Congenital heart disease (CHD) is a congenital disorder primarily affecting newborns and children. Children with CHD have a greater risk of experiencing growth delays or disorders compared to healthy children. CHD also affects various aspects of a child\'s development. The aim of this study was to determine the association of CHD types (cyanotic and acyanotic) with the growth and development status of children. A cross-sectional study was conducted among CHD patients at a national reference hospital in Sumatra, H. Adam Malik General Hospital in Medan, Indonesia. The children\'s growth status was assessed using the WHO growth chart, and the developmental condition was evaluated through the Denver Developmental Screening Test-II. Chi-squared test and Fisher\'s exact test were used to assess the association between the type of CHD with growth and development status in children. Using a consecutive sampling method, a total of 53 individuals were included in this study. Almost half of CHD patients (48.1%) were within the age group of 0-2 years and more than half (61.1%) were girls. Acyanotic CHD (74.1%) was more prevalent than cyanotic CHD (25.9%), with ventricular septum defect (VSD) as the most common diagnosis. A total of 37% of children with CHD suffered from malnutrition, whereas the remaining 62.9% had good nutrition. The Denver Developmental Screening Test-II indicated that 81.4% of the children were normal, whereas 18.5% had developmental disorders. Our data suggested a significant association between cyanotic CHD and poor growth status in children based on weight-for-age, weight-for-length, and body mass index (BMI)-for-age. However, there was no association between the type of CHD and developmental status in children. This study highlights that the type of CHD is significantly associated with the growth status of children, but not with their developmental status.

UNASSIGNED: Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes.
UNASSIGNED: We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD.
UNASSIGNED: In this prospective observational study, eligible infants and children ≤21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental \"delay\" and \"no delay\" groups.
UNASSIGNED: Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental \"delay\" and \"no delay\" groups (P = 0.04).
UNASSIGNED: Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery.

Growth retardation, malnutrition, and failure to thrive are some of the consequences associated with congenital heart diseases. Several metabolic factors such as hypoxia, anoxia, and several genetic factors are believed to alter the energetics of the heart. Timely diagnosis and patient management is one of the major challenges faced by the clinicians in understanding the disease and provide better treatment options. Metabolic profiling has shown to be potential diagnostic tool to understand the disease.
The present experiment was designed as a single center observational pilot study to classify and create diagnostic metabolic signatures associated with the energetics of congenital heart disease in cyanotic and acyanotic groups.
Metabolic sera profiles were obtained from 35 patients with cyanotic congenital heart disease (TOF) and 23 patients with acyanotic congenital heart disease (ASD and VSD) using high resolution 1D 1H NMR spectra. Univariate and multivariate statistical analysis were performed to classify particular metabolic disorders associated with cyanotic and acyanotic heart disease.
The results show dysregulations in several metabolites in cyanotic CHD patients versus acyanotic CHD patients. The discriminatory metabolites were further analyzed with area under receiver operating characteristic (AUROC) curve and identified four metabolic entities (i.e. mannose, hydroxyacetone, myoinositol, and creatinine) which could differentiate cyanotic CHDs from acyanotic CHDs with higher specificity.
An untargeted metabolic approach proved to be helpful for the detection and distinction of disease-causing metabolites in cyanotic patients from acyanotic ones and can be useful for designing better and personalized treatment protocol.

The optimal dose of tranexamic acid in minimizing perioperative bleeding is uncertain. We compared efficacy of two different doses of tranexamic acid in reducing post-operative blood loss and its side effects in patients with congenital cyanotic heart disease undergoing cardiac surgery.
Prospective observational study at a pediatric cardiac center in South India.
Consecutive cyanotic patients undergoing cardiac surgery were divided into groups I and II to receive either 10 mg/kg or 25 mg/kg of tranexamic acid administered as triple dose regime after induction, during cardiopulmonary bypass, and after protamine. Post-operative blood loss at 24 hours, blood component utilization, incidence of renal dysfunction and seizures were compared.
Totally, 124 patients were recruited, 62 in each group. The pre-operative variables and cardiopulmonary bypass time were comparable. Patients receiving 25 mg/kg had lower post-operative blood loss compared to patients in lower dose group (8.04 ± 8.89 vs 12.41 ± 19.23 ml/kg/24 hours, P = 0.03). There was no difference in the transfused volume of packed red cells (9.21 ± 7.13 ml/kg vs 12.41 ± 9.23 ml/kg, P = 0.712), fresh frozen plasma (13.91 ± 13.38 ml/kg vs 11.02 ± 8.04 ml/kg, P = 0.19), platelets (9.03 ± 6.76 ml/kg vs 10.90 ± 6.9 ml/kg, P = 0.14) or cryoprecipitate (0.66 ± 0.59 ml/kg vs 0.53 ± 0.54 ml/kg, P = 0.5) in group II and I, respectively. Two patients developed renal dysfunction secondary to low cardiac output in lower dose group. There were no seizures.
Tranexamic acid administered at a dose of 25 mg/kg as triple dose regime is associated with lower post-operative blood loss compared to a lower dose of 10 mg/kg in cyanotic patients undergoing cardiac surgery without causing major adverse effects.

