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Background: Complex patients requiring operations on the left ventricular outflow tract, aortic valve, or thoracic aorta after previous repair of aortopathy constitute a challenging group, with limited information guiding decision-making. We aimed to use our institutional experience to highlight management challenges and describe surgical pearls to address them. Methods: Forty-one complex patients with surgery on the left ventricular outflow tract, aortic valve, or aorta at Cleveland Clinic Children’s between 2016 and 2021 following previous repair of aortic pathology were retrospectively reviewed. Patients with known connective tissue disease or single ventricle circulation were excluded. Results: Median age at index procedure was 23 years (range 0.25–48) with median of 2 prior sternotomies. Previous aortic operations included subvalvular (n = 9), valvular (n = 6), supravalvular (n = 13), and multi-level surgeries (n = 13). Four deaths occurred in median follow-up of 2.5 years. Mean left ventricular outflow tract gradients improved significantly for patients with obstruction (34.9 ± 17.5 mmHg versus 12.6 ± 6.0 mmHg; p < 0.001). Technical pearls include the following: 1) liberal use of anterior aortoventriculoplasty with valve replacement; 2) primarily anterior aortoventriculoplasty following the subpulmonary conus in contrast to more vertical incision for post-arterial switch operation patients; 3) pre-operative imaging of mediastinum and peripheral vasculature for cannulation and sternal re-entry; and 4) proactive use of multi-site peripheral cannulation. Conclusions: Operation to address the left ventricular outflow tract, aortic valve, or aorta following prior congenital aortic repair can be accomplished with excellent outcomes despite high complexity. These procedures commonly include multiple components, including concomitant valve interventions. Cannulation strategies and anterior aortoventriculoplasty in specific patients require modifications.

We report the case of a young female with juvenile idiopathic arthritis presenting with cardiac tamponade secondary to an unusual pericardial mass. Pericardial masses are typically incidental findings. In rare circumstances they can cause compressive physiology warranting urgent intervention. She required surgical excision which revealed a pericardial cyst encapsulating a chronic solidified hematoma. Though certain inflammatory disorders are associated with myopericarditis, to our knowledge this is the first reported case of a pericardial mass in a well-controlled young patient. We theorize her immunosuppressant therapy resulted in hemorrhage into a pre-existing pericardial cyst, suggesting the need for further follow-up in those on adalimumab therapy.

Aims Seeking to account for accessory atrioventricular conduction potentially leading to ventricular pre-excitation, Mahaim in the mid-20th century had described pathways between the atrioventricular conduction axis and the muscular ventricular septum. We aimed to look for such ‘paraspecific’ connections in adult human hearts. Methods and results We serially sectioned 21 hearts, covering the triangle of Koch and the aortic root, and assessing the atrioventricular node, the penetration of the conduction axis, and the bundle branches in our search for fasciculo-ventricular connections. We also calculated the length of the non-branching bundle, and if present the origin of the fasciculo-ventricular connections. The non-branching bundle was 3.6 ± 1.7 mmin length, varying from 1.7 mm to 7.2 mm. Fasciculo-ventricular connections were found in more than half of the hearts, making direct contact with the muscular septum at an average of 3.5 ± 1.7 mm from the origin of the left bundle branch, with the site of origin varying from 1.1 mm to 5.5 mm from the first fascicle of the left bundle branch. In three hearts, additional fasciculo-fascicular connections were observed in the left bundle branch. Two loops were small, but one loop extended over 9.5 mm. Conclusion We endorse the finding of Mahaim that fasciculo-ventricular pathways exist in most human hearts. We presume the identified connections had the capability of producing ventricular pre-excitation. More studies are needed to determine the potential clinical manifestations.

