Background: Systemic sclerosis (SSc) is a disease characterized by the fibrosis of the skin and internal organs. The nature and functional significance of myocardial damage is controversial. Systematic endomyocardial biopsy in this disease has not yet been performed.
Methods: The hypothesis that increased myocardial fibrous tissue deposition occurs in patients with systemic sclerosis with no signs or symptoms of heart failure and normal left systolic ventricle function was tested in 16 SSc patients and 10 controls. Endomyocardial biopsy specimens were obtained from the right ventricular septum in SSc patients. Myocardial specimens were obtained from the same area in controls. Masson's trichrome staining was used for collagen fiber identification. Interstitial (ICVF) and perivascular collagen volume fraction (PCVF) was quantified by videomorphometry. There was a significant increase in the ICVF in patients with SSc compared with the controls, in spite of normal systolic left ventricular function. However, it was not observed in the PCVF.
Conclusions: It is possible to identify cardiac remodeling, characterized by myocardial fibrosis deposits, particularly within the interstitium in SSc patients before the any signs or symptoms of heart failure appear.