Research Newsletter 2017

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Registered Charity No. 1050845

Research Highlights 2017 Inside this issue. . .

P2 CRY International Conference P3 European Conferences P4 Interview with Professor Sharma P5 Interview with Dr Dhutia P6 Papers/Articles P7 Presentations P8 CRY’s Research Programme

Offering help and support to affected families @CRY_UK CardiacRiskintheYoung

CRY International Medical Conference


The 12th annual CRY Conference on 13 October 2017 was a fantastic success. CRY Consultant Cardiologist, Professor Sanjay Sharma, was a key part of the event, serving as the chair to many presentations to moderate, whilst also presenting himself. After former CRY Research Fellow Dr Sabiha Gati and Dr Nabeel Sheikh, Professor Sharma gave the third talk of the day, entitled ‘International recommendations; Bringing it all together.’ He began by emphasising just how much research is happening because of CRY’s support and proceeded to explain how the interpretation of ECG results has progressed in recent years, including the influence that CRY has had with the introduction of the improved refined criteria thanks to years of vital research. CRY was also well represented by several of our Research Fellows, including Dr Aneil Malhotra, who spoke after Professor Sharma on ‘Screening elite football players; The FA experience.’ Dr Malhotra stressed the prevalence of cardiac issues in adolescent football, which was the leading cause of death in 23 adolescent football players, who died between 2002 and 2016, at 35%. Screening is typically mandatory for elite athletes and professional footballers, whereas young people at an amateur

Presentations Updates on the interpretation of the athlete’s ECG “The athlete’s ECG; The things you should clear” Dr Sabiha Gati “ECG indices of cardiomyopathy; The things you should not miss” Dr Nabeel Sheikh “International recommendations; Bringing it all together” Professor Sanjay Sharma “Screening elite football players; The FA experience” Dr Aneil Malhotra “ECG Screening of young individuals; Myths and reality” Dr Harshil Dhutia

“Arrhythmogenic Right Ventricular Cardiomyopathy; Overlap with arrhythmia syndromes” Dr Elijah Behr “Arrhythmogenic Right Ventricular Cardiomyopathy; Novel treatment options” Dr Angeliki Asimaki Developments in Sports Cardiology “Adaptations of the female athlete’s heart” Dr Gherardo Finocchiaro “Effect of regular training in novice marathon runners” Dr Andrew D’Silva

level are not routinely offered the opportunity to be screened. Former CRY Research Fellow, Dr Harshil Dhutia, reiterated this message in his presentation on ‘ECG Screening of young individuals; Myths and reality?’ One of the biggest issues with sudden cardiac death among young, grass roots level athletes is that so few are screened. This only heightens the importance of CRY’s screening programme and the need to raise awareness. After the first break of the morning, CRY Research Fellow Dr Gherardo Finocchiaro continued the day with his presentation, ‘Adaptations of the female athlete’s heart.’ Dr Finocchiaro discussed some of the differences between the hearts of men and women and mentioned some startling statistics in their respective rates of sudden cardiac death (SCD): men are 10 times more likely to die of SCD than women in professional sports and 20 times more likely to die of SCD in recreational sports. Professor Mary Sheppard, Consultant Cardiac Pathologist at CRY’s Centre for Cardiac Pathology, gave a talk that highlighted just how much CRY’s research is progressing. Professor Sheppard began by thanking CRY for its support, adding that the increasing number of referrals received (including consent for research purposes) at the CRY Centre for Cardiac Pathology greatly helps CRY’s research into better understanding the causes of young sudden cardiac death. In addition to past and present CRY doctors, other experts such as Professor Mats Börjesson from Gothenburg, Sweden, also made it apparent just how big an impact CRY has. He then proceeded to discuss the prevalence of coronary artery anomalies.

“The heart of the master athlete” Dr Ahmed Merghani

To conclude the day’s presentations, myheart cardiologist Dr Michael Papadakis discussed the exercise recommendations for athletes with cardiomyopathy and the factors that determine the intensity of exercise they can complete and any lifestyle changes they may need.

Arrhythmogenic Cardiomyopathy; Current challenges and future perspectives

“Prevalence of coronary artery anomalies; Data from a large CT database” Professor Mats Börjesson

After all the presentations, there were two sports cardiology case study sessions that covered electrical disease, cardiomyopathies and structural disease.

