How best can we name the channels seen in the setting of deficient ventricular septation?

Surgical repair of channels between the ventricles is enhanced when the surgeon knows precisely where to place a patch, or baffle, so as to restore septal integrity. The paediatric cardiologist should provide the necessary information. Communication will be enhanced if the same words are used to account for the structures in question. Currently, however, the same term, namely "ventricular septal defect," is used to account for markedly different areas within the heart. Closure of perimembranous defects found in hearts with concordant or discordant ventriculo-arterial connections restores the integrity of the ventricular septum, at the same time separating the systemic and pulmonary blood streams. When both arterial trunks arise from the right ventricle, in contrast, the surgeon when placing a baffle so as to separate the blood streams, does not close the channel most frequently described as the "ventricular septal defect." In this review, we show that the perimembranous lesions as found in hearts with concordant or discordant ventriculo-arterial connections are the right ventricular entrances to the areas subtended beneath the hinges of the leaflets of the aortic or pulmonary valves. When both arterial trunks arise from the right ventricle, and the channel between the ventricles is directly subaortic, then the channel termed the "ventricular septal defect" is the left ventricular entrance to the comparable space subtended beneath the aortic root. We argue that recognition of these fundamental anatomical differences enhances the appreciation of the underlying morphology of the various lesions that reflect transfer, during cardiac development, of the aortic root from the morphologically right to the morphologically left ventricle.

Pilot phase experience of the International Quality Improvement Collaborative catheterization registry.

OBJECTIVES: To describe the development of a quality collaborative for congenital cardiac catheterization centers in low and middle-income countries (LMICs) including pilot study data and a novel procedural efficacy measure. BACKGROUND: Absence of congenital cardiac catheterization registries in LMICs led to the development of the International Quality Improvement Collaborative Congenital Heart Disease Catheterization Registry (IQIC-CHDCR). As a foundation for this initiative, the IQIC is a collaboration of pediatric cardiac surgical programs from LMICs. Participation in IQIC has been associated with improved patient outcomes. METHODS: A web-based registry was designed through a collaborative process. A pilot study was conducted from October through December 2017 at seven existing IQIC sites. Demographic, hemodynamic, and adverse event data were obtained and a novel tool to assess procedural efficacy was applied to five specific procedures. Procedural efficacy was categorized using ideal, adequate, and inadequate. RESULTS: A total of 429 cases were entered. Twenty-five adverse events were reported. The five procedures for which procedural efficacy was measured represented 48% of cases (n = 208) and 71% had complete data for analysis (n = 146). Procedure efficacy was ideal most frequently in patent ductus arteriosus (95%) and atrial septal defect (90%) device closure, and inadequate most frequently in coarctation procedures (100%), and aortic and pulmonary valvuloplasties (50%). CONCLUSIONS: The IQIC-CHDCR has designed a feasible collaborative to capture catheterization data in LMICs. The novel tool for procedural efficacy will provide valuable means to identify areas for quality improvement. This pilot study and lessons learned culminated in the full launch of the IQIC-CHDCR.

Management of undernutrition and failure to thrive in children with congenital heart disease in low- and middle-income countries.

Poor growth with underweight for age, decreased length/height for age, and underweight-for-height are all relatively common in children with CHD. The underlying causes of this failure to thrive may be multifactorial, including innate growth potential, severity of cardiac disease, increased energy requirements, decreased nutritional intake, malabsorption, and poor utilisation of absorbed nutrition. These factors are particularly common and severe in low- and middle-income countries. Although nutrition should be carefully assessed in all patients, failure of growth is not a contraindication to surgical repair, and patients should receive surgical repair where indicated as soon as possible. Close attention should be paid to nutritional support - primarily enteral feeding, with particular use of breast milk in infancy - in the perioperative period and in the paediatric ICU. This nutritional support requires specific attention and allocation of resources, including appropriately skilled personnel. Thereafter, it is essential to monitor growth and development and to identify causes for failure to catch-up or grow appropriately.

Global perspective on training and staffing for paediatric cardiac critical care.

This manuscript provides a global perspective on physician and nursing education and training in paediatric cardiac critical care, including available resources and delivery of care models with representatives from several regions of the world including Africa, Israel, Asia, Australasia, Europe, South America, and the United States of America.

