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Introduction: Permanent pacemaker implantation (PPI) in neonates is challenging with respect to indications, device selection, implantation technique, and long-term outcomes. Complex anatomy, the need for long-term pacing with high rates, and a problematic postoperative period are the major problems. Methods: We prospectively followed up 22 newborns who underwent PPI below 28 days of life at our institute. Results: The median age at implantation was 2 days (interquartile range 1-9 days), and 9% were born preterm. The average heart rate before implantation was 46.4 ± 7.2 bpm. Maternal lupus antibodies were positive in 8 (36.4%) neonates, whereas 11 (50.0%) had associated congenital heart disease. Nineteen neonates underwent single chamber (VVI) and three underwent dual chamber (DDD) pacemaker implantation. Over a median follow-up of 46 months (range 2-123 months), the average ventricular pacing percentage was 87.5 ± 24.9%, with a stable pacing threshold. Seven children underwent pulse generator replacement due to battery depletion at a median age of 47 months. Pacing-induced ventricular dysfunction was seen in five children at a median age of 23.6 months, and two underwent upgradation to cardiac resynchronization therapy. Overall mortality was 13.6%, all due to tissue hypoperfusion and lactic acidosis in the postimplantation period. Conclusions: PPI in neonates has a favorable outcome with excellent lead survival. Overall mortality is 13.6%, which is predominantly in the postimplantation period and related to myocardial dysfunction. [ABSTRACT FROM AUTHOR]

Background : Open-heart surgery is challenging in preterm neonates and infants, and its feasibility in low-resource settings has not been defined. We describe our institutional experience with open-heart surgeries performed on consecutive preterm infants. Materials, Methods and Results : This was a single-center retrospective cohort from a tertiary hospital in Southern India and included consecutive preterm neonates (<37 weeks) admitted for open-heart surgery. This report is limited to babies who were <3 months at the surgery. The salient features of the 15 preterm included twin gestation: 7 (46.7%); median gestational age at birth: 35 weeks (28-36 weeks); median corrected gestational age at surgery: 37 weeks (33-40 weeks); birth weight: 1.75 kg (1.0-2.6 kg); weight at surgery: 1.8 kg (1.2-2.9 kg); and small for gestational age: 12 (80%). The heart defects included transposition of the great arteries (7), total anomalous pulmonary venous return (3), large ventricular septal defect (VSD) (1), and VSD with coarctation of the aorta (4). Eleven (73%) were mechanically ventilated preoperatively and five had preoperative sepsis. The mean cardiopulmonary bypass time was 169.7 ± 61.5 min, and cross-clamp time was 99.7 ± 43.8 min. There was no inhospital mortality; one baby expired during follow-up at 1 month. Postoperative mechanical ventilation duration was 126.50 h (84.25-231.50 h), and intensive care unit stay was 13.5 days (9-20.8). The total hospital stay was 39 days (11-95 days). Two children (13.3%) had postoperative sepsis. Conclusion : Through collaborative multidisciplinary management, excellent outcomes are feasible in low-resource environments for selected preterm neonates undergoing corrective open-heart operations. [ABSTRACT FROM AUTHOR]

In response to the survey among early career pediatric cardiologists from India and the accompanying editorial, we invited comments and suggestions from thought leaders and senior functionaries in the field. We have summarized the thoughts and suggestions as a mini-symposium. [ABSTRACT FROM AUTHOR]

Objective: To study the applicability of on table extubation (OTE) protocol following congenital cardiac surgery in a low-resource setting and its impact on the length of intensive care unit (ICU) stay, hospital stay, hospitalization cost, parental anxiety, and nurse anxiety. Materials and Methods: In this prospective, nonrandomized, observational single-center study, we included all children above 1 year of age undergoing congenital cardiac surgery. We evaluated them for the feasibility of OTE using a prespecified protocol following separation from cardiopulmonary bypass. The data were prospectively collected on 60 children more than 1 year of age, belonging to the Risk Adjustment for Congenital Heart Surgery 1, 2, 3, and 4 groups and divided into two groups: those who underwent successful OTE and those who were ventilated for any duration postoperatively (30 children in each group). Duration of hospital stay, ICU stay, and total hospital cost were collected. Anxiety levels of the primary caregiver (nurse) in the ICU and the mother were assessed immediately after the arrival of the child in the ICU using the State Trait Anxiety Inventory (STAI). Results: Children who were extubated immediately following congenital cardiac surgery had significantly shorter ICU stay (median 20 [19, 22] h vs. 22 [20, 43] h [P < 0.05]). Patients extubated on table had a significant reduction in hospital cost {median Rs. 161,000 (138,330; 211,900), approximately USD 1970 (P < 0.05)} when compared to children who were ventilated postoperatively {median Rs. 201,422 (151,211; 211,900), approximately USD 2464}. The anxiety level in mothers was significantly less when their child was extubated in the operating room (STAI 36.5 ± 5.4 vs. 47.4 ± 7.4, P < 0.001). However, for the same subset of patients, anxiety level was significantly higher in the ICU nurse (STAI 46.0 ± 5.6 vs. 37.8 ± 4.1, P < 0.05). Conclusion: OTE following congenital cardiac surgery is associated with a shorter duration of ICU stay and hospital stay. It also reduces the total hospital cost and the anxiety level in mothers of children undergoing congenital heart surgery. However, the primary bedside caregiver during the child's ICU stay had increased anxiety managing patients with OTE. [ABSTRACT FROM AUTHOR]

