Phospholamban Immunostaining is A Highly Sensitive and Specific Method For Diagnosing Phospholamban p.Arg14del Cardiomyopathy

Phospholamban (PLN) p.Arg14del cardiomyopathy is associated with an increased risk of malignant ventricular arrhythmias and severe heart failure, and a poor prognosis from late adolescence. It can be diagnosed in whole heart specimens, but rarely in right ventricular biopsy specimens, by PLN immunohistochemistry (IHC) showing PLN-containing aggregates concentrated in cardiomyocytes in dense perinuclear aggresomes. The purpose of this study was to determine whether PLN IHC can be used to diagnose PLN p.Arg14del cardiomyopathy using apical left ventricular (LV) myocardial specimens harvested during LV assist device (LVAD) implantation.

Source: Cardiovascular Pathology - May 29, 2017 Category: Cardiology Authors: Wouter P. te Rijdt, Z. Joy van der Klooster, Edgar T. Hoorntje, Jan D.H. Jongbloed, Paul A. van der Zwaag, Folkert W. Asselbergs, Dennis Dooijes, Rudolf A. de Boer, J. Peter van Tintelen, Maarten P. van den Berg, Aryan Vink, Albert J.H. Suurmeijer Source Type: research