Hepatopulmonary syndrome differential diagnosis: Difference between revisions
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* Diffusion impairment | * Diffusion impairment | ||
* Decreased | * Decreased DLCO | ||
* Reduced lung volumes | |||
* Reduced | |||
| style="background: #F5F5F5; padding: 5px;" |'''Frequently nonspecific''' | | style="background: #F5F5F5; padding: 5px;" |'''Frequently nonspecific''' | ||
'''and subtle''' | '''and subtle''' | ||
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<br /> | <br /> | ||
| style="background: #F5F5F5; padding: 5px;" |Plays no | | style="background: #F5F5F5; padding: 5px;" | | ||
* Plays no direct role in diagnosis | |||
role in | |||
diagnosis | |||
| style="background: #F5F5F5; padding: 5px;" |Triad of | | style="background: #F5F5F5; padding: 5px;" |Triad of | ||
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* ABG (widened '''alveolar-arterial oxygen gradient''' | * ABG (widened '''alveolar-arterial oxygen gradient''' | ||
* '''Intra-pulmonary vascular dilation''' or arterio-venous communications that result in a '''right-to-left intrapulmonary shunt.''' | * '''Intra-pulmonary vascular dilation''' or arterio-venous communications that result in a '''right-to-left intrapulmonary shunt.''' | ||
| style="background: #F5F5F5; padding: 5px;" | | | style="background: #F5F5F5; padding: 5px;" |N/A | ||
|- | |- | ||
| style="background: #DCDCDC; padding: 5px; text-align: center;" |PPH | | style="background: #DCDCDC; padding: 5px; text-align: center;" |PPH | ||
| style="background: #F5F5F5; padding: 5px;" |+ | | style="background: #F5F5F5; padding: 5px;" | + | ||
* D[[Dyspnea|yspnea]] | |||
* Fatigue | |||
* S[[Syncope|yncope]]. | |||
| style="background: #F5F5F5; padding: 5px;" |+ | | style="background: #F5F5F5; padding: 5px;" |+ | ||
| style="background: #F5F5F5; padding: 5px;" |- | | style="background: #F5F5F5; padding: 5px;" |- | ||
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| style="background: #F5F5F5; padding: 5px;" |Hypoxemia | | style="background: #F5F5F5; padding: 5px;" |Hypoxemia | ||
| 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;" | | ||
| 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:''' | |||
| style="background: #F5F5F5; padding: 5px;" | | * 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;" |HHT | | 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;" |SaO2<96% | |||
| style="background: #F5F5F5; padding: 5px;" |Shunt in favor of AVM | |||
| 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 | |||
|} | |} | ||
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{{Reflist|2}} | {{Reflist|2}} | ||
[[Category: | [[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% |
|
|
Frequently nonspecific
and subtle
|
+ | + (could distinguish between intracardiac and intrapulmonary shunt)
|
|
Triad of
|
N/A |
PPH | + | + | - | - | + | Hypoxemia |
|
PFT cannot distinguish PPH from other pulmonary disorders with diffusion impairment
|
|
- | -
|
|
|
N/A |
HHT | + | - | + | + | - | SaO2<96% | Shunt in favor of AVM |
|
|
+ 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) |
|
- 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
- ↑ 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