Pediatric Cardiology Department Research Articles

Registry as a tool for assessing economic costs of managing pediatric patients with hypertrophic cardiomyopathy

Background. Hypertrophic cardiomyopathy (HCM) is one of the most common form of cardiomyopathy in children, with an estimated annual incidence of 2.9 per 100,000 children. The management of patients with HCM requires high healthcare costs. Registry-based medical research is an important tool in assessing health care decision making. Objective. To analyze the costs of the state for the management of pediatric HCM based on the data of the pediatric patient registry. Materials and methods. The study was performed on the basis of the Department of Pediatric Cardiology and Medical Rehabilitation of the Almazov National Medical Research Centre. Currently, the electronic database includes information on 159 children with HCM, including 52 (34 %) girls. The mean age of onset of HCM was 5 years [0; 11], and 68 (44.4 %) children had the onset of myocardial hypertrophy before the age of 1 year. Medical costs included direct medical costs of drug therapy, surgical treatments (implantation of a cardioverter-defibrillator and myectomy) and hospitalization, and indirect costs which included the costs of family caregiving; social benefits due to disability, and death impact on the gross domestic product. Results. The total economic impact of HCM in the context of government spending, is RUB 107.8 million, including medical costs of RUB 27.96 million, direct nonmedical costs of RUB 13.17 million, and indirect costs of RUB 66.7 million. Indirect costs accounted for the lion’s share (61.8 %) of economic impact. Direct medical costs were 26 % of economic impact. The average impact of family caregiving accounted to RUB 11,263.89, and total value were RUB 1.04 million. Conclusion. The intermediate results of our study allow to estimate the economic impact of pediatric HCM to the state. Optimization of financial resource use will help reduce the economic impact on the state to the healthcare sector in the future.

Prediction of cyanotic and acyanotic congenital heart disease using machine learning models.

Congenital heart disease is most commonly seen in neonates and it is a major cause of pediatric illness and childhood morbidity and mortality. To identify and build the best predictive model for predicting cyanotic and acyanotic congenital heart disease in children during pregnancy and identify their potential risk factors. The data were collected from the Pediatric Cardiology Department at Chaudhry Pervaiz Elahi Institute of Cardiology Multan, Pakistan from December 2017 to October 2019. A sample of 3900 mothers whose children were diagnosed with cyanotic or acyanotic congenital heart disease was taken. Multivariate outlier detection methods were used to identify the potential outliers. Different machine learning models were compared, and the best-fitted model was selected using the area under the curve, sensitivity, and specificity of the models. Out of 3900 patients included, about 69.5% had acyanotic and 30.5% had cyanotic congenital heart disease. Males had more cases of acyanotic (53.6%) and cyanotic (54.5%) congenital heart disease as compared to females. The odds of having cyanotic was 1.28 times higher for children whose mothers used more fast food frequently during pregnancy. The artificial neural network model was selected as the best predictive model with an area under the curve of 0.9012, sensitivity of 65.76%, and specificity of 97.23%. Children having a positive family history are at very high risk of having cyanotic and acyanotic congenital heart disease. Males are more at risk and their mothers need more care, good food, and physical activity during pregnancy. The best-fitted model for predicting cyanotic and acyanotic congenital heart disease is the artificial neural network. The results obtained and the best model identified will be useful for medical practitioners and public health scientists for an informed decision-making process about the earlier diagnosis and improve the health condition of children in Pakistan.

