[Etiological diagnosis of pulmonary hypertension: A cause of difficult diagnosis]

Rev Mal Respir. 2019 Mar;36(3):350-354. doi: 10.1016/j.rmr.2018.11.004. Epub 2018 Nov 22.
[Article in French]

Abstract

Introduction: Schistosomiasis associated pulmonary arterial hypertension belongs to group 1 of the pulmonary hypertension classification and should be considered in any patient with pulmonary hypertension returning from an endemic area.

Case report: A 17-year-old patient was hospitalized for pulmonary hypertension detected during the initial assessment of viral hepatitis B-related cirrhosis with portal hypertension. The initial assessment established the diagnosis of pulmonary hypertension secondary to viral hepatitis B-cirrhosis. The patient's hepatic and haemodynamic condition deteriorated and he was treated with intravenous epoprostenol. This allowed subsequent performance of a liver transplantation. Epoprostenol could then be discontinued. Unexpectedly, histology of the liver explant revealed florid schistosomiasis in addition to hepatitis B cirrhosis.

Conclusion: The diagnosis of pulmonary arterial hypertension associated with schistosomiasis may be difficult. It is necessary to repeat the serological studies and, sometimes, to obtain a rectal biopsy. The treatment of pulmonary arterial hypertension associated with schistosomiasis is based on specific therapies and antiparasitic treatment.

Keywords: Hypertension artérielle pulmonaire; Hypertension portale; Hypertension pulmonaire; Liver transplantation; Portal hypertension; Pulmonary arterial hypertension; Pulmonary hypertension; Schistosomiase; Schistosomiasis; Transplantation hépatique.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Animals
  • Diagnosis, Differential
  • Epoprostenol / administration & dosage
  • Hepatitis B / complications
  • Hepatitis B / diagnosis
  • Hepatitis B / parasitology
  • Hepatitis B / therapy
  • Humans
  • Hypertension, Portal / diagnosis
  • Hypertension, Portal / parasitology
  • Hypertension, Portal / therapy
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / parasitology
  • Hypertension, Pulmonary / therapy
  • Liver Transplantation
  • Male
  • Praziquantel / administration & dosage
  • Schistosoma mansoni
  • Schistosomiasis mansoni / complications*
  • Schistosomiasis mansoni / diagnosis*
  • Schistosomiasis mansoni / therapy

Substances

  • Praziquantel
  • Epoprostenol