Recurrent Ventricular Tachycardia in Patient with Friedreich's Ataxia in the Absence of Clinical Myocardial Disease

Abstract

We report a 33-year-old man with recurrent loss of consciousness due to ventricular tachyarrhythmia with a history of Friedreich's ataxia. The patient's symptom was improved after implantation of a single-lead implantable cardiac defibrillator. The clinical, genetic, echocardiographic, and electrocardiographic features are discussed in brief.

2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders

“…91,97 Ventricular arrhythmias have been described in patients with normal left ventricular function, underscoring the need for monitoring in patients who present with palpitations, syncope, or other symptoms suggestive of arrhythmias, with careful screening in asymptomatic patients (Figure 4). 193 AF has also been found to be common as cardiac disease progresses. 6,12 An increased prevalence of ECG preexcitation has been reported in mitochondrial disorders, especially in MELAS, but is rarely associated with symptomatic arrhythmias.…”

Management and therapy for cardiomyopathy in Friedreich’s ataxia

“…In one cohort, 21% of FRDA subjects had supraventricular beats noted, but otherwise no results have been reported [54]. Comments have also been made on the presence of atrial and ventricular arrhythmias in FRDA, but no study has compared the frequency of such events [55].…”

Friedreich’s Ataxia: a review from a cardiology perspective

“…Patients should be risk stratified for sudden death and medical therapy should be optimised prior to device insertion. The implantation of an ICD for recurrent syncope due to ventricular tachycardia in a patient with FA has been described [13]. Two other case reports regarding ICD placement in neuromuscular disorders have been published, one on a patient with Emery Dreifuss Syndrome, in whom a biventricular ICD was implanted [26], and one on a patient with a Desmin related cardiomyopathy in whom a prophylactic ICD was implanted [27].…”

“…A case report published recently described recurrent ventricular tachycardia (VT) and also atrial fibrillation in a patient with FA in the absence of myocardial disease. The patient underwent ICD insertion and subsequently received three appropriate therapies for VT [13]. Sudden death may occur in FA patients, the mechanism of which is not well characterised.…”