Pediatric Cardiology Department Research Articles

Incidence, clinical profile and short term outcome of cerebral abscess in cyanotic congenital heart diseases

BackgroundBrain abscess is a serious infection of brain parenchyma in patients with cyanotic congenital heart disease (CCHD) and around 25–46 % of unrepaired CCHD patients develop brain abscess. Aim of this study was to determine the incidence, clinical features, microbiology and factors associated with early and short term outcome of cerebral abscess in CCHD. Methodology: This is a retrospective study, conducted at Pediatric cardiology department, National Institute of Cardiovascular Diseases (NICVD) Karachi. The data was collected from January 2019 to December 2021. All CCHD patients between 1 and 25 years of age were included. Data of patients with cerebral abscess was reviewed. ResultsAmong the 544 pediatric patients hospitalized in the last two years, brain abscesses were identified in 51 (9.3 %). Polycythemia (31.4 %) was the most significant contributing factor, especially in patients aged above 10 years. The most frequently seen CCHD was tetralogy of fallot (TOF) 60.8 %. Majority of the patients (84.3 %) had a single abscess while 15.7 % had multiple abscesses. E coli (9.7 %) was the most common isolated pathogen. Immediate complication identified was cerebral edema in 22 (43.1 %). Four patients (7.8 %) died, 47 (92.2 %) patients completed treatment course. 45.1 % had complete recovery however, 17 (33.3 %) had neurological deficits, 8 (15.7 %) had seizures and 2 (3.9 %) patients had residual abscess. Prolonged hospitalization was observed in patients of age group <10 years. ConclusionIn patients with underlying CCHD,early referrals and intervention are key to mitigating the severe consequences of cerebral abscesses and can drastically improve patient outcomes.

The Burden of Neonatal Referrals on a Pediatric Cardiology Service: A Local Center Experience.

Congenital heart disease (CHD) is a common occurrence in live births, with some exhibiting critical congenital heart disease; therefore, cardiology services should be available around the clock to ensure timely diagnosis and management. This study aims to describe the workload and the need for pediatric cardiac services in a maternity hospital for newborn referrals. Moreover, the study describes the indications for neonatal cardiology consultations. The prospective cohort study was conducted over four months, from January to April 2022, in the Prince Sultan Cardiac Center Al Qassim region of Saudi Arabia. Prince Sultan Cardiac Center's pediatric cardiology department provides cardiac services to the Maternity and Children Hospital Buraidah Al Qassim. Out of the total 2,606 live births during the study period, the cardiology team evaluated 352 neonates. Neonates less than 30 days of age who were born in the maternity hospital were enrolled in the study. The outborn babies referred from other centers as suspected congenital heart disease for whom a cardiac evaluation was done were excluded. In addition, babies assessed in the emergency room and born elsewhere were excluded. Only new consultations have been considered, excluding follow-up consultations. Data about patients' demographic, clinical and echocardiographic findings were recorded on Google Forms and converted to a Google spreadsheet. The Google spreadsheet's inbuilt statistical software was used for analysis. Categorical data were presented as percentages, and numerical data as median and range. The cardiology team evaluated 352 neonates from 2,606 live births over four months, accounting for 13.5 per 100 live births. The median weight was 2.8 kilograms, with a 0.5-4.3 kilogram range. Males comprised 187 (53%), and females comprised 165 (47%). Moreover, full-term, preterm, and post-term accounted for 236 (67%), 113 (32%), and 3 (0.8%) of patients, respectively. The common indications for neonatal cardiac referral were respiratory distress 60 (17%), infants born to diabetic mothers 50 (14%), abnormal fetal echocardiogram 49 (13.9%), family history of abortion or neonatal death 31 (8.8%), and congenital anomalies 30 (8.5%). Systolic murmur was the commonest clinical finding that prompted cardiology referrals 82 (23.2%), followed by desaturation 38 (10.7%) and dysmorphic features 31 (8.8%). Among the congenital cardiac defects, an isolated atrial septal defect (ASD) was seen in 66 (18.5%), isolated patent ductus arteriosus in 50 (14.2%), and ventricular septal defect in 21 (5.9%). Moreover, 13 (4.4%) lesions were critical CHDs. Finally, 27 (7.6%) had adiagnosis of pulmonary hypertension. Knowing the burden of neonatal cardiac assessment on pediatric cardiology services in any maternity center may help the healthcare authorities to allocate resources and optimize the delivery of cardiac services among the neonatal population. Properly allocating pediatric cardiologists to the needed centers may optimize neonatal cardiac services. Moreover, it may decide on the number of pediatric cardiologists that need to be trained each year to meet the requirements of neonatal cardiac services.

