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ORPHA:185Malformation syndrome
Also called Congenital pulmonary venolobar syndrome · Epibronchial right pulmonary vein syndrome · Halasz syndrome · Hypogenetic lung syndrome
What it is
A rare congenital malformation characterized by a combination of cardiopulmonary anomalies including partial anomalous pulmonary venous connection of the right lung to the inferior caval vein leading to the creation of a left-to-right shunt, and varying degrees of right lung hypoplasia, along with a systemic arterial supply to the right lung.
Key facts
- Prevalence
- 1-9 / 100 000 (at birth, Europe)
- Age of onset
- Adolescent, Adult, Antenatal, Childhood, Infancy, Neonatal
- Inheritance
- Not applicable
- Classified as
- Malformation syndrome
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
11Sometimes5–29%
19- Abnormal hemidiaphragm morphology
- Anomalous pulmonary venous return
- Bronchogenic cyst
- Cough
- Descending aorta hypoplasia
- Double outlet right ventricle
- Hernia
- Hypoplasia of the diaphragm
and 11 more in this range
Rare1–4%
9- Abnormality of the genitourinary system
- Abnormality of the vertebral column
- Anomalous origin of left coronary artery from the pulmonary artery
- Heart block
- Interrupted inferior vena cava with azygous continuation
- Left superior vena cava draining to coronary sinus
- Pneumothorax
- Tricuspid atresia
and 1 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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