AI-enabled ECGs have previously been shown to accurately predict patient sex in adults and correlate with sex hormone levels. We aimed to test the ability of AI-enabled ECGs to predict sex in the pediatric population and study the influence of pubertal development. AI-enabled ECG models were created using a convolutional neural network trained on pediatric 10-second, 12-lead ECGs. The first model was trained de novo using pediatric data. The second model used transfer learning from a previously validated adult data-derived algorithm. We analyzed the first ECG from 90,133 unique pediatric patients (aged ≤18 years) recorded between 1987–2022, and divided the cohort into training, validation, and testing datasets. Subgroup analysis was performed on prepubertal (0–7 years), peripubertal (8–14 years), and postpubertal (15–18 years) patients. The cohort was 46.7% male, with 21,678 prepubertal, 26,740 peripubertal, and 41,715 postpubertal children. The de novo pediatric model demonstrated 81% accuracy and an area under the curve (AUC) of 0.91. Model sensitivity was 0.79, specificity was 0.83, positive predicted value was 0.84, and the negative predicted value was 0.78, for the entire test cohort. The model’s discriminatory ability was highest in postpubertal (AUC = 0.98), lower in the peripubertal age group (AUC = 0.91), and poor in the prepubertal age group (AUC = 0.67). There was no significant performance difference observed between the transfer learning and de novo models. AI-enabled interpretation of ECG can estimate sex in peripubertal and postpubertal children with high accuracy.
The primary focus of my year as president of the American Society of Echocardiography (ASE) is our membership. Integral to this vision is the education of our members. Education has been and continues to be a major priority for ASE, and our Society has a wide range of diverse educational activities that I would like to highlight.
One of the highlights of being President of our Society is the opportunity to interact with our ASE staff on a daily basis. Dr. Alan Pearlman, ASE Past President and ASE Historian, in the recent issue of Echo Magazine1, very elegantly detailed the history of our ASE staff development in the early years of our Society. As a corollary to this, I would like to introduce and highlight our amazing current ASE staff with hopes of educating our members on the tremendous support that they provide for us as a Society.
Background Early detection of left and right ventricular systolic dysfunction (LVSD and RVSD respectively) in children can lead to intervention to reduce morbidity and death. Existing artificial intelligence algorithms can identify LVSD and RVSD in adults using a 12‐lead ECG; however, its efficacy in children is uncertain. We aimed to develop novel artificial intelligence–enabled ECG algorithms for LVSD and RVSD detection in pediatric patients. Methods and Results We identified 10 142 unique pediatric patients (age≤18) with a 10‐second, 12‐lead surface ECG within 14 days of a transthoracic echocardiogram, performed between 2002 and 2022. LVSD was defined quantitatively by left ventricular ejection fraction (LVEF). RVSD was defined semiquantitatively. Novel pediatric models for LVEF ≤35% and LVEF <50% achieved excellent test areas under the curve of 0.93 (95% CI, 0.89–0.98) and 0.88 (95% CI, 0.83–0.94) respectively. The model to detect LVEF <50% had a sensitivity of 0.85, specificity of 0.80, positive predictive value of 0.095, and negative predictive value of 0.995. In comparison, the previously validated adult data‐derived model for LVEF <35% achieved an area under the curve of 0.87 (95% CI, 0.84–0.90) for LVEF ≤35% in children. A novel pediatric model for any RVSD detection reached a test area under the curve of 0.90 (0.87–0.94). Conclusions An artificial intelligence–enabled ECG demonstrates accurate detection of both LVSD and RVSD in pediatric patients. While adult‐trained models offer good performance, improvements are seen when training pediatric‐specific models.
It has been my distinct honor and privilege to participate in editorial roles for all three of ASE's flagship journals. I find it remarkable how each of these journals can produce monthly content that is vital to our Society's mission and core values. I want to personally express my gratitude to each of these editors and their dedicated editorial teams and have asked them to update our membership on each of their journal's activities and highlights over the past year.
This past month, the International Congenital Heart Defects Awareness Day was celebrated on February 14th. This day is meant to recognize and highlight children and adults living with congenital heart disease, celebrate their journey, and hopes to increase public awareness of these heart conditions. I wanted to take this opportunity to address the ways ASE has been providing education and awareness in this important population over the past year.
