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Background  A 5‐year‐old, healthy English Springer Spaniel died suddenly 4 months after delivering a litter of 7 puppies. Within 4 months of the dam's death, 3 offspring also died suddenly.    Hypothesis  Abnormal cardiac repolarization, caused by an inherited long  QT  syndrome, is thought to be responsible for arrhythmias leading to sudden death in this family.    Animals  Four remaining dogs from the affected litter and 11 related dogs.    Methods  Physical examination and resting  ECG  were done on the littermates and 9 related dogs. Additional tests on some or all littermates included echocardiogram with Doppler, Holter monitoring, and routine serum biochemistry. Blood for  DNA  sequencing was obtained from all 15 dogs.    Results  Three of 4 littermates examined, but no other dogs, had prolonged  QT  intervals with unique T‐wave morphology.  DNA  sequencing of the  KCNQ 1 gene identified a heterozygous single base pair mutation, unique to these 3 dogs, which changes a conserved amino acid from threonine to lysine and is predicted to change protein structure.    Conclusions and Clinical Importance  This family represents the first documentation in dogs of spontaneous familial  QT  prolongation, which was associated with a  KCNQ 1 gene mutation and sudden death. Although the final rhythm could not be documented in these dogs, their phenotypic manifestations of  QT  interval prolongation and abnormal  ECG  restitution suggested increased risk for sudden arrhythmic death. The  KCNQ 1 gene mutation identified is speculated to impair the cardiac repolarizing current  I  Ks,  similar to  KCNQ 1 mutations causing long  QT  syndrome 1 in humans.

Sudden Death Associated with QT Interval Prolongation and KCNQ 1 Gene Mutation in a Family of English Springer Spaniels