Assessment of Left Ventricular Endocardial Fibroelastosis in Fetuses With Aortic Stenosis and Evolving Hypoplastic Left Heart Syndrome

2010 ◽  
Vol 106 (12) ◽  
pp. 1792-1797 ◽  
Author(s):  
Doff B. McElhinney ◽  
Melanie Vogel ◽  
Carol B. Benson ◽  
Audrey C. Marshall ◽  
Louise E. Wilkins-Haug ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Ventricular ◽  
Left Heart ◽  
Endocardial Fibroelastosis ◽  
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 1212 LEFT VENTRICULAR AND CORONARY ARTERY MORPHOLOGY IN THE HYPOPLASTIC LEFT HEART SYNDROME

1981 ◽  
Vol 15 ◽  
pp. 644-644
Author(s):  
Willian O'Connor ◽  
James Cash ◽  
Carol Cottrill ◽  
Gregory Johnson ◽  
Jacqueline A Noonan
Keyword(s):  
Coronary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Ventricular ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
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.

scholarly journals Assessment of Structural and Functional Abnormalities of the Myocardium and the Ascending Aorta in Fetus with Hypoplastic Left Heart Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-9
Author(s):  
Yan Jiang ◽  
Yali Xu ◽  
Jinliang Tang ◽  
Hongmei Xia
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Left Ventricular ◽  
Ascending Aorta ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Histological Changes ◽  
Left Heart Syndrome ◽  
Rv Function ◽  
Normal Controls ◽  
Pulmonary Artery Diameter

Aims. To detect anatomical and intrinsic histopathological features of the ascending aorta and left ventricular (LV) myocardium and evaluate right ventricular (RV) function in fetuses with hypoplastic left heart syndrome (HLHS).Methods. Twenty-five fetuses diagnosed with HLHS were followed up in the antenatal and postpartum periods. 12 necropsy heart specimens were analyzed for morphological and histological changes.Results. Prenatal echocardiography and pathologic anatomy displayed the typical characteristics of HLHS as a severe underdevelopment of the LV in the form of mitral stenosis or atresia or as aortic atresia or stenosis, with a decreased ratio of aortic diameter to pulmonary artery diameter (median of 0.49 with a range of 0.24 to 0.69,p≤0.001) and a higher ratio of RV diameter to LV diameter (median of 2.44 with a range of 1.33 to 6.25,p≤0.001). The RV volume, stroke volume, and cardiac output in HLHS fetuses were increased compared with the gestational age-matched normal controls (p<0.01). Histological changes in the 12 HLHS specimens included LV myocardial fibrosis, aortic elastic fragmentation, and fibrosis.Conclusions. In addition to severe anatomical deformity, distinct histological abnormalities in the LV myocardium and aortic wall were identified in the fetuses with HLHS. RV function damage may be potentially exists.

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