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Start free with EleplanCongenital heart block
ORPHA:60041Disease
Also called Congenital atrioventricular block
What it is
Congenital heart block (CHB) is a rare disorder of atrioventricular conduction, characterized by absence of conduction of atrial impulses to the ventricles with slower ventricular rhythm (atrioventricular dissociation). CHB can occur in association with immunological evidence of maternal connective disease (autoimmune CHD), fetal structural CHD or can be idiopathic.
Key facts
- Prevalence
- 1-9 / 100 000 (at birth)
- Age of onset
- Infancy, Neonatal
- Inheritance
- Not applicable
- Classified as
- Disease
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
Sometimes5–29%
21- Bradycardia
- Congestive heart failure
- Crackles
- Cyanosis
- Exercise intolerance
- Fatigue
- Feeding difficulties in infancy
- Gallop rhythm
and 13 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
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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