Factors linked with the delayed diagnosis of congenital heart diseases. A single center experience from a tertiary care hospital of Pakistan.

Objective: To note the factors linked with delayed diagnosis of congenital heart diseases (CHDs) among children. Study Design: Cross-sectional study. Setting: Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi, Pakistan. Period: September 2022 to February 2023. Material &amp; Methods: We analyzed children aged up to 15 years who presented for the first time with echocardiographic evidence for the diagnosis of CHDs. Socio-demographic data along with clinical information like gender, age, residential status, maternal education status, history of maternal fetal echocardiography and the frequency of delayed diagnosis of CHDs were noted on a specially made proforma. Parent or guardians were asked for possible reasons of delayed diagnosis for CHDs. Results: In a total of 250 children, the mean age was 2.33±3.81 years while 162 (64.8%) children were aged below 2 years. There were 138 (55.2%) boys. Residential status of 159 (63.6%) children was rural while maternal educational status of 99 (39.6%) was illiterate. There were 164 (65.6%) children who were diagnosed to have cyanotic CHDs while 86 (34.4%) had acyanotic CHDs. Frequency of delayed diagnosis was noted among 182 (72.8%) children. In these 182 cases, delayed first consultation was the commonest factor behind delayed diagnosis noted in 78 (42.9%) children while missed diagnosis by the pediatrician was the 2nd most common factors linked with the delayed diagnosis of CHDs as described by 62 (34.1%) parents. Conclusion: Almost 3/4th of the studied children had delayed diagnosis of CHDs while cyanotic CHDs were the most common CHD types. Delayed first consultant and missed diagnosis by the pediatrician were the most common factors linked with delayed diagnosis of CHDs.

Clinical profile of infective endocarditis: a single centre experience

Background: Infective endocarditis (IE) is an illness which still has a high rate of morbidity and mortality despite the availability of improved diagnostic and treatment amenities in the developing world. With an increasing number of children with congenital heart disease (CHD) undergoing corrective treatments, improved pediatric intensive care, better antimicrobial treatments, and a relative decrease in rheumatic heart disease (RHD) over the years, the epidemiology of pediatric IE in Bangladesh may be undergoing a change. The present study aims to describe the epidemiological, clinical, laboratory profile and outcome of IE among selected patients. Methods: This prospective cross-sectional study was conducted with a total of 35 patient of IE from January 2020 to December 2021, who were admitted in pediatric cardiology department, Bangladesh Shishu hospital and institute were enrolled in this study which was diagnosed according to modified Duke criteria. Results: There were 35 children diagnosed with IE during this period. The mean age at presentation was 84 months (range: 2-216 months). CHD (n=28/35, 80%) was the most common predisposing condition. A total of 20 % (7/35) patients had no preexisting structural heart disease. Staphylococcus aureus was the most common etiological agent. Blood culture positive IE was (n=18, 51.4%), blood culture-negative IE was (n=17, 48.6 %). Six patients (17.1%) died during the hospital stay. Conclusions: Increasingly younger children are being diagnosed with IE in Bangladesh and a significant number of them are in the setting of a structurally normal heart.

Percutaneous Transcatheter Retrieval of Central Venous Port Fragments in Pediatric Patients; A Single-center Experience From the Pediatric Cardiology Department.

Split/fracture and embolization of central venous/shunt catheters are rare but serious complications in children. Percutaneous retrieval of intravascular foreign bodies is an important minimal invasive treatment. This study is aimed to represent our largest pediatric sample experience till now of 17 years from a single institution. Another aim is to compare the results regarding the removal or leaving in place of embolized or ruptured intravascular or cardiac venous catheter parts in children. A total of 26 cases were included in this study. Any pediatric patient with normal coagulation parameters and a fractured catheter fragment was included in this study. Other intravascular foreign bodies related to interventional devices and/or pacemaker/implantable cardiac defibrillator leads were excluded from this study. Twenty-six patients, of whom 25 had oncologic diseases and 1 had a ventriculoatrial shunt, were included. The median age was 83.5 months (between 20 mo and 18 y) at treatment.Superior vena cava (9 cases), followed by the right atrium (5 cases), were the most two common sites of embolization for cardiovascular foreign bodies. The success rate of percutaneous retrieval was 92.3% in all patients. There were neither complications nor deaths. The retrieval technique revealed a predisposition for extraction through the femoral vein (96.1%) and using snare techniques (100%). Additional catheters like pigtail, National Institutes of Health, or ablation catheters were used for stabilization in selective cases in which the permanent central venous fragments stuck to the vessels. A tractional maneuver and capturing the ruptured material in the middle were other trick points for successful retrieval. Patients were asymptomatic in 76.9% of cases (20/26). Percutaneous retrieval of cardiovascular foreign bodies is a reasonable, safe, and effective way in children when the catheter fragments are free and mobile. It should be considered the preferred treatment option instead of surgery. In patients where catheter fragments are stuck and are adherent to vessels, it could be left, and followed up by anticoagulation. Novel techniques accompanied by an experienced team could be helpful in difficult cases.

