DB99.3

ICD-11 MMS 2026-01

Portopulmonary hypertension

Description

This is the coexistence of portal and pulmonary hypertension, and is a serious complication of liver disease, present in 0.25 to 4% of all patients suffering from cirrhosis.

Index terms

  • Portopulmonary hypertension

Exclusions from above levels

  • 5D00.0 – amyloid degeneration of liver
  • BD71.Y – hepatic vein thrombosis
  • DB94 – alcoholic liver disease
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  • DB95 – toxic liver disease
  • DB98.3 – portal vein thrombosis
  • LB20.00 – cystic disease of liver (congenital)
  • ME10.00 – hepatomegaly NOS
  • ME10.1 – Unspecified jaundice
  • 01 – Certain infectious or parasitic diseases
  • 02 – Neoplasms
  • 05 – Endocrine, nutritional or metabolic diseases
  • 06 – Mental, behavioural or neurodevelopmental disorders
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • DB99.0 – Chronic liver disease
  • DB99.1 – Hepatic cyst
  • DB99.2 – Hepatorenal syndrome
  • DB99.4 – Hepatopulmonary syndrome
  • DB99.5 – Hepatic encephalopathy
  • DB99.6 – Intrahepatic cholestasis, not elsewhere classified
  • DB99.7 – Hepatic failure without mention whether acute or chronic
  • DB99.8 – Chronic hepatic failure
  • DB99.Y – Other specified diseases of liver

Foundation entity

Portopulmonary hypertension – http://id.who.int/icd/entity/1065786072

Allowed postcoordination axes

Part of: DB99: Certain specified diseases of liver › 13: Diseases of the digestive system