scholarly journals Congenital Anomalies of the Kidney and Urinary Tract in Children with Congenital Heart Defects

2020 ◽  
Vol 45 (2) ◽  
pp. 307-313
Author(s):  
Dapeng Jiang ◽  
Qi Wang ◽  
Zhengzhou Shi ◽  
Jie Sun
Keyword(s):  
Urinary Tract ◽  
Congenital Heart Defects ◽  
Congenital Anomalies ◽  
Congenital Heart ◽  
Medical Center ◽  
Heart Defects ◽  
Clinical Manifestations ◽  
Number Of Patients ◽  
Significant Difference ◽  
History Of

Background/Aims: To investigate the incidence and clinical characteristics of congenital anomalies of the kidney and urinary tract (CAKUT) in children with congenital heart defects (CHD). Methods: We retrospectively analyzed the clinical data of children with CHD with CAKUT admitted to the Shanghai Children’s Medical Center affiliated with the Shanghai Jiao Tong University School of Medicine between September 2018 and March 2019. Patients underwent routine examinations for liver, kidney, and coagulation function, and urinary tract ultrasonography, and we summarized patients’ clinical manifestations and imaging abnormalities. Results: A total of 1,410 children with CHD were diagnosed and treated in our hospital. The total number of patients with abnormal urogenital systems was 104, and hydronephrosis was the most common abnormality, followed by vesicoureteral reflux and duplication of the kidney and ureter. The overall prevalence of CAKUT was 7.4%. There was no statistically significant difference for maternal age, sex, parity, gestational age, and history of medication during pregnancy between the patients with CAKUT and those without CAKUT. Conclusion: The incidence of CAKUT in our patients with CHD was significantly higher than that in the general population. We recommend urinary ultrasonography as a routine examination for children with CHD for early detection of CAKUT, to avoid missed diagnoses, and to initiate appropriate treatment.

scholarly journals Epigenomic and Transcriptomic Dynamics During Human Heart Organogenesis

2020 ◽  
Vol 127 (9) ◽  
Author(s):  
Jennifer VanOudenhove ◽  
Tara N. Yankee ◽  
Andrea Wilderman ◽  
Justin Cotney
Keyword(s):  
Gene Expression ◽  
Congenital Heart Defects ◽  
Human Heart ◽  
Congenital Heart ◽  
Heart Defects ◽  
Regulatory Control ◽  
Whole Genome Sequencing Data ◽  
Regulatory Sequences ◽  
Sequencing Data ◽  
Number Of Patients

Rationale: There is growing evidence that common variants and rare sequence alterations in regulatory sequences can result in birth defects or predisposition to disease. Congenital heart defects are the most common birth defect and have a clear genetic component, yet only a third of cases can be attributed to structural variation in the genome or a mutation in a gene. The remaining unknown cases could be caused by alterations in regulatory sequences. Objective: Identify regulatory sequences and gene expression networks that are active during organogenesis of the human heart. Determine whether these sites and networks are enriched for disease-relevant genes and associated genetic variation. Methods and Results: We characterized ChromHMM (chromatin state) and gene expression dynamics during human heart organogenesis. We profiled 7 histone modifications in embryonic hearts from each of 9 distinct Carnegie stages (13–14, 16–21, and 23), annotated chromatin states, and compared these maps to over 100 human tissues and cell types. We also generated RNA-sequencing data, performed differential expression, and constructed weighted gene coexpression networks. We identified 177 412 heart enhancers; 12 395 had not been previously annotated as strong enhancers. We identified 92% of all functionally validated heart-positive enhancers (n=281; 7.5× enrichment; P <2.2×10 −16 ). Integration of these data demonstrated novel heart enhancers are enriched near genes expressed more strongly in cardiac tissue and are enriched for variants associated with ECG measures and atrial fibrillation. Our gene expression network analysis identified gene modules strongly enriched for heart-related functions, regulatory control by heart-specific enhancers, and putative disease genes. Conclusions: Well-connected hub genes with heart-specific expression targeted by embryonic heart-specific enhancers are likely disease candidates. Our functional annotations will allow for better interpretation of whole genome sequencing data in the large number of patients affected by congenital heart defects.

scholarly journals Palivizumab for prophylaxis of respiratory syncytial virus infections in preterm neonates

