title: Non-invasive screening for pulmonary hypertension in idiopathic pulmonary fibrosis.
abstract: Pulmonary hypertension (PH) is a common complication of idiopathic pulmonary fibrosis (IPF) that is associated with poor prognosis. Noninvasive screening for PH in IPF patients is challenging and a combination of several noninvasive determinations can improve discrimination.
  
  
  
  We included 235 IPF patients who underwent right heart catheterization (RHC) as part of the lung transplant evaluation. We measured electrocardiographic (ECG) and echocardiographic variables as well as the pulmonary artery (PA) and ascending aorta (AA) diameters on chest CT. We recorded results of arterial blood gases (ABG), pulmonary function (PFT) and 6-min walk tests (6MWT).
  
  
  
  Several variables were predictors of PH in IPF patients in univariable models including a lower arterial oxygenation and 6MWT distance; worse right ventricular (RV) function, rightward deviation of the QRS axis and a higher FVC/DLCOc ratio, PA/AA diameter ratio, and estimated RV systolic pressure. In multivariable analysis, a worse RV function and higher PA/AA ratio remained predictors of PH (c-index 0.75 (0.65-0.84)). Similarly, a worse RV function, a higher PA/AA ratio and a rightward QRS axis deviation were independent predictors of precapillary PH (c-index 0.86 (0.76-0.92)). A combination of PA/AA diameter ratio <1.1, a QRS axis <90° and normal RV function showed a negative predictive value of 85% for precapillary PH.
  
  
  
  There are significant differences in ECG, echocardiographic, chest CT, PFT and ABG parameters between IPF patients with and without PH. However, these noninvasive tests alone or combination have limited discrimination ability for PH screening in IPF.
authors: Department of Internal Medicine, Cleveland Clinic, Cleveland, OH, USA. Electronic address: alkukhl@ccf.org.
authors: Laith
authors: L
authors: Alkukhun
authors: Respiratory Institute Biostatistics Core, Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, USA. Electronic address: wangx6@ccf.org.
authors: Xiao-Feng
authors: XF
authors: Wang
authors: Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA; Department of Chest Diseases, Faculty of Medicine, Assiut University, Assiut, Egypt. Electronic address: Ahmedm2@ccf.org.
authors: Mostafa K
authors: MK
authors: Ahmed
authors: Department of Pulmonary Medicine, Billings Clinic, Billings, MT, USA. Electronic address: mbaumgartner@billingsclinic.org.
authors: Manfred
authors: M
authors: Baumgartner
authors: Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address: budevm@ccf.org.
authors: Marie M
authors: MM
authors: Budev
authors: Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address: dweikr@ccf.org.
authors: Raed A
authors: RA
authors: Dweik
authors: Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address: tonella@ccf.org.
authors: Adriano R
authors: AR
authors: Tonelli
mesh_terms: D000368:Aged; D001011:Aorta / Q000000981:diagnostic imaging; D001784:Blood Gas Analysis / Q000379:methods; D006328:Cardiac Catheterization; D004452:Echocardiography / Q000379:methods; D004562:Electrocardiography / Q000379:methods; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D016040:Lung Transplantation / Q000592:standards*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D012129:Respiratory Function Tests / Q000379:methods; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods; D016278:Ventricular Function, Right / Q000502:physiology; D000070857:Walk Test / Q000379:methods
keywords: Computed tomography scan; Echocardiography; Electrocardiogram; Idiopathic pulmonary fibrosis; Pulmonary hypertension