scholarly journals Reduced ascending aorta distensibility relates to adverse ventricular mechanics in patients with hypoplastic left heart syndrome: Noninvasive study using wave intensity analysis

2012 ◽  
Vol 144 (6) ◽  
pp. 1307-1314 ◽  
Author(s):  
Giovanni Biglino ◽  
Silvia Schievano ◽  
Jennifer A. Steeden ◽  
Hopewell Ntsinjana ◽  
Catriona Baker ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Ascending Aorta ◽  
Wave Intensity ◽  
Intensity Analysis ◽  
Left Heart ◽  
Wave Intensity Analysis ◽  
Hypoplastic Left Heart ◽  
Ventricular Mechanics ◽  
Left Heart Syndrome

A Non-Invasive Study Using MR-Derived Wave Intensity Analysis to Highlight the Effect of Surgical Arch Reconstruction on Ventriculo-Arterial Coupling in Patients With Hypoplastic Left Heart Syndrome

2012 ◽  
Author(s):  
Giovanni Biglino ◽  
Hopewell Ntsinjana ◽  
Jennifer A. Steeden ◽  
Catriona Baker ◽  
Silvia Schievano ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Hypoplastic Left Heart Syndrome ◽  
Surgical Reconstruction ◽  
Intensity Analysis ◽  
Left Heart ◽  
Wave Intensity Analysis ◽  
Mr Images ◽  
Hypoplastic Left Heart ◽  
Non Invasive ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a congenital heart disease whose staged surgical palliation aims to progressively separate the systemic and pulmonary circulations. The first stage or Norwood procedure [1] involves surgical reconstruction of the aortic arch, usually with pulmonary homograft patch [2]. Recent evidence suggested that, because of this extensive reconstruction, HLHS patients have abnormal elastic properties [3] and reduced distensibility [4,5] of the ascending aorta. However, the impact of the reconstructed aorta and its abnormal elastic properties on ventricular mechanics, i.e. ventriculo-arterial coupling mismatch, has not been assessed. In the light of this mismatch, a change in impedance on the arterial side will reflect on the ventricular side and quantification of this phenomenon may provide mechano-energetic information for further understanding a complex physiology such as palliated HLHS with aortic arch surgical reconstruction. In this study we suggest that wave intensity analysis (WIA) is a valid method for studying ventriculo-arterial coupling, as WIA is a hemodynamic index able to assess the performance of the heart and its interaction with arterial system. Previously, distensibility quantification necessitated either of invasive [5] or cuff [4] arterial pressure monitoring, or multiple magnetic resonance (MR) images acquisitions for transit time wave speed calculation [6]. Instead, here we propose a non-invasive and semi-automated method based only on MR images analysis, with single slice analysis for estimate of local distensibility. The method, including WIA, was developed as a plug-in for DICOM viewer OsiriX, and applied to two cohorts of single ventricle patients.

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.

Acute ischaemic heart block in hypoplastic left heart syndrome

2014 ◽  
Vol 26 (1) ◽  
pp. 151-154
Author(s):  
Dale A. Burkett ◽  
Neil Wilson ◽  
Max B. Mitchell ◽  
Adel K. Younoszai
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Heart Block ◽  
Ascending Aorta ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Complex Cases

AbstractIn hypoplastic left heart syndrome, thrombosis of the native ascending aorta is rare and often fatal; there are no previously reported cases presenting with acute heart block. We review a case of native ascending aorta thrombosis in a 2-year-old boy with hypoplastic left heart syndrome, presenting with acute heart block. This case highlights the benefit of multi-modality imaging in complex cases.

scholarly journals NovelSMAD3Mutation in a Patient with Hypoplastic Left Heart Syndrome with Significant Aortic Aneurysm

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Kristi K. Fitzgerald ◽  
Abdul Majeed Bhat ◽  
Katrina Conard ◽  
James Hyland ◽  
Christian Pizarro
Keyword(s):  
Aortic Aneurysm ◽  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart ◽  
Pathogenic Mutation ◽  
Ascending Aorta ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
First Case ◽  
Aortic Aneurysm And Dissection ◽  
Left Heart Syndrome

Aneurysms-osteoarthritis syndrome (AOS) caused by haploinsufficiency ofSMAD3is a recently described cause of syndromic familial thoracic aortic aneurysm and dissection (TAAD). We identified a novelSMAD3mutation in a patient with hypoplastic left heart syndrome (HLHS) who developed progressive aortic aneurysm requiring surgical replacement of the neoaortic root, ascending aorta, and proximal aortic arch. Family screening for the mutation revealed that his father, who has vascular and skeletal features of AOS, and his brother, who is asymptomatic, also have the pathogenic mutation. This is the first case report of aSMAD3mutation in a patient with hypoplastic left heart syndrome. This case highlights the importance of genetic testing for known causes of aneurysm in patients with congenital heart disease who develop aneurysmal disease as it may significantly impact the management of those patients and their family members.

scholarly journals Aortic morphometry and microcephaly in hypoplastic left heart syndrome

2007 ◽  
Vol 17 (2) ◽  
pp. 189-195 ◽  
Author(s):  
Amanda J. Shillingford ◽  
Richard F. Ittenbach ◽  
Bradley S. Marino ◽  
Jack Rychik ◽  
Robert R. Clancy ◽  
...  
Keyword(s):  
Birth Weight ◽  
Aortic Arch ◽  
Hypoplastic Left Heart Syndrome ◽  
Ascending Aorta ◽  
Full Term ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Neurodevelopmental Outcomes ◽  
Term Infants ◽  
Left Heart Syndrome

Microcephaly is a marker of abnormal fetal cerebral development, and a known risk factor for cognitive dysfunction. Patients with hypoplastic left heart syndrome have been found to have an increased incidence of abnormal neurodevelopmental outcomes. We hypothesized that reduced cerebral blood flow from the diminutive ascending aorta and transverse aortic arch in the setting of hypoplastic left heart syndrome may influence fetal growth of the brain. The purpose of our study, therefore, was to define the prevalence of microcephaly in full-term infants with hypoplastic left heart syndrome, and to investigate potential cardiac risk factors for microcephaly. We carried out a retrospective review of full-term neonates with hypoplastic left heart syndrome. Eligible patients had documented indexes of birth weight, and measurements of length, and head circumference, as well as adequate echocardiographic images for measurement of the diameters of the ascending aorta and transverse aortic arch. We used logistic regression for analysis of the data. A total of 129 neonates met the criterions for inclusion, with 15 (12%) proving to have microcephaly. The sizes of their heads were disproportionately smaller than their weights (p less than 0.001) and lengths (p less than 0.001) at birth. Microcephaly was associated with lower birth weight (p less than 0.001), lower birth length (p equal to 0.007), and a smaller diameter of the ascending aorta (p equal to 0.034), but not a smaller transverse aortic arch (p equal to 0.619), or aortic atresia (p equal to 0.969). We conclude that microcephaly was common in this cohort of neonates with hypoplastic left heart syndrome, with the size of the head being disproportionately smaller than weight and length at birth. Microcephaly was associated with a small ascending aorta, but not a small transverse aortic arch. Impairment of somatic growth may be an additional factor in the development of microcephaly in these neonates.

Right Ventricular Mechanics in the Fetus with Hypoplastic Left Heart Syndrome

2013 ◽  
Vol 26 (5) ◽  
pp. 515-520 ◽  
Author(s):  
Shobha Natarajan ◽  
Anita Szwast ◽  
Zhiyun Tian ◽  
Margaret McCann ◽  
Debbra Soffer ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Ventricular Mechanics ◽  
Left Heart Syndrome
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