Aims The arrangement of the conduction axis is markedly different in various mammalian species. Knowledge of such variation may serve to question the validity of using animals as prospective models for design of systems for clinical use. Methods and results We compared the arrangement of the atrioventricular conduction axis in human, murine, canine, porcine, and bovine hearts, examining serially sectioned datasets from 20 human, 16 murine, 3 porcine, 5 canine, and 1 bovine hearts. We also analysed computed tomographic datasets obtained from bovines and one human heart. Unlike the situation in the human heart, there is no formation of an atrioventricular fibrous membranous septum in the murine, canine, porcine, nor bovine hearts. Canine, porcine, and bovine hearts also lack an infero-septal recess, when defined as a fibrous plate supporting the buttress of the atrial septum. In these species, half of the non-coronary leaflet is directly opposed to the ventricular septal surface. Conclusion There is a long right-sided non-branching component of the axis, which skirts the attachment of the non-coronary sinus of the aortic root. In the bovine heart, moreover, the left bundle branch usually extends intramyocardially as a solitary tape before surfacing and ramifying on the left ventricular septal surface. The difference in the atrioventricular conduction axis between species may influence the anatomical substrates for atrioventricular re-entry tachycardia, as well as providing inferences for assessing the risks of transcatheter implantation of the aortic valve. Further studies are now needed to assess these possibilities.

Surgeons and electrophysiologists performing accessory pathway ablation procedures have used the term ‘posteroseptal’ region. This area, however, is neither septal nor posterior, but paraseptal and inferior; paraseptal because it includes the fibro-adipose tissues filling the pyramidal space and not the muscular septum itself and inferior because it is part of the heart adjacent to the diaphragm. It should properly be described, therefore, as being inferior and paraseptal. Pathways in this region can be ablated at three areas, which we term right inferior, mid-inferior, and left inferior paraseptal. The right- and left inferior paraseptal pathways connect the right and left atrial vestibules with the right and left paraseptal segments of the parietal ventricular walls. The mid-inferior paraseptal pathways take a subepicardial course from the myocardial sleeves surrounding the coronary sinus and its tributaries. Our review addresses the evolution of the anatomical concept of the inferior paraseptal region derived from surgical and catheter ablation procedures. We also highlight the limitations of the 12-lead electrocardiogram in identifying, without catheter electrode mapping, which are the pathways that can be ablated without a coronary sinus, or left heart approach.

The pathways for excitation of the atrioventricular node enter either superiorly, as the so-called ‘fast’ pathway, or inferiorly as the ‘slow’ pathway. However, knowledge of the specific anatomical details of these pathways is limited. Most of the experimental studies that established the existence of these pathways were conducted in mammalian hearts, which have subtle differences to human hearts. In this review, the authors summarise their recent experiences investigating human cardiac development, correlating these results with the arrangement of the connections between the atrial myocardium and the compact atrioventricular node as revealed by serial sectioning of adult human hearts. They discuss the contributions made from the atrioventricular canal myocardium, as opposed to the primary ring. Both these rings are incorporated into the atrial vestibules, albeit with the primary ring contributing only to the tricuspid vestibule. The atrial septal cardiomyocytes are relatively late contributors to the nodal inputs. Finally, they relate our findings of human cardiac development to the postnatal arrangement.

Coronary angiography remains the standard for diagnosis of cardiac transplant vasculopathy (CAV), but it is invasive. Non-invasively derived left ventricle (LV) global myocardial work (GMW) indices have not been evaluated. We aimed to assess for correlations between LV GMW and the presence of CAV in a pediatric population. 24 heart transplant patients and 24 normal controls were prospectively enrolled. Patients were age-matched into groups with: orthotopic heart transplant and CAV (OHT-CAV; 6 patients, 33% male, mean age 13.5 years [SD 4.2]), orthotopic heart transplant without CAV (OHT; 18 patients, 67% male, mean age 11.1 years [SD 4.8]), and normal healthy controls (42% male, mean age 12.8 years [SD 5.0]). Transplant patients underwent cardiac catheterization with coronary angiography within 3 months of echocardiogram. Post-processing of echocardiograms with speckle-tracking echocardiography and derivation of GMW indices was performed. OHT-CAV patients had decreased global work efficiency (GWE) compared to OHT (mean difference = 7.01 [1.76, 12.25], adjusted p

