Scimitar syndrome is a rare variant (5%) of partial abnormal pulmonary venous return. Surgery is required when pulmonary overcirculation is present. Following repair, Scimitar vein stenosis occurs in approximately 20%. We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. This tunneling technique allowed tension-free anastomosis and minimal Scimitar vein rotation.

In this study, we describe an infant case of pulmonary atresia with intact ventricular septum associated with ventriculo-coronary arterial communication for which a modified Blalock–Taussig shunt operation was performed. He experienced repeated myocardial ischaemic attacks. Further examination revealed pulmonary sequestration in the right lower lobe. He therefore underwent a bidirectional Glenn operation and coil occlusion of the feeding arteries. His myocardial ischaemic attacks subsequently improved.

Hepatoblastoma is the most common primary liver tumour in children; however, there are no reported incidences in the single-ventricle congenital heart disease population. We present three cases in patients with single-ventricle heart disease with a mean age at diagnosis of 19.7 (±4) months. The diagnosis was made prior to Fontan completion in all three cases. These represent the only documented hepatoblastoma cases in single-ventricle patients.

Aneurysms of the pulmonary arteries and trunk are rare entities. The Waterston shunt is a palliative procedure for children with cyanotic CHD due to obstruction of the pulmonary outflow. Described complications are distortion of the pulmonary artery and pulmonary arterial hypertension. We report a patient with a giant right pulmonary artery aneurysm in relation to a Waterston shunt.

Background: In this study, we aim to investigate the simulation of the cardiovascular system using an electronic circuit model under normal and pathological conditions, especially the Eisenmenger syndrome. Methods and Results: The Eisenmenger syndrome includes a congenital communication between the systemic and pulmonary circulation, with resultant pulmonary arterial hypertension and right-to-left reversal of flow through the defect. When pulmonary vascular resistance exceeds systemic vascular resistance, it results in hypoxaemia and cyanosis. The Westkessel model including Resistor-Inductance-Capacitance pi-segments was chosen in order to simulate both systemic and pulmonary circulation. The left and right heart are represented by trapezoidal shape stiffness for better simulation results. The Eisenmenger syndrome is simulated using a resistance (septal resistance) connected between the left ventricle and right ventricle points of the model. Matlab® is used for the model implementation. In this model, although there is a remarkable increase in the pulmonary artery pressure and right ventricle pressure, left ventricle pressure, aortic pressure, aortic flow, and pulmonary compliance decrease in the Eisenmenger syndrome. In addition, left-to-right septal flow reversed in these diseases. Conclusion: Our model is effective and available for simulating normal cardiac conditions and cardiovascular diseases, especially the Eisenmenger syndrome.

Outpatient management of patients with structurally or functionally abnormal cardiac valves combines an ability to record an appropriate history, a directed physical examination, a knowledge of both the interpretation as well as the context of any associated testing, and an understanding of the care guidelines in the medical literature. This article attempts to integrate these various features to guide the clinician towards more appropriate and timely management of patients with cardiac valve disease.

Abnormalities of chromosomes 11 and 22 are associated with congenital cardiac disease and/or various syndromes. We present a patient with partial trisomy for the long arms of chromosomes 11 and 22, the result of a maternal balanced reciprocal translocation between these two chromosomes. Our patient was dysmorphic and had coarctation of the aorta, an atrioventricular septal defect with common atrioventricular junction and exclusively atrial shunting, patency of the arterial duct, supracardiac totally anomalous pulmonary venous connection, a single kidney, and tracheobronchomalacia. This patient is unusual in having extensive left-sided cardiac involvement, a feature not usually found in this condition.

We describe two infants having an atrioventricular septal defect in the setting of a double inlet atrioventricular connection, but with patency of the left-sided valvar orifice and an imperforate right-sided valvar component, and a further case with atrioventricular septal defect and an imperforate Ebstein's malformation, all producing the haemodynamic effect of tricuspid atresia. We make comparisons with the arrangement in trisomy 16 mice, in whom deficient atrioventricular septation is seen at times with the common atrioventricular junction exclusively connected to the left ventricle, a situation similar to that seen in two of our infants. We also review previous reports emphasising the important theoretical implication of the findings despite their rarity.

