Significant challenges face developing countries as a result of the maldistribution of access to healthcare throughout the world, specifically access to paediatric cardiac care. Sustainable paediatric cardiac programmes must be established in developing countries to provide care to all children with congenital heart disease. Education and research are essential components to sustainable paediatric cardiac programmes in developing countries to define local problems and the incidence of disease, and to generate solutions thereto related. Research can contribute to developing local expertise, improving technology, providing opportunities for local talent, generating financial resources, enhancing the dignity of people, and the facilitating resolution of health problems throughout the world. Clinical trials conducted in developing countries should meet the same ethical standards as trials based in developed countries.

The aim of this study was to determine the left ventricular myocardial deformation and segmental myocardial dysfunction by speckle tracking echocardiography and tissue Doppler imaging among the operated patients with anomalous origin of the left coronary artery from the pulmonary artery. The study was conducted on 12 patients diagnosed with anomalous origin of the left coronary artery from the pulmonary artery, who had been operated upon between 2001 and 2013 at the medical centres of Shiraz University of Medical Sciences, Shiraz, Iran. The mean age of the patients at the time of surgical correction was 12.6 years ranging from 6 months to 43 years, and the duration of postoperative follow-up was between 1 and 12 years. Comparison of the strain rate between the patients with acceptable ejection fraction and the control group by tissue Doppler imaging showed significant differences between the two groups regarding the lateral wall (p<0.001), but not the septal wall of the left ventricle (p=0.65). Moreover, the strain values by the speckle tracking method revealed significant differences between the patient and the control group regarding the global strain (p=0.016) and anterior, lateral, and posterior segments of the left ventricle. Although postoperative conventional echocardiography revealed normal global left ventricular function with acceptable ejection fraction, abnormal myocardial deformation of the variable segments of the left ventricle with regional and global myocardial dysfunction were well defined by speckle tracking echocardiography.

We assessed parental attitudes towards congenital cardiovascular malformations in their children in a cross-sectional study in Egypt. Parents face many problems related to concerns about their child’s prognosis, but these associations with parental stress have never been evaluated in Egypt or examined in relation to religiosity in a predominantly Muslim society. Accordingly, we conducted interviews in Cairo with mothers of 99 sequential infants born with conotruncal heart malformations (cases) and 65 mothers of age-matched controls. The survey assessed healthcare access and usage, knowledge of congenital cardiovascular malformations, religiosity, the Locus of Control Scale, and the Parenting Stress Index. Results showed that 45% of the mothers of cases had correct knowledge about their child’s diagnosis; 85% were satisfied with the clinical care; and 79% reported that the cost of care was burdensome. Compared with parents of cases, parents of controls were more likely to report stress overall and all its subscales. Regarding belief about locus of control over health, God as a determining factor was given the highest endorsement. Mothers in the congenital cardiovascular malformations group reported a higher level of parental locus of control than did those in the control group. The correlations between stress and locus of control were stronger in the control than in the case group. Religiosity was related neither to stress nor to locus of control. Future studies can explore the roles that personal, familial, and societal factors play in exacerbating or reducing stress levels among parents of sick children, particularly in developing countries where economic pressures are acute.

Neonates with critical CHD have evidence, by imaging, of preoperative brain injury, although the timing is unknown. We used circulating postnatal serum glial fibrillary acidic protein as a measure of acute perinatal brain injury in neonates with CHD. Glial fibrillary acidic protein was measured on admission and daily for the first 4 days of life in case and control groups; we included two control groups in this study – non-brain-injured newborns and brain-injured newborns. Comparisons were performed using the Kruskal–Wallis test with Dunn’s multiple comparisons, Student’s t-test, and χ2 test of independence where appropriate. In aggregate, there were no significant differences in overall glial fibrillary acidic protein levels between CHD patients (n=56) and negative controls (n=23) at any time point. By day 4 of life, 7/56 (12.5%) CHD versus 0/23 (0%) normal controls had detectable glial fibrillary acidic protein levels. Although not statistically significant, the 5/10 (50%) left heart obstruction group versus 1/17 (6%) conoventricular, 0/13 (0%) right heart, and 1/6 (17%) septal defect patients trended towards elevated levels of glial fibrillary acidic protein at day 4 of life. Overall, glial fibrillary acidic protein reflected no evidence for significant peripartum brain injury in neonates with CHD, but there was a trend for elevation by postnatal day 4 in neonates with left heart obstruction. This pilot study suggests that methods such as monitoring glial fibrillary acidic protein levels may provide new tools to optimise preoperative care and neuroprotection in high-risk neonates with specific types of CHD.

