Hepatopulmonary syndrome differential diagnosis: Difference between revisions

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[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Hepatopulmonary syndrome]]
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Hepatopulmonary_syndrome]]
{{Hepatopulmonary syndrome}}
 
{{CMG}}; {{AE}} {{Soroush}}
{{CMG}}; {{AE}} {{Soroush}}
==Overview==
==Overview==
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
Hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).
 
OR
 
[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].


==Differentiating [Disease name] from other Diseases==
==Differentiating Hepatopulmonary Syndrome from other Diseases==
[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
Mild hypoxemia occurs in 30 percent of patients with chronic liver disease. It may be due to common cardiopulmonary diseases such as congestive heart failure, chronic obstructive pulmonary disease (COPD) or pneumonia. Pulmonary vascular bed malfunction also might be responsible for certain conditions such as Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH).


OR
Hepatopulmonary syndrome (HPS) must be differentiated from [[portopulmonary hypertension]] (PPH) and [[hereditary hemorrhagic telangiectasia]] (HHT).


[Disease name] must be differentiated from [differential dx1], [differential dx2], and [differential dx3].
Portopulmonary hypertension is often confused with HPS. However, HPS and PPH are not the same disease. Although both are abnormalities of the pulmonary vasculature resulting from liver disease, '''HPS is characterized by vasodilatation''' and hypoxemia whereas '''PPH is characterized by obstruction or narrowing (vasoconstriction)''' of blood vessels with resulting pulmonary arterial hypertension.


OR
===Differentiating  hepatopulmonary syndrome from other diseases on the basis of pulse oximetry, arterial blood gas (ABG) analysis , contrast enhanced echocardiography; 99mTc scan: lung perfusion scintigraphy with technetium 99mTc labeled macro aggregated albumin, chest CT scan , pulmonary angiography, and pulmonary function test.===


As [disease name] manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtype. [Subtype name 1] must be differentiated from other diseases that cause [clinical feature 1], such as [differential dx1] and [differential dx2]. In contrast, [subtype name 2] must be differentiated from other diseases that cause [clinical feature 2], such as [differential dx3] and [differential dx4].
On the basis  pulse oximetry, arterial blood gas (ABG) analysis,  contrast enhanced echocardiography, 99mTc scan (lung perfusion scintigraphy with technetium 99mTc labeled macro aggregated albumin), chest CT scan , pulmonary angiography, and pulmonary function test, hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).<ref name="pmid28759737">Krynytska I, Marushchak M, Mikolenko A, Bob A, Smachylo I, Radetska L et al. (2017) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=28759737 Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).] ''Bosn J Basic Med Sci'' 17 (4):276-285. [http://dx.doi.org/10.17305/bjbms.2017.2020 DOI:10.17305/bjbms.2017.2020] PMID: [https://pubmed.gov/28759737 28759737]</ref>
 
===Differentiating [disease name] from other diseases on the basis of [symptom 1], [symptom 2], and [symptom 3]===
 
