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The most startling feature of Modernization of Indian Tradition is that it was so ahead of its times when it appeared in 1973 that most of us missed its full import. In those years there was, in the main, a simplistic understanding of modernisation as its markers were primarily technological achievements and western lifestyles, even affectations. In this book, Professor Yogendra Singh broke away from such formulaic renditions and instead proposed a social and relational view of modernisation where the principal emphasis was on how people interacted with one another and not on prowess facility with superior technical facilities or exterior presentation. Today we can appreciate the relevance of this approach for the drawbacks of correlating modernisation with things has proved to be inadequate, when not misleading, as it gives a skewed appreciation of the subject. Consequently, Enlightenment too gets a fresh coating for it now largely centres around the conditions of knowledge generation, which includes interpersonal relationships, and not on finished products of science. A person may, therefore, be very knowledgeable yet may remain quite un-modern when interacting with others.

Standard of care (SOC) anticoagulation for thromboembolism (TE) prevention in children with cardiac disease includes low molecular weight heparins or vitamin K antagonists. Limited data exists for alternate use of direct oral anticoagulants in children.The investigators aimed to obtain safety and efficacy data for edoxaban in children.We performed a phase 3, multinational, prospective, randomized, open-label, blinded-endpoint trial in patients 18 years of age with cardiac disease (ENNOBLE-ATE [Edoxaban for Prevention of Blood Vessels Being Blocked by Clots (Thrombotic Events) in Children at Risk Because of Cardiac Disease] trial). Patients were randomized 2:1 to age- and weight-based oral edoxaban once daily vs SOC for 3 months (main study period), stratified by cardiac diagnosis. Both groups could continue in an open-label edoxaban extension arm through 1 year. The primary endpoint was adjudicated clinically relevant bleeding (CRB). The main secondary endpoint was symptomatic TE or asymptomatic intracardiac thrombosis.The modified intention-to-treat cohort included 167 children. One patient per group experienced a nonmajor CRB in the main period. Treatment-emergent adverse events occurred in 46.8% (51 of 109) with edoxaban and 41.4% (24 of 58) with SOC. One SOC patient experienced 2 TE events (DVT with PE). Among 147 children in the extension, 1 CRB event (0.7%) and 4 TEs occurred (2.8%; 2 strokes and 2 of 33 Kawasaki disease patients with coronary artery thromboses and/or myocardial infarctions).Edoxaban is a potential alternative mode of thromboprophylaxis in children with cardiac disease showing low rates of CRB and TEs with advantages of once daily dosing and infrequent monitoring requirement. (ENNOBLE-ATE [Edoxaban for Prevention of Blood Vessels Being Blocked by Clots] (Thrombotic Events) in Children at Risk Because of Cardiac Disease trial; NCT03395639).

Background This report describes 9 high-risk neonates and infants with hypoplastic left heart syndrome (HLHS) who were stabilized with a combined hybrid approach and ventricular assist device (VAD) insertion (HYBRID+VAD) in preparation for heart transplantation. Methods A total of 9 patients with HLHS (7 neonates, 2 infants) presented with anatomic or physiologic features associated with an increased risk for conventional univentricular palliation with the Norwood operation (large coronary sinusoids or fistulas, severe tricuspid regurgitation, cardiogenic shock, restrictive atrial septum). These patients underwent combined VAD insertion (Berlin EXCOR, Berlin Heart, Inc, Berlin, Germany) and Stage 1 hybrid palliation (application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed). During this same era, at the Congenital Heart Center, University of Florida, Gainesville, Florida, 46 neonates underwent a Norwood operation, 4 neonates underwent a hybrid approach “Stage 1” without VAD, and 3 patients with HLHS were supported with prostaglandin while awaiting heart transplantation. Results At HYBRID+VAD insertion, the median age was 20 days (range, 13 to 143 days), and median weight was 3.25 kg (range, 2.43 to 4.2 kg). Six patients survive (67%), and 3 patients died (33%). Five survivors are at home doing well after successful heart transplantation, and 1 survivor is doing well in the intensive care unit on VAD support while awaiting transplantation. Only 1 of 6 survivors (16.7%) required intubation more than 10 days after HYBRID+VAD insertion. In 8 patients no longer undergoing VAD support, the median length of VAD support was 119.5 days (range, 56 to 196 days). Conclusions High-risk patients with HLHS who are suboptimal candidates for Norwood palliation can be successfully stabilized with pulsatile VAD insertion along with hybrid palliation while awaiting cardiac transplantation. These patients may be extubated and optimized for transplantation while undergoing VAD support.

