<div>Original article</div><div>One-stage repair of transposition
complex and interrupted aortic arch in children</div><div>Liang Zhang, MD<sup>1</sup> (mhwzyh@163.com); Muzi Li,
MD<sup>2</sup> (limuzifw@163.com); Shoujun Li,
MD<sup>1</sup> (kszcyh@sina.com);
Jun
Yan, MD<sup>1</sup>(jiadetgyx@163.com);
Qiang Wang, MD<sup>1</sup> (txzzyx@sina.com)</div><div><sup>1</sup>Center
for Pediatric Cardiac Surgery, Fuwai Hospital,
National
Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences
and Peking Union Medical College, Beijing, China</div><div><sup>2</sup>Department of Echocardiography, Fuwai Hospital,
National Center for Cardiovascular Diseases, Chinese Academy of Medical
Sciences, and Peking Union Medical College, Beijing, China</div><div>Short running title: Interrupted aortic arch repair</div><div>Corresponding Author:</div><div>Qiang Wang, MD</div><div>Fuwai Hospital, 167 Beilishi Road, Xicheng District, Beijing, China</div><div>Phone: 86-13691061902</div><div>Fax: 86-010-88398496</div><div>E-mail address: txzzyx@sina.com</div><div>Ethics and integrity policy statements:</div><ul><li>Funding statement: This work was supported by the Fundamental Research
Funds for the Central Universities (3332020021).</li><li>Conflict of interest disclosure: The authors have no conflicts of
interest to declare.</li><li>Institutional Review Board approval or waiver: The study was conducted
in accordance with the Declaration of Helsinki (as revised in 2013)
and was approved by the Ethics Committee of Fuwai Hospital (approval
no. 2021-1506).</li><li>Patient consent statement: The requirement of individual consent was
waived due to the retrospective nature of the study.</li><li>Data availability statement: Data presented in this article can be
made available upon reasonable request to the corresponding author</li></ul><div>Abstract:</div><div>Background/Aim: A transposition complex with an interrupted aortic arch
(IAA) is rare and surgically challenging because of its anatomical
diversity and complexity. Herein, we aimed to present our 20-year
experience
with one-stage arterial switch surgery associated with IAA repair.</div><div>Methods: From January 2000 to April 2017, 11 patients were diagnosed
with transposition complex and IAA and underwent one-stage repair at our
center. These patients were retrospectively reviewed. Two patients had
transposition of the great arteries, while the others had double outlet
right ventricles, of whom eight had subpulmonary ventricular septal
defects (Taussig-Bing anomalies), and one had a non-committed
ventricular septal defect. In terms of the IAA, three patients underwent
repair by extended end-to-end anastomosis, and one 16-mm prosthetic
vascular graft was replaced in an elder patient. The remaining patients
underwent autologous pericardial patch enlargement. All the variables
were summarized and reported with descriptive statistics.</div><div>Results: Three early deaths occurred in this study. The median follow-up
time was approximately 5 years (range: 3 – 14 years).
No
late deaths were reported. Only one patient required percutaneous
re-intervention for recurrent coarctation. Moderate aortic regurgitation
was observed in three patients. However, there was no requirement for
aortic valvuloplasty or valve replacement. One patient had more than
moderate tricuspid regurgitation. The other survivors are presently
healthy.</div><div>Conclusions: Although one-stage repair for transposition complex and IAA
still has non-negligible mortality even in older children, the late
outcomes of survivors are acceptable. Owing to the high rate of valve
regurgitation, closer follow-up is necessary for these patients.</div><div>Keywords: Congenital heart disease, transposition complex, interrupted
aortic arch (IAA), one-stage repair, cardiac surgery</div><div>Introduction:</div><div>Patients with transposition complex and interrupted aortic arch (IAA)
are very rare. It has been reported that only 6–10% of infants with
IAA have transposition of the great arteries (TGA).<sup>1</sup>In the past, a staged repair was preferred. Primary IAA repair was
performed via a left thoracotomy, followed by an arterial switch
operation (ASO) after several months, which has been reported to be
associated with high mortality. During the last two decades, a one-stage
repair of IAA at the same time as ASO has been generally accepted as the
treatment of choice.<sup>2</sup></div><div>However, even in the recent era, these patients still represent a
high-risk subgroup of candidates, and only a few reports have been
published on this
group.
Herein, we report our surgical experience using several approaches since
the year 2000 with one-stage ASO and IAA repair.</div><div>Materials and Methods:</div>