On the basis [symptom 1], [symptom 2], and [symptom 3], [disease name] must be differentiated from [disease 1], [disease 2], [disease 3], [disease 4], [disease 5], and [disease 6].
{|
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! rowspan="4"  style="background: #4479BA; color: #FFFFFF; text-align: center;|Diseases
! rowspan="4"  style="background: #4479BA; color: #FFFFFF; text-align: center;|Diseases
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Clinical manifestations'''
| colspan="5" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
! colspan="7" rowspan="2"  style="background: #4479BA; color: #FFFFFF; text-align: center;|Para-clinical findings
! colspan="7" rowspan="2"  style="background: #4479BA; color: #FFFFFF; text-align: center;|Para-clinical findings
| colspan="1" rowspan="4"  style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Gold standard'''
| colspan="1" rowspan="4"  style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Gold standard'''
Line 33: Line 25:
|-
|-
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Symptoms'''
| colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|'''Symptoms'''
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical examination
! colspan="2" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
|-
|-
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|Histopathology
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;|Histo
pathology
|-  
|-  
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Symptom 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Respiratory symptoms
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;|Symptom 2
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;|Chronic liver disease symptoms
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Symptom 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Platypnea
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical exam 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Orthodeoxia
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical exam 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Chronic liver disease signs
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Physical exam 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Pulse oximetry
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Arterial blood gas (ABG) analysis
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Lab 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Pulmonary
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging 1
function test
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Chest CT scan/
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Imaging 3
Pulmonary angiography
! style="background: #4479BA; color: #FFFFFF; text-align: center;|99mTc scan
! style="background: #4479BA; color: #FFFFFF; text-align: center;|Contrast enhanced echocardiography
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 1
| style="background: #DCDCDC; padding: 5px; text-align: center;" |HSP
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+ (in most of the patients)
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+ (in most of the patients)
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |SaO2<96%
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* 15 mmHg ≤ AaPO2
* PaO2< 80 mm Hg
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Diffusion impairment
* Decreased DLCO
* Reduced lung volumes
| style="background: #F5F5F5; padding: 5px;" |'''Frequently nonspecific'''
'''and subtle'''
* Dilated peripheral pulmonary vessels
* Increased [[pulmonary artery]] to [[bronchus]]
*ratios
* Characteristic findings of intrapulmonary [[vascular]] dilatations
* Direct arterio-venous communications may be less commonly seen.
<br />
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |  + (could distinguish between intracardiac and intrapulmonary shunt)
* In intracardiac shunting: three cardiac cycles after the appearance of the bubbles in the right heart chambers.
* In intrapulmonary shunting: four to six cardiac cycles after the appearance of the bubbles in the right heart chambers.
<br />
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Plays no direct role in diagnosis
| style="background: #F5F5F5; padding: 5px;" |Triad of
* liver disease
* ABG (widened '''alveolar-arterial oxygen gradient'''
* '''Intra-pulmonary vascular dilation''' or arterio-venous communications that result in a '''right-to-left intrapulmonary shunt.'''
| style="background: #F5F5F5; padding: 5px;" |N/A
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 2
| style="background: #DCDCDC; padding: 5px; text-align: center;" |PPH
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
 
| style="background: #F5F5F5; padding: 5px;" |
* D[[Dyspnea|yspnea]]
| style="background: #F5F5F5; padding: 5px;" |
* Fatigue
| style="background: #F5F5F5; padding: 5px;" |
* S[[Syncope|yncope]].
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Hypoxemia
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Used for Pulmonary hypertension  Class Identification
* PaO2 is normal or only slightly lower than normal
* PaCO2 is decreased as a result of alveolar hyperventilation
| style="background: #F5F5F5; padding: 5px;" |'''PFT cannot distinguish PPH from other pulmonary disorders with diffusion impairment'''
* Decreased diffusion capacity
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Central '''pulmonary''' artery dilatation
* Abrupt narrowing or tapering of peripheral '''pulmonary''' vessels
* Right ventricular hypertrophy
* Right ventricular and atrial enlargement
* Dilated bronchial arteries
| style="background: #F5F5F5; padding: 5px;" |-
| style="background: #F5F5F5; padding: 5px;" | -
* Right heart [[dysfunction]] secondary to [[pulmonary hypertension]]
*Diagnosis and staging is based on the followings:
*Pulmonary artery pressure >25 mmHg
*Pulmonary capillary wedge pressure<15 mmHg
*Pulmonary vascular resistance
*>240syn·s·cm<sup>−5</sup>
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Plays no direct role in diagnosis
| style="background: #F5F5F5; padding: 5px;" |<br />
* '''The diagnosis of portopulmonary hypertension is based on hemodynamic criteria:'''
* Portal hypertension and/or liver disease (clinical diagnosis—ascites/varices/splenomegaly)
* Mean pulmonary artery pressure—MPAP > 25 mmHg at rest
* Pulmonary vascular resistance—PVR > 240 dynes s cm−5
* :Pulmonary artery occlusion pressure— PAOP < 15mmHg or transpulmonary gradient—TPG > 12 mmHg where TPG = MPAP − PAOP.
*
| style="background: #F5F5F5; padding: 5px;" |N/A
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 3
| style="background: #DCDCDC; padding: 5px; text-align: center;" |HHT
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |-
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |SaO2<96%
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Shunt in favor of AVM
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
!Diseases
!Symptom 1
! colspan="1" rowspan="1" |Symptom 2
!Symptom 3
!Physical exam 1
! colspan="1" rowspan="1" |Physical exam 2
!Physical exam 3
!Lab 1
!Lab 2
!Lab 3
!Imaging 1
!Imaging 2
!Imaging 3
!Histopathology
|'''Gold standard'''
!Additional findings
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 4
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 5
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Differential Diagnosis 6
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Most patients have normal resting pulmonary function values
* Others might present with restrictive or an obstructive pattern
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* Arteriovenous malformation (AVM) in lungs, liver, and other organs
| style="background: #F5F5F5; padding: 5px;" |+ Due to AVM and hence shunts
| style="background: #F5F5F5; padding: 5px;" |+ Due to AVM and hence shunts
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
* [[TGF beta signaling pathway|TGFβ signalling]] pathway impairment
* A[[Arteriovenous malformation|rteriovenous malformations]] (AVMs) in various internal organs
<br />
| style="background: #F5F5F5; padding: 5px;" |Hereditary hemorrhagic telangiectasia is a clinical diagnosis that is based on the presence of three of four criteria (i.e., epistaxis, telangiectasias, visceral arteriovenous malformations, or family history of the disease)
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
# Spontaneous recurrent [[epistaxis]]
# Multiple teleangiectasias on typical locations (see above)
# Proven visceral AVM (lung, liver, brain, spine)
# First-degree family member with HHT
|}
|}
* '''[[Platypnea]]''' (increased shortness of breath when the body is in a vertical position) and '''orthodeoxia''' (3-10 mmHg reduction in РаО2 in capillary blood during transition from horizontal to vertical position)
* '''99mTc scan''': lung perfusion scintigraphy with technetium 99mTc labeled macro aggregated albumin
* '''Chronic liver disease symptoms including,''' Itching, easy bruising, abdominal fullness, decreased libido, abdominal distension, and fatigue.
* '''Chronic liver disease signs''' including [[spider angioma]], [[Red palms|red palm]]<nowiki/>s, [[edema]], [[gynecomastia]]
* '''Respiratory symptoms''' including [[Dyspnea|shortness of breath]], clubbed fingers, and [[cyanosis]].
*Hepatopulmonary syndrome ('''HPS''') must be differentiated from portopulmonary hypertension ('''PPH''') and [[hereditary hemorrhagic telangiectasia]] '''(HHT''').
*Severity of HPS is defined based on [[Pulmonary gas pressures|PaO2]], while below 50 is extremely sever, 50-60 is sever, and more than 60 is defined as moderate to mild.


