Arrhythmogenic cardiomyopathy (ACM) is a myocardium disease characterized by phenotypic features of myocardial scarring due to fibrofatty myocardial replacement often associated with global or regional ventricular dysfunction. For years after arrhythmogenic right ventricular cardiomyopathy (ARVC) was first described, the left ventricle (LV) was generally considered normal or minimally involved. In recent years, however, LV involvement has been recognized. It usually presents with early-on arrhythmias more than heart failure symptoms compared to dilated cardiomyopathy. It can be right ventricular, biventricular, or left ventricular. The underlying pathophysiology involves either desmosomal or non-desmosomal mutations. Phospholamban (PLN) mutation is one of those and is associated with more severe arrhythmias and SCD. Primary prevention with ICD implantation should be considered in these patients, even the ones with an ejection fraction greater than 35%. In addition, if such patients progress to Stage D heart failure, they need to be evaluated for advanced heart failure therapies.
Khan, Wahab J; Asif, Muhammad; Chaudhry, Hammad S; Aslam, Sadia; Nadeem, Ifrah; and Chowdhury, Mohammed A.
"Arrhythmogenic Left Ventricular Cardiomyopathy Associated with a Phospholamban Gene Mutation in a Young Female: A Case Report and Literature Review,"
Journal of Community Hospital Internal Medicine Perspectives: Vol. 13:
4, Article 5.
Available at: https://scholarlycommons.gbmc.org/jchimp/vol13/iss4/5