Rohit Gupta1, Robert P Baughman2, Steven D Nathan3, Athol U Wells4, Vasilis Kouranos4, Esam H Alhamad5, Daniel A Culver6, Joseph Barney7, Eva M Carmona8, Francis C Cordova9, Marloes Huitema10, Mary Beth Scholand11, Marlies Wijsenbeek12, Sivagini Ganesh13, Surinder S Birring14, Laura C Price15, Stephen John Wort15, Oksana A Shlobin3. 1. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, USA. Electronic address: rohit.gupta@tuhs.temple.edu. 2. Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA. 3. The Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, VA, USA. 4. Interstitial Lung Disease/Sarcoidosis Unit, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College, London, UK. 5. Division of Pulmonary Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia. 6. Department of Pulmonary Medicine, and Department of Inflammation and Immunity, Cleveland Clinic, Cleveland, OH, USA. 7. The University of Alabama at Birmingham School of Medicine, Birmingham, AL, USA. 8. Pulmonary and Critical Care, Mayo Clinic, Rochester, MN, USA. 9. Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, USA. 10. Department of Cardiology, Sint Antonius Hospital, Nieuwegein, the Netherlands. 11. University of Utah School of Medicine, Salt Lake City, UT, USA. 12. Centre of Excellence for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus Medical Centre, Rotterdam, the Netherlands. 13. Pulmonary, Critical Care and Sleep Medicine, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA. 14. Centre for Human & Applied Physiological Sciences, School of Basic & Medical Biosciences, Faculty of Life Sciences & Medicine, King's College London, London, UK. 15. National Pulmonary Hypertension Service, Royal Brompton Hospital, London, UK.
Abstract
INTRODUCTION: Sarcoidosis associated pulmonary hypertension (SAPH) is a leading contributor to sarcoidosis-related mortality. The 6-min walk test (6MWT) is widely used in assessment of cardiorespiratory conditions. A reduced 6-min walk distance (6MWD) has been associated with increased mortality in SAPH. We examined patients from the Registry of Sarcoidosis Associated Pulmonary Hypertension (ReSAPH) who had performed 6MWT at enrollment to identify variables that affect 6MWD, and the prognostic value of 6MWT variables regarding death or lung transplantation. MATERIAL AND METHODS: ReSAPH patients with available 6MWT were included. Variables analyzed using pre-defined cutoffs included 6MWD, initial and end of test Borg dyspnea score, oxygen saturation, and heart rate at beginning, end, and after 1-min recovery, absolute change in oxygen saturation, modified distance-saturation product (mDSP), and the heart rate recovery at 1-min (HRR). FINDINGS: 174 patients met inclusion criteria; 48 patients died and 8 underwent lung transplantation. Patients with 6MWD<300 m had a higher chance of dying or undergoing transplantation compared to those with 6MWD>300 m (p = 0.012). No associations with outcome were observed with mDSP cutoff 200 m%, desaturation≥5% and oxygen saturation<88% at end of 6MWT, or multiple HRR cutoffs (13,14,16). 6MWD correlated with initial Borg score, (p = 0.001), DLCO% (p = 0.0001) and sPAP (p = 0.031) on multivariate analysis. These variables were significant for both pre- and post-capillary PH subgroups. 6MWD also correlated with fatigue assessment scale (FAS) (p = 0.015). CONCLUSION: Of the parameters evaluated, 6MWD had the greatest prognostic value in SAPH which correlated with other physiologic and hemodynamic variables. 6MWT captures the multidimensional effects of sarcoidosis.
INTRODUCTION: Sarcoidosis associated pulmonary hypertension (SAPH) is a leading contributor to sarcoidosis-related mortality. The 6-min walk test (6MWT) is widely used in assessment of cardiorespiratory conditions. A reduced 6-min walk distance (6MWD) has been associated with increased mortality in SAPH. We examined patients from the Registry of Sarcoidosis Associated Pulmonary Hypertension (ReSAPH) who had performed 6MWT at enrollment to identify variables that affect 6MWD, and the prognostic value of 6MWT variables regarding death or lung transplantation. MATERIAL AND METHODS: ReSAPH patients with available 6MWT were included. Variables analyzed using pre-defined cutoffs included 6MWD, initial and end of test Borg dyspnea score, oxygen saturation, and heart rate at beginning, end, and after 1-min recovery, absolute change in oxygen saturation, modified distance-saturation product (mDSP), and the heart rate recovery at 1-min (HRR). FINDINGS: 174 patients met inclusion criteria; 48 patients died and 8 underwent lung transplantation. Patients with 6MWD<300 m had a higher chance of dying or undergoing transplantation compared to those with 6MWD>300 m (p = 0.012). No associations with outcome were observed with mDSP cutoff 200 m%, desaturation≥5% and oxygen saturation<88% at end of 6MWT, or multiple HRR cutoffs (13,14,16). 6MWD correlated with initial Borg score, (p = 0.001), DLCO% (p = 0.0001) and sPAP (p = 0.031) on multivariate analysis. These variables were significant for both pre- and post-capillary PH subgroups. 6MWD also correlated with fatigue assessment scale (FAS) (p = 0.015). CONCLUSION: Of the parameters evaluated, 6MWD had the greatest prognostic value in SAPH which correlated with other physiologic and hemodynamic variables. 6MWT captures the multidimensional effects of sarcoidosis.
Keywords:
Registry of sarcoidosis associated pulmonary hypertension; Sarcoidosis; Sarcoidosis associated pulmonary hypertension; Six-minute walk distance; Six-minute walk test