Pediatric Cardiology

Background: Heterotaxy syndrome (HS) results from failure of the developing embryo to establish normal leftright asymmetry of thoraco-abdominal organs resulting to complex congenital heart disease, pulmonary anomalies, asplenia or polysplenia syndromes with various complications including recurrent infections. It has an estimated incidence of 1 in 6,000 to 1 in 20,000 live births with high mortality of up to 85% in infancy. Objective: To highlight the management challenges of a child with Heterotaxy syndrome (right isomerism and asplenia) seen at

Introduction: Pericardial effusion (PE) is an abnormal accumulation of fluid in the pericardial space, which, if severe, is associated with high mortality. The causes are diverse, including infective and non-infective. Few studies have looked at the spectrum of severity and causes in Northern Nigeria. The aim was to determine the spectrum of causes and severity of pericardial effusion, as well as the outcome among children seen at the

Sir, Charcot-Marie-Tooth disease (CMT) due to ganglioside-induced differentiation-associated protein 1 (GDAP1) mutations is rare. The GDAP1 expression in the Schwann cells and in the outer mitochondrial membrane is responsible for the complex interplay whereby it can cause axonal as well as demyelinating neuropathy. Although GDAP1 is also expressed in the brain, no clinical involvement has been documented so far in any patient. The purpose of this letter is to report an Indian family with a novel GDAP1 mutation which also appears to be the first report in literature.

Follow-up studies on patients operated on for congenital heart defects have shown good anatomical results and long-term survival. To date, there have been few studies on such patients with regard to long-term psychosocial outcome and quality of life. In this study, two cohorts of patients operated on before the age of 15 years, one for tetralogy of Fallot (TOF) and the other for atrial septal defect (ASD), were investigated 20 and 30 years after operation regarding quality of life. The combined cohort had a higher educational level than average. There was no connection between quality of life and physical health as judged from the New York Heart Association classification. The TOF group rated their quality of life higher than the ASD group, but both groups had lower figures at the 30-year than at the 20-year follow-up. Fewer patients in the TOF than in the ASD group considered that their lives were affected by the heart disease. It is concluded that the severity of the heart disease is not necessarily congruent with estimated quality of live and that mild heart defects, such as ASD, can have a considerable impact on later life quality. Surprisingly few TOF patients were affected negatively, a finding which might reflect development of a specific coping strategy in these patients during childhood.

ABSTRACT
BACKGROUND: Congenital heart disease surgery is related to
significant postoperative complications which have been associated
with poor patient outcomes. However, the prevalence, predictors,
and effect of complications on outcomes have only been evaluated
in a few numbers of studies. This study aimed to assess the magnitude of postoperative complications after congenital heart surgery,
their predictors, and the associations between complications and
patient outcomes.
METHODS: A retrospective, single-center cross-sectional study
was done at the Cardiac Center of Ethiopia (CCE) on children who
had undergone cardiac surgery between 2009 and 2022. All 919
pediatric patients aged below 18 years who had undergone congenital heart surgery were included in the study.
RESULT: Of the 919 patients who underwent surgery in the cardiac center of Ethiopia, Ventricular septal defect( VSD) was the most
common diagnosis (41%) and VSD patch closure (39.4%) was the
most common surgical procedure. The presence of at least one
complication was identified in 39.5% of patients. Of all patients,
11.3% had cardiac, 23.1% had extra-cardiac complications, and
5.3%% had major complications. Higher age at diagnosis, lower
weight at admission, cyanotic congenital heart disease, higher Risk
adjustment for congenital heart surgery score (RACH-1 score),
higher vasoactive inotropic score, Cardiopulmonary bypass use
during surgery, higher cardiopulmonary bypass time, higher aortic
cross-clamp time, higher duration of surgery, and the higher number of surgeries were associated with complications. The duration
of mechanical ventilation, length of intensive care unit stays, and
length of hospital stay was significantly prolonged in patients with
complications.
CONCLUSION: Congenital heart disease surgeries pose a high
risk of complications, and these complications are associated with
poor patient outcomes in Ethiopia. Therefore, predicting complications based on risk factors and early detection and treatment is crucial to improve the patient's outcome.

Background This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD). Methods A total of 49 consecutive children with 22q11.2 and CHD were retrospectively identified. The CHD consisted of tetralogy of Fallot and variances (n = 22), interrupted aortic arch (n = 10), ventricular septal defect (n = 8), truncus arteriosus (n = 6), and double aortic arch (n = 1). Extracardiac anomalies were present in 46 of 47 children. Results The median follow-up time was 8.5 years (range, 3 months to 23.5 years). Cardiac surgical repair was performed for 35 children, whereas 5 had palliative surgery, and 9 never underwent cardiac surgery. The median age at repair was 7.5 months (range, 2 days to 5 years). The mean hospital stay was 35 days (range, 7-204 days), and the intensive care unit stay was 15 days (range, 3-194 days). Significant postoperative complications occurred for 26 children (74%), and surgery for extracardiac malformations was required for 21 patients (43%). The overall mortality rate was 22% (11/49), with 1-year survival for 86% and 5-year survival for 80% of the patients. A total of 27 cardiac reinterventions were performed for 16 patients (46%) including 15 reoperations and 12 interventional catheterizations. Residual cardiac findings were present in 25 patients (71%) at the end of the follow-up period. Conclusions Children with microdeletion 22q11.2 and CHD are at high risk for mortality and morbidity, as determined by both the severity of the cardiac lesions and the extracardiac anomalies associated with the microdeletion.