“Arrhythmogenic Right Ventricular Cardiomyopathy; Not so right-sided after all” Professor Mary Sheppard

“Exercise recommendations in athletes with cardiomyopathy; An update” Dr Michael Papadakis


Research Highlights 2017

For videos of all the presentations visit Cardiac Risk in the Young

European Conferences ESC Congress

BCS Conference

The 2017 European Society of Cardiology Congress was held in Barcelona, Spain, as over 31,700 healthcare professionals assembled from a record 153 countries. Several CRY doctors were at the event to represent us and provide their expert insight on a variety of topics. Dr Gherardo Finocchiaro presented on ‘Cardiac symptoms before unexpected sudden cardiac death in the young: Data from a large pathology registry’ and CRY Research Fellow, Dr Stathis Papatheodorou, presented a poster on ‘Comprehensive familial evaluation in Sudden Arrhythmic Death Syndrome (SADS) families leads to significant yields of Brugada syndrome (BrS)’. As the Medical Director of the London Marathon, Professor Sanjay Sharma’s presentation discussing how to deal with heatstroke and a collapse contained excellent advice. He broke down the ‘ABC Management’ of how to handle an exerciserelated collapse, which focuses on the steps of checking ‘Airway’, ‘Breathing’ and ‘Circulation’.

The annual British Cardiovascular Society Conference, hosted at the Manchester Central Convention Complex, featured a main theme of “cardiology at the extremes” this year. Multiple CRY doctors were in attendance, including Dr Gherardo Finocchiaro and Dr Keerthi Prakash (below), who provided insightful poster presentations on, respectively, the impact of body size on cardiac structure for those at risk, and the difference in exercise performance for those with Hypertrophic Cardiomyopathy based on ethnicity.

Professor Sharma also gave expert tips on how to manage heatstroke, how to prepare for a marathon and how much you should drink during the race. CRY research was also presented by Professor Silvia Priori, who gave a presentation on Dr Harshil Dhutia’s paper highlighting the importance of having experienced cardiologists for interpreting ECG results correctly. Professor Priori also commented that Dr Dhutia’s paper is “one of the most important studies in sports cardiology.” One standout achievement from the conference was Dr Papatheodorou winning the award for the best moderated poster presentation for his poster entitled ‘Validation of the proposed Shanghai Brugada Syndrome Score (SBrS) in a cohort of relatives of Sudden Arrhythmic Death Syndrome (SADS) victims.’

Cardiac Risk in the Young

Dr Aneil Malhotra spoke about testmyheart and the growth of CRY’s research, which helped highlight the work we are doing alongside the bereavement support and screenings we provide. Professor Sharma also attended and addressed the importance of exercise, which is a key message to enforce, given the worry that some have when sudden cardiac deaths occur during exertion.

EuroPrevent Many CRY doctors spoke at the EuroPrevent conference in 2017. Dr Michael Papadakis presented multiple pieces of CRY Research, including “Effect of ethnicity, age, gender and sporting discipline on the athlete’s ECG”, whilst Professor Mary Sheppard provided her specialist knowledge in pathology by presenting “Contemporary studies of sudden cardiac death in young athletes.” A full list of the presentations given by CRY doctors at EuroPrevent 2017 can be found on page 7. Research Highlights 2017



Interview with Professor Sanjay Sharma What is the impact of exercise on the heart?

Participation in regular intensive exercise (more than four hours per week) is associated with a collection of structural and functional changes within the heart that also impact on the surface ECG. The ECG in athletes shows sinus bradycardia and large QRS complexes. The magnitude of these changes is dependent on several factors, including age, sex, ethnicity and type of sport. On occasion, the ECG may show features that overlap with those also observed in patients with cardiomyopathy. This issue is particularly relevant in athletes of African or Afro-Caribbean origin (black) and endurance athletes.

borderline variant do not require further investigation. Such practice has been validated in two large studies from the UK and Qatar which have shown increased specificity without compromising sensitivity. Indeed the false positive rate with the ESC 2010 recommendations was as high as 25%, but with current recommendations is expected to be less than 3%. Our own experience in the UK reveals a false positive rate of 2.5%. How has your research into black athletes impacted the new recommendations? The new recommendations are more specific about the normal repolarisation patterns in black athletes (anterior T-wave inversion preceded by J-point elevation and ST segment elevation). This data is also derived from the largest study in black athletes, published in the EHJ in 2010, which showed that 12% of black athletes reveal this repolarisation pattern in the absence of any overt pathology on cardiac MRI, exercise stress test and Holter. As such, these patterns are now classified as normal variants. The previous guidelines did not include the black athlete’s ECG pattern in any category.