SYNCOPA IN CHILDREN AND ADOLESCENTS OUR EXPERIENCE

<p><strong>Introduction</strong>: Syncope is a sudden, short-lived, transient loss of consciousness associated with the inability to maintain postural tone. The aim of this paper is: to determine the frequency of syncope in children and adolescents in our conditions, to analyze the characteristics of syncopal episodes and clinical presentation in order to identify the etiology of seizures, and to provide diagnostic protocols, i.e. guidelines for streamlining clinical trials.</p> <p><strong>Methodology</strong>: The diagnosis of the disease was made clinically on the basis of well-taken anamnestic data and a detailed description of the quality of the attack, physical examination and routine laboratory analyzes: (CBC, glycemia, standard ECG). Additional tests were selectively performed: Holter ECG, ergometry, Tilt table test, Echocardiogram, NMR, EEG.</p> <p><strong>Results</strong>: Out of the total number of examined children in the outpatient Children’s Hospital, Department of Pediatric Cardiology, 139 patients (0.6%) reported due to short-term loss of consciousness. The largest number of children was between 15 and 18 years old. Ninety-two ( 66%) of those 139 were girls, and 47 (34%) were boys (p <0.05). Regarding the cause of syncope, it was found in 117 (84%) patients, and in 22 (16%) children the cause of syncope was unknown (p <0.05). Etiologically spreaking, syncope was divided into 3 groups: autonomic (vasovagal, situational, orthostatic, increased vagus tone in athletes) was the most common, in 88 patients (75%), cardiogenic in 5 (4%) and non-cardiogenic in 24 patients.(21%). There was also a recurrence of the attack. In two children, the syncopal attack was repeated 4 times.</p> <p><strong>CONCLUSION</strong>: Syncope most often occurs in teenagers and is mostly benign. To assess syncope, it is necessary to gather a detailed history of the attack, to conduct a detailed physical examination and routine laboratory analyzes: CBC, glycemia and standard ECG. Supplementary diagnostics should be performed exclusively in patients where there is a reasonable suspicion of heart disease or neurological diseases. </p>

Malnutrition and Growth Failure in Cyanotic and Acyanotic Congenital Heart Diseases with and Without Pulmonary Hypertension

Objective: To determine the status of malnutrition and growth failure in cyanotic and acyanotic congenital heart diseases with and without pulmonary hypertension. Study Design: Cross sectional study. Place and Duration of Study: Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases, Tando Muhammad Khan, Pakistan, from Jan 2020 to Oct 2021. Methodology: A total of 250 children, with echocardiography confirmed congenital heart disease, of both genders and aged below 5 years were included. All cases were divided into 4 groups, these being, acyanotic congenital heart disease with PH, acyanotic congenital heart disease without pulmonary hypertension, cyanotic congenital heart disease with pulmonary hypertension and cyanotic Congenital heart disease without pulmonary hypertension. Gender, age, body weight, height, area of residence and malnutrition status were determined in all cases and compared in between study groups. Results: There were 41(16.4%) patients with acyanotic PH, 115(46.0%) acyanotic without PH, 34(13.6%) cyanotic with pulmonary hypertension and 60(24.0%) cyanotic without pulmonary hypertension among a total of 250 cases. Significant differences were noted in terms of age, weight, and height among different study groups (p<0.001). Nutritional status as normal was noted in 51(20.4%) cases, mild malnutrition in 50(20.0%), moderate malnutrition in 65(26.0%) and 84(33.6%) with severe malnutrition. Children having cyanotic congenital heart disease and pulmonary hypertension were also found to have failure to thrive (p=0.009). Conclusion: Malnutrition and growth failure were found to be very prevalent among children with CHDs under the age of 5 years. Malnutrition was significantly more pronounced among children having cyanotic CHDs with PH.

Looking through the crystal ball feasibility of tele-echocardiography using smart glasses in neonates: a pilot study.

In recent years, the importance of telemedicine has increased significantly. Especially in the field of echocardiography, virtual reality glasses offer the possibility of real-time data transmission without restrictions in the examination process. In particular, the care of critically ill newborns with suspected CHD might be improved by allowing a specialized paediatric cardiologist to remotely guide an echocardiographic examination. The current study aims to prove whether novices, under Google Glass guidance by a paediatric cardiologist, can perform an appropriate neonatal echocardiography. The current study is a prospective monocentric single-blinded pilot study. Participants were supposed to perform two test runs: The first test run was "unguided" and the second test run was instructed via Google Glass. A validated training simulator for neonatal echocardiography "EchocomNeo, Echocom GmbH" was used. The study took place at the Leipzig Heart Center, Department of Pediatric Cardiology from April 2022 to November 2022. A total of 21 medical students were enrolled. In total 252 views (126 views in each test run) were recorded. The overall performance was significantly higher in the Google Glass guided test run compared to "unguided" (structure score: 77.6% vs. 63.2%. p < 0.001 and quality score: 58.7% vs. 47.2%, p < 0.001). Also, the time was significantly lower in the Google Glass guided test run than in the unguided test run, p = 0.014. Google Glass guidance by a paediatric cardiologist could optimize the performance of novices in echocardiography using a standardized neonatal echo-simulator with structural normal cardiac anatomy.

Frequency and pattern of adult congenital heart disease in a tertiary care cardiac hospital: Reasons associated with delayed diagnosis.

