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.

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 Improvement in right ventricular function by mitral valve closure in hypoplastic left heart syndrome

2021 ◽  
Author(s):  
Dai Asada ◽  
Yoko Kawai ◽  
Yoshinobu Maeda ◽  
Masaaki Yamagishi
Keyword(s):  
Mitral Valve ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Hypoplastic Left Heart Syndrome ◽  
Right Ventricular Function ◽  
Left Ventricular ◽  
Valve Closure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Abstract A male neonate presented with the aortic/mitral stenotic variant of hypoplastic left heart syndrome, wherein the suprasystemic left ventricular pressure and relatively large left ventricle had shifted the intraventricular septum. Despite bilateral pulmonary artery banding, the stroke volume was difficult to maintain owing to the compressed right ventricle, causing heart failure symptoms. Percutaneous balloon aortic valvuloplasty decreased the left ventricular pressure, restoring the right ventricular function. Norwood procedure with mitral valve closure after catheter intervention reduced the left ventricular size and improved the right ventricular function. This paper refers to the potential of mitral valve closure for hypoplastic left heart syndrome.

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

Tricuspid Valve and Right Ventricular Function Throughout the Hybrid Palliation Strategy for Hypoplastic Left Heart Syndrome and Variants

2020 ◽  
pp. 215013512094769
Author(s):  
Sergio A. Carrillo ◽  
Karen M. Texter ◽  
Christina Phelps ◽  
Yubo Tan ◽  
Patrick I. McConnell ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Tricuspid Valve ◽  
Multiple Time ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Risk Of Death ◽  
Left Heart Syndrome ◽  
Rv Function ◽  
Hybrid Palliation

Background: Tricuspid valve (TV) and right ventricular (RV) function are major determinants of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS). We sought to retrospectively evaluate these parameters throughout the hybrid palliation strategy. Methods: From 2002 to 2018, 203 patients with HLHS and variants presented for hybrid stage I (HS1). Echocardiographic evaluation of tricuspid regurgitation (TR) and RV function was assessed at multiple time points. Clinical outcomes including tricuspid valvuloplasty, transplantation, and death were reviewed. Results: The most prevalent HLHS subtype was aortic atresia/mitral atresia. The presence of significant TR and/or RV dysfunction was 14.78% and 9.36%, respectively, at the time of initial HS1. There were 185 survivors following HS1 (91.13%, n = 185/203), while 147 patients underwent comprehensive stage II or bidirectional Glenn shunt (72.41%, n = 147/203). Tricuspid valvuloplasty was undertaken in nine patients (4.86%, n = 9/185). Ultimately, 100 patients underwent the Fontan procedure. The odds of development of significant TR and/or RV dysfunction were not statistically different throughout the stages of palliation (TR: odds ratio [OR] = 0.14-0.25, P = .5260; RV dysfunction: OR = 0.02-0.13, P = .3992). However, the risk of death and/or transplant was 2.5- to 3.8-fold when either were present alone or in combination (TR: OR = 2.58, P = .0356; RV dysfunction: OR = 3.84, P = .0262). Transplant-free survival at 15 years was 44.8%. Conclusion: Following hybrid palliation for HLHS, the majority of survivors have normal RV and TV functions. Tricuspid valvuloplasty was required in few patients. Once significant TR and/or RV dysfunction ensues, there is a two- to three-fold risk of death and/or transplant.

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