[Updated clinical classification of pulmonary hypertension]. / Pulmoner arter hipertansiyonunun güncel klinik siniflandirilmasi.

In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (YPPH) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1". Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4. (J Am Coll Cardiol 2013;62:D34-41) a 2013 by the American College of Cardiology Foundation.

Rheumatic fever & rheumatic heart disease: the last 50 years.

Rheumatic fever (RF) and rheumatic heart disease (RHD) continue to be a major health hazard in most developing countries as well as sporadically in developed economies. Despite reservations about the utility, echocardiographic and Doppler (E&D) studies have identified a massive burden of RHD suggesting the inadequacy of the Jones' criteria updated by the American Heart Association in 1992. Subclinical carditis has been recognized by E&D in patients with acute RF without clinical carditis as well as by follow up of RHD patients presenting as isolated chorea or those without clinical evidence of carditis. Over the years, the medical management of RF has not changed. Paediatric and juvenile mitral stenosis (MS), upto the age of 12 and 20 yr respectively, severe enough to require operative treatement was documented. These negate the belief that patients of RHD become symptomatic ≥20 years after RF as well as the fact that congestive cardiac failure in childhood indicates active carditis and RF. Non-surgical balloon mitral valvotomy for MS has been initiated. Mitral and/or aortic valve replacement during active RF in patients not responding to medical treatment has been found to be life saving as well as confirming that congestive heart failure in acute RF is due to an acute haemodynamic overload. Pathogenesis as well as susceptibility to RF continue to be elusive. Prevention of RF morbidity depends on secondary prophylaxis which cannot reduce the burden of diseases. Primary prophylaxis is not feasible in the absence of a suitable vaccine. Attempts to design an antistreptococcal vaccine utilizing the M-protein has not succeeded in the last 40 years. Besides pathogenesis many other questions remain unanswered.

Editorial Board Composition Among Pediatric Cardiology Journals: Time to Cast the Net Wider.

Background: No data currently exist on the diversity of editorial board members (EBMs) of pediatric cardiology journals. Objectives: The objective was to investigate the editorial boards of 5 pediatric cardiology journals to assess the composition of these boards in terms of the geographical, gender, and economic representation of their members. Methods: Information on EBMs was collected directly from 5 journal websites accessed in February 2022. The following data were collected: country of practice (including World Bank geographical and income classification), institution of practice, role on editorial board, and whether an individual held a role on 1 or more of the boards included. Results: A total of 455 EBMs were identified. A total of 369 (81%) were male. All editors-in-chief were male, and 4 were from the United States. EBMs practicing in North America accounted for 278 individuals (61%) of the editorial boards reviewed. The next majority of EBMs are practicing within Europe and Central Asia (23%, n = 103), East Asia and Pacific (7%, n = 31), Middle East and North Africa (4%, n = 18), and Latin America and Caribbean (4%, n = 16). Less than 2% (n = 9) practice in Sub-Saharan Africa and South Asia. Over 90% (n = 415) practice in high-income countries. There was no representation from low-income countries. Conclusions: Women and pediatric cardiologists practicing in countries outside of Europe and North America were underrepresented on the editorial boards of the journals studied. Diversifying composition of editorial boards may provide greater representation of underserved areas and encourage broader avenues of investigation and research.

Pulmonary Hypertension Registry of Kerala, India (PRO-KERALA): One-year outcomes.