2017 ◽  
Vol 229 (05) ◽  
pp. 259-260
Author(s):  
Ludwig Gortner
Keyword(s):  
Respiratory Syncytial Virus ◽  
Congenital Heart Defects ◽  
Congenital Anomalies ◽  
Congenital Heart ◽  
Heart Defects ◽  
The United States ◽  
Preterm Neonates ◽  
Virus Infections ◽  
Number Of Children ◽  
Syncytial Virus

Recent data indicate that the number of children cared for respiratory syncytial virus (RSV) infections in hospitals is in the range of 50,000 to 100,000 per year in the United States 3. Major risk factors include apart from prematurity specifically below 29 weeks, congenital heart defects, Down syndrome and further congenital anomalies. The American Academy of Pediatrics recommended in 2014 a restriction of Palivizumab prophylaxis to preterm infants of <29 weeks of gestation and those with a high-risk course postnatally and higher gestational ages apart from specific indications resulting from congenital anomalies including congenital heart defects 1.

scholarly journals A randomized control trial: Effects of teach back method on self-efficacy among mothers of children with congenital heart defects

2018 ◽  
Vol 8 (7) ◽  
pp. 106
Author(s):  
Azza A. Ghoneim ◽  
Aml AbdElrazk Fathalla
Keyword(s):  
Congenital Heart Defects ◽  
Self Efficacy ◽  
Congenital Heart ◽  
Heart Defects ◽  
Nursing Intervention ◽  
University Hospital ◽  
Health Concern ◽  
Discharge Instructions ◽  
Significant Difference ◽  
Randomized Control

Background/Objective: Congenital Heart Defects (CHD) remain a major health concern all over the world particularly Egypt where the prevalence of CHD is 1.0 per 1,000. Nurses are instrumental in supplying information. The teach-back method is a technique used for improving patient understanding and outcomes. This study aimed to evaluate the effect of teach back method on self-efficacy and satisfaction among mothers of children with congenital heart defects.Methods: The design of this study was randomized control trail. A sample of 60 children with congenital heart defects and their mothers participated in this study. It conducted at Menofia University hospital. Tools of this study included Self Efficacy Scale; Teach back Discharge Education Audit and Satisfaction Assessment.Results: The current study revealed that the majority of nurses were unfamiliar with teach-back method and there was significant difference between mothers in the experimental and control groups regarding their self-efficacy.Conclusions: This study concluded that mothers who received discharge instructions through teach back method had increased self-efficacy and high level of satisfaction. Therefore, pediatric nurses should integrate teach back method as a routine nursing intervention in the discharge plan for children with congenital heart defects.

scholarly journals A clinical importance of natriuretic peptides in pediatric practice

2017 ◽  
Vol 21 (1) ◽  
pp. 117 ◽  
Author(s):  
E. V. Saperova ◽  
I. V. Vakhlova
Keyword(s):  
Congenital Heart Defects ◽  
Natriuretic Peptides ◽  
Conflict Of Interest ◽  
Congenital Heart ◽  
Heart Defects ◽  
Clinical Importance ◽  
Volume Overload ◽  
Disease Outcomes ◽  
History Of

<p class="a">Natriuretic peptides (NUP) are peptide hormones secreted by cardiomyocytes in response to a volume overload of the heart. Emphasis in the article is placed on the history of NUP discovery, their chemical structure and pathophysiological mechanisms of action. The data on determination of NUP reference values in children with cardiac pathologies are analyzed. A NUP clinical significance in early diagnosis of heart failure in children, including those with congenital heart defects is shown. Also considered is NUP dynamics after surgical correction of congenital heart defects. A conclusion is drawn that NUP play an important prognostic role in evaluating early and late postoperative periods and disease outcomes on a whole.</p><p align="JUSTIFY">Received 11 October 2016. Accepted 22 December 2016.</p><p align="JUSTIFY"><strong>Funding: </strong>The study had no sponsorship.</p><p align="JUSTIFY"><strong>Conflict of interest: </strong>The authors declare no conflict of interest.</p><p align="JUSTIFY"><strong>Author contributions</strong></p><p align="JUSTIFY">All authors contributed equally at all stages of the research.</p>

scholarly journals Prevalence of Congenital Heart Defects in Neonates of Drug Abusing Mothers