Introduction: Biologic sex and hormonal concentrations shape ECG parameters due to sex hormone effects on cardiac function. AI-enabled ECGs have previously been shown to accurately predict patient sex in adults and correlate with sex hormone levels. Aims: We aimed to test the ability of AI-enabled ECGs to predict pediatric patient sex and explore the influence of pubertal development. Methods: Two AI-enabled ECG models were created using a convolutional neural network trained on pediatric 10-second, 12-lead surface ECGs. The first model was trained de novo using pediatric data. The second model used transfer learning from a previously validated adult data-derived algorithm. We analyzed the first ECG from 89,063 pediatric patients (aged <19 years) recorded from 1988-2022, and divided the cohort into training, validation, and testing datasets. Subgroup analysis was performed on prepubertal (0-7 years), pubertal (8-14 years), and post-pubertal (15-18 years) patients. Results: The cohort consisted of 46.6% males, with 21,434 prepubertal, 26,300 pubertal, and 41,329 post-pubertal children. No significant performance difference was observed between the two models. Both models demonstrated 81% accuracy and an overall AUC of 0.91 in the testing cohorts, with high discriminatory ability in post-pubertal teenagers (AUC = 0.98). (Figure 1) Conclusions: AI-ECG predicts pediatric patient sex with varying performance across pubertal stages. The highest discriminatory ability is observed post-puberty, decreasing in pubertal and prepubertal children. The results suggest hormonal and physiological changes during puberty may influence cardiac electrophysiology measurably. More research is required to interpret CNN-predicted sex discordance with actual sex and its implications for future cardiovascular risk.
Background: Atrial septal defects (ASD) and ventricular septal defects (VSD) are among the most common forms of congenital heart disease (CHD). Early detection and intervention for these defects are crucial to prevent complications. Given advancements in AI technology, the development of AI-enabled ECG models for detecting these defects is of significant interest. Aims: This study aimed to create AI models using pediatric ECG data for the detection of ASD, VSD, and a combined model detecting either ASD or VSD. Methods: We analyzed a dataset of 7,795 pediatric patients from Mayo Clinic who had undergone echocardiography between 2002 and 2022, with corresponding 10-second, 12-lead surface ECGs taken within 14 days of echocardiography. This dataset included 259 patients with ASDs and 139 patients with VSDs (single or multiple). The control group was comprised of 7,397 patients without significant or complex CHD. For cases and controls, we included patients with all forms of electrocardiographic abnormalities and those with valvular lesions who had less than moderate stenosis or regurgitation. The dataset was divided into training, validation, and testing subsets to develop convolutional neural network (CNN) models for ASD, VSD, and either ASD or VSD detection. Results: Our ASD and VSD detection models achieved test AUCs of 0.82 (95% CI 0.76, 0.88) and 0.80 (95% CI 0.70, 0.90), respectively. Meanwhile, a combined model that detected either ASD or VSD demonstrated a test AUC of 0.81 (95% CI, 0.76, 0.86), sensitivity of 0.73, specificity of 0.76, PPV of 0.14, and NPV of 0.98 (Figure 1). Conclusions: AI-enabled ECG models demonstrate promising performance in detecting ASD and VSD in pediatric patients. A combined model that detects either ASD or VSD also shows robust results. Future research should focus on multi-center validation of these models, refining their diagnostic capacities, and investigating their potential for early intervention in CHDs.
In this month's President's Message, I would like to continue my theme of highlighting ASE's core values. I believe that our Society's commitment to scholarly activities and cutting-edge research truly embodies our core values of excellence and advancing knowledge. These research efforts are pivotal to our membership and to our strategic vision to be the leader in all facets of cardiovascular ultrasound. I have asked Daniel Forsha, co-chair of our Research Committee, to update our members regarding our research mission at ASE and how it has impacted his and many others' academic careers.
