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Late-onset severe long QT syndrome
Babken Asatryan1, André Schaller2, Deborah Bartholdi2
1University Clinic of Cardiology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Summary
Congenital Long QT syndrome can manifest late in life, causing fatal arrhythmias like torsades de pointes. Early genetic counseling and assessment are vital for families with unexplained sudden death history.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Congenital Long QT syndrome (LQTS) is a genetic disorder affecting heart repolarization.
- LQTS can lead to life-threatening ventricular arrhythmias, including torsades de pointes (TdP).
- Family history of sudden unexplained death is a critical indicator for potential LQTS.
Observation:
- A 77-year-old male presented with torsades de pointes arrhythmia as the initial clinical manifestation.
- The patient had a family history of sudden unexplained death.
- This presentation occurred despite no prior known cardiac symptoms.
Findings:
- This case highlights that congenital Long QT syndrome can manifest with potentially fatal arrhythmias in elderly, previously asymptomatic individuals.
- Torsades de pointes can be the first and only presenting sign of LQTS, even late in life.
- Genetic predisposition for LQTS may remain latent until advanced age.
Implications:
- Vigilant clinical assessment is crucial in families with a history of sudden unexplained death.
- Genetic counseling is essential for identifying at-risk asymptomatic relatives.
- Late-onset LQTS necessitates considering genetic cardiac channelopathies in elderly patients presenting with unexplained arrhythmias.
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