Key result
Familial long-QT syndrome presents with sudden cardiac death and recurrent syncope, necessitating thorough electrocardiographic and clinical evaluation of surviving relatives.
Why the study?
How should family members of a patient with suspected Long-QT syndrome be evaluated and treated?
How should family members of a patient with suspected Long-QT syndrome be evaluated and treated?
This clinical vignette highlights the presentation of familial Long-QT syndrome and raises the question of appropriate evaluation and management for surviving family members.
Supports ECG screening of relatives in suspected cases; extends classic descriptions of familial LQTS inheritance.
After a 13-year-old girl dies suddenly while playing basketball, her family comes to the clinic for medical evaluation. Her parents' resting electrocardiograms (ECGs) are normal, but her 9-year-old sister's ECG has an abnormally long QT interval. The family history is notable for recurrent syncope in the maternal grandmother's female relatives. How should family members be evaluated and treated?
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Dan M. Roden (2008) studied this question. Familial long-QT syndrome presents with sudden cardiac death and recurrent syncope, necessitating thorough electrocardiographic and clinical evaluation of surviving relatives.
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