It is frequent, in the current era, to encounter congenital cardiac malformations described in terms of “cor triatriatum”. But can hearts be truly found with three atrial chambers? The morphological method, emphasised by Van Praagh et al, states that structures within the heart should be defined on the basis of their most constant components. In the atrial chambers, it is the appendages that are the most constant components, and to the best of our knowledge, hearts can only possess two appendages, which can be of either right or left morphology. The hearts described on the basis of “cor triatriatum”, nonetheless, can also be analysed on the basis of division of either the morphologically right or the morphologically left atriums. In this review, we provide a description of cardiac embryology, showing how each of the atrial chambers possesses part of the embryological body, along with an appendage, a vestibule, a venous component, and a septum that separates them. We then show how it is, indeed, the case that the hearts described in terms of “cor triatriatum” can be readily understood on the basis of division of these atrial components. In the right atrium, it is the venous valves that divide the chamber. In the left atrium, it is harder to provide an explanation for the shelf that produces atrial division. We also contrast the classic examples of the divided atrial chambers with the vestibular shelf that produces supravalvar stenosis in the morphologically left atrium, showing that this form of obstruction needs to be distinguished from the fibrous shelves producing intravalvar obstruction.

Agenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.

Introduction: The term cor triatriatum is used to describe a fibromuscular partition that divides an atrium into two compartments. It was first used by Borst in 1905, although the lesion had been described before by Church in 1868. Both described divided left atrial chamber (also referred to as cor triatriatum sinister). The incidence of the divided left atrial chamber among patients with congenital heart disease is reported at 0.1% and 0.4% in an autopsy study. Divided right atrial chamber – also referred to as cor triatriatum dexter – is even rarer to an extent that there is no reported incidence and only few hundred cases have been reported so far. A search through pubMed library revealed only 90 publications on humans, either under the term “dexter” or “dextrum”. We would like to report a new association of bilateral divided atrial chambers and pulmonary vein stenosis, with a review of the literature.

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.

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

Objective: There are limited data on the outcomes of children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. The primary aim of this project is to identify the aetiology and outcomes of extracorporeal membrane oxygenation in children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. Patients and methods: We conducted a retrospective review of all children ≤18 years supported with delayed extracorporeal membrane oxygenation after cardiac surgery between the period January, 2001 and March, 2012 at the Arkansas Children’s Hospital, United States of America, and Royal Children’s Hospital, Australia. The data collected in our study included patient demographic information, diagnoses, extracorporeal membrane oxygenation indication, extracorporeal membrane oxygenation support details, medical and surgical history, laboratory, microbiological, and radiographic data, information on organ dysfunction, complications, and patient outcomes. The outcome variables evaluated in this report included: survival to hospital discharge and current survival with emphasis on neurological, renal, pulmonary, and other end-organ function. Results: During the study period, 423 patients undergoing cardiac surgery were supported with extracorporeal membrane oxygenation at two institutions, with a survival of 232 patients (55%). Of these, 371 patients received extracorporeal membrane oxygenation <7 days after cardiac surgery, with a survival of 205 (55%) patients, and 52 patients received extracorporeal membrane oxygenation ≥7 days after cardiac surgery, with a survival of 27 (52%) patients. The median duration of extracorporeal membrane oxygenation run for the study cohort was 5 days (interquartile range: 3, 10). In all, 14 patients (25%) received extracorporeal membrane oxygenation during active cardiopulmonary resuscitation with chest compressions. There were 24 patients (44%) who received dialysis while being on extracorporeal membrane oxygenation. There were eight patients (15%) who had positive blood cultures and four patients (7%) who had positive urine cultures while being on extracorporeal membrane oxygenation. There were nine patients (16%) who had bleeding complications associated with extracorporeal membrane oxygenation runs. There were 10 patients (18%) who had cerebrovascular thromboembolic events associated with extracorporeal membrane oxygenation runs. Of these, 19 patients are still alive with significant comorbidities. Conclusions: This study demonstrates that mortality outcomes are comparable among children receiving extracorporeal membrane oxygenation ≥7 days and <7 days after cardiac surgery. The proportion of patients receiving extracorporeal membrane oxygenation ≥7 days is small and the aetiology diverse.