Why is it important to understand these changes?

How are the new recommendations approaching T-wave inversions?

Recognition of these factors is important to reduce the false positive rate during ECG reporting in athletes. The original European Society of Cardiology (ESC) recommendations for interpretation of the athlete’s ECG were associated with a high false positive rate because they did not allow for ethnic differences and focused mainly on leisure athletes rather than highly competitive athletes. Studies in predominantly white athletes have shown that the false positive rate with the new recommendations is less than 3%.

The new recommendations incorporate T-wave inversion in leads V1 and V2 in the normal category based on data in 14,000 young white patients, of which 350 (with anterior T-wave inversion) were investigated comprehensively for cardiomyopathy. The recommendations also demonstrate methods of differentiating anterior T-wave inversion in athletes from anterior T-wave inversion observed in black and white patients with cardiomyopathy based on the preceding J point and ST segment which was published in the EHJ (2015).

Why are these new international recommendations for interpreting ECGs so important?

Why is age an important part of the new recommendations?

There have been several developments in the interpretation of the athlete’s ECG since 2010 (Seattle criteria and the refined criteria) which are all incorporated in the new international recommendations. Most of the studies leading to these developments were conducted at St George’s, University of London, and funded by CRY. The international recommendations seek to provide just one set of uniform recommendations that all sports cardiologists adhere to. The paper discusses the evolution of ECG criteria and refers to the foundation set by the ESC 2010 recommendations. What are the major changes to the way ECGs are interpreted in the new recommendations? As opposed to just two categories – normal (type 1 patterns) and abnormal (type 2 patterns) – the current recommendations have a borderline variant category. This category has been derived from two large studies published in the European Heart Journal (EHJ) in 2014, which revealed that (1) voltage criteria for atrial enlargement of axis deviation in isolation don’t usually represent cardiac pathology in young athletes and (2) voltage criterion for right ventricular hypertrophy is common in athletes and does not equate to right ventricular pathology (Zaidi). Based on these findings we have proposed that asymptomatic athletes with just one


Research Highlights 2017

For the first time the ECG recommendations consider age and make allowances for anterior T-wave inversion (V1-V3) in athletes aged under 16, which was absent in the 2010 recommendations. These recommendations are also derived from large cohorts of paediatric athletes and one paper was published in the EHJ. What about long QT and short QT? How are these new recommendations different? The QT interval cut-offs are less conservative and this is explained in the manuscript. The basis of a definitely prolonged QT interval in the athlete (> 500 msec) was also published in the EHJ in 2008, but was not clear in the ESC 2010 recommendations. The new definition of a short QT interval (< 320 msec) in an athlete has been validated in a large study of over 10,000 young British individuals (Dhutia) which was published last year. What is a take home message for those interested in taking forward screening? How is this paper going to help them? For the first time the authors propose a minimum set of recommendations for investigating athletes with specific ECG abnormalities which was totally absent in the 2010 recommendations.