In medical research, continuous variables are often categorised into two or more groups before being included in the analysis; this practice often comes with a cost, such as loss of power in analysis, less reliable estimates, and can often leave residual confounding in the results. In this research report, we show this by way of estimates from a regression analysis looking at the association between acute kidney injury and post-operative mortality in a sample of 194 neonates who underwent the Norwood operation. Two models were developed, one using a continuous measure of renal function as the main explanatory variable and second using a categorised version of the same variable. A continuous measure of renal function is more likely to yield reliable estimates and also maintains more statistical power in the analysis to detect a relation between the exposure and outcome. It also reveals the true biological relationship between the exposure and outcome. Categorising a continuous variable may not only miss an important message, it can also get it wrong. Additionally, given a non-linear relationship is commonly encountered between the exposure and outcome variable, investigators are advised to retain a predictor with a linear term only when supported by data. All of this is particularly important in small data sets which account for the majority of clinical research studies.

Cardiac tumours are relatively uncommon, particularly in children. Myofibroma is an extremely rare variety of cardiac tumour, which nearly always arises in the context of infantile myofibromatosis. Herein, we present a case of a solitary cardiac myofibroma causing right ventricular outflow tract obstruction in a 2-month-old male infant.

Proton pump inhibitors are widely used agents in the treatment of dyspepsia, and their effects on ventricular repolarisation through ion channels are well-known. Our aim is to evaluate the change in ventricular repolarisation parameters on electrocardiogram before and after proton pump inhibitor treatment. This study included 69 patients who had symptoms such as burning stomach pain, bloating, nausea, and heartburn for at least 3 months. Electrolyte levels of the patients were measured before and after treatment, and 12-lead electrocardiograms were taken at the initial and 1st month follow-up visit. Heart rate, QT interval, corrected QT (QTc), QT dispersion (QTd), QTc dispersion (QTcd), Tp-e measurements, and Tp-e/QT ratio were calculated and compared. Thirty-nine of the patients were girls, 30 were boys, and the mean age was 13.16 ± 3.02 years. Electrolyte levels of the patients before and after treatment were within the normal range. There was no statistically significant difference in the QTc, the Tp-e duration, or the Tp-e/QT ratio of the patients before and after treatment. We did not find a significant prolongation in the QTc duration or any other ventricular repolarisation parameters after proton pump inhibitor treatment in children with dyspepsia. We did not observe ventricular arrhythmia in our patients during follow-up. However, different results might be obtained with a larger sample and a longer follow-up period. These patients may have an increased risk of developing ventricular arrhythmias. Therefore, precaution should be taken when using drugs that prolong the QT period, and follow-up with serial electrocardiograms should be planned.

We present a case of an 11-year-old Indonesian female who was referred to our facility after surgical excision of brain abscess. The patient has been previously diagnosed with right atrial isomerism, complete atrioventricular septal defect, and L-Transposition of great arteries. Multiple staged surgeries are required for the management of this condition.

Charles H. Mayo stated “the great contribution we can make is to prepare the oncoming generations to think they can and will think for themselves”. The Hippocratic Oath also indicates that physicians have an obligation to transmit their knowledge to the next generation. Hence, it is important to find time and resources for education even in busy clinical practices.

Premature ventricular contractions are a rare side effect of filgrastim, reported mainly in elderly men. Here we report the case of a 9-year-old child with thalassaemia who developed frequent premature ventricular contractions after three doses of filgrastim were given for deferiprone-induced agranulocytosis. The arrhythmia resolved 3 weeks after discontinuation of filgrastim. Children treated with filgrastim should be carefully monitored for potentially serious arrhythmia.

“The oath of hippocrates states the following:To hold him who has taught me this art as equal to my parents and to live my life in partnership with him, and if he is in need of money to give him a share of mine, and to regard his offspring as equal to my brothers in male lineage and to teach them this art – if they desire to learn it – without fee and covenant; to give a share of precepts and oral instruction and all the other learning to my sons and to the sons of him who has instructed me and to pupils who have signed the covenant and have taken an oath according to the medical law, but no one else”.