Conduction problems still occur following transcatheter aortic valvar replacement. With this in mind, we have assessed the relationship of the conduction axis to the aortic root. We used serial histological sections, made perpendicular to the base of the triangle of Koch in nine hearts, and perpendicular to the aortic root in 11 hearts. We first defined the extent of the fibrous tissues forming the boundaries of an infero-septal recess of the subaortic outflow tract, found in all datasets but one. When the recess was present, the axis penetrated through its rightward wall, giving rise to the left bundle branch prior to entering the outflow tract. The axis itself was usually on the crest of the ventricular septum, but could be deviated leftward or rightward. Its proximity to the virtual basal plane reflected the angulation of the muscular septum. On average, the superior edge of the left bundle was within 3.3 mm of the hinge of the right coronary leaflet, with a range from 0.4 to 10.2 mm. The arrangement was markedly different in the case lacking an infero-septal recess. Our findings necessitated a redefinition of the right fibrous trigone and the central fibrous body. The atrioventricular conduction axis, having entered the aortic root, is usually closest at the hinge of the right coronary leaflet. Knowledge of the depth of the infero-septal recess, and the angulation of the muscular ventricular septal, may help to avoid conduction problems following transcatheter implantation of the aortic valve.

Professor Liesl Zühlke is the focus of our fifth in a series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Professor Zühlke (nee Hendricks) was born in Cape Town, South Africa. She would attend medical school in her hometown at University of Cape Town, graduating in 1991. Professor Zühlke then went on to complete a Diploma in Child Health at College of Medicine in Cape Town followed by completion of her Paediatric and Paediatric Cardiology training in 1999 and 2007, respectively. She would subsequently complete her Masters of Public Health (Clinical Research Methods) at the University of Cape Town, completing her dissertation in 2011 on computer-assisted auscultation as a screening tool for cardiovascular disease, under the supervision of Professors Landon Myer and Bongani Mayosi.Professor Zühlke began her clinical position as a paediatric cardiologist in the Department of Paediatrics and Child Health at the Red Cross War Memorial Children’s Hospital in Cape Town, South Africa in 2007. In this role, she has been instrumental in developing a transitional clinic at the paediatric hospital, is a team member of the combined cardio-obstetric and grown-up congenital heart disease clinics, each of which are rare in South Africa, with very few similar clinics in Africa. Professor Zühlke would continue her research training, completing her Doctorate at the University of Cape Town in 2015, with her dissertation on the outcomes of asymptomatic and symptomatic rheumatic heart disease under the supervision of Professor Bongani Mayosi and Associate Professor Mark Engel. In 2015, in affiliation with the University of Cape Town and the Department of Paediatrics and the Institute of Child Health, she established The Children’s Heart Disease Research Unit, with the goals to conduct, promote and support paediatric cardiac research on the African continent, facilitate Implementation Science and provide postgraduate supervision and training in paediatric cardiac research. In 2018, she would subsequently complete her Master of Science at the London School of Economics in Health Economics, Outcomes and Management of cardiovascular sciences. Professor Zühlke currently serves as the acting Deputy-Dean of Research at the Faculty of Health Sciences, University of Cape Town.Professor Zühlke has achieved the highest leadership positions within cardiology in South Africa, including President of the Paediatric Cardiac Society of South Africa and President of the South African Heart Association. She is internationally regarded as a leader in research related to rheumatic heart disease. Professor Zühlke’s work includes patient, family and health advocacy on a global scale, being involved in the development of policies that have been adopted by major global organisations such as the World Health Organization. In addition to her clinical and research efforts, she is highly regarded by students, colleagues and graduates as an effective teacher, mentor and advisor. This article presents our interview with Professor Zühlke, an interview that covers her experience as a thought leader in the field of Paediatric Cardiology, specifically in her work related to rheumatic heart disease, Global Health and paediatric and congenital cardiac care in resource-limited settings.