Background Pediatric patients with moderate and great complexity congenital heart disease (CHD) may benefit from coordinated transfer to adult congenital heart disease (ACHD) centers to reduce the risk of complications; however, there are a variety of transfer practices. We examined the impact of referral order placement at the last pediatric cardiology visit on time to transfer to an ACHD center. Methods We analyzed data collected from pediatric patients with moderate and great complexity CHD who were eligible to transfer to our tertiary center’s accredited ACHD center. We examined transfer outcomes and time-to-transfer between those with a referral order placed at the last pediatric cardiology visit and those without using Cox proportional hazards modeling. Results The sample (n = 65) was 44.6% female and mean age at study start was 19.5 years (± 2.2). Referral orders were placed for 32.3% of patients at the last pediatric cardiology visit. Those who had a referral order placed at...

Background Dextro-transposition of the great arteries with intact ventricular septum (d-TGA/IVS) requires early arterial switch operation (ASO) to preserve left ventricular function, but delayed presentations complicate outcomes due to LV regression and hypoxemia. Alternative bridging strategies are essential for late-presenting patients to improve surgical feasibility. Case report We present a six-month-old male patient with dextrocardia, situs inversus, d-TGA/IVS who experienced persistent cyanosis despite prior balloon atrial septostomy (BAS). On admission, the patient exhibited severe hypoxemia (SpO₂ 33%), metabolic acidosis, and LV regression (LV mass index: 36-41 g/m2). Echocardiography confirmed a restrictive atrial septal defect (3.5 mm) and the absence of a patent ductus arteriosus (PDA). Given the prohibitive risk of immediate ASO, an emergency transcatheter intervention was performed. PDA recanalization was attempted. Following successful wire passage, balloon angioplasty and stent deployment restored systemic-to-pulmonary shunting, improving oxygen saturation to 56%. To further augment intercirculatory mixing, a 10.0 mm × 29 mm Omnilink Elite stent was implanted across the interatrial septum, increasing oxygen saturation to 85%. The patient demonstrated stable post-procedural hemodynamics and was subsequently bridged to elective ASO, which was performed successfully after two months. Conclusion Transcatheter PDA recanalization and interatrial septal stenting represent a viable bridge to ASO in latepresenting d-TGA/IVS patients. This minimally invasive approach expands treatment options in resource-limited settings where early surgical intervention is not always feasible.

Transcatheter closure of arterial duct (AD) remains challenging in low-weight patients and using Amplatzer Duct Occluder devices is still considered off-label in infants < 6 kg. This study aimed to report a large, single-center experience of percutaneous AD closure in infants < 6 kg as well as to compare the most frequently used devices, Amplatzer Duct Occluder type I (ADO I) and Amplatzer Duct Occluder type II Additional Sizes devices (ADO II-AS) (St. Jude Medical Corp, St. Paul, MN, USA). From March 2000 to March 2017, among the 762 patients submitted to percutaneous closure of AD at our Institution, 33 were infants < 6 kg (age 4.8 ± 2.1 months; weight 5.0 ± 0.9 kg). Fourteen patients (45%) underwent ADO I (Group I) and 19 patients ADO II-AS (Group II) device implantation. AD diameter was 2.6 ± 0.8 (range 1.5-4.0) mm resulting in QP/QS of 2.6 ± 0.0.9 (range 1.1-4.5). Successful device deployment was achieved in all patients without procedural morbidity or mortality. Proce...

Significant and balanced PA growth following arterial duct (AD) stenting has already been consistently reported in literature. However, to date, no data are available about the role of this approach as palliation of congenital heart disease with a duct-dependent discontinuous pulmonary artery (dPA). The aim of this study was to evaluate the fate of a dPA of ductal origin following trans-catheter AD stabilization. Angiographic PA evaluation was performed in seven patients submitted to neonatal AD stenting as palliative recruitment of dPA. Five patients showed discontinuity of one PA, while two patients had both PAs served by bilateral ducts. PA growth was evaluated as per the Nakata index, McGoon ratio as well as dPA (n = 9) versus heart-dependent PA (hPA; n = 5) size and z-score changes. AD stabilization was performed using coronary stents dilated to 3.2 ± 0.3 mm (median 3.4), with significant increase of O2 saturation (from 83 ± 11 to 95 ± 5%, p < 0.02). Control angiography was ...

OBJECTIVES: Hybrid palliation for hypoplastic left heart syndrome (HLHS) is associated with mortality and late ventricular dysfunction. Increased ventricular workload and coronary perfusion limitation may be the important factors. Using mathematical modelling, this study investigated the effects of differing hybrid configurations on the demands on this single ventricle circulation. A multicompartmental Windkessel model of hybrid HLH-aortic atresia circulation was adopted, with a time-varying elastance representing ventricular functionality. The effects of diameter increases in bilateral pulmonary artery bandings (PABs) (+0.5, 2.5-4 mm) and ductal stent (+1, 4-10 mm) on cardiovascular haemodynamics, systemic oxygenation and ventricular energetics were assessed. RESULTS: Simulations showed that an increase in PAB diameter of 2.5-4 mm resulted in an increased Q (0.61-2.66), and diastolic stent backflow (-0.2 to -0.78 l/min) with reduced systemic perfusion (0.82-0.77 l/min) and diastolic pressures (48.3-41.2 mmHg). Arterial and venous saturations increased, SaO 2 (%) was 62-88 and SvO 2 41-65. To maintain mean systemic pressures, substantial increases in cardiac output (1.3-2.8 l/min) and ventricular stroke work (576-1360 mmHg ml) were required. A decrease in the ductal stent diameter over the range 10-7 mm had a negligible haemodynamic effect: reduced systemic systolic pressure (77-72 mmHg) and increase in ventricular stroke work (781-790 mmHg ml). When the ductal diameter was restricted to <7 mm, it resulted in a significant reduced systemic flow and increased stroke work. Optimal hybrid configuration was defined at PAB 3 mm and ductal stent ≥7 mm. In this model, increasing the PAB diameter, or a stent diameter <7 mm, substantially increased single ventricle workload and reduced systemic perfusion and diastolic pressure. This may compromise myocardial oxygen demand-supply, particularly in the setting of retrograde-dependent coronary perfusion.

