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.

Incidence and impact of acute kidney injury in patients with hypoplastic left heart syndrome following the hybrid stage 1 palliation

2020 ◽  
pp. 1-7
Author(s):  
Tyler W. Cunningham ◽  
Yubo Tan ◽  
Catherine D. Krawczeski ◽  
John D. Spencer ◽  
Shasha Bai ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Prenatal Diagnosis ◽  
Hypoplastic Left Heart Syndrome ◽  
Lower Incidence ◽  
Kidney Injury ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Stage 1 ◽  
Left Heart Syndrome ◽  
Hybrid Stage 1 Palliation

Abstract Objective: Acute kidney injury leads to worse outcomes following paediatric cardiac surgery. There is a lack of literature focusing on acute kidney injury after the Hybrid stage 1 palliation for single ventricle physiology. Patients undergoing the Hybrid Stage 1, as a primary option, may have a lower incidence of kidney injury than previously reported. When present, kidney injury may increase the risk of post-operative morbidity and mortality. Methods: A retrospective, single centre review was conducted in patients with hypoplastic left heart syndrome who underwent Hybrid Stage 1 from 2008 to 2018. Acute kidney injury was defined as a dichotomous yes (meeting any injury criteria) or no (no injury) utilising two different criteria utilised in paediatrics. The impact of kidney injury on perioperative characteristics and 30-day mortality was analysed. Results: The incidence of acute kidney injury is 13.4–20.7%, with a severe injury rate of 2.4%. Patients without a prenatal diagnosis of hypoplastic left heart syndrome have a higher incidence of kidney injury than those prenatally diagnosed, (40% versus 14.5%, p = 0.024). Patients with acute kidney injury have a significantly higher incidence of 30-day mortality, 27.3%, compared to without, 5.6% (p = 0.047). Discussion: The incidence of severe acute kidney injury after the Hybrid Stage 1 palliation is low. A prenatal diagnosis may be associated with a lower incidence of kidney injury following the Hybrid Stage 1. Though uncommon, severe acute kidney injury following Hybrid Stage 1 may be associated with higher 30-day mortality.

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.

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

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.

scholarly journals Effect of acid–base balance on postoperative course in children with hypoplastic left heart syndrome after the modified Norwood procedure

Medicine ◽  
2017 ◽  
Vol 96 (34) ◽  
pp. e7739
Author(s):  
Marcin Gładki ◽  
Tomasz Składzień ◽  
Rafał Żurek ◽  
Elżbieta Broniatowska ◽  
Elżbieta Wójcik ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Norwood Procedure ◽  
Left Heart ◽  
Acid Base Balance ◽  
Hypoplastic Left Heart ◽  
Acid Base ◽  
Postoperative Course ◽  
Base Balance ◽  
Left Heart Syndrome

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.

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