DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

I27 Other pulmonary heart diseases

  • Header · not billable

I27 is not billable. The category heads 17 billable codes in FY2027, grouped under 5 subdivisions.

ICD-10 code by name

What is the ICD-10 code for pulmonary hypertension, unspecified?
Pulmonary hypertension ICD-10 code: I27.20 Pulmonary hypertension, unspecified. Search ICD-10 for pulmonary hypertension
What is the ICD-10 code for pulmonary arterial hypertension?
Pulmonary arterial hypertension ICD-10 code: I27.21 Secondary pulmonary arterial hypertension. Alphabetic Index: Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonaryarterial (associated) (drug-induced) (toxin-induced) → I27.21. Search ICD-10 for pulmonary arterial hypertension

Where it sits

  1. Chapter 9 Diseases of the circulatory system (I00-I99)
  2. I26-I28 Pulmonary heart disease and diseases of pulmonary circulation (I26-I28)
  3. I27 Other pulmonary heart diseases
Billable codes
17
Subdivisions
5
CC / MCC codes
4 / 0
HCC V28 codes
13
New FY2027
0
Index entries
59

Codes in I27

  1. I27.0
    Primary pulmonary hypertension
    BillableCC226
    Inclusion terms 4, Excludes1 4

    Inclusion terms

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

    Excludes1

    • persistent pulmonary hypertension of newborn (P29.30) P29.30
    • pulmonary hypertension NOS (I27.20) I27.20
    • secondary pulmonary arterial hypertension (I27.21) I27.21
    • secondary pulmonary hypertension (I27.29) I27.29
  2. I27.1
    Kyphoscoliotic heart disease
    BillableCC226
  3. I27.2
    Other secondary pulmonary hypertension
    Header
    Excludes1 1, Code also 1

    Excludes1

    • Eisenmenger's syndrome (I27.83) I27.83

    Code also

    • associated underlying condition
  4. I27.20
    Pulmonary hypertension, unspecified
    Billable226
    Inclusion terms 1

    Inclusion terms

    • Pulmonary hypertension NOS
  5. I27.21
    Secondary pulmonary arterial hypertension
    Billable226
    Inclusion terms 2, Code also 10

    Inclusion terms

    • (Associated) (drug-induced) (toxin-induced) pulmonary arterial hypertension NOS
    • (Associated) (drug-induced) (toxin-induced) (secondary) group 1 pulmonary hypertension

    Code also

    • associated conditions if applicable, or adverse effects of drugs or toxins, such as:
    • adverse effect of appetite depressants (T50.5X5) T50.5X5
    • congenital heart disease (Q20-Q28) Q20 Q28
    • human immunodeficiency virus [HIV] disease (B20) B20
    • polymyositis (M33.2-) M33.2
    • portal hypertension (K76.6) K76.6
    • rheumatoid arthritis (M05.-) M05
    • schistosomiasis (B65.-) B65
    • Sjögren syndrome (M35.0-) M35.0
    • systemic sclerosis (M34.-) M34
  6. I27.22
    Pulmonary hypertension due to left heart disease
    Billable226
    Inclusion terms 1, Code also 4

    Inclusion terms

    • Group 2 pulmonary hypertension

    Code also

    • associated left heart disease, if known, such as:
    • multiple valve disease (I08.-) I08
    • rheumatic mitral valve diseases (I05.-) I05
    • rheumatic aortic valve diseases (I06.-) I06
  7. I27.23
    Pulmonary hypertension due to lung diseases and hypoxia
    Billable226
    Inclusion terms 1, Code also 6

    Inclusion terms

    • Group 3 pulmonary hypertension

    Code also

    • associated lung disease, if known, such as:
    • bronchiectasis (J47.-) J47
    • cystic fibrosis with pulmonary manifestations (E84.0) E84.0
    • interstitial lung disease (J84.-) J84
    • pleural effusion (J90) J90
    • sleep apnea (G47.3-) G47.3
  8. I27.24
    Chronic thromboembolic pulmonary hypertension
    Billable226
    Inclusion terms 1, Code also 1

    Inclusion terms

    • Group 4 pulmonary hypertension

    Code also

    • associated pulmonary embolism, if applicable (I26.-, I27.82) I26 I27.82
  9. I27.29
    Other secondary pulmonary hypertension
    Billable226
    Inclusion terms 5, Code also 9

    Inclusion terms

    • Group 5 pulmonary hypertension
    • Pulmonary hypertension with unclear multifactorial mechanisms
    • Pulmonary hypertension due to hematologic disorders
    • Pulmonary hypertension due to metabolic disorders
    • Pulmonary hypertension due to other systemic disorders

    Code also

    • other associated disorders, if known, such as:
    • chronic myeloid leukemia (C92.10-C92.22) C92.10 C92.22
    • essential thrombocythemia (D47.3) D47.3
    • Gaucher disease (E75.22) E75.22
    • hypertensive chronic kidney disease with end stage renal disease (I12.0, I13.11, I13.2) I12.0 I13.11 I13.2
    • hyperthyroidism (E05.-) E05
    • hypothyroidism (E00-E03) E00 E03
    • polycythemia vera (D45) D45
    • sarcoidosis (D86.-) D86
  10. I27.8
    Other specified pulmonary heart diseases
    Header
  11. I27.81
    Cor pulmonale (chronic)
    Billable226
    Inclusion terms 1, Excludes1 1, Code also 1

