scholarly journals Hypoplastic left heart syndrome with parchment left ventricle: A rare perinatal autopsy case report

2015 ◽  
Vol 1 (2) ◽  
pp. 26-28
Author(s):  
K. R. Chatura ◽  
G. V. Neethu ◽  
K. S. Mavintop
Keyword(s):  
Case Report ◽  
Left Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Autopsy Case ◽  
Hypoplastic Left Heart ◽  
Perinatal Autopsy ◽  
Left Heart Syndrome

scholarly journals Distention of the Immature Left Ventricle Triggers Development of Endocardial Fibroelastosis: An Animal Model of Endocardial Fibroelastosis Introducing Morphopathological Features of Evolving Fetal Hypoplastic Left Heart Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Shogo Shimada ◽  
Christian Robles ◽  
Ben M. W. Illigens ◽  
Alejandra M. Casar Berazaluce ◽  
Pedro J. del Nido ◽  
...  
Keyword(s):  
Animal Model ◽  
Left Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Endocardial Fibroelastosis ◽  
Hypoplastic Left Heart ◽  
Fetal Imaging ◽  
Adult Rat ◽  
Human Fetal Heart ◽  
Left Heart Syndrome

Background.Endocardial fibroelastosis (EFE), characterized by a diffuse endocardial thickening through collagen and elastin fibers, develops in the human fetal heart restricting growth of the left ventricle (LV). Recent advances in fetal imaging indicate that EFE development is directly associated with a distended, poorly contractile LV in evolving hypoplastic left heart syndrome (HLHS). In this study, we developed an animal model of EFE by introducing this human fetal LV morphopathology to an immature rat heart.Methods and Results.A neonatal donor heart, in which aortic regurgitation (AR) was created, was heterotopically transplanted into a recipient adult rat. AR successfully induced the LV morphology of evolving HLHS in the transplanted donor hearts, which resulted in the development of significant EFE covering the entire LV cavity within two weeks postoperatively. In contrast, posttransplants with a competent aortic valve displayed unloaded LVs with a trace of EFE.Conclusions.We could show that distention of the immature LV in combination with stagnant flow triggers EFE development in this animal model. This model would serve as a robust tool to develop therapeutic strategies to treat EFE while providing insight into its pathogenesis.

Abstract 238: Single-Cell Transcriptomic Analysis and Patient-Specific IPSCs Reveal Dysregulated Cell Cycle in Coronary Endothelial Cell in Hypoplastic Left Heart Syndrome

2020 ◽  
Vol 127 (Suppl_1) ◽  
Author(s):  
Mingxia Gu ◽  
Yifei Miao ◽  
Xin Zhou ◽  
Lei Tian ◽  
Marcy Martin ◽  
...  
Keyword(s):  
Cell Cycle ◽  
Endothelial Cell ◽  
Left Ventricle ◽  
Single Cell ◽  
Hypoplastic Left Heart Syndrome ◽  
Human Heart ◽  
G1 Phase ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a single ventricle congenital heart disease that results in severe underdevelopment of the left ventricle, mitral valve, aortic valve, and ascending aorta. Early serial postmortem examinations also revealed a high rate of coronary anomalies in HLHS, which included multiple ventriculo-coronary arterial connections as well as thick-walled and kinked coronary arteries. A previous study showed that fetal hypoplastic left hearts had a reduced endothelial cell (EC) population and lower capillary density compared with normal hearts. However, the mechanism underlying coronary abnormalities associated with HLHS remains unknown. Thus, we generated induced pluripotent stem cells derived ECs (iPSC-ECs) from three HLHS patients and three age-matched controls. Single Cell RNA-Seq (scRNA-seq) profiling identified both endocardial (NPR3 + /CDH5 + ) and coronary endothelial populations (APLN + /CDH5 + ) from the heterogeneous iPSC-ECs. Intriguingly, a subcluster of the coronary endothelial cells (CECs) with cell cycle arrest was specifically enriched in HLHS patients. Further cell cycle analysis showed that 30.6% of the HLHS cells were trapped in the G1 phase, while the majority of the control CECs entered cell cycle normally. Additionally, the cell cycle differences between control and HLHS was only seen in CECs, not in the endocardial population. To verify our transcriptomic analysis, we applied negative cell sorting (NPR3 - /CDH5 + ) on iPSC-ECs to purify CECs (iCECs) and confirmed that HLHS iCECs showed profound reduction of cell cycle/proliferative genes ( KI67, PCNA, CCNA2, CCNB1 ) and abnormal induction of CCND2 , which is the hallmark of G1 phase. BrdU assays also indicated suppressed proliferation in HLHS iCECs. Furthermore, we profiled the transcriptome from a human heart with an underdevelopment left ventricle (ULV) at single cell resolution. When compared to the normal human heart, pathway enrichment analysis of differentially expressed genes in ULV hearts demonstrated reduced cell proliferation in the CEC subpopulation. Here, we identified that CECs from HLHS patients exerted proliferative defects that can potentially impede the development of vascular/capillary structure and cause related functional deficiencies. Reformation of the coronary defect provides a promising therapeutic strategy to prevent HLHS deterioration.