To determine the distribution pattern and possible reasons of delayed diagnosis of congenital heart disease in adult population. The analytical, cross-sectional study was conducted at the Department of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi, from July to December 2021, and comprised patients of either gender aged 18 years and above with congenital heart disease confirmed by echocardiographic examination. Age, gender, weight, height, provincial affiliation, marital status, number of children, if any, duration of disease and New York Heart Association classification were noted on a predesigned proforma. Patterns of congenital heart disease types and reasons behind delayed diagnosis, if noted, were recorded. Data was analysed using SPSS 26. Of the 129 patients, 68(52.7%) were males. The overall mean age was 25.8±9.8 years. Mean age at the time of diagnosis was 12.4±14.8 years. There were 91(70.5%) cases with delayed diagnosis. There was no significant gender difference with respect to delayed diagnosis (p>0.05). Delayed referral 39(42.9%), delayed consultation 22(24.2%), delayed diagnosis by the doctor 21(23.1%), and social factors 9(9.9%) were the most frequent reasons behind delayed diagnosis. Ventricular septal defect was the most frequently defect 38(29.5%), followed by Tetralogy of Fallot 32(24.8%). Right ventricular dysfunction was the most common complication of delayed diagnosis 35(27.1%). Delayed diagnosis of congenital heart disease was reported in over two-thirds of the cases, with ventricular septal defect being the most frequent type of defect, and delayed referral being the most common reason.

8137 Prevalence Of Overweight And Obesity In Dominican Children With Congenital Heart Disease

Abstract Disclosure: L.D. Rojas Rodríguez: None. M.J. Mateo Pérez: None. L.C. López Sención: None. V.T. San Martin: None. A. Grullón: None. Background: Overweight and obesity can be detrimental to the health of children with congenital heart disease, potentially leading to or contributing to other chronic medical conditions such as hypertension. This, in turn, may increase the cardiovascular demands of these patients whose lives are already affected by a defective heart anatomy. Objective: To determine the prevalence of overweight and obesity in Dominican children with congenital heart disease. Methods: An observational cross-sectional study was conducted to determine the prevalence of overweight and obesity in children with congenital heart disease attending the Pediatric Cardiology Department at Children's Hospital (Santo Domingo, Dominican Republic), from July to December 2022. The study population was classified into categories of underweight, normal weight, overweight or obesity using the CDC Body Mass Index-for-age growth chart (2022), based on their age, weight, height, and sex. The parents of the patients were interviewed to gather information about their children’s medical history, as well as their diet type and level of physical activity. Diet type was assessed through a questionnaire about daily meals; diets in which processed foods and simple carbohydrates were predominant were categorized as unhealthy, while those with a predominance of proteins and complex carbohydrates were categorized as healthy. Physical activity levels were measured using the Assessment of Physical Activity Level Questionnaire (APALQ). Additionally, blood pressure values were categorized as normal or elevated/high based on the American Academy of Pediatrics Clinical Practice Guidelines for Screening and Management of High Blood Pressure in Children and Adolescents (2017). Results: A total of 84 patients with congenital heart disease were analyzed, with 11.9% (10/84) categorized as having overweight and 23.8% (20/84) categorized as having obesity. Among patients with overweight or obesity, unhealthy eating habits predominated in 66.7% (20/30), while a sedentary level of physical activity was reported in 56.7% (17/30). Additionally, among patients with overweight or obesity, 36.7% (11/30) had elevated/high blood pressure. Conclusion: About one-third of Dominican patients with congenital heart disease were found to have overweight or obesity. Additionally, about one-third of those with excess weight were found to have elevated/high blood pressure. These findings stress the need for urgent implementation of personalized interventions and educational programs focused on fostering healthier lifestyles. The ultimate goal is to reduce cardiovascular risks and improve the overall well-being of children living with congenital heart disease. Presentation: 6/1/2024

How to diagnose macrophage activation syndrome early: own experience of the Department of Paediatric Cardiology and Rheumatology in Lodz

How to diagnose macrophage activation syndrome early: own experience of the Department of Paediatric Cardiology and Rheumatology in Lodz

Use of ultrasound for vascular access during cardiac catheterisation in children with congenital heart disease: a Swiss multicentre cohort study.

