OBJECTIVE: Systemic sclerosis (SSC; scleroderma)-associated pulmonary arterial hypertension (PAH) is a major cause of mortality in SSc patients and represents an important diagnostic and therapeutic target. Our aims were to evaluate the relationship between echocardiogram-derived right-sided heart hemodynamics and gold standard right-sided heart catheterization (RHC) measurements in a scleroderma population and to investigate whether this relationship is modified by a subset of pulmonary hypertension. METHODS: We performed RHC and echocardiography on the same day, with pulmonary function testing in 21 consecutive subjects with scleroderma and precapillary pulmonary hypertension (mean ± SD age 57 ± 10 years, 81% women). RESULTS: RHC measures, including pulmonary arterial systolic and mean pressure and pulmonary vascular resistance (PVR), correlated strongly with echocardiogram-derived data. RHC-derived PVR was negatively associated with right ventricular (RV) systolic performance, as measured by tricuspid annular plane systolic excursion (TAPSE; rho = -0.70, P < 0.001), tissue Doppler tricuspid s' velocity (rho = -0.68, P = 0.002), and RV fractional area change (rho = -0.78, P < 0.001). Correlations with TAPSE and s' velocity were strengthened when forced vital capacity %/diffusing capacity of the lung for carbon monoxide % ≥1.6 was used to identify pure PAH phenotypes in SSc. Bland-Altman analyses demonstrated strong agreement between RHC and echocardiogram-derived hemodynamic measures. CONCLUSION: Our findings suggest that echocardiography may play a clinical role in identifying pulmonary hypertension and RV dysfunction noninvasively, particularly in a subset of SSc patients stratified by pulmonary function testing. This method may establish specific disease phenotypes with differential cardiovascular impact and prove useful as a marker of disease progression/risk stratification in SSC patients that warrants further investigation in larger cohorts.
OBJECTIVE:Systemic sclerosis (SSC; scleroderma)-associated pulmonary arterial hypertension (PAH) is a major cause of mortality in SSc patients and represents an important diagnostic and therapeutic target. Our aims were to evaluate the relationship between echocardiogram-derived right-sided heart hemodynamics and gold standard right-sided heart catheterization (RHC) measurements in a scleroderma population and to investigate whether this relationship is modified by a subset of pulmonary hypertension. METHODS: We performed RHC and echocardiography on the same day, with pulmonary function testing in 21 consecutive subjects with scleroderma and precapillary pulmonary hypertension (mean ± SD age 57 ± 10 years, 81% women). RESULTS: RHC measures, including pulmonary arterial systolic and mean pressure and pulmonary vascular resistance (PVR), correlated strongly with echocardiogram-derived data. RHC-derived PVR was negatively associated with right ventricular (RV) systolic performance, as measured by tricuspid annular plane systolic excursion (TAPSE; rho = -0.70, P < 0.001), tissue Doppler tricuspid s' velocity (rho = -0.68, P = 0.002), and RV fractional area change (rho = -0.78, P < 0.001). Correlations with TAPSE and s' velocity were strengthened when forced vital capacity %/diffusing capacity of the lung for carbon monoxide % ≥1.6 was used to identify pure PAH phenotypes in SSc. Bland-Altman analyses demonstrated strong agreement between RHC and echocardiogram-derived hemodynamic measures. CONCLUSION: Our findings suggest that echocardiography may play a clinical role in identifying pulmonary hypertension and RV dysfunction noninvasively, particularly in a subset of SSc patients stratified by pulmonary function testing. This method may establish specific disease phenotypes with differential cardiovascular impact and prove useful as a marker of disease progression/risk stratification in SSC patients that warrants further investigation in larger cohorts.
Authors: Paul R Forfia; Micah R Fisher; Stephen C Mathai; Traci Housten-Harris; Anna R Hemnes; Barry A Borlaug; Elzbieta Chamera; Mary C Corretti; Hunter C Champion; Theodore P Abraham; Reda E Girgis; Paul M Hassoun Journal: Am J Respir Crit Care Med Date: 2006-08-03 Impact factor: 21.405
Authors: D Mukerjee; D St George; C Knight; J Davar; A U Wells; R M Du Bois; C M Black; J G Coghlan Journal: Rheumatology (Oxford) Date: 2004-01-06 Impact factor: 7.580
Authors: Amr E Abbas; F David Fortuin; Nelson B Schiller; Christopher P Appleton; Carlos A Moreno; Steven J Lester Journal: J Am Coll Cardiol Date: 2003-03-19 Impact factor: 24.094
Authors: D Mukerjee; D St George; B Coleiro; C Knight; C P Denton; J Davar; C M Black; J G Coghlan Journal: Ann Rheum Dis Date: 2003-11 Impact factor: 19.103
Authors: George Hung; Valentina Mercurio; Steven Hsu; Stephen C Mathai; Ami A Shah; Monica Mukherjee Journal: Curr Rheumatol Rep Date: 2019-12-07 Impact factor: 4.592