Background: Bacterial infection (BI) after congenital heart surgery (CHS) is associated with increased morbidity and is difficult to differentiate from systemic inflammatory response syndrome caused by cardiopulmonary bypass (CPB). Procalcitonin (PCT) has emerged as a reliable biomarker of BI in various populations. Aim: To determine the optimal PCT threshold to identify BI among children suspected of having infection following CPB. Setting and Design: Single-center retrospective observational study. Setting and Design: Single-center retrospective observational study. Statistical Analysis: The Wilcoxon rank-sum test was used for nonparametric variables. The diagnostic performance of PCT was evaluated using a receiver operating characteristic (ROC) curve. Results: Ninety-eight patients were included. The median age was 2 months (25th and 75th interquartile of 0.1-7.5 months). Eleven patients were included in the BI group. The median PCT for the BI group (3.42 ng/mL, 25th and 75th interquartile of 2.34-5.67) was significantly higher than the median PCT for the noninfected group (0.8 ng/mL, 25th and 75th interquartile 0.38-3.39), P = 0.028. The PCT level that yielded the best compromise between the sensitivity (81.8%) and specificity (66.7%) was 2 ng/mL with an area under the ROC curve of 0.742. Conclusion: A PCT less than 2 ng/mL makes BI unlikely in children suspected of infection after CHS.

Background: Rheumatic heart disease (RHD) is a global cause of early heart failure. Early RHD is characterized by valvar regurgitation, leading to ventricular distention and possible elaboration of amino-terminal pro-brain natriuretic peptide (NT-proBNP). We investigated the ability of NT-proBNP to distinguish cases of latent RHD detected by echocardiographic screening from the controls. Materials and Methods: Ugandan children (N = 44, 36% males, mean age: 12 ± 2 years) with latent RHD (cases) and siblings (controls) by echocardiography were enrolled. Cases and controls were matched for age and sex, and they had normal hemoglobin (mean: 12.8 mg/dL). Children with congenital heart disease, pregnancy, left ventricular dilation or ejection fraction (EF) below 55%, or other acute or known chronic health conditions were excluded. RHD cases were defined by the World Heart Federation (WHF) 2012 consensus guideline criteria as definite. Controls had no echocardiography (echo) evidence for RHD. At the time of echo, venous blood samples were drawn and stored as serum. NT-proBNP levels were measured using sandwich immunoassay. Paired t-tests were used to compare NT-proBNP concentrations including sex-specific analyses. Results: The mean NT-proBNP concentration in the cases was 105.74 ± 67.21 pg/mL while in the controls, it was 86.63 ± 55.77 pg/mL. The cases did not differ from the controls (P = 0.3). In sex-specific analyses, male cases differed significantly from the controls (158.78 ± 68.82 versus 76 ± 42.43, P = 0.008). Female cases did not differ from the controls (75.44 ± 45.03 versus 92.30 ± 62.35 respectively, P = 0.4). Conclusion: Serum NT-proBNP did not distinguish between latent RHD cases and the controls. Sex and within-family exposures may confound this result. More investigation into biomarker-based RHD detection is warranted.

Background: Heart disease is the major cause of death in thalassemia patients. Repeated blood transfusions and hemolysis cause iron overload and also disrupts the hydroxylation and synthesis of vitamin D, causing vitamin D deficiency. Vitamin D deficiency is associated with cardiac dysfunction. Objective The purpose of this study was to determine the association between vitamin D levels and left ventricular function and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels in thalassemia major children with iron overload. Patients and Methods: A cross-sectional study was conducted in March-April 2015 in the thalassemia clinic, Department of Child Health, Dr. Hasan Sadikin General Hospital, Bandung, Indonesia. Thirty-four children with thalassemia were enrolled consecutively. Serum vitamin D and NT-proBNP levels were measured with electrochemiluminescence (ECLIA) method and echocardiography was performed to assess ventricular function. Results: Significant correlations were found between vitamin D levels and left ventricular ejection fraction (LVEF) (r = 0.399, P = 0.019) and fractional shortening (FS) (r = 0.394, P = 0.021). There was also significant correlation between vitamin D and NT-proBNP levels (r = -0.444, P = 0.008). Chi-square analysis also showed a relationship between vitamin D and NT-proBNP (P = 0.019) levels. There was a difference in NT-proBNP levels among thalassemia major children with iron overload (P = 0.020). Post hoc analysis showed that there was a significant difference in NT-proBNP levels between those with vitamin D deficiency and those with normal vitamin D levels (P = 0.012). Conclusion: There is an association between vitamin D and left ventricular function and NT-proBNP levels in children with thalassemia major and iron overload. Vitamin D can be considered in patients with thalassemia having vitamin D deficiency.

