by Charles Christoph Roehr, Silke Wilitzki, Bernd Opgen-Rhein, Karim Kalache, Hans Proquitté, Christoph Bührer, Gerd Schmalisch
Aortic 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 Methods
17 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. Results
Abnormal 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. Conclusion
Both, 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.