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Case Reports: Rare disease
Portopulmonary hypertension: a patient with shortness of breath
  1. Aidan Ryan1,
  2. Nahima Miah1 and
  3. Mohamed Saleh2
  1. 1Medway Maritime Hospital, Gillingham, Kent, UK
  2. 2Gastroenterology, Medway Maritime Hospital, Gillingham, Kent, UK
  1. Correspondence to Dr Aidan Ryan; aidan.ryan1{at}nhs.net

Abstract

Portopulmonary hypertension (PoPH) is a poorly understood complication of liver disease which affects about 10% of patients with pulmonary hypertension. This case report outlines the difficulties in diagnosing and managing a patient with advanced disease, and the impact of these delays on the patient.

PoPH has a significant risk of mortality with a 2-year survival rate of 67%. There are also few treatment options available and those which do exist are associated with multiple contraindications and risks. Patients with PoPH commonly present with dyspnoea, pulmonary hypertension and portal hypertension. The presence of coexisting chronic liver disease is also sometimes present. Traditional management for heart failure can temporarily alleviate symptoms but there is no proven long-term benefit. As a result, an understanding of the pathophysiology, diagnostics and management is crucial to ensure the best possible patient outcomes.

  • Portal hypertension
  • Pulmonary hypertension

https://doi.org/10.1136/bcr-2021-244803

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    Footnotes

    • Contributors The article was researched and written by Dr AR and Dr NM, with the supervision of Dr MS, who assisted with edits and guidance.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.