Global myocardial work (GMW) is an emerging method to characterize left ventricle (LV) function with potential advantages over both ejection fraction and global longitudinal strain (GLS). We aimed to determine the feasibility and reproducibility for echocardiographic-derived GMW in a healthy pediatric population; establish normal reference values; and investigate the influence of age, gender, and other clinical factor on normal reference ranges. We prospectively enrolled 212 individuals (median age of 9 years; interquartile range, 6 to 12 years, 112 female). Global work index (GWI), global constructive work (GCW), global wasted work (GWW), and global work efficiency (GWE) were measured from LV pressure-strain loops. Quantification of GMW was performed using a GE Vivid E95 system and available software package (Echopac V.203, GE). The mean LV EF was 64 ± 3% with GLS of -21.3 ± 1.5%. GWI was 1688 ± 219 mmHg% with mean GWE of 96.5 ± 1.4%. The GCW was 1959 ± 207 mmHg%, and the mean GWW of 61.1 ± 30.9 mmHg%. No significant difference was found in MW indices across age group and gender (p > 0.05 for all). There were significant correlations between both GWI and GCW with GLS and systolic blood pressure (p < 0.001), but not with GWE and GWW. Linear regression model revealed that GWI and GCW were more closely correlated with systolic blood pressure than GLS. LV MW indices had good intra-observer and inter-observer reproducibility. This study establishes both the feasibility and reference ranges for non-invasive echocardiographic indices of GMW in healthy children. Myocardial work appears to be a complementary modality to assess LV performance in children.

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Dr. Katarina Hanséus is the focus of our fourth in a series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Hanséus was born in Malmö, Sweden. She attended undergraduate school in her home town in Malmö, Sweden, graduating in 1974. Dr. Hanséus then went on to complete medical school at University of Lund in Lund, Sweden, graduating in 1980, where additionally she completed a Doctoral Dissertation in the evaluation of cardiac function and chamber size in children using Doppler and cross-sectional echocardiography. Under the Swedish Board of National Welfare, Dr. Hanséus completed her authorisation as a paediatrician in 1986, followed by her authorisation as a paediatric cardiologist in 1988, at University of Lund. She was appointed head of Paediatric Cardiology in 2000 at the Children’s Heart Center, Skane University Hospital, Lund, Sweden. The programme at Lund serves as one of the two national referral centres for comprehensive paediatric and congenital cardiac care, including paediatric cardiac surgery, in Sweden. From 2006 to 2013, she served as the clinical and administrative head of the Department of Neonatology, Paediatric Surgery, Paediatric Intensive Care, Paediatric Cardiology, and Paediatric Cardiac Surgery, returning as the head of Paediatric Cardiology in 2013, for which she currently holds the position.Dr. Hanséus is a recognised leader in the field of Paediatric Cardiology and has been involved in leadership within the Swedish Pediatric Society, the Swedish Association for Pediatric Cardiology, and the Association for European Paediatric and Congenital Cardiology throughout her career. Within the Association for European Paediatric and Congenital Cardiology, she served as the Secretary General from 2011 to 2016, the President Elect in 2018, and is the current President serving from 2019 until 2022. This article presents our interview with Dr. Hanséus, an interview that covers her experience as a leader in the field of Paediatric Cardiology, including the history and goals of the Association for European Paediatric and Congenital Cardiology, and her role and vision as their current President.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Progressive aortic dilation is common in Marfan syndrome (MFS) and Loeys–Dietz syndrome (LDS). Risk factors for progression are poorly understood. Normal variation in the aortic root (AoR) rotational position relative to the left ventricular base may impact this risk. We aimed to assess the relationship between the rotational position of the AoR and aortic dimensions in this population. Patients with a genetic diagnosis of MFS or LDS were included. AoR and ascending aorta (AAo) dimensions were measured from the first and most recent transthoracic echocardiogram. The AoR rotational angle was measured in the parasternal short-axis plane in diastole. Linear regression was used to study the correlation between AoR rotation angle and aortic dimensions. 53 MFS and 14 LDS patients were included (age 11.5 ± 5.8 years at first TTE and 21.2 ± 7.2 years at most recent, 68% male). The mean indexed AoR and AAo values were 2.26 ± 0.58 cm/m2 and 1.64 ± 0.35 cm/m2 at the first TTE and 1.98 ± 0.39 cm/m2 and 1.45 ± 0.25 cm/m2 at the most recent TTE, respectively. The mean AoR rotational angle was 8 ± 14°. AoR rotational angle was central (− 9 to + 14°) in 42, clockwise (≥ + 15°) in 19, and counterclockwise (≤ −10°) in 6. The six outliers with counterclockwise position were excluded. There was a positive association between the AoR rotation angle and most recent TTE indexed AoR (r2 = 0.08, p = 0.02) and AAo sizes (r2 = 0.08, p = 0.02). There was no association between AoR rotational angle and rate of change in indexed AoR size (p = 0.8). There was a positive association between AoR rotation angle and rate of change in indexed AAo size (r2 = 0.10, p = 0.01). There is an association between clockwise rotational position of the AoR and increased AoR and AAo dimensions in children and young adults with MFS and LDS patients. The rotational position of the AoR may guide follow-up in these patient populations. However, this potential risk factor for dilation warrants further investigation.

