European Heart Journal

Remme, Carol Ann; Leclercq, Christophe; Behr, Elijah R

doi: 10.1093/eurheartj/ehab698pmid: 34617990

Cardiac arrhythmia genetics—need for a multi-disciplinary network in Europe Sudden cardiac death affects approximately three in every 100 000 European children and adults under the age of 35 every year. While it is uncommon, the impact on families and society is disproportionately greater than most sudden deaths occurring in older people. Most deaths are unheralded by prior symptoms or a family history and yet a majority are due to cardiac genetic disorders, mainly inherited arrhythmia syndromes such as long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, and cardiomyopathies (Figure 1).1 There is a clear need to identify patients and their families to mitigate their risk and prevent sudden death. The mission of the recently founded European Cardiac Arrhythmia Genetics (ECGen) focus group of the European Heart Rhythm Association (EHRA) is to address this need by improving education, training, patient guidelines and research in the field of inherited arrhythmia syndromes, and genetic aspects of arrhythmia in Europe and further afield.2 The foundation for this focus group originated from the European Reference Network (ERN) for rare cardiac disease, GUARD-Heart, which was established to provide equality in patient care across Europe, facilitate education and training, and establish a collaborative network for governance and research. However, since the mission of GUARD-Heart extends well beyond the current and future coverage of the ERN, a need was recognized for a pan-European representative group of electrophysiology professionals and their colleagues working in the field of cardiac genetics as applied to arrhythmia syndromes, arrhythmias, and sudden death. Figure 1 Open in new tabDownload slide Causes of sudden cardiac death in the young (age 0–40 years) based on post-mortem findings. ARVC, arrhythmogenic right ventricular cardiomyopathy; CPVT, catecholaminergic polymorphic ventricular tachycardia; HCM, hypertrophic cardiomyopathy; LQTS, long QT syndrome; SADS, sudden arrhythmic death syndrome. Figure 1 Open in new tabDownload slide Causes of sudden cardiac death in the young (age 0–40 years) based on post-mortem findings. ARVC, arrhythmogenic right ventricular cardiomyopathy; CPVT, catecholaminergic polymorphic ventricular tachycardia; HCM, hypertrophic cardiomyopathy; LQTS, long QT syndrome; SADS, sudden arrhythmic death syndrome. ECGen is a multidisciplinary group and will therefore ensure collaboration with existing stakeholders, including (i) the ESC Working Group for Myocardial and Pericardial Diseases (WGMPD), which addresses guidelines, education, training, and research in the field of cardiomyopathy and its genetic basis, (ii) the Association for European Cardiovascular Pathology (AECVP), which focuses on pathological and diagnostic guidelines and training in the investigation of sudden death including the role for genetic studies, (iii) the European Society of Human Genetics (ESHG), which similarly has a broad interest in sudden death and cardiac genetics with a strong educational programme, (iv) the newly founded ESC Council for Cardiovascular Genomics (CCG) whose mission is to encourage research, education, and the sharing of genomic knowledge, (v) the ESC Working Group on Cardiac Cellular Electrophysiology (WGCCE), providing a direct link with basic and translational arrhythmia research, (vi) the arrhythmia and genetic working groups of the European Association of Preventive Cardiology (EAPC), and (vii) the Association of Cardiovascular Nursing & Allied Professions (ACNAP). The objectives of ECGen The objectives of the multi-disciplinary ECGen focus group, as depicted in Figure 2, are to: 1. Develop and harmonize patient pathways and guidelines for clinical cardiology and genetic management of patients with arrhythmia syndromes and arrhythmic genetic disorders on behalf of EHRA and in collaboration with other European and international stakeholders, including the European Reference Network GUARD-Heart. 2. Provide a framework for delivering education and training in the genetic arrhythmia field to European physicians and allied professionals. 3. Provide a platform for interaction with national and European patient interest groups. 4. Promote collaboration of members to collect data for research as well as interact with other stakeholders where objectives are shared. 