Background: Children with decompensated heart failure are at high risk for arrhythmias, and ventricular assist device placement is becoming a more common treatment strategy. The impact of ventricular assist devices on arrhythmias and how arrhythmias affect the clinical course of this population are not well described. Methods and results: A single-centre retrospective analysis of children receiving a ventricular assist device between 1998 and 2011 was performed. In all, 45 patients received 56 ventricular assist devices. The median age at initial placement was 13 years (interquartile range 6–15). The median duration of support was 10 days (range 2–260). The aetiology of heart failure included cardiomyopathy, transplant rejection, myocarditis, and congenital heart disease. In all, 32 patients (71%) had an arrhythmia; 19 patients (42%) had an arrhythmia before ventricular assist device and eight patients (18%) developed new arrhythmias on ventricular assist device. Ventricular tachycardia was most common (25/32, 78%). There was no correlation between arrhythmia and risk of death or transplantation (p=0.14). Of the 15 patients who weaned from ventricular assist device, post-ventricular assist device arrhythmias occurred in nine (60%), with five (33%) having their first arrhythmia after weaning. Patients with ventricular dysfunction after ventricular assist device were more likely to have arrhythmias (p<0.02). Conclusions: Arrhythmias, especially ventricular, are common in children requiring ventricular assist device. They frequently persist for those able to wean from ventricular assist device.

Introduction: Dilated cardiomyopathy is usually idiopathic and may arise secondary to infections or metabolic or genetic causes. Another rare cause is hypocalcaemia. Owing to the fact that calcium plays an essential role in excitation and contraction of myocardial muscle, myocardial contractility may decline in patients with hypocalcaemia. Materials and Methods: Patients with symptoms of congestive heart failure and rickets-related hypocalcaemia were assessed clinically and by echocardiography in a paediatric cardiology clinic. Echocardiography was performed for all patients. Rickets was diagnosed according to the clinical, laboratory, and radiologic findings. Maternal lifestyle and living conditions were investigated, and the maternal 25-OH vitamin D3 blood level was measured. Results: We evaluated eight patients who developed heart failure as a result of severe hypocalcaemia associated with rickets between August, 1999 and June, 2012. The age distribution of the patients was 3–12 months. Laboratory results were consistent with advanced-stage rickets. Severe hypocalcaemia was detected in all patients. The maternal 25-OH vitamin D3 levels were low. Echocardiography revealed increased pre-treatment left ventricle end-systolic and end-diastolic diameters for age and reduced ejection fraction and fractional shortening. After clinical improvement, the patients were discharged. Conclusions: Severe hypocalcaemia associated with rickets must always be kept in mind among the causes of dilated cardiomyopathy and impaired cardiac function in infants. If diagnosed and treated in time, dilated cardiomyopathy and severe heart failure related to rickets respond well.

Introduction: We aimed to evaluate changes in the cerebral blood supply in children during vasovagal syncope and to clarify the diagnostic value of transcranial Doppler for vasovagal syncope. Materials and methods: Patients were divided into three groups. Group 1 consisted of 31 patients who were symptomatic and whose head-up tilt test was positive. Group 2 comprised 21 patients who were symptomatic but whose tilt test was negative. Group 3 included 22 healthy children. For the diagnosis of vasovagal syncope, the tilt test was applied. For the subjects of the patient and control groups, the tilt test was repeated. The flow rates of bilateral middle cerebral arteries were continuously and simultaneously recorded with temporal window transcranial Doppler. Results: There were no statistically significant differences between the three groups with respect to age and gender distribution (p>0.05). When the bed was at an upright position, the maximum blood flow rate of the right middle cerebral artery was lower in Group 1 than in Group 2, although the decrease was more significant in comparison to the healthy control group (p<0.05). The minimum blood flow rate of the right middle cerebral artery was lower in Group 1 than the Group 2, although the decrease was more significant in comparison with the healthy control group (p<0.05). The maximum blood flow rate of the left middle cerebral artery was significantly lower in Group 1 than in the control group (p<0.05). Conclusion: Minimum and maximum blood flow rates are significantly decreased in patients tilt test (+) patients with vasovagal syncope during orthostatic stress.