Cardiac Risk in the Young

Interview with Dr Harshil Dhutia Edited excerpts from an interview with Dr Dhutia, who was a CRY Research Fellow from 2013 to 2016. His paper “Inter-Rater Reliability and Downstream Financial Implications of Electrocardiography Screening in Young Athletes” was published in the American Heart Association in August 2017. Why did you investigate the variation in ECG interpretation among cardiologists of differing experience? As with any subjective test, there is concern regarding the variation of the ECG as a screening tool. Prior to this study, there was a limited appreciation of the degree of variation in ECG interpretation in athletes from small studies. Furthermore, I wanted to investigate the impact of newer ECG criteria on ECG interpretation, as well as investigate trends in clinical practice amongst cardiologists when faced with a relatively large real life sample of athlete ECGs. How did you conduct your study? Eight cardiologists (including four who routinely screen young athletes with ECGs) independently reported on the ECGs of 400 young, competitive athletes consecutively screened through the CRY programme in 2014. The cardiologists were blinded to the clinical history of the athletes and reported the ECG in accordance with three major interpretation criteria including the refined criteria, which was derived from research supported by CRY. In addition to reporting the ECG findings, the cardiologists provided recommendations on further investigations following ECG interpretation based on their routine clinical practice. We then investigated the inter-observer agreement for ECG interpretation and further investigations amongst the two groups of cardiologists. Even with differences between inexperienced and experienced cardiologists, does the use of CRY’s refined criteria help increase the detection of abnormalities overall? The refined criteria improved the degree of inter-observer agreement for ECG interpretation, especially amongst less experienced cardiologists, and reduced the gap in agreement between experienced and inexperienced cardiologists. This is pertinent as screening is often undertaken by a variety of physicians of different experience. The study was not designed or powered to test detection of disease. However, we have already shown that the refined criteria significantly reduces the proportion of abnormal ECGs from screening requiring further investigation without compromising the ability to identify serious cardiac disease (Dhutia et al, JACC 2016). Is CRY continuing to work on the reduction of false positives and the cost of ECGs/further testing? Research from CRY has formed the blue-print for the recently published international recommendations for ECG

Cardiac Risk in the Young

interpretation in athletes. We have shown that these new guidelines have further reduced the false positive rate to the 3% mark and reduced the cost of screening (Dhutia et al; JACC 2017). Such a low false positive rate represents significant progress in our understanding of ECG patterns in young athletes and will likely be acceptable to the most stringent critics of ECG screening. The cost of cardiovascular evaluation per athlete was $175 for inexperienced cardiologists and only $101 for experienced cardiologists. There was also a notable margin in the amount of ECGs categorised as abnormal and the amount of athletes referred for further testing between both groups of cardiologists. What do you think is the best way to tackle the difference, both financial and technical, between inexperienced and experienced cardiologists? We found that inexperienced cardiologists were more likely to categorise ECGs as abnormal compared to experienced cardiologists with lower inter-observer agreement rates. However, even amongst experienced cardiologists, interobserver agreement was moderate at best. These findings suggest that whilst experience is useful, formal training and possibly accreditation is required to support physicians involved in ECG interpretation in young athletes, either for screening or for diagnostic purposes. The MSc in sports cardiology and the ECG interpretation course at SGUL are examples of tangible methods to achieve the necessary training for ECG interpretation in athletes. Likewise, inexperienced cardiologists were more likely to request a higher frequency of further investigations (such as echocardiogram, MRI, exercise test and Holter) following ECG interpretation with poor-fair inter-observer agreement for these tests. Experienced cardiologists demonstrated a higher degree of inter-observer agreement (ranging from fair-moderate) than inexperienced cardiologists, which is reflected in the lower cost per athlete screened for screening performed by this group. These findings are not entirely surprising given that there is very little guidance on investigating asymptomatic young athletes with ECG abnormalities. In this regard, the recently published international recommendations are unique in that they provide a list of minimum investigations for ECG abnormality. Such guidance will hopefully reduce variation in individual clinical practice which should improve efficiency and reduce costs. What do you hope to research next? I hope to report on the outcomes of the CRY general population screening programme of nearly 27,000 young individuals. The results are highly anticipated in the scientific world as it is now appreciated that sudden cardiac death in non-competitive athletes is higher than previously thought, questioning the ethics of limiting screening to elite athletes, as is the case at present. Preliminary findings were presented at the American Heart Association and EuroPrevent with excellent feedback. Research Highlights 2017



Papers/Articles • “Pre-participation Cardiac Screening in Young Athletes: In Search of the Golden Chalice.” Papadakis M, Sharma S. The Canadian Journal of Cardiology, January 2017.

“Physical activity confers substantial health benefits and promotes longevity of life. Paradoxically, however, intensive exercise is also occasionally associated with sudden cardiac death and generates considerable attention when a high-profile young athlete is affected. In the majority of cases, exercise is the trigger for fatal arrhythmias in athletes with quiescent cardiac disease.” • “Pre-participation Cardiovascular Evaluation for Athletic Participants to Prevent Sudden Death: Position Paper from the EHRA and the EACPR, Branches of the ESC. Endorsed by APHRS, HRS, and SOLAECE.” Mont L et al. European Journal of Preventive Cardiology, January 2017.