5. Collaborate with WGMPD, AECVP, ESHG, CCG, WGCCE, EAPC, and ACNAP. Figure 2 Open in new tabDownload slide Overview of the objectives of ECGen. Figure 2 Open in new tabDownload slide Overview of the objectives of ECGen. ECGen committee composition and membership During the first year, an Initial Committee was formed by members of the ERN GUARD-Heart and EHRA, which established the process for electing the first Elected Focus Group as well as procedures and membership rules. The ECGen Elected Committee is multi-disciplinary and consists of (i) a Chairperson, Vice-Chairperson (deputizes for Chairperson as required), and Past-Chairperson, who will all be consultant physicians, and (ii) members (at least 9), including five consultant physicians of whom one must be a paediatric consultant physician, one trainee physician, one genetic nurse, one genetic counsellor, and one scientist.2 The ECGen Chairperson is a member of the EHRA Board as well as the ESC Council for Cardiovascular Genomics. ECGen members may be appointed by the EHRA President and the chairpersons of various EHRA committees to contribute to the association activities (Scientific Documents Committee, Education Committee, Congress Scientific Program Committee, Scientific Initiatives Committee). ECGen currently has 86 ordinary members; these require status as either a trainee or fully qualified adult or paediatric cardiologist, geneticist, pathologist, scientist, registered nurse, or genetic counsellor with an interest in the field of cardiac arrhythmia genetics. Membership application forms can be filled in online at My ESC account under ‘New application’ and will be reviewed monthly. As an integral part of EHRA, all ECGen members must be members of the association. Ongoing and planned activities of ECGen Prior to the first ECGen election, the focus group initial committee had already embarked on several initiatives. The Scientific Documents Committee in collaboration with ECGen successfully proposed an Expert Consensus Statement on the State of Genetic Testing for Cardiac Disease led by EHRA, in collaboration with the Heart Rhythm Society, Asia Pacific Heart Rhythm Society, and the Latin America Heart Rhythm Society, due to report in 2022. ECGen members also represented EHRA on the 2020 APHRS/HRS expert consensus statement on the investigation of decedents with sudden unexplained death and patients with sudden cardiac arrest, and of their families.3 Furthermore, the initial ECGen committee proposed to the Scientific Initiatives Committee to produce a survey on the provision of services and screening for patients and their families with Inherited Arrhythmia Syndromes, which identified significant heterogeneity in patient management across European centres and underscored the need for dedicated units and specialist services.4,5 As a first initiative of the full elected committee, a survey of ECGen members was held in October 2020 to gain insight into the areas of priority. The three preferred areas of activities identified included development of (i) recommendations and/or position statements, (ii) European and international research networks, and (iii) educational seminars, symposia, and webinars (for results, see Figure 3). Following this survey, several activities have been initiated to address the identified needs. In collaboration with other stakeholders, joint educational session proposals have been submitted for EHRA and ESC Congress, and a number of webinars have been organized, including the recent one on ‘Primary electrical disorders: between ECG and genes’ with Drs. Eric Schulze-Bahr, Michael Gollob, and Michael Ackerman, and another planned for January 2022 titled ‘Cardiovascular genetics in practice: What to know for today and tomorrow’. In addition, the EHRA Scientific Initiatives Committee developed a second survey based on a proposal from ECGen, this time on Sudden Death in the Young (SUDY). This survey was prepared and distributed among EHRA Research Network and ERN members and was aimed at gaining an understanding of (i) the provision across Europe of services for families of decedents, the current heterogeneity of autopsy practice and post-mortem genetic studies, and the availability of family service referrals and psychological support and (ii) the variation of family investigation protocols and the role for provocation studies. Results from this survey indicated a significant heterogeneity of service provision and variable adherence to guidelines and highlighted the need for the improvement and expansion of existing specialist structures and access to autopsy.6 Figure 3 Open in new tabDownload slide Results from the ECGen member survey (n = 50 respondents) with the three preferred areas of activities indicated in pink. Figure 3 Open in new tabDownload slide Results from the ECGen member survey (n = 50 respondents) with the three preferred areas of activities indicated in pink. The membership has also been