Introduction: Neonatal screening for congenital heart defects at birth can miss some heart defects, sometimes few critical ones, and the scenario is even worse in those neonates who had never undergone a neonatal checkup (home deliveries). Immunization clinic can serve as a unique opportunity as the second checkpoint for the screening of the children. A history- and examination-based test can serve as an effective tool to screen out children with heart defects. Aims and Objectives: The aim of this study was to establish the sensitivity and specificity of a clinical screening tool for the identification of congenital heart defects at the first visit of an infant after birth for immunization. Materials and Methods: This is a cross-sectional observational study which the consecutive children presenting at 6 weeks of age for immunization or any child presenting for the first time (outborn delivery) till 6 months of age in the immunization clinic were subjected to detailed history and examination and findings were recorded on a predesigned pro forma and a clinical score was calculated. All these children were then subjected to echocardiography for confirmation of the diagnosis of congenital heart disease (CHD), and the sensitivity and specificity of the test were recorded Observations and Results: A total of 970 babies were screened, out of them 31 were diagnosed with CHD and 18 had undergone neonatal screening at birth. A clinical score of 3 or more had more chances of detecting CHD. The sensitivity of the cutoff score as 3 was 96.77% and specificity was 98.72, with a positive predictive value of 71.43%, a negative predictive value of 99.89%, and an accuracy of 98.66%. Conclusions: The history- and examination-based tool is an effective method for early identification of CHD and can easily be used by peripheral workers working in remote places with poor resources enabling prompt referral.

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Isolated left ventricular hypoplasia is an entity with uncertain natural history and etiology. The presentation could vary from being asymptomatic to sudden death. This form of cardiomyopathy has been reported in infants as well as in adults. This case report aims to alert physicians to this diagnosis and the undeniable advantage of cardiac magnetic resonance.

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Heart failure due to congenital heart disease and cardiomyopathies is a significant burden in the pediatric population. Pharmacological strategies for the management of pediatric heart failure are largely based on the extrapolation of adult data and Delphi process based on expert opinion. There are differences in the etiology, clinical course, and outcome of pediatric heart failure as compared to adult, thus the results of adult heart failure trials cannot be simply extrapolated to pediatric patients. There have been a lot of newer drugs for adults with heart failure, but there is a void for pediatric population with heart failure due to many reasons. Early results of multi-centric randomized control PANORAMA HF Trial and subsequent Food and Drug Administration approval for Angiotensin Receptor and Neprylisin Inhibitor (Sacubitril / Valsartan) for pediatric patients have tried to fill in this void and paved the way for a newer class of drugs for heart failure with proven benefits in pediatric patients.

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Objectives: The aim of this study is to evaluate HHV-6 and PVB19 infection using polymerase chain reaction (PCR) and immunofluorescent assay (IFA) in the myocardium of pediatric patients with dilated cardiomyopathy (DCM) and the impact of viral persistence in the cardiac allograft after heart transplantation (HT). Methods: Multiplex droplet digital PCR was used to analyze the prevalence of viral sequences in myocardial samples from 48 pediatric DCM patients and 10 control subjects. Of the 48 DCM patients, 44 underwent HT. After HT, consecutive endomyocardial biopsy (EMB) samples were analyzed for the presence of PVB19 and HHV-6 antigens using IFA and the patients were evaluated for rejections, coronary vasculopathy, and graft loss. Results: Of the 48 DCM patients, 14 had positive viral PCR results in explanted/autopsy hearts. Among them, PVB19 was found in 8/48, HHV6 in 4/48, both PVB19 and HHV6 in 1/48, and enterovirus in one, but no adenovirus was found. The EMB samples obtained after HT were positive for PVB19 and HHV-6 in 7/44 and 3/44 cases, respectively. Viral presence in both the explanted heart and the cardiac allograft was demonstrated in 4 patients, 3 of whom were positive for PVB19, and one of whom was positive for HHV-6 pretransplant. Coronary vasculopathy and graft loss were more common in patients with PVB19-positive myocardial tissues versus those who were PVB19-negative. Conclusions: There is an association between PVB19 and HHV-6 infection and DCM in children. The study suggests the persistence of PVB19 and HHV-6 in the host can lead to subsequent viral reactivation in the transplanted heart, even in those recipients who do not have active myocarditis. PVB19 in the cardiac allograft tended toward higher adverse post-HT events.

