Introduction: Persistent foetal tachyarrythmias complicated by hydrops fetalis carry a poor prognosis, with foetal death reported in excess of a quarter despite treatment. We present our experience with direct intraperitoneal amiodarone administration in eight hydropic foetuses with resistant supraventricular tachycardia. Methods: Amiodarone was injected slowly into foetal peritoneal cavity under ultrasound guidance. All mothers were loaded with oral amiodarone before the procedure and maintained on it. The procedure was repeated guided by foetal rhythm. Result: All eight cases had severe hydrops with a median foetal heart rate of 255 bpm (range 240–300 bpm), and the median gestational age was 27+1 weeks (range 21–33+3 weeks) at presentation. In six cases, the average time for supraventricular tachycardia to revert to sinus rhythm from the first procedure was 11.5 days. In one case, intravascular injection of amiodarone into the umbilical vein was performed before intraperitoneal injection, which resulted in conversion to sinus rhythm sustained until delivery. In the last case, supraventricular tachycardia and severe hydrops persisted and the baby was delivered 5 days later at 34 weeks’ gestation. Hydrops resolved in five foetuses with a mean resolution time of 28.4 days. The mean gestational age at delivery was 34+5 days and seven of eight cases survived beyond the neonatal period with good postnatal outcomes. Conclusion: Intraperitoneal administration of amiodarone is a relatively simple and effective strategy in refractory supraventricular tachycardia complicated by severe hydrops. The intraperitoneal route assures delivery of the drug to the severely hydropic foetus and enables a bolus dose to be delivered for sustained absorption.

Heart failure in children is a complex clinical syndrome with multiple aetiologies. The underlying disorders that lead to heart failure in children differ significantly from those in adults. Some clinical biomarkers for heart failure status and prognosis appear to be useful in both age groups. This review outlines the use and the present status of biomarkers for heart failure in paediatric cardiology. Furthermore, clinical scenarios in which development of new biomarkers might address management or prognosis are discussed. Finally, strategies for proteomic discovery of novel biomarkers and application to practice are described.

Introduction: Haemodynamically significant patent ductus arteriosus is a significant cause of morbidity and mortality in pre-term infants. This retrospective study was conducted to investigate the usefulness of lower-dose paracetamol for the treatment of patent ductus arteriosus in pre-term infants. Materials and Methods: A total of 13 pre-term infants who received intravenous paracetamol because of contrindications or side effects to oral ibuprofen were retrospectively enrolled. In the first patient, the dose regimen was 15 mg/kg/dose, every 6 hours. As the patient developed significant elevation in transaminase levels, the dose was decreased to 10 mg/kg/dose, every 8 hours in the following 12 patients. Echocardiographic examination was conducted daily. In case of closure, it was repeated after 2 days and when needed thereafter in terms of reopening. Results: A total of 13 patients received intravenous paracetamol. Median gestational age was 29 weeks ranging from 24 to 31 weeks and birth weight was 950 g ranging from 470 to 1390 g. The median postnatal age at the first intravenous paracetamol dose was 3 days ranging from 2 to 9 days. In 10 of the 13 patients (76.9%), patent ductus arteriosus was closed at the median 2nd day of intravenous paracetamol ranging from 1 to 4 days. When the patient who developed hepatotoxicity was eliminated, the closure rate was found to be 83.3% (10/12). Conclusion: Intravenous paracetamol may be a useful treatment option for the treatment of patent ductus arteriosus in pre-term infants with contrindication to ibuprofen. In our experience, lower-dose paracetamol is effective in closing the patent ductus arteriosus in 83.3% of the cases.

Background: Cyanotic CHD comprises up to 25% of cases of all causes of CHD. Rationale: There is lack of data about the present spectrum of congenital cyanotic heart disease in the paediatric age group. Objective: The present study was undertaken to determine the spectrum of patients with congenital cyanotic heart disease in the paediatric age group in tertiary paediatric cardiac care clinic. Design: Prospective observational study. Setting: Paediatric cardiac clinic of a tertiary cardiac care centre. Methods: All children aged 0–18 years with suspected cyanotic CHD were provisionally included in this study. They underwent a thorough echocardiographic evaluation, and those patients who had definitive diagnosis of congenital cyanotic heart disease were included for final analysis. Results: A total of 119 children met the inclusion criteria. Tetralogy of Fallot and its variant were the most common congenital cyanotic heart disease with proportion of about 44%. Other common malformations were double outlet right ventricle (14%), pulmonary atresia with ventricular septal defect (8%), total anomalous pulmonary venous connection (7%), d-transposition of the great arteries (9%), tricuspid valve anomalies − tricuspid atresia and Ebstein’s anomaly − hypoplastic left-heart syndrome, truncus arteriosus, and complex CHD such as single ventricle. Conclusion: Tetralogy of Fallot and its variants were the most common cyanotic heart disease diagnosed in our patients. As there were a significant proportion of cases with complex cyanotic CHD, paediatric cardiologists should be familiar with the diagnosis and management of all these complex congenital malformations of the heart.

