Background: Cardiac rhabdomyoma (CR) often shows spontaneous regression and needs close follow-up. These tumors may be associated with tuberous sclerosis complex (TSC), caused by the disinhibition of m-TOR protein. Objective: The aim of the study is to observe the efficacy of everolimus in infants with significant CR. Materials and Methods: This is a single-center prospective observational study including infants with significant CR causing either clinical symptoms or obstruction to the blood flow. Everolimus was administered at a dose of 4.5 mg/M2/wk till the symptomatic improvement. Liver and renal function tests were monitored during treatment. Results: There were six cases of suspected CR included in the study. Median age and weight of patients were 5 days (range: 1–90 days) and 3.2 kg (range: 2.2–4.5 kg), respectively. One patient was excluded after surgical excision biopsy during concomitant closure of associated large perimembranous ventricular septal defect confirmed it as a fibroma. The remaining all five cases showed regression of tumor during mean follow-up of 6.1 ± 5.1 months. One child developed varicella infection necessitating temporary discontinuation of medicine during follow-up. One case had sudden death at 4 months of age. Conclusion: Everolimus appears to be useful in selected cases of symptomatic CR. Multicentric studies are needed to determine its safety and efficacy in larger population.

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Background: Evaluating outcome and identifying predictors of major complications among children undergoing cardiac surgery are essential to improve care. We evaluated short-term outcomes of postcardiac surgery and predictors of major complications in a national referral hospital in Indonesia. Methods: A prospective cohort study was conducted from April 2014 to March 2015 on all children undergoing cardiac surgery. Participants were followed up from the time of surgery until hospital discharge and 30-day mortality. We performed univariate and multivariate logistic regression using STATA 12-1 to identify predictors of postsurgical major complications. Results: A total of 257 patients (median age: 36 months) were recruited; 217 (84.1%) had complications, including low cardiac output syndrome (19.8%), arrhythmia (18.6%), sepsis (17.4%), and pleural effusion (14.8%). Forty-nine (19%) patients had major complications, including cardiac arrest (5%), need for emergency chest opening (3.9%), and multiple organ failure (7.4%). 12.8% died during hospital stay, and 30-day mortality was 13.6%. Predictors of major complications were cyanotic congenital heart disease (odds ratio [OR]: 4.6, 95% confidence interval [CI]: 1.5–14.2), longer duration of cardiopulmonary bypass (CPB, OR: 4.4, 95% CI: 1.5–13.4), high inotropes (OR: 13.1, 95% CI: 3.2–54.2), and increase in lactate >0.75 mmol/L/h or more in the first 24 h (OR: 37.1, 95% CI: 10.1–136.3). Conclusion: One-fifth of children undergoing cardiac surgery experienced major complications with around 13% mortality. Cyanotic congenital heart disease, longer duration of CPB, high inotropes on leaving operating theater, and increase in blood lactate are associated with major complications in children after cardiac surgery.

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Background: Cardiovascular morbidity (CVM) is the main etiology of mortality in children and adolescents with chronic kidney disease (CKD). CKD associated cardiovascular mortality is more common in children with diastolic cardiac dysfunction which was considered as an early indicator for death, while increased left ventricular mass (LVM) is a strong independent risk factor for these patients. Vitamin D deficiency was previously studied as one of the risk factors for CVM. Aim: The aim of the work was to investigate the relationship between biomarkers of mineral bone disorder including serum 25(OH) Vitamin D3 (25-OH D3), phosphorus and calcium × phosphorus (Ca×Po4) product with diastolic cardiac function and LVM in children and adolescents with CKD. Subjects and Methods: This was a cross-sectional observational study. Participants were classified into two groups: Group I including 86 pediatric patients with CKD (stages 4 or 5) and Group II including 40 healthy controls. Group I was subdivided into IA included children with diastolic dysfunction and IB included cases without diastolic dysfunction. 25-OH D3 level was measured by enhanced chemiluminescence method and intact parathyroid hormone (iPTH) by electrochemiluminescence method. Parameters for diastolic function and LVM were assessed by Doppler echocardiography, tissue Doppler imaging, and M-mode echocardiography. Results: 25-OH D3 level was significantly lower in Group I when compared to Group II. Diastolic dysfunction was present in 48.8% of the studied patients and was significantly associated with increased serum phosphorus and calcium-phosphorus product but not with decreased level of 25-OH D3. There was a significant positive correlation between LVM and iPTH. Conclusions: Hyperphosphatemia and high Ca×Po4 product were considered of prognostic value as they predict early diastolic dysfunction and increased LVM in children with CKD.