Heart transplantation (HT) is the gold standard for managing end-stage heart failure. Multiple quality metrics, including length of stay (LOS), have been used in solid organ transplantation. However, limited data are available regarding trends and factors influencing LOS after pediatric HT. We hypothesized that various donor, peri-transplant and recipient factors affect LOS after pediatric HT.We analyzed patients18years at time of HT from January 2005 to December 2018 in the Pediatric Heart Transplant Society database, and examined LOS trends, defined prolonged LOS (PLOS = LOS30days after HT), identified factors associated with PLOS and assessed outcomes.Of 4827 patients undergoing HT, 4414 patients were discharged and included for analysis. Overall median LOS was 19days[13,34]. Median LOS was longer in patients with congenital heart disease(CHD = 25days[15,43] than with cardiomyopathy(CM = 17days[12,27] across all ages. Median LOS in age1year was 26-days[16,45.5] and in age10year was 16days[11,26]. PLOS was seen in 1313 patients(30%). Patients with PLOS were younger, smaller and had longer CPB times. There was no difference in utilization of VAD at HT between groups, however, ECMO use at listing(8.45% vs 2.93%,p0.05) and HT was higher in the PLOS group(9.22% vs 1.58%,p0.05). PLOS was more common in patients with previous surgery, CHD, single ventricle physiology, recipient history of cardiac arrest or CPR, end organ dysfunction, lower GFR, use of mechanical ventilation at HT and Status 1A at HT.We present novel findings of LOS distribution and define PLOS after pediatric HT, providing a quality metric for individual programs to utilize and study in their practice.

Objective: The objective of this study was to nd the prevalence of thyroid dysfunction in transfusion-dependent thalassemia (TDT) and nontransfusion dependent thalassemia (NTDT) children, and to nd the correlation between serum ferritin levels with thyroid functions. Methods: This cross-sectional study was conducted in the department of Pediatrics and Thalassemia clinic of a tertiary care teaching institute in Kolkata, India. A total of 63 children were included in our study who satised the clinical and diagnostic criteria of TDT and NTDT and fullled the inclusion-exclusion criteria. Laboratory parameters studied were hemoglobin (Hb) %, thyroid-stimulating hormone (TSH), free thyroxin (FT4), and serum ferritin. Results: In our study, 32 (50.8%) were of TDT, and 31(49.2%) were NTDT. E-β thalassemia were detected in 60.3% (n=38) of thalassemic children, 81.58% (n=31) of them behaved as NTDTand 18.42% (n=7) as TDT. Thyroid dysfunction were detected in 28.57% (n=18) of total cases. Most of these, 25.40% (n=16) as subclinical hypothyroidism, and 3.17% (n=2) as overt hypothyroidism. Among TDT patients with mean age of 81.00 (± 33.0) months had subclinical thyroid dysfunction in 37.5% (n=12) . Among NTDTs with mean age of 85.62 (± 27.75) months had subclinical hypothyroidism in 12.9% (n=4). Overt hypothyroidism were detected in 1 (one) of each TDT and NTDT. Signicant correlation with the serum ferritin level and thyroid dysfunction could not be established. Conclusion: Thalassemic children should undergo screening of thyroid functions to detect subclinical hypothyroidism from school going age in addition to routine monitoring of serum ferritin level.

