Arrhythmogenic right ventricular cardiomyopathy in an asymptomatic patient

Introducere: Arrhythmogenic right ventricular cardi-omyopathy is predominantly a genetically determined heart muscle disorder that is characterized mainly by fibrofatty replacement of the right ventricular myocardium, which may act as a substrate for ventricular arrhythmias and sudden cardiac death. This results in abnormalities and aneurysms to global dilatation and dysfunction, with or without left ventricular involvment. ARVC is three times more prevalent in men than women and the prevalence is 1:2500 or 1:5000, considering sudden death. Many patients with ARVC, paricularly those with sporadic cases, remain clinically asymptomatic for decades, making the condition difficult to diagnose. The role of therapy is to prevent life-threatening arrhytmia and improve the quality of life.

Case presentation: A 60- year old patient, known with arrhythmogenic right ventricular cardiomyopathy, pre-sents in the cardiology department for palpitations and dizziness. Physical examination revealed obesity grade I, irregular heart sounds, HR= 55bpm, ABP= 130/90. ECG presented atrial fibrillation with slow ventricular rate, HR= 50 bpm, inverted T in precordial leads V1-V6 and ventricular extrasystoles. TTE revealed right ventricular and atrial enlargement, right ventricle apex intensely trabeculated, lateral wall hypokinesis and tri-cuspid regurgitation gr. I. The patient underwent Hol-ter monitoring which showed us that the minimum heart rate was 40 bpm and the maximum heart rate was 54 bpm. There were a total of 2544 ventricular beats. These were comprised of 2024 single beats, 43 couplets and 197 ventricular bigeminy events and 0 tachycardiac events. There were also 171 pause intervals defined greater than 2.4 seconds. MRI was performed and revealed right ventricle dilated with aspect of aneurys-mal dilatation of the lateral wall basal segment, with accentuation of trabeculations, with suggestive areas for intramyocardial fatty tissue, lateral and inferior wall with late contrast outlet at this level (mid wall type), with segmental kinetic disorders (akinetic, hypokinetic and dyskinetic areas) and impaired systolic function, elements that advocate for arrhythmogenic cardiomyopathy of the right ventricle. The patient was managed as atrial fibrillation with anticoagulation, antiarrhythmic and beta-blockers presenting a problem due to the low ventricular rate. Considering ARVC, the patient is a candidate for defibrillator implantation for preventing life-threatening arrhythmia, and regarding the modi-fications brought by ECG and Holter monitoring, the attribution of pacemaker should also be discussed.

Particularity: Asymptomatic patient for a long period of time, is diagnosed with ARVS following rou-tine investigations, resulting in a difficult therapeutic approach considering the presence of other rhythm disorders. The therapeutic management of patients with ARVC has evolved over the years and continues to be a chalange. Implantable defibrillator therapy is the most logical therapeutic strategy for patients with ARVC, because the natural history is primarily characterized by the risk of SCD and, only secondarily, by contractile dysfunction leading to progresive heart failure. A single-chamber ICD system is recommended in order to minimize the incidence of long-term lead-related complications, mostly in young patients.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)