Since Takotsubo cardiomyopathy was first described by Sato in 1990, multiple cases have been reported, but only few in children, among whom this type of cardiomyopathy is to some extent underappreciated. A series of children with this syndrome were therefore reviewed, drawing comparison with cases reported by others. The review addresses the current challenges in diagnosis, presentation, triggers, clinical course, management, and possible pathogenic mechanisms of the entity.

Objective: To make a historical comparison on the long-term psychosocial outcome of cardiothoracic surgery during childhood. Methods: Adult patients operated for tetralogy of Fallot or transposition of the great arteries between 1980 and 1990 (recent sample) were compared with patients who underwent surgery and were investigated 10 years earlier (historical sample). In addition, atrial switch and arterial switch patients within the recent sample were compared. Psychosocial functioning was measured using standardised, validated psychological questionnaires. Results: Although the recent sample of patients overall shows a favourable quality of life, impairments were found in income, living conditions, relationships, offspring, and occupational level. Compared with the historical sample, the recent sample showed no significant improvements on psychosocial functioning, except for a better educational level. The amount of educational problems, such as learning difficulties, was still high compared with normative data. Recently operated patients with transposition of the great arteries (arterial switch) scored significantly better on the Short Form-36 vitality scale (p = 0.02) compared with historical patients with transposition of the great arteries (atrial switch). Conclusions: Despite improvements in medical treatment over the past few decades, hardly any change was found in the psychosocial outcome of the recent patient sample compared with the historical patient sample. In particular, the percentage of patients needing special education and showing learning problems remained high, whereas income was low compared with normative data.

Background: This study aimed to examine the differences between arterial and inferior caval vein oxygen saturation, fractional oxygen extraction, and the shunt index, which were calculated in the diagnosis of patent ductus arteriosus. Methods: Twenty-seven preterm infants were included in this study and were divided into two groups according to patent ductus arteriosus. Among them, 11 (41%) infants had haemodynamically significant patent ductus arteriosus and 16 (59%) did not have significant patent ductus arteriosus. Synchronous arterial and venous blood gases were measured during the first post-natal hours after the insertion of umbilical catheters. The differences between arterial and inferior caval vein oxygen saturation, inferior body fractional oxygen extraction, and the shunt index were calculated. Echocardiography was performed before the 72nd hour of life in a selected group of patients who had haemodynamically significant patent ductus arteriosus. Ibuprofen treatment was administered to patients with patent ductus arteriosus. Echocardiography was performed on the 72nd hour of life in preterm infants without any clinical suspicion of patent ductus arteriosus. Results: The early measured differences between arterial and inferior caval vein oxygen saturation and inferior body fractional oxygen extraction were found to be lower and the shunt index was found to be higher in the haemodynamically significant patent ductus arteriosus group than in the group without haemodynamically significant patent ductus arteriosus. Conclusion: We found that the shunt index, calculated in the first hours of life as ≥63%, predicted haemodynamically significant patent ductus arteriosus with a sensitivity of 78% and specificity of 82% in preterm newborns.

Objective: Temporary pacing wires play a crucial role in the diagnosis and therapy of post-operative arrhythmia after surgery for congenital heart disease. At present, bipolar pacing wires are used in most institutions. In case of functional failure of these wires, a unipolar mode of stimulation and sensing should be theoretically possible as a rescue procedure. Methods: We tested the feasibility of the customised unipolar mode in 18 post-operative patients with congenital heart disease (age 9.2 ± 13.9 months, weight 6.3 ± 3.8 kg, and cardiopulmonary bypass time 70 ± 29 minutes). As there are two possible unipolar configurations, there are twice the number of testing parameters; of those, we compared sensing (mV) and pacing thresholds (V at 0.5 ms). Results: Atrial sensing was significantly better in the unipolar modes (p < 0.001, p < 0.003). The ventricular unipolar sensing did not differ significantly in the “better” of the two possible configurations from the bipolar values (p = 0.363). For the unipolar pacing thresholds, only the “better” unipolar configuration did not differ significantly from the bipolar measurements (atrial: p = 0.058, ventricular: p = 0.138). There was no exit block or undersensing. Conclusion: The results demonstrate that unipolar stimulation and sensing using bipolar epicardial temporary pacing wires is feasible. In the case of failure of bipolar temporary pacing wires, this modality represents an easy rescue measure that in such cases should always be considered.

