiences. It may seem a little old fashioned, but bringing target audiences together for a brief (one to three hour) seminar with information delivered by Electrophysiologists and other subject matter experts and with ample time for dialogue, is still highly effective. A series of a dozen or more seminars, with three or four aimed at each target audience, would be the cornerstone of an awareness campaign.

This information could be supported and enhanced by development of a teaching video and other on-line tools. Stakeholder organizations could provide links to this information on their respective websites and direct members with questions or concerns to the online material. Another longstanding vision of PACED is funding for a traditional electronic media (television and radio) public service announcement (PSA) campaign.

The messaging in the awareness campaign will be designed to initially steer at risk children to their family physician and ultimately to the regional centre of excellence for paediatric cardiology where a comprehensive work-up and definitive diagnosis will be completed. A few of the measurable outcomes of this initiative will include:

1.      An increase in the number of patients being appropriately referred to the regional centre of excellence.

 

2.      A decrease in the number of patients being inappropriately referred.

3.      An increase in the diagnosis of IHRD’s in the regions paediatric population

4.      A decrease in incidence of sudden cardiac arrest in the paediatric population

A Canadian Pilot in ECG Screening

There is a worldwide movement toward ECG screening for teenagers, especially competitive athletes, for the purpose of identifying underlying heart rhythm disorders including both cardiomyopathies and channelopathies. To date we are unaware of any ECG screening projects conducted in Canada. It would be instructive to complete a pilot that includes enough subjects to give the study horsepower, a minimum of 1,000 and perhaps even double that number. From the very beginning we wish to state that we would want no more than one third of study participants to be competitive athletes. Genetic diseases do not recognize athletic ability and will visit any child without discrimination.

The most referenced screening program was completed in northern Italy and attributes an 89% decrease in sudden cardiac arrest deaths amongst competitive athletes in the region to a comprehensive screening program. A more recent and equally compelling study from Switzerland was presented at the 2012 ESC Congress in Munich. The study found 1:250 athletes screened ultimately received a diagnosis of a potentially lethal heart arrhythmia http://www.medicalnewstoday.com/releases/249534.php

In order to simplify the inclusion process we propose that study be conducted with subjects that have attained the age of majority. A university would be an ideal place to complete the study. The magnitude and the endpoints for a screening program would be determined by funding levels and in-kind contributions. The barrier that we have encountered when proposing ECG screening programs in Canada has been finding qualified Cardiologists willing and/or able to read the ECG’s. Many screening programs in the US and UK have cardiologists that donate their time to the program.

Both of the programs outlined above could be completed as research projects with publication being a core objective or they could be done simply as community awareness and screening programs. The first option would give the programs far greater credibility. The second option would allow for faster, lower cost implementation and perhaps a greater reach at the grassroots level. In either case children and families affected by IHRD’s will be identified.

The prevalence of IHRD’s is debated amongst Electrophysiologists with numbers above and below a rough mean of 1 in 500 being commonly used. If we use the mean it suggests that there are 28,000 Canadians under the age of 35 living with an IHRD.  There are likely two students in every typical Ontario High School living with an IHRD. Identifying these children and their affected family members and getting them the treatment that they require is the endpoint of this proposal.

The Knowledge Trapped Inside an AED

Reading this awesome commotio cordis "save" story today reminded me of an issue that I have been raising for a number of years.

http://www.thecommunityvoice.com/article.php?id=6321

It seems counter intuitive, certainly to a lay person like myself, that an AED is far more likely to work on a 60 year old fan collapsed in the stands than on a 20 year old athlete lying on the field. It seems as if the younger (healthier?) heart should be a better candidate for a jump start. However the data is clear, in the two studies below no intercollegiate athletes were saved although several received a shock from an AED.

http://www.ncbi.nlm.nih.gov/pubmed/21081638
http://www.ncbi.nlm.nih.gov/pubmed/16177599

The obvious question is - Why?  Electrophysiologists have a pretty good understanding of why, or at least why they think AED's often don't work on young athletes. The word "acidosis" usually comes up, along with a number of other popular theories.

My first question is - Can we be doing more to understand what is happening to the heart of a young athlete that arrests during or shortly after intense physical activity?

My second question is - With greater understanding can we develop new algorithms for AED's and new protocols for trained responders that will result in higher survival rates for young athletes?

My third question is - Should we/us/somebody/anybody be setting up an event data registry, that includes the downloaded ECG data from the AED, for all cardiac arrest events involving young people.