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Mohammad Radgoodarzi ◽  
Elahe Norouzi ◽  
Zahra Vahedi ◽  
Mitra Salavati ◽  
Ameneh Yaghoubi ◽  
...  
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart Defect ◽  
Congenital Heart ◽  
Heart Defects ◽  
Normal Population ◽  
Color Doppler ◽  
Newborn Infants ◽  
Teaching Hospitals ◽  
Heart Defect ◽  
Significant Difference

Background: Congenital heart defects (CHDs) are the most common congenital malformations at birth. Substance abuse has increased dramatically over the past two decades. It also can affect neonates of drug-abusing mothers. Objectives: This study aimed to elucidate the possible association of maternal drug abuse with CHDs in their newborn infants. Patients and Methods: In this study, 72 neonates who were born during 6 years in three teaching hospitals are studied. Echocardiography was performed by a single pediatric cardiologist using two-dimensional and color Doppler echocardiography. The data were analyzed using descriptive statistics. Results: 1) Of 72 included cases, 38 (52.78%) had abnormal echocardiographic findings; 2) from 38 abnormal echocardiography, 35 (48.61% of total and 92.11% of abnormal echoes) had mild congenital heart defect (CHD), and 3 had complex CHD; 3) There was no significant difference in the prevalence of neonatal congenital heart defect with the type of misused drugs (opiates or methamphetamines). Conclusions: In our study, the prevalence of CHD in newborns of drug abuser mothers was significantly higher than the normal population of infants. Hence, echocardiographic screening of these newborns seems to be logical.

scholarly journals The incidence of congenital heart defects in the world regarding the severity of the defect

2016 ◽  
Vol 73 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Vesna Miranovic
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Methodological Approach ◽  
Live Births ◽  
Significant Difference ◽  
The World

Bacground/Aim. Congenital heart defects (CHDs) are structural or functional abnormalities of the heart present at birth even if they are detected much later. Their importance lies in the fact that, depending on the severity, they change the quality of life, and may be life threating. In addition, we should not ignore the high costs of treating people with congenital heart disease. The aim of this study was to analyze the incidence of congenital heart disease in relation to the severity in the world based on the available literature. Methods. All the available literature on the incidence of CHD cases regarding the severity of CHD published from 1955 to 2012 was analyzed. The researcher was able to read the titles and abstracts of 128 papers on the subject. Due to methodological inconsistency, 117 of the papers were rejected. Based on the criteria of reliability, availability and comparability, our analysis included 11 studies testing CHD incidence regarding the severity of the defect conducted all over the world. The Yates' ?2-test was used to compare the observed incidences. Results. The frequency of severe congenital heart defects, ranged from 0.414 to 2.3/1,000 live births, the incidence of moderate congenital heart defects from 0.43 to 2.6/1,000 live births while in the group of minor congenital heart defects the incidence ranged from 0.99 to 10.3/1000 live births. There were no statistically significant differences in the incidence of mild, moderate and severe CHDs. Conclusion. The results obtained studying of the available data suggest that no statistically significant difference in the incidence of mild, moderate and severe congenital heart defects. A universal methodological approach to the incidence of CHD is essential.

scholarly journals Maternal sociodemographic characteristics, early pregnancy behaviours, and livebirth outcomes as congenital heart defects risk factors - Northern Ireland 2010-2014

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Hafi Saad ◽  
Marlene Sinclair ◽  
Brendan Bunting
Keyword(s):  
Risk Factors ◽  
Northern Ireland ◽  
Birth Outcomes ◽  
Congenital Heart Defects ◽  
Early Pregnancy ◽  
Gestational Age ◽  
Congenital Heart ◽  
Heart Defects ◽  
Sociodemographic Characteristics ◽  
History Of

