Portopulmonary hypertension
Alerts and Notices
Synopsis

Clinical presentation of PPHTN includes manifestations of portal hypertension (ascites, lower extremity edema, encephalopathy, varices, and splenomegaly) along with symptoms of PAH (dyspnea on exertion, orthopnea, fatigue, chest pain, and syncope).
Codes
ICD10CM:I27.29 – Other secondary pulmonary hypertension
SNOMEDCT:
445237003 – Portopulmonary Hypertension
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Pulmonary artery hypertension unrelated to liver disease
- Hepatopulmonary syndrome
- Interstitial lung disease
- Obstructive sleep apnea
- Chronic obstructive pulmonary disease (COPD)
- Emphysema
- Drug toxicity (chemotherapeutics such as cyclophosphamide and bleomycin; amiodarone, fenfluramine / phentermine, aminorex, toxic rapeseed oil, cocaine, amphetamines)
- Radiation-induced lung disease
- Mitral stenosis
- Alpha-1 antitrypsin deficiency
- Congestive heart failure
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Management Pearls
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Therapy
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References
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Last Reviewed:05/17/2018
Last Updated:05/31/2021
Last Updated:05/31/2021