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Involvement of the heart in children with tuberculosis (TB) generally affects the pericardium; however, the myocardium of a child being affected alone and leading to systolic heart dysfunction has rarely ever been reported. We report a case of a 12-year-old child who presented to Pediatric Emergency Services with severe hypotension (BP

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.

Organ-specific vasculitis is an uncommon, delayed complication of COVID-19 infection. It is usually seen in mildly symptomatic or asymptomatic patients. Underlying endothelitis is the most likely pathophysiological mechanism for such a manifestation. We report two infants with renal artery stenosis, most likely consequent to COVID-19 infection.

Abstract Background Statins and aspirin have been proposed for treatment of COVID-19 because of their anti-inflammatory and anti-thrombotic properties. Several observational studies have shown favourable results. There is a need for a randomised controlled trial. Methods In this single-center, open-label, randomised controlled trial, 900 RT-PCR positive COVID-19 patients requiring hospitalisation, were randomly assigned to receive either atorvastatin 40 mg (Group A, n = 224), aspirin 75 mg (Group B, n = 225), or both (Group C, n = 225) in addition to standard of care for 10 days or until discharge whichever was earlier or only standard of care (Group D, n = 226). The primary outcome variable was clinical deterioration to WHO Ordinal Scale for Clinical Improvement ≥ 6. The secondary outcome was change in serum C-reactive protein, interleukin-6, and troponin I. Results The primary outcome occurred in 25 (2.8%) patients: 7 (3.2%) in Group A, 3 (1.4%) in Group B, 8 (3.6%) in Group C, and 7 (3.2%) in Group D. There was no difference in primary outcome across the study groups (P = 0.463). Comparison of all patients who received atorvastatin or aspirin with the control group (Group D) also did not show any benefit [Atorvastatin: HR 1.0 (95% CI 0.41–2.46) P = 0.99; Aspirin: HR 0.7 (95% CI 0.27–1.81) P = 0.46]. The secondary outcomes revealed lower serum interleukin-6 levels among patients in Groups B and C. There was no excess of adverse events. Conclusions Among patients admitted with mild to moderate COVID-19 infection, additional treatment with aspirin, atorvastatin, or a combination of the two does not prevent clinical deterioration. Trial Registry Number CTRI/2020/07/026791 ( http://ctri.nic.in ; registered on 25/07/2020)

Background : Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes. Aim : We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD. Methods : In this prospective observational study, eligible infants and children ≤21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental “delay” and “no delay” groups. Results : Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental “delay” and “no delay” groups (P = 0.04). Conclusion : Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery.

Justification : In recent years, there has been increasing recognition of children with heart disease in our country. These children belong to different age groups and have untreated, partially treated, or completely treated heart disease. The role of physical activity for optimal physical, emotional, and psychosocial well-being for children is well understood. There is a challenge for the parents and the medical professionals to take a decision regarding the type of physical activity safe for the child as heart disease may affect the hemodynamic demands. Most of the existing international guidelines focus on competitive sports in operated heart disease children. This may be of limited use when we have a mixed population of children with heart disease, different types of sports in our country and where a larger subset is looking for recommendations to leisure time activities. Process : The Pediatric Cardiac Society of India decided to formulate recommendations for physical activity in children with heart diseases. A committee of experts, who were well-versed with the subject of physical activity in children with heart disease, volunteered to take up the task of writing the guidelines. The recommendations emerged following deliberations of the committee members, on the virtual platform as well as mails. The final version of manuscript was approved by all committee members and all members are co-authors of this manuscript. The different types of physical activities were defined including leisure sports and competitive sports. The exercise was classified based on the mechanical action of muscles involved into dynamic and static components. Each type of exercise was then classified based on the intensity into low, medium, and high. Recommendations for the type of physical activity for individual heart lesions were decided based on the rationale available. Objectives : The recommendations here are made with an intention to provide general guidelines for physical activity in children with operated and unoperated heart diseases, not excluding a need for individualizing a plan, serial assessment, and comprehensive checkup in special situations. Recommendations : We hope the recommendations mentioned below would provide basic clarity in planning physical activity in children with heart disease. This is with the hope to encourage physically active life, at the same time ensuring a safety net.

