Combination of echocardiographic and pulmonary function test measures improves sensitivity for diagnosis of systemic sclerosis-associated pulmonary arterial hypertension: analysis of 2 cohorts.
OBJECTIVE
To evaluate routinely collected non-invasive tests from 2 systemic sclerosis (SSc) cohorts to determine their predictive value alone and in combination versus right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH).


METHODS
We evaluated 2 cohorts of patients who were at risk or with incident PAH: (1) The Pulmonary Hypertension Assessment and Recognition Outcomes in Scleroderma (PHAROS) cohort and (2) an inception SSc cohort at Cochin Hospital, Paris, France. Estimated right ventricular systolic pressure (eRVSP) as determined by transthoracic echocardiogram (TTE) and pulmonary function test (PFT) measures was evaluated, and the predictive values determined. We then evaluated patients with PAH missed on TTE cutoffs that were subsequently identified by a PFT measure.


RESULTS
In the PHAROS cohort (n = 206), 59 (29%) had RHC-defined PAH. An eRVSP threshold of 35-50 mm Hg failed to diagnose PAH in 7% to 31% of patients, 50% to 70% of which (n = 2-13) were captured by PFT measures. In the Cochin cohort (n = 141), 10 (7%) patients had RHC confirmed PAH. An eRVSP threshold of 35-50 mm Hg missed 0% to 70% (n = 0-7) of patients, of which 0% to 68% (n = 0-6) were met by PFT measures. The combination of TTE and PFT improved the negative predictive value for diagnosing PAH.


CONCLUSIONS
In 2 large SSc cohorts, screening with TTE and PFT captured a majority of patients with PAH. TTE and PFT complement each other for the diagnosis of PAH.
From the University of Michigan Scleroderma Program; Division of Rheumatology, Department of Medicine, Georgetown University, Washington, DC, USA; Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; St. Joseph's Hospital and Medical Center, Division of Pulmonary, Department of Medicine, Phoenix, AZ; David Geffen School of Medicine at UCLA, Division of Pulmonary, Department of Medicine, Los Angeles, CA; Arizona State University, Biodesign Institute, Tempe, AZ; Department of Biostatistics, University of Michigan, School of Public Health, Ann Arbor, MI, USA; and Paris XIII University, Cardiology Department, Avicenne Hospital, Bobigny, France.
Heather
H
Gladue
Virginia
V
Steen
Yannick
Y
Allanore
Rajeev
R
Saggar
Rajan
R
Saggar
Paul
P
Maranian
Veronica J
VJ
Berrocal
Jerome
J
Avouac
Christophe
C
Meune
Mona
M
Trivedi
Dinesh
D
Khanna
D000328:Adult; D000368:Aged; D015331:Cohort Studies; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology*; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D012680:Sensitivity and Specificity; D014463:Ultrasonography
DIAGNOSIS; ECHOCARDIOGRAM; PULMONARY ARTERIAL HYPERTENSION; PULMONARY FUNCTION TESTS; PULMONARY HYPERTENSION; SYSTEMIC SCLEROSIS