Our study aimed to assess the first-attempt success rate of ultrasound (US) and anatomical landmark (ALM) guidance for vascular cannulation in children with congenital heart disease in Switzerland. A prospective observational multicentre study was conducted from January 2022 to January 2023 in three university hospitals in Switzerland. We included patients with congenital heart disease aged 0 to 18 years who were scheduled for elective cardiac catheterisation. Periprocedural data were collected anonymously. The choice of vessel and guidance technique were at the operator's discretion. 177 arterial and 240 venous cannulations in 253 patients were analysed. The median age and weight were 4.4 years (interquartile range [IQR] 1.2-8.8) and 15 kg (IQR 8-27), respectively. Nearly all operators, 97.5%, were deemed experienced. The femoral artery and femoral vein were the most preferred vessels. US guidance was used in 62% of procedures, predominantly within populations who were younger, smaller and of lower weight.The success rate at the first attempt was notably higher with US guidance for arterial cannulation (US: 80%, ALM: 37%, p <0.001) and venous cannulation (US: 78% vs ALM: 47%, p <0.001). Overall success rates for venous and arterial cannulation were comparable between ultrasound and anatomical landmark guidance. A total of 45 complications (10.5%) occurred, with immediate uncomplicated haematoma being the most frequent. US guidance is preferred for the localisation and cannulation of vessels during cardiac catheterisation within paediatric cardiology departments in Switzerland. Given its high success rate for first-attempt cannulation, US warrants additional research to evaluate its potential in reducing complications.

Serum Albumin and Its Influence on Immediate Outcomes in Children with Congenital Heart Disease Undergoing Cardiac Surgery

Background: Hypoalbuminemia is associated with morbidity and mortality in critically ill children and is a well-recognized predictor of general surgical risk. It frequently occurs in patients with congenital heart disease (CHD). Moreover, cardiopulmonary bypass (CPB)-induces an inflammatory response, and the overall surgical stress can affect albumin concentration greatly Objective: To estimate pre-operative, per-operative and post-operative serum albumin level and to see its influence on immediate outcomes in children with congenital heart disease undergoing cardiac surgery with cardiopulmonary bypass Methods: This cross-sectional observational study was conducted in Paediatric cardiology department, BSMMU in a view to find out the influence of pre-operative, per-operative and post-operative serum albumin level on immediate outcomes in children with congenital heart disease undergoing cardiac surgery with cardiopulmonary bypass(CPB).The study included total 48 patients of CHD who underwent CPB. Patients were selected according to inclusion and exclusion criteria. Result: Mean pre-operative serum albumin was 3.5±0.9 mg/dl whereas per-operative and post-operative serum albumin was 3.3±0.7mg/dl and 3.1±0.7 mg/dl respectively.CPB time was a bit higher in study population (98.4± 29.1min) where as other outcome parameters were within normal range. Duration of hospital stay was 12.2±3.1days. 22 patient (45.8%) developed valvular regurgitation and 6 patient (12.5%) developed pericardial effusion in post-operative echocardiography. Infection was evident by positive blood culture in 7 patients (14.8%). Pre, per and post-operative hypoalbuminaemia was associated with poor outcome after CPB. Conclusion: Pre, per and post-operative hypoalbuminaemia is a prudent indicator for speculating poor immediate outcome following CPB in children with CHD. (Bangladesh Heart Journal 2024; 39(2): 121-126

Outcome of Early and Late Onset Fontan Operation in Patients with Univentricular Heart Repair

Objective: To determine the outcome of early and late-onset Fontan operation in patients with univentricular heart repair. Study Design: Quasi-experimental study. Place and Duration of the Study: Department of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan, from Jan 2019 to Dec 2021. Methodology: During the study period, 121 patients of both genders, up to any age, with functionally univentricular hearts and who planned to undergo Fontan procedures were included. All cases were divided into two groups, Group-A had patients who underwent extra-cardiac Fontan at age ≤6 years, while Group B had patients who underwent extra-cardiac Fontan at age &gt;6 years. Pre-procedure diagnosis, demographic, anatomic and hemodynamic characteristics, post-Fontan procedure morbidity and mortality were noted. Results: As per inclusion/exclusion criteria, 121 cases underwent Fontan surgery during the study period. There were 61(50.4%) male patients. Overall, the mean age was 7.02±4.6 years. Seventy-six 76(61.2%) cases were in Group-A and 45(38.8%) in Group-B. Mortality (within 30 days post-surgery) was reported in 11(9.1%) patients. Except for the mean duration of chest tube drainage ((0.010), there was no difference in terms of post-Fontan surgery outcomes between both study groups (p&gt;0.05). Conclusion: The age of the patients at the time of the Fontan procedure did not affect the outcomes.

