PMID: 18203320Jan 19, 2008Paper

Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: comparison of noninvasive tests with results of right-heart catheterization

The Journal of Rheumatology
Vivien HsuJames R Seibold

Abstract

Pulmonary hypertension (PH) is an ominous complication in patients with scleroderma (systemic sclerosis, SSc). We compared noninvasive assessment of PH with pulmonary artery (PA) pressures obtained by right-heart catheterization (RHC). Forty-nine patients with SSc were evaluated for suspected PH based on clinical findings, progressive dyspnea, and pulmonary function tests (PFT). PH was defined as mean PA pressure > or = 25 mm Hg, or > or = 30 mm Hg after exercise, with normal pulmonary capillary wedge pressure (PCW). Doppler echocardiography (echo) and cardiac magnetic resonance imaging (MRI) were performed within 4 hours of RHC, and the predictive accuracy of the tests was compared. RHC identified 24/49 (49%) patients with PH. The noninvasive cutpoints were: estimated right ventricular systolic pressure > 47 mm Hg by echo; diameter of the main PA > 28 mm by MRI; and the ratio of forced vital capacity to diffusion capacity (%FVC/%DLCO) > 2.0 by PFT. Echo classified 38 subjects correctly (14/24 with and 24/25 without PH; sensitivity 58%, specificity 96%). The area under receiver-operating characteristic curve (AUC) was 0.84 for echo. MRI measurement of PA diameter had a sensitivity of 68% and specificity 71% (AUC 0.78). PFT eval...Continue Reading

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