Between January, 2002 and December, 2011, 27 patients (19 boys) underwent pericardiectomy. The mean age was 9.3 ± 4.96 years (range 0.4 to 15 years) and the mean duration of symptoms was 16.9 ± 22.15 months. In all, 25 patients had dyspnoea; eight were in New York Heart Association (NYHA) class IV; six had bacterial pericarditis; and 18 were on anti-tuberculosis treatment, although only nine had records suggesting tuberculosis. There were nine patients who underwent pre-operative pigtail catheter drainage of pericardial fluid. Surgical procedures were complete pericardiectomy (n = 20), partial pericardiectomy (n = 6), and pleuropericardial window (n = 1).The mean pre-operative right atrial pressure was 20.4 ± 4.93 mmHg. There were six hospital deaths due to low cardiac output (n = 5) and arrhythmia (n = 1). The mean intensive care unit stay was 2.7 ± 1.2 days and mean post-operative stay was 9.9 days. The mean right atrial pressure dropped to 8.7 ± 1.15 mmHg. Adverse outcomes defined as death/prolonged intensive care unit stay, prolonged post-operative stay were not associated with sex, diagnosis of tuberculosis or pyopericardium, or the duration of symptoms or pre-operative right atrial pressure. Younger patients had prolonged intensive care unit stay (p = 0.03) but not increased mortality. Advanced NYHA class predicted death (p = 0.02). The mean follow-up was 23.1 ± 23.8 months. All except one survivor are in NYHA class I and off all cardiac medications. Despite adequate surgery, pericardiectomy in children is associated with a high mortality, which is related to delayed surgery and poor pre-operative general condition. No specific pre-operative variable other than worse pre-operative NYHA class is a predictor of survival. Therefore, early pericardiectomy should be undertaken in such patients.

Objective: To determine whether blood levels of the brain-specific biomarker glial fibrillary acidic protein rise during cardiopulmonary bypass for repair of congenital heart disease. Methods: This is a prospective observational pilot study to characterise the blood levels of glial fibrillary acidic protein during bypass. Children <21 years of age undergoing bypass for congenital heart disease at Johns Hopkins Hospital and Texas Children's Hospital were enrolled. Blood samples were collected during four phases: pre-bypass, cooling, re-warming, and post-bypass. Results: A total of 85 patients were enrolled between October, 2010 and May, 2011. The median age was 0.73 years (range 0.01–17). The median weight was 7.14 kilograms (range 2.2–86.5). Single ventricle anatomy was present in 18 patients (22%). Median glial fibrillary acidic protein values by phase were: pre-bypass: 0 ng/ml (range 0–0.35); cooling: 0.039 (0–0.68); re-warming: 0.165 (0–2.29); and post-bypass: 0.112 (0–0.97). There were significant elevations from pre-bypass to all subsequent stages, with the greatest increase during re-warming (p = 0.0001). Maximal levels were significantly related to younger age (p = 0.03), bypass time (p = 0.03), cross-clamp time (p = 0.047), and temperature nadir (0.04). Peak levels did not vary significantly in those with single ventricle anatomy versus two ventricle repairs. Conclusion: There are significant increases in glial fibrillary acidic protein levels in children undergoing cardiopulmonary bypass for repair of congenital heart disease. The highest values were seen during the re-warming phase. Elevations are significantly associated with younger age, bypass and cross-clamp times, and temperature nadir. Owing to the fact that glial fibrillary acidic protein is the most brain-specific biomarker identified to date, it may act as a rapid diagnostic marker of brain injury during cardiac surgery.

Objective: The aim of this study was to explore the acute psychosocial risk in families with infants undergoing surgery for a congenital heart disease and, secondarily, to explore the psychosocial impact of antenatal versus post-natal diagnoses. Method: The study sample comprised 39 caregivers (28 mothers) of 29 children diagnosed with a congenital heart disease and requiring surgery within the first 4 weeks of life. Psychosocial risk was measured using the Psychosocial Assessment Tool, which was adapted to include four novel items examining infant risk factors, namely, sleeping, feeding, crying, and bonding difficulties. Parents’ psychosocial risk was measured within 4 weeks after their child's surgery and stratified into a three-tiered framework: Universal, Targeted, and Clinical risk. Results: Of the total sample, 61.5% of parents were classified as Universal, that is, at lowest risk; 35.9% as Targeted, and 2.6% as Clinical. The within-family parent total Psychosocial Assessment Tool score correlations were non-significant, and there were no differences between families of infants who received post-natal versus antenatal diagnosis or single ventricle versus biventricular repair. Linear regression found that a higher parent education significantly predicted a lower total Psychosocial Assessment Tool score. Conclusions: Findings indicate that, although the majority of parents adapt to the acute stress of surgery for a serious cardiac illness in their infant, the remaining 38.5% report an increased psychosocial risk associated with higher rates of emotional distress, which may impact on the parental quality of life and capacity for optimal parenting. The distribution of psychosocial risk in parents of children undergoing surgery for a congenital heart disease is consistent with that described for parents of children with other serious paediatric diagnoses.

