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

Charles Christoph Roehr,Silke Wilitzki,Bernd Opgen-Rhein,Gerd Schmalisch,Karim Kalache,Christoph Bührer,Hans Proquitté,Irwin Reiss

doi:10.1371/journal.pone.0024903

Charles Christoph Roehr, Silke Wilitzki + Show 6 more

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https://doi.org/10.1371/journal.pone.0024903

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Journal: PLoS ONE	Publication Date: Sep 23, 2011
Citations: 25	License type: CC BY 4.0

Affiliation: Charité - Universitätsmedizin Berlin

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.

Highlights

Aortic arc anomalies (AAA) are rare congenital cardiac anomalies; they represent approx. 1–3% of all cardiovascular anomalies [1]
Compared to RAAA infants, infants with double-sided aortic arch anomalies (DAAA) had significantly more expiratory flow limitations in the tidal breathing flow-volume loops (TBFVL), (86% vs. 30%, p,0.05) and a significantly increased Raw (p = 0.015)
In right-sided aortic arches (RAAA) the aorta originates from the left ventricle and takes a rightward turn before its descent through the mediastinum, whereas double-sided aortic arch anomalies (DAAA) are combinations of a right-sided and a left sided aortic arch [2]

Summary

Introduction

Aortic arc anomalies (AAA) are rare congenital cardiac anomalies; they represent approx. 1–3% of all cardiovascular anomalies [1]. In right-sided aortic arches (RAAA) the aorta originates from the left ventricle and takes a rightward turn before its descent through the mediastinum, whereas double-sided aortic arch anomalies (DAAA) are combinations of a right-sided and a left sided aortic arch [2] This duplication often forms a vascular ring around the trachea and/or oesophagus, which tends to compress these [3,4,5]. A RAAA, together with the ligamentum arteriosum (the remnant of the ductus arteriosus Botalli) may cause compression of the mediastinal structures by forming a so called aortic sling Both anomalies may cause stridor, cough, dyspnoea, dysphagia and recurrent lower airway infections [6,7,8,9]. We studied the degree of airway obstruction in infants with AAA by neonatal lung function testing (LFT)

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