“Prospective studies are necessary to clarify if exercise is indeed a risk factor for SCD and the level of exercise that is safe in patients with a diagnosis of HCM. Such studies will inform exercise prescription in these patients which is currently lacking, leading to either a sedentary lifestyle because of overprotection or in some cases to potentially detrimental exercise behaviours.” • “Utility of Post-Mortem Genetic Testing in Cases of Sudden Arrhythmic Death Syndrome.” Lahrouchi N, Raju H, Lodder EM et al. Journal of the American College of Cardiology, May 2017. “Our data highlighted the predominant role of catecholaminergic polymorphic ventricular tachycardia and long QT syndrome, especially the RYR2 gene, as well as the minimal yield from other genes. Furthermore, we showed the enhanced utility of combined clinical and genetic evaluation. Furthermore, we showed the enhanced utility of combined clinical and genetic evaluation.” • “Ethical Considerations for Genetic Testing in the Context of Mandated Cardiac Screening Before Athletic Participation.” Magavern EF, Badalato L, Finocchiaro G et al. Genetics in Medicine, May 2017.

• “Anterior T-Wave Inversion in Young White Athletes and Nonathletes: Prevalence and Significance.” Malhotra A, Dhutia H, Gati S et al. Journal of the American College of Cardiology, January 2017.

• “Emergency Response Facilities Including Primary and Secondary Prevention Strategies Across 79 Professional Football Clubs in England.” Malhotra A, Dhutia H, Gati S et al. British Journal of Sports Medicine, June 2017.

“[Anterior T-Wave Inversion - ATWI] is present in 2.3% of the young white population and is more common in women and in athletes. Almost 80% of ATWI is confined to leads V1 to V2 and has a poor diagnostic yield for cardiac pathology, implying that this ECG pattern could be considered a normal phenomenon in asymptomatic individuals without a family history of cardiomyopathy or premature SCD.”

“The majority of football clubs in England have satisfactory prevention strategies and emergency response planning in line with European recommendations. Additional improvements such as increasing awareness of European guidelines for emergency planning, AED training and mentorship with financial support to lower division clubs are necessary to further enhance cardiovascular safety of athletes and spectators and close the gap between the highest and lower divisions.”

• “International Recommendations for Electrocardiographic Interpretation in Athletes.” Sharma S, Drezner J, Baggish A et al. European Heart Journal, February 2017. “The international consensus standards presented on ECG interpretation and the evaluation of ECG abnormalities serve as an important foundation for improving the quality of cardiovascular care of athletes.” • “International Recommendations for Electrocardiographic Interpretation in Athletes.” Sharma S, Drezner J, Baggish A et al. Journal of the American College of Cardiology, February 2017. • “Grey Zones in Cardiomyopathies: Defining Boundaries Between Genetic and Iatrogenic Disease.” Quarta G, Papadakis M, Donna PD et al. Nature Reviews Cardiology, February 2017. “In this Review, three of the most common and controversial areas are discussed, including left ventricular hypertrophy; left ventricular dilatation, noncompaction, and fibrosis; and arrhythmias originating from the right ventricle. A systematic and cautious approach is necessary in patients with mild phenotypes suggestive of, but not definitely diagnostic for, cardiomyopathies.” • “Sudden Cardiac Death in Pre-Excitation and Wolff-ParkinsonWhite: Demographic and Clinical Features.” Finocchiaro G, Papadakis M, Behr E et al. Journal of the American College of Cardiology, March 2017. “This study sought to describe the clinical and pathological features of [sudden cardiac death] cases with a premorbid diagnosis of [WolffParkinson-White].” • “Preventing Stroke and Assessing Risk in Women.” Keteepe-Arachi T, Sharma S. The Practitioner, March 2017. • “International Criteria for Electrocardiographic Interpretation in Athletes: Consensus Statement.” Drezner J, Sharma S, Baggish A et al. British Journal of Sports Medicine, May 2017. “The international consensus standards presented on ECG interpretation and the evaluation of ECG abnormalities serve as an important foundation for improving the quality of cardiovascular care of athletes. As new scientific data become available, revision of these recommendations may be necessary to further advance the accuracy of ECG interpretation in the athletic population.” • “Sudden Cardiac Death.” Finocchiaro G, Papadakis M, Sharma S et al. European Heart Journal, May 2017.