Objectives : We aimed to compare the performance of pulse-oximetry screening in detecting nonductus-dependent cyanotic congenital heart defects (CCHDs). Methods : In a prospective cross-sectional study, we recorded post ductal saturation of neonates (

Background: Costs can be a major barrier to medication adherence in low and middle-income countries and are an important target for policy-level interventions. The use of benzathine penicillin G (BPG) for secondary prevention of rheumatic heart disease (RHD) averts substantial morbidity and mortality, yet the total out-of-pocket costs for patients receiving this intervention are unknown. Objective: To estimate the total out-of-pocket costs for obtaining BPG prophylaxis among RHD patients in India. Methods: We prospectively collected self-reported drug-, transportation-, and provider-related costs for secondary prophylaxis among RHD patients presenting for follow-up to a tertiary care centre in New Delhi, India. Monthly costs were estimated by adjusting unit costs by frequency of drug administration. Results: The cost data provided by 420 patients [mean age (±SD) 11.6 (±2.9) years] was analysed. Majority of the patients were male (65.2%), hailed from rural areas (87.1%), and belonged to lower socioeconomic strata (73.3%). The median monthly total out-of-pocket costs (IQR) for obtaining BPG injections was Indian rupee (INR) 62.5 (42.5–117.0). The median costs for procuring the drug (IQR) was INR 34.0(30.0–39.0). Whereas median costs (IQR) for health care provider and transportation was INR 16.0 [0–32.0]) and INR 11 [0–31.0] respectively. When expressed as mean (SD), the costs for transportation constituted 50% of the total costs, whereas the mean cost for drug procurement and drug administration constituted 30% and 22% of the total costs respectively. Conclusion: RHD patients receiving BPG prophylaxis incur substantial out-of-pocket costs, with transportation costs constituting nearly half of the total expenditures. National investments in RHD control must be strategically directed at improving health care access and drug supply in order to lower the total costs of secondary prophylaxis and improve adherence rates.

Aims : The utility of beta-blocker therapy in infants with heart failure (HF) due to significant left-to-right shunt lesions is not known. The study aimed to assess the efficacy and safety of propranolol in infants with HF due to moderate-to-large ventricular septal defect (VSD). Methods : The prospective randomized trial included 80 infants with HF and moderate-to-large VSD, randomly allocated to receive either conventional therapy alone (n = 40) or propranolol plus conventional therapy (n = 40). The primary endpoint was a composite of all-cause mortality, hospitalization for HF and/or chest infection, and referral for surgery. The secondary clinical outcomes were the individual components of the composite endpoint. In addition, the patients were followed up to detect safety outcomes, for example, bronchospasm, bradyarrhythmia, and worsening HF symptoms. Results : The addition of propranolol therapy to the conventional medications did not result in significant improvement in the primary composite endpoint (32.50% vs. 52.50%; P = 0.07). There was a trend toward improvement, but the study is underpowered for this important question. However, propranolol therapy significantly decreased the risk of hospitalization (12.50% vs. 32.50%; P = 0.03) and worsening of Ross HF class (5.41% vs. 28.21%; P = 0.01) as compared to conventional therapy (estimated number needed to treat = 5). Propranolol did not result in any significant safety concerns in these infants except bronchospasm in an infant. Conclusions : Propranolol therapy in infants with significant left-to-right shunt may prevent worsening in HF symptoms and hospitalization and is well tolerated. However, it does not reduce mortality or need for surgery.

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.

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 Background Inflammation has been implicated in the initiation and perpetuation of non‐valvular atrial fibrillation (AF). However, there is a lack of similar data on AF in rheumatic heart disease (RHD). The objective of this study was to analyze the association of inflammation as measured by serum inflammatory biomarkers with AF in rheumatic mitral stenosis (Rh‐MS). Methods A comparative cross‐sectional analytical study was conducted on 181 Rh‐MS patients in normal sinus rhythm (NSR; n = 69), subclinical transient AF (SCAF; detected by 24‐hours Holter monitoring; n = 30) and chronic AF (n = 82). Serum hs‐CRP, IL‐6, and sCD‐40L were assessed using ELISA immunoassay and compared in all groups of Rh‐MS with or without AF. Results We found significantly higher serum hs‐CRP and sCD‐40L levels in the overall AF (Chronic AF + SCAF) group (hs‐CRP: 4.5 ± 3.4 vs 2.3 ± 2.9 mg/L, P

