Background: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and visceral organs, in which the heart is frequently (40–70% of patients) and severely involved. Pulmonary hypertension affects 10–15% of patients with SSc and is one of the most important complications adversely influencing their survival.
Case Report: The case report presents a 59-year-old male patient with advanced systemic sclerosis whose initial examination revealed pulmonary hypertension, rhythm and atrioventricular conduction disturbances, and elevated level of NT-proBNP. After six months the patient deteriorated; an increase in NT-proBNP level and progression of pulmonary hypertension were observed.
Conclusions: The described case is followed by a discussion of cardiovascular involvement in systemic sclerosis and emphasizes that heart involvement in SSc may have very serious clinical implications.

Keywords: Heart Block - complications, Hypertension, Pulmonary - complications, Scleroderma, Localized - complications, Scleroderma, Systemic - complications