encouraged to participate in research networks, for example a recent call was made to join the International Registry on Atrial Arrhythmias and Channelopathies. Furthermore, opportunities to join and collaborate with multiple research registries led by Guard-HEART will shortly be extended to the ECGen membership. Engagement with ACNAP has been initiated with the aim of promoting and recognizing the role of the cardiac genetic specialist nurse in Europe, particularly in the management of SUDY families. Similarly, collaboration with the Young EP committee has begun by exploring opportunities for observer ships, mentorships, and fellowships in the post-COVID era involving EP centres offering specialist cardiac genetics clinics led by ECGen members. Finally, proposals for scientific documents are being prepared on drug repurposing and personalized therapy in primary electrical disease and the management of arrhythmia syndromes during pregnancy. Conclusion ECGen will continue to forge opportunities for education and training, patient management, and research in the field of cardiac arrhythmia genetics, by encouraging a multidisciplinary membership and collaboration across, ESC and Europe. We welcome all EHRA members with an interest in inherited arrhythmogenic diseases to join ECGen. Conflict of interest: none declared. References 1 Semsarian C , Ingles J, Wilde AA. Sudden cardiac death in the young: the molecular autopsy and a practical approach to surviving relatives . Eur Heart J 2015 ; 36 : 1290 – 1296 . Google Scholar Crossref Search ADS PubMed WorldCat 2 https://www.escardio.org/Sub-specialty-communities/European-Heart-Rhythm-Association-(EHRA)/About/the-european-cardiac-arrhythmia-genetics-focus-group-ecgen-of-ehra. 3 Stiles MK , Wilde AAM, Abrams DJ, Ackerman MJ, Albert CM, Behr ER, Chugh SS, Cornel MC, Gardner K, Ingles J, James CA, Jimmy Juang JM, Kääb S, Kaufman ES, Krahn AD, Lubitz SA, MacLeod H, Morillo CA, Nademanee K, Probst V, Saarel EV, Sacilotto L, Semsarian C, Sheppard MN, Shimizu W, Skinner JR, Tfelt-Hansen J, Wang DW. 2020 APHRS/HRS expert consensus statement on the investigation of decedents with sudden unexplained death and patients with sudden cardiac arrest, and of their families . Heart Rhythm 2021 ; 18 : e1 – e50 . Google Scholar Crossref Search ADS PubMed WorldCat 4 Conte G , Scherr D, Lenarczyk R, Gandjbachkh E, Boulé S, Spartalis MD, Behr ER, Wilde A, Potpara T. Diagnosis, family screening, and treatment of inherited arrhythmogenic diseases in Europe: results of the European Heart Rhythm Association Survey . Europace 2020 ; 22 : 1904 – 1910 . Google Scholar Crossref Search ADS PubMed WorldCat 5 Conte G , Wilde A, Behr ER, Scherr D, Lenarczyk R, Gandjbachkh E, Crotti L, Brugada-Sarquella G, Potpara T. Importance of dedicated units for the management of patients with inherited arrhythmia syndromes . Circ Genom Precis Med 2021 ; 14 : e003313 . Google Scholar Crossref Search ADS PubMed WorldCat 6 Behr ER , Scrocco C, Wilde AAM, Marijon E, Crotti L, Iliodromitis KE, Remme CA, Kosiuk J, Rudaka I, Brugada GS, Frampton K, Schulze-Bahr E, Jubele K, de Asmundis C, Hofman N, Tfelt-Hansen J, Boveda S, Conte G. Investigation on Sudden Unexpected Death in the Young (SUDY) in Europe: results of the European Heart Rhythm Association Survey . Europace 2021 . doi: 10.1093/europace/euab176 . Google Scholar OpenURL Placeholder Text WorldCat Crossref Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: [email protected]. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)

Carbone, Federico

doi: 10.1093/eurheartj/ehab713pmid: 34618010

Since the concept of metabolic syndrome (MetS) was firstly formalized by Professor Reaven in 1988, insulin resistance was identified as the underlying common factor driving the associated cardiovascular (CV) risk. Ten years later the World Health Organization attempted to provide a comprehensive definition in order to identify and treat these high-risk individuals.1 Some argued that a mere factional clash would then drive the evolution of this definition, swinging between the will to promote or dilute the role of insulin resistance. On the contrary, such debate led to a critical revision of MetS paradigm and paved the way for the current knowledge on CardioMetabolic risk.2 Nevertheless, some shortcomings still undermine the effective consensus around this definition, so that even Reaven himself has come to wonder if MetS diagnosis is really necessary.3 1. European Society of Cardiology/American Heart Association (ESC/AHA) guideline for primary prevention of CV disease do not encompass MetS: ESC SCORE risk charts did not consider MetS in CV risk estimation and even the latest released guidelines do not include body mass index (BMI) and/or waist circumference as CV risk modifiers. AHA generally acknowledges that pooled cohort equations may underestimate CV risk in MetS—then considered as risk-enhancing factor—and claims to revise the 10-year CV risk estimation in MetS patients at borderline/intermediate risk. Nevertheless, although the sum (MetS as a whole) should be considered clinically relevant, for any risk factors treatment as per clinical guidelines is still recommended. 