Longitudinal analysis of myocardial function and cerebral perfusion in fetuses with left heart defects

AbstractTransseptal puncture (TSP) is a standard procedure to obtain access to the left heart. However, data on TSP in infants and children particularly with congenital heart defects (CHD) is sparse. Safety and efficacy of TSP in infants and children < 18 years with normal cardiac anatomy and with CHD were assessed. 327 TSP were performed in a total of 300 individuals < 18 years from 10/2002 to 09/2018 in our tertiary pediatric referral center. Median age at TSP was 11.9 years (IQR 7.8–15; range: first day of life to 17.9 years). 13 subjects were < 1 year. Median body weight was 43.8 kg (IQR 26.9–60; range: 1.8–121 kg). CHD was present in 28/327 (8.6%) procedures. TSP could be successfully performed in 323/327 (98.8%) procedures and was abandoned in 4 procedures due to imminent or incurred complications. Major complications occurred in 4 patients. 3 of these 4 subjects were ≤ 1 year of age and required TSP for enlargement of a restrictive atrial septal defect in complex CHD. Two of these babies deceased within 48 h after TSP attempt. The third baby needed urgent surgery in the cath lab. Pericardial effusion requiring drainage was noted in the forth patient (> 1 year) who was discharged well later. Minor complications emerged in 5 patients. The youngest of these individuals (0.3 years, 5.8 kg) developed small pericardial effusion after anterograde ballon valvuloplasty for critical aortic stenosis. The remaining 4/5 patients developed small pericardial effusion after ablation of a left-sided accessory atrioventricular pathway (6.1–12.2 years, 15.6–34.0 kg). TSP for access to the left heart was safe and effective in children and adolescents > 1 year of age. However, TSP was a high-risk procedure in small infants with a restrictive interatrial septum with need for enlargement of interatrial communication.

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

PEDIATRICS ◽

10.1542/peds.85.6.977 ◽

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.

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Standardization of the Perioperative Management for Neonates Undergoing the Norwood Operation for Hypoplastic Left Heart Syndrome and Related Heart Defects

Pediatric Critical Care Medicine ◽

10.1097/pcc.0000000000002478 ◽

2020 ◽

Vol 21 (9) ◽

pp. e848-e857

Author(s):

Shobha S. Natarajan ◽

Alyson Stagg ◽

April M. Taylor ◽

Heather M. Griffis ◽

Christy K. Bosler ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Perioperative Management ◽

Heart Defects ◽

Left Heart ◽

Norwood Operation ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

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The genetics of hypoplastic left heart syndrome

Cardiology in the Young ◽

10.1017/s1047951100005722 ◽

1999 ◽

Vol 9 (6) ◽

pp. 627-632 ◽

Cited By ~ 31

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.

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

BioMed Research International ◽

10.1155/2015/360209 ◽

2015 ◽

Vol 2015 ◽

pp. 1-6 ◽

Cited By ~ 2

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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Zabiegi kardiologii interwencyjnej u noworodków

Nowa Pediatria ◽

10.25121/np.2020.24.1.8 ◽

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.

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Introduction to a Norwood Program in an Emerging Economy: Learning Curve of a Single Center

The Heart Surgery Forum ◽

10.1532/hsf98.2013222 ◽

2013 ◽

Vol 16 (6) ◽

pp. 313 ◽

Cited By ~ 1

Author(s):

Murat Başaran ◽

Eylem Tunçer ◽

Füsun Güzelmeriç ◽

Nihat Çine ◽

Naci Öner ◽

...

Keyword(s):

Learning Curve ◽

Hypoplastic Left Heart Syndrome ◽

Emerging Economies ◽

Cerebral Perfusion ◽

Cerebral Oximetry ◽

Left Heart ◽

Single Center ◽

Hypoplastic Left Heart ◽

Stage 1 ◽

Left Heart Syndrome

Background: There has been a notable improvement in the outcome of stage 1 palliation for hypoplastic left heart syndrome (HLHS) in recent years. Nevertheless, developing a new Norwood program requires a steep learning curve, especially in emerging economies where rapid population growth brings a high volume of patients but, on the other hand, resources are limited. In this paper we aimed to summarize the initial results of a single center.Methods: Hospital records of 21 patients were reviewed for all patients having a stage 1 palliation procedure for HLHS between May 2011 and May 2013. There were 13 male (62%) and 8 female (38%) patients. Median age was 14 days (range, 4-74 days) and median weight was 3030 g (2600-3900 g). HLHS was defined as mitral or aortic stenosis or atresia (or both) in the presence of normally related great vessels and a hypoplastic left ventricle. Transthoracic echocardiography was the diagnostic modality used in all patients. All procedures but one were performed using an antegrade selective cerebral perfusion method and moderate hypothermia. Cerebral perfusion was monitored with cerebral oximetry in all patients. Modified ultrafiltration was routinely used in all patients.Results: Overall hospital mortality was 47.6% (n = 10). Mortality rates considerably decreased from the first year to second year (69% and 12.5% respectively). No risk factors were identified for mortality.Conclusions: Surgical palliation of neonates with hypoplastic left heart syndrome continues to be a challenge. To decrease the overall mortality nationwide and improve outcomes, a referral center with a dedicated team is necessary in emerging economies.

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

University of the Future: Re-Imagining Research and Higher Education ◽

10.29117/quarfe.2020.0196 ◽

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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Prenatal diagnosis and management of hypoplastic left heart syndrome: Single center results

Zeynep Kamil Medical Journal ◽

10.14744/zkmj.2021.50479 ◽

2021 ◽

pp. 16-20

Author(s):

Yunus Emre PURUT

Keyword(s):

Prenatal Diagnosis ◽

Hypoplastic Left Heart Syndrome ◽

Heart Defects ◽

Fontan Procedure ◽

Classical Type ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Tertiary Center ◽

The Mean ◽

Left Heart Syndrome

Objective: Hypoplastic left heart syndrome (HLHS) is the most common reason for neonatal deaths among congenital heart defects. Numerous studies showed that pre- natal diagnosis improves prognosis. We aimed to review the prenatal assessment of associated extracardiac anomalies, postnatal outcomes, and surgical management in cases of HLHS that were detected in our center. Material and Methods: The records of patients diagnosed with HLHS evaluated be- tween March 2017 and April 2020. A detailed anatomy scan was performed, and karyotype analysis was recommended to all patients. Due to poor perinatal prognosis, termination of pregnancy (TOP) was offered an option to families. Serial ultrasono- graphic examinations every 2–4 weeks. Postnatal echocardiography was performed, and the prenatal diagnosis was confirmed in all offspring. Surgical outcomes were recorded. Results: Sixteen patients were recruited in our study. The mean gestational age at diagnosis was 20.2±5.1 weeks. About 68.7% of cases were defined as classical type HLHS, and the remaining 31.3% were determined as variant type HLHS. TOP was performed in 9 (56.7%) patients. The mean follow-up interval was 16.4±4.7 months. Urge septostomy was performed in 2 (28.5%) cases after birth due to foramen ovale restriction. Three (42.8%) cases died before the first operation. Norwood procedure was performed in 4 (57.1%) cases. Two cases died after this operation. Glenn shunt and Fontan procedure were performed in the remaining two offspring. The total sur- vival rate was 28.5%. Conclusion: HLHS has high perinatal morbidity and mortality. Prenatal diagnosis allows the family for the fate of pregnancy and planned delivery in a tertiary center.

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