The Natural History of the Ductus Arteriosus in Association with Other Congenital Heart Defects

1975 ◽  
Vol 18 (4) ◽  
pp. 541-572 ◽  
Author(s):  
Donald E. Cassels ◽  
Saroja Bharati ◽  
Maurice Lev
Keyword(s):  
Natural History ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
History Of

Hypoplastic Left Heart Syndrome: Natural History in a Geographically Defined Population

PEDIATRICS ◽  
1990 ◽  
Vol 85 (6) ◽  
pp. 977-983
Author(s):  
Cynthia D. Morris ◽  
Jacquelyn Outcalt ◽  
Victor D. Menashe
Keyword(s):  
Natural History ◽  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart ◽  
Heart Defects ◽  
Norwood Procedure ◽  
Secular Change ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
History Of ◽  
Left Heart Syndrome

Advances in surgical treatment of hypoplastic left heart syndrome with the Norwood procedure and cardiac transplantation have made essential the understanding of the natural history of hypoplastic left heart syndrome. In a geographically defined population, we ascertained the prevalence of hypoplastic left heart syndrome in children born in Oregon from 1971 through 1986. Clinical and anatomic data were extracted from the charts of the 98 affected children and the survival rate was calculated. Hypoplastic left heart syndrome occurred in 0.162 per 1000 live births in Oregon during this period. No syndrome complex was prevalent and 84% were free of other congenital malformations. However, there was an increased occurrence of congenital heart defects in first-degree relatives of probands with hypoplastic left heart syndrome. Of the affected children 15 ± 4% died on the first day of life, 70 ± 5% died within the first week, and 91 ± 3% died within 30 days. No secular change in survival occurred during the study. Palliation with the Norwood procedure was performed in 20 children. Although survival was significantly improved with this surgery (P = .01), the effect was observed principally through 30 days of life and only one of these children remains alive. Hypoplastic left heart syndrome is a lethal congenital heart defect in children and poses management and ethical dilemmas.

scholarly journals Congenital Heart Defects Are Rarely Caused by Mutations in Cardiac and Smooth Muscle Actin Genes

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Tatiana Khodyuchenko ◽  
Anna Zlotina ◽  
Tatiana Pervunina ◽  
Dmitry Zverev ◽  
Anna Malashicheva ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Smooth Muscle Actin ◽  
Ductus Arteriosus ◽  
Control Group ◽  
Missense Mutations ◽  
Muscle Actin ◽  
Cardiac Actin

Background. Congenital heart defects (CHDs) often have genetic background due to missense mutations in cardiomyocyte-specific genes. For example, cardiac actin was shown to be involved in pathogenesis of cardiac septum defects and smooth muscle actin in pathogenesis of aortic aneurysm in combination with patent ductus arteriosus (PDA). In the present study, we further searched for mutations in humanα-cardiac actin (ACTC1) and smooth muscleα-actin (ACTA2) genes as a possible cause of atrial septum defect type II (ASDII) and PDA.Findings. Total genomic DNA was extracted from peripheral blood of 86 individuals with ASDs and 100 individuals with PDA. Coding exons and flanking intron regions ofACTC1(NM_005159.4) andACTA2(NM_001613) were amplified by PCR with specific primers designed according to the corresponding gene reference sequences. PCR fragments were directly sequenced and analyzed. Sequence analysis ofACTC1andACTA2did not identify any nucleotide changes that altered the coding sense of the genes. InACTC1gene, we were able to detect one previously described nucleotide polymorphism (rs2307493) resulting in a synonymous substitution. The frequency of this SNP was similar in the study and control group, thus excluding it from the possible disease-associated variants.Conclusions. Our results confirmed that the mutations inACTC1gene are rare (at least <1%) cause of ASDII. Mutations inACTA2gene were not detected in patients with PDA, thus being excluded from the list of frequent PDA-associated genetic 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 Spectrum of associated congenital heart defects in patients with “Non-Cardiac Congenital defects at a tertiary care children hospital in Pakistan”

2021 ◽  
Vol 37 (3) ◽  
Author(s):  
Muhammad Sohail Arshad ◽  
Muhammad Aslam ◽  
Shahnab Ahmad ◽  
Muhammad Kashif
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Vascular Malformations ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Tertiary Care ◽  
Primary Diagnosis ◽  
Congenital Defects