BACKGROUND Prediction of post-transplant health outcomes for pediatric heart transplantation is critical for risk stratification and high-quality post-transplant care. OBJECTIVE The purpose of the current study is to examine the use of machine learning models to predict rejection and mortality for pediatric heart transplant recipients. METHODS Various ML models were used to predict rejection and mortality at 1-, 3-, and 5-years post-transplant in pediatric heart transplant recipients using the United Network for Organ Sharing data from 1987-2019. Variables utilized for predicting post-transplant outcomes included donor and recipient, medical and social factors. SHAP (SHapley Additive exPlanations) were calculated to estimate the importance of each variable for prediction. RESULTS Random forest and adaptive boosting (AdaBoost) models were the best performing algorithms for different prediction windows across outcomes. Random forest outperformed other ML algorithms in predicting five of the six outcomes (AUROC: 0.664 and 0.706 for 1-year and 3-year rejection, 0.697, 0.758 and 0.763 for 1-year, 3-year and 5-year mortality). AdaBoost achieved the best performance for prediction of 5-year rejection with AUROC 0.705. CONCLUSIONS The current study demonstrates the comparative utility of ML approaches for modeling post-transplant health outcomes using registry data. ML approaches can identify unique risk factors and their complex relationship with outcomes, thereby identifying at-risk patients and informing the transplant community about the future potential of these innovative approaches to improve pediatric post-heart transplant care. Future studies are required to translate the information derived from prediction models to optimize counseling, clinical care, and decision-making within pediatric organ transplant centers.

Introduction: Inflammatory demyelinating disease like combined central and peripheral demyelination (CCPD) could have varied clinical presentation depending upon the topographical distribution of neural involvement. Case presentation: A seven-year-old child had presented with fever followed by acute onset flaccid paralysis and urinary retention. Weakness in the lower limbs as reported was ascending and symmetric in nature, while no history of trauma, band-like sensation or altered sensorium were documented. Superficial and deep tendon reflexes of both the lower limbs were absent. Routine blood investigations had revealed neutrophilic leucocytosis only. Serum IgM antibody for scrub typhus was found positive. CSF study didn’t show cyto-protein dissociation. NCV had demonstrated absence of F wave and H reflex in the peripheral nerves of lower limbs. Anti-ganglioside antibody profiles were negative. Subsequent investigations including MRI brain and spinal cord had revealed acute onset CCPD. Conclusion: Acute onset combined central and peripheral demyelination in a child had presented as acute flaccid paralysis of the lower limbs and the condition was temporally association with scrub typhus.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu’s arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu’s arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu’s arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu’s arteritis within the paediatric community.

COVID-19 pandemic continues to evolve and new variants like Delta and Omicron have been discovered. REGEN-COV is a recombinant human monoclonal antibody to the spike protein of SARS-CoV-2 which received emergency use authorisation for treatment and post-exposure prophylaxis in patients with high risk of progression to severe disease. We review our experience with use of REGEN-COV in paediatric heart transplant patients.

The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted.

Background: Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. Methods: We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Results: Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

We honour a great man and a true giant. Lodewyk H.S. van Mierop (March 31, 1927 – October 17, 2021), known as Bob, was not only a Paediatric Cardiologist but also a dedicated Scientist. He made many significant and ground-breaking contributions to the fields of cardiac anatomy and embryology. He was devoted as a teacher, spending many hours with medical students, Residents, and Fellows, all of whom appreciated his regularly scheduled educational sessions. Those of us who were fortunate to know and spend time with him will always remember his great mind, his willingness to share his knowledge, and his ability to encourage spirited and fruitful discussions. His life was most productive, and he will long be remembered by many through his awesome and exemplary scientific contributions.His legacy continues to influence the current and future generations of surgeons and all providers of paediatric and congenital cardiac care through the invaluable archive he established at University of Florida in Gainesville: The University of Florida van Mierop Heart Archive. Undoubtedly, with these extraordinary contributions to the fields of cardiac anatomy and embryology, which were way ahead of his time, Professor van Mierop was a true giant in Paediatric Cardiology. The invaluable archive he established at University of Florida in Gainesville, The University of Florida van Mierop Heart Archive, has been instrumental in teaching medical students, Residents, Medical Fellows, and Surgical Fellows. Only a handful of similar archives exist across the globe, and these archives are the true legacy of giants such as Dr. van Mierop. We have an important obligation to leave no stone unturned to continue to preserve these archives for the future generations of surgeons, physicians, all providers of paediatric and congenital cardiac care, and, most importantly, our patients.