Objective: By employing the widely used and accepted methodologies of case-mix complexity adjustment in congenital cardiac surgery, we tried to evaluate our performance and use the ABC scores for a case complexity selection that may have different outcomes in various centres. Methods: We analysed outcomes of cardiac surgical procedures – with or without cardiopulmonary bypass – performed in our institution between January, 2008 and December, 2011. Data were collected from the European Association for Cardio-Thoracic Surgery database. Together with prospective collection of these data, the data of all patients sent abroad to foreign cardiosurgical centres were recorded. Results: During the period of study, 634 operations were performed; among them, 60% were performed in Croatia and 40% in foreign cardiosurgical centres. The number of operations performed in Croatia showed a linear increase: 55, 78, 121, and 126 operations performed in the years 2008, 2009, 2010, and 2011, respectively. Early mortality rates were 1.82%, 5.41%, 3.64%, and 3.48% in 2008, 2009, 2010, and 2011, respectively. The increase in the number of operations was followed by a satisfactory low average mortality rate of 3.85%. The mean ABC score complexity for operations performed in Croatia was 5.77. We determined a linear correlation between ABC score and early mortality, especially for the more complex operations. Conclusion: The use of standardised risk scores allows selection of complex cardiac diseases, which may have very different outcomes in various centres. In our case, those with higher ABC scores were correctly identified and referred for treatment abroad. In this way, we allowed gradual progress of the cardiosurgical model in Croatia and maintained an enviably low mortality rate.

Aims: Advances in medical treatment have resulted in increased life expectancy in congenital heart disease. Consequently, the focus of management has shifted from reducing mortality to reducing long-term morbidity with the goal of improving quality of life. A predictor of quality of life might be N-terminal pro-brain natriuretic peptide, a well-established marker for heart failure. We aimed to determine the association between N-terminal pro-brain natriuretic peptide and quality of life in patients with congenital heart disease. Methods: We collected blood samples from consecutive patients who were initially operated between 1968 and 1980 (47.8% women; mean age 40.2±5.4 years). The 36-item Short-Form Health Survey was completed to assess subjective health status as a measure of quality of life. Analysis was performed for the entire group and for subgroups defined as simple versus complex congenital heart diseases. Median N-terminal pro-brain natriuretic peptide level was 15.2 pmol/L (overall range 1.3–299.3 pmol/L). N-terminal pro-brain natriuretic peptide levels were associated with the subdomain physical functioning (β=−0.074, p=0.031). This association remained significant after adjustment for age and sex (β=−0.071, p=0.038) and after adjustment for age, sex, body mass index, left ventricular function, and renal function (β=−0.069, p=0.048). In complex congenital heart disease, the association between N-terminal pro-brain natriuretic peptide and physical functioning remained significant in multivariable analysis (β=−0.076, p=0.046). No associations were found in the simple congenital heart disease group or on the other health status subdomains. Conclusion: In adults operated for congenital heart disease, N-terminal pro-brain natriuretic peptide is associated with the subdomain physical, primarily in the complex subgroup.

Introduction: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression. Methods: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution. The primary endpoints were post-operative bronchoscopic and clinical evidence of persistent airway compression and need for reinterventions on the pulmonary arteries. Results: A total of 19 patients were included during the study period. The mean age at repair was 4.1±3.0 years (range 10 months–11 years). In all, seven patients had pre-operative bronchoscopic evidence of airway compression, which was managed by pulmonary artery reduction plasty in four patients and Lecompte manoeuvre in three patients. There were no peri-operative deaths. In patients with pulmonary artery plasty, two had no post-operative airway compression, one patient had improved compression, and one patient had unchanged compression. In patients managed with a Lecompte manoeuvre, two patients had no or trivial airway compression and one had improved compression. There were six late reinterventions or reoperations on the pulmonary arteries – two out of four in the pulmonary artery plasty group and one out of three in the Lecompte group. Conclusions: Most late-presenting patients with absent pulmonary valve syndrome do not have airway compression. Either pulmonary artery reduction plasty or the Lecompte manoeuvre can relieve proximal airway compression, without a significantly different risk of pulmonary artery reintervention between techniques.