Investigation of the presence of atopy in children visiting the paediatric cardiology department due to chest pain.

The cases of chest pain in children are usually not of cardiac origin. To investigate asthma and other atopic diseases in children with chest pain not of cardiac origin. Children aged 6-18 years who were seen for chest pain were included in the study. Haematologic parameters, pulmonary function tests, and skin prick tests were performed. Atopic diseases and environmental factors were investigated. The non-cardiac chest pain group (Group 1) included 88 children (female: 53.4%) with a mean age of 11.9 ± 3.4 years; the control group (Group 2) included 29 children (female: 53.8%) with a mean age of 11.4±2 years (p > 0.05). A family history of atopy (22.7%) and skin prick test positivity (28.4%) was more common in Group 1 than Group 2 (p = 0.006 and p = 0.017, respectively). The rate of presence of all environmental factors except stove use and mould was significantly higher in Group 1 (54.5%) than Group 2 (3.4%) (p < 0.001). Asthma was diagnosed in 44.3% and allergic rhinitis in 9.1% of patients in Group 1. Idiopathic chest pain, musculoskeletal system disorders, gastroesophageal reflux, and pneumonia were identified in 23.9%, 11.4%, 8%, and 3.4% of patients in Group 1, respectively. In this study, the most common cause of non-cardiac chest pain was asthma. The local prevalence of asthma is higher than normal, and this may have affected the results of this study. A detailed history and physical examination will accurately establish the cause of chest pain in most children.

Pattern of Congenital Heart Diseases among Less than 5 Years Old Children of Bangladesh-An Observational Study from a Pediatric Cardiac Center

Introduction: Congenital Heart Disease (CHD) is the most common congenital problems. CHD is about 25% of all congenital malformations and is the most common type of heart disease among children. It may present in different ages from birth to adolescence. Worldwide, CHD is relatively common with a prevalence ranging from 1.0-6.6 per 1000 live births. Aim of the Study: The aim of this study was to find out the pattern of congenital heart diseases among less than 5 years old Children of a pediatric cardiac center. Methods: This was a retrospective observational study and conducted in the Department of pediatric cardiology of Combined Military Hospital Dhaka, Bangladesh during the period from July, 2018 to July, 2020. A total of 1000 patients from outdoor and indoor patient registry who were diagnosed and treated as a case of congenital heart disease within the mentioned period were included in the study. Result: Majority of our patients was male (58%) compared to female (42%). Among all patients, 29.80% had VSD, followed by 20.4% &amp; 14.9% had ASD &amp; TOF respectively and 72% &amp; 28% of our patients had Acyanotic &amp; cyanotic CHD respectively.VSD &amp; TOF was the most common in patients with acyanotic &amp; cyanotic CHD respectively. Recurrent RTI, pneumonia, heart failure, infective endocarditis, and Cyanotic spell are common complications of congenital heart disease. Down syndrome and congenital rubella syndrome are the main syndromic association in congenital heart diseases. The majority (97%) of our patients improved, and 3% died. Conclusion: Our study focuses on the congenital heart diseases of children of Bangladesh. Among the acyanotic congenital heart disease, VSD is the maximum &amp; among the cyanotic congenital heart disease TOF is the highest in number. With early diagnosis, treatment and regular follow up, mortality rate is very low and the prognosis is excellent.

Comparison of Short-Term Outcomes of Transcatheter versus Surgical Closure of Atrial Septum Defect at a Tertiary Cardiac Care Facility

