Kearns-Sayre Syndrome With Persistent Ventricular Tachycardia Refractory to Shocks and Medications

Cardiovascular conduction delay makes up part of the triad associated with Kearns-Sayre syndrome (KSS). Although there have been a few reported cases of prolonged Qtc and polymorphic ventricular tachycardia associated with this disease, despite the use of automatic implantable cardioverter defibrillators (AICD) for secondary prevention, some cases have been reported where the use of AICD did not help. We present a case of a 62-year-old male with KSS who came to the emergency department (ED) after two episodes of syncope. He already had an automatic AICD placed at the age of 34. Our patient had Qtc prolongation which is an unusual finding in KSS. He also had recurrent ventricular tachycardia (VT) refractory to medications and multiple shocks from his AICD, which progressed to a VT storm. He eventually passed away after the withdrawal of care, as his prognosis worsened. We recommend that a more clear guideline will help manage this devastating disease, resulting in mortality reduction.