[Kearns-Sayre syndrome: recurrent syncope and atrial flutter].

Kearns-Sayre syndrome is a rare disease linked to mitochondrial inheritance. The characteristic diagnostic triad consists of progressive external ophthalmoplegia, pigmentary degeneration of the retina, and atrioventricular block.1 It may also be associated with mental retardation, ataxia, deafness, muscle weakness, and endocrine disorders, such as diabetes mellitus or hypothyroidism. We present the case of a 37-yearold man with a noticeable phenotypic manifestation (Figure 1). From childhood, he had progressively presented photophobia, progressive loss of vision, external ophthalmoplegia, neurosensory deafness, Addison’s syndrome, muscle weakness, and ataxia. His present clinical symptoms consisted of recurrent syncope and atrial flutter. The association of Kearns-Sayre syndrome with atrial flutter has not been described. Twelve years before the current episode, he had been cardiologically assessed and diagnosed with Kearns-Sayre syndrome. At that time, the patient was asymptomatic, but the electrocardiogram showed right bundle-branch block and left anterior fascicular block. Periodic follow-up was prescribed. In the successive follow-ups, the patient remained asymptomatic with no changes in the electrocardiogram. The onset of the current clinical symptoms started during the postoperative of a cholecystostomy, when the patient presented several episodes of syncope. The electrocardiogram disclosed atypical flutter rhythm and regular conduction with an approximate ventricular frequency of 106 bpm, right bundle-branch block, and left anterior fascicular block (Figure 2). The echocardiogram was normal. An electrophysiological study established the diagnosis of cavotricuspid isthmusdependent flutter and counterclockwise rotation around the tricuspid valve. Isthmus ablation was performed successfully. After recovery of sinus rhythm, the atrioventricular conduction study showed prolongation of the HV interval and a short LETTERS TO THE EDITOR