DEMOGRAPHIC CHARACTERISTICS, HEMODYNAMIC PROPERTIES, TREATMENTS AND OUTCOMES OF PATIENTS WITH ISOLATED VENTRİCULAR SEPTAL DEFECT: A RETROSPECTIVE STUDY

Aim: Determine the demographic characteristics, hemodynamic properties, treatments, prognosis and complications of patients diagnosed with isolated ventricular septal defect (VSD). Material and Methods: The study was a retrospective study of 268 patients diagnosed with isolated VSD in OMUTF Department of Pediatric Cardiology between January-2010 and December-2018. The cases included in the study were accessed from the database of our hospital. The statistical analysis of data was processed with SPSS v.21 program. Results: Of the patients, 56.3% were female and 43.7% were male. The most frequent period in which VSDs were diagnosed (50%) was the first 30 days of life. Muscular VSD was the most common type (56.3%). Small VSDs were detected in 83.6%. Membranous defects have a higher tendency to be larger (35.8%). Murmur was detected in 91%. Spontaneous closure was observed in 44.4%, and the rate of spontaneous closure in the first 2 years of life was 58.8%. The spontaneous closure rate of muscular defects was higher. Surgical closure was performed for 5.5%. The need of surgical closure for large membranous defects was higher. Pulmonary hypertension was detected in 2.9% of the patients. Discussion: VSD is one of the common acyanotic congenital heart diseases. Although it is generally asymptomatic, it can develop to pulmonary hypertension and Eisenmenger syndrome. In case of delayed diagnosis time, the need for medical or surgical treatment will not be met. The complication and mortality rates of surgical treatment are low. However, we think that it is important to inform families before surgery.

A case of pediatric brain abscess secondary to rhinosinusitis

Acute bacterial rhinosinusitis is a common childhood infection and a frequent complication of viral infections or allergic inflammations of the upper respiratory tract. Untreated cases may have serious complications as meningitis, orbital cellulitis, epidural and brain abscesses (1,2). Brain abscesses rarely seen pediatric infections with high mortality and morbidity rates, are mostly observed secondary to extrasinus spread of rhinosinusitis and acute otitis media, hematogenous spread and penetrating trauma (3). The most common predisposing factors are immunodeficiencies, cyanotic heart and hematologic diseases. A 16-year-old boy was admitted to the emergency service due to seizure. It was learned from his history that he was admitted to the hospital 10 days ago due to headache and high fever. With the diagnosis of acute sinusitis, azithromycin and ornidazole treatment was started, but there was no improvement in his complaints.. On physical examination, clouding of consciousness and postnasal purulent discharge were detected. Remarkable laboratory test results: WBC: 22.500/mm3; platelets 156.000/mm3, C-reactive protein 226 mg/L. Brain tomography revealed a 4-cm suspicious abscess or mass lesion in the right frontal region of the brain compatible with right frontal and ethmoid sinusitis. Upon consultation with pediatric neurology and neurosurgery departments, cranial MRI and MR spectrometry were performed which revealed a lesion compatible with a 4- cm brain abscess, brain edema , shift, and acute sinusitis in the right frontal region. Treatment with cefotaxime, vancomycin and metroinidazole was started for acute sinusitis, and hypertonic sodium infusion and leveteresitam treatment for brain edema. Surgical drainage of the abscess &gt; 2 cm was performed by neurosurgery. Gram staining and culture of the abscess material were unremarkable. Upon evaluation by pediatric immunology, cardiology and hematology departments, immunodeficiency, cyanotic heart disease and hematologic disease were not detected. The lesion was evaluated as inappropriately treated brain abscess secondary to acute sinusitis. In the 2nd week of treatment, edema surrounding the abscess, shift and pressure regressed, but the the abscess did not shrink, so the treatment with meropenem, vancomycin and metroinidazole was initiated. Antiedema therapy was discontinued, and antibiotherapy was maintained for 9 weeks until the abscess size was &lt; 2 cm. When the abscess shrinked below 1 cm in the control cranial images he was discharged with follow-up recommendations. Acute bacterial sinusitis is one of the common, and clinically diagnosed infectious diseases of childhood. And current guidelines recommend amoxicillin, amoxicillin-clavulanic acid and cephalosporins (ie.cefdinir) as the first-line treatment. Considering the high drug resistance in our country, macrolides are not the first choice in patients without a history of penicillin allergy (2). If not treated appropriately, serious cranial complications preventable with timely appropriate antibiotherapy may develop. Brain abscesses are rare but mortal infectious diseases in childhood. The most common cause is the extrasinus spread of infections such as acute rhinosinusitis (3). Combination of medical and surgical treatment is usually recommended for abscesses &gt; 2 cm. For abscesses &lt; 2 cm and inoperable multiple abscesses only medical treatment is an option. Although the duration of treatment depends on the size, location, causative agent and treatment response, medical treatment is recommended for approximately 6-12 weeks until the abscess is &lt; 2 cm (4)

