DOI: 10.12890/2026_006600

Whipple’s disease complicated by severe pulmonary hypertension and right ventricular failure

Authors

  • Marwan Joma Department of Internal Medicine, University of Toledo College of Medicine and Life Sciences, Toledo, USA
  • Abdulrahman M. Karam Department of Internal Medicine, University of Toledo College of Medicine and Life Sciences, Toledo, USA
  • Bisher Sawaf Department of Internal Medicine, University of Toledo College of Medicine and Life Sciences, Toledo, USA
  • Wasef Sayeh Division of Gastroenterology, University of Toledo College of Medicine and Life Sciences, Toledo, USA
  • Waqar Khan Department of Pathology, ProMedica Toledo Hospital, Toledo, USA
  • Fadi Safi Division of Pulmonary and Critical Care Medicine, Department of Medicine, The University of Toledo, Toledo, USA
  • Yaseen Alastal Division of Gastroenterology, University of Toledo College of Medicine and Life Sciences, Toledo, USA

abdulrahman.karam@outlook.com

Keywords

Whipple’s disease, pulmonary hypertension, ventricular failure

Abstract

Whipple’s disease is a rare, chronic systemic infection caused by Tropheryma whipplei that commonly presents with gastrointestinal manifestations but may involve multiple extraintestinal organs. Cardiac involvement, particularly culture-negative endocarditis, is described; however, pulmonary complications such as pulmonary hypertension (PH) are uncommon. We report a case of Whipple’s disease presenting with severe pre-capillary pulmonary hypertension and right ventricular (RV) failure with reversibility following antimicrobial therapy. A 62-year-old woman with seropositive rheumatoid arthritis on adalimumab, presented with one month of profuse watery diarrhoea, abdominal pain, dizziness and weakness. Imaging revealed diffuse mesenteric and para-aortic lymphadenopathy. Upper endoscopy demonstrated oedematous whitish granular villi in the duodenum, and biopsies showed periodic acid–Schiff–positive, acid-fast bacillus–negative foamy macrophages diagnostic of Whipple’s disease. Transthoracic echocardiography revealed RV dilation and dysfunction with pulmonary hypertension, which was confirmed on right heart catheterisation as severe pre-capillary PH with elevated pulmonary vascular resistance and normal wedge pressure. The patient developed RV-predominant cardiogenic shock requiring inotropic and vasopressor support. Following initiation of intravenous ceftriaxone her haemodynamics improved rapidly, with normalisation of cardiac output and marked reduction in pulmonary pressures on repeat catheterisation. Gastrointestinal symptoms resolved, immunosuppressive therapy was discontinued, and she was discharged on prolonged antimicrobial therapy. This case underscores PH as a rare but potentially reversible manifestation of Whipple’s disease. Recognition of this entity is critical, as PH-specific vasodilator therapy alone is often ineffective, whereas early targeted antimicrobial treatment can result in rapid and profound clinical and haemodynamic recovery, even in cases presenting with severe RV failure.

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References

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Published

2026-04-30

Issue

Vol. 13 No. 5 (2026)

Categories

How to Cite

1.
Joma M, Karam AM, Sawaf B, Sayeh W, Khan W, Safi F, et al. Whipple’s disease complicated by severe pulmonary hypertension and right ventricular failure. EJCRIM [Internet]. 2026 Apr. 30 [cited 2026 Nov. 3];13(5). Available from: https://ejcrim.com/index.php/ejcrim/article/view/6600

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