Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic Derangements

Pulmonary hypertension (PH) associated with Whipple’s disease (WD-PH) is extremely rare, and the underlying pathophysiological processes are incompletely understood. Alterations in hemodynamics can be severe, with right ventricular (RV) dysfunction being common. A case involving a 23-year-old man wi...

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Main Authors: Salah Najm, Joud Hajjar, Robert P Nelson, Ramana S Moorthy, Karen M Wolf, Tim Lahm
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Canadian Respiratory Journal
Online Access:http://dx.doi.org/10.1155/2011/365645
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author Salah Najm
Joud Hajjar
Robert P Nelson
Ramana S Moorthy
Karen M Wolf
Tim Lahm
author_facet Salah Najm
Joud Hajjar
Robert P Nelson
Ramana S Moorthy
Karen M Wolf
Tim Lahm
author_sort Salah Najm
collection DOAJ
description Pulmonary hypertension (PH) associated with Whipple’s disease (WD-PH) is extremely rare, and the underlying pathophysiological processes are incompletely understood. Alterations in hemodynamics can be severe, with right ventricular (RV) dysfunction being common. A case involving a 23-year-old man with WD-PH who exhibited a dramatic vasodilator response during right heart catheterization despite severely altered pulmonary hemodynamics and concomitant RV dysfunction is reported. While the patient’s symptoms responded poorly to treatment with nifedipine and sildenafil, significant improvement in dyspnea, RV dysfunction and pulmonary pressures were noted following antibiotic therapy. The present report highlights that despite severely elevated pulmonary artery pressures and RV dysfunction in WD-PH patients, a highly significant vasodilator response and dramatic improvement with antibiotic therapy may be observed. Furthermore, the case highlights the phenomenon of PH in the setting of inflammation, suggesting that adequate control of the inflammatory response can be accompanied by a marked improvement in hemodynamics in certain types of PH.
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spelling doaj-art-df38ba1fd0634a3e8f47539e6e5e50602025-02-03T05:51:48ZengWileyCanadian Respiratory Journal1198-22412011-01-01185e70e7210.1155/2011/365645Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic DerangementsSalah Najm0Joud Hajjar1Robert P Nelson2Ramana S Moorthy3Karen M Wolf4Tim Lahm5Department of Medicine, St Vincent Hospital Indianapolis, USADepartment of Medicine, St Vincent Hospital Indianapolis, USADepartment of Medicine, St Vincent Hospital Indianapolis, USAAssociated Vitreoretinal and Uveitis Consultants Indianapolis, St Vincent Hospital Indianapolis, and Department of Ophthalmology, USADepartment of Medicine, St Vincent Hospital Indianapolis, USADepartment of Medicine, St Vincent Hospital Indianapolis, USAPulmonary hypertension (PH) associated with Whipple’s disease (WD-PH) is extremely rare, and the underlying pathophysiological processes are incompletely understood. Alterations in hemodynamics can be severe, with right ventricular (RV) dysfunction being common. A case involving a 23-year-old man with WD-PH who exhibited a dramatic vasodilator response during right heart catheterization despite severely altered pulmonary hemodynamics and concomitant RV dysfunction is reported. While the patient’s symptoms responded poorly to treatment with nifedipine and sildenafil, significant improvement in dyspnea, RV dysfunction and pulmonary pressures were noted following antibiotic therapy. The present report highlights that despite severely elevated pulmonary artery pressures and RV dysfunction in WD-PH patients, a highly significant vasodilator response and dramatic improvement with antibiotic therapy may be observed. Furthermore, the case highlights the phenomenon of PH in the setting of inflammation, suggesting that adequate control of the inflammatory response can be accompanied by a marked improvement in hemodynamics in certain types of PH.http://dx.doi.org/10.1155/2011/365645
spellingShingle Salah Najm
Joud Hajjar
Robert P Nelson
Ramana S Moorthy
Karen M Wolf
Tim Lahm
Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic Derangements
Canadian Respiratory Journal
title Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic Derangements
title_full Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic Derangements
title_fullStr Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic Derangements
title_full_unstemmed Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic Derangements
title_short Whipple’s Disease-Associated Pulmonary Hypertension with Positive Vasodilator Response Despite Severe Hemodynamic Derangements
title_sort whipple s disease associated pulmonary hypertension with positive vasodilator response despite severe hemodynamic derangements
url http://dx.doi.org/10.1155/2011/365645
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