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Hypoplastic Left Heart Syndrome

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3. Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation

Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation PEDSCCM.org Criteria abstracted from series in Review Posted: founded 1995 Questions or comments?

2015 PedsCCM Evidence-Based Journal Club

4. The Degree of Left Ventricular Hypoplasia Is Associated with Tricuspid Regurgitation Severity in Infants with Hypoplastic Left Heart Syndrome. (PubMed)

The Degree of Left Ventricular Hypoplasia Is Associated with Tricuspid Regurgitation Severity in Infants with Hypoplastic Left Heart Syndrome. Tricuspid valve regurgitation (TR) increases adverse outcomes in children with hypoplastic left heart syndrome (HLHS). Changes in tricuspid valve (TV) annulus and leaflet geometry have been described but the underlying causes for TR in HLHS remain uncertain. We aimed to examine the association between left ventricular (LV) size and TR in infants

2019 Pediatric Cardiology

5. Effects of Gestational Age on Early Survivability in Neonates With Hypoplastic Left Heart Syndrome

Effects of Gestational Age on Early Survivability in Neonates With Hypoplastic Left Heart Syndrome Prematurity increases pre- and postoperative mortality in children with congenital heart disease. There are no large, multicentered, studies that have evaluated this relation specifically in neonates with hypoplastic left heart syndrome (HLHS). We sought to determine the impact of gestational age (GA) on survival to Stage 1 palliation surgery and hospital discharge in infants with HLHS. We

2018 EvidenceUpdates

6. Effectiveness of interstage home monitoring on healthcare outcomes in infants with hypoplastic left heart syndrome: a systematic review protocol. (PubMed)

Effectiveness of interstage home monitoring on healthcare outcomes in infants with hypoplastic left heart syndrome: a systematic review protocol. What is the effectiveness of home monitoring on interstage survival rates, weight gain, hospital admissions and cost savings in infants with hypoplastic left heart syndrome?

2017 JBI database of systematic reviews and implementation reports

7. Extracorporeal Membrane Oxygenation-Supported Cardiopulmonary Resuscitation Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome

Extracorporeal Membrane Oxygenation-Supported Cardiopulmonary Resuscitation Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome PEDSCCM.org Criteria abstracted from series in Review Posted: founded 1995 Questions or comments?

2014 PedsCCM Evidence-Based Journal Club

8. Interrelationship between hemodynamics, brain volumes and outcome in Hypoplastic Left Heart Syndrome. (PubMed)

Interrelationship between hemodynamics, brain volumes and outcome in Hypoplastic Left Heart Syndrome. The long-term impact of altered hemodynamics after Stage II in children with hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC) on cerebral growth and neurodevelopmental outcome is unknown. We aimed to investigate whether elevated central venous and atrial filling pressures before Fontan procedure may be associated with smaller brain volumes and poorer

2019 Annals of Thoracic Surgery

9. Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum. (PubMed)

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum. Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe (...) procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.Copyright © 2018 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

2019 Mayo Clinic Proceedings

10. Smaller brain volumes at two years of age in patients with hypoplastic left heart syndrome - Impact of surgical approach. (PubMed)

Smaller brain volumes at two years of age in patients with hypoplastic left heart syndrome - Impact of surgical approach. Brain growth in hypoplastic left heart syndrome (HLHS) is reduced before and after birth. Little is known about further brain growth until two years of age before Fontan procedure and the potential impact of type of surgery.In a prospective, two-center study 29 patients with HLHS and variants were treated by Norwood (n = 5) or Hybrid procedure (n = 24). At two years of age

2019 International journal of cardiology

11. Outcomes of hypoplastic left heart syndrome and fetal aortic valvuloplasty in a country with suboptimal postnatal management. (PubMed)

Outcomes of hypoplastic left heart syndrome and fetal aortic valvuloplasty in a country with suboptimal postnatal management. Fetal aortic stenosis (AoS) may progress to hypoplastic left heart syndrome (HLHS) in utero. There are currently no data, pre- or postnatal, describing survival of fetuses or neonates with AoS or HLHS in a country with suboptimal postnatal management.Prospective cohort study performed in Mexico, including cases diagnosed with AoS and HLHS within a 6-year period. AoS

2019 Prenatal diagnosis

12. Hypoplastic left heart syndrome with anomalous circumflex artery arising from left pulmonary artery. A description of combined surgical and interventional cardiac treatment (PubMed)

Hypoplastic left heart syndrome with anomalous circumflex artery arising from left pulmonary artery. A description of combined surgical and interventional cardiac treatment 30302111 2018 11 14 1734-9338 14 3 2018 Postepy w kardiologii interwencyjnej = Advances in interventional cardiology Postepy Kardiol Interwencyjnej Hypoplastic left heart syndrome with anomalous circumflex artery arising from left pulmonary artery. A description of combined surgical and interventional cardiac treatment. 314

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2018 Postępy w kardiologii interwencyjnej = Advances in interventional cardiology

13. Necrotizing Enterocolitis in Infants with Hypoplastic Left Heart Syndrome Following Stage 1 Palliation or Heart Transplant. (PubMed)

