Skip to main content

Main menu

  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us

Clinical Medicine Journal

  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

User menu

  • Log in

Search

  • Advanced search
RCP Journals
Home
  • Log in
  • Home
  • Our journals
    • Clinical Medicine
    • Future Healthcare Journal
  • Subject collections
  • About the RCP
  • Contact us
Advanced

Clinical Medicine Journal

clinmedicine Logo
  • ClinMed Home
  • Content
    • Current
    • Ahead of print
    • Archive
  • Author guidance
    • Instructions for authors
    • Submit online
  • About ClinMed
    • Scope
    • Editorial board
    • Policies
    • Information for reviewers
    • Advertising

When common things aren't so common: A case report of hepatopulmonary syndrome

Emma Keelan, Chris Ferguson and Rebecca Kerr
Download PDF
DOI: https://doi.org/10.7861/clinmed.2021-0142
Clin Med May 2021
Emma Keelan
ABelfast City Hospital, Belfast, UK
Roles: ST6 respiratory medicine
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: ekeelan01@qub.ac.uk
Chris Ferguson
ABelfast City Hospital, Belfast, UK
Roles: ST6 respiratory medicine
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Rebecca Kerr
BBelfast City Hospital, Belfast, UK
Roles: foundation trainee year 2
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
Loading

ABSTRACT

A 60-year-old smoker with a history of liver cirrhosis and chronic obstructive pulmonary syndrome (COPD) presented with hypoxic respiratory failure. This was felt secondary to an exacerbation of COPD. Despite treatment, the patient required 10 L of oxygen to achieve saturations of 88% on ambulation. Interstitial lung disease, pulmonary emboli and pulmonary hypertension were excluded as potential aetiologies of hypoxia. Given the history of cirrhosis, hepatopulmonary syndrome was postulated. Contrast echocardiography suggested an extracardiac shunt; a technetium-99m macroaggregated albumin scan confirmed the diagnosis.

KEYWORDS:
  • hypoxia
  • hepatopulmonary syndrome (HPS)
  • shunt
  • cirrhosis

Case presentation

A 60-year-old woman with a 30 pack-year smoking history presented with increasing shortness of breath over 4 weeks. Her past medical history included hypertension, chronic obstructive pulmonary syndrome (COPD) and alcoholic liver disease with cirrhosis. Chest X-ray demonstrated bilateral lower zone infiltrates. Inflammatory indices were normal. The patient remained hypoxic on 15 L oxygen, requiring admission to intensive care for high flow nasal oxygen and ultimately intubation.

A computed tomography pulmonary angiography demonstrated no pulmonary emboli but noted marked emphysema. No growth was seen on blood and sputum cultures. Bronchoalveolar lavage excluded atypical pathogens including mycobacterium. Transthoracic echocardiography showed normal right ventricular and left ventricular function, with no evidence of valvular disease or pulmonary hypertension. Antibiotics and oral steroids were commenced despite normal inflammatory indices.

The patient was extubated after 4 days and discharged to the respiratory ward, where finger clubbing, spider naevi and fine bibasal crepitations were noted on examination. Oxygen saturations were 87% on room air and 90% on 3 L oxygen. Serum eosinophils, N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, ACE level, extrinsic allergic alveolitis screen, rheumatoid factor, creatine kinase, hepatitis and vasculitic screen were normal. FEV1 was 1.76 L (92% predicted), forced vital capacity (FVC) 2.65 L (103% predicted), with an obstructive ratio of 66%. Repeat chest X-ray raised the possibility of basal reticulonodular change.

Differential diagnosis

A COPD exacerbation was felt to be the most likely aetiology of the patient's respiratory failure, yet the persistent hypoxia, finger clubbing and reticulonodular changes on X-ray necessitated the exclusion of interstitial lung disease (ILD). High-resolution computed tomography showed no established fibrosis but ground glass opacities in the lower lobes, felt to represent resolving inflammatory change. The ILD multidisciplinary meeting advised empirical treatment with oral steroid and diuretics, yet the patient remained hypoxic and, on ambulatory assessment, walked 30 m before desaturating to SaO2 75% on 10 L oxygen.

