Abnormal aortic arch shape and vortical flow patterns are associated with descending aortic dilatation in patients with hypoplastic 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.

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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

ASME 2012 Summer Bioengineering Conference, Parts A and B ◽

10.1115/sbc2012-80262 ◽

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.

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Aortic Arch Obstruction After the Norwood Procedure for Hypoplastic Left-Heart Syndrome

Journal of the American College of Cardiology ◽

10.1016/j.jacc.2011.03.050 ◽

2011 ◽

Vol 58 (8) ◽

pp. 877-879 ◽

Cited By ~ 4

Author(s):

John J. Lamberti

Keyword(s):

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Norwood Procedure ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Arch Obstruction ◽

Left Heart Syndrome

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The Retrograde Aortic Arch in the Hybrid Approach to Hypoplastic Left Heart Syndrome

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2009.06.115 ◽

2009 ◽

Vol 88 (6) ◽

pp. 1939-1947 ◽

Cited By ~ 54

Author(s):

Serban C. Stoica ◽

Alistair B. Philips ◽

Matthew Egan ◽

Roberta Rodeman ◽

Joanne Chisolm ◽

...

Keyword(s):

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Hybrid Approach ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

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Arch intervention following stage 1 palliation in hypoplastic left heart syndrome is associated with slower feed advancement: a report from the National Pediatric Quality Cardiology Improvement Collaborative

Cardiology in the Young ◽

10.1017/s1047951120000177 ◽

2020 ◽

Vol 30 (3) ◽

pp. 396-401

Author(s):

Alicia H. Chaves ◽

Carissa M. Baker-Smith ◽

Geoffrey L. Rosenthal

Keyword(s):

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Left Heart ◽

Feeding Problems ◽

Hypoplastic Left Heart ◽

Feeding Difficulties ◽

Arch Obstruction ◽

Z Scores ◽

Stage 1 ◽

Left Heart Syndrome

AbstractIntroduction:Infants undergoing stage 1 palliation for hypoplastic left heart syndrome may have post-operative feeding difficulties. Although the cause of feeding difficulties in these patients is multi-factorial, residual arch obstruction may affect gut perfusion, contributing to feeding intolerance. We hypothesised that undergoing arch reintervention following stage 1 palliation would be associated with post-operative feeding difficulties.Methods:This was a retrospective cohort study. We analysed data from the National Pediatric Cardiology Quality Improvement Collaborative, which maintains a multicentre registry for infants with hypoplastic left heart syndrome discharged home following stage 1 palliation. Patients who underwent arch reintervention (percutaneous or surgical) prior to discharge following stage 1 palliation were compared with those who underwent non-aortic arch interventions after stage 1 palliation and those who underwent no intervention. Median post-operative days to full enteral feeds and weight for age z-scores were compared. Predictors of post-operative days to full feeds were identified.Results:Among patients who underwent arch reintervention, post-operative days to full enteral feeds were greater than for those who underwent non-aortic arch interventions (25 versus 16, p = 0.003) or no intervention (median days 25 versus 12, p < 0.001). Arch intervention, multiple interventions, gestational age, and the presence of a gastrointestinal anomaly were predictors of days to full feeds.Conclusions:Repeat arch intervention is associated with a longer time to achieve full enteral feeding in patients with hypoplastic left heart syndrome after stage 1 palliation. Further investigation of this association is needed to understand the role of arch obstruction in feeding problems in these patients.

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Neonatal hemodynamics in patients with hypoplastic left heart syndrome

Cardiology in the Young ◽

10.1017/s1047951104006250 ◽

2004 ◽

Vol 14 (S1) ◽

pp. 22-26

Author(s):

James C. Huhta

Keyword(s):

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Venous Return ◽

Left Heart ◽

Genetic Abnormality ◽

Hypoplastic Left Heart ◽

Doppler Evaluation ◽

Cardiac Diagnosis ◽

Left Heart Syndrome ◽

Rule Out

The neonate with hypoplastic left heart syndrome presents a challenge for clinical diagnosis and management. Three diagnostic goals must be met. First, it is necessary to make an etiologic cardiac diagnosis so as to rule out any genetic abnormality. Second, the anatomic cardiac diagnosis is made by segmental echocardiographic analysis, including details of the atrial arrangement, venous return, the patency of the arterial duct, atrial anatomy, and the arrangement of the aortic arch. Finally, the physiologic cardiac diagnosis is made by Doppler evaluation. In some patients, the diagnosis of hypoplastic left heart syndrome is not synonymous with functionally univentricular physiology, and a bi-ventricular repair can be achieved.1

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Incidence of and Risk Factors for Aortic Arch Interventions After the Comprehensive Stage II Procedure for Hypoplastic Left Heart Syndrome

Pediatric Cardiology ◽

10.1007/s00246-021-02739-2 ◽

2021 ◽

Author(s):

Annaka Gilmore ◽

Jo Ann Davis ◽

Samantha Low ◽

Joanne Chisolm ◽

John Kelly ◽

...

Keyword(s):

Risk Factors ◽

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Stage Ii ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome

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Early outcomes and computational fluid dynamic analyses of chimney reconstruction in the Norwood procedure

Interactive CardioVascular and Thoracic Surgery ◽

10.1093/icvts/ivz040 ◽

2019 ◽

Vol 29 (2) ◽

pp. 252-259 ◽

Cited By ~ 2

Author(s):

Satoshi Asada ◽

Masaaki Yamagishi ◽

Keiichi Itatani ◽

Yoshinobu Maeda ◽

Satoshi Taniguchi ◽

...

Keyword(s):

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

Fluid Dynamic ◽

Norwood Procedure ◽

Patient Specific ◽

Shear Stresses ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Flow Profiles ◽

Left Heart Syndrome

Abstract OBJECTIVES The ideal configuration of a reconstructed aortic arch in the Norwood procedure for hypoplastic left heart syndrome is still a matter of debate. Chimney reconstruction was developed to avoid postoperative complications and turbulent flow in the aortic arch. This study sought to clarify early outcomes of the procedure and verify its haemodynamic advantages using computational fluid dynamics (CFD). METHODS Fourteen consecutive patients with hypoplastic left heart syndrome or a variant who underwent chimney reconstruction in the Norwood procedure between January 2013 and March 2018 were enrolled. Median age and body weight at the time of operation were 2.5 months and 4.1 kg, respectively. Thirteen patients (93.9%) had been palliated with previous bilateral pulmonary artery (PA) banding. In addition, patient-specific CFD models of neoarches based on postoperative computed tomograms from 6 patients were created and the flow profiles analysed. RESULTS Survival rates at 1, 3 and 5 years were 76.6%, 67.3% and 67.3%, respectively. No patient developed left PA compression by neoaorta, neoaortic dilation or neoaortic insufficiency. Only 2 patients (14.3%) required surgical intervention for recoarctation. Fontan completion was performed on 5 patients. On CFD analysis, all reconstructed aortic arches showed low energy loss (9.16–14.4 mW/m2) and low wall shear stresses. CONCLUSIONS Chimney reconstruction was a feasible technique when homografts were not readily available. CFD analyses underscored the fact that this technique produced excellent flow profiles. Larger studies should be conducted to clarify long-term outcomes.

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