Hypogenetic Lung Syndrome

Pulmonary Venolobar Syndrome

Scimitar Syndrome

Hypogenetic Lung Syndrome

Pulmonary Venolobar Syndrome

Scimitar Syndrome

© William Herring, MD, FACR

Hypogenetic Lung SyndromeGeneral

Associated with:

Hypoplastic pulmonary artery, anomalousvenous return

Diaphragmatic hernia

Hemivertebrae and scoliosis

CHD: ASD, VSD, PDA, TOF

Location: right-sided predominance

40% are asymptomatic

Hypogenetic Lung SyndromeImaging

Hypoplasia, aplasia of one or more lobes ofright lung

Scimitar vein (90%)=PAPVR from belowdiaphragm to IVC, portal

Small hilus

Small right hemithorax and mediastinal shift

Cardiac dextroposition

Rib notching

Scimitar Vein, Hypogenetic RightLung

Scimitar Vein, Hypogenetic RightLung

Hypogenetic Lung SyndromeDDX

Dextrocardia

Hypoplastic Lung

Swyer-James syndrome

Hypogenetic Lung SyndromeHorseshoe Lung

Uncommon variant of hypogeneticlung syndrome in which RLL crossesmidline between esophagus and heartand joins left lung

The End