    Inclusion terms

    • Cor pulmonale NOS

    Excludes1

    • acute cor pulmonale (I26.0-) I26.0

    Code also

    • , if applicable, right heart failure (I50.81-) I50.81
  12. I27.82
    Chronic pulmonary embolism
    BillableCC267
    Excludes1 1, Use additional code 1

    Excludes1

    • personal history of pulmonary embolism (Z86.711) Z86.711

    Use additional code

    • code, if applicable, for associated long-term (current) use of anticoagulants (Z79.01) Z79.01
  13. I27.83
    Eisenmenger's syndrome
    Billable226
    Inclusion terms 3, Code also 5

    Inclusion terms

    • Eisenmenger's complex
    • (Irreversible) Eisenmenger's disease
    • Pulmonary hypertension with right to left shunt related to congenital heart disease

    Code also

    • underlying heart defect, if known, such as:
    • atrial septal defect (Q21.1-) Q21.1
    • Eisenmenger's defect (Q21.8) Q21.8
    • patent ductus arteriosus (Q25.0) Q25.0
    • ventricular septal defect (Q21.0) Q21.0
  14. I27.84
    Fontan related circulation
    Header
  15. I27.840
    Fontan-associated liver disease [FALD]
    BillableCC
  16. I27.841
    Fontan-associated lymphatic dysfunction
    Billable
    Code also 4

    Code also

    • associated conditions such as:
    • chylothorax (J94.0) J94.0
    • Fontan associated protein-losing enteropathy (K90.49) K90.49
    • plastic (obstructive) bronchitis (J44.89) J44.89
  17. I27.848
    Other Fontan-associated condition
    Billable
    Use additional code 1

    Use additional code

    • code to specify the Fontan associated condition
  18. I27.849
    Fontan related circulation, unspecified
    Billable
  19. I27.89
    Other specified pulmonary heart diseases
    Billable226
  20. I27.9
    Pulmonary heart disease, unspecified
    Billable226
    Inclusion terms 1

    Inclusion terms

    • Chronic cardiopulmonary disease

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

Sources of each column.

Instructions

Notes that also apply

Chapter 9 instructions (Excludes2 10): printed on the chapter 9 page.

Index entries

  • Arrillaga-Ayerza syndrome (pulmonary sclerosis with pulmonary hypertension) → I27.0
  • Ayerza's disease or syndrome (pulmonary artery sclerosis with pulmonary hypertension) → I27.0
  • Cardiopathia nigra → I27.0
  • Arteriosclerosis, arteriosclerotic (diffuse) (obliterans) (of) (senile) (with calcification)pulmonary (idiopathic) → I27.0
  • Disease, diseasedAyerza's (pulmonary artery sclerosis with pulmonary hypertension) → I27.0
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)lesser circulation → I27.0
  • SyndromeArrillaga-Ayerza → I27.0
  • SyndromeAyerza (-Arrillaga) → I27.0
  • Syndromecardiacos negros → I27.0
  • Kyphoscoliosis, kyphoscoliotic (acquired)heart (disease) → I27.1
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonary → I27.20
  • Corpulmonale → I27.81
  • Pancake heartwith cor pulmonale (chronic) → I27.81
  • ComplexEisenmenger's (ventricular septal defect) → I27.83
  • Disease, diseasedEisenmenger's (irreversible) → I27.83
  • Eisenmenger'scomplex or syndrome → I27.83
  • SyndromeEisenmenger's → I27.83
  • ConditionFontan-associated, specified NEC → I27.848
  • CirculationFontan related → I27.849
  • Disease, diseasedcardiopulmonary, chronic → I27.9
  • Disease, diseasedheart (organic)black → I27.0
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonaryprimary (idiopathic) → I27.0
  • Sclerosis, scleroticpulmonaryartery → I27.0
  • Syndromepulmonaryarteriosclerosis → I27.0
  • Disease, diseasedheart (organic)kyphoscoliotic → I27.1
  • Disease, diseasedpulmonaryhypertensive (vascular) → I27.20
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonaryarterial (associated) (drug-induced) (toxin-induced) → I27.21
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonarygroup 1 (associated) (drug-induced) (toxin-induced) → I27.21
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonarygroup 2 → I27.22
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonarygroup 3 → I27.23
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonarychronic thromboembolic → I27.24
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonarygroup 4 → I27.24
  • Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)pulmonarygroup 5 → I27.29
  • Corpulmonalechronic → I27.81
  • Embolism (multiple) (paradoxical)pulmonary (acute) (artery) (vein)chronic → I27.82
  • Disease, diseasedliver (chronic) (organic)Fontan-associated (FALD) → I27.840
  • DysfunctionlymphaticFontan-associated → I27.841
  • Disease, diseasedheart (organic)pulmonary (chronic) → I27.9
  • Disease, diseasedpulmonaryheart → I27.9
  • Disease, diseasedpulmonaryhypertensive (vascular)primary (idiopathic) → I27.0

19 more, longer entries are not listed; the search box finds them by word.

NCHS Alphabetic Index. Index first, then the Tabular List.

Hierarchy