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Clinical Implications of Airway Anomalies and Stenosis in Patients with Heterotaxy Syndrome
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  • Mei-Hwan Wu,
  • Ching-Chia Wang,
  • En-Ting Wu,
  • Frank Lu,
  • Shyh-Jye Chen
Mei-Hwan Wu
National Taiwan University Hospital

Corresponding Author:wumh@ntu.edu.tw

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Ching-Chia Wang
National Taiwan University Hospital
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En-Ting Wu
National Taiwan University Hospital
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Frank Lu
National Taiwan University Hospital
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Shyh-Jye Chen
National Taiwan University Hospital
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In heterotaxy syndrome, bronchopulmonary situs usually reflects atrial situs, resulting in either right (RAI) or left atrial isomerism (LAI). This study determines airway anomalies and its implications in patients with heterotaxy. This retrospective study included 223 patients with heterotaxy syndrome who received an integrated cardiac computed tomography evaluation. Patient database from 1995 to 2020 was reviewed. The patients were examined by a congenital heart disease team comprising pediatric cardiologists, radiologists, pulmonologists, and cardiovascular surgeons. Among the 223 patients, 189 (84.8%, M/F=1.66) had RAI and 29 had LAI (13.0%, M/F=0.71). Five patients had indeterminate isomerism (2.2%, M/F=1.5). Discordant bronchopulmonary and atrial situs occurred in 4% patients, while discordant bronchopulmonary, atrial, and splenic situs occurred in 23.2% patients. Lower airway stenosis was observed in 61 patients (27.4%), including 27.5%, 20.7%, and 60% RAI, LAI, and indeterminate isomerism patients, respectively (p=0.189). One patient had an intrinsic long segment lower tracheal stenosis and received slide tracheoplasty. Initial cardiac operation was performed in 213 patients. Higher surgical mortality occurred in patients with RAI (19.5% vs. none for LAI and indeterminate isomerism, p=0.038). In patients with RAI, lower airway anomaly/stenosis increased the duration of ventilator usage (p=0.030) but did not affect surgical mortality. Total anomalous pulmonary venous return to systemic veins and pulmonary venous stenosis were major surgical risk factors. Bronchopulmonary isomerism shares a similar isomeric pattern to cardiac atrial appendage. Lower airway anomalies/stenosis was common in patients with heterotaxy, resulting in prolonged ventilator therapy in patients with RAI.
09 Dec 2021Submitted to Pediatric Pulmonology
09 Dec 2021Submission Checks Completed
09 Dec 2021Assigned to Editor
10 Dec 2021Reviewer(s) Assigned
23 Dec 2021Review(s) Completed, Editorial Evaluation Pending
31 Dec 2021Editorial Decision: Revise Major
10 Mar 20221st Revision Received
11 Mar 2022Submission Checks Completed
11 Mar 2022Assigned to Editor
11 Mar 2022Reviewer(s) Assigned
12 Apr 2022Review(s) Completed, Editorial Evaluation Pending
18 Apr 2022Editorial Decision: Revise Minor
28 Apr 20222nd Revision Received
28 Apr 2022Submission Checks Completed
28 Apr 2022Assigned to Editor
28 Apr 2022Reviewer(s) Assigned
04 May 2022Review(s) Completed, Editorial Evaluation Pending
07 May 2022Editorial Decision: Accept