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Aims : There are limited data regarding critical congenital heart disease (CCHD) from middle-income countries (MIC). This study aims to determine the birth prevalence, rate of late diagnosis, and influence of timing of diagnosis on the outcome of CCHD. Setting and Design : Retrospective observational cohort study in the State of Johor, Malaysia. Subjects and Methods : All infants born between January 2006 and December 2015 with a diagnosis of CCHD, defined as infants with duct-dependent lesions or cyanotic heart disease who may die without early intervention. The late diagnosis was defined as a diagnosis of CCHD after 3 days of age. Results : Congenital heart disease was diagnosed in 3557 of 531,904 live-born infants and were critical in 668 (18.7%). Of 668, 347 (52%) had duct-dependent pulmonary circulation. The birth prevalence of CCHD was 1.26 (95% confidence interval: 1.16–1.35) per 1000 live births, with no significant increase over time. The median age of diagnosis was 4 days (Q1 1, Q3 26), with 61 (9.1%) detected prenatally, and 342 (51.2%) detected late. The highest rate of late diagnosis was observed in coarctation of the aorta with a rate of 74%. Trend analysis shows a statistically significant reduction of late diagnosis and a significant increase in prenatal detection. However, Cox regression analysis shows the timing of diagnosis does not affect the outcome of CCHD. Conclusions : Due to limited resources in the MIC, the late diagnosis of CCHD is high but does not affect the outcome. Nevertheless, the timing of diagnosis has improved over time.

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Objective: Pulmonary valve regurgitation (PR) and right ventricular (RV) dilatation are important residual findings after surgical repair of tetralogy of Fallot (TOF). We sought to describe the natural course of RV dilatation over time in patients with severe PR after TOF repair and to determine risk factors for quick progression of RV dilatation and dysfunction. Methods: Data of 85 consecutive TOF patients with PR and RV dilatation, undergoing serial cardiovascular magnetic resonance (CMR) scans between July 2002 and December 2016 in two institutions, were retrospectively reviewed. The dataset was analyzed regarding right and left ventricular (LV) volume and function and potential risk factors of progressive RV dilatation Results: There was no significant increase in RV end-diastolic volumes (RVEDV_i) indexed body surface area (BSA) (median 150 [81–249] vs. 150 [82–260] mL/m²) and end-systolic volumes indexed for BSA (RVESV_i) (75 [20–186] vs. 76 [39–189] mL/m²) between the first and last CMR in the overall group. Similarly, there were no significant changes in LV volumes indexed for BSA (LVEDV_i 78 [56–137] vs. 81 [57–128] mL/m² and LV end-systolic volume index 34 [23–68] vs. 35 [18–61] mL/m2). Global function remained also unchanged for both ventricles. RVEDVi increased statistically significantly (≥ 20 mL/m2) in twenty patients (24%) from 154 mL/m² (87–237) to 184 mL/m² (128–260, P < 0.001). LV dimensions showed a similar trend with LVEDVi increase from 80 ml/m² (57–98) to 85 ml/m² (72–105, P = 0.002). Shorter time interval between repair and first CMR was the only risk factor predictive for progressive RV dilatation. Conclusion: In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years. RV dilatation seems to progress early after surgery and subsequently stabilize. RV dilatation significantly progresses in a subgroup of 24% of patients, with a shorter time interval since surgical repair.

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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.