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, for which patients require surgical intervention at a very young age. Although these patients have long survival rates, they are at risk of chronic complications and frequently require re-operations with the most common being pulmonary valve replacement (PVR). However, the decision for surgical intervention is currently based on clinical indications and the right timing for PVR remains ambiguous [1,2]. The overall objective of this work is to identify a computational metric that will help assess the right timing for surgical intervention in adults with repaired tetralogy of Fallot. This current study concerns a preliminary computational analysis of blood flow in simplified geometries of the pulmonary bifurcation. The focus lies on the effect of geometric and haemodynamic parameters on the wall shear stress patterns around the pulmonary bifurcation.

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Background: Mechanical ventilation is extensively used in neonatal intensive care units. However, many risk factors are associated with mortality rates in neonates on mechanical ventilation. Various techniques have been introduced to assess the weaning time from mechanical ventilation with least risks including diaphragmatic ultrasound. This study aimed at evaluating the role of diaphragmatic ultrasound in predicting failure of weaning from mechanical ventilation in both Full Term (FT) and Preterm (PT) neonates by measuring diaphragmatic excursion before extubation using and determines a cut off value for diaphragmatic excursion for expecting weaning failure. Patients and Methods: A prospective cohort study was performed in the neonatal intensive care unit (NICU) of Mataria Teaching Hospital (MTH) from June 2018 to May 2019. All full and pre-term neonates who need mechanical ventilation from first day of admission were included major congenital anomalies, need surgical intervention, congenital diaphragmatic hernia, weaned before 72 hours, pleural effusion, hepato-splenomegaly were excluded. Results: Neonates with low gestational age, presence of respiratory distress, longer period on mechanical ventilation and higher ventilator setting were more prone to weaning failure. Additionally, excellent sensitivity and specificity of diaphragmatic ultrasound in prediction of weaning failure in neonates especially in full term whether using excursion of right and left hemidiaphragm. Conclusion: Bedside ultrasound can predict weaning failure through measuring right or left diaphragmatic excursion in full term and preterm neonates.

Background: Mechanical ventilation is extensively used in neonatal intensive care units. However, many risk factors are associated with mortality rates in neonates on mechanical ventilation. Various techniques have been introduced to assess the weaning time from mechanical ventilation with least risks including diaphragmatic ultrasound. This study aimed at evaluating the role of diaphragmatic ultrasound in predicting failure of weaning from mechanical ventilation in both Full Term (FT) and Preterm (PT) neonates by measuring diaphragmatic excursion before extubation using and determines a cut off value for diaphragmatic excursion for expecting weaning failure. Patients and Methods: A prospective cohort study was performed in the neonatal intensive care unit (NICU) of Mataria Teaching Hospital (MTH) from June 2018 to May 2019. All full and pre-term neonates who need mechanical ventilation from first day of admission were included major congenital anomalies, need surgical intervention, congenital diaphragmatic hernia, weaned before 72 hours, pleural effusion, hepato-splenomegaly were excluded. Results: Neonates with low gestational age, presence of respiratory distress, longer period on mechanical ventilation and higher ventilator setting were more prone to weaning failure. Additionally, excellent sensitivity and specificity of diaphragmatic ultrasound in prediction of weaning failure in neonates especially in full term whether using excursion of right and left hemidiaphragm. Conclusion: Bedside ultrasound can predict weaning failure through measuring right or left diaphragmatic excursion in full term and preterm neonates.

Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multiinstitutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status.

OBJECTIVES: A second paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. Participating hospitals contributed pre-, peri-and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (< _19 years of age) performed from 1 January 2000 to 1 July 2019 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS: Twenty-nine hospitals contributed 398 registered implants in 353 patients (150 female, 203 male) to the registry. The most frequent aetiology of heart failure was any form of cardiomyopathy (61%), followed by congenital heart disease and myocarditis (16.4% and 16.1%, respectively). Competing outcomes analysis revealed that a total of 80% survived to transplant or recovery or are ongoing; at the 2year follow-up examination, 20% died while on support. At 12 months, 46.7% received transplants, 8.7% were weaned from their device and 18.5% died. The 3-month adverse events rate was 1.69 per patient-year for device malfunction including pump exchange, 0.48 for major bleeding, 0.64 for major infection and 0.78 for neurological events. The overall survival rate was 81.5% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age) (P = 0.01) and lower weight (<20 kg) (P = 0.015). Transplant rates at 6 months continue to be low (33.2%) The fact that the EUROMACS registry is embedded within the European Association for Cardio-Thoracic Surgery Quality Improvement Programme offers opportunities to focus on improving outcomes.