Objective India has been struggling with infant malnutrition for decades. There is a need to identify suitable platforms for community engagement to promote locally feasible, resource efficient Infant and Young Child Feeding (IYCF) interventions. This study aims to explore if and how schools could represent a site for community engagement in rural India, acting as innovation hubs to foster positive change in partnership with the Angawadi centres.Design Five-phase formative study; A parallel mixed methods approach structured by a socioecological framework was used for data collection at individual, household and community levels. This paper focuses on the qualitative findings.Setting This study was undertaken in nine villages within two blocks, ‘Ghatol’ and ‘Kushalgarh’, in the Banswara district of Rajasthan, India.Participants 17 schools were identified. Interviews were conducted with local opinion leaders and representatives in the education sector, including principals, schoolteachers, block and district education officers. Across the nine study villages, information was gathered from 67 mothers, 58 paternal grandmothers using Focus Discussion Groups (FDGs) and 49 key respondents in Key Informant Interviews.Results Schools were considered an important community resource. Challenges included limited parental participation and student absenteeism; however, several drivers and opportunities were identified, which may render schools a suitable intervention delivery site. Enrolment rates were high, with schools and associated staff encouraging parental involvement and student attendance. Existing initiatives, including the mid-day meal, play opportunities and education on health and hygiene, further highlight the potential reliability of schools as a platform for community mobilisation.Conclusions Schools have been shown to be functional platforms frequently visited and trusted by community members. With teachers and children as change agents, schools could represent a suitable setting for community mobilisation in future wider scale intervention studies. Expanding the supportive environment around schools will be essential to reinforce healthy IYCF practices in the long term.

Objective Prospective data on clinical profile, natural history and outcomes of NSAA (non-specific aortoarteritis) in children is limited. We initiated this prospective study to evaluate the short-term and medium-term outcomes of NSAA in children.Design Prospective observational study.Setting Tertiary care hospital in India.Patients We included 28 consecutive children (

Abstract Objectives To assess the impact of adding statin (atorvastatin) and/or aspirin on clinical deterioration in patients infected with SARS-CoV-2 who require hospitalisation. The safety of these drugs in COVID-19 patients will also be evaluated. Trial design This is a single-centre, prospective, four-arm parallel design, open-label, randomized control trial. Participants The study will be conducted at National Cancer Institute (NCI), Jhajjar, Haryana, which is a part of All India Institute of Medical Sciences (AIIMS), New Delhi, and has been converted into a dedicated COVID-19 management centre since the outbreak of the pandemic. All RT-PCR confirmed cases of SARS-CoV-2 infection with age ≥ 40 years and < 75 years requiring hospital admission (patients with WHO clinical improvement ordinal score 3 to 5) will be included in the trial. Written informed consent will be taken for all recruited patients. Patients with a critical illness (WHO clinical improvement ordinal score > 5), documented significant liver disease/dysfunction (aspartate transaminase [AST] / alanine aminotransferase [ALT] > 240), myopathy and rhabdomyolysis (creatine phosphokinase [CPK] > 5x normal), allergy or intolerance to statins or aspirin, prior statin or aspirin use within 30 days, history of active gastrointestinal bleeding in past three months, coagulopathy, thrombocytopenia (platelet count < 100000/ dl), pregnancy, active breastfeeding, or inability to take oral or nasogastric medications will be excluded. Patients refusing to give written consent and taking drugs that are known to have a significant drug interaction with statin or aspirin [including cyclosporine, HIV protease inhibitors, hepatitis C protease inhibitor, telaprevir, fibric acid derivatives (gemfibrozil), niacin, azole antifungals (itraconazole, ketoconazole), clarithromycin and colchicine] will also be excluded from the trial. Intervention and comparator In this study, the benefit and safety of atorvastatin (statin) and/or aspirin as adjuvant therapy will be compared with the control group receiving usual care for management of COVID-19. Atorvastatin will be prescribed as 40 mg oral tablets once daily for ten days or until discharge, whichever is earlier. The dose of aspirin will be 75 mg once daily for ten days or until discharge, whichever is earlier. All other therapies will be administered according to the institute’s COVID-19 treatment protocol and the treating physician’s clinical judgment. Main outcomes All study participants will be prospectively followed up for ten days or until hospital discharge, whichever is longer for outcomes. The primary outcome will be clinical deterioration characterized by progression to WHO clinical improvement ordinal score ≥ 6 (i.e., endotracheal intubation, non-invasive mechanical ventilation, pressor agents, renal replacement therapy, ECMO requirement, and mortality). The secondary outcomes will be change in serum inflammatory markers (C-reactive protein and Interleukin-6), Troponin I, and creatine phosphokinase (CPK) from time zero to 5th day of study enrolment or 7th day after symptom onset, whichever is later. Other clinical outcomes that will be assessed include progression to Acute Respiratory Distress Syndrome (ARDS), shock, ICU admission, length of ICU admission, length of hospital admission, and in-hospital mortality. Adverse drug effects like myalgia, myopathy, rhabdomyolysis, hepatotoxicity, and bleeding will also be examined in the trial to assess the safety of the interventions. Randomisation The study will use a four-arm parallel-group design. A computer-generated permuted block randomization with mixed block size will be used to randomize the participants in a 1:1:1:1 ratio to group A (atorvastatin with conventional therapy), group B (aspirin with conventional therapy), group C (aspirin + atorvastatin with conventional therapy), and group D (control; only conventional therapy). Blinding (masking) The study will be an open-label trial. Numbers to be randomised (sample size) As there is no existing study that has evaluated the role of aspirin and atorvastatin in COVID-19 patients, formal sample size calculation has not been done. Patients satisfying the inclusion and exclusion criteria will be recruited during six months of study period. Once the first 200 patients are included in each arm (i.e., total 800 patients), the final sample size calculation will be done on the basis of the interim analysis of the collected data. Trial Status The institutional ethical committee has approved the study protocol (Protocol version 3.0 [June 2020]). Participant recruitment starting date: 28th July 2020 Participant recruitment ending date: 27th January 2021 Trial duration: 6 months Trial registration The trial has been prospectively registered in Clinical Trial Registry – India (ICMR- NIMS): Reference no. CTRI/2020/07/026791 (registered on 25 July 2020)]. Full protocol The full protocol is attached as an additional file, accessible from the Trials website (Additional file 1). In the interest of expediting dissemination of this material, the familiar formatting has been eliminated; this Letter serves as a summary of the key elements of the full protocol.