Non-invasive imaging markers in patients with repaired tetralogy of Fallot (rTOF) are still being investigated to inform clinical decision making. Atrial function is a prognostic indicator in many acquired and congenital heart diseases. We sought to examine the relationship between cardiac MRI (CMR)-derived indices of left atrial (LA) function, native left ventricular (LV) T1 values, biventricular systolic function, and exercise capacity in rTOF. Sixty-six patients with rTOF without prior pulmonary valve replacement who underwent CMR (median age 18.5 years) were identified. Twenty-one adult rTOF patients (age range 19–32 years) were compared with 20 age-matched healthy volunteers (age range 19–34 years). LA reservoir, conduit, and pump global longitudinal strain (GLS) and strain rate (SR) were determined by tissue tracking. Native LV T1 values were measured on rTOF patients. Pearson correlations were performed to determine bivariate associations. Adult rTOF patients had higher pump GLS, pump:conduit, and pump:reservoir GLS ratios, and lower conduit:reservoir GLS ratio, LV ejection fraction (EF), and right ventricular EF compared to controls (p

Global myocardial work (GMW) provides a metric of left ventricular (LV) function and energy consumption. Its non-invasive assessment by echocardiography correlates with invasive measures and normal values have been reported in healthy adults. We aimed to establish normal values in a healthy adolescent population. Fifty-two healthy adolescents (mean age = 14.5 ± 2.0 years, range 11–19 years, 62% male) with normal echocardiograms were included. Brachial cuff blood pressure was obtained immediately following apical imaging in the supine position. Post-processing of echocardiograms for speckle tracking strain measurement and derivation of global myocardial work indices from LV pressure–strain loops was performed. The mean global work index (GWI) was 1802.0 ± 264.4 mmHg% with mean global work efficiency of 95.5 ± 1.1%. The mean global constructive work (GCW) was 2054.5 ± 297.3 mmHg%, and the mean global wasted work 83.8 ± 28.1 mmHg%. On multivariable analysis, there were significant associations between both GWI and GCW with systolic blood pressure (β coefficient = 0.57, p

The essence of so-called heterotaxy is the potential disharmony between the arrangement of the bronchuses, abdominal organs, and the atrial appendages. Accurate description of the heart, however, can only be provided by specific description of these features, all of which are readily discernible in the clinical setting. We argue that, when accurate description of the atrial and visceral arrangement is provided, along with appropriate description of the intracardiac findings, no further accuracy is gained by suggesting that an individual heart is “heterotaxic”.

