Whipple's disease-associated pulmonary hypertension with positive vasodilator response despite severe hemodynamic derangements

Salah Najm, Joud Hajjar, Robert Nelson, Ramana S. Moorthy, Karen M. Wolf, Tim Lahm

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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.

Original languageEnglish
JournalCanadian Respiratory Journal
Volume18
Issue number5
StatePublished - Sep 2011

Fingerprint

Whipple Disease
Vasodilator Agents
Pulmonary Hypertension
Right Ventricular Dysfunction
Hemodynamics
Anti-Bacterial Agents
Pressure
Lung
Nifedipine
Cardiac Catheterization
Dyspnea
Pulmonary Artery
Therapeutics
Inflammation

Keywords

  • Cytokines
  • Hemodynamics
  • Inflammation
  • Right ventricular dysfunction
  • Tropheryma whipplei

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Whipple's disease-associated pulmonary hypertension with positive vasodilator response despite severe hemodynamic derangements. / Najm, Salah; Hajjar, Joud; Nelson, Robert; Moorthy, Ramana S.; Wolf, Karen M.; Lahm, Tim.

In: Canadian Respiratory Journal, Vol. 18, No. 5, 09.2011.

Research output: Contribution to journalArticle

@article{e4ee361eb7c7495c9aa672df8fc8dcff,
title = "Whipple's disease-associated pulmonary hypertension with positive vasodilator response despite severe hemodynamic derangements",
abstract = "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.",
keywords = "Cytokines, Hemodynamics, Inflammation, Right ventricular dysfunction, Tropheryma whipplei",
author = "Salah Najm and Joud Hajjar and Robert Nelson and Moorthy, {Ramana S.} and Wolf, {Karen M.} and Tim Lahm",
year = "2011",
month = "9",
language = "English",
volume = "18",
journal = "Canadian Respiratory Journal",
issn = "1198-2241",
publisher = "Pulsus Group Inc.",
number = "5",

}

TY - JOUR

T1 - Whipple's disease-associated pulmonary hypertension with positive vasodilator response despite severe hemodynamic derangements

AU - Najm, Salah

AU - Hajjar, Joud

AU - Nelson, Robert

AU - Moorthy, Ramana S.

AU - Wolf, Karen M.

AU - Lahm, Tim

PY - 2011/9

Y1 - 2011/9

N2 - 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.

AB - 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.

KW - Cytokines

KW - Hemodynamics

KW - Inflammation

KW - Right ventricular dysfunction

KW - Tropheryma whipplei

UR - http://www.scopus.com/inward/record.url?scp=84860449078&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84860449078&partnerID=8YFLogxK

M3 - Article

C2 - 21969933

AN - SCOPUS:84860449078

VL - 18

JO - Canadian Respiratory Journal

JF - Canadian Respiratory Journal

SN - 1198-2241

IS - 5

ER -