Background: Pulmonary stenosis in patients with common arterial trunk protects the pulmonary vasculature. In our recently published prospective study of common arterial trunk, some patients with sinusal origin of the pulmonary arterial segment had pulmonary stenosis induced by systolic excursion of a truncal valvar leaflet. We aimed to determine the detailed morphologic characteristics of this unusual finding. Methods and Results: All 70 patients underwent echocardiography and computed tomographic angiography as per predefined study protocol. In selected cases, we also performed cardiac catheterization. Among 27 patients with aortic dominance, we found sinusal origin of the pulmonary arterial segment. In 5 of these patients, pulmonary stenosis was induced by systolic excursion of a truncal valvar leaflet. In all these patients, the truncal valve was trisinusate, albeit with asymmetric sinuses. The pulmonary arterial segment arose from the largest left sinus with its relatively large leaflet obstructing the pulmonary orifice during systole. In the remaining 22 patients, without pulmonary stenosis but with sinusal origin of pulmonary arterial segment, the truncal valve was quadrisinusate in 7, bisinusate in 2, and trisinusate in 13. None of the patients with quadrisinusate and bisinusate truncal valves had pulmonary stenosis. Among the 13 patients with trisinusate valves, the sinuses of the truncal valve were symmetrical in 6, while in 7, the pulmonary orifice originated from a smaller asymmetric sinus. Conclusion: Pulmonary stenosis, produced by a relatively large leaflet of an asymmetric truncal sinus, may prevent early development of pulmonary vascular disease. Timely recognition of this unusual mechanism of pulmonary stenosis is important for optimal management.

Introduction: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. Objectives: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. Recommendations: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described. Keywords: Congenital heart disease, Intervention, Surgery

A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

Micro-RNAs (miRs) encapsulated inside urinary exosomes (uEs) have the potential as early biomarkers. Previously, we reported that a rise in uE miR-451 predicted albuminuria in diabetic rats; however, whether the rise was protective or detrimental, and occurred in response to injury or general hyperglycemia, was unknown. To address this, we studied both human and rat models of renal disease. In humans, uE miR-451 was approximately twofold higher in subjects with early-stage chronic kidney disease (CKD; serum creatinine < 2.0 mg/dl; n = 28), as compared to age-matched healthy controls (n = 23), and had a significant negative correlation with estimated glomerular filtration rate (eGFR) (r2 = −0.10, p = 0.01). Subgroup analysis of CKD subjects showed that those without diabetes had slightly (∼30%) but significantly higher uE miR-451 as compared to those with diabetes, with no differences in albumin excretion, eGFR, serum sodium, and potassium. Using human proximal tubule (hPT) cells, we found that locked nucleic acid (LNA) inhibition of miR-451 resulted in a significant increase in the messenger RNA (mRNA) expression of kidney-injury-associated miR-451 targets, e.g., CAB39, TBX1, and YWHAZ, as compared to treatment with a control LNA. Moreover, hPT cells and their secreted exosomes showed an increase in miR-451 in response to mechanical injury but not high glucose (20 versus 5 mM). For further proof of concept, in diabetic rats, we showed that atorvastatin (AT), a treatment proven to attenuate renal injury without affecting systemic glucose levels, reduced uE miR-451 with the concomitant restoration of renal miR-451. These data elucidate the stimuli for renal miR-451 expression and exosomal release and support its role as a therapeutic target and early biomarker for renal injury in humans.

