Hereditary sick sinus syndrome

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Hereditary sick sinus syndrome

ORPHA:166282Disease

What it is

A rare cardiac rhythm disease, usually of the elderly, characterized by electrocardiographic findings of sinus bradycardia, atrial fibrillation, atrial tachycardia sinus arrest, or sino-atrial block, and that manifest with symptoms like syncope, dizziness, palpitations, fatigue, or even heart failure. It results from malfunction of the cardiac conduction system, probably secondary to degenerative fibrosis of nodal tissue in the elderly or secondary to cardiac disorders in younger patients.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
Adult, Elderly
Inheritance
Autosomal dominant, Autosomal recessive
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.

Genes

GNB2Disease-causing germline mutation(s)
HCN4Disease-causing germline mutation(s)
MYH6Major susceptibility factor
SCN5ADisease-causing germline mutation(s)

ICD-10 codes

I49.5filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 13663MEDDRA 10040639MESH D012804MONDO 0012061OMIM 163800OMIM 182190OMIM 608567OMIM 614090OMIM 619464UMLS C0340491

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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