The complexity of ventricular septal defects in early infancy led to development of new mini-invasive techniques based on collaboration of cardiac surgeons with interventional cardiologists, called hybrid procedures. Hybrid therapies aim to combine the advantages of surgical and interventional techniques in an effort to reduce the invasiveness. The aim of this study was to present our approach with mVSD patients and initial results in the development of a mini-invasive hybrid procedure in the Gdansk Hybrid Heartlink Programme (GHHP) at the

Wstęp: W rzetelnej ocenie wyników leczenia należy brać pod uwagę różnorodność leczonej populacji pacjentów. Bez uwzględnienia tych danych analizowanie, a tym bardziej porównywanie, wyników jest obarczone dużym, nieakceptowalnym błędem. Do analizy śmiertelności po leczeniu wad wrodzonych serca w okresie 16 lat zastosowaliśmy cztery narzędzia do stratyfikacji ich złożoności: Aristotle Basic Complexity Score (ABC Score), Risk Adjustment for Congenital Heart Surgery-1 Categories (RACHS-1 Categories),

Objective: The analysis of the second harvest of the STS Congenital Heart Surgery Database produced meaningful outcome data and several critical lessons relevant to congenital heart surgery outcomes analysis worldwide. Methods: This data harvest represents the first STS multi-institutional experience with software utilizing the nomenclature and database requirements adopted by the STS and EACTS (April 2000 Annals of Thoracic Surgery). Members of the STS Congenital Heart Committee analyzed the STS data. Results: This STS harvest includes data from 16 centers (12787 cases, 2881 neonates, 4124 infants). In 2002, the EACTS reported similar outcome data utilizing the same database definitions (41 centers, 12736 cases, 2245 neonates, 4195 infants). Lessons from the analysis include: (1) Death must be clearly defined. (2) The Primary Procedure in a given operation must be documented. (3) Inclusionary and exclusionary criteria for all diagnoses and procedures must be agreed upon. (4) Missing data values remain an issue for the database. (5) Generic terms in the nomenclature lists, that is terms ending in Not Otherwise Specified (NOS), are redundant and decrease the clarity of data analysis. ( ) Methodology needs to be developed and implemented to assure and verify data completeness and data accuracy. 'Operative Mortality' and 'Mortality Assigned to this Operation' were defined by the STS and EACTS; these definitions were not utilized uniformly. 'Thirty Day Mortality' was problematic because some centers did not track mortality after hospital discharge. Only 'Mortality Prior to Discharge' was consistently reported. Designation of Primary Procedure for a given operation determines its location for analysis. Until Complexity Scores lead to automated methodology for choosing the Primary Procedure, the surgeon must designate the Primary Procedure. Inclusionary and exclusionary criteria for all diagnoses and procedures have been developed in an effort to define acceptable concomitant diagnoses and procedures for each analysis. Improvements in data completeness can be achieved using a variety of techniques including developing more functional techniques of data entry at individual institutions and software improvements. Future versions of the STS Congenital Database

Kawasaki disease (KD) is an acute, self-limited vasculitis of childhood with a unique predilection for the coronary arteries, representing the leading cause of acquired heart disease in children in developed countries. The condition’s clinical significance lies in its potential to cause coronary artery aneurysms (CAAs), myocardial infarction, and sudden death if not promptly recognized and treated. Intravenous immunoglobulin (IVIG) combined with aspirin remains the cornerstone of therapy, yet up to 20% of patients exhibit treatment resistance, necessitating exploration of alternative and adjunctive strategies. Recent clinical trials have investigated biologic agents such as infliximab, etanercept, and anakinra, as well as immunomodulators including cyclosporine A and anti-thrombotic agents like abciximab. Adjunctive therapies, including plasma exchange, have shown promise in refractory cases. Prognosis is closely tied to coronary artery involvement, with early diagnosis and intervention markedly improving outcomes; however, even angiographically resolved aneurysms can predispose to late atherosclerotic changes. Complications range from myocarditis and valvulitis to life-threatening coronary thrombosis. Long-term management demands an interprofessional approach, coordinated follow-up into adulthood, and patient education to mitigate late cardiovascular morbidity and mortality. This review synthesizes current evidence from ongoing trials, outlines prognostic determinants, and emphasizes both acute-phase and lifelong management priorities.

Chylothorax is a rare but potentially serious and well recognized cause of pleural effusion. Chylothorax commonly occurs following general thoracic and cardiac procedures. Additionally it sometimes develops secondary to non-surgical trauma, i.e. weight lifting. Here we report two additional cases with chylothorax. Somatostatin was added to conservative treatment protocol to increase effectiveness of therapy in both cases. Although the first case required surgical intervention, we avoided any surgical procedure in the second patient with conservative management combined with somatostatin.

Left ventricular hypertrophy (LVH) is independently associated with a higher risk of cardiovascular morbidity and mortality in adults. Adiposity is a risk factor for LVH, independent of blood pressure. Potential causes of this nonhemodynamic pathogenesis identified in adults include adverse body fat distribution, insulin resistance, dyslipidemia, and obstructive sleep apnea syndrome (OSA). In severely obese adolescents, the determinants of obesity-induced changes in left ventricular structure are poorly characterized. Cardiac ultrasonographic, demographic, anthropometric, and comorbidity-related data were prospectively collected in adolescents with severe obesity refractory to conservative treatment who presented for surgical therapy. Differences between adolescents with LVH and without LVH were evaluated using independent samples t, chi-square, or Fisher's exact test. Multivariable linear regression analysis was performed to evaluate associations with left ventricular structural changes, corrected for body mass index (BMI) z score. Forty-three patients entered analysis, of whom 24 (55.8%) showed LVH. The most common geometrical change was eccentric LVH (eLVH), occurring in 21 subjects (48.8%). Demographic and anthropometric variables did not differ between patients with and without LVH. Independent of BMI z score, left ventricular mass index was significantly associated with apnea-hypopnea index (AHI) (regression parameter B = 0.8; 95% CI 0.3 to 1.2). Interventricular septum thickness (IVST) was significantly associated with HOMA-IR values (B = 0.1; 95% CI 0.04 to 0.2), HDL-cholesterol (B = -1.2; 95% CI -2.2 to 0.1), and triglyceride levels (B = 0.5; 95% CI 0.001 to 0.9). LVH, especially eLVH, is highly prevalent amongst severely obese adolescents. Adverse changes in cardiac structure, increased IVST in particular, are independently associated with several nonhemodynamic comorbidities that are common in this population, namely OSA, insulin resistance, and dyslipidemia. Left ventricular hypertrophy • Left ventricular geometry • Severe obesity • Adolescents Abbreviations AHI Apnea hypopnea index BMI Body mass index cLVH Concentric left ventricular hypertrophy cLVR Concentric left ventricular remodeling eLVH Eccentric left ventricular hypertrophy DBP Diastolic blood pressure HOMA-IR Homeostatic model assessment of insulin resistance IVST Interventricular septal thickness LVEDD Left ventricular end-diastolic dimension LVESD Left ventricular end-systolic dimension * Ali Talib