Introduction and Aims : Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome. Methods and Results : We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5--9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up (n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias (P value-- 0.05). The 5-year survival rate of DCM patients was 59%. Conclusion : A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts. [ABSTRACT FROM AUTHOR]

Background : We present a large Indian series of Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) infection. The aim of the study is to present the incidence and pattern of cardiac involvement in children with MIS-C and their short-term follow-up. Methods and Results : Consecutive 144 children younger than 18 years of age diagnosed with MIS-C referred for cardiac evaluation between June 1 and November 30, 2021, were included and were followed up till February 2022. In addition to the demographics, details of COVID-19 infection, and biomarkers, their cardiovascular assessment (echocardiogram and electrocardiogram) was documented at baseline and on follow-up. The median age of children with MIS-C was 60 (24-104) months. Abnormal cardiac imaging was noted in 59% of children. Ventricular dysfunction was noted in 13.9% and coronary abnormalities were noted in 25.7% of children. The median duration when the first cardiac abnormality was reported was 7 (5-10) days. The distribution of age categories between children with and without cardiac abnormality was comparable. Children with cardiac abnormalities were followed up for a median duration of 47 (30-58) days. Complete resolution was documented in 92% of children after a median duration of 20 (9-38) days. There were no readmissions or deaths during follow-up. Conclusion : Cardiac involvement in children with MIS-C is frequent with coronary abnormalities and ventricular dysfunction being the most common manifestations. Most children exhibit complete clinical and myocardial recovery with appropriate anti-inflammatory therapy. Studies on long-term outcome of these children are needed. [ABSTRACT FROM AUTHOR]

Background : The availability of nomograms is crucial for the correct interpretation of pediatric and neonatal echocardiograms. Echocardiographic Z-score applications/websites use Western nomograms as reference, which may not be an appropriate standard for gauging Indian neonates. Currently available Indian pediatric nomograms either have not included neonates or have not been specifically designed for neonates. This gross underrepresentation of neonates renders available nomograms unreliable for use as standards for comparison. Objectives : The objective of this study was to collect normative data for the measurement of various cardiac structures using M-Mode and two-dimensional (2D) echo in healthy Indian neonates and to derive Z-scores for each measured parameter. Methods : Echocardiograms were performed on healthy term neonates (within first 5 days of life). Birth weight and length were recorded, and body surface area was calculated using Haycock's formula. Twenty M-mode and 2D-echo parameters were measured (including left ventricular dimensions, atrioventricular valves, and semilunar valves' annuli sizes, pulmonary artery and branches, aortic root, and arch). Results : We studied 142 neonates (73 males) with a mean age of 1.83 ± 1.12 days and mean birth weight of 2.89 ± 0.39 Kg. Regression equations with linear, logarithmic, exponential and square root models were tested to select the best model of fit for the relationship between birth weight and each echocardiographic parameter. Scatter plots and nomogram charts with Z-scores were prepared for each echocardiographic parameter. Conclusions : Our study provides nomograms with Z-scores for term Indian neonates weighing between 2 kg and 4 kg at birth, within first 5 days of life, for a set of echocardiographic parameters that are frequently used in clinical practice. This nomogram has poor predictability for babies at extremes of birth weight. There is a need for further indigenous studies to include neonates at extremes of weight, both term, and preterm. [ABSTRACT FROM AUTHOR]

Background: Outcome data of children with heart disease who acquired COVID-19 infection are limited. Aims : We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. Settings and Design: This is a retrospective, multicentric, observational study. Materials and Methods: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. Results: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046). Conclusions: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes. [ABSTRACT FROM AUTHOR]

Background: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID-19 on pediatric cardiac care in India. Aims: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. Settings and Design: This is a retrospective, multicentric, observational study. Methods: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. Results : The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). Conclusions: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic. [ABSTRACT FROM AUTHOR]

