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.

Abstract 19315: Geometry, Reintervention, and Growth Patterns in the Reconstructed Aortic Arch With Interdigitating Technique in Patients With Hypoplastic Left Heart Syndrome and Variants

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Christoph Haller ◽  
Devin Chetan ◽  
Matthew Doyle ◽  
Arezou Saedi ◽  
Rachel Parker ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Ventricular Function ◽  
Hypoplastic Left Heart Syndrome ◽  
Growth Characteristics ◽  
Stage Ii ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Z Scores ◽  
Arch Reconstruction ◽  
Left Heart Syndrome

Objectives: The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) is very effective to minimize the recoarctation rate. Little is known on the aortic arch’s growth characteristics and the resulting clinical impact. Methods: 139 patients with HLHS underwent staged palliation between 2007 and 2014. 72 patients who underwent Norwood arch reconstruction with the interdigitating technique were included. Dimensions of the ascending aorta (AA), transverse arch (TA), isthmus (IA) and descending aorta (DA) in pre-stage II (P1, n=50) and pre-Fontan (P2, n=21) angiograms were measured and geometry and growth characteristics of the aortic arches were analyzed. Correlations between the aortic dimensions and clinical outcomes were assessed. Results: There were significant increases in diameters in all segments between P1 and P2 (p < .0005). The z-scores in AA, TA and IA were unchanged between P1 and P2 (p = .931/.425/.121), but increased significantly in DA at P2 (p = .039). The percent increase in diameters were comparable among 4 segments (mean, 146% in IA, 144 in DA, p=.648). There were correlations in dimensions and z-scores between P1 and P2 in AA (p = .029/.013) and TA (p = .001/ < .0005), but no correlations were found in IA (p = .140/.747) and DA (p = .075/.432). The most significant tapering in the arch dimension occurred between TA and IA in both time points (P1, 67.3% vs. P2, 61.1%, p=.303). The reverse coarctation index (TA/IA ratio) at P1 (r = .381, p = .042), but not coarctation index (CoAI, IA/DA ratio) at P1 (p = .774) had a significant correlation with post-stage II ventricular function. Balloon dilatation for recoarctation was needed in 2 (2.7%) patients prior to stage II palliation. CoAI at P1 was a predictor for ventricular dysfunction at latest follow-up (p=.017). Conclusions: Aortic arch growth after interdigitating reconstruction in HLHS is substantial and relatively constant. The isthmus growth is proportional to other segments. Overall reintervention rate for recoarctation is exceptionally low. CoAI prior to stage II palliation may be associated with long-term ventricular function.

Outcomes of Hypoplastic Left Heart Syndrome in Low-Birth-Weight Patients

2011 ◽  
Vol 32 (8) ◽  
pp. 1175-1181 ◽  
Author(s):  
Sarah Gelehrter ◽  
Carlen G. Fifer ◽  
Aimee Armstrong ◽  
Jennifer Hirsch ◽  
Robert Gajarski
Keyword(s):  
Birth Weight ◽  
Low Birth Weight ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

Neurodevelopmental Outcomes After Staged Palliation for Hypoplastic Left Heart Syndrome

2008 ◽  
Vol 63 (7) ◽  
pp. 419-421 ◽  
Author(s):  
Sarah Tabbutt ◽  
Alex S. Nord ◽  
Gail P. Jarvik ◽  
Judy Bernbaum ◽  
Gil Wernovsky ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Neurodevelopmental Outcomes ◽  
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 Neurodevelopment and quality of life for children with hypoplastic left heart syndrome: current knowns and unknowns

2011 ◽  
Vol 21 (S2) ◽  
pp. 88-92 ◽  
Author(s):  
Caren S. Goldberg ◽  
Kathleen Mussatto ◽  
Daniel Licht ◽  
Gil Wernovsky
Keyword(s):  
Quality Of Life ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Neurodevelopmental Outcomes ◽  
Current State ◽  
Left Heart Syndrome

AbstractThe aim of this review is to describe the current state of knowledge related to neurodevelopmental outcomes and quality of life for children with hypoplastic left heart syndrome and to explore future questions to be answered for this group of children.

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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