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Atrial septal defect morphology and stenting in hypoplastic left heart syndrome after hybrid palliation

Published online by Cambridge University Press:  11 September 2017

Lilia Oreto ,
Giuseppe Mandraffino ,
Lucia Manuri ,
Michele B. Saitta ,
Salvatore Agati ,
Concetta Zito ,
Fiore S. Iorio ,
Scipione Carerj  and
Paolo Guccione
Lilia Oreto*
Affiliation:
Bambino Gesù Pediatric Hospital, Mediterranean Pediatric Cardiology Center, Taormina, Messina, Italy Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
Giuseppe Mandraffino
Affiliation:
Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
Lucia Manuri
Affiliation:
Bambino Gesù Pediatric Hospital, Mediterranean Pediatric Cardiology Center, Taormina, Messina, Italy
Michele B. Saitta
Affiliation:
Bambino Gesù Pediatric Hospital, Mediterranean Pediatric Cardiology Center, Taormina, Messina, Italy
Salvatore Agati
Affiliation:
Bambino Gesù Pediatric Hospital, Mediterranean Pediatric Cardiology Center, Taormina, Messina, Italy
Concetta Zito
Affiliation:
Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
Fiore S. Iorio
Affiliation:
Bambino Gesù Pediatric Hospital, Medical and Surgical Department of Pediatric Cardiology, Rome, Italy
Scipione Carerj
Affiliation:
Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
Paolo Guccione
Affiliation:
Bambino Gesù Pediatric Hospital, Medical and Surgical Department of Pediatric Cardiology, Rome, Italy
*
Correspondence to: L. Oreto, MD, Department of Clinical and Experimental Medicine, Cardiology, University Hospital, Via Consolare Valeria 100, Messina 98100, Italy. Tel: +39 090 2212 969; Fax: +390909572363; E-mail: liliaoreto@hotmail.com
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Abstract

Aims

The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect.

Methods and results

From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension. The mean gradient was then measured invasively. Restrictive defect occurred in 11/27 patients (40%). The restrictive group showed three ostium secundum defects (27%) and eight complex morphologies (73%). Conversely, in the non-restrictive group, we observed 11 ostium secundum defects (69%) and five complex morphologies (31%). Early balloon atrioseptostomy was required in three cases. Late restriction occurred in eight patients and was dealt with balloon dilation, stenting, or atrioseptectomy. There was no significant difference between restrictive and non-restrictive groups in terms of early or 12-month survival.

Conclusions

Complex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.

Keywords

Restrictive atrial septal defectstentinghypoplastic left heart syndromehybrid palliationmalalignement atrial septal defect
Type
Original Articles
Information
Cardiology in the Young , Volume 28 , Issue 2 , February 2018 , pp. 252 - 260
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Copyright
© Cambridge University Press 2017 

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References

1. Feinstein, JA, Benson, DW, Dubin, AM, et al. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 2012; 59: 544.Google Scholar
2. Emani, SM, del Nido, PJ. Strategies to maintain biventricular circulation in patients with high-risk anatomy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2013; 16: 37–42.Google Scholar
3. Hickey, EJ, Caldarone, CA, McCrindle, BW. Left ventricular hypoplasia: a spectrum of disease involving the left ventricular outflow tract, aortic valve, and aorta. J Am Coll Cardiol 2012; 59: S43–S54.Google Scholar
4. Chin, AJ, Weinberg, PM, Barber, G. Subcostal two-dimensional echocardiographic identification of anomalous attachment of septum primum in patients with left atrioventricular valve underdevelopment. J Am Coll Cardiol 1990; 15: 678–681.CrossRefGoogle ScholarPubMed
5. Park, MV, Fedderly, RT, Frommelt, PC, et al. Leftward displacement of septum primum in hypoplastic left heart syndrome. Pediatr Cardiol 2013; 34: 942–947.Google Scholar
6. Hoque, T, Richmond, M, Vincent, JA, Bacha, E, Torres, A. Current outcomes of hypoplastic left heart syndrome with restrictive atrial septum: a single-center experience. Pediatr Cardiol 2013; 34: 1181–1189.Google Scholar
7. Rychik, J, Rome, JJ, Collins, MH, DeCampli, WM, Spray, TL. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 1999; 34: 554–560.Google Scholar
8. Tchervenkov, CI, Jacobs, JP, Weinberg, PM, et al. The nomenclature, definition and classification of hypoplastic left heart syndrome. Cardiol Young 2006; 16: 339–368.Google Scholar
9. Holzer, RJ, Wood, A, Chisolm, JL, et al. Atrial septal interventions in patients with hypoplastic left heart syndrome. Catheter Cardiovasc Interv 2008; 72: 696–704.Google Scholar
10. Vida, VL, Bacha, EA, Larrazabal, A, et al. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center. Ann Thorac Surg 2007; 84: 581–585.Google Scholar
11. Schranz, D. Hybrid approach in hypoplastic left heart syndrome. Heart 2014; 100: 750–751.Google Scholar
12. Galantowicz, M, Cheatham, JP, Phillips, A, et al. Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg 2008; 85: 2063–2070.Google Scholar
13. Schranz, D, Bauer, A, Reich, B, et al. Fifteen-year single center experience with the “Giessen Hybrid” approach for hypoplastic left heart and variants: current strategies and outcomes. Pediatr Cardiol 2015; 36: 365–373.Google Scholar
14. Trezzi, M, Cetrano, E, Carotti, A. Hybrid palliation for hypoplastic left heart syndrome: navigating the grey zone. J Thorac Cardiovasc Surg 2015; 150: 435–436.Google Scholar
15. Taketazu, M, Barrea, C, Smallhorn, JF, Wilson, GJ, Hornberger, LK. Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome. J Am Coll Cardiol 2004; 43: 1902–1907.CrossRefGoogle ScholarPubMed
16. Marshall, AC, van der Velde, ME, Tworetzky, W, et al. Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum. Circulation 2004; 110: 253–258.Google Scholar
17. Kalish, BT, Tworetzky, W, Benson, CB, et al. Technical challenges of atrial septal stent placement in fetuses with hypoplastic left heart syndrome and intact atrial septum. Catheter Cardiovasc Interv 2014; 84: 77–85.Google Scholar
18. Kim, E, Sobczyk, WL, Yang, S, Mascio, C, Austin, EH, Recto, M. Restrictive tunnel patent foramen ovale and left atrial hypertension in single-ventricle physiology: implications for stent placement across the atrial septum. Pediatr Cardiol 2008; 29: 1087–1094.Google Scholar

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