Aims and Objectives: There is a paucity of data regarding the outcomes of Heart transplantation in children from the Indian subcontinent. The data of patients under the age of 18 undergoing an isolated heart transplantation was analyzed for patient clinical profiles and risk factors for early and medium-term mortality. Hospital mortality was defined as death within 90 days of transplantation and medium-term survival as follow up of up to 6 years. Materials and Methods: A total of 97 patients operated between March 2014 and October 2019 were included in this study.Data was collected about their INTERMACS status, pulmonary vascular resistance, donor heart ischemic times, donor age, donor to recipient weight ratio and creatinine levels. Results: The age range was from 1 to 18 with a mean of 10.6 ± 4.6 years. 67 % patients were in INTERMACS category 3 or less.12 children were on mechanical circulatory support at the time of transplant. The 90 day survival was 89 %.The risk factors for hospital mortality was lower INTERMACS category (odd's ratio 0.2143, P = 0.026), elevated creatinine (odd's ratio 5.42, P = 0.076) and elevated right atrial pressure (odd's ratio 1.19, P = 0.015).Ischemic time, pulmonary vascular resistance (PVR) and PVR index (PVRI) had no effect on 90 day survival. Kaplan Meier estimates for 5 year survival was 73 %.The medium term survival was affected by INTERMACS category (Hazard ratio 0.7, P =.078) , donor age > 25 (Hazard ratio 1.6, P = 0.26) and raised serum creatinine values.(Hazard ratio 2.7, P = 0.012). All the survivors are in good functional class. Conclusions: Excellent outcomes are possible after heart transplantation in a pediatric population even in a resource constrained environment of a developing economy. More efforts are needed to promote pediatric organ donation and patients need to be referred in better INTERMACS category for optimal outcomes. [ABSTRACT FROM AUTHOR]

Background: Purulent pericarditis, if not recognized and managed timely, it can lead to significant morbidity and mortality. There are no guidelines for the management of purulent pericardial effusion in pediatric patients. Aim: The study describes our experience with the management of 22 patients admitted with a primary diagnosis of purulent pericardial effusion seen over a 7-year period. Materials and Methods: Hospital records of 22 children admitted to the pediatric intensive care unit with purulent pericardial effusion during January 2012-December 2018 were retrospectively analyzed. Results: The mean age of presentation was 4.6 years. The most common presentation was fever. History of antecedent trauma was present in 27.27% of patients. Empyema was the most common associated infection. Staphylococcus aureus was the most commonly isolated organism. Out of 22, pericardial drainage was done in 13 patients (59%). Only one of these patients required pericardiectomy later on. Six (27.2%) patients responded to antibiotics alone. Three (13.6%) patients died before any intervention could be planned. Conclusion: Echocardiography guided percutaneous pericardiocentesis and pigtail catheter placement are a safe and effective treatment for purulent pericardial effusion. When pericardial drainage is not amenable, close monitoring of the size of effusion by serial echocardiography is required. Small residual pericardial effusion may be managed conservatively. [ABSTRACT FROM AUTHOR]

Background: Pediatric heart transplantation is a now a well-established and standard treatment option for end stage heart failure for various conditions in children. Due to logistic issues, it is not an option for in most pediatric cardiac centres in the third world. Aim: We sought to describe our early experience in the current era in India. Methods: This is a short term retrospective chart review of pediatric patients who underwent heart transplantation at our centre. Mean/Median with standard deviation /range was used to present data. Results: Twenty patients underwent orthotopic heart transplant between January 2016 and June 2019. The median age at transplant was 12.4 years (range 3.3 to 17.3 years). The median weight was 23.2kg (range 10 80kg). The mean donor/recipient weight ratio was 1.62± 0.84. The mean ICU stay was 12.1days. The mean follow up post transplant was 2.03± 0.97years (range 10 days 3.57 years). The 1 month and the 1 year survival was 100%. Biopsies were positive for significant rejection in 7 patients (35%). At the time of last follow up, 3 patients (15%) had expired. The major post transplant morbidities were mechanical circulatory support (n=3), hypertension with seizure complex (n=3), post transplant lympho proliferative disorder (n=1), pseudocyst of pancreas (n=1), coronary allograft vasculopathy (n=3) and systemic hypertension (n=7). All surviving patients (n=17) were asymptomatic at last follow up. Conclusion: The results suggest acceptable short term outcomes in Indian pediatric patients can be achieved after heart transplantation in the current era. Significant rejection episodes and coronary allograft vasculopathy need careful follow up. [ABSTRACT FROM AUTHOR]

