Pulmonary Vascular Disease: Fellow Case Report Slide: Pulmonary Disorders |

Successful Treatment of Idiopathic Pulmonary Vasculopathy With Targeted Pulmonary Vasodilator Therapy FREE TO VIEW

Mariam Anis, MD; Kathryn Wikenheiser-Brokamp, PhD; Jean Elwing, MD
Author and Funding Information

University of Cincinnati Medical Center, Cincinnati, OH

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1141A. doi:10.1016/j.chest.2016.08.1251
Text Size: A A A
Published online

SESSION TITLE: Fellow Case Report Slide: Pulmonary Disorders

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Tuesday, October 25, 2016 at 04:30 PM - 05:30 PM

INTRODUCTION: Pulmonary vasculopathy is associated with a diverse group of diseases that manifest with various histopathological features. We describe a patient with idiopathic pulmonary arteriopathy and normal hemodynamics on right heart catheterization (RHC) who benefitted markedly from bosentan and subsequent tadalafil with resolution of chronic hypoxic respiratory failure.

CASE PRESENTATION: A 44 year old African American female with a history of hypertension and fibromyalgia presented to our center in 2006 for evaluation of progressive dyspnea since 2001. She had been treated with methotrexate and prednisone without improvement. Pulmonary function tests (PFTs) revealed moderate restriction with severely reduced diffusion capacity (DLCO). No parenchymal pathology or lymphadenopathy was found on a high resolution CT scan. CT angiogram was negative for pulmonary embolism. Extensive cardiac and rheumatologic workup was negative. Patient endorsed NYHA class IV symptoms requiring >10 liters of oxygen. Open lung biopsy revealed pulmonary arteriopathy characterized by muscularization of arterioles and concentric mural thickening of muscular arteries with muscular hypertrophy and intimal expansion. No venous pathology or thromboembolic disease was identified. RHC showed normal hemodynamics. Due to progressive respiratory failure, she was trialed on bosentan with resultant rapid and sustained resolution of hypoxia. In 2009 an interruption in therapy led to clinical deterioration. She improved with reinitiation. She remained well until 2014 when she developed increased dyspnea. Due to concern for waning bosentan effect, she was transitioned to tadalafil with full recovery. She continues to do well without supplemental oxygen requirement.

DISCUSSION: Pulmonary arteriopathy is well described with cardiopulmonary diseases. It involves muscular arteries accompanying bronchioles and arterioles within alveolar septae. Etiologic considerations include pulmonary vascular disease, cardiac abnormalities, parenchymal lung disease, thromboembolic disease, drug reactions and connective tissue disease.

CONCLUSIONS: We present the first case of idiopathic pulmonary vascular disease unexplained by a systemic condition with a remarkable and sustained response to pulmonary vascular targeted therapy. Isolated pulmonary vasculopathy should be considered in unexplained hypoxic respiratory failure.

Reference #1: Pietra GG, Capron F et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol. 2004;43:(12 Suppl S):25S-32S.

DISCLOSURE: The following authors have nothing to disclose: Mariam Anis, Kathryn Wikenheiser-Brokamp, Jean Elwing

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543