Congenital Abnormalities

Pulmonary Agenesis

• No lung

• No vasculature

• No bronchus/airwat

Vascular

Proximal Interruption of Pulmonary artery

• More common on right

• If on left, associated with other congenital heart abnormalities

• Result of failure of proximal 6th aortic arch to form in embryogenesis

• Will see unilateral abscence of pulmonary artery, hypoplastic lung on affected side, parenchymal abnormalities (cystic, etc.) on affected side

Double Aortic Arch

• Encircles trachea and esophagus and therefore may cause stridor

• Will have posterior indentation on esophagus, likely from right arch

• Will have anterior indentation of trachea from left arch

• Cannot differentiate from RA w/ALS on fluoro alone

Left Aortic Arch with Aberrant Right Subclavian

• Posterior indentation on esophagus

• Typically asymptomatic

• If have symptoms, commonly dysphagia, called dysphagia lusoria

Pulmonary Sling

• Abnormal left pulmonary artery which arises from the right pulmonary artery and goes between trachea and esophagus

• Posterior indentation on trachea

• Anterior indentation on esophagus

• Results in entrapment of the trachea

• Associated with

  • Tracheal anomalies (bronchus suis, complete tracheal rings, tracheomalacia)

  • Congenital heart anomalies

Note DDx for vascular cause of stridor

• Double aortic arch

• Right arch with aberrant left subclavian

• Pulmonary sling (only one with a left arch!)

Partial Anomalous Pulmonary Vascular Return

Heterotaxy Syndromes

• Abnormal location of organs commonly with isomerism (mirror image)

• Highly associated with congenital cardiac anomalies and splenic anomalies

Left isomerism

• Presents later in life

• Hyparterial bronchus (bronchus below the artery) as seen on plain film

• Polysplenia (left has a spleen, so you are mirroring the left and therefore have another on the right now)

• Bilateral bi-lobed lung

• Intestinal malrotation

• Azygous continuation of IVC

References: