Refractory ventricular tachycardia storm associated with severe hypokalemia in Fanconi syndrome

Rodriguez, Alex P.; Badiye, Amit; Lambrakos, Litsa K.; Ghodsizad, Ali; Myerburg, Robert J.; Goldberger, Jeffrey J.

In polymorphic VT, electrolyte abnormality correction is one of the mainstays of therapy. Especially in this case, against the backdrop of Fanconi syndrome and stage IV chronic kidney disease, the patient's profound hypokalemia was thought to be a major determinant of the recurrent arrhythmias. Despite the aggressive repletion of potassium in the setting of acute or chronic renal insufficiency, serial blood draws showed a downward trend in serum K 1 levels, which made the arrhythmia resistant to treatment. Animal studies have demonstrated that hypokalemia-induced arrhythmogenicity stems from prolonged ventricular repolarization, slowed conduction, and abnormal pacemaker activity. 4 Intravenous beta blocker administration is also indicated in a variety of situations of incessant VT, including acute myocardial ischemia and long QT syndrome (LQTS), and has been used with favorable results and mortality improvement. 1 In this case, initial intravenous propranolol use in the emergency department was precluded by periodic bradycardia episodes that consistently deteriorated into VT and VF. However, after placement of a temporary pacemaker, concomitant use of beta blockers is feasible, as was seen in our patient. Amiodarone may also be effective in suppressing and controlling malignant VT storm episodes. 5 Ultimately, ECMO placement along with sedation and intubation, stellate ganglionic blockade, antiarrhythmic drug therapy, rapid ventricular pacing, and aggressive electrolyte replacement succeeded in decreasing the arrhythmia burden and attaining hemodynamic stability.