Although bearing in mind the dictum that ‘common things are common’, the team was uneasy regarding the patient's diagnosis as the degree of hypoxia appeared out of keeping with the severity of emphysema. This prompted a review of the case and, given the presence of cirrhosis, hepatopulmonary syndrome was postulated. Oxygen saturations were assessed supine and standing, at 90% and 79% on room air, respectively, confirming platypnoea–orthodeoxia syndrome. Contrast echocardiography confirmed an extracardiac shunt with opacification of the left heart chambers after seven cardiac cycles (Fig 1), confirming hepatopulmonary syndrome. A hepatology referral was made for consideration of a liver transplant and the patient was discharged on long-term oxygen (LTOT).

Fig 1.
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig 1.

Contrast echocardiography showing extracardiac shunt with opacification of the left heart chambers after seven cardiac cycles, confirming hepatopulmonary syndrome.

Outcome and follow-up

During transplant work-up, concern was expressed regarding the extent of the patient's emphysema, with her degree of hypoxaemia considered an unreliable marker of HPS severity. A technetium-99m macroaggregated albumin (TcMAA) study was completed, revealing a shunt fraction of 30.7%. Despite the degree of shunt, our patient was declined for liver transplant.

Discussion

HPS is a rare cause of hypoxaemia, reported in 4–32% of patients with liver disease.1 Characterised by liver disease, intrapulmonary vascular dilatations (IPVDs) and arterial de-oxygenation in the absence of other cardiorespiratory abnormalities, HPS can be subtyped into type 1 and type 2. Type 1 patients typically display smaller diffuse vascular dilations while type 2 patients have larger, discrete IPVDs comparable to arteriovenous malformations (AVMs). True AVMs are rare. The driver of pulmonary dilatation has been postulated as nitric oxide; however, the exact cause remains elusive.2

When considering a diagnosis of HPS it is useful to assess for orthodeoxia (defined as a SpO2 decrease of ≥ 5% from supine to standing).3 This decrease in SpO2 occurs due to V/Q mismatching from the redirection of blood to the lung bases when standing, an area where IPVDs predominate. Orthodeoxia can also be identified using arterial blood gas analysis with a decrease of ≥ 0.5 kPa in PaO2 from supine to upright diagnostic. Yet the European Respiratory Society (ERS) caution against using PaO2 alone when diagnosing HPS, as this may underestimate oxygen impairment due to reflex hyperventilation.4 Consequently the A-a oxygen gradient is recommended, with a gradient of ≥2 kPa when upright on room air (or 2.7 kPa in those aged over 65) the defined cut-off.4,5

Investigations

Patients with suspected HPS should receive chest imaging and pulmonary function testing to exclude other causes of hypoxia. Spirometry is typically normal (unless comorbid respiratory disease exists). TLCO is usually impaired. Chest X-rays are often normal. Transthoracic contrast echocardiography is recommended by the ERS as the first-line investigation.5 In those with HPS and a right-to-left shunt, agitated saline causes opacification in the left and right heart chambers as the bubbles bypass the pulmonary capillary bed.

For patients with coexistent cardiorespiratory disease, it may be useful to quantify the degree of shunt using radionuclide perfusion scanning with TcMAA. In healthy subjects, the TcMAA is filtered by the pulmonary capillaries, whereas in HPS the particles bypass the lungs, becoming trapped in the brain and kidneys. The proportion of TcMAA bypassing the lungs is used to quantify the extent of the shunt. The need for invasive pulmonary angiography is rare, as contrast CT often excludes larger IPVDs; as such, it is only advised when attempting to identify type 2 patients with IPVDs amenable to embolisation.6

Treatment

HPS is a progressive disease associated with impaired quality of life. Untreated, mortality is twice that of cirrhotic patients without HPS.

While not a cure, LTOT is the first-line supportive therapy. Liver transplantation remains the only definitive treatment, with 85% of patients demonstrating resolution of vascular dilatations and normalisation of oxygenation at 1 year post-transplant.7 Severe hypoxaemia and a pre-transplant shunt fraction >20% on TcMAA confers greater transplant mortality.7,8 In light of the poor prognosis with HPS and favourable transplant outcomes, the International Liver Transplant Society recommend expedited transplant referrals in patients with severe HPS.

Summary

  • Hepatopulmonary syndrome (HPS) is a rare cause of hypoxaemia.