Objective: To compare the short-term outcomes of transcatheter versus surgical closure of atrial septal defect (ASD) at a tertiary cardiac care facility in South Punjab, Pakistan.&#x0D; Study Design: Prospective comparative study.&#x0D; Place and Duration of the Study: Department of Paediatric Cardiology and Paediatric Cardiac Surgery, Chaudhary Pervez Elahi Institute of Cardiology, Multan Pakistan, from Jul 2019 to Jun 2021.&#x0D; Methodology: A total of 110 patients with moderate to large isolated secundum ASDs without severe pulmonary hypertension and planned to undergo closure of secundum ASD by either surgical approach or device closure were included. All surgical and transcatheter procedures were performed adopting standard techniques. Intra-procedural or post-procedural outcomes were noted.&#x0D; Results: In a total of 110 patients, 59(53.6%) underwent transcatheter closure of ASD, while surgical closure was done in the remaining 51(46.4%) patients. Post-procedural complications were significantly more in the Surgical Closure Group in comparison to the Transcatheter Closure Group, [14(27.5%) vs 5(8.5%) (p=0.026)]. None of the cases in the Transcatheter Closure Group required inotropes in the post-procedure period in comparison to 24(47.1%) cases (p&lt;0.001). The total cost of treatment was significantly higher in transcatheter closure patients compared to surgical closure patients (1504±60.4 US$ versus 1448±75.4 US$, p&lt;0.001). Overall, successful closure was noted in 108(98.2%) cases. No mortality was recorded.&#x0D; Conclusion: Overall, short-term outcomes of transcatheter ASD closure were comparable to the surgical approach, but patients with transcatheter ASD closure had significantly less duration of hospitalization and low rates of post-procedural complications.Keywords: Atrial septum defect, Mortality, Surgical closure, Transcatheter closure.

Relationship Between Transthoracic Echocardiography and Transesophageal Echocardiography in the Anatomic and Hemodynamic Assessment of Secundum Atrial Septal Defects in Children and Adults

Objective: To determine the relationship between transthoracic and transesophageal echocardiography in the anatomic and hemodynamic assessment of secundum atrial septal defects in children and adults.&#x0D; Study Design: Cross-sectional study.&#x0D; Place and Duration of Study: Department of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan, from Jul 2020 to Jun 2021.&#x0D; Methodology: Seventy-seven patients above three years of age with secundum atrial septal defects were included. Linear regression was used to determine any statistically significant difference between transthoracic and transesophageal echocardiography approaches.&#x0D; Results: Of 77 patients, there were 37(48.1%) male and 40(51.9%) female. No significant difference was noted in terms of any of the clinical parameters during transthoracic echocardiography and transesophageal echocardiography. As per transthoracic echocardiography, 35(45.5%) were noted to be deviceable, while in transesophageal echocardiography, 36(46.8%) patients were found to be deviceable (p=0.872). No emergency exploration or deaths occurred. Linear regression showed no statistically significant difference between transthoracic echocardiogram and transesophageal echocardiography findings in terms of atrial septal defects size (p=0.226), total septal length (p=0.930), RV systolic pressure (p=0.440), RV functions and TAPSE (0.748) except LV ejection fraction function findings (p&lt;0.001).&#x0D; Conclusion: Anatomic and hemodynamic assessment of secundum atrial septal defects in children and adults correlated well with transthoracic and transesophageal echocardiography.

Prenatal diagnosis, 4D spatiotemporal image correlation imaging, pregnancy, and postnatal outcome of partial Uhl's anomaly.

Journal Article Accepted manuscript Prenatal diagnosis, 4D spatiotemporal image correlation imaging, pregnancy, and postnatal outcome of partial Uhl’s anomaly Get access Balaganesh Karmegaraj, DM Balaganesh Karmegaraj, DM Sowmi Fetal heart centre, Tirunelveli, Tamilnadu and Fetal cardiology Division, Department of Paediatric Cardiology, Amrita institute of Medical Sciences and research centre, Kochi, Kerala, India Address for correspondence: Dr Balaganesh Karmegaraj MD., DM, Clinical Assistant Professor, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, AIMS Ponekkara PO, Kochi, Kerala, India Pin: 682 041, Tel: +91 484 285 3570 , Fax. +91 484 2802020, Mobile: +9197461 31803. Email: pedsheartkbg@gmail.com https://orcid.org/0000-0001-8728-2140 Search for other works by this author on: Oxford Academic PubMed Google Scholar European Heart Journal - Cardiovascular Imaging, jead133, https://doi.org/10.1093/ehjci/jead133 Published: 07 June 2023 Article history Received: 06 May 2023 Editorial decision: 04 June 2023 Accepted: 06 June 2023 Published: 07 June 2023

First results of maintenance of register in electronic form in pediatric patients with hypertrophic cardiomyopathy

