OBJECTIVE: A prospective study to evaluate echocardiography and gas transfer (DLCO) by comparison with cardiac catheterization in discriminating between patients with and without systemic sclerosis-associated pulmonary arterial hypertension (SScPAH). METHOD: A total of 137 (52 with and 85 without pulmonary fibrosis) had echocardiography and lung function tests within 3 months of their definitive invasive study. RESULTS: At cardiac catheterization 99 of these patients were found to have PAH, while PAH was excluded in 38. Echocardiographically estimated tricuspid gradient (TG) showed a moderate positive correlation (r(2) = 0.44, P<0.005) with both mean pulmonary pressure and invasively determined tricuspid gradient. DLCO showed a weak correlation (r(2 )= 0.09, P = 0.006), when compared with mean pulmonary arterial pressure. In total, 97% of patients with an echocardiographically determined TG of > 45 mmHg were found to have pulmonary hypertension at catheterization. However, no threshold could be defined with either screening test that safely excluded PAH. CONCLUSIONS: The positive predictive accuracy of currently used non-invasive tests are adequate for the diagnosis of advanced PAH provided sufficiently high thresholds (TG > 45 mmHg or DLCO < 55% predicted) are used. These tests cannot be relied upon to exclude pulmonary hypertension where pre-test probability is high.
OBJECTIVE: A prospective study to evaluate echocardiography and gas transfer (DLCO) by comparison with cardiac catheterization in discriminating between patients with and without systemic sclerosis-associated pulmonary arterial hypertension (SScPAH). METHOD: A total of 137 (52 with and 85 without pulmonary fibrosis) had echocardiography and lung function tests within 3 months of their definitive invasive study. RESULTS: At cardiac catheterization 99 of these patients were found to have PAH, while PAH was excluded in 38. Echocardiographically estimated tricuspid gradient (TG) showed a moderate positive correlation (r(2) = 0.44, P<0.005) with both mean pulmonary pressure and invasively determined tricuspid gradient. DLCO showed a weak correlation (r(2 )= 0.09, P = 0.006), when compared with mean pulmonary arterial pressure. In total, 97% of patients with an echocardiographically determined TG of > 45 mmHg were found to have pulmonary hypertension at catheterization. However, no threshold could be defined with either screening test that safely excluded PAH. CONCLUSIONS: The positive predictive accuracy of currently used non-invasive tests are adequate for the diagnosis of advanced PAH provided sufficiently high thresholds (TG > 45 mmHg or DLCO < 55% predicted) are used. These tests cannot be relied upon to exclude pulmonary hypertension where pre-test probability is high.
Authors: Sangmee Bae; Rajeev Saggar; Marcy B Bolster; Lorinda Chung; Mary Ellen Csuka; Chris Derk; Robyn Domsic; Aryeh Fischer; Tracy Frech; Avram Goldberg; Monique Hinchcliff; Vivien Hsu; Laura Hummers; Elena Schiopu; Maureen D Mayes; Vallerie McLaughlin; Jerry Molitor; Nausheen Naz; Daniel E Furst; Paul Maranian; Virginia Steen; Dinesh Khanna Journal: Ann Rheum Dis Date: 2012-02-02 Impact factor: 19.103
Authors: Rajeev Saggar; Dinesh Khanna; Daniel E Furst; Shelley Shapiro; Paul Maranian; John A Belperio; Neeraj Chauhan; Philip Clements; Alan Gorn; S Sam Weigt; David Ross; Joseph P Lynch; Rajan Saggar Journal: Arthritis Rheum Date: 2010-12
Authors: Dinesh Khanna; Heather Gladue; Richard Channick; Lorinda Chung; Oliver Distler; Daniel E Furst; Eric Hachulla; Marc Humbert; David Langleben; Stephen C Mathai; Rajeev Saggar; Scott Visovatti; Nezam Altorok; Whitney Townsend; John FitzGerald; Vallerie V McLaughlin Journal: Arthritis Rheum Date: 2013-12