Introduction: In this prospective study, we compared the efficacy and safety of ibuprofen, indomethacin, and paracetamol in the closure of patent ductus arteriosus (PDA) in preterm neonates. Materials and Methods: This randomized prospective study was conducted in the Division of Pediatric Cardiology, M. D. M and Umaid Hospital, Jodhpur. A total of 105 preterm neonates with gestational age <37 weeks and hemodynamically significant PDA (hs-PDA) diagnosed clinically and confirmed by echocardiography were enrolled. All neonates were randomly assigned in a ratio of 1:1:1 to oral indomethacin (Group A, 3 doses at an interval of 12 h with a starting dose of 0.2 mg/kg), oral ibuprofen (Group B, 10 mg/kg ibuprofen followed by 5 mg/kg/day for 2 days), or IV paracetamol (Group C, 15 mg/kg every 6 hourly for 3 consecutive days). After the completion of the first course, neonates were assessed clinically as well as by echocardiography to confirm PDA closure. If PDA remained open, the second course of the same drug was given and repeat assessment was done within 24 h of the last dose. In addition to an echocardiographic examination, complete blood counts, renal and liver function tests were performed. Results: Our study shows that there was no significant difference observed in PDA closure among all the three treatment groups after the completion of two courses of treatment. The cumulative rate of PDA closure was 68% in the indomethacin group, 77.14% in the ibuprofen group, and 71.43% in the paracetamol group (P = 0.716). There were no significant changes found in Hb, platelet, blood urea nitrogen (BUN), creatinine, and liver enzymes after treatment in the paracetamol group (P > 0.05). BUN and serum creatinine levels were significantly increased after treatment in indomethacin and ibuprofen groups (P < 0.0001 and P < 0.05, respectively). Conclusion: Our study shows that IV paracetamol is as effective as indomethacin and ibuprofen in promoting the closure of hs-PDA in premature infants with a better safety profile. [ABSTRACT FROM AUTHOR]

The author reflects on the universal heart coverage (UHC) model for pediatric heart disease in India. Topics discussed include the burden of pediatric heart disease in the country which is considered to be the largest among all nations in the world, the resources available pediatric heart care, and the need for all stakeholders to understand the massive shortfall of available resources for pediatric care.

Hypocalcemia is a less known but treatable cause for dilated cardiomyopathy, leading to severe heart failure in children. Cardiogenic shock related to hypocalcemic cardiomyopathy is a rare event. We describe 5 infants presenting with cardiogenic shock over 3 years, who were found to have severe hypocalcemia as a sole cause of myocardial dysfunction. The patients responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized within months of treatment. In any case of cardiogenic shock, hypocalcemia should be included in the differential diagnosis and must be investigated. [ABSTRACT FROM AUTHOR]

Objectives: To study the blood pressure of school going children in a rural area and its relationship with the anthropometric indices. Methods: A prospective, cross-sectional study was carried out from November 2006 to December 2007 in the school going children between the ages of 6-17 years from eight different schools in the rural areas of Wardha district. The height, weight, systolic blood pressure (SBP), and diastolic blood pressure (DBP) were recorded in both sexes followed by complete clinical examination with special emphasis on cardiovascular system. Hypertension (HT) was defined as SBP or DBP exceeding the 95th percentile for age, gender, and height on at least three separate occasions, 1-3 weeks apart. SPSS software was used to analyze the data. Coefficient correlation tests were employed to assess the relation between BP and anthropometric variables. Results: Of 2643 school children, 1227 were boys and 1416 girls with a male to female ratio of 1:1.16. In boys, SBP and DBP increased with age except a marginal decline in SBP at the age of 17 years (-0.09) and decrease in the DBP (-1.29) at 16 years of age. In girls, SBP and DBP also increased with age except at 11 years, wherein there was a mild decrease in SBP (-0.09) as well as the DBP (-0.24). Correlation coefficient analysis showed highly significant positive correlation of height with SBP and DBP. There was a significant correlation of SBP and DBP with the weight, and body mass index (BMI). The prevalence of HT was 5.75% (i.e., 3.25% for systolic HT and 2.49% for diastolic HT). Conclusion: We recommend that there is a need for checking BP to detect HT in children, so that remedial measures can be initiated as early as possible. [ABSTRACT FROM AUTHOR]

Pulmonary artery thrombosis in neonates is a rare entity. We describe two neonates with this diagnosis; their presentation, evaluation, and management. These cases highlight the importance of this differential diagnosis when evaluating the cyanotic neonate. [ABSTRACT FROM AUTHOR]