Background: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Methods: We performed a retrospective review of 73 consecutive patients who underwent repair for TOF/APV between January 2005–August 2015. Mean age was 6.4 ± 5.6 years (28 days–22 years). The right ventricular outflow tract (RVOT) was reconstructed using varied techniques. Freedom from RVOT gradients and re-operation was studied. Results: There were four (5.5%) early deaths, two each in infants and older children. Median ICU stay was 2 days (range, 1–12 days). Mean ICU stay for, infants, children and adults, was 6.5 ± 6.04, 2.75 ± 2.45, and 2.33 ± 1.03 days, respectively (p = 0.0762). Median hospital stay was 6 days (range, 4 to 15 days). Mean hospital stay for, infants and children and adults was 7 ± 2, 6.75 ± 2.39, and 6.33 ± 1.63 days, respectively (p = 0.325). Mean follow up was 65 ± 36.6 months (median 56 months, range 7–126 months). On follow up echocardiography, 14 (21.21%) had no pulmonary regurgitation. 21 (31.81%) had mild PR patients, 8 (12.12%) moderate PR and 19 (28.78%) had severe PR. There were five (7.5%) reoperations. Five and ten-year survival was 95% ± 2.12 and 92.3% ± 3.45 respectively. Freedom from RVOT reoperation was 93 ± 2.62% and 89 ± 3.87% at 5 and 10 years. Conclusions: In contrast to children and adults with TOF/APV, infants carry significant early mortality. But the mid-term outcome for patients who survive the initial repair of TOF/APV is acceptable. However, these patients require constant surveillance and irrespective of the methods of RVOT management, the reoperation rates are expected to be high as more of these patients survive into adulthood.

Background: Atypical hemolytic uremic syndrome (aHUS), an important cause of acute kidney injury (AKI), is characterized by dysregulation of the alternative complement pathway. Autoantibodies to factor H (FH), a chief regulator of this pathway, account for a distinct subgroup. While high anti-FH titers predict relapse, they do not correlate well with disease activity and their functional characterization is required.Methods: Of 781 patients

A 27-year-old female presented with generalized body swelling and progressive shortness of breath for the past 4 months. On examination, she had a loud continuous murmur, elevated jugular venous pressure, and gross ascites. The examination findings, electrocardiogram, chest radiography, and echocardiogram are discussed in a step-wise manner to arrive at a diagnosis and plan of management of a patient with ruptured sinus of Valsalva aneurysm is discussed along with review of relevant literature.

Objective: The adjustable pulmonary artery band (APAB) has been demonstrated by us earlier to be superior to the conventional pulmonary artery banding (CPAB), in terms of reduced early morbidity and mortality. In this study, we assessed the adequacy of the band and its complications over the mid-term. Methods: Between 2002 and 2012, 73 patients underwent adjustable PAB, and their operative and follow-up data were collected and analyzed. Results: There was one early death following the APAB. Follow-up data were available for 57 patients of which 44 patients (61.7%) underwent definitive repair, 10 were awaiting definitive repair, and 3 patients were kept on medical follow-up because of inadequate fall in pulmonary artery (PA) pressures. 14 patients (19%) were lost to follow-up. Major PA distortion or stenosis was absent in the majority. 1 patient had pseudoaneurysm of the main pulmonary artery (MPA) with sternal sinus infection and required surgical reconstruction. 1 patient had infective endocarditis of the pulmonary valve managed medically. Band migration was not encountered. There were two deaths after definitive repair and one after APAB. Conclusions: Patients undergoing APAB fulfilled the desired objectives of the pulmonary artery banding (PAB) with minimum PA complications in the mid-term. This added to the early postoperative benefits, makes the APAB an attractive alternative to the CPAB.

Bioelectrical impedance analysis (BIA) is a method for the assessment of nutritional status. We studied the effect of graft function on nutritional status in postrenal 45 transplant patients with borderline to good allograft function using BIA. The patients had a mean serum creatinine of 1.42 ± 0.42 mg% and mean glomerular filtration rate (GFR) of 45.1 ± 14.1 mL/min. Based on BIA-derived GFR, the patients were divided into two groups; group 1: borderline graft function GFR 4.0) in extracellular water (P