Necrotizing Enterocolitis in Infants with Hypoplastic Left Heart Syndrome Following Stage 1 Palliation or Heart Transplant. Previous studies of necrotizing enterocolitis (NEC) among infants with hypoplastic left heart syndrome (HLHS) were conducted in single centers or had small sample sizes. This study aimed to determine the mortality rate and the risk factors for NEC among infants with HLHS who were discharged over a 10-year period (2004-2013) from 41 Pediatric Health Information System (...) affiliated children's hospitals. Either stage 1 palliation and/or heart transplant were completed prior to patient's death or hospital discharge. We compared the characteristics of infants with HLHS who did not develop NEC and those who developed medical or surgical NEC and of patients who had medical vs. surgical NEC. The primary outcome was mortality over time and by birth weight category (low birth weight [LBW], birth weight < 2500 vs. ≥ 2500 g). Multivariable analyses were performed to identify

2018 Pediatric Cardiology

14. Evolution of Left Ventricular Size in Late Survivors of Surgery for Hypoplastic Left Heart Syndrome. (PubMed)

Evolution of Left Ventricular Size in Late Survivors of Surgery for Hypoplastic Left Heart Syndrome. Aortic atresia and mitral atresia are predictors of adverse events in early life in patients with hypoplastic left heart syndrome, but it is unclear whether late outcomes are also affected, and whether this impact is related to a small hypertrophied left ventricle (LV) that impairs right ventricle (RV) function.Thirty patients with hypoplastic left heart syndrome surviving with a Fontan (...) to 57.7, p = 0.029).Patients with aortic atresia and mitral atresia have worse outcomes even after Fontan independently of a small, thick, hypertrophied LV. The most severely hypoplastic LVs do not grow proportionally as much as the RVs.Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

2017 Annals of Thoracic Surgery

15. Congenital diaphragmatic hernia with heart defect has a high risk for hypoplastic left heart syndrome and major extra-cardiac malformations - 10-year national cohort from Finland. (PubMed)

Congenital diaphragmatic hernia with heart defect has a high risk for hypoplastic left heart syndrome and major extra-cardiac malformations - 10-year national cohort from Finland. Congenital diaphragmatic hernia (CDH) has a well-known risk of congenital heart defects with poor prognosis. This study was conducted to determine the national total prevalence and prenatal detection rates of CDH with heart defects and its association with major extra-cardiac malformations and to further evaluate (...) was 41%. The total prevalence (483/10 000) and live birth prevalence (500/10 000) of hypoplastic left heart syndrome were 124 and 250 times higher than in the general population in Finland, respectively. Additional major extra-cardiac malformations were found in 68% of cases. The survival rate for CDH with major heart defects was 11 and 38% with minor heart defects.The total prevalence of hypoplastic left heart syndrome was significantly higher in CDH patients than in the general population

2017 Acta Obstetricia et Gynecologica Scandinavica

16. The Optimal Timing of Stage-2-Palliation for Hypoplastic Left Heart Syndrome: An analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Dataset. (PubMed)

The Optimal Timing of Stage-2-Palliation for Hypoplastic Left Heart Syndrome: An analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Dataset. In infants requiring 3-stage single-ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage 2 palliation (S2P), a physician-modifiable factor, on long-term survival is not well understood. We hypothesized that an optimal interval (...) between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics.The National Institutes of Health/National Heart, Lung, and Blood Institute Pediatric Heart Network Single Ventricle Reconstruction Trial public data set was used. Transplant-free survival (TFS) was modeled from (1) Norwood to S2P and (2) S2P to 3 years by using parametric hazard analysis. Factors associated with death or heart

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2017 Circulation

17. Hypoplastic Left Heart Syndrome with Congenital Complete Heart Block. (PubMed)

Hypoplastic Left Heart Syndrome with Congenital Complete Heart Block. Congenital complete atrioventricular block (CCAVB) is a rare condition with an incidence of 1 of 20,000 live births. Hypoplastic left heart syndrome (HLHS) occurs more frequently than CCAVB and occurs in 1 of 5000 live births. HLHS in association with CCAVB is exceedingly rare. In this report, we describe a rare case of HLHS and CCAVB diagnosed in utero. Postnatal diagnosis, management and outcome are presented as well

2017 Pediatric Cardiology

18. Variation in care for infants undergoing the Stage II palliation for hypoplastic left heart syndrome (PubMed)

Variation in care for infants undergoing the Stage II palliation for hypoplastic left heart syndrome The Single Ventricle Reconstruction trial randomised neonates with hypoplastic left heart syndrome to a systemic-to-pulmonary-artery shunt strategy. Patients received care according to usual institutional practice. We analysed practice variation at the Stage II surgery to attempt to identify areas for decreased variation and process control improvement.Prospectively collected data were available

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2018 Cardiology in the young

19. Impact of Hybrid Stage 1 Palliation for Hypoplastic Left Heart Syndrome: Histopathological Findings. (PubMed)

Impact of Hybrid Stage 1 Palliation for Hypoplastic Left Heart Syndrome: Histopathological Findings. The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (...) ; moreover, it was significantly higher in HS1P AA group than in primary group. Capillary vascular density was significantly lower in all hypoplastic left heart syndrome groups than in TAC group. At the sub-endocardial layer, collagen type I/III ratios were higher in HS1P AA group than in other hypoplastic left heart syndrome groups. The proportions of N-cadherin immunolocalized to myocyte termini were lower in all hypoplastic left heart syndrome groups than in TAC group. Right ventricle in hypoplastic

2018 Pediatric Cardiology

20. Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015. (PubMed)

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015. To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales.The UK's national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000

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2018 Heart

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