iPHORECAST

PHOspholamban RElated CArdiomyopathy STudy

1 Current vacancy for iPHORECAST

Bachelor and Master students

Project: BIOSTAT-CHF, Early Synergy, GIPS-IV trial, Adiposity in Heart Failure with Preserved Ejection Fraction, iPHORECAST, KETONE-HF, RACE‐8‐HF, RED-CVD, SECRETE-HF, Selenium and Heart Failure, STOP-HF, PLN cardiomyopathy, AF RISK, APAF-CRT, RACE 9, RASTA AF Research line: Heart Failure, Ischemic Heart Diseases, Experimental Cardiology, Atrial Fibrillation

More about this vacancy

iPHORECAST

In the Netherlands ≈15% of idiopathic dilated cardiomyopathy (DCM) and ≈10% arrhythmogenic right ventricular cardiomyopathy (ARVC) patients carry a single (founder) mutation in the gene encoding Phospholamban, PLN R14del. Analogous to other inherited cardiomyopathies, the natural course of the disease is age-related (“age-related penetrance”); after a presymptomatic phase of variable length many PLN R14del-carriers progress to overt disease, and are diagnosed with either DCM or ARVC.

PLN is a regulator of the sarcoplasmic reticulum Ca2+-ATPase (SERCA2a) pump in cardiac muscle and thereby important for maintaining Ca2+ homeostasis. Cardiac fibrosis is an early manifestation of disease. The hypothesis of iPHORECAST is that treatment of presymptomatic PLN R14del-carriers with eplerenone, which by virtue of its mineralocorticoid(aldosterone)-blocking properties is a strong antifibrotic agent, reduces disease progression and postpones onset of overt disease. iPHORECAST is a multicenter, prospective, randomized trial, carried out in UMCG, Amsterdam UMC, UMCU and Antonius Hospital Sneek. Half of the subjects group receive a 50 mg tablet of eplerenone once daily and the other half receives no treatment. Duration of treatment is 3 years. The study started in 2014 and the last subject of the 84 asymptomatic PLN R14del-carriers that have been included will leave the study beginning 2021.The primary endpoint is a composite of MRI findings (LV and RV volumes and function, and gadolinium late enhancement), ECG findings and arrhythmias. The results of this innovative trial are expected in 2021.

People involved

Principal investigators

Maarten van den Berg

Cardiologist

Rudolf de Boer

Cardiologist