Scimitar syndrome

Also known as: congenital pulmonary venolobar syndrome; Hypogenetic lung syndrome

Anomalous right pulmonary venous drainage to the inferior vena cava with right lung hypoplasia.

Medical specialty: Cardiology
Subspecialty: Cardiology / Congenital heart disease
Sex & age distribution: Male: affected; Female: affected, slightly more; Children: yes, infant and adult forms
Gene(s) involved: None recorded

Etiology & mechanism

Failure of normal right pulmonary vein connection.

Gene(s) involved (0)

No gene recorded.

Symptoms & signs (7)

cyanosisdextroposition of heartdyspneafailure to thrivepulmonary hypertensionrecurrent chest infectionsscimitar sign on chest x-ray

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