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


[[Category: (name of the system)]]
[[Category:Surgery]]
[[Category:Medicine]]
[[Category:Pulmonology]]
[[Category:Cardiology]]
[[Category:Gastroentrology]]
[[Category:Up-To-Date]]

Latest revision as of 17:55, 6 September 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

Hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).

Differentiating Hepatopulmonary Syndrome from other Diseases

Mild hypoxemia occurs in 30 percent of patients with chronic liver disease. It may be due to common cardiopulmonary diseases such as congestive heart failure, chronic obstructive pulmonary disease (COPD) or pneumonia. Pulmonary vascular bed malfunction also might be responsible for certain conditions such as Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH).

Hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).

Portopulmonary hypertension is often confused with HPS. However, HPS and PPH are not the same disease. Although both are abnormalities of the pulmonary vasculature resulting from liver disease, HPS is characterized by vasodilatation and hypoxemia whereas PPH is characterized by obstruction or narrowing (vasoconstriction) of blood vessels with resulting pulmonary arterial hypertension.

Differentiating hepatopulmonary syndrome from other diseases on the basis of pulse oximetry, arterial blood gas (ABG) analysis , contrast enhanced echocardiography; 99mTc scan: lung perfusion scintigraphy with technetium 99mTc labeled macro aggregated albumin, chest CT scan , pulmonary angiography, and pulmonary function test.

On the basis pulse oximetry, arterial blood gas (ABG) analysis, contrast enhanced echocardiography, 99mTc scan (lung perfusion scintigraphy with technetium 99mTc labeled macro aggregated albumin), chest CT scan , pulmonary angiography, and pulmonary function test, hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).[1]

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histo

pathology

Respiratory symptoms Chronic liver disease symptoms Platypnea Orthodeoxia Chronic liver disease signs Pulse oximetry Arterial blood gas (ABG) analysis Pulmonary

function test

Chest CT scan/

Pulmonary angiography

99mTc scan Contrast enhanced echocardiography
HSP + + (in most of the patients) + + + (in most of the patients) SaO2<96%
  • 15 mmHg ≤ AaPO2
  • PaO2< 80 mm Hg


  • Diffusion impairment
  • Decreased DLCO
  • Reduced lung volumes
Frequently nonspecific

and subtle

  • Dilated peripheral pulmonary vessels
  • Increased pulmonary artery to bronchus
  • ratios
  • Characteristic findings of intrapulmonary vascular dilatations
  • Direct arterio-venous communications may be less commonly seen.


