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.
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