| Autor: |
Sikking MA; Department of Cardiology, Maastricht University Medical Centre, Cardiovascular Research Institute Maastricht (CARIM), 6229 HX Maastricht, The Netherlands., Stroeks SLVM; Department of Cardiology, Maastricht University Medical Centre, Cardiovascular Research Institute Maastricht (CARIM), 6229 HX Maastricht, The Netherlands., Henkens MTHM; Department of Pathology, Maastricht University Medical Centre, 6229 HX Maastricht, The Netherlands.; Netherlands Heart Institute (NLHI), 3511 EP Utrecht, The Netherlands., Venner MFGHM; Department of Cardiology, Maastricht University Medical Centre, Cardiovascular Research Institute Maastricht (CARIM), 6229 HX Maastricht, The Netherlands., Li X; Department of Pathology, Maastricht University Medical Centre, 6229 HX Maastricht, The Netherlands., Heymans SRB; Department of Cardiology, Maastricht University Medical Centre, Cardiovascular Research Institute Maastricht (CARIM), 6229 HX Maastricht, The Netherlands.; Centre for Molecular and Vascular Biology, Department of Cardiovascular Sciences, Katholieke Universiteit Leuven, 3000 Leuven, Belgium., Hazebroek MR; Department of Cardiology, Maastricht University Medical Centre, Cardiovascular Research Institute Maastricht (CARIM), 6229 HX Maastricht, The Netherlands., Verdonschot JAJ; Department of Cardiology, Maastricht University Medical Centre, Cardiovascular Research Institute Maastricht (CARIM), 6229 HX Maastricht, The Netherlands.; Department of Clinical Genetics, Maastricht University Medical Centre, 6229 HX Maastricht, The Netherlands. |
| Abstrakt: |
Dilated cardiomyopathy (DCM) has a genetic cause in up to 40% of cases, with differences in disease penetrance and clinical presentation, due to different exogeneous triggers and implicated genes. Cardiac inflammation can be the consequence of an exogeneous trigger, subsequently unveiling a phenotype. The study aimed to determine cardiac inflammation in a cohort of genetic DCM patients and investigate whether it associated with a younger disease onset. The study included 113 DCM patients with a genetic etiology, of which 17 had cardiac inflammation as diagnosed in an endomyocardial biopsy. They had a significant increased cardiac infiltration of white blood, cytotoxic T, and T-helper cells ( p < 0.05). Disease expression was at a younger age in those patients with cardiac inflammation, compared to those without inflammation ( p = 0.015; 50 years (interquartile range (IQR) 42-53) versus 53 years (IQR 46-61). However, cardiac inflammation was not associated with a higher incidence of all-cause mortality, heart failure hospitalization, or life-threatening arrhythmias (hazard ratio 0.85 [0.35-2.07], p = 0.74). Cardiac inflammation is associated with an earlier disease onset in patients with genetic DCM. This might indicate that myocarditis is an exogeneous trigger unveiling a phenotype at a younger age in patients with a genetic susceptibility, or that cardiac inflammation resembles a 'hot-phase' of early-onset disease. |
