Advanced systemic sclerosis complicated by pulmonary hypertension and complete atrioventricular block: a case report

Med Sci Monit. 2007 Oct;13(10):CS124-7.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Heart Block / complications*
  • Humans
  • Hypertension, Pulmonary / complications*
  • Male
  • Middle Aged
  • Scleroderma, Localized / complications
  • Scleroderma, Systemic / complications*