Importance Whether vigorous intensity exercise is associated with an increase in risk of ventricular arrhythmias in individuals with hypertrophic cardiomyopathy (HCM) is unknown.Objective To determine whether engagement in vigorous exercise is associated with increased risk for ventricular arrhythmias and/or mortality in individuals with HCM. The a priori hypothesis was that participants engaging in vigorous activity were not more likely to have an arrhythmic event or die than those who reported nonvigorous activity.Design, Setting, and Participants This was an investigator-initiated, prospective cohort study. Participants were enrolled from May 18, 2015, to April 25, 2019, with completion in February 28, 2022. Participants were categorized according to self-reported levels of physical activity: sedentary, moderate, or vigorous-intensity exercise. This was a multicenter, observational registry with recruitment at 42 high-volume HCM centers in the US and internationally; patients could also self-enroll through the central site. Individuals aged 8 to 60 years diagnosed with HCM or genotype positive without left ventricular hypertrophy (phenotype negative) without conditions precluding exercise were enrolled.Exposures Amount and intensity of physical activity.Main Outcomes and Measures The primary prespecified composite end point included death, resuscitated sudden cardiac arrest, arrhythmic syncope, and appropriate shock from an implantable cardioverter defibrillator. All outcome events were adjudicated by an events committee blinded to the patient's exercise category.Results Among the 1660 total participants (mean [SD] age, 39 [15] years; 996 male [60%]), 252 (15%) were classified as sedentary, and 709 (43%) participated in moderate exercise. Among the 699 individuals (42%) who participated in vigorous-intensity exercise, 259 (37%) participated competitively. A total of 77 individuals (4.6%) reached the composite end point. These individuals included 44 (4.6%) of those classified as nonvigorous and 33 (4.7%) of those classified as vigorous, with corresponding rates of 15.3 and 15.9 per 1000 person-years, respectively. In multivariate Cox regression analysis of the primary composite end point, individuals engaging in vigorous exercise did not experience a higher rate of events compared with the nonvigorous group with an adjusted hazard ratio of 1.01. The upper 95% 1-sided confidence level was 1.48, which was below the prespecified boundary of 1.5 for noninferiority.Conclusions and Relevance Results of this cohort study suggest that among individuals with HCM or those who are genotype positive/phenotype negative and are treated in experienced centers, those exercising vigorously did not experience a higher rate of death or life-threatening arrhythmias than those exercising moderately or those who were sedentary. These data may inform discussion between the patient and their expert clinician around exercise participation.
As I articulated in my previous JASE President’s Message, our core values are the foundation of our Society. Last month, I highlighted diversity as one of ASE’s important core values. This month, I would like to highlight another of our core values – ASE Cares. This core value was originally championed during the ASE presidency of Madhav Swaminathan, MD, MMCi, FASE, and I have asked him to give us an update of this core value in action.“People do not care how much you know until they know how much you care.”–Theodore Roosevelt In early 2019, burnout among healthcare workers was attracting the attention of institutional leaders. Well-being and resilience were widely discussed, and many organizations were acknowledging burnout as an existential threat. Leaders struggled not only to understand its etiology, but also what actions we could take to address burnout. We approached this problem using the traditional model of diagnose and treat the illness. It had been our mantra for centuries. But this time, it was different. We were the ones who were “ill.” Burnout is a personal experience, and well-being is strongly influenced by those around us. As a member-based organization, ASE recognized the need for a purposeful pivot. Could we see it differently? Could we tackle it differently? ASE has consistently prioritized its mission to educate, inform, and advocate for our members. With burnout posing a threat to any progress we made as a Society, ASE quickly recognized that well-being was the glue for our culture that would keep us together and help us thrive in this challenging environment. Caring is not just a personal virtue, but a powerful force that could shape an entire organization. That became the foundation for the ASE Cares campaign in 2019. We aimed to create a sense of belonging within ASE and brainstormed ways we could make our members feel more valued. Perhaps belonging can be best defined as being missed when you are absent. How could ASE be more inclusive and help all our members feel that they are cared for and like they belonged in the ASE family? Caring can be contagious. By fostering an environment where compassion and empathy would thrive, ASE sought to create a culture that would inspire its members to care deeply for each other and their patients. We began thinking about how we could make our Scientific Sessions and courses, guideline documents, advocacy, and member services more inclusive. In 2019, we introduced childcare on site at our Scientific Sessions in Portland to facilitate participation of parents of young children. We also introduced private pods for nursing mothers. At the 2023 Scientific Sessions, there were meditation rooms to allow for prayer or quiet reflection. A “bark park” was introduced in 2022 to allow attendees some ‘down time’ with puppies and enhance their sense of well-being. At our