Although usually shown in embryology textbooks, the presence of the fifth pair of pharyngeal arch arteries has long been controversial. To the best of our knowledge, six pairs of bilaterally symmetrical arteries developing within the pharyngeal arches are yet to be found in any mammalian or avian species. Collateral channels between the distal ends of the fourth and sixth arch arteries, in contrast, have been found in up to half of all developing mouse embryos. In only one human embryo, again to the best of our knowledge, has a channel been found that extends from the aortic sac to the dorsal aorta, and hence qualifies as an arch artery. Despite these confounding factors in terms of the developmental heritage of the fifth arch arteries, the purported channels are invoked with increasing frequency to describe various lesions discovered in the setting of the congenitally malformed heart. Persistence of the artery of the fifth arch was initially proposed to explain double-barrelled aorta. It was subsequently proposed to account for various systemic-to-pulmonary channels feeding the pulmonary circulation in the setting of pulmonary atresia. It has also been claimed to persist so as to explain abnormal branching of the brachiocephalic arteries from the aortic arch. In the light of the ongoing doubts concerning the existence of the arteries of the fifth arch themselves, we have reviewed the various descriptions of purported fifth arch arteries within the world literature. We have then sought to validate the descriptions on the basis of our own understanding of development, for this purpose providing images of the remoulding arch arteries in the mouse so as to substantiate our conclusions. While accepting that our own interpretations are speculative, we suggest that more convincing alternative explanations can be advanced to account for the majority of lesions currently interpreted on the basis of persistence of the arteries of the fifth arches. Although the interpretations do not necessarily change the therapeutic approaches to the channels, appropriate description is important in terms of their classification.

A biomarker is any measurable, surrogate characteristic, which reflects either the presence or the absence of a disease state. This can be a blood test, an imaging characteristic, an exercise parameter, and even a genetic profile. Serum biomarkers are particularly attractive in that their cost to the patient is relatively low in terms of money, time, risk, and ease of obtaining a sample. The potential benefits of a good biomarker are manifold. This manuscript will review serum biomarkers of proposed utility in paediatric heart failure, especially with respect to their ability to aid clinical decision making, diagnosis, and prognosis.

Objective: Protocols for the placement of temporary pacing wires vary among institutions. Our current protocol is to selectively place temporary pacing wires in those patients who develop haemodynamically significant intra-operative arrhythmia. We wished to identify how effective our current protocol is at identifying who will develop post-operative arrhythmia and need temporary pacing wires. Methods: The charts of 880 patients over 8 years who underwent cardiopulmonary bypass were reviewed to find patients who developed intra-operative arrhythmia, had temporary pacing wires placed, and whether or not they developed post-operative arrhythmia and required utilisation of the pacing wires. Results: A total of 87 (9.9%) out of 880 patients who required cardiopulmonary bypass over 8 years had intra-operative arrhythmia and had temporary pacing wires placed. Of these, 59 (67.8%) had post-operative arrhythmia and utilised the pacing wires, whereas 28 (32.2%) did not have post-operative arrhythmia or utilise the pacing wires. In all, seven patients who did not have intra-operative arrhythmia or temporary pacing wires placed developed post-operative arrhythmia. Conclusion: Intra-operative arrhythmia is predictive of post-operative arrhythmia (70.2%) and our protocol is a sensitive means of identifying those who will develop post-operative arrhythmia (89.3%).

