Cardiac arrhythmias and rhabdomyolysis in Bartter–Gitelman patients

Abstract

Recent data demonstrate that patients affected with hypokalemic salt-losing tubulopathies are prone to acute cardiac arrhythmias and rhabdomyolysis. The tendency to these potentially fatal complications is especially high if chronic hypokalemia is severe, in patients with diarrhea, vomiting or a prolonged QT interval on standard electrocardiography, in patients on drug management with compounds prolonging the electrocardiographic QT interval (including antiarrhythmic agents, some antihistamines, macrolides, antifungals, psychotropics, ß 2 -adrenergic agonists or cisapride), following acute alcohol abuse and during exercise. Cardiac arrhythmias and rhabdomyolysis occur with sufficient frequency in hypokalemic salt-losing tubulopathies to merit wider awareness of their presence and the preparation of specific prevention and management recommendations.

Phenotype–genotype correlation and follow-up in adult patients with hypokalaemia of renal origin suggesting Gitelman syndrome

“…A few case reports have emphasized the possibility of prolonged QTc intervals with increased risk of syncope and cardiac arrest, perhaps promoted by myocardial perfusion abnormalities (30,31). According to these results, it is difficult to determine the exact risk of cardiac complications in GS patients, but cardiac assessment seems to be recommended.…”

Renal Potassium Handling and Associated Inherited Tubulopathies Leading to Hypokalemia

“…Early replacement of electrolytes is important and in common with Bartter's syndrome, NSAIDs have been used to good effect to promote growth in these children (Liaw, et al, 1999). Patients with Gitelman's syndrome have very good long term prognosis, however sudden cardiac deaths associated with prolonged QT intervals and cardiac arrhythmias have been reported (Cortesi, et al, 2010). In keeping with this less than benign phenotype, Gitelman's patients may have severe symptoms which are debilitating.…”

Understanding Bartter syndrome and Gitelman syndrome

“…[19] Prompt electrolyte and fluid repair, oral potassium supplementation, potassium sparing diuretics, cyclo-oxygenase inhibitors, and renin-angiotensin blockers become life-saving in such emergencies. [19] Finally, in familial cases, both conditions are conveyed by autosomal recessive transmission. [18,20] Clinical characteristics distinct to each syndrome in Gitelman syndrome, [3] the defect resides at the distal convoluted tubule (DCT).…”

“…[16][17][18] Although the chronic hypokalemia can be mildly symptomatic, it can be aggravated by diarrhea or vomiting, precipitating prolonged QT interval, increased risk of rhabdomyolysis, cardiac arrhythmia, syncope, and sudden death. [19] Alcohol abuse, cocaine or other drug abuse can also precipitate life-threatening arrhythmia. [19] Prompt electrolyte and fluid repair, oral potassium supplementation, potassium sparing diuretics, cyclo-oxygenase inhibitors, and renin-angiotensin blockers become life-saving in such emergencies.…”

“…[19] Alcohol abuse, cocaine or other drug abuse can also precipitate life-threatening arrhythmia. [19] Prompt electrolyte and fluid repair, oral potassium supplementation, potassium sparing diuretics, cyclo-oxygenase inhibitors, and renin-angiotensin blockers become life-saving in such emergencies. [19] Finally, in familial cases, both conditions are conveyed by autosomal recessive transmission.…”