Hepatopulmonary syndrome differential diagnosis

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

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

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).

Diseases	Clinical manifestations					Para-clinical findings							Gold standard	Additional findings
	Symptoms			Physical examination
						Lab Findings			Imaging			Histopathology
	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						SaO2<96%	15 mmHg ≤ AaPO2 PaO2< 80 mm Hg			+	+
PPH
HHT
Diseases						Lab 1	Lab 2	Lab 3	Imaging 1	Imaging 2	Imaging 3	Histopathology	Gold standard	Additional findings
Differential Diagnosis 4
Differential Diagnosis 5
Differential Diagnosis 6

• 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.