Comparison of right ventricular deformation and dyssynchrony in patients with different subtypes of hypoplastic left heart syndrome after Fontan surgery using two-dimensional speckle tracking

2011 ◽  
Vol 21 (6) ◽  
pp. 677-683 ◽  
Author(s):  
Colin Petko ◽  
Inga Voges ◽  
Jana Schlangen ◽  
Jens Scheewe ◽  
Hans-Heiner Kramer ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Speckle Tracking ◽  
Two Dimensional ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Intraventricular Dyssynchrony ◽  
Fontan Surgery ◽  
Left Heart Syndrome

AbstractBackgroundThe left ventricle in patients with hypoplastic left heart syndrome may influence right ventricular function and outcome. We aimed to investigate differences in right ventricular deformation and intraventricular dyssynchrony between hypoplastic left heart syndrome patients with different anatomical subtypes and left ventricle sizes after Fontan surgery using two-dimensional speckle tracking.Patients and methodsWe examined 29 hypoplastic left heart syndrome patients aged 5.4 plus or minus 2.8 years after Fontan surgery and compared 15 patients with mitral and aortic atresia with the remaining 14 patients with other anatomic subtypes. We used two-dimensional speckle tracking to measure the global and regional systolic longitudinal strain and strain rate as well as intraventricular dyssynchrony.ResultsGlobal strain (−19.5, 2.8% versus −17.4, 3.9%) and global strain rate (−1.0, 0.2 per second versus −0.9, 0.3 per second) were not different between groups. The mitral and aortic atresia group had higher strain in the basal septal (−13.0, 5.0% versus −3.9, 9.3%, p = 0.003) and mid-septal (−19.4, 4.7% versus −13.2, 6.5%, p = 0.009) segments, and higher strain rates in the mid-septal segment (−1.14, 0.3 per second versus −0.95, 0.4 per second, p = 0.047), smaller left ventricle area (0.18, 0.41 square centimetre versus 2.83, 2.07 square centimetre, p = 0.0001), and shorter wall-to-wall delay (38, 29 milliseconds versus 81, 57 milliseconds, p = 0.02).ConclusionSignificant differences in regional deformation and intraventricular dyssynchrony exist between the mitral and aortic atresia subtype with small left ventricles and the other anatomic subtypes with larger left ventricles after Fontan surgery.

scholarly journals Double shunt technique for hybrid palliation of hypoplastic left heart syndrome: a case report

2011 ◽  
Vol 6 (1) ◽  
Author(s):  
Marcelo Biscegli Jatene ◽  
Patrícia M Oliveira ◽  
Rafael A Moysés ◽  
Ieda Biscegli Jatene ◽  
Carlos A Pedra ◽  
...  
Keyword(s):  
Case Report ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Hybrid Palliation

First-Trimester Diagnosis of Hypoplastic Left Heart Syndrome: A Case Report

2021 ◽  
Vol 37 (2) ◽  
pp. 185-192
Author(s):  
Emily Hildebrand
Keyword(s):  
Case Report ◽  
Mitral Valve ◽  
Aortic Valve ◽  
Early Detection ◽  
Hypoplastic Left Heart Syndrome ◽  
First Trimester ◽  
Cardiac Anomaly ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) comprises a spectrum of left-sided heart anomalies resulting from left outflow or inflow obstruction. Obstruction most often occurs at the levels of the aortic valve and/or mitral valve due to stenosis or atresia. HLHS is a lethal cardiac anomaly if not treated within the first week of life. Knowledge of sonographic features could aid in early detection, which results in better planning and management of pregnancy. This report presents a case in which HLHS was discovered during a routine dating and viability sonogram, performed at 12 weeks’ gestation.

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