2. The mismatch between MetS and obesity: during the first decade of 20th century the awareness of excess body weight/obesity burden significantly grew, but far from the concept of MetS. The concept of ‘obesity paradox’ was born, keeping scientists hostage for a decade further. 3. The rationale behind MetS definition is unclear: the foresight in MetS consensus definition was to identify high waist circumference—rather than BMI—as adiposity index and essential criterion for MetS definition. However, the role of other MetS determinants is currently challenged and even the concept of syndrome might be itself questioned. The sick fat, also referred to as ‘adiposopathy’, may be now considered the paradigm of global CardioMetabolic risk. The sine qua non of adiposopathy is visceral fat deposition associated with inflammatory/adipokine dysregulation and ectopic fat deposition.4 This paradigm shift may allow relevant steps forward and deserves to be deeply discussed. First, the focus on visceral adipose tissue may provide a key to understand the obesity paradox, highlighting the inherent limits of BMI use in clinical practice. As second, the emerging role of inflammation—whose consecutio with visceral adipose tissue dysfunction has yet to be clarified—seems to follow what observed at the beginning of the 2000s, when the shift from vulnerable plaque to vulnerable patient occurred. Furthermore, far from being a single entity, obesity encompasses a heterogeneous group of phenotypes: classical, metabolically healthy, normal weight, and osteosarcopenic obesities.5 Considering inflammatory dysregulation as the underlying mechanism of these different phenotypes is an intriguing hypothesis, a potential ‘theory of everything’, that deserves further investigations.6 Investigating ectopic fat deposition finally allows at appreciating the full pathogenic potential of dysfunctional adipose tissue, giving an integrated perspective and recognizing the importance of the cross-talk between adipose tissue and other body systems.7 The worldwide obesity pandemic claims for urgent interventions at several levels. The failure of BMI flags the need for implementing anthropometric indexes of central obesity. With the contribution of artificial intelligence, advances in body fat imaging will be greatly contributing to validate the concept of adiposopathy and some of these might be soon applied for clinical use (e.g. dual-energy X-ray absorptiometry and rapid MRI scan).8 The establishment of a working group on visceral obesity should then become a priority for cardiology societies. To date International Atherosclerosis Society and International Chair on CardioMetabolic Risk are the only ones to have produced a consensus statement claiming for waist circumference assessment as a vital sign in clinical practice.9 AHA has already published a scientific statement on obesity and CV disease,10 but including visceral adiposity assessment in the next guideline update appears now mandatory. Alongside pathophysiological and diagnostic considerations, there are indeed many therapeutic implications ranging from lifestyle modifications, pharmacotherapy and even surgical interventions. Concerning the latter, results are so impressive that such approach is increasingly known as metabolic rather than bariatric surgery. After 20 years, a lot of progresses and some missteps, it is now time to consider CardioMetabolic medicine as a subspecialty for which clinicians from different backgrounds should be trained (Figure 1). Scientific societies are therefore called upon to provide the tools to tackle this silent pandemic. Conflict of interest: none to be declared. Figure 1 Open in new tabDownload slide Step forward in CardioMetabolic Medicine during 20th century. Since the end of 19th century clustering of CardioMetabolic risk factors has become a matter of fact. However, the kind of and the extent to which they contribute to the overall CardioMetabolic risk is still matter of debate. This path would retrace what occurred in the study of atherosclerosis where inflammation has been finally recognized as leading determinant in atherosclerosis pathophysiology. Despite some missteps also obesity is now increasingly recognized as systemic