Background: Previous studies have demonstrated structural changes in the heart and cardiac dysfunction in foetuses with intrauterine growth restriction. There are no available data that evaluated left ventricular dimensions and mass in neonates with symmetric and asymmetric intrauterine growth restriction. Therefore, we aimed to evaluate left ventricular dimensions, systolic functions, and mass in neonates with symmetric and asymmetric intrauterine growth restriction. We also assessed associated maternal risk factors, and compared results with healthy appropriate for gestational age neonates. Methods: In all, 62 asymmetric intrauterine growth restriction neonates, 39 symmetric intrauterine growth restriction neonates, and 50 healthy appropriate for gestational age neonates were evaluated by transthoracic echocardiography. Results: The asymmetric intrauterine growth restriction group had significantly lower left ventricular end-systolic and end-diastolic diameters and posterior wall diameter in systole and diastole than the control group. The symmetric intrauterine growth restriction group had significantly lower left ventricular end-diastolic diameter than the control group. All left ventricular dimensions were lower in the asymmetric intrauterine growth restriction neonates compared with symmetric intrauterine growth restriction neonates (p>0.05), but not statistically significant except left ventricular posterior wall diameter in diastole (3.08±0.83 mm versus 3.54 ±0.72 mm) (p<0.05). Both symmetric and asymmetric intrauterine growth restriction groups had significantly lower relative posterior wall thickness (0.54±0.19 versus 0.48±0.13 versus 0.8±0.12), left ventricular mass (9.8±4.3 g versus 8.9±3.4 g versus 22.2±5.7 g), and left ventricular mass index (63.6±29.1 g/m2 versus 54.5±24.4 g/m2 versus 109±28.8 g/m2) when compared with the control group. Conclusions: Our study has demonstrated that although neonates with both symmetric and asymmetric intrauterine growth restriction had lower left ventricular dimensions, relative posterior wall thickness, left ventricular mass, and mass index when compared with appropriate for gestational age neonates, left ventricular systolic functions were found to be preserved. In our study, low socio-economic level, short maternal stature, and low maternal weight were found to be risk factors to develop intrauterine growth restriction. To our knowledge, our study is the first to evaluate left ventricular dimensions, wall thicknesses, mass, and systolic functions in neonates with intrauterine growth restriction and compare results with respect to asymmetric or symmetric subgroups.

Cor triatriatum dexter is an extremely rare congenital anomaly of the right atrium. It occurs because of the persistence of the right sinus venosus valve, resulting in partitioning of the right atrium. Most of the described cases of cor triatriatum dexter in the literature were incidental findings on echocardiogram or at necropsy.

We present a case report of a 7-year-old girl who was referred to us for further assessment, with a possible diagnosis of coarctation of the aorta. Initial investigations confirmed not only the presence of a long segment coarctation of the aorta, but also a large obstructive membrane in the right atrium.

A catheter intervention was performed to stent the coarctation segment, and the fibro-muscular shelf in the right atrium was surgically resected.

Objective: The aim of this study was to determine developmental enamel defects and dental treatment conditions in children with congenital heart disease by comparing them with a control group of healthy children. Methods: Children included in the study were referred to a paediatric dentistry for dental examination and treatment after undergoing routine examination in a paediatric clinic. Results: The congenital heart disease group included 72 children and the control group included 56 healthy children. Children in the age group of 3–14 years were included in this study. The mean age of the congenital heart disease group and control group was 6.24±2.85 and 6.73±3.01, respectively. The mean values of the decayed, missing, and filled indices for primary and permanent teeth in the congenital heart disease group were 2.80±3.77 and 0.81±1.63, respectively. In the control group, the values were 1.87±3.31 and 0.72±1.46, respectively. The care score for primary teeth was 3.6% in the congenital heart disease group and 13.3% in the control group. The enamel defect was detected in at least one permanent tooth in seven out of 72 children (9.7%) in the congenital heart disease group and in three out of 56 children (5.3%) in the control group. Conclusion: Although there was no significant difference in the development of dental caries or the prevalence of enamel defects between children with congenital heart disease and healthy children, the care score was low in children with congenital heart disease. In addition, children with congenital heart disease had a higher rate of pulled primary teeth and delayed treatment of decayed teeth.

Purpose: Previous studies have shown that the underlying pathophysiologic mechanism in children with breath holding may be generalised autonomic dysregulation. Thus, we performed cardiac rhythm and heart rate variability analyses using 24-hour Holter monitoring to evaluate the cardiac effects of autonomic dysregulation in children with breath-holding spells. Methods: We performed cardiac rhythm and heart rate analyses using 24-hour Holter monitors to evaluate the cardiac effects of autonomic dysregulation in children during a breath-holding spell. Our study group consisted of 68 children with breath-holding spells – 56 cyanotic type and 12 pallid type – and 39 healthy controls. Results: Clinical and heart rate variability results were compared between each spell type – cyanotic or pallid – and the control group; significant differences (p<0.05) in standard deviation of all NN intervals, mean of the standard deviations of all NN intervals for all 5-minute segments, percentage of differences between adjacent RR intervals >50 ms, and square root of the mean of the sum of squares of the differences between adjacent NN intervals values were found between the pallid and cyanotic groups. Conclusions: Holter monitoring for 24 hours and heart rate variability parameters, particularly in children with pallid spells, are crucial for evaluation of cardiac rhythm changes.

We describe the technique of closure of native right ventricular outflow tract by Amplatzer muscular ventricular septal defect device because of severe regurgitation in a patient who had tetralogy of Fallot repair with conduit at 3 years of age followed by percutaneous Melody valve implant 6 years later.