Around 15% of children and adolescents experience at least one episode of syncope until adulthood. Excluding cardiac disease, the majority of syncopes are of reflex origin and benign in nature. In this situation, a tilt test is conducted to reproduce symptoms and to evaluate cardiovascular adaptations to orthostatism, but its mechanisms are not yet well defined. Here, we investigated haemodynamics and autonomic activity during tilt in young patients. Patients (n=113) with unexplained syncope were enrolled. Tilt followed a standard protocol without provocative agents. A positive response (fainters) was defined as a sudden development of syncope or presyncope associated with hypotension, bradycardia, or both. Haemodynamic parameters, autonomic activity, and baroreflex sensibility were evaluated. Data were analysed on baseline; immediately after tilting; on tilt adaptation; before fainting or before tilt-down for non-fainters; and on tilt-down. A total of 45 patients experienced syncope after a mean time of 18 minutes. During tilting up, fainters showed lower blood pressure and peripheral resistance values, which decreased progressively with time together with baroreflex sensibility. Sympathetic tone increased massively along time till syncope. No changes in cardiac output and heart rate were observed. Results show a strong effort of the autonomic nervous system to adapt to orthostatic stress through different magnitudes of sympathetic output, which was maximal before syncope without apparent modifications of parasympathetic tone. These changes suggest an imbalance between both branches of the autonomic nervous system, not enabling a time-progressive adaptation and leading the subject to faint.

Right ventricular function is a crucial determinant of long-term outcomes of children with heart disease. Quantification of right ventricular systolic and diastolic performance by echocardiography is of paramount importance, given the prevalence of children with heart disease, particularly those with involvement of the right heart, such as single or systemic right ventricles, tetralogy of Fallot, and pulmonary arterial hypertension. Identification of poor right ventricular performance can provide an opportunity to intervene. In this review, we will go through the different systolic and diastolic indices, as well as their application in practice. Quantification of right ventricular function is possible and should be routinely performed using a combination of different measures, taking into account each disease state. Quantification is extremely useful for individual patient follow-up. Laboratories should continue to strive to optimise reproducibility through quality improvement and quality assurance efforts in addition to investing in technology and training for new, promising techniques, such as three-dimensional echocardiography.

Introduction: The aim of this prospective study was to determine the frequency and composition of cerebral microemboli in a paediatric population during transcatheter atrial septal defect closure. Methods: Multi-frequency transcranial Doppler was used to detect microembolic signals in the middle cerebral artery of 24 patients. Embolic signals were automatically identified and differentiated according to their composition, gaseous or solid. The procedure was divided into five periods: right cardiac catheterisation; left cardiac catheterisation; pulmonary angiography; balloon sizing; and device placement. Results: Microemboli were detected in all patients. The median number of signals was 63 and over 95% gaseous. The total number of microembolic signals detected during two periods – balloon sizing and sheath placement and device placement – was not significantly different (median: 18 and 25, respectively) but was significantly higher than each of the other three periods (p<0.001). In eight patients, the device was opened more than once and the number of embolic signals decreased with each successive device deployment. There was no correlation between the number of microembolic signals and fluoroscopic time, duration of procedure, age, or device size. Conclusion: This is the first study to investigate the timing and composition of cerebral microemboli in a paediatric population during cardiac catheterisation. Microembolic signals were related to specific catheter manipulations but were not associated with fluoroscopic time or duration of procedure.

Objective: To evaluate the psychosocial status of mothers and fathers in response to their infant’s diagnosis of hypoplastic left heart syndrome. Design: A study on interviews with parents whose children had survived staged surgery. Setting: Tertiary hospital paediatric ICU. Subjects: A total of 29 parents (16 mothers and 13 fathers) of surviving children. Intervention: A semi-structured face-to-face interview was conducted to explore experiences of parents in response to their infant’s diagnosis, their interaction with the doctor delivering the diagnosis, their deliberation about staged surgery, and their reasons for this choice. Measurement and Main Results: All parents were devastated about their infant’s diagnosis, and most (83%) of them said that the time of the diagnosis and the aftermath was the worst time of their lives. Parents reported helpful and unhelpful communication at this time. Although all parents in this study chose surgery for their infant, when faced with the choice, 17 of them made an immediate decision “to protect their infant’s life”, 8 were initially unsure when their infant was diagnosed in utero, and 4 were unsure when the infant was diagnosed after birth. Parents also experienced loss and other stressors. Conclusions: All parents of the infants diagnosed with hypoplastic left heart syndrome experienced intense loss and stressors. Physicians need to be sensitive to the needs and thinking of the parents when discussing treatment options before surgery. The nature of the relationship with the doctor at this time can support parents or be a further source of stress.

