Early Lung Function Testing in Infants with Aortic Arch Anomalies Identifies Patients at Risk for Airway Obstruction

Abstract

BackgroundAortic arch anomalies (AAA) are rare cardio-vascular anomalies. Right-sided and double-sided aortic arch anomalies (RAAA, DAAA) are distinguished, both may cause airway obstructions. We studied the degree of airway obstruction in infants with AAA by neonatal lung function testing (LFT).Patients and Methods17 patients (10 RAAA and 7 DAAA) with prenatal diagnosis of AAA were investigated. The median (range) post conception age at LFT was 40.3 (36.6–44.1) weeks, median body weight 3400 (2320–4665) g. Measurements included tidal breathing flow-volume loops (TBFVL), airway resistance (Raw) by bodyplethysmography and the maximal expiratory flow at functional residual capacity (V′maxFRC) by rapid thoracic-abdominal compression (RTC) technique. V′maxFRC was also expressed in Z-scores, based on published gender-, age and height-specific reference values.ResultsAbnormal lung function tests were seen in both RAAA and DAAA infants. Compared to RAAA infants, infants with DAAA had significantly more expiratory flow limitations in the TBFVL, (86% vs. 30%, p<0.05) and a significantly increased Raw (p = 0.015). Despite a significant correlation between Raw and the Z-score of V′maxFRC (r = 0.740, p<0.001), there were no statistically significant differences in V′maxFRC and it's Z-scores between RAAA and DAAA infants. 4 (24%) infants (2 RAAA, 2 DAAA) were near or below the 10th percentile of V′maxFRC, indicating a high risk for airway obstruction.ConclusionBoth, infants with RAAA and DAAA, are at risk for airway obstruction and early LFT helps to identify and to monitor these infants. This may support the decision for therapeutic interventions before clinical symptoms arise.

A Case of Prenatal Presentation with Double Aortic Arch

“…Respiratory symptoms can develop in infants with either an isolated RAA or DAA [11]. However, respiratory symptoms develop in most patients with prenatal diagnosis of DAA 1 and are more severe in infants with DAA than in those with an isolated RAA [11].…”

“…However, respiratory symptoms develop in most patients with prenatal diagnosis of DAA 1 and are more severe in infants with DAA than in those with an isolated RAA [11]. Symptoms of DAA mimic "asthma-like" respiratory symptoms during infancy and the diagnosis of DAA is often delayed in infants without prenatal suspicion or diagnosis of DAA [4].…”

Recurrent respiratory infections caused by a double aortic arch: The diagnostic role of spirometry

“…These cases are mostly associated with a condition of tracheomalacia due to the prolonged tracheal compression caused by the vascular ring 2,5,22–24 . Recently, in a group of infants with a prenatal echocardiographic diagnosis of aortic arch abnormalities, neonatal lung function tests were able to diagnose airway obstruction and to support the rational choice of a surgical therapeutic approach, before the occurrence of clinical manifestations 25 . When compared with other diagnostic tests used to assess airway obstruction, including CT scan, MRI and bronchoscopy, spirometry results to be a sensitive, less expensive, non invasive and non radiating tool.…”

“… 2,5,22–24 Recently, in a group of infants with a prenatal echocardiographic diagnosis of aortic arch abnormalities, neonatal lung function tests were able to diagnose airway obstruction and to support the rational choice of a surgical therapeutic approach, before the occurrence of clinical manifestations. 25 When compared with other diagnostic tests used to assess airway obstruction, including CT scan, MRI and bronchoscopy, spirometry results to be a sensitive, less expensive, non invasive and non radiating tool. Therefore, spirometry should be performed in all patients affected by DAA within the context of a pre-surgical setting aimed to evaluate the degree of pulmonary function impairment, as well as in follow-up post-surgical evaluations, in order to assess lung function recovery.…”