Background. The problem of managing children with hypertrophic cardiomyopathy (HCM) remains relevant due to the high risk of sudden cardiac death (SCD). Registers of patients with HCM contribute to a better understanding of the course of the disease and its outcomes.Objective. To study the structure of CMP with a hypertrophic phenotype, as well as to identify clinical, molecular and genetic features of the course of HCM and outcome in children based on anamnestic, clinical and instrumental data from an electronic database.Design and method/ Currently, the database includes complete information on 80 children from 0 to 18 years of age inclusive with a hypertrophic phenotype of CMP. The study was performed on the basis of the Department of Pediatric Cardiology and Medical Rehabilitation of the V.A. Almazov” of the Ministry of Health of Russia. The database contains the history of the disease and family history, the results of clinical and laboratory-instrumental examination, the results of molecular genetic research. The register is dynamically updated.Results. The mean age of onset of HCM was 1.75 [0.02; 10.00] years. There were more boys than girls — 54 (67.5 %). The main complaints were decreased exercise tolerance in 35 (43.8 %) children and shortness of breath in 30 (37.5%) children. All children had signs of heart failure (HF): 53 (66.3 %) had signs of functional class 2 HF, 26 (32.5 %) — functional class 1 and 1 (1.2 %) — functional class 3. Ventricular arrhythmias were recorded in 23 (29 %) children, unstable ventricular tachycardia in 2 (2.5 %) children. Myocardial fibrosis of the left ventricle according to the results of MRI of the heart was found in 60.7 %. A lethal outcome was registered in 5 % (n = 4) of cases on average at 241 ± 117 days of life. All the deceased had phenocopies of HCM.Conclusion. Maintaining an electronic database of patients with HCM will allow a better understanding of the influence of factors, including genetic ones, on the course, outcomes, and prognosis of the disease in the pediatric population.

Thinking of the Moms.

Department of Pediatric Cardiology, Cleveland Clinic, Cleveland, OH. Dr. Yildirim has disclosed that she does not have any potential conflicts of interest. For information regarding this article, E-mail: [email protected]

Evaluating Types of TAPVR on Echocardiography and CT Angiography in Paediatric Patients Admitted in Tertiary Care Hospital: A Comparative Study

Objective: To evaluate the types of TAPVR on Echocardiography and CT Angiography in Paediatric Patients Study design: A comparative study Place and Duration: Pediatric cardiology department and CT Angiography department of the National Institute of Cardiovascular Diseases (NICVD) Karachi from January 2019 to December 2021 Methodology: Patients who have TAPVR confirmed by CT angiography and detected by echocardiography were included in the research. Cases having anomalies in the systemic veins were eliminated. Demographic variables (age, gender, weight, etc.), echocardiographic results (such as type of TAPVR, obstructive and non-obstructive), and CT angiography findings were collected for the study using a preset structured proforma. We evaluated the diagnostic disparity between the two modalities. Results: In the present study there were 107 patients, 67.3% were male gender, 56.1% with age less than 6 months, the median age was 6 months, median weight was 4.5 kg. On echocardiography, there were 83.2% non-obstructive, 47.7% supracardiac and 36.4% cardiac TAPVR. On CT angiography, there were 66.4% were non-obstructive, 53.3% supracardiac and 23.4% cardiac TAPVR. Of the associated cardiac diseases, in common 86.9% were found with atrial septal defect, 13.1% were persistent left superior vena cava, 12.1% were patent ductus arteriosus, 8.4% were a ventricular septal defect, 6.5% were of pulmonary atresia, and 3.7% were complete endocardial cushion defect. Conclusion: As compared to echocardiography, CT angiography is more reliable for the detection of obstructive and mixed types of TAPVR. Detection of the supracardiac type of TAPVR is also almost the same as on echocardiography and CT angiography. As compared to CT angiography, echocardiography over diagnoses the non-obstructive type of TAPVR. CT angiogram is also useful in detecting other associated lesions such as heterotaxy, abnormal coronaries, presence of bilateral superior vena cava and coronary sinus. Keywords: TAVPR, echocardiography, CT angiography, cardiac disorders

Neurological consequences and short–term outcome of children with diagnosis of cyanotic congenital heart diseases at a Tertiary Care Cardiac Setup of Pakistan: A single center study.

Objective: To determine the neurological consequences and short–term outcome of children with diagnosis of cyanotic congenital heart diseases (CHDs) at a tertiary care cardiac setup of Pakistan. Study Design: Single Center Observational Cohort study. Setting: Department of Pediatric Cardiology, National Institute of Cardiovascular Disease (NICVD), Karachi, Pakistan. Period: November 2021 to October 2022. Material &amp; Methods: A total of 80 children of both genders, aged between 1 month to 12 years with known cyanotic CHD as per 2D transthoracic echocardiography were admitted in Pediatric Cardiology Department of NICVD, Karachi and were considered. At the time of enrollment, demographic characteristics along with presenting complaints were noted. Necessary baseline laboratory and radiological investigations were ordered and clinical neurological examination was done. Children were discharged and short term outcomes were labeled after 3 months follow up. Results: In a total of 80 children with cyanotic CHDs, most common underlying diagnosis was tetralogy of fallot, found in 41 (51.3%) children. The CT brain revealed ischemic infarct, brain abscess and gross oedema as the most frequent observations revealed in 47 (58.8%), 40 (50.0%) and 37 (46.3%) children. Surgical management of underlying neurological abnormality was advised in 23 (28.8%) children. After 3 months follow-up, complete recovery was observed among 43 (53.8%) children. CT findings at the time of enrollment as haemorrhagic infarct (p&lt;0.001), brain abscess (p=0.001) and atrophic changes (p=0.032) were significantly associated with incomplete recovery. Surgery as a mode of treatment for the underlying neurological disease was associated with good recovery outcomes (p&lt;0.001). Conclusion: Most common underlying diagnosis among children with cyanotic CHDs was tetralogy of fallot. CT findings at the time of enrollment as haemorrhagic infarct, brain abscess and atrophic changes, as well as hypotonia, down going plantars and developmental delay at the time of presentation were significantly linked with incomplete recovery.