Background Prediction of post-transplant health outcomes for pediatric heart transplantation is critical for high quality post-transplant care. The purpose of the current study is to examine the use of machine learning models to predict late acute rejection, hospitalizations, and mortality for pediatric heart transplant recipients. Methods Various traditional machine learning and deep learning models were used to predict late acute rejection, hospitalizations, and mortality at 1-, 3-, and 5-years post-transplant in pediatric heart transplant recipients using the national United Network for Organ Sharing data. Variables predicting post-transplant outcomes included donor and recipient medical and social predictors. SHAP (SHapley Additive exPlanations) were calculated to estimate the importance of each variable for prediction and to increase the interpretability of modeling results. Results Logistic regression, random forest, and adaptive boosting models were the best performing algorithms for different prediction windows across outcomes. Based on the area under the receiver operating curve, models predicting rejection and mortality were found to be more accurate than those predicting hospitalizations. Conclusions Machine learning approaches can identify unique risk factors, their complex relationship with outcomes using prediction modeling, thereby aiding in identifying at-risk patients, providing a solid foundation for improved clinical care, risk stratification, and guiding decision-making within pediatric organ transplant centers. The current study demonstrates the value of machine learning approaches for modeling post-transplant health outcomes using patient-level data and informs the transplant community about the future potential of these innovative approaches to improve pediatric post-heart transplant care.

HT recipients experience high levels of medication non-adherence during adolescence. This pilot study examined the acceptability and feasibility of an asynchronous DOT mHealth application among adolescent HT recipients. The app facilitates tracking of patients' dose-by-dose adherence and enables transplant team members to engage patients. The DOT application allows patients to self-record videos while taking their medication and submit for review. Transplant staff review the videos and communicate with patients to engage and encourage medication adherence.Ten adolescent HT recipients with poor adherence were enrolled into a single-group, 12-week pilot study examining the impact of DOT on adherence. Secondary outcomes included self-report measures from patients and parents concerning HRQOL and adherence barriers. Long-term health outcomes assessed included AR and hospitalization 6 months following DOT.Among 14 adolescent HT patients approached, 10 initiated the DOT intervention. Of these, 8 completed the 12-week intervention. Patients and caregivers reported high perceptions of acceptability and accessibility. Patients submitted 90.1% of possible videos demonstrating medication doses taken. MLVI values for the 10 patients initiating DOT decreased from 6 months prior to the intervention (2.86 ± 1.83) to 6 months following their involvement (2.08 ± 0.87) representing a 21.7% decrease in non-adherence, though not statistically significant given the small sample size.Result of this pilot study provides promising insights regarding the feasibility, acceptability, and potential impact of DOT for adolescent HT recipients. Further randomized studies are required to confirm these observations.

Medication non-adherence causes poor outcomes in paediatric organ transplantation. COVID-19 pandemic has led to an exponential use of mobile health approaches for patient care. Herein, we describe a pilot intervention study using mobile video directly observed therapy building on emerging trends in research and clinical practice pertaining to medication adherence in paediatric organ transplantation.

We reviewed our management strategy and outcome data for all 179 patients with pediatric and/or congenital heart disease who underwent 183 heart transplants from 01/01/2011-12/31/2021 and evaluated the impact of elevated PRA.High PRA was defined as PRA10%. Univariate associations with long-term survival were assessed with Cox proportional hazards models. Impact of high PRA on survival was estimated with multivariable models.PRA10% was present in 60/183=32.8% transplants, who were more likely to have prior cardiac surgery, higher number of prior cardiac surgeries, prior sternotomy, prior heart transplant, and positive crossmatch (24/60=40.0% versus 11/123=8.9%), P0.0001. Univariate associations with long-term survival include: acquired heart disease (versus congenital or re-transplant): HR=0.18(.053-.593),P=0.005; prior cardiac surgery: HR=5.6(1.32-23.75),P=0.020; number of prior cardiac surgeries: HR=1.3 for each additional surgery (1.12-1.50),P=0.0004; single ventricle: HR=2.4(1.05-5.48),P=0.038; and preoperative renal dysfunction: HR=3.4(1.43-7.49),P=0.002. In multivariate analysis, high PRA does not impact survival when controlling for each of the factors shown in univariable analysis to be associated with long-term survival. Kaplan-Meier survival estimates-One-year(95% Confidence Intervals);Five-year(95% Confidence Intervals): All patients=93.6%(89.9%-97.3%);85.8%(80.0%-92.1%). PRA10%=96.6%(93.4%-99.9%);86.7%(79.6%-94.3%). PRA10%=86.7%(78.0%-96.4%); 83.8%(74.0%-95.0%). Despite high PRA being associated with higher mortality at one year (14.9% versus 3.8%,P=0.035), no significant difference exists in Kaplan-Meier overall survival at five years post-transplant in patients with and without high PRA (log-rank P=0.4).In our cohort, five-year survival in patients with high PRA (PRA10%) is similar to patients without high PRA (PRA10%), despite the presence of more risk factors in those with high PRA. Individualized immunomodulatory strategies can potentially mitigate the risk of high PRA.