Objective: The use of covered stents to close fenestration in total cavopulmonary connection is presented. Methods: We retrospectively reviewed data of all patients undergoing the procedure of a covered stent to close fenestration of total cavopulmonary connection between 2005 and 2012. Results: A total of 50 patients met the inclusion criteria. Median age and weight were 7.7 years and 20 kg, respectively. Median interval between Fontan completion and fenestration closure was 13 months. The femoral vein was used in 42 patients and the jugular vein in eight patients. Of the patients, seven received two stents. Covered stents were CP stents in 42 patients and Atrium Advanta V12 in eight patients. BIB balloons were used in 24 patients and simple balloons in 18 patients. Simultaneous occlusion of venous collaterals was observed in five patients. Median procedural and fluoroscopy times were 49 and 8 minutes, respectively. Mean central venous pressure rose from 10 to 12 mmHg. Mean oxygen saturation increased from 88% to 96%. Full occlusion was confirmed in 47 patients. The remaining had residual shunts: two patients had intracardiac Fontan, and one patient had a stent that could not be fully opened. Following the procedure, five patients had local bleeding, and three delayed discharge 48 hours after the procedure. There was no thromboembolic event after a mean follow-up of 49 months. Conclusion: Covered stent is a good option to close fenestration in extracardiac total cavopulmonary connection. It is safe, easily achievable with low fluoroscopy time, with very low risk of complication or failure. Good results are sustainable when excluding patients with none circular pathway.

Stem cell therapy has the optimistic goal of regenerating the myocardium as defined by re-growth of lost or destroyed myocardium. As applied to patients with heart failure, many confuse or limit the regenerative definition to just improving myocardial function and/or decreasing myocardial scar formation, which may not be the most important clinical outcome to achieve in this promising field of molecular medicine. Many different stem cell-based therapies have been tested and have demonstrated a safe and feasible profile in adult patients with heart failure, but with varied efficacious end points reported. Although not achieved as of yet, the encompassing goal to regenerate the heart is still believed to be within reach using these cell-based therapies in adult patients with heart failure, as the first-generation therapies are now being tested in different phases of clinical trials. Similar efforts to foster the translation of stem cell therapy to children with heart failure have, however, been limited. In this review, we aim to summarise the findings from pre-clinical models and clinical experiences to date that have focussed on the evaluation of stem cell therapy in children with heart failure. Finally, we present methodological considerations pertinent to the design of a stem cell-based trial for children with heart failure, as they represent a population of patients with very different sets of issues when compared with adult patients. As has been taught by many learned clinicians, children are not small adults!

Background: Altered mesenteric perfusion may be a contributor to the development of necrotising enterocolitis in patients with hypoplastic left heart syndrome. The goal of this study was to document mesenteric flow patterns in patients with hypoplastic left heart syndrome pre- and post-hybrid procedure. Methods: A prospective study on all patients with hypoplatic left heart syndrome undergoing the hybrid procedure was conducted. Doppler ultrasound analysis of the coeliac and superior mesenteric artery was performed. Results: A total of 13 patients were evaluated. There was a significant difference in the coeliac artery effective velocity-time intergral pre- and post-hybrid procedure (8.69±3.84 versus 12.51±4.95 cm, respectively). There were significant differences in the superior mesenteric artery antegrade velocity-time integral pre- and post-hybrid procedure (6.86±2.45 versus 10.52±2.64 cm, respectively) and superior mesenteric artery effective velocity-time integral pre- and post-hybrid procedure (6.22±2.68 versus 9.73±2.73 cm, respectively). There were no significant differences between the coeliac and superior mesenteric artery Doppler indices in the pre-hybrid procedure; there were, however, significant differences in the post-hybrid procedure between coeliac and superior mesenteric artery antegrade velocity-time integral (13.8 2±5.60 versus 10.52±2.64 cm, respectively) and effective velocity-time integral (13.04±4.71 versus 9.73±2.73 cm, respectively). Conclusion: Doppler mesenteric indices of perfusion improve in patients with hypoplastic left heart syndrome after the hybrid procedure; however, there appears to be preferential flow to the coeliac artery versus the superior mesenteric artery in these patients post-procedure.

We present a case of percutaneous removal of a large right atrial thrombus formed after insertion of a ventriculoatrial shunt for hydrocephalus.