Table 1.

Population characteristics, echocardiographic and invasive findings.

CharacteristicsN = 35
Age, yrs65 ± 12
Female, n (%)20 (57)
Weight, kg64 ± 12
Height, cm163 ± 10
Body mass index, kg/m223.7 ± 7.4
Pulmonary hypertension, n (%)25 (71)
NYHA, n (%)
  I3 (9)
  II12 (34)
  III19 (54)
  IV1 (3)
SSc subset, n (%)
  Limited cutaneous30 (86)
  Diffuse cutaneous5 (15)
Disease duration, mos5.3 ± 4.7
SSc-related organ involvement, n (%)
  Skin35 (100)
  Lung15 (43)
  Heart2 (6)
  Gastrointestinal*2 (6)
  Kidney2 (6)
  Muscle1 (3)
Immunological profile, n (%)
  Anticentromere antibodies16 (46)
  Anti–Scl-706 (17)
  Anti-RNA polymerase III2 (6)
Echocardiographic findings
  Left ventricular ejection fraction, %66 ± 8
  PASP, mmHg51 ± 16
  Right atrial pressure, mmHg6 ± 2
  TRV, m/s3.5 ± 0.6
  TVIRVOT15 ± 5
  Pulmonary acceleration time, ms3 ± 21
  TAPSE, mm20 ± 5
  Tricuspid annular S wave, m/s11 ± 3
Right heart catheterization findings
  PVR, Wood units4.8 ± 2.3
  mPAP, mmHg31 ± 8
  PASP, mmHg53 ± 15
  PCWP, mmHg9 ± 4
  CO, l/min5.0 ± 1.0
  Right ventricular–to right atrial pressure gradient, mmHg45 ± 17
  Right atrial pressure, mmHg6 ± 4
  • * Gastrointestinal involvement excepting gastroesophageal reflux. Values are mean ± SD unless otherwise specified. NYHA: New York Heart Association; SSc: systemic sclerosis; PASP: pulmonary arterial systolic pressure; TRV: peak tricuspid regurgitant velocity; TVIRVOT: right ventricular outflow tract time-velocity integral; PCWP: pulmonary capillary wedge pressure; TAPSE: tricuspid annular plane systolic excursion; PVR: pulmonary vascular resistance; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; CO: cardiac output.