Background: The gold standard for determining the left ventricular ejection fraction is cardiac magnetic resonance imaging. Other parameters for determining the ejection fraction such as M-mode echocardiography are operator-dependant and often inaccurate. Assessment of the displacement of the mitral valve annulus using two-dimensional speckle tracking echocardiography may provide an accurate and simple method of determining the left ventricular ejection fraction in children. Method: We retrospectively studied 70 healthy 9-year-old children with no history of cardiovascular disease who had been assessed using cardiac magnetic resonance imaging and two-dimensional transthoracic echocardiography. Mitral displacement was determined using the tissue motion annular displacement (TMAD) feature of Philips QLAB version 9. The midpoint displacement of the mitral valve was calculated, and the predicted left ventricular ejection fraction was compared with magnetic resonance imaging-derived and M-mode-derived ejection fractions. Results: The mean ejection fraction derived from magnetic resonance imaging (64.5 (4.6)) was similar to that derived from the TMAD midpoint (60.9 (2.7), p = 0.001) and the M-mode (61.9 (7), p = 0.012). The TMAD midpoint correlated strongly with the magnetic resonance imaging-derived ejection fraction (r = 0.69, p < 0.001), as did the predicted fraction (r = 0.67, p < 0.001). The M-mode ejection fraction showed a poor linear correlation with both magnetic resonance imaging-derived and TMAD-derived fractions (r = 0.33 and 0.04, respectively). Conclusion: TMAD of the mitral valve is a simple, effective, and highly reproducible method of assessing the ejection fraction in normal children. It shows a strong linear correlation with magnetic resonance imaging-derived ejection fraction and is superior to M-mode-derived ejection fractions.

Objective: At present, the haemodynamic diagnosis of orthostatic intolerance is based mainly on the head-up tilt table test, which is sometimes risky for patients. Thus, it is important to find objective and safe methods to differentiate haemodynamic patterns of orthostatic intolerance cases. Methods: In all, 629 children with orthostatic intolerance, either vasovagal syncope or postural orthostatic tachycardia syndrome, were included in the multi-centre clinical study. We analysed the association between the clinical manifestation and haemodynamic patterns of the patients. Results: Syncope after motion with a prodrome of chest distress or palpitations and the concomitant symptom(s) after a syncopal attack, with debilitation, dizziness or headache, were the most important variables in predicting the diagnosis of vasovagal syncope. The overall diagnostic accuracy was 71.5%. Conclusion: Complaint of syncope after motion with prodromal chest distress or palpitation and the concomitant symptom after a syncopal attack, with subsequent debilitation, dizziness or headache, were the most important variables in the diagnosis of vasovagal syncope in children with orthostatic intolerance.

Background: The aim of this study was to evaluate our experience in central extracorporeal life support with an integrated left ventricular vent in children with cardiac failure. Methods: Eight children acquired extracorporeal life support with a left ventricular vent, either after cardiac surgery (n = 4) or during an acute cardiac illness (n = 4). The ascending aorta and right atrium were cannulated. The left ventricular vent was inserted through the right superior pulmonary vein and connected to the venous line on the extracorporeal life support such that active left heart decompression was achieved. Results: No patient died while on support, seven patients were successfully weaned from it and one patient was transitioned to a biventricular assist device. The median length of support was 6 days (range 5–10 days). One patient died while in the hospital, despite successful weaning from extracorporeal life support. No intra-cardiac thrombus or embolic stroke was observed. No patient developed relevant intracranial bleeding resulting in neurological dysfunction during and after extracorporeal life support. Conclusions: In case of a low cardiac output and an insufficient inter-atrial shunt, additional left ventricular decompression via a vent could help avoid left heart distension and might promote myocardial recovery. In pulmonary dysfunction, separate blood gas analyses from the venous cannula and the left ventricular vent help detect possible coronary hypoxia when the left ventricle begins to recover. We recommend the use of central extracorporeal life support with an integrated left ventricular vent in children with intractable cardiac failure.

Objectives: Our aim was to evaluate patients who were treated by percutaneous stent implantation. Methods: Patients with aortic coarctation (n = 35) who had been treated with 38 stents – 12 bare and 26 covered – were evaluated. The demographics and procedural and follow-up data were recorded from hospital registers and compared according to patient specifications, for example, weight and coarctation nature. Results: The procedure was successful in all patients. There was a statistically significant difference between the patients with native coarctation (n = 17) and those with recurrent coarctation (n = 18) in terms of pre-procedural blood pressures, systolic gradients, coarctation diameters, and the ratio of the coarctation site diameter to the descending aorta diameter. Although all patients received antihypertensive drugs before the procedure, the drug was discontinued in 26 patients during follow-up (p < 0.001). Stent migration was observed in four patients with recurrent coarctation (11.4%), and peripheral arterial injury was seen in three patients (8.5%). The mean follow-up time was 34 ± 16 months. On average, 21 (6–42) months after the procedure, six patients underwent cardiac catheterisation. At least 2 years after the procedure, tomography was performed in 20 patients (57.2%). Patients who were evaluated by multi-slice computerised tomography revealed no pathologies. There was no statistically significant difference between the five patients weighing less than 20 kg and the other 30 patients in terms of demographic and procedural characteristics, procedure success and complication rates, and follow-up data. Conclusion: Stent implantation for aortic coarctation is a method yielding satisfactory results in reducing coarctation gradients, efficient enlargement of the lesion area, and resolution of hypertension for children, including those weighing less than 20 kg.