leadership retreat in 2019, we developed our latest strategic plan, described our vision, and ensured that our core values were clearly defined. Once established, our core value of diversity and inclusion was embedded in all our actions. There would be more diverse panels at our meetings, a broader range of speakers from all spheres of ASE, a wide representation in the authorship of our guideline documents, and a volunteer workforce that represented every facet of our membership. The goal was to ensure that no one felt excluded, and everyone felt a sense of belonging. “Who are we missing?” was the essential question in every selection process. We introduced the ASE GEM (Going the Extra Mile) award to honor members who went above and beyond, embodying ASE’s core values. Fellow members choose nominees who showed caring and compassion. We also paid closer attention to our traditional awards to ensure our implicit biases did not influence decisions about awardees. To ensure fairness and inclusivity, we once again asked ourselves, “Who are we missing?” When ASE embraced caring as a core value and implemented the ASE Cares campaign, we realized that compassion and empathy can have a profound impact not only within a professional organization but beyond in healthcare. We recognized that caring can be a catalyst for personal and professional growth. We also hoped that our efforts at ASE would inspire other organizations to prioritize caring as a core value and eventually foster a more compassionate society for all. When Covid-19 struck, ASE was well-positioned to help our members navigate a crucial moment in history marked by unfamiliar and uncomfortable social isolation. Caring for each other was critical through much of 2020 and remains important now. The ASE Cares campaign is part of ASE’s fabric, and caring is firmly in our DNA, as we move forward with our mission and strategic goals. While the bricks of our organizational structure are clearly visible, it is the mortar of caring that binds us together and is fundamental to our mission. In the years to come, ASE Cares will evolve to include other aspects of our Society such as advocacy, healthy member initiatives, and well-being programs that focus on all our members, including those who are the future of our specialty. ASE Cares serves as a reminder that when caring becomes the heartbeat of an organization, remarkable transformations are possible. Madhav Swaminathan, MD, MMCi, FASE, is a tenured Professor and Vice Chair for Faculty Affairs in the Department of Anesthesiology at Duke University School of Medicine in Durham, North Carolina. He has worked as a cardiac anesthesiologist and intensivist at Duke since 2000 and is a past ASE President. Benjamin W. Eidem, MD, FASE, is the Director of Pediatric and Congenital Echocardiography at Mayo Clinic and is a Professor of Pediatrics and Medicine at Mayo Clinic College of Medicine in the Departments of Pediatrics and Cardiology. He has been an ASE member since 1995. This text also appears in the October Echo magazine (http://ASEcho.org/EchoMagazine/).
As I continue my quest to highlight our ASE core values, there could not be a more shining example than the ASEF. The ASEF truly embodies all our ASE core values: Diversity, Excellence, Professionalism, Ethical Behavior, Advancing Knowledge, and Caring Community. I have invited Dr. James Kirkpatrick, MD, FASE, our ASEF Board Chair and our ASE.23 Scientific Sessions Chair, to update our membership on the mission and incredible successes of the ASEF in 2023 as well as to provide a strategic vision as we move into 2024:
It was one of my highest honors as incoming president of ASE to appoint members of our Society to vacancies in our committees and task forces. As I previously elaborated upon in my initial President’s Message in the July issue of JASE and Echo magazine, one of my primary focuses for this year will be on our membership. I thought it would be helpful to remind our members about the depth and breadth of our committees and task forces with hopes of enabling you to position yourselves to participate and excel in these opportunities. To do so, I will present ten tips to hopefully make each of you a successful candidate for these various roles in our Society.(1)Review the current committees and task forces at ASE The best way to participate at ASE is to have a working knowledge of our various committees and task forces. This can be facilitated by reviewing these on our ASE website (https://www.asecho.org/about-ase/get-involved/). You will find the various responsibilities and charges for each committee and task force listed to give you insights into what each committee or task force does at ASE. The current updated membership of each committee and task force is also provided which enables you to communicate with these members to get a better “feel” of their specific committee or task force responsibilities.(2)Be flexible in your initial ASE participation One of the great things about ASE is the ongoing opportunity to serve in many diverse committees and task forces over your years of membership. You may feel that some committees and task forces may not be in your ideal skill set or area of interest; however, every committee or task force will afford you the chance to get a wider vision of ASE as well as to network with other members along with the leadership of those committees. Over my years at ASE, I have served on numerous committees and task forces, and I believe that each of these opportunities gave me a much better appreciation of what our Society is all about.(3)Be ready and stay informed While most committee and task force assignments begin after our annual Scientific Sessions, there continue to be additional opportunities for participation throughout each year. Many of these may be ideal for you. So, stay informed on Connect@ASE and Echo magazine as well as other social media posts from ASE detailing these opportunities.