Introduction: There are few data on heart failure (HF) burden and none available on the community prevalence of HF in India. We conducted a study aimed at determining the HF prevalence in a rural community as well as tertiary hospital care setting in North India. We also reviewed the existing literature regarding the estimated and projected prevalence of HF in India. Methodology: All adults (>20 years) with chronic breathlessness in six villages under a primary health care center in Northern India were identified and evaluated with standardized questionnaire and physical examination by trained health care workers. HF was diagnosed by standardized criteria and a transthoracic echocardiogram was performed in all subjects. In the hospital study, 500 consecutive patients presenting to our tertiary referral hospital were evaluated for the diagnosis of HF. For the systematic review, all published studies addressing HF or the burden of risk factors in India were identified. Projections for the absolute HF burden were made using local data and global studies of HF incidence, morbidity, and mortality. Results: Among the surveyed rural adult population of 10,163 patients, chronic breathlessness was present in 128 (1.3%). HF was present in 9% (n = 12), of which 67% (n = 8) had preserved left ventricular (LV) systolic function and 33% (n = 4) had LV systolic dysfunction. Therefore, the prevalence of HF in this general community was 1.2/1000. All patients with HF and preserved ejection fraction had poorly controlled hypertension. In the hospital study, of 500 consecutive patients, 20.4% had HF. Rheumatic heart disease (52%) was the most common cause followed by ischemic heart disease (17%). The mean age of presentation was 39 ± 16 years. The prevalence of HF in the outpatient department patients was 22.5% below 30 years and 14.9% above 50 years, reflecting the young population of HF. For the estimates concerning HF burden in India, projections were made using both age-specific extrapolations from developing countries and data regarding development of HF in the presence of risk factors. The estimated prevalence of HF is about 1% of the total population or about 8–10 million individuals. The estimated mortality attributable to HF is about 0.1–0.16 million individuals per year. Conclusions: While our hospital data are consistent with the HF burden and etiology expected in a government tertiary hospital setting, our community-based study is the first of its kind reported from India. The community study demonstrates a surprisingly low prevalence of symptomatic HF in the surveyed villages. This could be partially explained by the rural farming-based community setting but is also likely due to under-reporting of symptoms. Our review of the projected national estimates suggests an alarming burden of HF in India despite a younger population than the developed nations. A significant proportion of this burden may be preventable with better screening and early and adequate treatment of the risk factors.

Objective: To assess the birth prevalence and pattern of congenital heart disease (CHD) using echocardiography in babies born in a community hospital of North India. Methods: A cross-sectional observational study conducted over a period of 3 years. Newborns born over a specific 8-h period of the day were recruited in the study. They underwent routine clinical examination and pulse oximetry, followed by screening echocardiography for diagnosing a CHD. Results: A total of 20,307 newborns were screened, among which 874 had abnormal echocardiograms; 687 had insignificant CHDs, 164 had significant CHDs, and 24 had other abnormal cardiac findings. The birth prevalence of significant CHDs was 8.07 per 1000 live births; 131 newborns had an acyanotic CHD (79.9%) and 33 a cyanotic CHD (20.1%). Ventricular septal defect (VSD) was the most common acyanotic CHD, present in 116 newborns, giving a prevalence of 5.7/1000 live births. Among the cyanotic CHD, transposition of great arteries was most common (prevalence 0.34/1000 live births). Conclusion: The CHD birth prevalence in our study is similar to the reported worldwide birth prevalence. Acyanotic CHD (mostly VSD) is seen in about three-fourths of babies born with CHD. The more sinister cyanotic CHD is present in remaining 25%.

Intradialytic hypotension (IDH) is a life-threatening condition. We evaluated the feasibility of blood volume monitoring (BVM) and blood temperature monitoring (BTM) in preventing IDH in patients prone to the same. Fourteen hemodynamically unstable end-stage renal disease patients who were prone to IDH and unable to achieve dry weight were given BVM treatment twice weekly for two weeks. Forty patients who were not on BVM treatment served as controls. Patients were anemic, had low serum albumin (3.4 ± 0.43 g/dL) and fluid overload and were edematous. Of the 40 patients in the control group, 18 patients experienced IDH and dialysis had to be terminated. The incidence of IDH was 5% in the control group. In the BVM group, the total volume of fluid removed during hemodialysis was between 2.0 and 4.5 L (mean 3.2 L). By the end of dialysis, the hemo-concentration increased by 34.8%. With use of BVM and BTM, the blood pressure did not drop below 120/80 mm Hg, the dialysis sessions were uneventful and none of the patients suffered symptoms of hypotension. There was a difference of 3 kg between weight achieved and dry weight of the patient, although there was a 14.2% reduction in extracellular water (ECW), 14.5% in plasma fluid and 14.5% decrease in interstitial fluid. Blood volume significantly correlated with post-dialysis intracellular water (ICW) (r = 0.722, P = 0.008) and ECW/ICW ratio (r = 0.698, P = 0.012). There was a significant correlation between systolic blood pressure and ECW (r = 0.615, P = 0.033). Diastolic blood pressure significantly correlated with post-dialysis ECW (r = 0.690, P = 0.008), plasma fluid post-dialysis (r = 0.632, P = 0.027) and interstitial fluid (r = 0.604, P = 0.038). The ECW/ICW ratio was high (1.13 ± 0.48; control 0.74), implying overhydration and expanded extracellular fluid. BVM should be included in the dialysis protocol where patient compliance to maintenance hemodialysis is poor and patients are constantly in volume overload.