Background: In some inherited connective tissue diseases with involvement of the cardiovascular system, for example, Marfan syndrome, early impairment of left ventricular function, which have been described as Marfan-related cardiomyopathy has been reported. Our aim was to evaluate the left ventricular function in young adults with mitral valve prolapse without significant mitral regurgitation using two-dimensional strain imaging and to determine the possible role of the transforming growth factor-β pathway in its deterioration. Methods: We studied 78 young adults with mitral valve prolapse without mitral regurgitation in comparison with 80 sex-matched and age-matched healthy individuals. Longitudinal strain and strain rates were defined using spackle tracking. Concentrations of transforming growth factor-β1 and β2 in serum were determined by enzyme-linked immunosorbent assays. Results: In 29 patients, classic relapse was identified with a leaflet thickness of ≥ 5 mm; 49 patients had a non-classic mitral valve prolapse. Despite the similar global systolic function, a significant reduction in global strain was found in the classic group (−15.5 ± 2.9%) compared with the non-classic group (−18.7 ± 3.8; p = 0.0002) and the control group (−19.6 ± 3.4%; p < 0.0001). In young adults with non-classic prolapse, a reduction in longitudinal deformation was detected only in septal segments. Transforming growth factor-β1 and β2 serum levels were elevated in patients with classic prolapse as compared with the control group and the non-classic mitral valve prolapse group. Conclusions: These changes in the deformations may be the first signs of deterioration of the left ventricular function and the existence of primary cardiomyopathy in young adults with mitral valve prolapse, which may be caused by increased transforming growth factor-β signalling.

Background: Hypertrophic cardiomyopathy is an important cause of disability and death in patients of all ages. Egyptian children may differ from Western and Asian patients in the pattern of hypertrophy distribution, clinical manifestations, and risk factors. Objectives: The aim of our study was to report the clinical characteristics and outcomes of Egyptian children with hypertrophic cardiomyopathy studied over a 7-year duration and to determine whether the reported adult risk factors for sudden cardiac death are predictive of the outcome in these affected children. Study design and methods: This retrospective study included 128 hypertrophic cardiomyopathy children. The data included personal history, family history, physical examination, baseline laboratory measurements, electrocardiogram, and Holter and echocardiographic results. Logistic regression analysis was used for the detection of risk factors of death. Results: Fifty-one out of 128 patients died during the period of the study. Of the 51 deaths, 36 (70.5%) occurred in patients presenting before 1 year of age. Only eight patients had surgical intervention. Extreme left ventricular hypertrophy, that is, interventricular septal wall thickness or posterior wall thickness Z-score >6, sinus tachycardia, and supraventricular tachycardia were found to be independent risk factors for prediction of death in patients with hypertrophic cardiomyopathy. Conclusions: At our Egyptian tertiary care centre, hypertrophic cardiomyopathy has a relatively worse prognosis when compared with reports from Western and Asian series. Infants have a worse outcome than children presenting after the age of 1 year. A poorer prognosis in childhood hypertrophic cardiomyopathy is predicted by an extreme left ventricular hypertrophy, the presence of sinus tachycardia, and supraventricular tachycardia.

Tetralogy of Fallot and coarctation of the aorta is an exceptional association. We report here four cases of infants referred for tetralogy of Fallot with or without pulmonary atresia associated with aortic coarctation from 1974 to 2013. All had a right aortic arch, and the coarctation was abnormally situated between the right common carotid and the right subclavian arteries. In all, two infants had an abnormal left subclavian artery and one child had DiGeorge syndrome. All underwent staged surgical repair of the left and right-sided obstructions. A review of the literature shows two types of coarctation in this context. In left aortic arch, coarctation is situated distal to the left subclavian artery. In right aortic arch, coarctation is distal to the right common carotid artery, mirror-image of interrupted left aortic arch type B, associated with anomalies of the branches of the aorta, and should be considered a complex anomaly of aortic arches in the setting of an outflow tract defect due to abnormal migration of cardiac neural crest cells. Screening for this unusual association is critical in the initial assessment of all patients with tetralogy of Fallot.