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Surgical Management of Tetralogy of Fallot with Unilateral Absence of the Pulmonary Artery

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Abstract

Tetralogy of Fallot with unilateral absence of the pulmonary artery (UAPA) is a rare congenital heart disease. The aim of the present study was to examine the surgical strategy for Tetralogy of Fallot with UAPA by summarizing our experience of its treatment. We retrospectively evaluated 17 patients admitted to our hospital for treatment between 2006 and 2017. All patients were diagnosed with absence of the left pulmonary artery. The Nakata Index (NI), NI Z score, and McGoon ratio of the existing pulmonary artery were calculated. All patients were divided into one-stage complete repair (group A) or palliative procedure (group B) groups according to these criteria and surgical treatments. There were nine treated patients in group A, with a mean NI of 595.6 ± 690.32 mm2/m2 (169.3–2433 mm2/m2) and a mean NI Z score of − 1.57 ± 3.02 (− 4.60 to 5.27). There were eight treated patients in group B, with a mean NI of 107.61 ± 49.49 mm2/m2 (53.15–216.39 mm2/m2) and a mean NI Z score of − 6.27 ± 1.56 (− 8.22 to − 3.54). The mean follow-up time in group A was 5.58 ± 3.42 years (1–11.7 years), while that in group B was 5.4 ± 3.42 years (0.6–12.1 years). No hospital deaths occurred and the follow-up results were good in both groups. In conclusion, development of a single existing pulmonary artery can be evaluated using the NI and the NI Z score. These evaluations can be used to select complete repair or palliative procedures for patients and achieve good results.

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Abbreviations

ALPA:

Absence of the left pulmonary artery

APV:

Absence of the pulmonary valve

ASD:

Atrial septal defect

CPB:

Cardiopulmonary bypass

CR:

Complete repair

CT:

Computed tomography

LSVC:

Left superior vena cava

MAPCAs:

Major aorto-pulmonary collateral arteries

MBTS:

Modified right-sided Blalock-Taussig shunt

NI:

Nakata index

NYHA:

New York heart association

PDA:

Patent ductus arteriosus

PFO:

Patent foramen ovale

poSpO2 :

Post-surgery percutaneous oxygen saturation

preSpO2 :

Pre-surgery percutaneous oxygen saturation

PS:

Pulmonary stenosis

PVP:

Pulmonary valvuloplasty

PVS:

Pulmonary valve stenosis

RAARDA:

Right aortic arch and right-side descending aorta

RVOT:

Right ventricular outflow tract

RVOTR:

Right ventricular outflow tract reconstruction

SBE:

Subacute bacterial endocarditis

TAP:

Transannular patch

TBPV:

Transluminal balloon pulmonary valvuloplasty

TOF:

Tetralogy of Fallot

UAPA:

Unilateral absence of the pulmonary artery

VSD:

Ventricular septal defect

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Acknowledgements

We acknowledge the role of our colleagues, perfusionists, nurses, and others involved in the care of the study patients. We also thank Liwen Bianji, Edanz Editing China (www.liwenbianji.cn/ac), for editing the English text of a draft of this manuscript. This study was supported by the National Key R&D Program of China [2017YFC1308100].

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Correspondence to Jing Zhang.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all patients, parents, or guardians included in the study.

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Yang, T., Sun, J., Xu, H. et al. Surgical Management of Tetralogy of Fallot with Unilateral Absence of the Pulmonary Artery. Pediatr Cardiol 40, 1026–1034 (2019). https://doi.org/10.1007/s00246-019-02109-z

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  • DOI: https://doi.org/10.1007/s00246-019-02109-z

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