Objective: The aim of this study was to determine normal values for fetal left ventricular (LV)-myocardial performance index (MPI) in Indian population and to assess its relation to advancing gestation and fetal heart rate (FHR). Materials and Methods: Two hundred pregnant women without any pregnancy-related complications and whose fetuses were shown to have structurally normal hearts were enrolled in this study. Doppler waveform involving simultaneous display of mitral inflow and LV outflow was obtained in all. Various intervals including isovolumetric contraction time (IVCT), isovolumetric relaxation time (IVRT), and ejection time (ET) were measured and then the MPI was calculated using the formula IVCT + IVRT/ET. Also the correlation between MPI and gestation age and FHR was assessed. We also reviewed the literature on the use of MPI for the assessment of fetal LV function. Results: The normal MPI in second and third trimester fetuses of Indian population was 0.42 ± 0.03. The mean IVCT was 33 ± 4 milliseconds (ms), mean IVRT was 39 ± 5 ms, and mean ET was 169 ± 9 ms. The mean heart rate was 148 ± 8 bpm and the mean PR interval was 111 ± 10 ms. There was no significant association of LV-MPI with either FHR or advancing gestation. Conclusion: MPI is a useful parameter for the assessment global cardiac function. MPI has the advantage of not being affected by FHR, ventricular size, and geometry or image quality. The review of literature shows its significant importance in monitoring complicated pregnancies.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a highly malignant inheritable cardiac channelopathy. The past decade and a half has provided exciting new discoveries elucidating the genetic etiology and pathophysiology of CPVT. This review of the current literature on CPVT aims to summarize the state of the art in our understanding of the genetic etiology and the molecular pathogenesis of CPVT, and how these relate to our current approach to diagnosis and management. We will also shed light on groundbreaking new work that will continue to refine the management of CPVT in the future. As our knowledge of CPVT continues to grow, further studies will yield a better understanding of the efficacy and pitfalls of established diagnostic approaches and therapies as well as help shape newer diagnostic and treatment strategies. Two separate searches were run on the National Center for Biotechnology Information's (NCBI) website. The first used the medical subject headings (MeSH) database using the term “catecholaminergic polymorphic ventricular tachycardia” that was run on the PubMed database using the age filter (birth to 18 years), and it yielded 58 results. The second search using the MeSH database with the search term “catecholaminergic polymorphic ventricular tachycardia,” applying no filters yielded 178 results. The abstracts of all these articles were studied and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles were further explored and read in full.

Pulmonary vascular disease develops early in untreated single ventricle patients with increased pulmonary flow. Pulmonary artery (PA) banding is done at a young age in these patients in order to protect the lung vasculature and maintain low pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). This also enables future completion of the single ventricle palliation. Pulmonary venous hypertension (PVH) secondary to left sided obstruction if present in addition in this setting contributes to the pulmonary arterial hypertension (PAH) but involves an element of reversibility of the PAH if the obstruction is relieved. We present two cases of single ventricle both of who re-presented late with PAH and PVH (secondary to mitral valve obstruction) and underwent delayed PA banding at 9.5 and 4.5 years of age respectively. Both patients however had different outcomes. The patient undergoing PA banding at 9.5 years successfully underwent a cavo-pulmonary shunt at the age of 12 years. The patient with PA banding at 4.5 years however, has residual PAH that presently precludes a cavo-pulmonary shunt.

Percutaneous treatment of totally occluded coarctation of the aorta has been reported predominantly in adults. The success and challenges of this procedure in children is reported in few patients. We report an outcome of percutaneous treatment of three children with completely occluded coarctation of the aorta. The age range was 9-14 years. All the patients had upper limb hypertension. One case had severe left ventricular dysfunction. In all cases, a pediatric Brockenbrough needle and a covered stent were implanted. Recanalization and implantation of a covered stent was successful in all patients. One of these patients developed transient postcoarctectomy syndrome. Percutaneous recanalization of totally occluded coarctation of the aorta using Brockenbrough needle and a covered stent in children is feasible and effective.

We report a 14-year-old boy who presented with acute chest pain, following the ingestion of loperamide for acute diarrhea. Twelve lead electrocardiogram (ECG) showed evidence of acute ischemia indicating acute coronary artery spasm. The changes reverted with treatment within a few hours with no permanent effect on myocardial function. This report highlights a rare side effect of loperamide, often debated in adults and never reported in adolescents.

Go to: We report a case of an atrial septal defect where a Figulla Occlutech device embolized into the right ventricle. As this device has no left atrial hub, we brought the bare device into the inferior vena cava and used a novel two-snare technique to slenderize the device into the sheath. This report highlights additional tips in transcatheter device retrieval.

