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

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

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.

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.

Catheter interventions in the staged management of hypoplastic left heart syndrome

2013 ◽  
Vol 24 (2) ◽  
pp. 212-219 ◽  
Author(s):  
Zdenka Reinhardt ◽  
Joseph De Giovanni ◽  
John Stickley ◽  
Vinay K. Bhole ◽  
Benjamin Anderson ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Left Pulmonary Artery ◽  
Stage Ii ◽  
Stage Iii ◽  
Catheter Intervention ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Fontan Palliation

AbstractAimTo analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome.MethodsThis study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.5 years.ResultsA staged Norwood/Fontan palliation for hypoplastic left heart syndrome was performed in 527 patients. The 30-day survival rate after individual stages was 76.5% at Stage I, 96.3% at Stage II, and 99.4% at Stage III. A total of 348 interventions were performed in 189 out of 527 patients. Freedom from catheter intervention in survivors was 58.2% before Stage II and 46.7% before Stage III. Kaplan–Meier freedom from intervention post Fontan completion was 55% at 10.8 years of follow-up. Post-stage I interventions were mostly directed to relieve aortic arch obstruction – 84 balloon angioplasties – and augment pulmonary blood flow – 15 right ventricle-to-pulmonary conduit interventions; post-Stage II interventions centred on augmenting size of the left pulmonary artery – 73 procedures and abolishing systemic venous collaterals – 32 procedures. After Stage III, the focus was on manipulating the size of the fenestration – 42 interventions – and the left pulmonary artery −31 procedures.ConclusionInterventional cardiac catheterisation constitutes an integral part in the staged palliative management of patients with hypoplastic left heart syndrome. Over one-third (37%) of patients undergoing staged palliation required catheter intervention over the follow-up period.

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