disease with different phenotypes in which inflammation would exert a critical pathophysiological role. Figure 1 Open in new tabDownload slide Step forward in CardioMetabolic Medicine during 20th century. Since the end of 19th century clustering of CardioMetabolic risk factors has become a matter of fact. However, the kind of and the extent to which they contribute to the overall CardioMetabolic risk is still matter of debate. This path would retrace what occurred in the study of atherosclerosis where inflammation has been finally recognized as leading determinant in atherosclerosis pathophysiology. Despite some missteps also obesity is now increasingly recognized as systemic disease with different phenotypes in which inflammation would exert a critical pathophysiological role. References 1 Alberti KG , Zimmet PZ; WHO Consultation . Definition, diagnosis and classification of diabetes mellitus and its complications. Part 1: diagnosis and classification of diabetes mellitus provisional report of a WHO consultation . Diabet Med 1998 ; 15 : 539 – 553 . Google Scholar Crossref Search ADS PubMed WorldCat 2 Eckel RH , Kahn R, Robertson RM, Rizza RA. Preventing cardiovascular disease and diabetes: a call to action from the American Diabetes Association and the American Heart Association . Diabetes Care 2006 ; 29 : 1697 – 1699 . Google Scholar Crossref Search ADS PubMed WorldCat 3 Reaven GM. The metabolic syndrome: is this diagnosis necessary? Am J Clin Nutr 2006 ; 83 : 1237 – 1247 . Google Scholar Crossref Search ADS PubMed WorldCat 4 Neeland IJ , Poirier P, Despres JP. Cardiovascular and metabolic heterogeneity of obesity: clinical challenges and implications for management . Circulation 2018 ; 137 : 1391 – 1406 . Google Scholar Crossref Search ADS PubMed WorldCat 5 Vecchie A , Dallegri F, Carbone F et al. Obesity phenotypes and their paradoxical association with cardiovascular diseases . Eur J Intern Med 2018 ; 48 : 6 – 17 . Google Scholar Crossref Search ADS PubMed WorldCat 6 Carbone FN , Migliola E, Bonaventura A et al. Circulating levels of sclerostin predict glycemic improvement after sleeve gastrectomy . Nutrients 2021 ; 13 : 623 . Google Scholar Crossref Search ADS PubMed WorldCat 7 Ferrara D , Montecucco F, Dallegri F, Carbone F. Impact of different ectopic fat depots on cardiovascular and metabolic diseases . J Cell Physiol 2019 ; 234 : 21630 – 21641 . Google Scholar Crossref Search ADS PubMed WorldCat 8 Zhang L , Sun J, Jiang B, Wang L, Zhang Y, Xie X. Development of artificial intelligence in epicardial and pericoronary adipose tissue imaging: a systematic review . Eur J Hybrid Imaging 2021 ; 5 : 14 . Google Scholar Crossref Search ADS PubMed WorldCat 9 Ross R , Neeland IJ, Yamashita S et al. Waist circumference as a vital sign in clinical practice: a Consensus Statement from the IAS and ICCR Working Group on Visceral Obesity . Nat Rev Endocrinol 2020 ; 16 : 177 – 189 . Google Scholar Crossref Search ADS PubMed WorldCat 10 Powell-Wiley TM , Poirier P, Burke LE, Despres JP et al. ; American Heart Association Council on Lifestyle and Cardiometabolic Health; Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; Council on Epidemiology and Prevention; and Stroke Council . Obesity and cardiovascular disease: a scientific statement from the American Heart Association . Circulation 2021 ; 143 : e984 – e1010 . Google Scholar Crossref Search ADS PubMed WorldCat Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: [email protected]. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)

Gatzoulis, Michael A; Grocott-Mason, Richard

doi: 10.1093/eurheartj/ehab817pmid: 34922355

Times have changed. The English language and technology have made communication easier and the world a smaller place. Yet disease burden is rampant—and global—and expectations of a long and good life are high, as should be. Healthcare systems do their best to provide care, but with new therapies and the ability to treat higher risk patients, all systems are finding resources insufficient to meet their population needs. Unless something else comes into play—and in good time—we will continue to struggle. Promoting health rather than treating disease has to be the way forward. This requires patient education,1 empowerment, and support. In addition, policymakers need to develop novel ways to encourage and support healthy life choices for the whole population. In this article, we focus on congenital heart disease (CHD); however, the principles apply universally. CHD is the most common inborn and global defect. Advances in diagnosis and treatment have revolutionized the prospects for these patients in the developed world;2 there is a lot of catching up to do elsewhere. In the developed