Familial prolonged atrial standstill presenting in infancy

SUMMARY Three of nine siblings were affected by atrial standstill. Clinical, electrophysiological, and histological findings in two of these cases were studied. Electrophysiological studies showed persistent atrial standstill in one and partial atrial standstill in the other. Biopsy specimens of the right atria showed amyloid deposits, but specimens of the right ventricles and skin did not. Neither patient had peripheral neuropathy, gastrointestinal disorder, or renal dysfunction. Familial atrial standstill is usually associated with peripheral neuropathy.In the two cases studied amyloid deposition mainly affected the atrium, rather than the ventricles or other organs, and caused atrial standstill.Amyloidosis is a cause of atrial standstill.'-3 The most common clinical feature of cardiac amyloidosis is rapidly progressive heart failure4 5; familial amyloidosis is usually also associated with peripheral neuropathy.6 We report on three cases of familial atrial standstill caused by amyloidosis in which there were no cardiac symptoms or peripheral neuropathy.

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Familial prolonged atrial standstill presenting in infancy

Cited by 4 publications

References 10 publications

Disturbances of Cardiac Rhythm

Disturbances of Cardiac Rhythm

Ablation of atrial reentrant tachycardia in children with silent atrium

Familial atrial standstill caused by amyloidosis.

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