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Background: There are no established criteria to decide suitability for Fontan fenestration closure. Our institution has the following criteria: an unobstructed Fontan pathway with no significant decompressing venovenous collaterals, baseline Fontan pressure ≤15 mmHg, baseline cardiac index ≤2 L/min/m², and a decrease in cardiac index ≥20% with test occlusion of the fenestration. Objective: The objective of the study was to review midterm outcomes following device closure of Fontan fenestration using institutional criteria. Materials and Methods: A retrospective review was performed of patients who underwent catheterization with prior fenestrated Fontan procedure between May 2005 and January 2015. Patients were classified as those who underwent successful closure (A), had closure deferred due to failure to meet criteria (B), or were not referred for closure (C). Results: There were 42 patients in Group A, 10 in Group B, and 150 in Group C. The mean Fontan pressure increased from 13.1 ± 2.1 to 14.5 ± 2.1mmHg in Group A and 14.6 ± 1.5 to 15.7 ± 2.2 mmHg in Group B (P = not significant). With test occlusion, cardiac index fell by 18.12% ± 15.68% in Group A and 33.75% ± 14.98% in Group B (P = 0.019). At a median of 46 month follow-up, oxygen saturation increased significantly from 85.15% ± 6.29% at baseline to 94.6% ± 4.43% (P < 0.001) in Group A but with no statistically significant difference in the rates of plastic bronchitis, protein-losing enteropathy, stroke, or heart transplantation between the three groups. Conclusions: Using institutional criteria, transcatheter device closure of Fontan fenestration was followed by significant increase in oxygen saturations and no statistically significant difference in morbidity or mortality between closure and nonclosure groups.

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We report the case of a 16-year-old girl diagnosed with myocarditis, although initial echocardiographic imaging was consistent with hypertrophic cardiomyopathy (HCM). The diagnosis of myocarditis was made with the findings of troponin elevation, presence of influenza A, and a more characteristic electrocardiogram. She eventually made a full recovery. Clinicians must be vigilant for such rare presentations of myocarditis masquerading as HCM.

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Introduction: Endothelin receptor antagonists (ERAs) are widely accepted as a specific treatment for pulmonary arterial hypertension. Unfortunately, consensus and recommendations are lacking for the treatment of patients who suffer from pulmonary arterial hypertension and congenital heart disease, including Eisenmenger syndrome. Objective: This meta-analysis aimed to compare the effect of ERA on patients with Eisenmenger syndrome. Methods: Electronic search on PubMed (MEDLINE), EBSCO, EuropePMC, Clinicaltrials.gov, and Google Scholar was done. Studies involving the use of ERAs on Eisenmenger syndrome patients were included. There were 18 studies included. The primary outcome of interest was the 6-min walking test distance before and after exposure to ERA. Results: There were 517 patients with Eisenmenger syndrome. The subjects had Eisenmenger syndrome secondary to congenital heart disorders, with WHO functional Class ranging from Class I–IV. The follow-up ranges from a mean of 4–60 months. Seventeen studies reported a statistically significant difference between pretreatment and the posttreatment result of 6-min walking test distance. Pooled mean difference comparing pre and posttreatment values yielded an increase of 55.24 m (42.15, 68.33) P < 0.001; moderate heterogeneity I² 51% P = 0.008. Pooled mean pulmonary vascular resistance index difference comparing pre and posttreatment values yielded a decrease of 4.76 woods unit (-6.86, -2.66), P < 0.001 favoring posttreatment; low heterogeneity I2 0%, P = 0.82. Pooled mean mean pulmonary arterial pressure difference comparing pre and posttreatment values yielded a decrease of 5.40 mmHg (-7.53, -3.28), P < 0.001 favoring posttreatment, low heterogeneity I² 0%, P = 0.65. Conclusion: Implementation of ERA in Eisenmenger improves 6-min walking distance and pulmonary vascular pressure indices. Earlier administration of ERA might be beneficial, further studies are needed to assess mortality benefit of this agent.

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Unilateral pulmonary agenesis or aplasia (UPA) in combination with congenital heart defects is rare and has not been reported in connection with transposition of the great arteries. This case demonstrated dextroposition of the fetal heart, and subsequent scans could not clearly visualize the right pulmonary artery. UPA should be considered in the workup and counseling for a family in the setting of fetal heart malposition, as there is a significant clinical impact.

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Deterioration following a successful balloon atrial septostomy (BAS) is not common. We report an unusual cause for the same. A thrombus in transit has not been previously reported following BAS.