UNASSIGNED: Oxidative stress can cause many diseases and increases the risk of post-operative complications in children with congenital heart disease. For these reasons, this study was aimed to investigate the differences between cyanotic and acyanotic paediatric patients who underwent heart surgery with markers of oxidative stress.
UNASSIGNED: Eighty five patients were included in the study. The samples taken before the surgery and within the first 24 h after the surgery were evaluated for haemoglobin (Hb), leukocytes, uric acid, glutathione (GSH), malondialdehyde and total antioxidant capacity. Cyanotic, acyanotic, hyperoxygenated, normo-oxygenated, cardiac surgery with or without cardiopulmonary bypass (CPB) comparisons were made.
UNASSIGNED: Positive correlation was found between age and pre-operative total antioxidant status values. Cyanotic and acyanotic patients did not have different antioxidant reserve capacities preoperatively. Although pre-operative thiobarbituric acid reactive substances (TBARS) levels were significantly lower in cyanotic patients, post-operative levels were higher. TBARS levels increased and GSH levels reduced postoperatively. The level of oxygenation did not cause a significant difference on markers of oxidative stress. The duration of CPB did not have negative effects on oxidative stress.
UNASSIGNED: Cyanotic and younger patients were found to be more vulnerable to oxidative stress. The increased levels of TBARS and the decreased levels of GSH could be the indicators of oxidative damage depending on many factors such as surgery, CPB, ischaemia/reperfusion, inflammation, iron overload and oxygenation. The level of oxygenation does not cause a noticeable difference in oxidative stress. CPB causes oxidative stress, but if it is conducted appropriately, the duration of CPB does not cause a significant negative impact on oxidative stress.

OBJECTIVE: To characterize, using magnetic resonance imaging (MRI), the distribution of blood flow and oxygen transport in human fetuses with subtypes of congenital heart disease (CHD) that present with neonatal cyanosis.
METHODS: Blood flow was measured in the major vessels of 152 late-gestation human fetuses with CHD and 40 gestational-age-matched normal fetuses, using cine phase-contrast MRI. Oxygen saturation (SaO2 ) was measured in the major vessels of 57 fetuses with CHD and 40 controls.
RESULTS: Compared with controls, we found lower combined ventricular output in fetuses with single-ventricle physiology, with the lowest being observed in fetuses with severe forms of Ebstein\'s anomaly. Obstructive lesions of the left or right heart were associated with increased flow across the contralateral side. Pulmonary blood flow was reduced in fetuses with Ebstein\'s anomaly, while those with Ebstein\'s anomaly and tricuspid atresia had reduced umbilical flow. Flow in the superior vena cava was elevated in fetuses with transposition of the great arteries, normal in fetuses with hypoplastic left heart, tetralogy of Fallot or tricuspid atresia and reduced in fetuses with Ebstein\'s anomaly. Umbilical vein SaO2 was reduced in fetuses with hypoplastic left heart or tetralogy of Fallot. Ascending aorta and superior vena cava SaO2 were reduced in nearly all CHD subtypes.
CONCLUSIONS: Fetuses with cyanotic CHD exhibit profound changes in the distribution of blood flow and oxygen transport, which result in changes in cerebral, pulmonary and placental blood flow and oxygenation. These alterations of fetal circulatory physiology may influence the neonatal course and help account for abnormalities of prenatal growth and development that have been described in newborns with cyanotic CHD. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

OBJECTIVE: Endothelin-1 plays an important role in the pathogenesis of severe pulmonary hypertension. The + 139 \'A\', adenine insertion variant in 5\'UTR of edn1 gene has been reported to be associated with increased expression of Endothelin-1 in vitro. The aim of present study was to explore the association of this variant with the circulating levels of Endothelin-1 in vivo using archived DNA and plasma samples from 38 paediatric congenital heart disease (cyanotic and acyanotic) patients with severe pulmonary hypertension.
RESULTS: The plasma Endothelin-1 levels were highly varied ranging from 1.63 to75.16 pg/ml. The + 139 \'A\' insertion variant in 5\'UTR of edn1 was seen in 8 out of 38 cases with only one acyanotic sample demonstrating homozygosity of inserted \'A\' allele at + 139 site (4A/4A genotype). The plasma Endothelin-1 levels in children with homozygous variant 3A/3A genotype were comparable in cyanotic and acyanotic groups. Lone 4A/4A acyanotic sample had ET-1 levels similar to the median value of ET-1 associated with 3A/3A genotype and was absent in cyanotic group presumably due to deleterious higher ET-1 levels. The discussed observations, limited by the small sample size, are suggestive of homozygous adenine insertion variant posing a risk in cyanotic babies with Severe Pulmonary Hypertension.