Outcome of Management of Tetralogy of Fallot in a Cardiac Center of a Tertiary Care Hospital in Bangladesh

Background: Congenital heart diseases (CHD) represent one of the major groups of birth defects and make up approximately 1% of human malformations. Incidence of congenital heart disease is 8-10/1000 live births. The incidence is higher in premature infants. In Bangladesh incidence of congenital heart diseases was found up to 25/1000 live birth in a one year study conducted in CMH Dhaka. Congenital cyanotic heart disease accounts for less than 25% of total congenital cardiac defects. In TOF the lungs are under-perfused as blood shunts from right to left bypassing the lungs. TOF constitutes 5-7% of congenital heart disease. It is the commonest congenital cyanotic heart disease in Bangladesh. Objective: The aim of this study was to assess outcome of management of Tetralogy of Fallot in a cardiac center of a tertiary care hospital in Bangladesh. Methodology: It was a retrospective observational study and was conducted in the department of Pediatric Cardiology of Combined Military Hospital (CMH), Dhaka, Bangladesh from January 2015 to December 2016. We included 50 patients with TOF taking treatment from our department. All the patients were divided into three groups according to treatment type. Medical management group, palliative surgery group and total intra cardiac repair group and their outcome were assessed. Results: In our study we found 6 (12%) patients were in medical management group, 8 (16%) were in palliative surgery group and 36 (72%) were in total intra cardiac repair group. Among them 33 (66%) were male and 17 (34%) were female. Majority patients 30 (60%) were in less than 3 years of age. In medical management group all the patients had oxygen saturation &lt;80%, 4 (66.68%) patients reduced frequency of hyper-cyanotic spell, 1 (16.67%) patient had cerebral abscess. In palliative surgery group 6 (75%) patients had &gt;90% oxygen saturation, 2 (25%) had &lt;90% oxygen saturation, tet spell reduced in all the cases. After total intra cardiac repair no patient had .....

Development of PR Restenosis after Percutaneous Balloon Pulmonary Valvuloplasty

Background: Pulmonary stenosis is not an uncommon congenital heart disease in our population. Various age groups present with severe pulmonary stenosis require intervention either surgical or percutaneous catheter-based. Often lifesaving surgical intervention is not feasible considering procedural risk, the patient’s physical condition, and young age. Percutaneous balloon pulmonary valvuloplasty is safe, effective, almost painless, economical, and less hospital stay in any age group with immediate and long-term success. This study aimed to analyze the rate of development of (Pulmonary Regurgitation) PR restenosis after Percutaneous Balloon Pulmonary Valvuloplasty (PBPV). Material Methods: This hospital-based prospective observational study was conducted in the Department of Paediatric Cardiology Combined Military Hospital (CMH), Dhaka from January to December 2021. 0-18 years aged 50 population with PS who were referred to the Paediatric Cardiology Department of CMH Dhaka during the study period were selected as study subjects as per inclusion criteria. The purposive Sampling technique was used in this study. Data were collected with a predesigned standard data collection sheet. Statistical significance was set as a 95% confidence level at a 5% acceptable error level. Categorical variables were expressed as frequency and percentages. Mean, standard deviation, and/or medians with interquartile ranges were used to express the continuous variables. Paired t-test was performed in categorical comparison. A p-value of &lt;0.05 was considered statistically significant. Collected data were analyzed using SPSS Version 24. Written informed consent was taken from every parent. Ethical clearance was taken from the Ethics Review Committee of CMH Dhaka for conducting the study. Results: 50(6.39%) had isolated valvular PS. The international standard for the prevalence of isolated PS is 8-10% which is very close to this study’s result. In this study, male predominance was found with the male-female ratio of 3:2 though there was no gender dominance found in other studies. Out of 50 cases, 3(6%) are neonates,7(14%) are infants, 27 (54%) are 1-5 years aged, and 13(26%) are more than 5 years aged. A total of 13(26%) cases developed PR, and all 13 patients developed trivial PR on D1 echo, out of them 1 patient developed mild PR on D30 Echo but in D90 and D180 Echo patients persisted the same mild PR. Out of 50 cases, 46(92%) were successful and 4 cases were procedure failed. None of the successful cases developed restenosis. Out of 4 procedure failed cases 1 patient developed restenosis on D30 follow up and 2 patients develop restenosis on the D90 follow-up. At D180 follow up none of the patients developed restenosis. All the restenosis cases had dysplastic pulmonary valves. One patient’s PS gradient persisted at 30-50 mm of Hg throughout the follow-up period. Overall restenosis in 180-day follow-up is 3(6%) and the remaining 47(94%) cases lead a healthy life in 6 months follow-up. Reballooning was done immediately after diagnosis of restenosis in stenosed cases. Conclusion: Immediate and intermediate-term (6 months) follow-up with Doppler echocardiography showed excellent outcomes in all cases except 3 (6.0%) restenosis cases and 13 (26.0%) insignificant PR cases. The baby who all had restenosis, had the risk factor, like- dysplastic PV. It may be concluded that balloon valvuloplasty is the procedure of choice for moderate, severe, and critical pulmonary valve stenosis.

