scholarly journals Unraveling Gene Expression Profiles of Cardiac Genes that Participate in Embryonic development of Congenital Heart Defects using Chick Embryo

2020 ◽  
Author(s):  
Amal Nasr ◽  
Aya Omar ◽  
Maha Alser ◽  
Huseyin Yalcin ◽  
Fatiha Benslimane
Keyword(s):  
Gene Expression ◽  
Left Atrial ◽  
Congenital Heart ◽  
Expression Profiles ◽  
Heart Defects ◽  
Gene Expression Profiles ◽  
Cardiac Markers ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a rare but serious subtype of congenital heart defect (CHDs) at which the hemodynamics are disturbed. In this project, HLHS was introduced surgically by left atrial ligation (LAL) to embryonic chicks and the subsequent effects of it were studied. Different tests were done post-LAL to study cardiac morphology, function, and gene expression of cardiac markers.

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.

Zabiegi kardiologii interwencyjnej u noworodków

2020 ◽  
Vol 24 (1) ◽  
Author(s):  
Bożena Werner
Keyword(s):  
Aortic Stenosis ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Atrial Septum ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Cardiac Catheter ◽  
Atrial Septostomy ◽  
Left Heart Syndrome

In the last 30 years, a great progress has been made in the treatment of congenital heart defects by interventional cardiology procedures. Cardiac catheter interventions are implemented in fetuses with critical aortic stenosis and pulmonary atresia with intact ventricular septum to promote the growth and improve function of the left or right ventricle. In a case of aortic stenosis it could prevent the evolution to hypoplastic left heart syndrome. Balloon atrial septostomy or stent placement is a lifesaving procedure in fetuses with hypoplastic left heart syndrome and highly restrictive or intact atrial septum. The majority of cardiac catheter interventional procedures in neonates are palliative and lifesaving. They include: balloon atrial septostomy or static balloon dilation or cutting balloon septostomy or stenting of the atrial septum in congenital heart defects with duct-dependent blood mixing, balloon valvuloplasty in neonates with critical aortic valve stenosis, stent implantation into the right ventricular outflow tract or arterial duct in cyanotic neonates with duct-dependent pulmonary circulations, angioplasty for stenotic vessels. The therapeutic cardiac interventions in neonatal period are: pulmonary valvuloplasty in patients with critical pulmonary stenosis and challenging transcatheter ductus arteriosus closure in preterm and extremely low birth weight infants.

scholarly journals Effect of left atrial ligation-driven altered inflow hemodynamics on embryonic heart development: clues for prenatal progression of hypoplastic left heart syndrome

2021 ◽  
Author(s):  
Huseyin Enes Salman ◽  
Maha Alser ◽  
Akshay Shekhar ◽  
Russell A. Gould ◽  
Fatiha M. Benslimane ◽  
...  
Keyword(s):  
Embryonic Development ◽  
Hypoplastic Left Heart Syndrome ◽  
Heart Development ◽  
Left Atrial ◽  
Heart Defects ◽  
Systemic Circulation ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
The Right ◽  
Left Heart Syndrome

AbstractCongenital heart defects (CHDs) are abnormalities in the heart structure present at birth. One important condition is hypoplastic left heart syndrome (HLHS) where severely underdeveloped left ventricle (LV) cannot support systemic circulation. HLHS usually initiates as localized tissue malformations with no underlying genetic cause, suggesting that disturbed hemodynamics contribute to the embryonic development of these defects. Left atrial ligation (LAL) is a surgical procedure on embryonic chick resulting in a phenotype resembling clinical HLHS. In this study, we investigated disturbed hemodynamics and deteriorated cardiac growth following LAL to investigate possible mechanobiological mechanisms for the embryonic development of HLHS. We integrated techniques such as echocardiography, micro-CT and computational fluid dynamics (CFD) for these analyses. Specifically, LAL procedure causes an immediate flow disturbance over atrioventricular (AV) cushions. At later stages after the heart septation, it causes hemodynamic disturbances in LV. As a consequence of the LAL procedure, the left-AV canal and LV volume decrease in size, and in the opposite way, the right-AV canal and right ventricle volume increase. According to our CFD analysis, LAL results in an immediate decrease in the left AV canal WSS levels for 3.5-day (HH21) pre-septated hearts. For 7-day post-septated hearts (HH30), LAL leads to further reduction in WSS levels in the left AV canal, and relatively increased WSS levels in the right AV canal. This study demonstrates the critical importance of the disturbed hemodynamics during the heart valve and ventricle development.

scholarly journals Clinical audit of pre- and postnatal diagnostics of obstructive congenital left heart defects at the perinatal center (level III)

2020 ◽  
Vol 25 (8) ◽  
pp. 3788
Author(s):  
O. V. Shumakova ◽  
E. L. Bokeria
Keyword(s):  
Aortic Arch ◽  
Congenital Heart ◽  
Clinical Audit ◽  
Heart Defects ◽  
Coarctation Of The Aorta ◽  
Venous Drainage ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Perinatal Center ◽  
Left Heart Syndrome

Aim. To compare the data of pre- and postnatal expert echocardiographic diagnostics of obstructive left heart defects (LHD) to identify a possible predictors and reasons for hypo- and overdiagnosis of this.Material and methods. A retrospective analysis of data from expert echocardiography of 194 fetuses and newborns with suspected obstructive LHD (coarctation of the aorta (CA), interruption of the aortic arch, aortic stenosis (AS), CA or interruption of the aortic arch with AS, hypoplastic left heart syndrome (HLHS)) was performed.Results. After birth, critical obstructive LHD was confirmed in 59,3% of newborns (115 out of 194): in cases of prenatally diagnosed CA — in 41% of children (42 out of 103); AS — in 67% (4 out of 6); CA with AS — in 65,9% (27 out of 41); HLHS — in 93,9% (39 out of 44); or interruption of the aortic arch with and without AS — in 100% (11 out of 11). After birth, the discrepancy in diagnoses of critical congenital heart defects was detected in 2 cases — obstructive LHD were not confirmed, but total anomalous pulmonary venous drainage (APVD) was detected. In children with a confirmed diagnosis of HLHS after birth, there was a hypodiagnosis of obstructive forms of APVD in three newborns.Conclusion. The probability of confirming a critical obstructive LHD after birth is higher, the more LHD were hypoplasized during intrauterine imaging. APVD may be “hemodynamically masked” as HLHS when prenatal echocardiography is performed. The combination of HLHS and obstructive forms of APVD can lead to HLHS “hemodynamic aggravation” due to an additional decrease in blood flow of left heart.

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