Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma

N Engl J Med. 1984 Jan 19;310(3):142-8. doi: 10.1056/NEJM198401193100302.


To investigate cardiopulmonary function in progressive systemic sclerosis with diffuse scleroderma, we studied 26 patients with maximal exercise and redistribution thallium scans, rest and exercise radionuclide ventriculography, pulmonary-function testing, and chest roentgenography. Although only 6 patients had clinical evidence of cardiac involvement, 20 had abnormal thallium scans, including 10 with reversible exercise-induced defects and 18 with fixed defects (8 had both). Seven of the 10 patients who had exercise-induced defects and underwent cardiac catheterization had normal coronary angiograms. Mean resting left ventricular ejection fraction and mean resting right ventricular ejection fraction were lower in patients with post-exercise left ventricular thallium defect scores above the median (59 +/- 13 per cent vs. 69 +/- 6 per cent [P less than 0.025], and 36 +/- 12 per cent vs. 47 +/- 7 per cent [P less than 0.025], respectively). We conclude that in progressive systemic sclerosis with diffuse scleroderma, abnormalities of myocardial perfusion are common and appear to be due to a disturbance of the myocardial microcirculation. Both right and left ventricular dysfunction appear to be related to this circulatory disturbance, suggesting ischemically mediated injury.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Aged
  • Cardiac Catheterization
  • Cardiomyopathies / etiology
  • Coronary Angiography
  • Coronary Circulation
  • Exercise Test
  • Heart / diagnostic imaging
  • Heart / physiopathology*
  • Humans
  • Microcirculation
  • Middle Aged
  • Radioisotopes
  • Radionuclide Imaging
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / diagnostic imaging
  • Scleroderma, Systemic / physiopathology*
  • Stroke Volume
  • Thallium


  • Radioisotopes
  • Thallium