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 including, shortness of breath, clubbed fingers, and cyanosis.	Chronic liver disease symptoms including, Itching, easy bruising, abdominal fullness, decreased libido, abdominal distension, and fatigue.	Platypnea	Orthodeoxia	Chronic liver disease signs including, spider angioma, red palms, edema, gynecomastia	Pulse oximetry	Arterial blood gas (ABG) analysis	Pulmonary function test	Chest CT scan	Pulmonary angiography	Contrast enhanced echocardiography
Differential Diagnosis 1
Differential Diagnosis 2
Differential Diagnosis 3
Diseases	Respiratory symptoms including, shortness of breath, clubbed fingers, and cyanosis.	Chronic liver disease symptoms including, Itching, easy bruising, abdominal fullness, decreased libido, abdominal distension, and fatigue.	Platypnea	Orthodeoxia	Chronic liver disease signs including, spider angioma, red palms, edema, gynecomastia	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)