Hepatopulmonary syndrome differential diagnosis

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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).[1]

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%
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).

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