Heart Care Info - Heart Disease Prevention & Treatment

Heart Transplantation for a Patient With Kearns–Sayre Syndrome and End‐Stage Heart Failure

DJ Homan, DM Niyazov, PW Fisher... - Congestive Heart ..., 2011 - Wiley Online Library A 24-year-old man with KearnsSayre Syndrome (KSS), multiple transient ischemic attacks, dilated cardiomyopathy (ejection fraction, 15%), and diastolic dysfunction was admitted with decompensated heart failure. The patient (Figure 1) also had had progressive muscle ...

The cardiac manifestations of KSS are the most important aspect of the disease for determining prognosis.3 Typically, patients develop cardiac conduction defects that progress to complete heart block and then manifest clinically as congestive heart failure, syncope, and/or sudden death. In all patients with mt DNA mutations (not just KSS patients), mortality among those with no cardiac disease is 26% vs 71% in patients with cardiomyopathy.4 More case reports exist for complete heart block as the etiology of death in KSS patients than any other arrhythmia, but some speculate that ventricular arrhythmias are the actual fatal cause of death since QT prolongation progressing to torsades de pointes has been reported.5 Currently, permanent pacemakers are indicated for all patients with neuromuscular diseases (including KSS) who have developed atrioventricular (AV) block, but prophylactic pacemaker placement prior to developing third degree or advanced second degree AV block is not supported by the most recent American College of Cardiology/American Heart Association/North American Society for Pacing and Electrophysiology guidelines.6 Additionally, there is currently no indication for automatic ICD placement for primary prevention in KSS patients, but many end up with an automatic ICD because they qualify based on the degree of their cardiomyopathy.