Acquired long QT syndrome
ICD-10 I45.8 · ICD-11 BC9Y

Acquired Long QT Syndrome with a Pathogenic Mutation and a Normal QTc Interval

This protocol covers the management of acquired long QT syndrome in patients who carry a confirmed pathogenic mutation but currently show a normal QTc interval on ECG. The absence of QTc prolongation does not eliminate risk in this population, and a distinct management approach applies.

A pathogenic mutation is present with a normal QTc interval at the time of assessment. Current evidence supports active management in this specific subgroup — an expectant approach alone is insufficient.

Beta-blocker therapy is part of the recommended management for patients in this mutation-positive, normal-QTc setting. The complete structured regimen — including agent selection and all accompanying guidance — is available in the full protocol.

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References

DOI: 10.1093/eurheartj/ehac262

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