Prevention of cardiac arrhythmias in pediatric patients with normotensive-hypokalemic tubulopathy

Abstract

Potassium deficiency predisposes to cardiac arrhythmias culminating in syncope or sudden death. Because of the uncertainty related to the possible occurrence of such cardiac arrhythmias in the context of normotensive-hypokalemic tubulopathies, 19 European pediatric nephrologists with a large experience of normotensive-hypokalemic tubulopathies were asked to answer a questionnaire. The responses suggest that inherited normotensive-hypokalemic tubulopathies per se do not strongly predispose to dangerous cardiac arrhythmias. However, cardiac arrhythmias may be acutely precipitated by drugs that prolong the QT interval, by diarrhea, or vomiting, and perhaps even by physical activity. Finally, the likelihood of dangerous arrhythmias in normotensive-hypokalemic tubulopathy is currently unknown.

Myocardial perfusion defects in Bartter and Gitelman syndromes

“…A questionnaire‐based inquiry performed among European paediatric nephrologists with a large experience with normotensive hypokalaemic tubulopathies, the only available in the literature, raised the current opinion that inherited normotensive hypokalaemic tubulopathies do not predispose per se to dangerous cardiac arrhythmias which, on the other hand, may be acutely precipitated by treatments with drugs prolonging QT interval, diarrhoea or vomiting, which further worsen hypokalaemia, and perhaps by intense physical activities such as competitive sports. Most of the nephrologists interviewed, however, felt that further investigations were important to assess the true hazard of dangerous arrhythmias in these patients, recommending the need to develop practical guidelines for a cardiac work‐up and management of children and adolescents with normotensive hypokalaemic tubulopathies [28]. Given the findings of our study, the same need also appears to adult patients with these syndromes.…”

Congenital long QT syndrome aggravated by salt-wasting nephropathy

“…Furthermore, arrhythmias or syncopal episodes in patients with Gitelman syndrome (or other inherited normotensivehypokalemic nephropathies) are surprisingly unusual despite hypokalemia. 13,14 No correlation was demonstrated between serum potassium or magnesium levels and QTc in the proband. Furthermore, failure of the QTc to shorten with potassium supplementation prompted referral to a cardiologist for suspected congenital LQTS.…”

C1q nephropathy in association with Gitelman syndrome: a case report

“…While GS has generally been considered to be a benign and asymptomatic disorder, this has been challenged recently in the literature [7][8][9]. Several authors have emphasized the potential for fatigue, musculoskeletal complaints, and even serious cardiac arrhythmias with GS [7][8][9].…”