: The primary objective of this study was to evaluate pediatric residents' ability to correctly identify electrocardiogram (ECG) findings and pair them to a corresponding cardiac diagnosis. : : Forty-six pediatric residents from the Johns Hopkins Children's Center were surveyed to evaluate their ability to interpret ECGs and factors affecting that ability. Included in the survey was a packet of 10 patient vignettes each accompanied by a 12-lead ECG. The primary outcome variable was the resident's score of correctly paired ECG findings with the appropriate cardiac diagnosis. One point was given for each pair correctly identified for a maximum of 10 points. Simple and multiple linear regression was used to estimate the magnitude and significance of any association between score of correct responses and resident year, completion of a pediatric cardiology rotation, self-rated ability to read ECGs, and training received in reading ECGs. The mean number of correct ECG findings and cardiac diagnosis pairings out of 10 for the PGY 1 group was 4.1 ± 3, PGY 2 group 4.9 ± 2.9, PGY 3 group 6.6 ± 2, and the PGY 4 group 6.8 ± 1.7. In the unadjusted linear regression model, the PGY 3 group correctly identified 2.4 more pairings compared to the PGY 1 group (P =0.02). Those who completed a pediatric cardiology rotation correctly identified 2.5 more pairings compared to those who did not complete a rotation (P=0.001). ECG interpretation significantly improved from PGY 1 to PGY 3. Educational programs involving ECG interpretation should target those diagnoses with high clinical severity and average to poor resident knowledge.

Please cite this article as: Ramamurthy HR et al., A novel approach to preparation of neonates with transposition of great arteries with intact ventricular septum for successful arterial switch operation: Combined balloon atrial septostomy and patent ductus arteriosus stenting, Medical Journal Armed Forces India,

Background Congenital Heart Disease stands as a prominent cause of infant mortality, with notable disparities in surgical outcomes evident between high-income and low-to middle-income countries. Objective This study presents a collaborative partnership between a local governmental entity and an international private organization to establish a high-quality Pediatric Cardiac Surgery Program in a post-conflict limited resource country, Iraq. Methods A descriptive retrospective study analyzed pediatric cardiac surgery procedures performed by a visiting pediatric heart surgery team from October 2021 to October 2022, funded by the Ministry of Health (MOH). We used the STS-EACTS complexity scoring model (STAT) to assess mortality risks associated with surgical procedures. Results A total of 144 patients underwent 148 procedures. Infants comprised 58.3% of the patients. The most common anomalies included tetralogy of Fallot, ventricular septal defect, and various single ventricle categories, constituting 76% of the patient cohort. The overall surgical mortality rate was 4.1%, with an observed/expected surgical mortality rate of 1.1 (95% CI 0.5, 2.3). There was no significant difference between our observed surgical mortality in Category 2, 3, and 4 and those expected/reported by the STS-EACTS Database (p = 0.07, p = 0.72, and p = 0.12, respectively). The expenses incurred by the MOH for conducting surgeries in Iraq were lower than the alternative of sending patients abroad for the same procedures. The partnership model between a local public entity committed to infrastructure development and funding and an international private organization delivering clinical and training services can provide the foundation for building sustainable, high-quality in situ programs in upper-middle-income countries. Congenital heart surgery • Limited resource country • Building in situ programs • Outcome of pediatric cardiac surgery • Governmental and private partnership, low-and middle-income countries * Fadi Bitar

Patients with hypoplastic left heart syndrome who have been palliated with the Fontan procedure are at risk for adverse neurodevelopmental outcomes, lower quality of life, and reduced employability. We describe the methods (including quality assurance and quality control protocols) and challenges of a multi-center observational ancillary study, SVRIII (Single Ventricle Reconstruction Trial) Brain Connectome. Our original goal was to obtain advanced neuroimaging (Diffusion Tensor Imaging and Resting-BOLD) in 140 SVR III participants and 100 healthy controls for brain connectome analyses. Linear regression and mediation statistical methods will be used to analyze associations of brain connectome measures with neurocognitive measures and clinical risk factors. Initial recruitment challenges occurred that were related to difficulties with: (1) coordinating brain MRI for participants already undergoing extensive testing in the parent study, and (2) recruiting healthy control subjects. Th...

Aberrant cerebellar development and the associated neurocognitive deficits has been postulated in infants with congenital heart disease (CHD). Our objective is to investigate the effect of postnatal head and somatic growth on cerebellar development in neonates with CHD. We compared term-born neonates with a history of CHD with a cohort of preterm-born neonates, two cohorts at similar risk for neurodevelopment impairment, in order to determine if they are similarly affected in the early developmental period. Study Design: 51 preterms-born healthy neonates, 62 term-born CHD neonates, and 54 term-born healthy neonates underwent a brain MRI with volumetric imaging. Cerebellar volumes were extracted through an automated segmentation pipeline that was developed in-house. Volumes were correlated with clinical growth parameters at both the birth and time of MRI. Results: The CHD cohort showed significantly lower cerebellar volumes when compared with both the control (p &lt; 0.015) and prete...