Clinical features and echocardiographic findings in children with ebstein’s anomaly: A descriptive case-series of 48 cases.

Objective: To determine the clinical features and echocardiographic findings among children presenting with Ebstein’s anomaly (EA). Study Design: Descriptive Case-series. Setting: Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases (NICVD), Karachi Pakistan. Period: July 2021 to June 2022. Material &amp; Methods: Children of both genders aged above 1 month up to 18 years, having echocardiographic confirmed EA were analyzed. Demographic data along with clinical features, presenting symptoms and echocardiographic findings were noted in all case. Results: In a total of 48 cases of EA, 38 (79.2%) were female. The mean age was 8.4±4.21 years. Cyanosis, exertional dyspnea, easy fatigue ability and exercise intolerance were the most frequent symptoms reported in 31 (64.6%), 27 (56.3%), 26 (54.2%) and 24 (50.0%) patients respectively. Arrhythmias and heart failure were the most frequent clinical features noted in 29 (60.4%) patients each. Small atrial septal defect (ASD) was noted in 27 (56.3%) patients while ventricular septal defect in 19 (39.6%) cases. There were 11 (22.9%) patients who presented timely with diagnosis of EA while 37 (77.1%) patients had late diagnosis. Conclusion: Majority of the patients with Ebstein’s anomaly were female. Cyanosis, exertional dyspnea, easy fatigue ability and exercise intolerance were the most frequent symptoms whereas arrhythmias and heart failure were the most frequent clinical features observed. ASD was the most commonly noted associated CHDs observed in almost half of the Ebstein’s anomaly case. Almost 3/4th of the patients had late diagnosis.

Clinical aspects and targeted inspiratory muscle training in children and adolescents with Fontan circulation: a randomized controlled trial.

Since 1968, thousands of patients with a morphologically or functionally univentricular heart have been treated with a total cavopulmonary connection/Fontan operation. Because of the resulting passive pulmonary perfusion, blood flow is assisted by the pressure shift during respiration. Respiratory training is known to improve exercise capacity and cardiopulmonary function. However, there are limited data on whether respiratory training can also improve physical performance after Fontan surgery. The aim of the present study was to clarify the effects of six months of daily home-based inspiratory muscle training (IMT) aimed at increasing physical performance by strengthening respiratory muscles, improving lung function and peripheral oxygenation. In this non-blinded randomized controlled trial, the effects of IMT on lung capacity and exercise capacity were measured in a large cohort of 40 Fontan patients (25% female; 12.3±2.2 years) who were under regular follow-up by the outpatient clinic of the Department of Congenital Heart Defects and Pediatric Cardiology of the German Heart Center Munich. After a lung function test and a cardiopulmonary exercise test, patients were randomly assigned in a parallel arm design to either an intervention group (IG) or a control group (CG) via stratified and computer-generated letter randomization from May 2014 to May 2015. The IG completed a daily, telephone-monitored IMT of three sets of 30 repetitions for six months with an inspiratory resistive training device (POWERbreathe medic®), the CG continued their usual daily activities without an IMT until the second examination within the timeframe of November 2014 until November 2015. After six months of IMT, lung capacity values in the IG (n=18) did not increase significantly compared to the CG [n=19; ΔFVC: IG: 0.21±0.16 l vs. CG 0.22±0.31 l; P=0.946 (CI: -0.16, 0.17); ΔFEV1: CG: 0.14±0.30 vs. IG: 0.17±0.20 P=0.707 (CI: -0.20, 0.14)]. Exercise capacity did not improve significantly, yet the maximum workload achieved trended to improve with an increase of 14% in the IG vs. 6.5% in the CG [P=0.113 (CI: -15.8, 1.76)]. There was a significant increase of oxygen saturation at rest in the IG compared to the CG [IG: 3.31%±4.09% vs. CG: 0.17%±2.92%, P=0.014 (CI: -5.60, -0.68)]. Compared to the CG, the mean oxygen saturation at peak exercise no longer dropped below 90% in the IG. This observation is thus not statistically significant, yet of clinical relevance. The results of this study show benefits of an IMT in young Fontan patients. Even if some data are not statistically significant, they may still be clinically relevant and may contribute to a multidisciplinary approach in patient care. IMT should therefore be an additional target and integrated into the training program to improve the prognosis of Fontan patients. German Clinical Trials Register; DRKS.de; registration ID: DRKS00030340.