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Objective: We compared the pre- and post-operative right ventricular (RV) function by tricuspid annular plane systolic excursion (TAPSE) between trans-right atrial (t-RA) versus t-RA/RV (RA/RV) approach for the repair of Tetralogy of Fallot (TOF). Patients and Methods: Fifty consecutive patients, 1–15 years of age, undergoing intracardiac repair of TOF between September 2015 and June 2016 were randomized into two groups based on the approach for repair as follows: t-RA or t-RA/RV approach. TAPSE was used for the assessment of pre- and post-operative RV function. Results: Age, body surface area, preoperative saturation, cardiopulmonary bypass and aortic cross-clamp times, inotropic score, postoperative intensive care unit, and hospital stay were similar in both the groups. However, t-RA/RV group had significant mediastinal drainage (169 ± 163 ml vs. 90.6 ± 58.7 ml, P < 0.05) and pleural effusions (8 vs. 2 patients, P < 0.05), but had better relief of RV outflow tract gradients. The mean follow-up was 23 ± 6.7 (median 26, range 21–29) months. There were no differences in arrhythmias in either group up to the 1^st month and at last follow-up. Preoperative TAPSE for t-RA and t-RA/RV was similar (1.49 ± 0.29 vs. 1.66 ± 0.34, P > 0.05) and so was the post-operative TAPSE at discharge (1.52 ± 0.30 vs. 1.43 ± 0.32, P > 0.05), at 1 month (1.6 ± 0.27 vs. 1.43 ± 0.032, P > 0.05) and at last follow-up (1.79 ± 0.15, median 1.8 vs. 1.72 ± 0.17, median 1.7 P > 0.05). Conclusion: Both t-RA and t-RA/RV approaches provide safe palliation for patients with TOF. A limited right ventriculotomy neither leads to deleterious effects on early RV function nor does it increase the incidence of arrhythmias at early follow-up. Larger studies with longer follow-up are needed to further address these issues.

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Tuberculat myocarditis is an extremely rare entity with few published reports. We report a 13 year old male who presented with ventriculat tachycardia. On evaluation he was observed to have MRI features and workup consistent with nodular variant of tubercular myocarditis. Child had fatal outcome on follow up.

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Background: Iron-induced cardiomyopathy remains the leading cause of mortality in patients with β-thalassemia major. Iron overload cardiomyopathy, which may be reversible through iron chelation, is characterized by early diastolic dysfunction. Amino-terminal pro-brain natriuretic peptide (NT-proBNP) is a sensitive biomarker of diastolic dysfunction. Aim: The aim of the study is to evaluate the diagnostic value of NT-proBNP as a surrogate marker of iron overload examined with magnetic resonance imaging T2-star (MRI T2*). Methods: Sixty-eight β-thalassemia major patients (10–18 years) with no signs of heart failure underwent NT-proBNP measurement before routine transfusion. All participants prospectively underwent cardiac MRI T2* examination within 3 months (median 19 days). Patients were divided as cardiac hemosiderosis (cardiac MRI T2* <20 ms) and nonhemosiderosis (cardiac MRI T2* >20 ms). Results: Of 68 patients, the male-to-female ratio was 1:1.1 and the median age was 14.1 years (range: 10–17.8 years). NT-proBNP levels were not different between hemosiderosis and nonhemosiderosis patients (P = 0.233). Further receiver operating characteristic analysis resulted in no significant correlation of NT-proBNP and MRI T2* (area under the curve 0.393, P = 0.233). Conclusion: Measurement of NT-proBNP levels cannot be used for early detection of cardiac iron overload in adolescent with β-thalassemia major.

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Background: Application of transannular patch (TAP) during the repair of tetralogy of Fallot (TOF) leads to the development of pulmonary regurgitation (PR). This PR is known to cause right ventricular (RV) volume overload and dysfunction which in turn leads to increase in both morbidity and mortality both in immediate and long-term periods. Here, we sought to analyze the effects of polytetrafluoroethylene (PTFE) pulmonary bicuspid valve on the early outcome of patients with TOF repair where TAP is needed. Subjects and Methods: This is a retroprospective, observational study where PTFE bicuspid pulmonary valve was incorporated in all consecutive patients undergoing repair of TOF involving the application of TAP. Postoperative inhospital course was assessed, and patients were followed till 6 months for the status of PR and peak RV outflow tract (RVOT) gradient. Results: 38 patients were enrolled in the study. The mean age of participants was 53.66 ± 78.67 months. Extubation in operating room was done in 31 (81.58%) patients. Duration of ventilator support was 10.48 ± 24.22 h, and duration of hospital stay was 7.95 ± 3.08 days. These data were compared with three recent studies where only TAP was applied. Extubation in operating room, mechanical ventilation time, and hospital stay were significantly improved in the present study. Out of 37 live patients, four (10.81%) patients had no PR, 28 (75%) had mild, 1 (2.7) had moderate, and 4 (10.81%) had severe PR. Average RVOT peak gradient was 18.8 ± 8.1 mmHg. Conclusion: Incorporation of PTFE bicuspid pulmonary valve during the repair of TOF appears to facilitate early extubation and thus reduces ventilator-associated comorbidities. Study needs further long-term follow-up to look for behavior of PTFE bicuspid valve.

