We report a young girl with classic Bartter disease (type III) with severe hypokalemia (< or = 2.0 mmol/l) who developed a prolonged heart rate-corrected QT interval of 510 ms (upper reference 430 ms) and ST segment depression in all leads. Holter electrocardiography was performed (with a plasma potassium level of 2.0 mmol/l) and it disclosed a stable sinus rhythm, a prolonged correct QT interval, more-evident ST segment depression during an increase in heart rate, a few single premature ventricular complexes, and nocturnal conduction abnormalities such as second-degree atrioventricular block 2:1. In the light of these results, the treatment was modified by increasing indomethacin from 1.5 to 3 mg/kg per day and adding spironolactone at a dose of 5 mg/kg per day. After 10 days, plasma potassium levels increased to 2.7 mmol/l and electrocardiographic abnormalities regressed. No other cardiac abnormalities were noted when the serum potassium was maintained > 2.5 mmol/l. In conclusion, this case report supports the link between arrhythmic events and chronic renal hypokalemic alkalosis in renal tubular disorders. We highlight the importance of standardizing the use of rest electrocardiography and 24-h Holter monitoring to diagnose arrhythmic events in children with severe hypokalemic renal disorders, especially in those with a plasma potassium < 2.5 mmol/l. The importance of beginning early medical treatment, to improve plasma potassium levels and reverse cardiac abnormalities, is emphasized.
[Show abstract][Hide abstract] ABSTRACT: Potassium deficiency may cause cardiac arrhythmias culminating in syncope or sudden death.
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.
Four patients died suddenly and three had severe syncope. These episodes occurred in the context of severe chronic hypokalaemia (< or =2.5 mmol/l) or were precipitated by acute diseases, which exacerbated hypokalaemia (< or =2.0 mmol/l).
In conclusion, severe chronic or acute hypokalaemia is hazardous in inborn salt-losing tubulopathies.
[Show abstract][Hide abstract] ABSTRACT: Antenatal Bartter syndrome typically presents with marked fetal polyuria that leads to polyhydramnios and premature delivery. In our case, polyhydramnios was detected at 26 weeks of gestation and amniotic fluid chloride level was high. Serial amnion-drains were performed. During indomethacine treatment, fetal bradycardia developed. The mother was given daily oral potassium to maintain normal serum level and prevent fetal arrhythmias. After birth, hypokalaemic alkalosis, low chloride, hyper-reninemia and hyperaldosteronemia were detected.
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