Childhood Kidney Diseases

  • 제27권2호
  • /
  • Pages.105-110
  • /
  • 2023
  • /
  • 2384-0242(pISSN)
  • /
  • 2384-0250(eISSN)
대한소아신장학회 (Korean Society of Pediatric Nephrology)
권호 표지
DOI QR코드

DOI QR Code

Hypokalemia as a risk factor for prolonged QT interval and arrhythmia in inherited salt-losing tubulopathy

  • Seong Ryeong Kang (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Yo Han Ahn (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Hee Gyung Kang (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Naye Choi (Department of Pediatrics, Seoul National University Children's Hospital)
  • 투고 : 2023.07.04
  • 심사 : 2023.08.31
  • 발행 : 2023.12.31
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

Purpose: To analyze electrocardiograms (ECGs) of patients with a salt-losing tubulopathy (SLT) and to determine the frequency and risk factors for long QT and arrhythmia. Methods: A total of 203 patients aged <19 years with SLT, specifically Bartter syndrome and Gitelman syndrome, who had a 12-lead ECG were included in this retrospective study. We analyzed the presence of an arrhythmia or prolonged corrected QT (QTc) on ECGs obtained for these patients. Demographic and laboratory data were compared between patients with abnormal and normal ECG findings. Results: Out of the 203 SLT patients, 38 (18.7%) underwent electrocardiography and 10 (40.0%) of 25 patients with inherited SLT had abnormal ECG findings, including prolonged QTc and arrhythmias. The abnormal ECG group had significantly lower serum potassium levels than the normal group (median [interquartile range]: 2.50 mmol/L [2.20-2.83] vs. 2.90 mmol/L [2.70-3.30], P=0.036), whereas other serum chemistry values did not show significant differences. The cutoff level for a significant difference in QTc interval was serum potassium level <2.50 mmol/L. One cardiac event occurred in a 13-year-old boy, who developed paroxysmal supraventricular tachycardia and underwent cardiac ablation. No sudden cardiac deaths occurred in this cohort. Conclusions: The incidence of ECG abnormalities in patients with inherited SLT was 40.0%, whereas the ECG screening rate was relatively low (18.7%). Therefore, we recommend ECG screening in patients with inherited SLT, especially in those with serum potassium level <2.50 mmol/L.

키워드

과제정보

We would like to express our deepest gratitude to the all patients in this study.

참고문헌

  1. Konrad M, Nijenhuis T, Ariceta G, Bertholet-Thomas A, Calo LA, Capasso G, et al. Diagnosis and management of Bartter syndrome: executive summary of the consensus and recommendations from the European Rare Kidney Disease Reference Network Working Group for Tubular Disorders. Kidney Int 2021;99:324-35. https://doi.org/10.1016/j.kint.2020.10.035
  2. Gitelman HJ, Graham JB, Welt LG. A new familial disorder characterized by hypokalemia and hypomagnesemia. Trans Assoc Am Physicians 1966;79:221-35.
  3. Blanchard A, Bockenhauer D, Bolignano D, Calo LA, Cosyns E, Devuyst O, et al. Gitelman syndrome: consensus and guidance from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int 2017;91:24-33. https://doi.org/10.1016/j.kint.2016.09.046
  4. Zelikovic I. Hypokalaemic salt-losing tubulopathies: an evolving story. Nephrol Dial Transplant 2003;18:1696-700. https://doi.org/10.1093/ndt/gfg249
  5. Podrid PJ. Potassium and ventricular arrhythmias. Am J Cardiol 1990;65:33E-44E. https://doi.org/10.1016/0002-9149(90)90250-5
  6. Bettinelli A, Tosetto C, Colussi G, Tommasini G, Edefonti A, Bianchetti MG. Electrocardiogram with prolonged QT interval in Gitelman disease. Kidney Int 2002;62:580-4. https://doi.org/10.1046/j.1523-1755.2002.00467.x
  7. Cortesi C, Lava SA, Bettinelli A, Tammaro F, Giannini O, Caiata-Zufferey M, et al. Cardiac arrhythmias and rhabdomyolysis in Bartter-Gitelman patients. Pediatr Nephrol 2010;25:2005-8. https://doi.org/10.1007/s00467-010-1580-4
  8. Pachulski RT, Lopez F, Sharaf R. Gitelman's not-so-benign syndrome. N Engl J Med 2005;353:850-1. https://doi.org/10.1056/NEJMc051040
  9. Malafronte C, Borsa N, Tedeschi S, Syren ML, Stucchi S, Bianchetti MG, et al. Cardiac arrhythmias due to severe hypokalemia in a patient with classic Bartter disease. Pediatr Nephrol 2004;19:1413-5. https://doi.org/10.1007/s00467-004-1611-0
  10. Nakane E, Kono T, Sasaki Y, Tokaji Y, Ito T, Sohmiya K, et al. Gitelman's syndrome with exercise-induced ventricular tachycardia. Circ J 2004;68:509-11. https://doi.org/10.1253/circj.68.509
  11. Scognamiglio R, Negut C, Calo LA. Aborted sudden cardiac death in two patients with Bartter's/Gitelman's syndromes. Clin Nephrol 2007;67:193-7. https://doi.org/10.5414/CNP67193
  12. Hacihamdioglu DO, Fidanci K, Kilic A, Gok F, Topaloglu R. QT and JT dispersion and cardiac performance in children with neonatal Bartter syndrome: a pilot study. Pediatr Nephrol 2013;28:1969-74. https://doi.org/10.1007/s00467-013-2517-5
  13. Goldenberg I, Moss AJ. Long QT syndrome. J Am Coll Cardiol 2008;51:2291-300. https://doi.org/10.1016/j.jacc.2008.02.068
  14. Scognamiglio R, Calo LA, Negut C, Coccato M, Mormino P, Pessina AC. Myocardial perfusion defects in Bartter and Gitelman syndromes. Eur J Clin Invest 2008;38:888-95. https://doi.org/10.1111/j.1365-2362.2008.02034.x