Case Report: We present a case of a newborn baby who developed biphasic stridor immediately after a normal vaginal delivery. Endoscopic assessment of the trachea revealed a pulsatile narrowing at the level of the thoracic trachea, suggestive of an external compression. A contrast-enhanced computed tomography scan of the thorax with three-dimensional reconstruction confirmed the diagnosis of DAA with compression of the trachea and esophagus.
Conclusion: Clinicians should strongly consider the possibility of a congenital vascular ring compression should an infant with a normal upper airway present with stridor. A precise diagnosis can be made by radiological examination.
STUDY DESIGN: This observational cross-sectional study includes 195 confirmed PPHN with a gestational age of ≥34 weeks without congenital heart disease. Multivariable logistic regression was used to identify risk factors for mortality.
RESULTS: The mortality rate was 16.4%, with the highest mortality with pulmonary hypoplasia. Of 195, 65% received iNO; 18% were iNO non-responders with the majority having pulmonary hypoplasia. Independent risk factors for mortality were the presence of reversal of flow at the descending aorta, pulmonary hypoplasia, APGAR scores ≤ 5 at 5 min, and idiopathic PPHN with an adjusted odds ratio of 15.9, 7.5, 6.7, and 6.4, respectively.
CONCLUSIONS: Despite the usage of iNO, mortality due to PPHN remains high and is related to etiology and cardiac function.