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


METHODS
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.
Ciurzyński
Michał
M
Department of Internal Medicine and Cardiology, Medical University of Warsaw, Poland. mciurzyn@amwaw.edu.pl
Bienias
Piotr
P
Szewczyk
Agnieszka
A
Lichodziejewska
Barbara
B
Błaszczyk
Maria
M
Liszewska-Pfejfer
Danuta
D
Pruszczyk
Piotr
P
D006327:Heart Block / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications*; D008297:Male; D008875:Middle Aged; D012594:Scleroderma, Localized / Q000150:complications; D012595:Scleroderma, Systemic / Q000150:complications*