Pulmonary hypertension induced by invasive cardiopulmonary exercise testing in patients with fibrotic interstitial lung diseases

• Grant number: 14/12212-5
• Support type: Scholarships abroad - Research
• Effective date (Start): August 05, 2014
• Effective date (End): August 04, 2015
• Field of knowledge: Health Sciences - Medicine - Medical Clinics
• Principal researcher: Rudolf Krawczenko Feitoza de Oliveira
• Grantee: Rudolf Krawczenko Feitoza de Oliveira
• Host: Aaron Bradley Waxman
• Home Institution: Escola Paulista de Medicina (EPM). Universidade Federal de São Paulo (UNIFESP). Campus São Paulo. São Paulo , SP, Brazil
• Research place: Brigham and Women's Hospital (BWH), United States

Abstract

Introduction: The presence of pulmonary hypertension (PH) represents a known marker for a poor prognosis in interstitial lung diseases (ILDs). Even mild elevations of pulmonary artery pressure (mPAP) at rest represent independent predictors of mortality in this population. This finding may indicate a subgroup of ILD patients at higher risk for developing PH. The invasive cardiopulmonary exercise testing (iCPET) unifies the functional evaluation obtained by incremental CPET, and the hemodynamic evaluation assessed at right heart catheterization. The iCPET represents one of the most complete diagnostic tools for the detection of early stages of PH, and for the evaluation of dyspnea of unknown etiology. Objectives: To determine the prevalence of PH at iCPET in patients with fibrotic ILD, with a respiratory limitation that is out of proportion to their resting objective data; to describe the hemodynamic, metabolic, ventilatory and cardiovascular mechanisms leading to dyspnea and exercise induced PH in this population. Methods: Prospective, cross-sectional, single-center study. Thirty consecutive patients with fibrotic ILD and a respiratory limitation that is out of proportion to their resting objective data (baseline dyspnea index <9 points and a mild ventilatory restriction with a forced vital capacity >70%) and a systolic pulmonary artery pressure <50 mmHg measured by resting echocardiography, will perform symptom limited iCPET. Rationale: iCPET is capable to differentiate precapillary PH and post-capillary PH, and it is also capable to correctly identify the physiological mechanism related to exercise intolerance, differentiating the limitation caused by pulmonary vasculopathy from the ventilatory limitation related to ILD. This procedure has not been properly studied in patients with ILDs. Therefore, studies using iCPET in this population may provide further pathophysiological knowledge in this area, and may help to identify subgroups of patients with potential benefit for early PH intervention, and may help identify patients for lung transplantation prioritization. (AU)

Scientific publications

(9)

(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)

OLIVEIRA, RUDOLF K. F.; WAXMAN, AARON B.; HOOVER, PAUL J.; DELLARIPA, PAUL F.; SYSTROM, DAVID M. Pulmonary Vascular and Right Ventricular Burden During Exercise in Interstitial Lung Disease. CHEST, v. 158, n. 1, p. 350-358, JUL 2020. Web of Science Citations: 0.

FARIA-URBINA, MARIANA; OLIVEIRA, RUDOLF K. F.; AGARWAL, MANYOO; WAXMAN, AARON B. Inhaled Treprostinil in Pulmonary Hypertension Associated with Lung Disease. Lung, v. 196, n. 2, p. 139-146, APR 2018. Web of Science Citations: 1.

OLDHAM, WILLIAM M.; OLIVEIRA, RUDOLF K. F.; WANG, RUI-SHENG; OPOTOWSKY, ALEXANDER R.; RUBINS, DAVID M.; HAINER, JON; WERTHEIM, BRADLEY M.; ALBA, GEORGE A.; CHOUDHARY, GAURAV; TORNYOS, ADRIENN; MACRAE, CALUM A.; LOSCALZO, JOSEPH; LEOPOLD, JANE A.; WAXMAN, AARON B.; OLSCHEWSKI, HORST; KOVACS, GABOR; SYSTROM, DAVID M.; MARON, BRADLEY A. Network Analysis to Risk Stratify Patients With Exercise Intolerance. Circulation Research, v. 122, n. 6, p. 864+, MAR 16 2018. Web of Science Citations: 9.

HUANG, WEI; OLIVEIRA, RUDOLF K. F.; LEI, HAN; SYSTROM, DAVID M.; WAXMAN, AARON B. Pulmonary Vascular Resistance During Exercise Predicts Long-Term Outcomes in Heart Failure With Preserved Ejection Fraction. JOURNAL OF CARDIAC FAILURE, v. 24, n. 3, p. 169-176, MAR 2018. Web of Science Citations: 6.

FARIA-URBINA, MARIANA; OLIVEIRA, RUDOLF K. F.; SEGRERA, SERGIO A.; LAWLER, LAURIE; WAXMAN, AARON B.; SYSTROM, DAVID M. Impaired systemic oxygen extraction in treated exercise pulmonary hypertension: a new engine in an old car?. PULMONARY CIRCULATION, v. 8, n. 1 JAN 24 2018. Web of Science Citations: 2.

HUANG, WEI; RESCH, STEPHEN; OLIVEIRA, RUDOLF K. F.; COCKRILL, BARBARA A.; SYSTROM, DAVID M.; WAXMAN, AARON B. Invasive cardiopulmonary exercise testing in the evaluation of unexplained dyspnea: Insights from a multidisciplinary dyspnea center. EUROPEAN JOURNAL OF PREVENTIVE CARDIOLOGY, v. 24, n. 11, p. 1190-1199, JUL 2017. Web of Science Citations: 23.

VAN RIEL, ANNELIEKE C. M. J.; SYSTROM, DAVID M.; OLIVEIRA, RUDOLF K. F.; LANDZBERG, MICHAEL J.; MULDER, BARBARA J. M.; BOUMA, BERTO J.; MARON, BRADLEY A.; SHAH, AMIL M.; WAXMAN, AARON B.; OPOTOWSKY, ALEXANDER R. Hemodynamic and metabolic characteristics associated with development of a right ventricular outflow tract pressure gradient during upright exercise. PLoS One, v. 12, n. 6 JUN 21 2017. Web of Science Citations: 2.

OLIVEIRA, RUDOLF K. F.; WAXMAN, AARON B.; AGARWAL, MANYOO; ESLAM, ROZA BADR; SYSTROM, DAVID M. Pulmonary haemodynamics during recovery from maximum incremental cycling exercise. European Respiratory Journal, v. 48, n. 1, p. 158-167, JUL 2016. Web of Science Citations: 14.

OLIVEIRA, RUDOLF K. F.; AGARWAL, MANYOO; TRACY, JULIE A.; KARIN, ABBEY L.; OPOTOWSKY, ALEXANDER R.; WAXMAN, AARON B.; SYSTROM, DAVID M. Age-related upper limits of normal for maximum upright exercise pulmonary haemodynamics. European Respiratory Journal, v. 47, n. 4, p. 1179-1188, APR 2016. Web of Science Citations: 30.