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Abstract: FR-PO555

Abnormal Electrocardiogram Incidence and Risk Factors in Hypokalemic Salt-Losing Tubulopathy

Session Information

Category: Fluid‚ Electrolyte‚ and Acid-Base Disorders

  • 1002 Fluid‚ Electrolyte‚ and Acid-Base Disorders: Clinical


  • Kang, Seong Ryeong, Seoul National University Children's Hospital, Seoul, Korea (the Republic of)
  • Choi, Naye, Seoul National University Children's Hospital, Seoul, Korea (the Republic of)
  • Min, Jeesu, Seoul National University Children's Hospital, Seoul, Korea (the Republic of)
  • Ahn, Yo Han, Seoul National University Children's Hospital, Seoul, Korea (the Republic of)
  • Kang, Hee Gyung, Seoul National University Children's Hospital, Seoul, Korea (the Republic of)
  • Lim, Seon Hee, Korean Society of Nephrology, Seocho-gu, Seoul, Korea (the Republic of)

Hypokalemic salt-losing tubulopathy including Batter’s/Gitelman’s syndrome (BS/GS) is characterized by hypokalemia, hyponatremia, and hypomagnesemia, which may prolong the corrected QT (QTc) interval and lead to cardiac arrhythmias. So far, 6 cases of arrhythmia or cardiac death in BS/GS have been reported. Despite its fatality, limited information is known about the frequency or risk factors of arrhythmia in patients with hypokalemic salt-losing tubulopathy.


A total of 83 patients under the age of 18 at diagnosis with hypokalemic salt-losing tubulopathy at our center were reviewed. Thirty-four of 83 patients had a 12-lead electrocardiogram during follow-up. Among them, the abnormalities of the rhythms and the QTc were investigated. Sex, age, body mass index (BMI), serum electrolytes level, and genetic background were compared between those with abnormal ECG and cases with normal ECG.


Eight of 34 (23.5%) patients showed abnormal heart rhythms, 21 (61.8%) prolonged QTc beyond borderline, and 7 showed both abnormal heart rhythms and prolonged QTc. These 22 (64.7%) patients were classified as abnormal ECG group. There was no difference in sex, age, BMI, and serum levels of sodium, potassium, magnesium, and calcium at the time of ECG when compared with the normal ECG group (p>0.05).
Two of 22 (9.09%) patients had the procedure for arrhythmia. One was performed catheter ablation and the other was inserted a pacemaker. There was no cardiac arrest during follow-up. Only 1 of 9 patients with follow-up ECG was normalized. Among the normal ECG group, 2 cases (16.7%) showed QTc prolongation in adults. Of 13 patients who performed echocardiography, 3 had dilated cardiomyopathy findings, and the rest are normal findings. Currently, 18 of 34 patients (52.9%) are under follow-up at our center, of which 6 have progressed to chronic kidney disease, and one is on hemodialysis.


Among hypokalemic salt-losing tubulopathy patients at our center, ECG abnormality was relatively high(64.7%). There were no significant differences in sex, age, BMI, and serum electrolyte levels. As arrhythmia can be fatal when it occurs, prevention is important. Therefore frequent follow-up is required to monitor laboratory findings, ECG, and patient compliance. Cardiologic evaluation should also be performed regularly.