Patients with congenital heart disease (CHD) are at risk for neurocognitive impairments. Little is known about the impact of CHD on the organization of large-scale brain networks. We applied graph analysis techniques to diffusion tensor imaging (DTI) data obtained from 49 adolescents with dextro-transposition of the great arteries (d-TGA) repaired with the arterial switch operation in early infancy and 29 healthy referent adolescents. We examined whether differences in neurocognitive functioning were related to white matter network topology. We developed mediation models revealing the respective contributions of peri-operative variables and network topology on cognitive outcome. Adolescents with d-TGA had reduced global efficiency at a trend level (p = 0.061), increased modularity (p = 0.012), and increased small-worldness (p = 0.026) as compared to controls. Moreover, these network properties mediated neurocognitive differences between the d-TGA and referent adolescents across ever...

Background and Purpose-Preterm birth is associated with alteration in cortico-thalamic development, which underlies poor neurodevelopmental outcomes. Our hypothesis was that preterm neonates with CHD would demonstrate abnormal thalamic microstructure when compared to critically ill neonates without CHD. A secondary aim was to identify any association between thalamic microstructural abnormalities and peri-operative clinical variables. Material and Methods-We compared thalamic DTI measurements in 21 preterm neonates with CHD to two cohorts of neonates without CHD: 28 term and 27 preterm neonates, identified from the same neonatal intensive care unit. Comparison was made with three other selected white matter regions using ROI manual based measurements. Correlation was made with postconceptional age and peri-operative clinical variables. Results-In preterm neonates with CHD, there were age-related differences in thalamic diffusivity (axial and radial) compared to the preterm and term non-CHD group, in contrast to no differences in anisotropy. Contrary to our hypothesis, abnormal thalamic and optic radiation microstructure was most strongly associated with an elevated first arterial blood gas pO 2 and elevated pre-operative arterial blood gas pH (p<0.05). -related thalamic microstructural abnormalities were observed in preterm neonates with CHD. Perinatal hyperoxemia and increased peri-operative serum pH was associated

Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multiinstitutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status.

OBJECTIVESPatients with hypoplastic left heart syndrome are at high risk of late adverse events after Fontan completion, but it is unclear whether their morphological characteristics influence these outcomes.METHODSRetrospective review of the data from the Australian and New Zealand Fontan Registry identified 185 patients with hypoplastic left heart syndrome who survived to hospital discharge after Fontan completion. Their outcomes were reviewed to identify predictors of adverse events with a particular focus on the impact of morphological characteristics. All available echocardiographic parameters were collected, and the hypoplasia of the left ventricle was subjectively considered to be mild, moderate or severe.RESULTSThe mean follow-up after the Fontan procedure was 6.4 ± 4.7 years. The median age at Fontan procedure was 4.41 years, 95% (176/185) of patients underwent an extracardiac conduit Fontan procedure and 71% (132/185) of those were fenestrated. At 15 years after Fontan, fr...

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Several cases of Kawasaki disease (KD) were unresponsive to the initial treatment with intravenous immunoglobulin (IVIG). We retrospectively analyzed all children admitted with KD to determine the occurrence and variables associated with the initial IVIG treatment failure. All patients who fulfilled the criteria for KD and were treated with a single dose (2 g/kg) of IVIG between January 1995 and August 2001 were enrolled. An analysis of the patients who had initially failed to respond to IVIG was performed. A total of 120 patients were enrolled during the study period. There were 68 boys (56.7%). Fourteen patients (11.6%) were found to be unresponsive to initial IVIG treatment. Patients who were anemic (Hb<10 g/dl), had a high neutrophil count (>75%), a high band count, and low albumin were at risk of failure to respond to a single dose of IVIG. We found no correlation among age, gender, days since starting IVIG treatment, and erythrocyte sedimentation rate (ESR) with failure of the initial IVIG treatment. There were 12 patients (10%) who developed coronary artery aneurysms. The failure of a single dose of IVIG treatment occured in up to 11.6% of our Kawasaki patients. We found that low hemoglobin (<10 g/dl), high neutrophil count (>75%), high band count, and a low albumin were associated with the requirement for retreatment with a second dose of IVIG.

Background Patients with single‐ventricle physiology who undergo the Fontan procedure are at risk for thrombotic events associated with significant morbidity and mortality. The UNIVERSE Study evaluated the efficacy and safety of a novel liquid rivaroxaban formulation, using a body weight–adjusted dosing regimen, versus acetylsalicylic acid (ASA) in children post‐Fontan. Methods and Results The UNIVERSE Study was a randomized, multicenter, 2‐part, open‐label study of rivaroxaban, in children who had undergone a Fontan procedure, to evaluate its dosing regimen, safety, and efficacy. Part A was the single‐arm part of the study that determined the pharmacokinetics/pharmacodynamics and safety of rivaroxaban in 12 participants before proceeding to part B, whereby 100 participants were randomized 2:1 to open‐label rivaroxaban versus ASA. The study period was 12 months. A total of 112 participants were enrolled across 35 sites in 10 countries. In part B, for safety outcomes, major bleeding ...

