Stridor as a Harbinger of Congenital Cardiovascular Anomaly

There is an intimate spatial relationship between the cardiovascular and airway structures. Central airway compression related to congenital cardiovascular anomalies should be considered in neonates, infants, and young children presenting with stridor.

From July 31, 1990 to December 31, 2018, 24 pediatric patients, including 18 males and 6 females, aged 1 day to 11.3 years old, presenting with stridor and/or lip cyanosis were enrolled in this study. At presentation, none of the patients had a known history of congenital heart disease. Patients with congenital bronchopulmonary vascular/foregut malformations, congenital pulmonary venolobar syndrome, congenital pulmonary malinosculations, Eisenmenger syndrome, secondary pulmonary hypertension, and idiopathic pulmonary arterial hypertension were excluded from this study. Available profiles of 24 patients were reviewed and the underlying congenital cardiovascular anomalies contributing to the clinical manifestation of stridor were analyzed, including chart recordings, chest radiograms, echocardiograms, computerized tomography, electrocardiograms, esophagograms, cardiac catheterization, magnetic resonance imaging, and bronchography.

Stridor was an early sign of congenital cardiovascular anomalies, including double aortic arch, right aortic arch (RAA) with Kommerell diverticulum, mirror-image right aortic arch with aortic diverticulum, anomalous right innominate artery, left pulmonary artery sling, RAA with tetralogy of Fallot and persistent fifth aortic arch, a vertical patent ductus arteriosus from a transverse left aortic arch, and absent pulmonary valve syndrome. Notably, chest radiography provided the first clue of RAA in 18 of the 24 patients.

Stridor can be a harbinger of congenital cardiovascular anomalies causing central airway compression in pediatric patients.