+ + (could distinguish between intracardiac and intrapulmonary shunt)
  • In intracardiac shunting: three cardiac cycles after the appearance of the bubbles in the right heart chambers.
  • In intrapulmonary shunting: four to six cardiac cycles after the appearance of the bubbles in the right heart chambers.


  • Plays no direct role in diagnosis
Triad of
  • liver disease
  • ABG (widened alveolar-arterial oxygen gradient
  • Intra-pulmonary vascular dilation or arterio-venous communications that result in a right-to-left intrapulmonary shunt.
N/A
PPH + + - - + Hypoxemia
  • Used for Pulmonary hypertension Class Identification
  • PaO2 is normal or only slightly lower than normal
  • PaCO2 is decreased as a result of alveolar hyperventilation
PFT cannot distinguish PPH from other pulmonary disorders with diffusion impairment
  • Decreased diffusion capacity
  • Central pulmonary artery dilatation
  • Abrupt narrowing or tapering of peripheral pulmonary vessels
  • Right ventricular hypertrophy
  • Right ventricular and atrial enlargement
  • Dilated bronchial arteries
- -
  • Right heart dysfunction secondary to pulmonary hypertension
  • Diagnosis and staging is based on the followings:
  • Pulmonary artery pressure >25 mmHg
  • Pulmonary capillary wedge pressure<15 mmHg
  • Pulmonary vascular resistance
  • >240syn·s·cm−5
  • Plays no direct role in diagnosis

  • The diagnosis of portopulmonary hypertension is based on hemodynamic criteria:
  • Portal hypertension and/or liver disease (clinical diagnosis—ascites/varices/splenomegaly)
  • Mean pulmonary artery pressure—MPAP > 25 mmHg at rest
  • Pulmonary vascular resistance—PVR > 240 dynes s cm−5
  • :Pulmonary artery occlusion pressure— PAOP < 15mmHg or transpulmonary gradient—TPG > 12 mmHg where TPG = MPAP − PAOP.
N/A
HHT + - + + - SaO2<96% Shunt in favor of AVM
  • Most patients have normal resting pulmonary function values
  • Others might present with restrictive or an obstructive pattern
  • Arteriovenous malformation (AVM) in lungs, liver, and other organs
+ Due to AVM and hence shunts + Due to AVM and hence shunts


Hereditary hemorrhagic telangiectasia is a clinical diagnosis that is based on the presence of three of four criteria (i.e., epistaxis, telangiectasias, visceral arteriovenous malformations, or family history of the disease)
  1. Spontaneous recurrent epistaxis
  2. Multiple teleangiectasias on typical locations (see above)
  3. Proven visceral AVM (lung, liver, brain, spine)
  4. First-degree family member with HHT
  • Platypnea (increased shortness of breath when the body is in a vertical position) and orthodeoxia (3-10 mmHg reduction in РаО2 in capillary blood during transition from horizontal to vertical position)
  • 99mTc scan: lung perfusion scintigraphy with technetium 99mTc labeled macro aggregated albumin
  • Chronic liver disease symptoms including, Itching, easy bruising, abdominal fullness, decreased libido, abdominal distension, and fatigue.
  • Chronic liver disease signs including spider angioma, red palms, edema, gynecomastia
  • Respiratory symptoms including shortness of breath, clubbed fingers, and cyanosis.
  • Hepatopulmonary syndrome (HPS) must be differentiated from portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT).
  • Severity of HPS is defined based on PaO2, while below 50 is extremely sever, 50-60 is sever, and more than 60 is defined as moderate to mild.

References

  1. Krynytska I, Marushchak M, Mikolenko A, Bob A, Smachylo I, Radetska L et al. (2017) Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). Bosn J Basic Med Sci 17 (4):276-285. DOI:10.17305/bjbms.2017.2020 PMID: 28759737