Background: Data is limited about the incidence of congenital heart disease in Jordan. The goal of this study is to determine the incidence and patterns of congenital heart diseases (CHD) among Jordanian infants evaluated at King Abdullah University Hospital. Methods: A retrospective chart review was conducted for all infants who had an echocardiogram evaluation in the 3-years period July 2016-June 2019. All included infants had a 2-dimentional echocardiogram with a Doppler vascular study performed by the same cardiologist. Infants with a structural congenital heart disease were included in the analysis. Results: A total of 1,028 infants were evaluated at the cardiology department during the study period. Eight hundred and sixty-five had an abnormal echo finding. Two hundred and ninety-eighth were diagnosed with CHD. The incidence of CHD was 25 per 1000 live births. Fifty one percent were premature infants. The majority of cases were mild CHD. Patent ductus arteriosus was the most common acyanotic lesion followed by ventricular septal defect and atrial septal defect with a prevalence of 44, 25, and 25%, respectively. Cyanotic CHD constituted 6% of all CHD. Tetralogy of Fallott was the most common cyanotic CHD. The main indication for referral was hearing a heart murmur during physical examination. Conclusion: Although the incidence of CHD in our cohort was relatively high, the majority of cases were acyanotic mild CHD with favorable prognosis. A wider population-based study is needed to evaluate the incidence and better understand the patterns and distribution of CHD at a national level.

The objective of this study is to assess the prevalence and risk factors for attention-deficit hyperactivity disorder (ADHD) in a large cohort of patients with congenital heart disease (CHD). Patients (n = 695) with CHD who were aged 6-15 years and visited the outpatient clinics in our hospital from June 2015 to May 2017 were enrolled. Their medical records were collected, and the Chinese version of the Swanson, Nolan, and Pelham rating scale (SNAP-IVc) and a questionnaire about neuropsychiatric care-seeking behavior were completed by parents and counselors. Of the 695 patients, the overall prevalence of ADHD was 12.4%, including 3.2% for the combined subtype, 6.8% for the inattentive-predominant subtype, and 2.4% for the hyperactivity/impulsive-predominant subtype. Only the inattention-predominant subtype was significantly more prevalent than in the general population. The prevalence of the inattention-predominant subtype was highest in the patients with cyanotic CHD, high severity index, and in those who had received surgery or cardiopulmonary bypass. Multivariate regression analysis indicated that the risk factors for inattention-related symptoms included postoperative seizure and previous cardiopulmonary bypass (odds ratio: 3.22 and 3.82; P = 0.027 and < 0.001, respectively). Only 58.7% of the patients with probable ADHD ever sought neuropsychiatric care, and only 27% regularly attended neuropsychiatric clinics. The inattention-predominant subtype of ADHD was more prevalent in our CHD patients, especially in those with cyanotic CHD, higher disease severity index, and in those who had undergone a surgical intervention. The percentage of patients receiving regular neuropsychiatric clinic follow-up was low.

BACKGROUND: Uncorrected congenital heart lesions in children keep them in a state of constant hypoxia with compromised quality of life and reduced life expectancy. This requires early diagnosis and interventions including prevention and treatment of the resultant anaemia. Unfortunately, congenital heart disease (CHD) often goes unrecognized and thus untreated.
OBJECTIVE: We determined the occurrence of CHD in children below 15 years at the Komfo Anokye Teaching Hospital (KATH), assessed the prevalence of relative iron deficiency anaemia in that cohort and the use of iron supplementation in these patients.
METHODS: We conducted a cross-sectional study, using a structured data collection tool, by retrospectively reviewing patient records from December 2015 to January 2010. Data was also obtained prospectively from January 2016 to March 2016.
RESULTS: Eighty cases (44 females and 36 males) of CHD were encountered. Tetralogy of Fallot was the most common (48.8%) CHD. Cases of cyanotic congenital heart disease were reported at autopsy. Of the 80 cases, 48 (72.7%) had signs of relative iron deficiency. Thirty (62.5%) of the 48 patients did not receive iron supplementation. In 14 cases, full blood count was not determined and yet 10 patients received iron at sub-optimal doses (<3 mg/kg/day) and one was given iron at 6 mg/kg/day.
CONCLUSIONS: CHD is a common phenomenon among newborns at KATH. Use of iron supplementation was suboptimal. Compliance with guidelines on the use of iron as well as structures for early detection of CHD for definitive interventions are advocated.