(4)Apply for FASE It is a distinct honor and privilege to be a fellow of our Society! I would highly encourage each of you to review the FASE criteria and application process on the ASE website (https://www.asecho.org/fase/). When it comes to committee and task force assignments, being FASE is a distinct advantage because it demonstrates your commitment to ASE and volunteers holding the FASE designation are placed first. I would love to have the year of my presidency have the highest number of new FASE members – so please let me know how I can help you to achieve this wonderful distinction in our Society!(5)Be patient One big piece of advice from my own experiences at ASE is to have patience in your desire and expectations to participate in our committees and task forces. I view my participation at ASE as a journey. Over my 25 years of ASE membership, there have been many committees and task forces that I was interested in; however, for many of these, I often needed to wait a year or two to be assigned to that opportunity. We often have more volunteers applying than we have open placements. So, view your membership participation at ASE as a journey and be patient for opportunities … because they will certainly come!(6)Consider participation in all areas of ASE In addition to committees and task forces, there are many additional areas to consider for participation at ASE. ASE has six councils (steering committees), including Cardiovascular Sonography, Circulation & Vascular Ultrasound, Pediatric & Congenital Heart Disease, Perioperative Echocardiography, and our two newest additions, Interventional Echocardiography and Critical Care Echocardiography. Each council has its own governance with several at-large positions available each year. In addition, each council has various opportunities for member participation including their own task forces, podcasts, writing groups, webinars, and other council-specific activities. Utilizing council participation is a great step to other areas of ASE service. In addition to committees, task forces, and councils, there are many additional areas at ASE for our members to make a “splash.” Be an ASE social media champion on Twitter. Participate in ASE webinars or our live courses, including the Scientific Sessions. My initial participation in our Society was the presentation of a scientific abstract at the Scientific Sessions, and this experience ignited my desire to make a difference in our Society. Specialty interest groups (SIGs) also provide an avenue for participation and collaboration. Current SIGs include Emerging Echo Enthusiasts, Cardio-Oncology, Neonatal Hemodynamics TnECHO, and Veterinary Medicine. Participation in educational areas of our Society is also an incredible opportunity and experience including product development, live courses, review courses, and webinars.(7)Participate in the ASE Foundation The ASE Foundation (ASEF) is a wonderful place to become involved at ASE. Global outreach has been a very meaningful and rewarding experience for many members of our Society. Travel grants and scholarships within ASEF enable many members to attend the Scientific Sessions and other activities. Funding of scholarly research is a wonderful mission of ASEF that enables many early career as well as seasoned investigators in our Society to fund their important research efforts. The ASEF also has its own Board of Directors with several at-large positions available. Finally, we can all participate in our Society through philanthropy and giving to the ASEF to enable all their fruitful efforts to continue.(8)Publish your important work in our flagship journals: JASE, CASE, and Echo magazine In addition to abstract submissions at the Scientific Sessions, ASE journals offer a diverse opportunity for our members to submit and publish their work. Each journal has a distinct “flavor” in their type of content which is ideal for all our members to have a choice for their submissions. I will highlight each of these journals in a future President’s Message.(9)Explore micro volunteer opportunities at ASE The advent of micro volunteer opportunities at ASE began under the leadership of our past president Ray Stainback. These opportunities are ideal for all members who want to start “small,” with a limited time commitment needed for participation, but enable members to dream “big” for future leadership opportunities in our Society. These can be experiences like product reviews, taking surveys, reviewing articles, etc.(10)Be a local liaison for ASE Local echo labs and echo societies are foundational to ASE. Being an ASE liaison in your echo lab or local echo society extends the reach and emboldens the mission and strategic reach of our Society. ASE has partnerships with over 70 labs and echo groups. Your advocacy efforts at the local, state, and regional level also can play an enormous role in the future of echocardiography as a specialty as well as ASE as a Society. In summary, ASE is truly the home for all users of ultrasound. We are very fortunate to have an amazing number of very talented members in all areas of our Society. It is my hope that each of our members can find their ideal areas of participation at ASE that can enable and advance their individual career goals as well as facilitate meaningful interactions with many colleagues in our Society.Benjamin W. Eidem, MD, FASE, is the Director of Pediatric and Congenital Echocardiography at Mayo Clinic and is a Professor of Pediatrics and Medicine at Mayo Clinic College of Medicine in the Departments of Pediatrics and Cardiology. He has been an ASE member since 1995. This text also appears in the August Echo magazine.