world, most patients now survive to adulthood, even those with complex disease.3 But with longevity comes increasing healthcare needs, requiring CHD, cardiovascular, and other medical expertise. The reality for many patients is a heavy history of previous interventions, ongoing residual haemodynamic and other lesions, which is challenging for the profession, compromising early success and attainment of full potential.4 If we take bacterial endocarditis, a life-threatening complication of CHD5 for example, patients should be aware of the signs and symptoms of the disease—which are non-specific—and resist antibiotics ‘over the counter’, unless blood cultures are drawn. More importantly, patients with CHD should know that the usual source of bacteria entry is the mouth and skin. Obviously, the best treatment for any condition is prevention and so patients should know how to minimize additional risks for bacterial endocarditis, i.e. healthy diet, avoid smoking and optimal gum/dental hygiene with good skin care and timely treatment of any skin disease, such as paronychia. Have we done a good job to communicate all this to our patients? Multiple committees and guidelines refer to antibiotic prophylaxis, when to consider it or not, for preventing bacterial endocarditis in patient groups at risk. This has a very limited effect, even when adhered to, however. We submit that a better defence against bacterial endocarditis, and for that matter for any infection, is to follow the new aspirational mantra of “being slim and athletic”. A healthy lifestyle including a good diet, daily physical activity etc, boosts the immune system, minimizes chronic gingival disease, and thus exposure to increased endocarditis risk and improves quality of life. We spend time with our patients discussing diet/cooking and the merits of optimal weight and exercise. We must learn how to cook and make time for it, in our busy lives. To do so is to invest in good health, eat whole foods in season, be sociable and entertaining, support the local economy—and it may also help us to save the planet. And we must learn this early in life, as toddlers at home and young pupils at school. And match it with daily exercise, the best medicine. What is the basis on which we restricted physical activity to our patients, a thing of the past? Why is it participation in sports at schools on a decline, and why are some cities, like London, not fully pedestrianized with cycling lanes everywhere, promoting physical activity and discovery of place, architecture and history? It is never too late. We must, in parallel promote oral health,6 introduce diets low in sugar and alcohol, avoid tobacco use, improve access to dental care and thus, reduce overlap with other non-communicable diseases. It is Education, Education, Education1 (of the patient, the profession and the public) that is the best gift of all, and this is where we must invest (Figure 1). We must do a better job to reassure the patients and their families with CHD, often diagnosed in utero, that there are excellent prospects and a life-long infrastructure to support their journey and provide them with a compass on how to maximize their potential, what to do in an emergency and how to plan their lives. Patients with a ‘single ventricle physiology’, the most complex form of CHD—and their families—should know that the best investment they can make after their Fontan operation (connecting the systemic venous return directly to the lungs in early childhood), is to be ‘slim and athletic’ and if female, and wishing to have a child that it would be best to do so in early adulthood and under the right supervision/multi-disciplinary support. This is all achievable and within reach, does not require a lot of resource but does require a different approach from the profession, the health care policy makers and the patients alike. We should develop new ‘low cost’ technologies8 to support patients to lead healthy and longer lives. Figure 1 Open in new tabDownload slide Education, engagement and empowerment of families/patients with Congenital Heart Disease from the time of diagnosis and throughout life. (Modified from reference 7). Figure 1 Open in new tabDownload slide Education, engagement and empowerment of families/patients with Congenital Heart Disease from the time of diagnosis and throughout life. (Modified from reference 7). Patients with CHD are a highly suited cohort to apply this necessary next step in global health with patient education, engagement and empowerment at its core: they are relatively young, motivated, technologically savvy, have a life-long disease and are thus interested in assuming this joint responsibility. Clinicians, policymakers