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.

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.

Fetal management of hypoplastic left heart syndrome

2020 ◽  
pp. 201-210
Author(s):  
Michael Harris ◽  
Gurleen Sharland
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart ◽  
Interatrial Septum ◽  
Atrial Septum ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Fetal Diagnosis ◽  
The Status ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a major form of congenital heart disease, with universal mortality if left untreated. Even with treatment, survival to eventual palliative univentricular Fontan circulation is in the order of 65% at the age of 5 years. The condition also carries with it a significant load in terms of comorbidities, recurrent interventions, and reduced quality of life. Fetal diagnosis of HLHS is important for accurately counselling the prospective parents on the available options during and after the pregnancy. It is crucial for the adequate planning of, and preparation for, delivery, with evidence emerging that babies delivered with such preparation are in better preoperative condition. Risk stratification is possible with the assessment of restrictive flow at the interatrial septum and the use of magnetic resonance imaging to assess the status of the fetal lungs in this situation. Fetal intervention in cases of a restrictive atrial septum is an emerging and challenging area, with limited evidence for efficacy currently. Due to the high-risk nature of this condition, it is difficult to prove that fetal diagnosis makes any significant impact on mortality.

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.

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