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Unroofed coronary sinus, an unusual form of interatrial communication, is a rare cardiac anomaly. It is not a true defect of the atrial septum. It is described as a partial (focal or fenestrated) or complete absence of the roof of the coronary sinus, resulting in a communication between the coronary sinus and left atrium. It is presumably the least common variety of defects associated with interatrial shunting. Such defects are often difficult to diagnose and may even be overlooked during surgery for complex congenital heart disease. In most cases, they are associated with a persistent left superior vena cava, pulmonary or tricuspid atresia, and hearts with isomeric right atrial appendages. We report a case of this unusual form of interatrial communication without any associated anomalies in a 7-year-old girl child who presented to us with exertional dyspnea.

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Aneurysmal dilatation of the vertical vein in a case of supracardiac total anomalous pulmonary venous connection (TAPVC) is an extremely rare condition. It has been reported to occur secondary to severe compression of the vertical vein when it traverses between the left bronchus and the left pulmonary artery. We present a 14-year-old male with supracardiac TAPVC with a vertical vein aneurysm, probably secondary to stenosis caused by a thin membranous web just proximal to the aneurysm.

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Objective: The objective of this study is to evaluate the neutrophil-to-lymphocyte ratio (NLR) and platelet–lymphocyte ratio, from the hemograms obtained from children and adolescents with dilated cardiomyopathy (DCM), and to correlate them with the levels of B-type natriuretic peptide (BNP) and with the clinical evolution of these patients in the long term. Materials and Methods: Follow-up of 57 patients with DCM was made retrospectively, with hemogram and BNP level determination being performed after optimized therapy for heart failure. We compared the findings of the patients' examinations that progressed with stability in relation to the occurrence of transplant listing, cardiac transplantation, or evolution to death. Results: The average age was 48 months, and the follow-up was 64 months. The average of the levels of neutrophils was greater in poor evolution group (7026 vs. 3903; P = 0.011) as well as the average of NLR (5.5 vs. 1.9; P = 0.034). The averages of hemoglobin, total leukocytes, lymphocytes, and platelets were similar in the groups. The area under the receiver operating characteristic curve for NLR in relation to the poor evolution was of 72.9%, being the best cutoff point of NLR ≥5.2 (sensitivity: 93.8% and specificity: 87.8%). Kaplan–Meier curves demonstrate that patients with NLR ≤5.2 (P = 0.001) and BNP <1000 pg/dl (P < 0.0001) presented greater survival. Conclusions: NLR (≥5.2) and lymphopenia (≤1000 lymphocyte/μL) were associated with a poor prognosis and a higher chance of evolution to death or cardiac transplant, similar to the findings for BNP.

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Scimitar syndrome (SS) can be repaired by different surgical techniques including direct implantation, intracardiac rerouting, and in situ pericardial channel to direct the Scimitar vein (SV) to the left atrium. The presence of several anatomical variations such as remote infradiaphragmatic drainage of the SV and abnormal situs makes the repair more challenging with conventional repair techniques. In this paper, we present our experience in using an extracardiac-ringed polytetrafluoroethylene conduit in two pediatric patients (14 months and 2 years old) with SS.

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Aortic arch obstruction and bronchial compression are possible postoperative complications in patients with truncus arteriosus communis (TAC) with interrupted aortic arch (IAA). We present a case of bronchial compression as adverse effect of right pulmonary artery (PA) stenting in an infant with TAC (Type 4A)-IAA (Type B) repair. Due to growth potential, self-expandable metal stent was applied for postoperative proximal right PA stenosis, which caused bronchial compression. Later, we found patient having bronchomalacia. Surgical removal was needed. Stent insertion might be an option for postoperative PA stenosis, but bronchial compression might be a potential risk, particularly in patients with bronchomalacia.

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Bicuspidity of both the semilunar valves is rarely reported. We report the first ever case of bilateral bicuspid semilunar valves in a case of transposition.

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Fontan completion in patients with complex cardiac anatomy, and specifically heterotaxy syndrome, can present unique physiologic considerations. For example, existing venous connections may be “unmasked” after a cavopulmonary anastomosis operation. We present the case of a child with heterotaxy, dextrocardia, single-ventricle physiology, and anomalous hepatic venous drainage that resulted in profound shunting and cyanosis. We addressed the problem utilizing a novel strategy with a “fenestrated” covered stent.

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