and teachers should act together to educate, and encourage and support all patients to make healthy life choices. In particular, the efforts should be most intensive for those at highest risk in society. The link between health outcomes and social deprivation are very strong, and multifactorial, hence a co-ordinated and concerted effort is clearly required. The Covid vaccination story has demonstrated what can be achieved with focus and investment. However, it has also illustrated the challenges of equity in the world, and the need to overcome some people’s suspicion and distrust of science and authority. There are also similarities in the need to address the greatest future health risk to all—climate change. We should strive to follow and utilize recent advances in science and technology to ensure a rich and fulfilling life. There is no better time than now, and not just for patients—for all of us. Conflict of interest: none declared. References 1 Gatzoulis MA. Adult congenital heart disease: education, education, education . Nat Clin Pract Cardiovasc Med 2006 ; 3 : 2 – 3 . Google Scholar Crossref Search ADS PubMed WorldCat 2 Brida M , Gatzoulis MA. Adult congenital heart disease: past, present and future . Int J Cardiol Congenit Heart Disease 2020 ; 1 : 100052 . Google Scholar Crossref Search ADS WorldCat 3 Diller GP , Kempny A, Alonso-Gonzalez R et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre . Circulation 2015 ; 132 : 2118 – 2125 . Google Scholar Crossref Search ADS PubMed WorldCat 4 Gatzoulis MA , Balaji S, Webber SA et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study . Lancet 2000 ; 356 : 975 – 981 . Google Scholar Crossref Search ADS PubMed WorldCat 5 Tutarel O , Alonso-Gonzalez R, Montanaro C et al. Infective endocarditis in adults with congenital heart disease remains a lethal disease . Heart 2018 ; 104 : 161 – 165 . Google Scholar Crossref Search ADS PubMed WorldCat 6 Baumgartner H , De Backer J, Babu-Narayan SV et al. ; ESC Scientific Document Group . 2020 ESC Guidelines for the management of adult congenital heart disease . Eur Heart J 2021 ; 42 : 563 – 645 . Google Scholar Crossref Search ADS PubMed WorldCat 7 Brida M , Gatzoulis MA. Adult Congenital Heart Disease: The Quest to Address the Simple and the Complex . JACC Case Rep 2021 ; 3 : 353 – 355 . Google Scholar Crossref Search ADS PubMed WorldCat 8 Diller GP , Kempny A, Babu-Narayan SV et al. Machine learning algorithms estimating prognosis and guiding therapy in adult congenital heart disease: data from a single tertiary centre including 10 019 patients . Eur Heart J 2019 ; 40 : 1069 – 1077 . Google Scholar Crossref Search ADS PubMed WorldCat Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: [email protected]. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)

Zhou, Zeming; Gu, Yuanrui; Zheng, Hong

doi: 10.1093/eurheartj/ehab877pmid: 34972211

A 50-year-old woman was presented with chest tightness and palpitations. The transthoracic echocardiogram (TTE) showed an unroofed coronary sinus (URCS) syndrome (Panel A and B) with the larger right ventricle (RV) and moderate tricuspid regurgitation (TR). The preoperative multi-slice computed tomography (MSCT) revealed the expanding coronary sinus (CS) with a 22 mm defect to the left atrium (LA) and 20 mm to the coronary sinus ostium (CSO) (Panel C and D). The type of URCS was Ib without left superior vena cava, and several minor coronary veins were returned to the LA. The pulmonary to systemic blood flow ratio was 2.2:1. Open in new tabDownload slide Open in new tabDownload slide During the operation, a 26 mm atrial septal defect occluder was implanted to close the CSO that the coronary vein flow was returned to the LA. However, the device had a large deformation, and the mitral valve was affected by the left disc (Panel E and G). Thus, we replaced a 26/24 mm patent ductus arteriosus (PDA) occluder (Starway Medical Tec., Beijing). The shape was satisfied without affecting the mitral valve Panel F and H). Femoral artery blood oxygenation was similar between before and after surgery (96.7 vs. 96.2%). During the 3-month follow-up, TTE showed a normal RV with a mild TR, and the result of MSCT was satisfied (Panel I and J). The symptoms were disappeared without any supraventricular arrhythmias until now. To our best knowledge, this is the first report of transcatheter closure for Ib-type URCS using a PDA occluder with satisfying results in the short term. This work was supported by National Key Research and Development Program of China [grant number 2018YFB1107100]. © The Author(s) 2021. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: [email protected] This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)