Pulmonary agenesis

Case contributed by Dr Liz Silverstone

Presentation

Right heart failure for transplant assessment

Patient Data

Age: 70 years
Gender: Female
X-ray

Small relatively opaque left hemithorax with mediastinal shift and elevated hemidiaphragm.

Large proximal right pulmonary arteries.

Scoliosis.

CT

Single right lung herniating across the midline.

No left bronchus or pulmonary artery.

Pulmonary hypertension due to large secundum ASD: RV dilated and hypertrophied.

Case Discussion

The absence of left bronchial stub distinguishes agenesis from hypoplasia.

ASD and scoliosis are features of VACTERL.

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