PATTERN OF CONGENITAL HEART DISEASE IN 0 TO 28 DAYS INFANTS OF DIABETIC MOTHERS

Objectives: To determine the frequency of patterns of congenital heart disease in 0 to 28 days infants of diabetic mothers. Study design: Cross Sectional Study Place and duration of study: This study was conducted at the department of pediatrics, Mardan Medical Complex Mardan from 10th October 2022 to 10th April 2023. Methods: A total of 152 neonates born to diabetic mothers and reported with congenital heart disease were included in the study. All neonates were sent to department of pediatric cardiology for echocardiographic evaluation. Data regarding patterns of congenital heart disease was recoded. Descriptive analysis was done by applying frequency and percentages. Results: The Mean±SD of age in this study was 7.934±3.53 days with an age range of 0 to 28 days. Male patients were 61.8% while females were 38.2%. Patent ducts arteriosus was the commonest congenital heart disease 49 (32.2%) followed by Patent foramen ovale 44 (28.9%), hypertrophic cardiomyopathy 21 (13.8%) and atrial septal defect 16 (10.5%), ventricular septal defect 10 (6.58%), transposition of great arteries 7 (6.50%) and tetralogy of fallot 5 (3.28%). Conclusion: Patent ducts arteriosus was the commonest pattern of congenital heart disease followed by patent foramen ovale, hypertrophic cardiomyopathy, atrial septal defect and others in infants of diabetic mother. Postnatal echocardiography screening programs for CHD for early diagnosis and management can help to reduce morbidity and mortality in these infants.

Professional quality of life in cardiovascular nurses: differences between cardiology and pediatric cardiology clinic - pilot study

Abstract Background/Introduction Nursing can be a rewarding profession as it involves helping others. However, it is demanding, particularly for Cardiovascular nurses who may be exposed to stressful situations, patient suffering, and death. Additionally, work-related issues such as shift work, work overload, and excessive bureaucracy can pose challenges. It is necessary to consider that caring for children presents unique challenges compared to caring for adults, which can impact the Professional Quality of Life (ProQoL) and its domains. Purpose In this study, we aimed to investigate differences in ProQoL between nurses working in pediatric cardiology and adult cardiology units. Methods Nurses working in pediatric cardiology and adult cardiology units were enrolled in the study. The Polish version of the ProQoL 5 questionnaire was collected using a paper form. The specific domains of satisfaction, job burnout, and stress were calculated according to the questionnaire key. Moreover, data on age, additional places of employment and working hours were collected. The study examined the relationship between these factors and ProQoL domain scores using the R-Spearman correlation. Scores from each domain were compared between clinics using U-Mann-Whitney tests and a two-sided Fisher's exact test. Results The study involved 13 pediatric cardiology staff: six midwives, six nurses, one paramedic, and 29 adult cardiology staff: 26 nurses, two paramedics and one medical caretaker. No differences were found in any of the ProQoL domains between those working with children and those working with adults: p=0.74 for satisfaction, p=0.90 for burnout and p=0.77 for stress. A trend of lower age was observed among those working in the Paediatric Cardiology Department. The majority of nurses, regardless of ward, reported high or intermediate job satisfaction, as well as low or moderate levels of stress and burnout. Only one person employed in the adult Cardiology department reported a low level of job satisfaction. Older age was associated with higher job satisfaction (R=0.32; p=0.04) and lower levels of burnout (R=-0.31; p=0.048). Additionally, a higher number of working hours per month was associated with burnout (R=0.41; p=0.007). Satisfaction was negatively associated with burnout (R=-0.51; p&amp;lt;0.001) and positively correlated with stress (R=0.35; p=0.04). However, burnout was not associated with stress (p=0.56). Conclusions Regardless of the different work specifications in departments, related to working with children or adults, the ProQoL does not differ significantly. Older age may be positively associated with ProQoL. It may be the result of greater work experience. A higher number of working hours per month is associated with burnout, so it is therefore appropriate to emphasize the importance of occupational health and encourage not to overwork. A multicentre study could further define the differences in ProQoL and the influencing factors.

Nationwide Survey on Transitional Care for Patients With Childhood-Onset Cardiomyopathy in Japan.

Background: Individuals transitioning into adulthood require age-appropriate medical care and delegation of decision-making authority from their parents to the patients themselves. Although there have been multiple observational and interventional studies on transitional care for patients with congenital heart disease (CHD) in the cardiovascular field, transitional care specific to childhood-onset cardiomyopathy (CM) remains unaddressed. Methods and Results: A nationwide questionnaire-based survey was performed in the pediatric cardiology departments of 151 facilities in Japan. Responses were obtained from 100 (66%) facilities with low transfer rates (<5%) for childhood-onset CM cases. The comparison between CHD-transferring and non-CHD-transferring facilities revealed a significantly higher transfer rate (83.9%) for childhood-onset CM cases in the CHD-transferring facilities (P<0.001). Regarding the transition programs, 72 (72%) facilities do not offer any programs for CM, while most (92%) facilities recognize its necessity. Finally, only 19 (19%) facilities provided a transition program, 10 of which were CHD based. Conclusions: To the best of our knowledge, this is the first study to demonstrate the poor transition/transfer care status of patients with childhood-onset CM in Japan. The transfer rate of CMs was lower than that of CHDs, and transition programs were less available. Referring to the system established for CHD could help develop a successful transitional care system for CM.

IMPACT OF MALNUTRITION ON PEDIATRIC HEART FAILURE ANALYZING Z-SCORES AT PRESENTATION

Pediatric heart failure represents a critical health concern characterized by the heart's inability to effectively pump blood, often resulting from congenital heart defects, myocarditis, and cardiomyopathies. Malnutrition has emerged as a recognized comorbidity in pediatric heart failure, significantly impacting patient outcomes. This study aimed to investigate the relationship between malnutrition and pediatric heart failure by analyzing Z-scores at presentation to guide targeted interventions and improve patient management. The study employed a retrospective observational design and was conducted at the Department of Pediatric Cardiology, Lady Reading Hospital, Peshawar, from January 2023 to January 2024. A total of 764 pediatric patients with heart failure were included in the analysis. Nutritional status was assessed using weight and height measurements, and Z-scores were calculated based on WHO Child Growth Standards. Data analysis was performed using IBM SPSS version 22. Results revealed varying degrees of malnutrition among pediatric heart failure patients, with 62.8% classified as severely malnourished, 28.4% as moderately malnourished, and 8.8% as mildly malnourished. The calculated Z-score of -3.2 indicated significant malnutrition among the study population. Furthermore, a negative correlation (r = -0.32, p &lt; 0.001) between Z-score and left ventricular ejection fraction was observed, suggesting a link between malnutrition severity and cardiac dysfunction. These findings underscore the high prevalence of malnutrition among pediatric heart failure patients and its substantial impact on outcomes. Early nutritional assessment and intervention are crucial for optimizing patient care and enhancing outcomes in children with heart failure. Collaborative, multidisciplinary approaches that integrate cardiac and nutritional management strategies can comprehensively address the diverse needs of this vulnerable population.

Clinical Presentation, Echocardiographic Findings, Treatment Options Available for Ventricular Septal Defect in Children: A Study in a Tertiary Care Military Hospital

Introduction: Ventricular Septal Defect (VSD) is the most common congenital heart defects. Early diagnosis, appropriate treatment and transcatheter VSD closure with newer devices in favorable cases, decrease the need for surgical interventions and mortality. Objectives: To determine the clinical presentation, echocardiographic findings and the treatment options available for VSD in Paediatric Cardiology Department, Combined Military Hospital (CMH) Dhaka. Methods: It was a cross sectional observational study carried out in the department of Paediatric Cardiology, CMH Dhaka. Forty six isolated ventricular septal defect cases were diagnosed by echocardiography. Clinical features, echocardiographic findings and treatment options of all 46 cases were observed and recorded in pre formed data sheet and statistical analysis was done in statistical software SPSS version 25. Results: Among the 46 patients of VSD, majority 20(43.48%) were in age group 0-6 months. Most of them were male 24(52%). Common presenting features were dyspnea on exertion 23(50%), cough 30(65.20%), head sweating 23(50%), and feeding problem in 34(73.91%). VSD in 27(58.6%) patients were &lt;5mm, 15(32.6%) were 5-10mm and 4(8.7%) had size &gt;10mm. Most of the VSDs were perimembranous 31(67.39%). Among the 46 cases medical management was given to 20(43.5%), device closure was done in 10(21.7%) patients and surgical interventions done in 16(34.8 %) patients. Conclusion: Most of the VSD patients presented at an earlier age some of them were undergone device closure and some require surgical interventions that were not suitable for device closure. Patients those presented lately developed pulmonary hypertension. JAFMC Bangladesh. Vol 19, No 1 (June) 2023: 22-26

