Table 3.
EBMT Recommendations 6 | ASSIST 1 | ASTIS 3 | SCOT 2 | Henrique-Neto et al. 10 | Henes et al. 8 | |
---|---|---|---|---|---|---|
PAH assessment | PASP > 40 mmHg at rest or > 45 mmHg post-fluid challenge (RHC) mPAP > 25 mmHg at rest or > 30 mmHg after fluid challenge (RHC) Decrease or lack of augmentation of CO after fluid challenge Pulmonary vascular resistance > 3 wood units |
PASP > 40 mmHg (RHC) mPAP > 25 mmHg (RHC) |
mPAP > 50 mmHg | PA peak systolic pressure > 55 mmHg (echo) PA peak systolic pressure 45-55 mmHg (echo) and mPAP by RHC > 25 mmHg at rest or 30 mmHg with exercise WHO Class III or IV symptoms Taking medications that lower pulmonary artery pressure |
Systolic pulmonary artery pressure > 40 mmHg by echocardiography or mPAP > 27 mmHg by right heart catheter |
Pulmonary arterial hypertension with PASP > 50 mmHg |
LV function | LVEF < 45% | LVEF < 40% | Clinical evidence of significant CHF (NYHA Class III or IV) LVEF < 50% by echocardiogram |
LVEF < 40% Moderate or severe diastolic dysfunction (echocardiography, CMR or RHC) |
Severe heart failure LVEF < 40% by cardiac echo | |
CMR abnormalities | D-sign or septal bounce | |||||
Coronary artery disease | Unrevascularised severe coronary artery disease | |||||
Arrhythmias | Untreated severe arrhythmia | Uncontrolled clinically significant arrhythmias | Ventricular arrhythmias | |||
Pericardial abnormalities | Cardiac tamponade Constrictive pericarditis |
Constrictive pericarditis | ||||
Other | Symptomatic cardiac disease Tricuspid annular plane systolic excursion < 1.8 cm |
Severe major organ involvement | Prior insertion of a pacemaker or cardioverter-defibrillator |
PAH pulmonary arterial hypertension; LV left ventricular; CMR cardiovascular magnetic resonance imaging; EBMT European Society for Blood and Marrow Transplantation; PASP pulmonary artery systolic pressure; mPAP mean pulmonary artery pressure; RHC right heart catheterisation; CO cardiac output; LVEF left ventricular ejection fraction; PA pulmonary artery; WHO World Health Organisation; CHF congestive heart failure; NYHA New York Heart Association; ASSIST Autologous Stem Cell Systemic Sclerosis Immune Suppression Trial; ASTIS Autologous Stem Cell Transplantion International Scleroderma; SCOT Scleroderma: Cyclophosphamide or Transplantation.