Objectives: To assess the frequency and pattern of associated congenital heart disease (CHD) among patients with “non-cardiac congenital defects”. Methods: An observational study was done at Paediatric Cardiology Department, The Children’s Hospital and The Institute of Child Health, Multan, Pakistan, from December 2018 to November 2019. Children from birth to 15 years having non-cardiac congenital defects, referred for cardiac evaluation from surgical unit during the study period were enrolled. Echocardiography was done to confirm diagnosis of CHD by consultant pediatric cardiologist. Results: Out of a total of 323 cases, 176 (54.5%) were male. Out of 323 patients, 160 (49.5%) belonged to age one month to one year. Vascular malformations were the most frequent primary diagnosis among our cases, seen in 69 (21.4%) children followed by cleft lip and palate 55 (17.2%), cleft palate only 52 (16.1%), Cleft lip only 40 (12.4%) and ARM high variety 33 (10.2%). CHD was found among 42 (13.0%) children while patent ductus arteriosus (PDA) and VSD were the commonest finding seen in 14 (33.3%) and 6 (14.3%) children respectively. Conclusion: Frequency of associated CHD among patients with non-cardiac congenital defects was high (13.0%). Children with cleft lip and/or palate should be given more attention because of the high incidence of CHD in this group. Echocardiography must be advised for the timely identification of any possible type of CHD. doi: https://doi.org/10.12669/pjms.37.3.3604 How to cite this:Arshad MS, Aslam M, Ahmad S, Kashif M. Spectrum of associated congenital heart defects in patients with “Non-Cardiac Congenital defects at a tertiary care children hospital in Pakistan”. Pak J Med Sci. 2021;37(3):---------. doi: https://doi.org/10.12669/pjms.37.3.3604 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Pharmacotherapy of Congenital Heart Defects

2004 ◽  
Vol 9 (3) ◽  
pp. 160-178
Author(s):  
Shannan Eades
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Pediatric Population ◽  
Ductus Arteriosus ◽  
Pharmacological Agents ◽  
Mild Heart Failure ◽  
Cardiovascular Defects

Congenital cardiovascular defects account for significant morbidity and mortality in the pediatric population. Complications of congenital heart disease are lesion-dependent and may range from mild heart failure with no cyanosis to severe cyanosis and shock. Pharmacotherapy of congenital heart disease is also lesion-dependent and usage may range from palliative agents (e.g., prostaglandin E1 for relaxation of aortic stricture) to corrective agents (e.g., indomethacin for closure of the ductus arteriosus). This review will discuss the aberrant pathophysiology and complications associated with specific congenital heart defects, as well as the selection of pharmacological agents used in the management of these defects.

Survival, Morbidities, and Developmental Outcomes among Low Birth Weight Infants with Congenital Heart Defects

2020 ◽  
Author(s):  
Mihai Puia-Dumitrescu ◽  
Laura N. Sullivan ◽  
David Tanaka ◽  
Kimberley Fisher ◽  
Rick Pittman ◽  
...  
Keyword(s):  
Birth Weight ◽  
Low Birth Weight ◽  
Congenital Heart Defects ◽  
Gestational Age ◽  
Congenital Heart ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Developmental Outcomes ◽  
Low Birth Weight Infants ◽  
Common Diagnosis

Objective Prematurity and low birth weight (LBW) are risk factors for increased morbidity and mortality in infants with congenital heart defects (CHDs). We sought to describe survival, inhospital morbidities, and 2-year neurodevelopmental follow-up in LBW infants with CHD. Study Design We included infants with birth weight (BW) <2,500 g diagnosed with CHD (except isolated patent ductus arteriosus) admitted January 2013 to March 2016 to a single level-IV academic neonatal intensive care unit. We reported CHD prevalence by BW and gestational age; selected in-hospital morbidities and mortality by infant BW, CHD type, and surgical intervention; and developmental outcomes by Bayley's scales of infant and toddler development, third edition (BSID-III) scores at age 2 years. Results Among 420 infants with CHD, 28 (7%) underwent cardiac surgery. Median (25th and 75th percentiles) gestational age was 30 (range: 27–33) weeks and BW was 1,258 (range: 870–1,853) g. There were 134 of 420 (32%) extremely LBW (<1,000 g) infants, 82 of 420 (20%) were small for gestational age, and 51 of 420 (12%) multiples. Most common diagnosis: atrial septal defect (260/420, 62%), followed by congenital anomaly of the pulmonary valve (75/420, 18%). Most common surgical procedure: pulmonary artery banding (5/28, 18%), followed by the tetralogy of Fallot corrective repair (4/28, 14%). Survival to discharge was 88% overall and lower among extremely LBW (<1,000 g, 81%) infants and infants undergoing surgery (79%). Comorbidities were common (35%); retinopathy of prematurity and bronchopulmonary dysplasia were most prevalent. BSID-III scores were available on 148 of 176 (84%); any scores <85 were noted in 73 of 148 (49%), with language being most commonly affected. Conclusion Among LBW infants with congenital heart disease, hospital mortality varied by BW and cardiac diagnosis. Key Points

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.

Sign in / Sign up

Export Citation Format

Share Document