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Assessment of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis: Comparison of Noninvasive Tests with Results of Right-Heart Catheterization
VIVIEN M. HSU, ABEL E. MOREYRA, ALAN C. WILSON, MEIR SHINNAR, DANIEL M. SHINDLER, JULIANNE E. WILSON, AMI DESAI, and JAMES R. SEIBOLD
ABSTRACT. Methods. 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 ≥ 25 mm Hg, 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. Results. 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 evaluation had a sensitivity of 71% and specificity of 72% (AUC 0.76). Conclusion. In evaluation of SSc with suspected PH, echo appeared to be the most useful among the noninvasive tests, mainly due to the high specificity, high positive predictive value, and highest AUC. However, due to the low sensitivity of noninvasive testing, RHC should remain the gold standard. (First Release Jan 15 2008; J Rheumatol 2008;35:458-65) Key Indexing Terms:
PULMONARY ARTERIAL HYPERTENSION
From the University of Medicine and Dentistry of New Jersey Scleroderma Program; Division of Cardiovascular Diseases and Hypertension, UMDNJ Robert Wood Johnson Medical School, New Brunswick, New Jersey; Yale University, New Haven, Connecticut; and University of Michigan, Ann Arbor, Michigan, USA. V.M. Hsu, MD, Assistant Professor, Director, Scleroderma Program; J.E. Wilson, RN, BSN, Research Coordinator, UMDNJ Scleroderma Program; A.E. Moreyra, MD, Professor; A.C. Wilson, PhD, Associate Professor; M. Shinnar, MD, Associate Professor, Director, Cardiac MRI; D.M. Shindler, MD, Professor, Medical Director, Echocardiography, Division of Cardiovascular Diseases and Hypertension, UMDNJ Robert Wood Johnson Medical School; A. Desai, MD, Yale University; J.R. Seibold, MD, Professor, Director, Scleroderma Program, University of Michigan, Ann Arbor. Address reprint requests to Dr. V.M. Hsu, UMDNJ Scleroderma Program, Clinical Research Center, Acute Care Bldg., 3rd floor, PO Box 19, New Brunswick, NJ 08903-0019. E-mail: hsuvm@umdnj.edu Accepted for publication October 15, 2007. |