Research Highlights 2017

• “Left atrial function and phenotypes in asymmetric hypertrophic cardiomyopathy.” Kobayashi Y, Wheeler M, Finocchiaro G et al. Echocardiography, June 2017. “Few studies have analyzed changes in left atrial (LA) function associated with different phenotypes of asymmetric hypertrophic cardiomyopathy (HCM). We sought to demonstrate the association of impairments in LA function with disease phenotype in patients with obstructive and nonobstructive HCM.” • “Reply: Are T-Inversions in Chest Leads Always Benign?” Malhotra A, Dhutia H, Gati S et al. Journal of the American College of Cardiology, July 2017. • “Sudden Unexplained Death in Alcohol Misuse (SUDAM) Patients Have Different Characteristics to Those Who Died From Sudden Arrhythmic Death Syndrome (SADS).” Sorkin T, Sheppard MN. Forensic Science, Medicine, and Pathology, July 2017. “SADS, unlike SUDAM, is often associated with heritable channelopathies that may affect surviving family members. Therefore, differentiating between SUDAM and SADS identifies families likely to benefit from screening for these mutations, thus preventing further sudden arrhythmic deaths.” • “Prevalence of Subclinical Coronary Artery Disease in Masters Endurance Athletes With a Low Antherosclerotic Risk Profile.” Merghani A, Maestrini V, Rosmini S et al. Circulation, July 2017. • “Inter-Rater Reliability and Downstream Financial Implications of Electrocardiography Screening in Young Athletes.” Dhutia H, Malhotra A, Yeo TJ et al. American Heart Association, August 2017. “Interpretation of the ECG in athletes and the resultant cascade of investigations are highly physician dependent even in experienced hands with important downstream financial implications, emphasizing the need for formal training and standardized diagnostic pathways.” • “Impact of the International Recommendations for Electrocardiographic Interpretation on Cardiovascular Screening in Young Athletes.” Dhutia H, Malhotra A, Finocchiaro G et al. Journal of the American College of Cardiology, August 2017. One vital benefit of the improved recommendations for ECG analysis is the reduction in costs. For instance, the overall cost of screening using the 2010 ESC Recommendations was $110 per athlete and $35,993 per serious diagnosis, whilst the refined criteria cut those costs to $87 and $28,510, respectively.

Cardiac Risk in the Young

Papers/Articles • “Effect of Sex and Sporting Discipline on LV Adaptation to Exercise.” Finocchiario G, Dhutia H, D’Silva A et al. Journal of the American College of Cardiology, August 2017.

individual’s self-determination and at the same time offers athletes the necessary protection.”

“Highly trained athletes generally show normal LV geometry; however, female athletes participating in dynamic sport often exhibit eccentric hypertrophy. Although concentric remodelling or hypertrophy in male athletes engaged in dynamic sport is relatively common, it is rare in female athletes and may be a marker of disease in a symptomatic athlete.” • “Clinical Characteristics and Circumstances of Sudden Cardiac Death in Hypertrophic Cardiomyopathy.” Finocchiaro G, Papadakis M, Sharma S et al. CardioPulse, August 2017. “Competitive sport is not a prerogative of young men and women and middle-age individuals increasingly engage in professional or amateur sport at a high level. Prospective studies are necessary to clarify if exercise is indeed a risk factor for SCD and the level of exercise that is safe in patients with a diagnosis of HCM.” • “European Association of Preventive Cardiology (EAPC) and European Association of Cardiovascular Imaging (EACVI) Joint Position Statement: Recommendations for the Indication and Interpretation of Cardiovascular Imaging in the Evaluation of the Athlete’s Heart.” Pelliccia A, Caselli S, Sharma S et al. European Heart Journal, September 2017. • “Cardiovascular Causes of Maternal Sudden Death. Sudden Arrhythmic Death Syndrome is Leading Cause in UK.” Krexi D, Sheppard MN. European Journal of Obstetrics, Gynecology, and Reproductive Biology, October 2017. “This study highlights sudden cardiac death in pregnancy or in the postpartum period, which is mainly due to SADS with underlying channelopathies and cardiomyopathy. We wish to raise awareness of these frequently under-recognised entities in maternal deaths and the need of cardiological screening of the family as a result of the diagnosis.” • “Time out: Ethical Reflections on Medical Disqualification of Athletes in the Context of Mandated Pre-participation Cardiac Screening.” Magavern EF, Finocchiaro G, Sharma S et al. British Journal of Sports Medicine. October 2017. “The ultimate goal should be a collaborative decision-making process that allows for individualised exercise prescription, respects an