We introduced primary arterial switch operation for the patient with transposition of great arteries and intact ventricular septum (TGA-IVS) who had more than 3.5 mm of posterior left ventricle (LV) wall thickness. Between January 2013 and June 2015, a total of 116 patients underwent arterial switch operation. Of the 116 patients, 26 with TGA-IVS underwent primary arterial switch operation at more than 30 days of age. The age and body weight (mean ± SD) at the operation were 120.4 ± 93.8 days and 4.1 ±1.0 kg, respectively. There was no hospital mortality. The thickness of posterior LV wall (preoperation vs postoperation; mm) was 4.04 ± 0.71 versus 5.90 ± 1.3; P &lt; .0001; interval: 11.8 ± 6.5 days. The left atrial pressure (mm Hg; postoperative day 0 vs 3) was 20.0 ± 3.2 versus 10.0 ± 2.0; P &lt; .0001; and the maximum blood lactate level (mmol/dL) was 4.7 ± 1.4 versus 1.4 ± 0.3; P &lt; .0001, which showed significant improvement in the postoperative course. All cases had delayed s...

The Single Ventricle Reconstruction (SVR) Trial was a randomized prospective trial designed to determine survival advantage of the modified Blalock-Taussig-Thomas shunt (BTTS) vs the right ventricle to pulmonary artery conduit (RVPAS) for patients with hypoplastic left heart syndrome. The primary aim of the long-term follow-up (SVRIII) was to determine the impact of shunt type on RV function. In this work, we describe the use of CMR in a large cohort follow up from the SVR Trial as a focused study of single ventricle function. The SVRIII protocol included short axis steady-state free precession imaging to assess single ventricle systolic function and flow quantification. There were 313 eligible SVRIII participants and 237 enrolled, ages ranging from 10 to 12.5 years. 177/237 (75%) participants underwent CMR. The most common reasons for not undergoing CMR exam were requirement for anesthesia (n = 14) or ICD/pacemaker (n = 11). A total of 168/177 (94%) CMR studies were diagnostic for ...

Patients with hypoplastic left heart syndrome who have been palliated with the Fontan procedure are at risk for adverse neurodevelopmental outcomes, lower quality of life, and reduced employability. We describe the methods (including quality assurance and quality control protocols) and challenges of a multi-center observational ancillary study, SVRIII (Single Ventricle Reconstruction Trial) Brain Connectome. Our original goal was to obtain advanced neuroimaging (Diffusion Tensor Imaging and Resting-BOLD) in 140 SVR III participants and 100 healthy controls for brain connectome analyses. Linear regression and mediation statistical methods will be used to analyze associations of brain connectome measures with neurocognitive measures and clinical risk factors. Initial recruitment challenges occurred that were related to difficulties with: (1) coordinating brain MRI for participants already undergoing extensive testing in the parent study, and (2) recruiting healthy control subjects. Th...

Neurodevelopmental outcomes following the Norwood procedure for single right ventricular lesions are worse than those in the normal population. It would be valuable to identify which patients at the time of Norwood discharge are at greatest risk of neurodevelopmental impairment later in childhood. As such, we sought to construct and validate a model to predict poor neurodevelopmental outcome using variables readily available to the clinician. Using data from 14-month neurodevelopmental outcome of the Single Ventricle Reconstruction (SVR) trial, a Classification and Regression Tree (CART) analysis model was developed to predict severe neurodevelopmental impairment, defined as a Psychomotor Development Index (PDI) of <70 on the Bayley Scales of Infant Development®-II. The model was then validated using data from subjects enrolled in the Infant Single Ventricle (ISV) trial. PDI scores were <70 in 138 of 313 subjects (44%). Predictors of PDI<70 were: post-Norwood length of ICU stay >46 days, genetic syndrome or other anomalies, birth weight <2.7 kg, additional cardiac surgical procedures, and use of ≥5 medications at hospital discharge. Using these risk factors, the CART model correctly identified 75% of SVR subjects with PDI<70. When the CART model was applied to 70 subjects from the ISV trial, the correct classification rate was 67%.

Objectives: Currently, the survival for the Norwood procedure for hypoplastic left heart syndrome is approximately 90% in selected centers. However, the development of tricuspid regurgitation remains a significant obstacle to successful staged repair in a subset of these patients. The results of tricuspid valve repair in this challenging patient population remain largely unknown. Methods: Twenty-eight patients with significant (3-4ϩ) tricuspid regurgitation after the Norwood procedure required tricuspid valve repair from August 1995 through December 2002. The clinical and Doppler-echocardiographic data were reviewed to determine the efficacy of repair and patient outcome. Results: Follow-up was 96% complete (27/28). Patients were divided into 2 groups on the basis of tricuspid regurgitation at late follow-up: those with a successful late outcome (0-2ϩ) and those with a poor outcome (3-4ϩ). There were 17 (63%) patients with a successful result and 10 (37%) with an adverse outcome. Age, weight, follow-up duration, valve anatomy, and stage of palliation were not significantly different between groups. Early postoperative 0 to 2ϩ regurgitation was associated with a durable result (P ϭ .012) and preserved ventricular function (P ϭ .04). Need for repair other than a partial annuloplasty was predictive of a poor outcome (P ϭ .04). Overall survival was 67% (18/27). Survival was 94% (16/17) for patients with a successful late result versus 20% (2/10) for those with a poor outcome (P ϭ .0002). Tricuspid valve repair can be accomplished in this challenging patient population with excellent results. Successful tricuspid valve repair is predictive of continued good valve function and preserved right ventricular function. Successful valve repair at late follow-up predicts excellent late survival. From the Divisions of Pediatric Cardiovascular Surgery a and Pediatric Cardiology,

Published data showing the intermediate effect of transcatheter device closure of atrial septal defect (ASD) in the pediatric age-group are scarce. The objective of the study was to assess the effects of transcatheter ASD closure on right and left ventricular functions by tissue Doppler imaging (TDI). The study included 37 consecutive patients diagnosed as ASD secundum by transthoracic echocardiography and TEE and referred for transcatheter closure at

Cardiac hydatid cyst is an uncommon lesion. The infection, often acquired by children during play with infected dogs, is most common in sheep-raising areas of the world. We report our clinical and surgical experience in the treatment of one of the youngest reported cardiac hydatidosis patients with multivisceral involvement.