Abstract Background Congenital Heart Defects (CHD) is the most commonly occurring congenital anomaly in Europe and a major paediatric health care concern. Investigations are needed to enable identification of CHD risk factors as studies have given conflicting results. This study aim was to identify maternal sociodemographic characteristics, behaviours, and birth outcomes as risk factors for CHD. This was a population based, data linkage cohort study using anonymised data from Northern Ireland (NI) covering the period 2010-2014. The study cohort composed of 94,067 live births with an outcome of 1162 cases of CHD using the International Statistical Classification of Diseases and Related Health Problems (ICD)-10 codes and based on the European Surveillance of Congenital Anomalies (EUROCAT) grouping system for CHD. CHD cases were obtained from the HeartSuite database (HSD) at the Royal Belfast Hospital for Sick Children (RBHSC), maternal data were extracted from the Northern Ireland Maternity System (NIMATS), and medication data were extracted from the Enhanced Prescribing Database (EPD). STATA version 14 was used for the statistical analysis in this study, Odds Ratio (OR), 95% Confident intervals (CI), P value, and logistic regression were used in the analysis. Ethical approval was granted from the National Health Service (NHS) Research Ethics Committee. Result In this study, a number of potential risk factors were assessed for statistically significant association with CHD, however only certain risk factors demonstrated a statistically significant association with CHD which included: gestational age at first booking (AOR = 1.21; 95% CI = 1.04-1.41; P < 0.05), family history of CHD or congenital abnormalities and syndromes (AOR = 4.14; 95% CI = 2.47-6.96; P < 0.05), woman’s smoking in pregnancy (AOR = 1.22; 95% CI = 1.04-1.43; P < 0.05), preterm birth (AOR = 3.01; 95% CI = 2.44-3.01; P < 0.05), multiple births (AOR = 1.89; 95% CI = 1.58-2.60; P < 0.05), history of abortion (AOR = 1.12; 95% CI = 1.03-1.28; P < 0.05), small for gestational age (SGA) (AOR = 1.44; 95% CI = 1.22-1.78; P < 0.05), and low birth weight (LBW) (AOR = 3.10; 95% CI = 2.22-3.55; P < 0.05). Prescriptions and redemptions of antidiabetic (AOR = 2.68; 95% CI = 1.85-3.98; P < 0.05), antiepileptic (AOR = 1.77; 95% CI = 1.10-2.81; P < 0.05), and dihydrofolate reductase inhibitors (DHFRI) (AOR = 2.13; 95% CI = 1.17-5.85; P < 0.05) in early pregnancy also showed evidence of statistically significant association with CHD. Conclusion The results of this study suggested that there are certain maternal sociodemographic characteristics, behaviours and birth outcomes that are statistically significantly associated with higher risk of CHD. Appropriate prevention policy to target groups with higher risk for CHD may help to reduce CHD prevalence. These results are important for policy makers, obstetricians, cardiologists, paediatricians, midwives and the public.

Abstract P040: Muscle Metaboreflex Regulation of Arterial Blood Pressure in Children with Congenital Heart Defects

Circulation ◽  
2016 ◽  
Vol 133 (suppl_1) ◽  
Author(s):  
Corey R Tomczak ◽  
Stephanie Fusnik ◽  
Elizabeth Hogeweide ◽  
Mark J Haykowsky ◽  
Michael K Stickland ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Flow Control ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Free Flow ◽  
Post Exercise ◽  
Muscle Metaboreflex ◽  
Arterial Blood ◽  
Significant Difference