Summary of the Consensus Statement: This statement has been prepared keeping Indian heart failure patients in mind. Optimal management of CHF improves quality of life, reduces hospitalization rates and prolongs survival for people with this condition. Echocardiography is the single most useful test in the evaluation of heart failure, and is necessary to confirm the diagnosis. Plasma B-natriuretic peptide (BNP) measurements may be useful in excluding CHF but not mandatory in India. Educate people with CHF about lifestyle changes (e.g., increase physical activity levels, reduce salt intake and manage weight). Educate people with CHF about CHF symptoms and how to manage fluid load. Avoid prescribing drugs that exacerbate CHF. Prescribe angiotensin-converting enzyme inhibitors (ACEI) at effective doses for people with all grades of systolic heart failure, and titrate to the highest recommended dose tolerated. Angiotensin II receptor antagonists (ARA) may be used as alternatives in people who cannot tolerate ACEIs. Mineralocorticoid receptor antagonists (MRAs) should also be used. For people with stabilised systolic heart failure, prescribe beta-blockers that have been shown to improve outcome in heart failure (e.g., bisoprolol, carvedilol, extended release metoprolol or nebivolol). Titrate to the highest recommended dose tolerated. Prescribe diuretics, digoxin and nitrates for people already using ACEIs and beta-blockers to manage symptoms as indicated. For people who have systolic heart failure (New York Heart Association (NYHA) Class II-IV) despite appropriate doses of ACEIs and diuretics, consider prescribing spironolactone. Eplerenone can be considered in certain setting especially post myocardial infarction though it is more expensive. Consider direct sinus node inhibition with ivabradine for people with CHF who have impaired systolic function, have had a recent heart failure hospitalisation and are in sinus rhythm with a heart rate >70 bpm despite beta blockers or where beta blockers are contraindicated Check for, and treat, iron deficiency in people with CHF to improve their symptoms, exercise tolerance and quality of life Consider assessing people with CHF for biventricular pacemakers and implantable defibrillators. Patients with end stage heart failure have an option for heart transplant and ventricular assist devices which is now available in select centers.

The 5 th Congress of Asia Pacific Pediatric Cardiac Society was held in New Delhi from 6-9 March 2014. This article describes the journey of preparing and hosting one of the largest international events in the specialty of Pediatric Cardiac Care ever held in India. A total of 938 delegates, including 400 from outside India, participated. The scientific program was inclusive keeping in mind the diverse background of delegates from the member nations. Large numbers of research papers were presented, mostly by fellows in training.

About 1.35 million babies are born with congenital heart disease each year globally. Most of these are expected to lead normal, productive lives if they are treated in time. However, 90% of babies born with congenital heart disease live in regions where medical care is inadequate or unavailable. The privilege of early diagnosis and timely intervention is restricted to only those born in developed countries. Added to the burden of congenital heart disease is rheumatic heart disease, which remains a global health problem in many low-income and middle-income countries. Providing optimal care for all these children is a daunting task, and requires funds and proper planning at various levels of the health care system. This article describes the burden of pediatric heart disease, including lacunae in the current state, as well as challenges and opportunities for providing optimal care to this large population of children.

Post-transplant hypertension is a major risk factor for graft loss and patient survival; therefore, hypertension following renal transplantation must be treated strictly. There seems to be a strong association between hypertension, total body water (TBW) and dry weight. In this study, we report the relationship of body water and body composition with hypertension in post-renal transplant patients. Forty-five post-transplant patients were enrolled in the study. Blood pressure (BP) was labeled as controlled if BP was 120/80 mm Hg and not under good control if BP was above 120/80 mm Hg. Based on the number of antihypertensive medications a patient was taking, patients were divided into two groups: Group 1 patients on one antihypertensive drug and Group 2 patients on more than one antihypertensive drug. Nutritional status of the patients was assessed. Body composition (water compartments, body fat and lean mass) was assessed using bioelectrical impedance analysis (BIA). Patient data were compared with 30 healthy volunteers. In patients, systolic BP was associated with TBW (P = 0.016), extracellular water (ECW Lt; r = 0.99), ECW% (r = 0.78) and diastolic BP with TBW% (P = 0.003), dry weight (r = 0.76) ECW% (r = 0.95) and percent intracellular water (ICW%) (r = 0.79). Compared with controls, ECW and ECW% was higher in patients, and the ICW% was less in patients. There was significant difference in the actual weight of the patients and BIA-derived dry weight, although patients were clinically not edematous. The study showed a significant increase in diastolic BP with increase in dry weight. Significant difference in TBW was observed when the patients were grouped on the basis of the antihypertensive medication a patient was taking (one antihypertensive drug versus more than one antihypertensive drug). This study also showed an association between hypertension and overhydration. BIA may be a useful tool for the clinical assessment of overhydration in non-edematous patients.