Apport du scanner cardiaque dans la prise en charge des cardiopathies congénitales : expérience de l'institut de cardiologie d'Abidjan à propos de 27 cas

Introduction and objectivesIn contrast to developed countries, cardiac CT is not widely used in West Africa for the assessment of congenital heart disease, and has only recently been introduced in Côte d'Ivoire. The lack of data prompted this study, the aim of which was to describe our experience of the contribution of CT to the management of congenital heart disease in the Ivorian cardiology setting. Patients and methodThis was a prospective study which took place in the pediatric cardiology department over a period of 9 months (September 2022 to June 2023) which included all patients with congenital heart disease explored by echocardiography and cardiac scan. ResultsThe average age was 5.7 ± 4.7 years with extremes of 5 months and 16 years. We noted a female predominance with a sex ratio of 0.52. The main heart diseases were: tetralogy of Fallot (37.1%), followed by pulmonary atresia with open septum (18.52%). The assessment of the anatomy of the pulmonary arteries (81.48%), the search for aortopulmonary collaterals (59.63%) and finally the assessment of the anatomy of the aorta (18.52%) were the main indications. The practice of cardiac CT scanning has enabled better accuracy in the diagnosis and management of congenital heart disease in several cases: an interruption of the aortic arch was found in 1 case initially suspected of hypoplasia of the aortic arch, three cases of partial abnormal pulmonary venous return and one case of total abnormal pulmonary venous return. Also, agenesis of the left pulmonary artery with birth anomaly of the right coronary artery in the assessment of tetralogy of Fallot was found in 1 case. The CT scan made it possible to specify the exact topography of the aortopulmonary collaterals in four cases of pulmonary atresia with an open septum. ConclusionCT has enabled better visualization of the arterial and venous pulmonary tree, identification and exact localization of aorto-pulmonary collaterals, precise description of the three-dimensional anatomy of the coronary arteries, and better precision of congenital anomalies of the aorta.

Improving Quality Health Care Services by Implementing DMAIC Approach in Paediatric Cardiology Department of Public Hospital of Sindh, Pakistan: A Case Study

Objectives: The hospital industry has changed its dimension from diagnosis to complete total patient satisfaction. This study aims to identify the critical variables that can enhance overall patient satisfaction in the hospital industry. The study identified three critical factors for any hospital to provide quality service. Methodology: The study adopted the lean Six Sigma process improvement methodology to identify the critical service factors in hospitals. Lean Six Sigma is a flexible problem-solving tool that comprehensively solves any problem. DMAIC process improvement methodology is used to define, measure, analyze, improve, and control the overall experience in the hospital. CTQ (critical to quality) was defined and represented by Kano methodology. After filtration through cause and effect analysis, three core dimensions and 25 sub-dimensions of critical quality were identified. The impacts of these 25 sub-dimensions were evaluated and prioritized using the Kano Model of patient satisfaction. This identifies the most critical service requirement. This study is an initiative for service quality improvement in the hospital industry. In the future, this technique can be modified and adopted by different public and private hospitals to enhance the practical implementation. Results: Findings indicate that healthcare organizations demonstrate enhanced system-wide capabilities, optimizing resource utilization. Understanding patient needs propels satisfaction and delight. Three overarching factors, with 25 sub-factors, significantly impact patient satisfaction, predominantly yielding positive effects across the categories. Conclusion: This research concludes that providing quality service is critical in the hospital industry. Quality service is pivotal in the evolving hospital industry, transitioning from a focus on diagnosis to holistic patient satisfaction. This study aims to identify critical variables essential for enhancing overall patient satisfaction, pinpointing key factors crucial for delivering quality service in any hospital.