ObjectiveWe explore shared decision making (DM) in guardians of children with heart disease by assessing the desired weight of influence on DM and factors that may alter the relative weight of parent or medical team influence.MethodsGuardians of patients 1 week in the paediatric cardiac intensive care unit (PCICU) were recruited. Twelve vignettes were designed including technical (antibiotic selection, intubation, peripherally inserted central catheter placement, ventricular assist device placement, heart transplant, organ rejection, heart rhythm abnormalities and resuscitation effort) and non-technical vignettes (cessation of life-sustaining therapies, depression treatment, obesity and palliative care referral). Participants responded to questions on DM characteristics and one question querying preference for relative weight of parent or medical team influence on DM.ResultsOf 209 participants approached, 183 were included. Most responded with equal desire of medical team and parental influence on DM in all vignettes (range 41.0%–66.7%). Technical scenarios formed one cluster based on DM characteristics, compared with non-technical scenarios. Factors that increase the relative weight of parental influence on DM include desired input and involvement in big-picture goals (OR 0.274, CI [0.217 to 0.346]; OR 0.794, CI [0.640 to 0.986]). Factors that increase the relative weight of medical team influence on DM include perception of medical expertise needed (OR 1.949 [1.630 to 2.330]), urgency (OR 1.373 [1.138 to 1.658]), benefit (OR 1.415 [1.172 to 1.710]), number of PCICU admissions (OR 1.134 [1.024 to 1.256]) and private insurance (OR 1.921 [1.144 to 3.226]).ConclusionAlthough factors may alter the weight of influence on DM, most parents desire equal parental and medical team influence on DM.

We report 15 high-risk neonates and infants with functionally univentricular circulation stabilized with initial surgical palliation plus ventricular assist device (VAD) insertion (PALLIATION+VAD) in preparation for transplantation.Fifteen functionally univentricular patients with ductal-dependent systemic circulation (8 hypoplastic left heart syndrome, 1 hypoplastic left heart syndrome-related malformation: 7 neonates, 2 infants) or ductal-dependent pulmonary circulation (6 hypoplastic right heart syndrome: 5 neonates, 1 infant) presented with anatomical and/or physiological features associated with increased risk for conventional univentricular palliation (large coronary sinusoids with ventricular-dependent coronary circulation, severe systemic atrioventricular valvar regurgitation, cardiogenic shock, or restrictive atrial septum). PALLIATION+VAD for patients with ductal-dependent systemic circulation was: VAD insertion plus application of bilateral pulmonary bands, stent placement in the arterial duct, and atrial septectomy, if needed. PALLIATION+VAD for patients with ductal-dependent pulmonary circulation was: VAD insertion plus stent placement in the arterial duct or systemic-to-pulmonary artery shunt with pulmonary arterioplasty, if needed.At PALLIATION+VAD, median age was 20 days (range, 4-143 days) and median weight was 3.47 kg (range, 2.43-4.86 kg). Ten patients (67%) survived and 5 patients (33%) died. All ten survivors are at home doing well after successful transplantation. Only 2 of 10 survivors (20%) required intubation10 days after PALLIATION+VAD. Median length of VAD support for all 15 patients was 138 days (range, 56-226 days).High-risk neonates with functionally univentricular hearts who are suboptimal candidates for conventional palliation can be successfully stabilized with pulsatile VAD insertion along with initial palliation while awaiting cardiac transplantation; these patients may be extubated, enterally nourished, and optimized for transplantation while on VAD.

