Severe syncope and sudden death in children with inborn salt-losing hypokalaemic tubulopathies

Abstract

Potassium deficiency may cause cardiac arrhythmias culminating in syncope or sudden death. An inquiry performed among physicians caring for a total of 249 patients with inborn salt-losing tubulopathies revealed that acute cardiac complications occurred in seven children. Four patients died suddenly and three had severe syncope. These episodes occurred in the context of severe chronic hypokalaemia (< or =2.5 mmol/l) or were precipitated by acute diseases, which exacerbated hypokalaemia (< or =2.0 mmol/l). In conclusion, severe chronic or acute hypokalaemia is hazardous in inborn salt-losing tubulopathies.