I27.0

Primary pulmonary hypertension

Billable FY2026 2025-10-01 → 2026-09-30

Exclusions 14 never code together

I27.0code

Excludes1

persistent pulmonary hypertension of newborn (P29.30)P29.30 P29.30
pulmonary hypertension NOS (I27.20)I27.20 I27.20
secondary pulmonary arterial hypertension (I27.21)I27.21 I27.21
secondary pulmonary hypertension (I27.29)I27.29 I27.29

I00-I99chapter

Excludes2

certain conditions originating in the perinatal period (P04-P96)P04-P96
certain infectious and parasitic diseases (A00-B99)A00-B99
complications of pregnancy, childbirth and the puerperium (O00-O9A)O00-O9A
congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)Q00-Q99
endocrine, nutritional and metabolic diseases (E00-E88)E00-E88
injury, poisoning and certain other consequences of external causes (S00-T88)S00-T88
neoplasms (C00-D49)C00-D49
symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)R00-R94
systemic connective tissue disorders (M30-M36)M30-M36
transient cerebral ischemic attacks and related syndromes (G45.-)G45.-

Excludes1 — never code together. The excluded condition and this one are mutually exclusive.

Excludes2 — not included here, but the patient may have both. Code both when documented.

Instructional notes 4

I27.0code

Inclusion terms

Heritable pulmonary arterial hypertension
Idiopathic pulmonary arterial hypertension
Primary group 1 pulmonary hypertension
Primary pulmonary arterial hypertension

Index entries leading here 8

Syndrome — see also Disease
- Arrillaga-Ayerza I27.0
- pulmonary
  - arteriosclerosis I27.0
- Ayerza (-Arrillaga) I27.0
- cardiacos negros I27.0
Arrillaga-Ayerza syndrome (pulmonary sclerosis with pulmonary hypertension) I27.0
Arteriosclerosis, arteriosclerotic (diffuse) (obliterans) (of) (senile) (with calcification) I70.90
- pulmonary (idiopathic) I27.0
Sclerosis, sclerotic
- pulmonary — see Fibrosis, lung
  - artery I27.0
Ayerza's disease or syndrome (pulmonary artery sclerosis with pulmonary hypertension) I27.0
Disease, diseased — see also Syndrome
- Ayerza's (pulmonary artery sclerosis with pulmonary hypertension) I27.0
- heart (organic) I51.9
  - black I27.0
- pulmonary — see also Disease, lung
  - hypertensive (vascular) I27.20
    - primary (idiopathic) I27.0
Cardiopathia nigra I27.0
Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic) I10
- pulmonary I27.20
  - primary (idiopathic) I27.0
- lesser circulation I27.0

Alongside this code 4 same parent — I27

I27.1 Kyphoscoliotic heart disease I27.2 Other secondary pulmonary hypertension category I27.8 Other specified pulmonary heart diseases category I27.9 Pulmonary heart disease, unspecified

Medications 2

34 Hypertension, Pulmonary drugs 2 Pulmonary Arterial Hypertension drugs

MED-RT files conditions by MeSH, which carves the world differently from ICD-10. These are name matches on this code's description, offered as candidates — not as an assertion that they are this code.

Additional references

AHA Coding Clinic® Licence required

Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.

Risk adjustment

CMS22HCC85 CMS28HCC226 ESRD21HCC85 ESRD24HCC85 RX08HCC183

Presence is a property of the code. Untick a model to hide it everywhere.

This code

Billable: yes
Code set: FY2026
Parent: I27
Block: I26-I28
Siblings: 4

Present FY2024–FY2027.