Effect of Pomegranate Extract On N-Terminal Pro Brain Natriuretic Peptide and Asymmetric Dimetylarginine Levels in Children with Pulmonary Artery Hypertension in Acyanotic Congenital Heart Disease

Background: Pulmonary arterial hypertension (PAH) is one of the complications due to acyanotic congenital heart disease. New treatment approach using natural ingredients from plants has been demonstrated in vitro to prevent and improve vascular remodeling in PAH. Objective: This study aimed to analyze the effect of pomegranate extract on N-terminal pro brain natriuretic peptide (NT pro BNP) and asymmetric dimethylarginine (ADMA) levels in children with PAH in acyanotic CHD. Method: This study is a double-blind, quasi-experimental text with pre-test, post-test, and control group approach to children aged one month to 10 years old in Outpatient Department of Pediatric Cardiology Department/Pediatrics Department RSUD Dr. Soetomo in the period of February-October 2022, alongside met the inclusion criteria. Data were analyzed using Wilcoxon and Mann-Whitney test with 95% CI. Result: The study cohort included 15 subjects in the placebo group and 15 subjects in pomegranate extract group. The study was predominantly female (53,3%) with a mean age of each group, i.e., placebo group was 49 months, and pomegranate extract group was 58 months. The mean level of NT pro-BNP in the pomegranate extract group was 29.31 ± 33.55 ng/L (p= 0.008) and its mean ADMA level was 5619 ± 6166 ng/L (p= 0.173). As for the difference of NT pro-BNP and ADMA level changes after the intervention did not show a significant difference (p-0,330; p=0,885, respectively). Conclusion: No significant effect towards NT pro- BNP and ADMA levels found with giving pomegranate extract to children with PAH due to acyanotic CHD.

Circular shunt in fetal absent pulmonary valve with tricuspid stenosis.

Pediatrics InternationalAccepted Articles e15480 CLINICAL NOTE Circular shunt in fetal absent pulmonary valve with tricuspid stenosis Tomiko Toyokawa, Tomiko Toyokawa Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Yao Municipal Hospital, Yao, Osaka, JapanSearch for more papers by this authorNoboru Inamura, Corresponding Author Noboru Inamura ki88mi@gmail.com orcid.org/0000-0002-6270-1158 Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan Correspondence Noboru Inamura, Department of Pediatrics, Kindai University, Faculty of Medicine; 377-2 Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan, Phone: +81-72-366-0221; Fax: +81-72-368-1566. Email: ki88mi@gmail.comSearch for more papers by this authorYukiko Kawazu, Yukiko Kawazu Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Fukuyama City Hospital, Fukuyama, Hiroshima, JapanSearch for more papers by this authorFutoshi Kayatani, Futoshi Kayatani Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, JapanSearch for more papers by this author Tomiko Toyokawa, Tomiko Toyokawa Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Yao Municipal Hospital, Yao, Osaka, JapanSearch for more papers by this authorNoboru Inamura, Corresponding Author Noboru Inamura ki88mi@gmail.com orcid.org/0000-0002-6270-1158 Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan Correspondence Noboru Inamura, Department of Pediatrics, Kindai University, Faculty of Medicine; 377-2 Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan, Phone: +81-72-366-0221; Fax: +81-72-368-1566. Email: ki88mi@gmail.comSearch for more papers by this authorYukiko Kawazu, Yukiko Kawazu Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, Japan Department of Pediatrics, Fukuyama City Hospital, Fukuyama, Hiroshima, JapanSearch for more papers by this authorFutoshi Kayatani, Futoshi Kayatani Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka, JapanSearch for more papers by this author First published: 19 January 2023 https://doi.org/10.1111/ped.15480 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi:10.1111/ped.15480. AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future.e15480 RelatedInformation