Dr Rodney Franklin is the focus of our third in a planned series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care.” Dr Franklin was born in London, England, spending the early part of his childhood in the United States of America before coming back to England. He then attended University College London Medical School and University College Hospital in London, England, graduating in 1979. Dr Franklin would then go on to complete his general and neonatal paediatrics training in 1983 at Northwick Park Hospital and University College Hospital in London, England, followed by completing his paediatric cardiology training in 1989 at Great Ormond Street Hospital for Children in London, England. During this training, he additionally would hold the position of British Heart Foundation Junior Research Fellow from 1987 to 1989. Dr Franklin would then complete his training in 1990 as a Senior Registrar and subsequent Consultant in Paediatric and Fetal Cardiology at Wilhelmina Sick Children’s Hospital in Utrecht, the Netherlands. He subsequently obtained his research doctorate at University of London in 1997, consisting of a retrospective audit of 428 infants with functionally univentricular hearts.Dr Franklin has spent his entire career as a Consultant Paediatric Cardiologist at the Royal Brompton & Harefield Hospital NHS Foundation Trust, being appointed in 1991. He additionally holds honorary Consultant Paediatric Cardiology positions at Hillingdon Hospital, Northwick Park Hospital, and Lister Hospital in the United Kingdom, and Honorary Senior Lecturer at Imperial College, London. He has been the Clinical Lead of the National Congenital Heart Disease Audit (2013–2020), which promotes data collection within specialist paediatric centres. Dr Franklin has been a leading figure in the efforts towards creating international, pan European, and national coding systems within the multidisciplinary field of congenital cardiac care. These initiatives include but are not limited to the development and maintenance of The International Paediatric & Congenital Cardiac Code and the related International Classification of Diseases 11th Revision for CHD and related acquired terms and definitions. This article presents our interview with Dr Franklin, an interview that covers his experience in developing these important coding systems and consensus nomenclature to both improve communication and the outcomes of patients. We additionally discuss his experience in the development and implementation of strategies to assess the quality of paediatric and congenital cardiac care and publicly report outcomes.

Turner syndrome is associated with an increased risk of aortic aneurysms and dissection. Recent 2017 clinical care guidelines recommend medical therapy to treat aortic dilatation, although whether this slows dilatation is unknown. We aimed to describe a pre-guideline cohort of Turner syndrome patients with aortic dilatation, the rate of dilatation following diagnosis, and post therapy dilatation rates. We conducted a retrospective review of Turner syndrome patients with a dilated aortic root or ascending aorta by current definitions. In total, 40 patients were included with 22 treated patients. Most patients had 45,X karyotype, were white, non-Hispanic, and received both growth hormone and estrogen. Except for hypertension, there were no differences in risk factors among treated and untreated groups. Bicuspid aortic valve was very common. Treatment group patients had significantly more dilated ascending aortas by absolute measurements and aortic size index. In an adjusted model, there was minimal change in aortic measures over time and this was not associated with medication use. In conclusion, in this cohort, Turner syndrome patients with aortic dilatation were more likely to be treated if they had hypertension and if they met multiple dilatation criteria. Further study is needed to establish medical therapy efficacy on dilatation progression.

Pectus excavatum (PE) is a chest wall deformity of variable severity and symptomatology. Existing female-specific literature highlights breast asymmetry and cosmetic reconstruction. We sought to evaluate gender differences in cardiopulmonary function. Cardiac MRIs, pulmonary function tests (PFTs), and cardiopulmonary exercise tests (CPETs) were reviewed in 345 patients undergoing preoperative evaluation for PE. Regression modeling was used to evaluate associations between gender and clinical endpoints of cardiopulmonary function. Mean age was 15.2 years, 19% were female, 98% were white. Pectus indices included median Haller Index (HI) of 4.8, mean depression index (DI) of 0.63, correction index (CI) of 33.6%, and Cardiac Compression Index (CCI) of 2.79. Cardiac assessment revealed decreased right and left ventricular ejection fraction (RVEF, LVEF) in 16% and 22% of patients, respectively. PFTs and CPETs were abnormal in ~ 30% of patients. While females had deeper PE deformities—represented by higher pectus indices—they had superior function with higher RVEF, LVEF Z-scores, FEV1, VO2 max, O2 pulse, work, and breathing reserve (p

