KB42

ICD-11 MMS 2026-01

Persistent pulmonary hypertension of the newborn

Description

Persistent pulmonary hypertension of the newborn is a cardiopulmonary disorder characterised by systemic arterial hypoxemia secondary to pulmonary hypertension and extrapulmonary right-to-left shunting across the foramen ovale and ductus arteriosus.

Coding note from above levels

Conditions arising in the perinatal period, even though death or morbidity occurs later, should, as far as possible, be coded to chapter 19, which takes precedence over chapters containing codes for diseases by their anatomical site. For children less than 28 days old, assume that a reported condition developed in the perinatal period, unless the duration is stated and the onset was after the first completed week of life.

Index terms

  • Persistent pulmonary hypertension of the newborn
  • PFC - [persistent fetal circulation] syndrome
  • PPHN - [Persistent pulmonary hypertension of the newborn]
Show allHide
  • fetal circulation
  • newborn pulmonary hypertension
  • persistent fetal circulation
  • persistent fetal circulation syndrome
  • primary pulmonary hypertension of newborn

Exclusions from above levels

  • http://id.who.int/icd/entity/2004408087 – congenital malformations of the heart and circulatory system
  • 01 – Certain infectious or parasitic diseases - acquired after birth
  • 02 – Neoplasms
Show allHide
  • 05 – Endocrine, nutritional or metabolic diseases
  • 08 – Certain congenital diseases of the nervous system
  • 20 – Congenital malformations, deformations and chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • 3A10 – Hereditary haemolytic anaemia
  • 4A01.03 – Transient hypogammaglobulinaemia of infancy
  • 9B71.3 – Retinopathy of prematurity
  • BC43 – congenital cardiomyopathy
  • DA93.0 – Paralytic ileus
  • EA50.Z – Pemphigus neonatorum
  • EH40.00 – Cradle cap
  • http://id.who.int/icd/entity/588616678 – Gastroenteritis or colitis of infectious origin
  • http://id.who.int/icd/entity/609214049 – Congenital gonococcal infection

Sibling codes

  • KB40 – Neonatal cardiac failure
  • KB41 – Cardiac arrhythmias in the neonate
  • KB44 – Transient myocardial ischaemia of newborn
  • KB45 – Neonatal hypertension
  • KB46 – Neonatal hypotension
  • KB47 – Benign or innocent cardiac murmurs in newborn
  • KB48 – Patent arterial duct of prematurity
  • KB4Y – Other specified cardiovascular disorders present in the perinatal or neonatal period
  • KB4Z – Cardiovascular disorders present in the perinatal or neonatal period, unspecified

Foundation entity

Persistent pulmonary hypertension of the newborn – http://id.who.int/icd/entity/346808536

Part of: 19: Certain conditions originating in the perinatal period