A 13-year-old boy presented with vague musculoskeletal pain and involvement of multiple small and large joints along with axial skeleton for the last 3 years, poorly responsive to aspirin. However, on account of presence of carditis and fulfilment of Jones criteria, a diagnosis of acute rheumatic fever (ARF) with atypical arthritis was made. We report this case to break the myth and sensitize pediatricians and rheumatologists to keep the possibility of atypical articular presentations, as in our case, in patients with ARF and prevent delayed diagnosis and treatment.

We report a successful surgical management of a case presented with a combination of aortopulmonary window (APW) with large ventricular septal defect (VSD) amounting to a single ventricle, with a view to highlight technical considerations during staged single-ventricle palliation.

An unusual case of a rare vascular ring, which has been called right aortic arch with aberrant left innominate artery, is presented. The appearance of this case led to the realization that there is really no innominate artery present in this anomaly but only the left dorsal aorta. We present a clarification of the nature and likely development of the vessels present.

Repair strategies in scimitar syndrome are varied and need to be individualized to the surgical anatomy. This report focuses on the repair achieved in a case with unusual anatomy of the scimitar vein.

Hereditary hemorrhagic teleangiectasia (HHT) is a genetic disorder, characterized by abnormal vessel formation and arteriovenous malformations (AVMs). The so-called “Curaçao criteria” are most commonly employed for the purposes of clinical diagnosis. However, children may not exhibit the full magnitude of symptoms and the Curaçao criteria appear to be less sensitive in this setting. We describe a family, in which two members were clinically diagnosed with HHT and referred for genetic testing. As there were phenotypic features suggesting the high likelihood of combined syndrome of juvenile polyposis with hereditary hemorrhagic teleangiectasia (JPHT), we proceeded with genetic testing of SMAD4 gene as initial step, which revealed a novel frameshift mutation. This case shows the variety of challenges that clinicians and genetic laboratories may face in complex cases such as combined JPHT syndrome. Knowledge of the syndrome features is of paramount importance as they could frequently point at the most appropriate gene to be tested.

Plastic bronchitis is a rare life-threatening complication of the Fontan operation. Transcatheter Fontan fenestration can ameliorate symptoms by decompressing elevated venous pressures. Transcatheter creation of a fenestration can be technically challenging in cases with complex venous anatomy. We report a case of a 5-year-old boy with heterotaxy, dextrocardia with unbalanced atrioventricular canal (AVC), atrial and visceral situs inversus, left-sided superior vena cava (SVC), and left-sided interrupted inferior vena cava (IVC) with azygos continuation. With few modifications to the equipment, a successful Fontan fenestration with stent implantation was performed via transjugular approach. At 2-year follow-up, his symptoms of plastic bronchitis improved significantly.

A 1.7 kg infant with obstructed supracardiac total anomalous pulmonary venous drainage (TAPVD) presented with severe pulmonary hypertension secondary to vertical vein obstruction. The child, in addition, had a large omphalocele that was being managed conservatively. The combination of low weight, unoperated omphalocele, and severe pulmonary hypertension made corrective cardiac surgery very high-risk. Therefore, transcatheter stenting of the stenotic vertical vein, as a bridge to corrective surgery was carried out. The procedure was carried out through the right internal jugular vein (RIJ). The stenotic segment of the vertical vein was stented using a coronary stent. After procedure, the child was discharged well to the referred hospital for weight gain and spontaneous epithelialization of the omphalocele. Stenting of the vertical vein through the internal jugular vein can be considered in very small neonates as a bridge to repair obstructed supracardiac total anomalous venous drainage.

If the atrial appendages lie adjacent to each other on same side of the great arteries, instead of encircling their roots, they are referred as juxtaposed. Right juxtaposition of atrial appendages is less common than left juxtaposition. The images demonstrate the classical radiological, echocardiographic, and surgical images of juxtaposed atrial appendages. Their clinical incidence, associations, and relevance during interventional and surgical procedures are discussed.

Cardiomyopathy is an important cause of morbidity and mortality in patients with Duchenne muscular dystrophy (DMD). Early recognition of myocardial involvement and initiation of therapy are important for improved outcomes. Cardiac magnetic resonance imaging (CMR) is a sensitive tool in early detection of myocardial fibrosis in these children.

Changes in left coronary artery flow pattern in anomalous left coronary from pulmonary artery can provide valuable insight into pathology and natural history of disease. We wish to discuss a case with pre and post operative left coronary flow pattern with mid term follow up.

Congenital ventricular diverticulum is a rare cardiac anomaly defined as a localized protrusion of the ventricular free wall. Although, it is usually asymptomatic, complications such as embolism, infective endocarditis, and arrhythmias can occur. The diagnosis can be made by echocardiography, cardiac magnetic resonance imaging, or catheter angiography. Surgical resection is the treatment of choice in symptomatic patients, whereas the management of asymptomatic patients often represents a therapeutic dilemma. We report here, a 9-month-old patient with asymptomatic congenital left ventricular (LV) diverticulum associated with epigastric hernia.