Approximately 32,000 infants are born with CHDs each year in the United States of America. Of every 1000 live births, 2.3 require surgical or transcatheter intervention in the first year of life. There are few more stressful times for parents than when their neonate receives a diagnosis of complex CHD requiring surgery. The stress of caring for these infants is often unrelenting and may last for weeks, months, and often years, placing parents at risk for developing post-traumatic stress disorder, as well as a drastic decrease in quality of life. Anxiety often peaks in the days and weeks after discharge from the hospital as families no longer have immediate access to nursing and medical staff. The purpose of this paper is to describe the methods of a randomised controlled trial that was designed to determine whether REACH would favourably affect parental and infant outcomes by decreasing parental stress, improve parental quality of life, increase infant stability, and decrease resource utilisation in infants with complex CHD.

The purpose of this study was to assess cardiorespiratory responses to submaximal exercise in patients with univentricular atrioventricular connection after the Fontan operation, and to evaluate whether changes occur during medium-term follow-up. Eighteen patients (age 12.1±5.5 years) underwent graded exercise test on a treadmill 2.3±1.4 year after the Fontan repair. Ventilatory gases were measured using breath-by-breath analysis. Results were compared to gender/age-matched controls. Twelve patients (age 14.2±5.4 years) were reevaluated 2.4±2.1 years after the first test. Aerobic exercise performance was subnormal in all patients during the first test. At the lowest level of exercise, the ventilatory threshold was already surpassed in 6/18 patients, while it was reduced in all other patients (p<0.001). All patients were in stable sinus rhythm throughout the test. Heart rate at all exercise levels was ±10% below normal (p<0.05). The respiratory frequency was increased at all exercise levels (p<0.001). The ventilatory equivalent for oxygen was increased (p<0.001), and the end-tidal tension of carbon dioxide was decreased (p<0.001). The ratio of physiological dead space/tidal volume was increased in all patients (p<0.001), while the normal decrease of this ratio during exercise was not observed. Upon reevaluation heart rate, respiratory rate, oxygen uptake, venti latory equivalent for oxygen, end-tidal carbon dioxide tension and physiological dead space did not change signifi cantly. Only a slight further decrease in ventilatory threshold was observed. Aerobic performance after the Fontan procedure ranges widely from just above resting metabolic rate to the lower limit of normal. Dyspnea during exercise is exacerbated by a decreased ventilatory threshold, increased physiological dead space, and decreased respir-atory efficiency. Cardiorespiratory response to exercise, nonetheless, remains relatively stable during medium-term follow-up.

The most appropriate way of describing the congenital cardiac malformations unified because the atrial chambers are joined across the atrioventricular junctions to morphologically inappropriate ventricles has long been contentious. In the past, the lesions have been described in such arcane terms as mixed levocardia,1 while “ventricular inversion” still retains it currency in some circles. As we will show in this review, the abnormal arrangements at the atrioventricular junctions can be found with various patterns, but most frequently the patients also have the arterial trunks arising from morphologically inappropriate ventricles. This combination is best described as congenitally corrected transposition, and will form the focus of our review. It is salutary to note that, when von Rokitansky gave the first description of this combination,2 one of his illustrations was ideally suited to aid the understanding of modern-day echocardiographers (Fig. 1). We hope to emulate von Rokitansky in our own review.

Takotsubo cardiomyopathy or transient apical ballooning syndrome very rarely presents in children. In all patients with takotsubo, it is estimated that only 3.5% will have recurrence. In this study, we describe a case of recurrent takotsubo cardiomyopathy in a child, likely triggered by status epilepticus.

The cardio-ankle vascular index is a measure of arterial stiffness, whereas oxidative stress underlies arterial pathology. This study aimed to investigate the association between the cardio-ankle vascular index and antioxidant-related gene polymorphisms in young Russians. A total of 89 patients (mean age, 21.6 years) were examined by the cardio-ankle vascular index and for 15 gene polymorphisms related to antioxidant enzymes including FMO3 (flavin-containing monooxygenase 3), GPX1 (glutathione peroxidase 1), and GPX4 (glutathione peroxidase 4). A higher cardio-ankle vascular index level was detected in carriers with the KK-genotype of FMO3 polymorphism rs2266782 than in those without (mean levels: 6.2 versus 5.6, respectively, p<0.05). Similarly, a higher cardio-ankle vascular index level was seen in carriers with the CC-genotype of GPX4 polymorphism rs713041 than in those without (6.0 versus 5.5, respectively, p<0.05). We did not observe significant associations between the cardio-ankle vascular index levels and the other gene polymorphisms. Although carriers with the LL-genotype of GPX1 polymorphism rs1050450 showed a higher diastolic blood pressure level than those without, the polymorphism did not affect the cardio-ankle vascular index level. This study showed a significant association between rs2266782 and rs713041 polymorphisms and arterial stiffness, as measured by the cardio-ankle vascular index, in young Russians. The pathways utilised by antioxidant enzymes may be responsible for early arterial stiffening in the Russian population.

