Pulmonary vascular dysfunction without pulmonary hypertension: A distinct phenotype in idiopathic pulmonary fibrosis

Author:

Nathan Steven D.1,Tehrani Benham2,Zhao Qiong2,Arias Rafael2,Kim Dennis3,Pellegrini Antonia1,Collins Ashley Claire1,Diviney Jack1,Chakravorty Shourjo3,Khangoora Vikramjit1,Shlobin Oksana A.1,Thomas Christopher1,Lavon Ben R.4,King Christopher S.1ORCID,Chandel Abhimanyu5ORCID

Affiliation:

1. Advanced Lung Disease and Transplant Program, Inova Heart and Vascular Institute Inova Fairfax Hospital Falls Church Virginia USA

2. Cardiology Department, Inova Heart and Vascular Institute Inova Fairfax Hospital Falls Church Virginia USA

3. Department of Medicine Inova Fairfax Hospital Falls Church Virginia USA

4. FLUIDDA New York New York USA

5. Department of Pulmonary and Critical Care Walter Reed National Military Medical Center Bethesda Maryland USA

Abstract

AbstractPulmonary vascular dysfunction in the absence of pulmonary hypertension (PH) has been observed in patients with idiopathic pulmonary fibrosis (IPF). We describe the prevalence and etiology of elevated pulmonary vascular resistance (PVR) without PH among patients with IPF. Hemodynamic, echocardiographic, and functional respiratory imaging (FRI) data was compared between patients with IPF without PH with normal (<3 wood units) and elevated PVR (≥3 wood units). Mortality between these two groups were compared to patients with IPF and PH. Of 205 patients with IPF, there were 146 patients without PH, of whom 114 (78.1%) had a normal PVR and 32 (21.9%) who had a high PVR. Functional testing and hemodynamics were similar in the two groups, except for the cardiac index which was significantly lower in patients with a high PVR (2.3 vs. 2.6 L/min/m2; p = 0.004). Echocardiographic comparison demonstrated a higher tricuspid regurgitant velocity in those with a high PVR (3.4 vs 3.0 m/s; p = 0.046). FRI revealed proportionately fewer large vessels as a proportion of the vasculature in the patients without PH and elevated PVRs. Among patients without PH, PVR was associated with increased mortality. In conclusion, patients with IPF without PH but a high PVR appear to be a distinct phenotype with a prognosis between those with and without PH, likely reflecting the continuum of vascular dysfunction. The basis for this unique hemodynamic profile could not be definitively discerned although FRI suggested an aberrant anatomical vascular response.

Publisher

Wiley

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