Great organizations carefully define their culture through their core values. But what exactly are “core values” and how do they impact our Society? The concept of institutional core values was first introduced by Collins and Porras in a 1994 book entitled Built to Last: Successful Habits of Visionary Companies.1 The authors commented that many of the best and most enduring organizations adhered to a set of principles that they termed “core values.” These deeply ingrained principles were intended to guide all an organization’s actions and strategic mission. These core values were the organization’s culture and the DNA that was foundational to the organization’s identity. They were to guide the organization and its people toward a shared mission or goal and were firmly embedded in everything the organization did. ASE has key core values for our Society (Table 1). The foundation of our Society’s strategic goals and mission is rooted in our core values. I believe that it is vital for all our membership to not only be aware of these core values or be able to articulate them but also have these values ingrained in each of our own DNA. Over this coming year, I plan to better define each of these core values as well as demonstrate the impact they have on our Society’s activities and our mission moving forward.Table 1ASE Core ValuesDiversityExcellenceProfessionalismEthical BehaviorAdvancing KnowledgeCaring Community Open table in a new tab In this current JASE President’s Message, I want to focus on diversity. Harvard University defines diversity as “The condition of being different or having differences – differences among people with respect to age, class, ethnicity, gender, health, physical and mental ability, race, sexual orientation, religion, physical size, education level, job and function, personality traits, and other human differences.”2 Being a diverse organization is not only the right thing to do but also has many additional tangible benefits including improved performance, increased member satisfaction and trust, facilitation of a larger talent pool, increased work output, enhanced innovation, and enabling better overall decision-making. Equally important ideals that go hand-in-hand with diversity are equity and inclusion. Equity can be defined as “the consistent and systematic fair, just, and impartial treatment of all individuals, including individuals who belong to underserved communities that have been denied such treatment” while inclusion can be defined as “the recognition, appreciation, and use of the talents and skills of employees of all backgrounds.”3 An inclusive Society provides and sustains a sense of belonging – it values and practices respect for the talents, beliefs, and backgrounds of its members. Equity and inclusivity make people feel respected and valued for who they are. Diversity, equity, and inclusion permeate ASE’s DNA in every facet of our Society, including our committees, task forces, councils, educational courses, and so many other areas. It is demonstrated throughout our Society’s leadership and plays a critical role in our Society’s overall health and ongoing strategic mission. These values enable and enhance our productivity, foster innovation, and promote increased membership engagement. To strive for diversity is not only important to do – it is the essential thing to do! It is our diversity that creates a Society that has all the right tools to succeed. But we cannot rest on our laurels – ASE must continue to be vigilant to ensure that we attract, develop, mentor, and promote the next generation of diverse leaders in all areas of our Society! Remember – great organizations carefully define their culture through their core values. Please join me in your commitment and in your practice of ASE’s core values – with everyone’s support there is truly no limit to our Society’s success!1.Collins JC, Porras JI. Built to Last: Successful Habits of Visionary Companies. HarperCollins Publishers, Inc. New York, New York. 1994.2.Harvard Human Resources Glossary of Diversity, Inclusion and Belonging (DIB) Terms. Accessed July 21, 2023. https://edib.harvard.edu/files/dib_glossary3.DEIA Definitions. U.S. Department of Housing and Urban Development. Accessed July 21, 2023. hud.gov/program_offices/administration/admabout/diversity_inclusion/definitions This text also appears in the September Echo magazine.Benjamin W. Eidem, MD, FASE, is the Director of Pediatric and Congenital Echocardiography at Mayo Clinic and is a Professor of Pediatrics and Medicine at Mayo Clinic College of Medicine in the Departments of Pediatrics and Cardiology. He has been an ASE member since 1995.