Researching cardiac arrest in the adult population is simplified by the fact that researchers can acquire data from thousands of events quite easily. Cardiac arrest in young athletes is relatively rare so to acquire even 1,000 data sets, with ECG attached, is a challenge. If there were a central registry where defibrillation program coordinators, athletic trainers and first responders from across the country could send event data it would provide researchers with the horsepower they need to make meaningful determinations. Collecting the ECG data from every event occurring at elementary schools, high schools, colleges, universities, and community sport venues would, within a few years, provide thousands of records for researchers to consider

The AED captures the earliest presenting rhythm and is more likely to contain answers than ECG's acquired by paramedics or in the ER. If the first ECG is captured after ROSC is achieved there is no record of the presenting rhythm. Knowing exactly what's happening electrically to an athletes heart within the first moments after collapse seems to me to be the first step in figuring out how to improve outcomes.

If one good thing comes out of the death of a young person it may be that the ECG downloaded from the AED (that failed to convert their tachyarrhythmia) contributes to saving young lives in the future. I know that over the past decade AED's have been applied to hundreds of collapsed athletes and sadly the ECG's captured by those devices have not contributed to a greater understanding of cardiac arrest in youth.

A Long QT Story from March 2013

A Plea to Parents and Family Physicians

The problem of misdiagnosis of Long QT Syndrome, often as a seizure disorder or epilepsy, has long been recognized and Electrophysiologists (EP's) have wrestled with how to solve the problem. Attached is a link to the abstract for Dr. Judith MacCormick's 2009 study of a cohort of 31 Long QT patients in New Zealand.

http://www.annemergmed.com/article/S0196-0644(09)00113-9/abstract

A parent reading this abstract might sum it up in one word, frightening. Four years on there has been marked improvement in the management of these patients but the misdiagnosis problem is far from being solved.

This past week I delivered an AED with training to a family in a town that is about a half hour drive from Toronto, Ontario, Canada, a part of the world where paediatric medicine is second to none. Their young teenager had just been diagnosed with Long QT type 2, and in addition to other therapies the patient's EP prescribed an AED for home and school. Multiple fainting/seizure episodes when the child was less than five years old had been misdiagnosed as a seizure disorder. Ten years on a near death event, triggered by physical activity, led to a proper diagnosis of Long QT Syndrome. Thankfully, the patient and the family cheated the odds for sudden cardiac death, but now they are faced with the challenge of ending a promising athletic career.

This close to (my) home story mirrors much of Dr. MacCormicks data from half a world away including a 10 year delay in diagnosis after an initial misdiagnosis of epilepsy. The greater concern of course is the significant number of preventable deaths in both the probands and their first degree relatives occurring during the long diagnostic delay, four in the NZ Study.

One of the common confounding factors is that fainting (syncope) associated with Long QT Syndrome and several other Inherited Heart Rhythm Disorders, often presents as seizure like activity. The likelihood that these types of events are neurological in origin is far greater than the likelihood that they are of a cardiac origin. However if a definitive neurological cause cannot be determined testing to rule out cardiac origin should be completed and interpreted by a physician that understands paediatric arrhythmia.

If you are the parent of a child, or know of a child or any person, that has ever been diagnosed with epilepsy or seizure disorder after experiencing one or more seizure/syncope episodes,   

• especially if the diagnosis was one of "idiopathic" epilepsy
• or if the diagnosis was made without an ECG and other testing to rule out cardiac origin
• or if the ECG was not interpreted by a Paediatric Cardiologist or EP
• or if the physician seemed in anyway non-committal or unsure in their diagnosis
• or if other first degree relatives have experienced seizure/syncope episodes
• or if your "instincts" tell you that the diagnosis should be revisited

you should work with the Family Physician to arrange for cardiac testing that will provide an accurate and up to date picture of the patients cardiac health

If you are a Family Physician that has a patient on your roster that meets any of the above criteria perhaps you would consider doing a new investigation of both the patient and other family members. Diagnosis and testing completed in the 20th century is particularly suspect and as the case noted above points out even a diagnosis from the 21st century can be incorrect and putting a patient at unnecessary risk of sudden death.

Whether you are family member or a Family Physician if you know of a person with a diagnosis of epilepsy or seizure disorder and it just doesn't seem to add up or sit well, consider circling back around to revisit possible cardiac origin. It may save a life, or two .....