BACKGROUND: Short term outcomes of patients with pulmonary hypertension are not available from low and middle-income countries including India. METHODS: We conducted a prospective study of 2003 patients with pulmonary hypertension, from 50 centres (PROKERALA) in Kerala, who were followed up for one year. Pulmonary hypertension (PH) was mainly diagnosed on the basis of Doppler echocardiography. The primary outcome was a composite end-point of all-cause death and hospital admission for heart failure. All cause hospitalisation events constituted the secondary outcome. RESULTS: Mean age of study population was 56 ± 16 years. Group 1 and Group 2 PH categories constituted 21.2% and 59% of the study population, respectively. Nearly two-thirds (65%) of the study participants had functional class II symptoms. 31% of Group 1 PH patients were on specific vasodilator drugs.In total, 83 patients (4.1%) died during the one-year follow-up period. Further, 1235 re-hospitalisation events (61.7%) were reported. In the multivariate model, baseline NYHA class III/IV (OR 1.87, 95% C.I. 1.35-2.56), use of calcium channel blockers (OR 0.18, 95% C.I. 0.04-0.77), vasodilator therapy (OR 0.5, 95% C.I. 0.28-0.87) and antiplatelet agents (OR 1.80, 95% C.I. 1.29-2.51) were associated with primary composite outcome at one-year (p < 0.05). CONCLUSION: In the PROKERALA registry, annual mortality rate was 4%. More than half of the patients reported re-hospitalisation events on follow up. Uptake of guideline directed therapies were suboptimal in the study population. Quality improvement programmes to improve guideline directed therapy may improve clinical outcomes of PH patients in India.

A Population Health Approach to Address the Burden of Congenital Heart Disease in Kerala, India.

Background: Congenital heart disease (CHD) has emerged as a leading contributor to infant mortality in many low-and middle-income countries (LMICs). We report early results of a population health program for CHD, implemented in the state of Kerala, India. Objective: Report on early results of a population-based program implementation in a LMIC to reduce mortality from CHD. Methods: We retrospectively analyzed the results of an innovative population-based program to address CHD. We devised, implemented and evaluated measures in the care continuum to address deficiencies in CHD care in Kerala, India, through structured capacity building initiatives that enabled early detection, prompt stabilization and expedited referral to a tertiary center. A comprehensive web-based application enabled real-time case registration, prioritization of treatment referrals, and tracking every child registered with CHD. Advanced pediatric heart care was delivered through a public-private partnership. Results: Early identification, referral, and treatment of infants with CHD were improved. The web-based application, 'Hridyam,' registered 502 cases in 2017 (Aug-Dec), 2190 in 2018 and 3259 in 2019; infants < 1 year of age constituted 56, 62 and 63% in these years, respectively. The number of heart operations managed through Hridyam rose from 208 to 624 and 1227 in the same years, with overall 30-day mortality of 2.4%. Overall- and CHD-related infant mortality in Kerala fell by 21.1% and 41.0%, respectively, over the same interval. Unmet challenges include lack of universal catchment and a 5% preoperative mortality rate. Conclusion: We demonstrate successful implementation of a population-based and real-time approach to reduce CHD mortality. We speculate that Hridyam has contributed to the observed decline in Kerala's IMR from 12 to 7 between 2016 and 2019. This model has potential applications for other conditions, and in other jurisdictions, especially LMICs considering building CHD capacity.

Status of eye health among tribal school children in South India.

Purpose: Global trends show a high prevalence of refractive errors among children. The prevalence of vision impairment due to uncorrected refractive errors among school children is increasing and the need for management of other ocular conditions is also reported. This study presents the status of eye health and pattern of daily activities among the school children of a tribal location in Tamil Nadu, South India. Methods: A cross-sectional study was conducted in 13 schools of Karumandurai cluster, Salem district in Tamil Nadu, India. A three-phased comprehensive school screening protocol was conducted to understand the prevalence of vision impairment, refractive error, and other ocular conditions along with a survey about the daily activities of the children at school and home. Results: Among the 3655 children screened, the prevalence of vision impairment was found to be 0.62% (n = 23, 95% confidence interval [CI] 0.42-0.94) and prevalence of refractive error was 0.30% (n = 11, 95%CI 0.17-0.54), among which 0.11% (n = 4) were already wearing spectacles. A total of 44 children (1.20%; 95%CI 0.90-1.61) were found to have other ocular problems and among them, 14 (0.38%) had visual acuity less than 20/30 (6/9). Almost 84% of children required surgical or specialty eye care services. Vision impairment was more in children with other ocular conditions compared to refractive errors (P < 0.001). Conclusion: The prevalence of vision impairment and refractive errors in this tribal area was less. Ocular conditions were more prevalent than refractive errors in this tribal region with the majority of children needing specialty or surgical eye care services. This implies the need for access to secondary or tertiary eye care centers.

Status of Eye Health among School Children in South India - Sankara Nethralaya School Children Eye Examination Study (SN-SEES).

