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

Background. Potassium deficiency may cause cardiac arrhythmias culminating in syncope or sudden death. Methods. 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. Results. Four patients died suddenly and three had severe syncope. These episodes occurred in the context of severe chronic hypokalaemia (≤2.5mmol/l) or were precipitated by acute diseases, which exacerbated hypokalaemia (≤2.0mmol/l). Conclusions. In conclusion, severe chronic or acute hypokalaemia is hazardous in inborn salt-losing tubulopathies.