As treatments of the extra-cardiac complications of muscular dystrophy (MD) improve, males with MD are more likely to develop cardiac disease. The impact of cardiomyopathy or heart failure (HF) and ventricular tachycardia (VT) on hospitalizations and in hospital mortality are not known. We performed an analysis of inpatient admission data for patients with MD using the Pediatric Health Information System database. We selected males who were 6 years or older with diagnosis codes of MD and cardiac disease including cardiomyopathy/HF and VT between 2003 and 2013. We created a logistic regression model to identify predictors of subsequent cardiac arrest or death in MD patients. We also compared hospital charges, lengths of stay and ages among MD patients with or without cardiac disease. Our logistic regression model showed that VT (OR 5.41, 95 % CI 2.83, 10.34) and cardiomyopathy/HF (OR 1.79, 95 % CI 1.05, 3.04) were risk factors for cardiac arrest or death. Of the 84 cardiac arrests or deaths in 3363 MD patients, 49 (58 %) were related to cardiac disease. Nineteen (39 %) of these events occurred in MD patients with VT. The mean hospital charges and the mean length of stay were greater and longer in MD patients with VT compared to those without cardiac disease and those with only cardiomyopathy/HF (p \ 0.05). Cardiac disease is a significant burden in hospitalized MD patients. Our results suggest that VT and cardiomyopathy/HF are associated with an increased risk of cardiac arrest or death in MD patients.

Background and aims Few data are currently available for Kawasaki disease (KD) below 12 months especially in Caucasians. This study aims to analyze clinical and laboratory features of KD among an Italian cohort of infants. Methods A retrospective chart review of KD children aged less than one year at time of disease onset between January 2008-December 2017 was performed. Clinical data, laboratory parameters, instrumental findings, treatment and outcome were collected in a customized database. Results Among 113 KD patients, 32 (28.3%) were younger than 1 year. Nineteen patients aged below 6 months, and three below 3 months. The median age was 5.7 ±2.7 months. The mean time to diagnosis was 7±3 days and was longer in the incomplete forms (8 ± 4 vs 6 ± 1 days). Conjunctival injection was present in 26 patients (81.2%); rash in 25 (78.1%); extremity changes in 18 (56.2%); mucosal changes in 13 (40.6%,) and lymphadenopathy only in 7 (21.8%). Mucosal changes were the least common features in incomplete forms (18.2%). Twenty-two patients (68.7%) had incomplete KD. Nineteen (59.4%) had cardiac involvement, of whom 13 (59.0%) had incomplete form. ESR, PCR and platelet values were higher in complete KD; especially, ESR resulted significantly higher in complete forms (80 ± 25.7 mm/h vs 50 ± 28.6 mm/h; p = 0.01). Conversely, AST level was statistically significant higher in patients with incomplete forms (95.4 ± 132.7 UI/L vs 29.8 ± 13.2 UI/L; p = 0.03). All patients received IVIG. Response was reported in 26/32 patients; 6 cases needed a second dose of IVIG and one required a dose of anakinra. Conclusion In our cohort, incomplete disease was commonly found, resulting in delayed diagnoses and poor cardiac prognosis. Infants with incomplete KD seem to have a more severe disease and a greater predilection for coronary involvement than those with complete KD. AST was significantly higher in incomplete forms, thus AST levels might be a new finding in incomplete forms' diagnosis. Eventually, we highlight a higher resistance to IVIG treatment. To our knowledge this is the first study involving an Italian cohort of patients with KD below 12 months. Kawasaki disease (KD) is an acute medium vessel vasculitis of childhood, typically involving coronary arteries . It is one of the most common pediatric vasculitis and the commonest cause of acquired

Cannon A waves are prominent, high-amplitude jugular venous pulsations resulting from atrial contraction against a closed tricuspid valve, reflecting a pathophysiologic hallmark of atrioventricular (AV) dyssynchrony. They are most commonly encountered in rhythm disturbances such as complete heart block, ventricular tachycardia, and pacemaker syndrome. Clinically, these exaggerated A waves manifest intermittently and are best appreciated on physical examination of the jugular venous pulse. However, the detection of Cannon A waves requires an astute understanding of the jugular waveform and its correlation with electrocardiographic and hemodynamic findings. While these waves are often irregular, they may present regularly in junctional or supraventricular rhythms. Differentiation from other venous waveform abnormalities, particularly giant A waves or V waves in tricuspid valve disease, is essential. Evaluation involves comprehensive rhythm assessment via ECG and structural assessment via echocardiography. Management focuses on correcting the underlying arrhythmia or AV dyssynchrony, often necessitating pacemaker therapy or rhythm control strategies. This review aims to elucidate the pathophysiology, clinical recognition, differential diagnosis, and interprofessional management strategies related to Cannon A waves, underscoring their diagnostic value in bedside medicine.

A very rare coronary artery anomaly, intercoronary communication, that may be lucky for some patients Uma anomalia muito rara na artéria coronária: We read the paper by Abreu et al. entitled 'Coronary arcade: A rare anomaly of the coronary circulation' with great interest, and we thank them for their interesting report. They asserted that the cause of the chest pain and ECG alterations observed during exercise testing in the case presented may be the result of transient disturbances in coronary blood flow during the test. Furthermore, they state that intercoronary communication (ICC) may be unrelated to ischemia, and that it may even function as a natural bypass, protecting against ischemia. 1 Herein, we present our case supporting their idea.

International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties. Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. IJCRI publishes Review Articles, Case Series, Case Reports, Case in Images, Clinical Images and Letters to Editor.