Purpose: To define the prevalence of visual impairment, refractive errors and other ocular problems among school children from the public schools of South India between 2011 and 2015.Methods: This was a cross-sectional study covering 296 schools in the three districts of Tamil Nadu. The school eye screening included visual acuity assessment, external eye examination, objective and subjective refraction, and direct ophthalmoscopy. Vision impairment was defined as logMAR visual acuity of less than 0.2 (Snellen equivalent of 6/9).Results: A total of 91545 children were included with a mean age of 12.9 ± 3.0 years. The prevalence of vision impairment was found to be 5.67% (95%CI 5.53-5.83) and spherical equivalent refractive error was 4.42% (95%CI 4.29-4.56). The prevalence of myopia, hyperopia, and 'other refractive errors' was found to be 3.57% (95%CI 4.01-4.27), 0.03% (95%CI 0.02-0.04), and 0.82% (95%CI 0.76-0.88) respectively. Strabismus, retina and neuro-ophthalmology-related abnormalities, ptosis, and corneal scars were the common ocular problems. In the rural region the prevalence of the refractive errors and the ocular problems were 2.92% and 2.32%, respectively.Conclusion: The study reports a lower prevalence of refractive errors and myopia in this population, much lesser compared to other reported studies from India. Rural regions exhibit an equal need for both refractive services and management of other ocular problems.

Graphene's non-equilibrium fermions reveal Doppler-shifted magnetophonon resonances accompanied by Mach supersonic and Landau velocity effects.

Oscillatory magnetoresistance measurements on graphene have revealed a wealth of novel physics. These phenomena are typically studied at low currents. At high currents, electrons are driven far from equilibrium with the atomic lattice vibrations so that their kinetic energy can exceed the thermal energy of the phonons. Here, we report three non-equilibrium phenomena in monolayer graphene at high currents: (i) a "Doppler-like" shift and splitting of the frequencies of the transverse acoustic (TA) phonons emitted when the electrons undergo inter-Landau level (LL) transitions; (ii) an intra-LL Mach effect with the emission of TA phonons when the electrons approach supersonic speed, and (iii) the onset of elastic inter-LL transitions at a critical carrier drift velocity, analogous to the superfluid Landau velocity. All three quantum phenomena can be unified in a single resonance equation. They offer avenues for research on out-of-equilibrium phenomena in other two-dimensional fermion systems.

Obesity in children & adolescents.

Worldwide, obesity trends are causing serious public health concern and in many countries threatening the viability of basic health care delivery. It is an independent risk factor for cardiovascular diseases and significantly increases the risk of morbidity and mortality. The last two decades have witnessed an increase in health care costs due to obesity and related issues among children and adolescents. Childhood obesity is a global phenomenon affecting all socio-economic groups, irrespective of age, sex or ethnicity. Aetiopathogenesis of childhood obesity is multi-factorial and includes genetic, neuroendocrine, metabolic, psychological, environmental and socio-cultural factors. Many co-morbid conditions like metabolic, cardiovascular, psychological, orthopaedic, neurological, hepatic, pulmonary and renal disorders are seen in association with childhood obesity. The treatment of overweight and obesity in children and adolescents requires a multidisciplinary, multi-phase approach, which includes dietary management, physical activity enhancement, restriction of sedentary behaviour, pharmacotherapy and bariatric surgery. A holistic approach to tackle the childhood obesity epidemic needs a collection of activities including influencing policy makers and legislation, mobilizing communities, restructuring organizational practices, establishing coalitions and networks, empowering providers, imparting community education as well as enriching and reinforcing individual awareness and skills. The implications of this global phenomenon on future generations will be serious unless appropriate action is taken.

Positron emission tomography-computed tomography-associated incidental neoplasms of the thyroid gland.