In a cross-sectional study, we assessed family functioning prior to outpatient visits and routine heart catheterisations in the families of paediatric heart transplant recipients. Caregivers rated higher short-term family functioning prior to outpatient visits, but not prior to catheterisations. This finding may indicate that family functioning benefits from the support provided during outpatient visits.

To assess the phenotypic variations found among hearts diagnosed at autopsy with hypoplastic left heart syndrome, with attention to implications related to this syndrome as an acquired disease of fetal life, rather than being the consequence of abnormal embryogenesis. We assessed 119 specimens, from 2 archives, diagnosed initially as representing hypoplastic left heart syndrome. Among the 119 specimens, the majority of which had been entered into the archives prior to the availability of surgical treatment for the syndrome, 36 (30%) had the combination of mitral and aortic atresia, 26 (22%) had mitral and aortic stenosis, and 57 (48%) had mitral stenosis combined with aortic atresia. Of the hearts with combined atresia, 92% (33 specimens) had slit-like left ventricles, compared to 12% (3 specimens) of hearts with stenosis of both aortic and mitral valves, and 2 hearts (4%) with mitral stenosis and aortic atresia (P0.001). Hypoplasia of the left atrial appendage was present in half (18 specimens, 51%) of those with combined atresia, as opposed to just 18% (10 specimens) of mitral stenosis combined with aortic atresia (P = 0.001). Small left ventricles with valves deemed proportional in size were found in 11 (42%) of those with combined mitral and aortic stenosis. Fibroelastosis was significantly more common in the hearts with mitral stenosis compared to those with mitral atresia (76% vs 11%, P0.001). The ascending aorta was significantly smaller in the hearts with aortic atresia. The variability in the morphologic findings support the notion that the lesions seen represent acquired disease occurring subsequent to closure of the embryonic interventricular communication, rather than representing abnormal embryogenesis.

Palliation of patients with hypoplastic left heart syndrome remains challenging. Although coronary ischemia can be catastrophic, the prevalence and pathologies of anomalies of the coronary arteries remains unknown. We reviewed 119 specimens with the features of hypoplastic left heart syndrome, focusing our attention on the aortic root and the coronary arteries. We found 36 (30%) specimens with the combination of mitral and aortic atresia, 26 (22%) with mitral and aortic stenosis, and 57 (48%) with mitral stenosis combined with aortic atresia. In 29 specimens (24%), the coronary arteries were not located in the center of any sinuses, while in 24 specimens (21%) at least 1 coronary artery was located very proximal to a raphe or commissure, with potential for obstruction. The specimens with combined stenosis were more likely to have eccentric positions of the coronary arteries (11 specimens, 42%), compared to the 3 specimens with combined atresia (9%, P = 0.009). The specimens with combined stenosis were also more likely to have positioning at risk for obstruction (9 specimens, 35%), compared to those with combined atresia (3 specimens, 9%, P = 0.05). Coronary arterial fistulous communications were found in 11 specimens (9%), significantly more frequently in specimens with mitral stenosis and aortic atresia (9 specimens, 16%, P = 0.041). The origins of the coronary arteries in patients with hypoplastic left heart syndrome place them at potential risk for ischemia, with fistulous communications being a particular risk in those with mitral stenosis combined with aortic atresia.

Marx has been misread primarily because the politicians who, in his name, powered communist regimes popularized a tendentious interpretation of his works. In particular, they justified authoritarianism and violence by emphasizing the “dictatorship of the proletariat” and the “animal theory of revolution” where the poor get poorer and eventually erupt in a cataclysmic fashion. Instead, if attention had been paid to Marx’s seminal concept—“socially necessary labor”—and his exhortation to win the minds of the working classes by participating in popular movements of the subalterns everywhere, then a new appreciation would emerge of the corpus of Marx’s contributions. As that has not quite happened, scholars like Weber, Arendt, and Friedman have misinterpreted Marx, rather willfully, and shot into prominence with their first book-length publications.