We currently perform open-heart procedures using bloodless priming of cardiopulmonary bypass circuits regardless of a patient’s body weight. This study presents results of this blood-saving approach in neonates and infants with a body weight of up to 7 kg. It tests with multivariate analysis factors that affect perioperative transfusion. A total of 498 open-heart procedures were carried out in the period 2014–2016 and were analysed. Priming volume ranged from 73 ml for patients weighing up to 2.5 kg to 110 ml for those weighing over 5 kg. Transfusion threshold during cardiopulmonary bypass was 8 g/dl of haemoglobin concentration. Transfusion factors were first analysed individually. Variables with a p-value lower than 0.2 underwent logistic regression. Extracorporeal circulation was conducted without transfusion of blood in 335 procedures – that is, 67% of cases. Transfusion-free operation was achieved in 136 patients (27%) and was more frequently observed after arterial switch operation and ventricular septal defect repair (12/18=66.7%). It was never observed after Norwood procedure (0/33=0%). Lower mortality score (p=0.001), anaesthesia provided by a certain physician (p=0.006), first chest entry (p=0.013), and higher haemoglobin concentration before going on bypass (p=0.013) supported transfusion-free operation. Early postoperative mortality was 4.4% (22/498). It was lower than expected (6.4%: 32/498). In conclusion, by adjusting the circuit, cardiopulmonary bypass could be conducted without donor blood in majority of patients, regardless of body weight. Transfusion-free open-heart surgery in neonates and infants requires team cooperation. It was more often achieved in procedures with lower mortality score.

Background: Coarctation of the aorta is a very common congenital heart malformation. It is frequently associated with other abnormalities. Echocardiography is the diagnostic modality for congenital heart disease. The carotid-subclavian artery index and the isthmus/descending aorta index were proposed for establishing the diagnosis of coarctation of the aorta. Objectives: The objectives were to evaluate such indexes and to look for other echocardiographic predictors of coarctation of the aorta. Method Echocardiography was reviewed for infants with coarctation of the aorta, as well as a control group, using the Echo PAC Dimension. Standard measurements were obtained from different sites of the aortic arch. Results: A total of 31 infants 3 months or less with coarctation of the aorta and 50 infants with no coarctation of the aorta were reviewed. Abnormal aortic valve was present in 65% of those with coarctation of the aorta. The diameters of the proximal and the distal transverse aortic arch were smaller in the coarctation of the aorta group. The distance between the aortic arch branches was longer in the coarctation of the aorta group. Apart from the ratio between distance 2 and the ascending aorta, other ratios/indexes were smaller in the coarctation of the aorta group than in the control group. Conclusion: The presence of abnormal aortic valve, a carotid subclavian index <1.1, I/AAo ratio <0.53, and DTA/AAo ratio <0.6 suggest the presence of coarctation of the aorta. Neonates with large patent ductus arteriosus and any of these findings need close observation until the patent ductus arteriosus closes. If the arch is difficult to assess by two-dimensional echocardiography, the patient may need further imaging to rule out coarctation of the aorta.

Wiskott-Aldrich syndrome is a rare X-linked disease, associated with immunodeficiency, infections, thrombocytopaenia, and eczema. Aortitis and formation of aneurysms have also been described. We describe here our experience with a 7-year-boy with this syndrome. He survived replacement of the aortic root because of an aneurysmal ascending aorta, and subsequent bone marrow transplantation.

An 8 year old girl presents with cyanosis, exertional dyspnoea, and palpitation only. X-ray displayed a typical coeur en sabot heart and auscultsation revealed a harsh ejection systolic murmur. Echocardiography confirmed Tetralogy of Fallot (McGoon ratio 1.3) and computed tomography was requested for further investigation of pulmonary stenosis. A vascular ring was detected, presenting asymptomatically and previously missed through echocardiography. The patient underwent a double-stage repair with ligation of an aortic arch. Recovery was unremarkable. This case highlights the limitations of echocardiography in detection of extra-cardiac anomalies.