Introduction: Metabolically sensitive afferent fibers in skeletal muscle can signal an increase the sympathetic control of arterial blood pressure during exercise. This process, called the muscle metaboreflex, is augmented in adults with cardiovascular disease and contributes to exercise intolerance. The role of the muscle metaboreflex on blood pressure control in children with congenital heart defects (CHD) is not known. Hypothesis: We tested the hypothesis that the mean arterial pressure (MAP) response to the muscle metaboreflex would be augmented in children with CHD compared to healthy controls. Methods: Twenty-six children with CHD (11±2 years; males=14; females=12) and 21 age- and sex-matched controls (11±3 years; males=10; females=11) were studied. CHD diagnoses included Tetralogy of Fallot (n=7), pulmonary or aortic stenosis (n=3), hypoplastic left or right heart syndrome (n=5), Ebstien’s anomaly (n=1), atrial or ventricular septal defect (n=5), transposition of the great arteries (n=2), double inlet left ventricle (n=1), heart transplantation (n=2), tricuspid or pulmonary atresia (n=2), coarctation of the aorta (n=2), and dilated cardiomyopathy (n=1). Testing included a 3 min rest period followed by isometric handgrip exercise at 30% of maximal voluntary contraction for 2 min. Exercise was followed by 3 min of either a free flow control condition or circulatory occlusion to isolate the muscle metaboreflex. Beat-by-beat MAP was recorded using finger plethysmography. Analyses were completed using mixed designs factorial ANOVA with P < 0.05 as the level of significance. Data are mean ± SD. Results: During the free flow control condition, there was no significant difference in resting MAP (75±9 mmHg vs. 78±9 mmHg), exercise MAP (86±14 mmHg vs. 87±12 mmHg), or free flow MAP (72±10 mmHg vs. 78±9 mmHg) between children with CHD vs. controls, respectively ( P > 0.05). MAP significantly increased from rest to exercise and returned to resting levels during the free flow control condition for both groups. For the circulatory occlusion condition, there was no significant difference in resting MAP (73±11 mmHg vs. 74±8 mmHg), exercise MAP (84±15 mmHg vs. 81±11 mmHg), or circulatory occlusion MAP (79±13 mmHg vs. 79±13 mmHg) between children with CHD vs. controls, respectively ( P > 0.05). MAP significantly increased from rest to exercise and MAP remained elevated above resting levels during circulatory occlusion for both children with CHD and controls ( P < 0.001 for all). Conclusions: Post-exercise circulatory occlusion significantly increases MAP in children with CHD and healthy children, with no significant difference in the magnitude of the MAP response between groups. We conclude that children with CHD demonstrate a normal muscle metaboreflex during post-exercise circulatory occlusion.

scholarly journals Pulmonary hypertension in infants with congenital heart defects: are leukotrienes involved?

1997 ◽  
Vol 6 (5-6) ◽  
pp. 323-326 ◽  
Author(s):  
A. Serraf ◽  
J-P. Gascard ◽  
J. Bruniaux ◽  
C. Labat ◽  
C. Planche ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Hypertension ◽  
Congenital Heart Defects ◽  
Pulmonary Blood Flow ◽  
Congenital Heart ◽  
Heart Defects ◽  
Blood Levels ◽  
Blood Samples ◽  
Number Of Patients ◽  
Pulmonary Arterial

The circulating levels of leukotriene E4in infants with congenital heart defects, increased pulmonary blood flow and pulmonary arterial hypertension, were determined and compared with infants with decreased pulmonary blood flow (Tetralogy of Fallot). There was no correlation (r=0.38) between the pulmonary arterial pressure (56 ± 4 mmHg) and the leukotriene E4levels (1.37 ± 0.67 ng/ml blood) measured in peripheral blood samples from the hypertensive group prior to surgery. There was considerable variation in the detectable leukotriene E4levels in blood samples from different patients. The levels detected in the blood samples between the two groups of patients was similar. These data suggest that neither the surgical repair during cardiopulmonary bypass nor the pulmonary hypertension appeared to modify the leukotriene E4blood levels in the small number of patients studied.

scholarly journals Anesthetic Management of a Patient With Ring 18 Syndrome

2021 ◽  
Vol 68 (3) ◽  
pp. 178-179
Author(s):  
Midori Maekawa ◽  
Makoto Yasuda ◽  
Haruka Sasaki ◽  
Yasuharu Tachinami ◽  
Kentaro Mizuta
Keyword(s):  
General Anesthesia ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Genetic Disorder ◽  
Genetic Material ◽  
Clinical Manifestations ◽  
Ring Chromosome ◽  
Chromosome 18 ◽  
Ring Chromosome 18

Ring 18 syndrome or ring chromosome 18 is an extremely rare genetic disorder involving the fusion of the 18th chromosomal ends to form a ring, often with genetic material loss of varying degrees. Although clinical presentation can be extremely variable, characteristic features usually include craniofacial malformations, delayed development, hypotonia, and other skeletal and congenital heart defects. We report the management of a 20-year-old male with ring chromosome 18 who underwent general anesthesia for dental treatment. Clinical manifestations for this patient included intellectual disability, short stature, hypertelorism, flat nasal bridge, micrognathia, a “carp-shaped” mouth, and aortic and pulmonary valve regurgitation. Although mask ventilation and oral intubation were easily performed, nasal intubation was difficult because of rhinostenosis. When providing general anesthesia for a patient with ring chromosome 18, anesthesiologists should evaluate the patient preoperatively for congenital heart defects and prepare for a potential difficult airway.

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