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.
authors.affiliation: Department of Medicine, St Vincent Hospital, Indianapolis, Indiana, USA.
authors.forename: Salah
authors.initials: S
authors.lastname: Najm
authors.forename: Joud
authors.initials: J
authors.lastname: Hajjar
authors.forename: Robert P
authors.initials: RP
authors.lastname: Nelson
authors.forename: Ramana S
authors.initials: RS
authors.lastname: Moorthy
authors.forename: Karen M
authors.initials: KM
authors.lastname: Wolf
authors.forename: Tim
authors.initials: T
authors.lastname: Lahm
mesh_terms: D001993:Bronchodilator Agents / Q000494:pharmacology; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D009569:Nitric Oxide / Q000494:pharmacology; D014664:Vasodilation / Q000187:drug effects; D018497:Ventricular Dysfunction, Right / Q000503:physiopathology; D008061:Whipple Disease / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D055815:Young Adult
chemical_list: D001993:Bronchodilator Agents; D009569:Nitric Oxide