• “Value of Strain Imaging and Maximal Oxygen Consumption in Patients With Hypertrophic Cardiomyopathy.” Moneghetti KJ, Stolfo D, Christle JW et al. The American Journal of Cardiology, October 2017. • “Management of Mature Athletes with Cardiovascular Conditions.” D’Silva A, Sharma S. Heart, October 2017. • “Cardiac Structure and Function in Elite Native Hawaiian and Pacific Islander Rugby Football League Athletes: An Exploratory Study.” Johnson C, Forsythe L, Somauroo J et al. The International Journal of Cardiovascular Imaging, November 2017. • “The Past, Present and Future Challenges in Epilepsy Related and Sudden Deaths and Biobanking.” Thom M, Boldrini M, Bundock E et al. Neuropathology and Applied Neurobiology, November 2017. • “Unravelling the Mystery Behind Sudden Death in the Young: A Wake Up Call for Nationwide Autopsy-based Approach.” Finocchiaro G, Sharma S, Sheppard MN. Europace, December 2017. • “Impact of Demographic Features, Lifestyle, and Comorbidities on the Clinical Expression of Hypertrophic Cardiomyopathy.” Finocchiaro G, Magavern EF, Sinagra G et al. Journal of the American Heart Association, December 2017. • “The Authors Reply (on Effect of Sex and Sporting Discipline on LV Adaptation to Exercise).” Finocchiaro G, Dhutia H, D’Silva A et al. Journal of the American College of Cardiology Cardiovascular Imaging, December 2017. • “Effects of International Electrocardiographic Interpretation Recommendations on African American Athletes.” Sharma S. Journal of the American Medical Association Cardiology, December 2017. • “Guidelines for Autopsy Investigation of Sudden Cardiac Death: 2017 Update from the Association for European Cardiovascular Pathology.” Basso C, Aguilera B, Banner J et al. Virchows Archiv European Journal of Pathology, December 2017. “Pathologists are responsible for determining the precise cause and mechanism of sudden death but there is still considerable variation in the way in which they approach this increasingly complex task.”

Publications •

“Fever vs. Drug: Battling with the Brugada syndrome substrate.” Behr, ER; Ensam, B. Heart Rhythm, 14 (4).

Presentations at BCS/ESC/EuroPrevent •

“Electrocardiographic differentiation between benign T-wave inversion and arrhythmogenic right ventricular cardiomyopathy.” Finocchiaro G, Papadakis M, Dhutia H, Sinagra G, Sharma S. Abstract session (poster) ESC Congress, 2017.

“Nationwide cardiovascular screening of young individuals: the diagnostic yield and implications on workload.” Dhutia H, Malhotra A, Finocchiaro G, Papadakis M and Sharma S, EuroPrevent, London, April 2017.

“Cardiac symptoms before unexpected sudden cardiac death in the young: Data from a large pathology registry.” Finocchiaro G, Papadakis M, Dhutia H, Sharma S, Sheppard MN. Abstract session (oral presentation) ESC Congress, 2017.

“Contemporary studies of sudden cardiac death in young athletes.” Sheppard MN, EuroPrevent, London, April 2017.

“Cost effective pre-participation screening in athletes: is it feasible?” Dhutia H, EuroPrevent, London, April 2017.

“Emergency response facilities including primary and secondary prevention strategies across professional soccer clubs in England.” Malhotra A, Finocchiaro G, Gati S, Keteepe-Arachi T, Rao P, Sahdev N, Reddin D, Cowie C, Papadakis M and Sharma S, EuroPrevent, London, April 2017.

“Discussion: The significance of myocardial scars and implications for sports participation.” Papadakis M, EuroPrevent, London, April 2017.