Dr. Jennifer Li is the focus of our second in a planned series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Li was born in Boston, Massachusetts, United States of America, and moved to Indianapolis, Indiana where she completed her secondary education. She then attended Stanford University, majoring in Chemistry and English and graduating with distinction in 1983. Dr. Li then attended Duke University School of Medicine, graduating in 1987. She then completed her internship at Children’s Hospital of Philadelphia in 1987–1989, returning to Duke University Medical Center to complete both her residency in general paediatrics in 1989–1990 followed by her fellowship in paediatric cardiology in 1990–1993. She would later complete her Master’s Degree in Health Sciences at Duke University in 2005.Dr. Li has spent her entire career as a paediatric cardiologist at Duke University Medical Center, where she was appointed a Professor of Pediatrics and Professor of Medicine in 2008 and has held the position as Beverly C. Morgan Endowed Professor of Pediatrics since 2012. She has served as the Chief of Paediatric Cardiology at Duke University Medical Center since 2006. She also was the Director of Paediatric Research at Duke Clinical Research Institute from 2001-2015. Dr. Li has played an instrumental role in evaluating the safety and efficacy of drugs in children, as well as in analysing and linking large multicentric databases to evaluate the outcomes, quality, and cost of paediatric and congenital cardiac care. Dr. Li has received funding from the National Institute of Health of the United States of America, as well as from industry and foundation grants. This article presents our interview with Dr. Li, an interview that covers her experience collaborating with governmental organizations and industry in the pursuit of common interests to design clinical drug trials, link and analyse large, multicentric databases, and improve paediatric health care.

Dr. Jane Newburger is the focus of our first in a planned series of interviews inCardiology in the Youngentitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Newburger was born in Manhattan, New York, United States of America. She was raised in the Bronx for her first six years of life, at which point her family moved to Yonkers, New York, where she spent the rest of her childhood. She then attended Bryn Mawr College where she majored in psychology. Dr. Newburger subsequently attended Harvard Medical School, graduating in 1974. She did her internship and residency in paediatrics at Boston Children’s Hospital in 1974–1976, followed by her fellowship at Boston Children’s Hospital in 1976–1979. She received her Masters in Public Health at the Harvard School of Public Health in 1980.Dr. Newburger has spent her entire career as a paediatric cardiologist at Boston Children’s Hospital and Harvard Medical School, where she was appointed a Professor of Pediatrics in 1999 and has held the position as Commonwealth Professor of Pediatrics since 2008. She has established herself as a leading clinical scientist within the field of paediatric cardiology, with expertise in leadership of multicentre and multidisciplinary research, including the building of collaborative groups. She has been continuously funded by the National Institute of Health since 1982, and amongst other areas has led the field in the areas of neurodevelopmental outcomes in congenital heart disease, improved methods of vital organ support, and management of Kawasaki disease. This article presents our interview with Dr. Newburger, an interview that covers her path towards becoming a clinical scientist, her interests spanning four decades of hard work, and her strategies to design and lead successful multicentre studies.

To assess atrial function in Fontan patients using cardiac MRI (CMR) and determine the relationships between atrial function, hemodynamics, and clinical outcomes. Two center chart review identified all Fontan patients with technically adequate CMR to assess atrial function within 1 year of cardiac catheterization and age-matched controls with CMR. Atrial reservoir, conduit, and pump global longitudinal strain and strain rate measurements were determined by CMR. Univariate and stepwise multivariable analysis were conducted to detect associations of atrial function measures with ventricular end diastolic pressure (EDP), cardiac index (CI), exercise capacity, liver stiffness and a composite outcome of heart transplantation, ventricular assist device or death. The study cohort was comprised of 33 Fontan patients and 30 age-matched controls. Fontan patients had lower atrial reservoir and conduit strain, and lower reservoir, conduit, and pump strain rate compared to age-matched controls. Atrial conduit strain was negatively associated with EDP and lower atrial pump-to-conduit strain ratio was associated with lower cardiac index in multivariable analysis. Lower atrial pump strain was associated with lower exercise capacity (per 1% increase: β = 2.3 ± 0.9, p = 0.03) and higher liver stiffness (per 1% increase: β = − 0.12 ± 0.03, p = 0.002). Higher atrial pump strain (HR per 1% increase = 0.53 [95% confidence interval 0.22, 0.83], p = 0.002) and ventricular ejection fraction (HR per 1% increase = 0.90 [95% confidence interval 0.80, 0.98], p = 0.02) were associated with lower risk for the composite adverse outcome. Atrial function is impaired in Fontan patients and is associated with worse CI, EDP, exercise performance, liver stiffness, and a higher risk for adverse outcomes.