Arrhythmogenic Right Ventricular Cardiomyopathy in a Patient Experiencing Out-of-Hospital Ventricular Fibrillation Arrest Twice: Case Report and Review of the Literature

Abstract

We present a case of a 62-year-old male who was admitted to the hospital with out-of-hospital ventricular fibrillation (VF) arrest. He had a VF arrest in 2011 and was admitted to another hospital. He had several investigations excluding cardiac magnetic resonance imaging, all of which were normal. He was playing tennis on both occasions when he experienced the VF arrest. His electrocardiogram on admission showed AF with partial right bundle branch block, inverted T waves in V1-V2, low voltage QRS complexes, ventricular ectopic in lead V1-V2, and prolonged QTc. His echocardiogram showed normal left ventricular function and a dilated right ventricle. Cardiac magnetic resonance imaging showed a dilated RV cavity size with impaired systolic function and dyskinetic region in the mid-ventricular free wall proximal to the insertion of the moderator band and late gadolinium enhancement in both right and left ventricles insertion points and mid-wall late gadolinium enhancement in the basal inferolateral wall suggestive of arrhythmogenic right ventricular cardiomyopathy. He had a single chamber VVI implantable cardioverter-defibrillator fitted for primary prevention and was discharged home. He had outpatient follow-up and showed good improvement and his implantable cardioverter-defibrillator checks were satisfactory and did not experience any shocks.

A Case Report of a Young Patient Presenting With Syncope Secondary to Arrhythmogenic Ventricular Cardiomyopathy

“…The clinical findings and morphological features of the disease support the non-ischaemic atrophy of the right ventricular myocardium rather than the congenital absence of the myocardium that leads to arrhythmias in adolescents and young adults [ 6 , 7 ]. AVC is the second leading cause of death in young patients following hypertrophic cardiomyopathy [ 1 ]. Thiene et al (1988) reported autopsy findings suggestive of AVC in a series of cases of exertional sudden cardiac deaths in young adults; the ECGs in these patients demonstrated right precordial leads with negative T waves and left bundle branch (LBBB) morphology type ventricular arrhythmia [ 8 ].…”

“…Khan et al (2022) reported a case of a 62-year-old patient who had two out-of-hospital cardiac arrests in a span of a decade and was successfully resuscitated both times. His coronary arteries were unobstructed and CMR findings were suggestive of ARVC, for which he had an ICD implanted for primary prevention [ 1 ]. Latt et al (2017) published a case report of a 33-year-old male athlete presenting with palpitations and chest pain, on a background of similar episodes five years previously [ 10 , 11 ].…”

“…Arrhythmogenic ventricular cardiomyopathy (AVC) is an inherited condition, first described by Frank and Fontain in 1978 [ 1 ]. They described it as characterized by total or partial replacement of right ventricular muscle with fibro-fatty tissue.…”

“…Other genes found to have alterations in patients with ARVC include plakoglobin (JUP), desmocollin 2 (DSC2), plakophilin 2 (PKP2), and desmoplasia (DSP) [ 3 ]. It is most commonly diagnosed on post-mortem examination following the death of an individual; however, younger patients may also present with sustained ventricular tachycardia, left-sided, right-sided, or biventricular failure [ 1 ]. There is a significant geographical variation in the distribution of the disease; for example, it is the most common cause of death in young individuals below 35 years of age and young athletes in Italy whereas it accounts for about 5% of sudden cardiac death in individuals < 65 and 3-4% in young athletes [ 4 ].…”

“…ARVC most commonly affects male patients and about 80% of individuals diagnosed with ARVC are < 40 years old [ 4 ]. It should commonly be suspected in individuals presenting with cardiac arrest, syncopal episodes, or arrhythmias and is commonly seen in patients with hypertrophic obstructive cardiomyopathy (HOCM) [ 1 , 4 ].…”