With the increasing use of 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) scans in oncology, the finding of thyroid incidentalomas, also popularly described as PET-associated incidental neoplasms (PAINs) of the thyroid gland is not unusual. The 18F-FDG PET-CT scans of all patients who underwent imaging for indications other than thyroid malignancy at our tertiary care center between January 1 and December 31, 2017, were retrospectively reviewed for PAINs of the thyroid. A total of 1737 18F-FDG PET-CT scans were done at our center in the year 2017. 288 thyroid incidentalomas were detected in the said period; the rate of PET-CT-detected thyroid incidentalomas being 16.58%, focal incidentalomas among them being 11.7%. Only 29 out of 204 patients (14.21%) with focal thyroid incidentalomas in our cohort underwent an aspiration cytology and/or ultrasound. The rate of malignancy among the PET detected focal thyroid incidentalomas in the cohort of patients with a proven diagnosis was 10.34%. Our study highlights the challenges in the evaluation and management of PAIN in a tertiary care oncology setting. None of the factors studied including nodule size and standardized uptake value predicted the risk of malignancy. Clinicians specializing in the management of thyroid nodules need to understand the clinical significance of the PAIN, and we hope that our unique experience adds to the limited clinical information available in this regard.

Challenges and Special Aspects of Pulmonary Hypertension in Middle- to Low-Income Regions: JACC State-of-the-Art Review.

Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart disease, human immunodeficiency virus, or schistosomiasis are highly prevalent. This consensus statement provides selective, tailored modifications to the current PH guidelines to address the specific challenges faced in MLIRs, resulting in the first pragmatic and cost-effective consensus recommendations for PH care providers, patients, and their families.

Minibands in twisted bilayer graphene probed by magnetic focusing.

Magnetic fields force ballistic electrons injected from a narrow contact to move along skipping orbits and form caustics. This leads to pronounced resistance peaks at nearby voltage probes as electrons are effectively focused inside them, a phenomenon known as magnetic focusing. This can be used not only for the demonstration of ballistic transport but also to study the electronic structure of metals. Here, we use magnetic focusing to probe narrowbands in graphene bilayers twisted at ~2°. Their minibands are found to support long-range ballistic transport limited at low temperatures by intrinsic electron-electron scattering. A voltage bias between the layers causes strong minivalley splitting and allows selective focusing for different minivalleys, which is of interest for using this degree of freedom in frequently discussed valleytronics.

Publisher Correction: Control of electron-electron interaction in graphene by proximity screening.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Common inflammatory markers after cardiac surgery in infants and their relation to blood stream sepsis.

BACKGROUND: Limited information exists on trends of common inflammatory markers after infant heart surgery and their role in identifying post-operative sepsis. METHODS: 275 consecutive infants undergoing cardiac surgery (231 with and 44 without Cardiopulmonary Bypass) were studied prospectively. Daily trends (0-4 day post-operative) of leucocyte counts, platelet counts and C-reactive protein were recorded. Association of these trends with early post-operative bloodstream sepsis, Cardiopulmonary Bypass and surgical outcomes were studied. OBSERVATIONS: Trends of these inflammatory markers were noted. While off-Cardiopulmonary Bypass Surgery, and sepsis were associated with a statisticaly insignificant rise in total leucocyte count peaking on first post-operative day, Cardiopulmonary Bypass exposure was associated with significant decline (p = 0.002), more pronounced with Cardiopulmonary Bypass-exposure exceeding 150 min. Percentage of neutrophils showed a rise (maximum on first post-operative day) but no significant association with sepsis or Cardiopulmonary Bypass.Platelet counts significantly declined after surgery, with nadir on 2nd POD (p < 0.001), the drop being more marked in patients operated on Cardiopulmonary Bypass (p < 0.005). Counts were significantly lower in patients exposed to >150 min Cardiopulmonary Bypass compared to those with shorter Cardiopulmonary Bypass. Septic patients had significantly lower platelet counts than uninfected patients, decline >2 SD from mean pre-operative level strongly associated with sepsis (p < 0.001).C-Reactive Protein levels rose markedly after surgery, peaking on 2nd POD; levels were significantly higher if operated on Cardiopulmonary Bypass. Cardiopulmonary Bypass >150 min was associated with lower mean C-Reactive Protein on first post-operative day, but significantly higher values on third and fourth post-operative days, as compared to Cardiopulmonary Bypass <150 min. Comparison of infected versus non-infected patients showed significantly higher mean C-Reactive Protein in the former group. CONCLUSION: While leucocyte count, platelet count and C-Reactive Protein emerged as useful markers of post-operative inflammatory response and reaction to Cardiopulmonary Bypass, they proved unsatisfactory predictors of early post-operative sepsis.

2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.