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.

It is generally agreed that most patients with refractory VT or VF often have underlying structural heart disease, 1 often associated with acute ischemia, reperfusion after transient ischemia, worsening heart failure, medications, underlying primary arrhythmia syndromes, hypokalemia and hypomagnesemia, and hyperthyroidism, among other factors. 3 A common pathway in patients with risk factors is the enhanced sympathetic catecholaminergic overdrive, which can augment and perpetuate the arrhythmia itself. 1 The management of electrical storm requires a basic knowledge of the arrhythmia mechanism and possible reversible etiologies, along with the implementation of aggressive abortive interventions. 1 Polymorphic VT, such as in this case, may result from acute ischemia or reperfusion, prolonged QT (both congenital and acquired), Brugada syndrome, CPVT, acute myocarditis, hypertrophic cardiomyopathy, and medications, among other causes. When seen in patients with a normal heart, VF is generally triggered by closely coupled monomorphic PVCs, bradycardia, and hypokalemia. 1 It is well accepted that myocardial ischemia is one of the most prevalent etiologies of polymorphic VT and VF. In this particular case, after a thorough evaluation of the causative factors, it appears that a combination of profound electrolyte disarrangements, ventricular ectopy, and mild myocarditis were responsible for the patient's clinical arrhythmias. Ischemia, in turn, though part of the differential, was less likely the culprit, as evidenced by the negative successive cardiac biomarkers, as well as preserved EF and absence of wall motion abnormalities seen on initial evaluation with echocardiography.