Arrhythmogenic Cardiomyopathy: A Review of a Rare Case of Biventricular Phenotype

Abstract

Arrhythmogenic cardiomyopathy is a rare hereditary structural heart disease, with various phenotypes, which mostly affects the right ventricle of the heart, resulting in fibrofatty replacement of the heart muscles and a proclivity to create spontaneous malignant cardiac arrhythmias that may lead to sudden death. Most previous reports were noted on young people. We report a case of its biventricular phenotype in a 61-yearold heavy truck driver who has a current medical history of diabetes mellitus and smoking and was incidentally diagnosed based on the Padua criteria after presenting to the hospital with complaints of lightheadedness and syncope. He eventually had an implantable cardioverter defibrillator, hence preventing death. We were able to correctly diagnose the case and prevent sudden cardiac death by instituting the necessary management.

An atypical case report of an arrhythmogenic cardiomyopathy in a 70-years-old patient with suggestive left ventricular signs

“…It was in 1977 that Guy Fontaine, a French cardiologist, and electrophysiologist, described arrhythmogenic cardiomyopathy, which is characterized by a fibrofatty replacement of the heart muscles [3] , predominantly affecting the RV. However, there are several phenotypes that classify the disease into the RV phenotype, the LV phenotype and the biventricular one ( Fig.…”

“…The disease has been described as autosomal dominant with variable penetrance [3] . A rare autosomal recessive variant has been described, which is associated with woolly hair and palmoplantar keratoderma (Naxos disease).…”

“…Palpitation is the most common symptom, manifesting in 27% to 67% of the cases [3] , and occurs due to ventricular arrhythmias. It was the main symptom for our patient.…”

“…It was the main symptom for our patient. Several functional signs are associated with the pathology, notably syncope (an alarming but inconsistent sign), dyspnea, and atypical chest pain [3] . The latter was ted in all 23 cases studied by Bariani et al [12] , with at least one episode described in 90% of them, as well as in a 26-year-old Afro-American patient who described chest pain lasting up to 6 weeks [3] .…”

“…Several functional signs are associated with the pathology, notably syncope (an alarming but inconsistent sign), dyspnea, and atypical chest pain [3] . The latter was ted in all 23 cases studied by Bariani et al [12] , with at least one episode described in 90% of them, as well as in a 26-year-old Afro-American patient who described chest pain lasting up to 6 weeks [3] . It is very important to note that many patients with ACM, particularly those with sporadic cases, remain clinically asymptomatic for decades, making the condition difficult to diagnose [9] .…”