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

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

Research output: Contribution to journalArticle

4 Scopus citations

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 (US)
Pages (from-to)e70-e72
JournalCanadian Respiratory Journal
Volume18
Issue number5
DOIs
StatePublished - 2011

Keywords

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

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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