Diphtheria continues to be reported from many parts of the world. Complete heart block is rare but often fatal complication of diphtheric myocarditis. We report six children with diphtheric myocarditis who presented with complete heart block. Three patients survived, one with persistent complete heart block. Aggressive supportive management including transvenous pacing may result in complete recovery in a significant number of children with diphtheric myocarditis.

Background: Amplatzer vascular plugs (AVPs) are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs). Aims: To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. Materials and Methods: All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. Results: A total of 39 AVPs were implanted in 31 patients. Thirteen (33%) were AVP type I and 23 (59%) were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM) (n = 7), aortopulmonary collaterals (n = 7), closure of a patent Blalock-Taussig shunt (n = 5), systemic AVM (n = 5), coronary AVM (n = 4), patent ductus arteriosus (PDA) (n = 3), pulmonary artery aneurysms (n = 3), and venovenous collaterals (n = 2). Deployment of the AVP was done predominantly via the 5 - 7F Judkin′s right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. Conclusions: AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow.

In India, the discipline of cardiology started in the late 1950s and at that time pediatric cardiology was practiced as a part of cardiology specialty. This article traces the history of pediatric cardiology in India. Dr. S. Padmawati and Dr. Kamala Vytilingam underwent training in pediatric cardiology at international centers in the early 1950s and early 1960s. Dr. N. Gopinath successfully closed a ventricular septal defect using a pump oxygenator at Christian Medical College, Vellore. Open heart surgery program kicked off in the 1960s with the tireless efforts of many other surgeons. Dr. Rajendra Tandon, trained for 2 years at Boston Children Hospital under Dr. Alexander Nadas, joined the Department of Cardiology at the All India Institute of Medical Sciences, New Delhi in 1963. This and many other stories are described.

Nephrolithiasis is associated with a variety of abnormalities in urinary composition. These abnormal urinary risk factors are due to dietary indiscretions, physiological-metabolic disturbances or both. Stone disease is morbid and costly, and the recurrence rates may be as high as 30-50% after 5 years. Efforts to prevent stone formation are, therefore, essential. Dietary factors play an important role in kidney stone formation. Tailored dietary recommendations based on metabolic evaluation should be offered to patients for the prevention of recurrence of stone formation. Dietary intervention and subsequent evaluations of therapeutic efficacy should be based on results from multiple 24-h urine collections. Urine flow of >1 ml/kg/h almost eliminates the risk of supersaturation for calcium oxalate, calcium phosphate and uric acid, thus protecting from the formation of corresponding kidney stones. In patients with cystenuria, the required urine flow may even be higher and, in cases such as primary xanthinuria, high fluid intake is required. Milk intake in these patients should be within the RDA of calcium and protein. In children, recommendation of a high fluid intake has only limited success. Nevertheless, each patient should be advised about adequate fluid intake to increase urine volume in accordance with body size. Although children with hypocitraturia may benefit from therapeutic agents that raise the urine citrate concentration, all children bearing residual fragments should be counseled on adequate fluid intake along with potassium citrate treatment to prevent stone regrowth or formation.

Aortopulmonary collaterals (APCs) are occluded either preoperatively or at the time of cardiac surgery in patients with pulmonary atresia and ventricular septal defect (PAVSD). If left untreated, APCs are an important cause of deterioration in the early postoperative period. We present here an unusual case with a large residual APC causing refractory low-output state in the early postoperative period. Usual arterial approach failed due to extensive angulation with ostial narrowing. The large residual APC was successfully closed with an Amplatzer duct occluder (ADO) device delivered through the transvenous route.

A 6-year-old boy presented with dual drainage of left upper pulmonary vein, with connection to innominate vein inaddition to its normal connection to the left atrium. Despite relief of aortic stenosis at the age of 3 years, significant left to right shunt persisted. The dual drainage allowed successful percutaneous closure of the levoatriocardinal vein without obstruction to the pulmonary venous flow to the left atrium.

Percutaneous perforation of pulmonary valve, using 0.014” guidewires meant for coronary artery chronic total occlusion (CTO), is increasingly being performed for select cases of pulmonary atresia with intact ventricular septum (PA-IVS). Despite growing experience, procedural failures and complications are not uncommon. Even in infants treated successfully, the orifice created in the atretic pulmonary valve is eccentric. In this report, we present usefulness of coronary microcatheter in alignment of perforating coronary guidewire to the center of atretic pulmonary valve resulting in central perforation.