Lung aplasia (right)

Case contributed by Hidayatullah Hamidi

Presentation

Respiratory distress. Echocardiography reported large ASD, situs inversus, levocardia, and suspected PAPVC.

Patient Data

Age: Neonate
Gender: Male
ct

Complete absence of right lung.
Rudimentary blinded right main bronchus.
The trachea and left bronchial tree are normal.
The heart is located in the right hemithorax.
The right pulmonary artery and vein are not seen.
ASD is seen.
Normal drainage of SVC and IVC to the right atrium.
Normal drainage left pulmonary veins to the left atrium.
The pulmonary trunk normally arises from the right ventricle.
Aorta normally arises from the left ventricle.
Aberrant right subclavian artery arising from the distal aortic arch distal to the level of origin of the left subclavian artery.

Compensatory hyperinflation of the left lung with some part of it herniating towards the right hemithorax.

Case Discussion

The features are diagnostic of right lung agenesis type II (Aplasia) with ASD. 

The presence of blinded rudimentary right bronchus is pathognomic for lung aplasia than agenesis. 

As no pulmonary vein is present on the right side, hence the echocardiologist has suspected partial anomalous pulmonary venous return.

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