scholarly journals German Heart Centre Munich—45 years of surgery in adults with congenital heart defects: from primary corrections of septal defects and coarctation to complex reoperations

2021 ◽  
Vol 11 (2) ◽  
pp. 492-502
Author(s):  
Jelena Pabst von Ohain ◽  
Eleonora Tonino ◽  
Harald Kaemmerer ◽  
Julie Cleuziou ◽  
Peter Ewert ◽  
...  
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Septal Defects

The clinic pattern of pulmonary artery vasoconstriction in children (morbus coelius)

1927 ◽  
Vol 23 (10) ◽  
pp. 1041-1045
Author(s):  
B. M. Deich
Keyword(s):  
Pulmonary Artery ◽  
Birth Defects ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Pulmonary Artery Stenosis ◽  
Septal Defects ◽  
Nosological Entity ◽  
Simultaneous Existence ◽  
Large Vessels

"Blue disease" is not a separate nosological entity, but represents a certain syndrome accompanying congenital heart defects. Despite the fact that this disease has long been known, nevertheless, its pathogenesis has not yet been definitively established. The former authors explained various individual forms of cardiac birth defects by this syndrome, but now it has been established that "blue disease" is a collective concept and occurs, according to Barye, in septal defects and anomalies of large vessels, and according to Fallop, in simultaneous existence of pulmonary artery stenosis and non-cavitary septum as well as in non-cavitary ductus botalus.

scholarly journals Coxsackievirus B3 Infection Early in Pregnancy Induces Congenital Heart Defects Through Suppression of Fetal Cardiomyocyte Proliferation

2021 ◽  
Vol 10 (2) ◽  
Author(s):  
Vipul Sharma ◽  
Lisa S. Goessling ◽  
Anoop K. Brar ◽  
Chetanchandra S. Joshi ◽  
Indira U. Mysorekar ◽  
...  
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Transforming Growth Factor ◽  
Heart Defects ◽  
Viral Myocarditis ◽  
Cardiomyocyte Proliferation ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
Cvb3 Infection ◽  
Viral Titers

Background Coxsackievirus B (CVB) is the most common cause of viral myocarditis. It targets cardiomyocytes through coxsackie and adenovirus receptor, which is highly expressed in the fetal heart. We hypothesized CVB3 can precipitate congenital heart defects when fetal infection occurs during critical window of gestation. Methods and Results We infected C57Bl/6 pregnant mice with CVB3 during time points in early gestation (embryonic day [E] 5, E7, E9, and E11). We used different viral titers to examine possible dose‐response relationship and assessed viral loads in various fetal organs. Provided viral exposure occurred between E7 and E9, we observed characteristic features of ventricular septal defect (33.6%), abnormal myocardial architecture resembling noncompaction (23.5%), and double‐outlet right ventricle (4.4%) among 209 viable fetuses examined. We observed a direct relationship between viral titers and severity of congenital heart defects, with apparent predominance among female fetuses. Infected dams remained healthy; we did not observe any maternal heart or placental injury suggestive of direct viral effects on developing heart as likely cause of congenital heart defects. We examined signaling pathways in CVB3‐exposed hearts using RNA sequencing, Kyoto Encyclopedia of Genes and Genomes enrichment analysis, and immunohistochemistry. Signaling proteins of the Hippo, tight junction, transforming growth factor‐β1, and extracellular matrix proteins were the most highly enriched in CVB3‐infected fetuses with ventricular septal defects. Moreover, cardiomyocyte proliferation was 50% lower in fetuses with ventricular septal defects compared with uninfected controls. Conclusions We conclude prenatal CVB3 infection induces congenital heart defects. Alterations in myocardial proliferate capacity and consequent changes in cardiac architecture and trabeculation appear to account for most of observed phenotypes.

scholarly journals An Unusual Mechanism of Closure of Muscular Ventricular Septal Defects

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Soham Dasgupta ◽  
Ashraf M. Aly
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Fibrous Tissue ◽  
Spontaneous Closure ◽  
Muscular Tissue ◽  
Tissue Formation ◽  
Ventricular Septal Defects ◽  
Septal Defects ◽  
Muscular Vsd

Ventricular septal defects (VSDs) are the most common congenital heart defects. Most of the small or moderate size (<6 mm) muscular VSDs close spontaneously within the first two years of life. The usual mechanism of spontaneous closure involves muscular tissue encroachment with superimposed fibrosis or primary fibrous tissue formation around the margins of the defect. We describe an unusual mechanism of spontaneous closure of a muscular VSD.

Abstract P140: Maternal Smoking During Pregnancy and Risk of Congenital Heart Defects: A Systematic Review and A Meta-Analysis

Circulation ◽  
2012 ◽  
Vol 125 (suppl_10) ◽  
Author(s):  
Laura Lee ◽  
Philip Lupo
Keyword(s):  
Congenital Heart Defects ◽  
Maternal Smoking ◽  
Congenital Heart ◽  
Heart Defects ◽  
Meta Analysis ◽  
Epidemiological Studies ◽  
Ventricular Septal Defects ◽  
Smoking During Pregnancy ◽  
Septal Defects ◽  
Maternal Smoking During Pregnancy

Objective: Congenital heart defects (CHDs) are the most common group of structural malformations occurring in approximately 1 out of every 100 births. In addition to being the most prevalent birth defect, CHDs are the leading cause of death due to congenital malformations. Despite their prevalence and clinical importance, little is known about the etiology of CHDs, and there are currently no strategies for reducing their prevalence. Maternal smoking during pregnancy has been suggested as a potential risk factor for congenital heart defects, but the evidence is inconclusive. We systematically reviewed epidemiological studies and conducted a meta-analysis of the association between maternal cigarette smoking during pregnancy and the risk of CHDs among the infants. Methods: From a search of published literature through July 2011, we extracted 35 original human epidemiological studies that examined the association between maternal smoking during pregnancy (including a month before conception) and the risk of CHDs in offspring. Odds ratios (ORs) and 95% confidence intervals (CIs) were extracted from each study or calculated from available data, comparing smoking to no smoking during pregnancy. Summary risk estimates were also calculated for a number of CHDs subtypes (e.g., conotruncal defects, ventricular septal defects) using both fixed- and random-effects models. Random effects estimates were reported if there was evidence of heterogeneity among the studies. Effects of paternal and environmental smoking were not considered in our analysis. Results: We observed statistically significant positive association between maternal smoking anytime during pregnancy and the risk of CHDs (OR = 1.10; 95% CI: 1.01–1.19; number of cases (n) = 19,552). Additionally, we found a positive and significant association between maternal smoking and the risk of conotruncal defects in offspring (OR = 1.14; 95% CI: 1.03–1.26; n = 1953). Although not statistically significant, stronger elevated risks were observed for ventricular septal defects (OR = 1.11; 95% CI: 0.87–1.41; n = 4605) and atrial septal defects (OR = 1.34; 95% CI: 0.97–1.86; n = 565). Conclusion: Our systematic review and meta-analysis suggest maternal smoking is modestly associated with an increased risk of CHDs and some CHDs subtypes. This work adds to the existing body of evidence that implicated maternal smoking during pregnancy as a possible risk factor for CHDs. Future studies should consider evaluating paternal and environmental smoking exposure to better understand the relationship between smoking and CHDs.

Thymic medullary structures: Microscopical picture of the thymic medullary structures in children with congenital heart defects

Biologia ◽  
2012 ◽  
Vol 67 (1) ◽  
Author(s):  
Renáta Mikušová ◽  
Paulína Gálfiová ◽  
Štefan Polák
Keyword(s):  
Neural Crest ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Ventricular Septal Defects ◽  
Electron Microscopic ◽  
Septal Defects ◽  
The Face ◽  
Thymic Medulla ◽  
Thymus Cells

AbstractThe aim of this work is to describe the structure of the thymus, especially its medullary part, in children with congenital heart defects. It is known that development of the thymus and the heart is also influenced by neural crest cells. During the early development of the heart and the thymus cells proliferate and migrate to their primordia. It is known that inadequate cephalic neural crest contribution during development of pharyngeal pouch derivatives results in defective organogenesis of the face, the thymus, parathyroid glands and also the heart. We studied the structure of the thymus in children with congenital heart defects from 0 to 12 years of life at light microscopic and electron-microscopic levels. Thymuses of the patients were surgically removed in the Children’s Cardiocenter in Bratislava. The results of our study confirmed the differences in the medullary structures of thymuses with chosen diagnoses. Hassall’s corpuscles in the thymic medulla were various in size and also in structure and number. The special structures of the thymic medullary region in children with ventricular septal defects and defects of outflow of the heart were big cystic Hassall’s corpuscles. In comparison with a size of Hassall’s corpuscles in normal thymuses the size of Hassall’s corpuscles in studied thymuses suprisingly ranged between 100–250 μm.

Congenital heart defects in La Réunion Island: a 6-year survey within a EUROCAT-affiliated congenital anomalies registry

2012 ◽  
Vol 22 (5) ◽  
pp. 547-557 ◽  
Author(s):  
Hélène Bourdial ◽  
Karim Jamal-Bey ◽  
Abdelhafid Edmar ◽  
Dominique Caillet ◽  
Françoise Wuillai ◽  
...  
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Pulmonary Valve Stenosis ◽  
La Réunion ◽  
Ventricular Septal Defects ◽  
Perinatal Deaths ◽  
Septal Defects ◽  
Total Prevalence

AbstractObjectivesThis study compares the prevalence and perinatal mortality of congenital heart defects on La Réunion with European (EUROCAT) standards.Methods and resultsData were extracted from a EUROCAT-affiliated congenital malformations registry, covering 88,025 births during the period 2002–2007, on the whole island territory. A total of 512 congenital heart defects were registered, including 424 live births, 18 foetal deaths from 16 weeks of gestation, and 70 terminations of pregnancy. The total prevalence of congenital heart defects was 5.8 per 1000 births and live birth prevalence was 4.8 per 1000. The total prevalence of non-chromosomal congenital heart defects was 5.1 per 1000 births, of which 3% were perinatal deaths, 33.3% prenatally diagnosed, and 11.6% termination of pregnancy. Severe non-chromosomal congenital heart defects – excluding ventricular septal defects, atrial septal defects, and pulmonary valve stenosis – occurred in 2.1 per 1000 births, of which 10.3% were perinatal deaths, 59.1% prenatally diagnosed, and 24.3% termination of pregnancy. Of the severe congenital heart defects, the rates of single ventricle (0.20‰), Ebstein anomaly (0.11‰), common arterial trunk (0.25‰), and atrioventricular septal defect (0.62‰) exceeded averages found in Europe, although coarctation of the aorta was infrequent. Conversely, rates of ventricular septal defects, atrial septal defects, and pulmonary valve stenosis were inferior to European standards. Slightly less than half of the congenital heart defects of chromosomal origin were associated with Down syndrome.ConclusionIn La Réunion, the total prevalence of congenital heart defects is far inferior to that found in Europe. The difference can be attributable to lower prevalences of mild congenital heart defects.

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