scholarly journals EP04.39: Hypoplastic left heart syndrome: early ultrasound diagnosis of fetal congenital heart defects

2017 ◽  
Vol 50 ◽  
pp. 280-280
Author(s):  
D. Ilic ◽  
M. Bogavac ◽  
Z. Belopavlovic ◽  
G. Keckes ◽  
Z. Tatic Stupar ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Ultrasound Diagnosis ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

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.

Prenatal ultrasound diagnosis of hypoplastic left heart syndrome in utero associated with hydrops fetalis

1982 ◽  
Vol 104 (6) ◽  
pp. 1368-1372 ◽  
Author(s):  
David J Sahn ◽  
Lewis Shenker ◽  
Kathryn L Reed ◽  
Lilliam M Valdes-Cruz ◽  
Richard Sobonya ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
In Utero ◽  
Prenatal Ultrasound ◽  
Hydrops Fetalis ◽  
Ultrasound Diagnosis ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

The genetics of hypoplastic left heart syndrome

1999 ◽  
Vol 9 (6) ◽  
pp. 627-632 ◽  
Author(s):  
Paul D. Grossfeld
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Heart Defects ◽  
Left Ventricular ◽  
Left Ventricular Cavity ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Term Survival ◽  
Surgical Options ◽  
Systemic Ventricle ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome is one of the most therapeutically challenging congenital cardiac defects. It accounts for as many as 1.5% of all congenital heart defects, but is responsible for up to one quarter of deaths in neonates with heart disease.1The management of hypoplastic left heart syndrome is controversial. Two surgical options exist:2,3the Norwood procedure, is a three stage repair in which the morphologically right ventricle is converted to function as the systemic ventricle. Alternatively, orthotopic transplantation can be performed. Although both surgical options have had improved outcomes, the prognosis for long-term survival is guarded, with a five year survival for either approach reported to be in the region of 50–60%. In this review, I explore the evidence for a genetic etiology for the “classic” hypoplastic left heart syndrome, defined as mitral and/or aortic atresia with hypoplasia of the left ventricular cavity and the other left-sided structures.

scholarly journals Umbilical Neutrophil Gelatinase-Associated Lipocalin Level as an Early Predictor of Acute Kidney Injury in Neonates with Hypoplastic Left Heart Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Piotr Surmiak ◽  
Małgorzata Baumert ◽  
Małgorzata Fiala ◽  
Zofia Walencka ◽  
Andrzej Więcek
Keyword(s):  
Acute Kidney Injury ◽  
Hypoplastic Left Heart Syndrome ◽  
Heart Defects ◽  
Kidney Injury ◽  
High Sensitivity ◽  
Left Heart ◽  
Acid Base Balance ◽  
Hypoplastic Left Heart ◽  
Umbilical Blood ◽  
Left Heart Syndrome

Acute kidney injury (AKI) is a primarily described complication after unbalanced systemic perfusion in neonates with congenital heart defects, including hypoplastic left heart syndrome (HLHS). The aim of the study was to compare the umbilical NGAL concentrations between neonates born with HLHS and healthy infants, as well as to analyze whether the determination of NGAL level could predict AKI in neonates with prenatally diagnosed HLHS. Twenty-one neonates with prenatally diagnosed HLHS were enrolled as study group and 30 healthy neonates served as controls. Perinatal characteristics and postnatal parameters were extracted from the hospital neonatal database. In umbilical cord blood, we determined plasma NGAL concentrations, acid base balance, and lactate and creatinine levels. In neonates with HLHS, complications (respiratory insufficiency, circulatory failure, NEC, IVH, and AKI) were recorded until the day of cardiosurgery. We observed in neonates with HLHS higher umbilical NGAL levels compared to controls. Among 8 neonates with HLHS and diagnosed AKI stage 1, we observed elevated NGAL levels in comparison to those newborns without AKI. Umbilical NGAL could predict, with high sensitivity and specificity, AKI development in study neonates. We suggest that the umbilical blood NGAL concentration may be an early marker to predict AKI in neonates with HLHS.

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