Myocardial strain offers new insights into ventricular performance, There are software packages from several different companies used to ascertain this, and little data is available in patients with single right ventricle (sRV) physiology. We aimed to compare the analysis of two strain software applications using a cohort of patients with sRV for both inter-vendor and inter-observer variability. Echocardiograms from 85 patients with sRV (122 separate studies) were prospectively evaluated. All had Glenn and/or Fontan palliation. Longitudinal 4-chamber (4LS), inflow/outflow (IO), circumferential, and radial strain were assessed using Velocity Vector Imaging (VVI, Seimens, Munich) and Automated Functional Imaging (AFI, General Electric, Boston) software. In a subset of 45 patients (61 separate studies), strain measurements were obtained by two sonographers so a paired “inter-observer” analysis could be performed. A moderate correlation between measurements made by the two systems was observed. Circumferential strain assessment had the highest R value (0.77) with all others having R values < 0.6. Both software packages showed modest inter-observer reproducibility for longitudinal and circumferential strain. VVI intraclass correlation coefficients (ICC) for 4LS and average circumferential strain (ACS) were 0.6 and 0.58, compared to 0.68 and 0.59 for AFI. Other than radial strain and VVI IO inferior strain, mean strain differences between AFI and VVI were ≤ 1%. Inter-observer variability is modest, however, mean differences are minimal suggesting reasonable clinical reliability. Inter-vendor variability is greater and not as clinically reliable. In patients with sRV, serial assessments with strain should be performed using the same software.
Introduction: Safety of vigorous exercise for individuals with appropriately-treated HCM remains debated. Physical activity practices in this population have not been described. The objective of this report is to describe physical activity pattern in HCM patients. Long term follow up which is ongoing will determine safety of vigorous exercise. Methods: The NIH-funded LIVE-HCM study prospectively enrolled individuals age 8-60 years with clinically overt HCM or patients with a positive genetic test for sarcomeric HCM. Participants (or parents, for children) answered surveys describing activity patterns. Vigorous exercise was defined as > 6 METS for > 60 hours per year. Clinical and demographic data were derived from medical record review. Results: Among 1798 participants, 745 (41%) reported exercising vigorously, including 296 competitive athletes, with 59 at high-school or college varsity level, 770 (43%) were exercising at moderate, and 283 (16%) at low levels. Of those working, 6% described jobs more active than walking, including 16 in protective services, and 11 in sports/coaching. Clinical/demographic data including imaging characteristics and treatments, are shown in Table, and were mostly similar amongst the exercise-groups. Conclusions: Many patients with HCM are engaged in vigorous exercise including competitive athletics. Prospective follow-up of this cohort is ongoing. Comparison of arrhythmic outcomes outcomes in vigorous exercisers vs moderate/low level exercisers will help determine the safety of exercise in HCM patients.
Ebstein anomaly and tricuspid valve dysplasia are the two most common congenital malformations of the tricuspid valve. Although Ebstein anomaly is considered a disorder of the tricuspid valve leaflets, it is more accurately described as an aberration in myocardial development and essentially is a cardiomyopathy. The importance of the Celermajer index emphasizes that the right atrium is severely enlarged in many patients with Ebstein anomaly. Embryonic regression of the right sinus venosus valve results in the formation the Eustachian and Thebesian valves. Incomplete regression of the fetal right sinus valve results in the persistence of membranous structures within the right atrium, most notably the Chiari network and cor triatriatum dextrum. Imaging of cor triatriatum dextrum is best facilitated in the subxiphoid imaging planes as well as the parasternal short-axis orientation.