  • The diagnostic difficulty surrounding the condition means it is often diagnosed late in the disease course.

  • When considering a diagnosis of HPS it is useful to assess for orthodeoxia.

It is important for physicians to re-evaluate a patient's diagnosis when the clinical picture does not adequately correlate with the patient's symptoms and signs.

  • © Royal College of Physicians 2021. All rights reserved.

References

  1. ↵
    1. Tumgor G
    . Cirrhosis and hepatopulmonary syndrome. World J Gastroenterol 2014;20:2586–94.
    OpenUrl
  2. ↵
    1. Nunes H
    , Lebrec D, Mazmanian M, et al. Role of nitric oxide in hepatopulmonary syndrome in cirrhotic rats. Am J Respir Crit Care Med 2001;164:879–85.
    OpenUrlCrossRefPubMed
  3. ↵
    1. Grilo-Bensusan I
    , Pascasio-Acevedo JM. Hepatopulmonary syndrome: What we know and what we would like to know. World J Gastroenterol 2016;22:5728–41.
    OpenUrl
  4. ↵
    1. Rodríguez-Roisin R
    , Krowka MJ, Hervé P, et al. Pulmonary–hepatic vascular disorders (PHD). Eur Respir J 2004;24:861–80.
    OpenUrlFREE Full Text
  5. ↵
    1. Porres-Aguilar M
    , Altamirano JT, Torre-Delgadillo A, et al. Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview. Eur Respir Rev 2012;21:223–33.
    OpenUrlAbstract/FREE Full Text
  6. ↵
    1. Poterucha JJ
    , Krowka MJ, Dickson ER, et al. Failure of hepatopulmonary syndrome to resolve after liver transplantation and successful treatment with embolotherapy. Hepatology 1995;21:96–100.
    OpenUrlCrossRefPubMed
  7. ↵
    1. Swanson KL
    , Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 2005;41:1122–9.
    OpenUrlCrossRefPubMed
  8. ↵
    1. Arguedas MR
    , Abrams GA, Krowka MJ, et al. Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation. Hepatology 2003;37:192–7.
    OpenUrlCrossRefPubMed
Back to top
Previous articleNext article

Article Tools

Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Citation Tools
When common things aren't so common: A case report of hepatopulmonary syndrome
Emma Keelan, Chris Ferguson, Rebecca Kerr
Clinical Medicine May 2021, 21 (3) 226-227; DOI: 10.7861/clinmed.2021-0142

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
When common things aren't so common: A case report of hepatopulmonary syndrome
Emma Keelan, Chris Ferguson, Rebecca Kerr
Clinical Medicine May 2021, 21 (3) 226-227; DOI: 10.7861/clinmed.2021-0142
del.icio.us logo Digg logo Reddit logo Twitter logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • ABSTRACT
    • Case presentation
    • Differential diagnosis
    • Outcome and follow-up
    • Discussion
    • Summary
    • References
  • Figures & Data
  • Info & Metrics

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • A case of antisynthetase syndrome presenting solely with life-threatening interstitial lung disease
  • Pulmonary CT imaging findings in fat embolism syndrome: case series and literature review
  • Acute quadriplegia and epileptic seizures in a 47-year-old man
Show more Acute medical care

Similar Articles

FAQs

  • Difficulty logging in.

There is currently no login required to access the journals. Please go to the home page and simply click on the edition that you wish to read. If you are still unable to access the content you require, please let us know through the 'Contact us' page.

  • Can't find the CME questionnaire.

The read-only self-assessment questionnaire (SAQ) can be found after the CME section in each edition of Clinical Medicine. RCP members and fellows (using their login details for the main RCP website) are able to access the full SAQ with answers and are awarded 2 CPD points upon successful (8/10) completion from:  https://cme.rcplondon.ac.uk

Navigate this Journal

  • Journal Home
  • Current Issue
  • Ahead of Print
  • Archive

Related Links

  • ClinMed - Home
  • FHJ - Home
clinmedicine Footer Logo
  • Home
  • Journals
  • Contact us
  • Advertise
HighWire Press, Inc.

Follow Us:

  • Follow HighWire Origins on Twitter
  • Visit HighWire Origins on Facebook

Copyright © 2021 by the Royal College of Physicians