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Heart defect-tongue hamartoma-polysyndactyly syndrome

ORPHA:1338· ICD-10 Q87.8

Definition

A rare, genetic, multiple congenital anomalies syndrome characterized by congenital heart defects (e.g. coarctation of the aorta with or without atrioventricular canal and subaortic stenosis), associated with tongue hamartomas, postaxial hand polydactyly and toe syndactyly.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Neonatal