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Ventricular septal defects (VSDs) comprise the most common congenital heart defect at birth. The chances of spontaneous closure of VSD depend on the size and location of the defect. Subpulmonic location is an unlikely site for the VSD to close spontaneously and known to have complications such as aortic valve prolapse and regurgitation. Percutaneous closure has become the preferred strategy for small–moderate-sized VSDs located in muscular, perimembranous areas. Subpulmonic location poses concerns due to the close proximity to the aortic valve. Herein, we present a case of percutaneous device closure of a subpulmonic VSD using ADO I occluder device.

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Ductal stenting is increasing as an alternative to surgical shunts in cyanotic newborns. However, most reports include newborns with patent ducts who are often on prostaglandin before the procedure. We report a successful late transcatheter recanalization of the closed ductus arteriosus in a 2-month-old infant with tetralogy of Fallot, right aortic arch, and an isolated left pulmonary artery (LPA). We achieved adequate LPA growth before complete repair. There were no procedural complications. This procedure is rare in Western countries due to universal pulse oximetry screening and increased fetal diagnoses. The procedure is technically challenging in the absence of any angiographic flow into the ductus. Evidence of a ductal ampulla increases the chances of success.

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Scimitar syndrome is a clinical triad of anomalous pulmonary venous drainage, lung hypoplasia, and anomalous aortic blood supply to the lung segment. When there is dual pulmonary venous drainage both to inferior vena cava and left atrium, it is called scimitar variant. A young child presenting with recurrent chest infections, dextroposition of the heart, and scimitar shadow on chest X-ray was identified to have scimitar variant after a detailed evaluation and managed successfully by catheter interventions. This report discusses the embryogenesis and angiographic findings of scimitar variants, indications for interventions, and issues in its management.

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The association of left isomerism with tetralogy of Fallot (TOF) is rare and reported as scattered case reports. Complex congenital cardiac and noncardiac malformations are known to be associated with left isomerism, but right-sided obstructive lesions are rare. We present our experience with a case of left isomerism with bilateral superior vena cava, interrupted inferior vena cava, and TOF managed by atrial septation and intracardiac repair.

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Congenital ventricular diverticulum is a rare congenital malformation of the heart. It remains mostly asymptomatic unless associated with other congenital cardiac defects. We present a case of a 4-month-old child who had dextrocardia, large inlet ventricular septal defect, amounting to single ventricle, and severe pulmonary artery hypertension. There was a right ventricular diverticulum which passed through the foramina of Morgagni to give rise to a pulsating lump in the epigastrium. Right ventricular diverticulum presenting with a pulsating mass in the abdomen is a rare entity. The confirmation of diverticulum was made with histopathology.

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We describe the use of a novel interventional approach to the histopathologic diagnosis of a ventricular septal tumor using intraoperative ultrasound-guided trans-epicardial biopsy without the need for cardiopulmonary bypass in a 2-year-old child. This novel approach has not been previously reported. Multidisciplinary collaboration between cardiothoracic surgery, cardiology, cardiac imaging, and interventional radiology provided the ability to perform cardiac biopsy. This technique may be used in specific cases of cardiac tumors where tissue diagnosis is important, but surgical resection is deemed excessively risky or impossible.

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A 16-year-old girl presented to the emergency department with orthopnea and exercise intolerance for 1 week. Electrocardiogram at that time showed rightward deviation of the cardiac axis, with nonspecific T-wave inversion in the lateral precordial leads and flattening in the inferior leads. She was discharged from the emergency department with cardiology follow-up. Subsequent echocardiogram demonstrated that “the heart is in a funny position in the chest,” and computed tomography of the chest was requested to evaluate for a mediastinal mass causing leftward displacement of the heart.

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Giant left atrial appendage aneurysms (LAAAs) are rare causes of recurrent cardioembolism and managed routinely by surgery. A first catheter closure of a giant LAAA is reported, when a recent cerebral infarct precluded immediate surgery. Planning included ostial measurement on multimodal imaging, echo navigation for septal puncture, rotational angiogram for profiling, overlay imaging for device placement, and cerebral embolic protection from thrombus debris.

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