“Left Ventricular Morphology in Elite Athletes Characterized by Extreme Anthropometry.” Finocchiaro G, Papadakis M, Dhutia H, Sharma S. Abstract session (poster) BCS Congress, 2017.

“Effect of ethnicity, age, gender and sporting discipline on the athlete’s ECG.” Papadakis M. EuroPrevent, London, April 2017.

“The mixed race athlete’s ECG: Not so black and white.” Malhotra A, Rao P, Dhutia H, Gati S, Keteepe-Arachi T, Finocchiaro G, D’Silva A, Yeo T.J., Papadakis M and Sharma S, EuroPrevent, London, April 2017.

For a full list of CRY’s research visit Cardiac Risk in the Young

Research Highlights 2017


Professor Sanjay Sharma, CRY Consultant Cardiologist The CRY Research Programme is overseen by Professor Sanjay Sharma, Professor of Inherited Cardiovascular Disease and Sports Cardiology at St George’s Hospital, London; Virgin Money London Marathon Medical Director; and was the London 2012 Olympic Cardiologist. The CRY Research Fellowship Programme funds doctors for up to three years who choose to specialise in the fields of inherited cardiac diseases, young sudden cardiac death, screening and sports cardiology. It is our unique expertise in sports cardiology and how athleticism, ethnicity and gender affect the ECG that has made CRY a leading international authority on cardiac screening. “Sports cardiology” is cardiac research and clinical

practice applied to fit and healthy young people. The knowledge gained from studying athletes better informs the diagnosis and management of all young people at risk from cardiac conditions. As of January 2017 there are 9 CRY Research Fellows at St George’s Hospital who divide their time between NHS clinics, CRY screenings and research. As well as the Fellows CRY are currently funding, 19 former Fellows have been trained as specialists by CRY and are now working in the NHS throughout the UK. Professor Sharma oversees the CRY National Screening Programme in which the CRY Research Fellows play a central role. Every person that CRY tests is asked to consent to having their data used anonymously for research purposes. This has developed a symbiotic relationship between research and screening; identifying young individuals at risk whilst learning from our experience and publishing these findings.

CRY’s Research Programme CRY’s research improves our understanding of the incidence, prevention, assessment and management of cardiac conditions that can cause young sudden cardiac death (YSCD). CRY research has shown: • Every week in the UK at least 12 young people die of undiagnosed heart conditions (Papadakis M et al. 2009) • 1 in 300 young people that CRY tests will have a potentially life-threatening heart condition (Wilson MG et al. 2008) • 80% of SADS deaths occur at rest or during sleep (Mellor G et al. 2014) • ARVC is the cardiac condition most likely to cause sudden death during exercise (Finocchiaro et al. 2016)

• Prevalence of cardiac conditions in young people

• Management of those at increased risk of YSCD

• Incidence of YSCD

• Exercise prescription

• Causes of YSCD

• Psychological adjustment

• Circumstances of YSCD

• Identification of those at increased risk of YSCD

• Risk stratification of those identified

• Screening

• Monitoring those identified

• Symptoms • Family history

17% of CRY’s Funding Supports Research Fundraising 16% Governance 4%

Awareness 17% Support 8%

Screening 38%

Research 17%

• The Jack Boulton Memorial Fund • The Nathan Butler Memorial Fund • The Matthew Cragg Memorial Fund • The Jeremy Cole Memorial Fund


Research Highlights 2017

CRY research grants fund fast-track, expert referral services at the CRY Centre for Cardiac Pathology (CRY CCP) and the CRY Centre for Inherited Cardiovascular Conditions and Sports Cardiology CRY research grants fund original research using data gathered from the CRY Centres and through the CRY National Screening Programme CRY research grants fund clinical doctors who are present at every CRY screening event to examine the results of young people tested and provide a consultation on the same day

In 2017 the following ringfenced funds made significant contributions to support CRY’s research team at St George’s Hospital: • The Neil Desai Memorial Fund • The Chris Haw Memorial Fund • The Joe Kellogg Memorial Fund • The Jannik Lam Memorial Fund

• The Chad Loveday Memorial Fund • The Alan Lumley Memorial Fund • The Rosie Mitchell Memorial Fund • The Jason Nixon Memorial Fund • The Oliver Thompson Memorial Fund

Cardiac Risk in the Young