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Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies

Published online by Cambridge University Press:  06 July 2018

Sophie C. Hofferberth ,
Jesse J. Esch ,
David Zurakowski ,
Christopher W. Baird ,
John E. Mayer  and
Sitaram M. Emani
Sophie C. Hofferberth
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
Jesse J. Esch
Affiliation:
Department of Cardiology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
David Zurakowski
Affiliation:
Departments of Anesthesia and Biostatistics, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
Christopher W. Baird
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
John E. Mayer
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
Sitaram M. Emani*
Affiliation:
Department of Cardiac Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA
*
Author for correspondence: S. M. Emani, MD, Department of Cardiac Surgery, Boston Children’s Hospital, Bader 273, 300 Longwood Avenue, Boston, MA 02115, USA. Tel: +617 355 6712; Fax: +1 617 730 0214; E-mail: [email protected]
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Abstract

Introduction

The optimal approach to unifocalisation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (pulmonary artery/ventricular septal defect/major aortopulmonary collaterals) remains controversial. Moreover, the impact of collateral vessel disease burden on surgical decision-making and late outcomes remains poorly defined. We investigated our centre’s experience in the surgical management of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals.

Materials and methods

Between 1996 and 2015, 84 consecutive patients with pulmonary artery/ventricular septal defect/major aortopulmonary collaterals underwent unifocalisation. In all, 41 patients received single-stage unifocalisation (Group 1) and 43 patients underwent multi-stage repair (Group 2). Preoperative collateral vessel anatomy, branch pulmonary artery reinterventions, ventricular septal defect status, and late right ventricle/left ventricle pressure ratio were evaluated.

Results

Median follow-up was 4.8 compared with 5.7 years for Groups 1 and 2, respectively, p = 0.65. Median number of major aortopulmonary collaterals/patient was 3, ranging from 1 to 8, in Group 1 compared with 4, ranging from 1 to 8, in Group 2, p = 0.09. Group 2 had a higher number of lobar/segmental stenoses within collateral vessels (p = 0.02). Group 1 had fewer catheter-based branch pulmonary artery reinterventions, with 5 (inter-quartile range from 1 to 7) per patient, compared with 9 (inter-quartile range from 4 to 14) in Group 2, p = 0.009. Among patients who achieved ventricular septal defect closure, median right ventricle/left ventricle pressure was 0.48 in Group 1 compared with 0.78 in Group 2, p = 0.03. Overall mortality was 6 (17%) in Group 1 compared with 9 (21%) in Group 2.

Discussion

Single-stage unifocalisation is a promising repair strategy in select patients, achieving low rates of reintervention for branch pulmonary artery restenosis and excellent mid-term haemodynamic outcomes. However, specific anatomic substrates of pulmonary artery/ventricular septal defect/major aortopulmonary collaterals may be better suited to multi-stage repair. Preoperative evaluation of collateral vessel calibre and function may help inform more patient-specific surgical management.

Keywords

Pulmonary atresiamajor aortopulmonary collateralsunifocalisationsurgery
Type
Original Article
Information
Cardiology in the Young , Volume 28 , Issue 9 , September 2018 , pp. 1091 - 1098
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Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Hofferberth SC, Esch JJ, Zurakowski D, Baird CW, Mayer JE, Emani SM. (2018) Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: collateral vessel disease burden and unifocalisation strategies. Cardiology in the Young 28: 1091–1098. doi: 10.1017/S104795111800080X

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