Chapter 9

I00–I99: Diseases of the Circulatory System

Diseases of the Circulatory System diagnostic classification updated for 2026.

Chapter Instructions

Excludes2(Not included here. Code separately if applicable.)
  • certain conditions originating in the perinatal period (P04-P96)
  • certain infectious and parasitic diseases (A00-B99)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
  • systemic connective tissue disorders (M30-M36)
  • transient cerebral ischemic attacks and related syndromes (G45.-)
Internal Codes
  • I26.96

    Fat embolism of pulmonary artery without acute cor pulmonale

  • I26.99

    Other pulmonary embolism without acute cor pulmonale

  • I27

    Other pulmonary heart diseases

  • I27.0

    Primary pulmonary hypertension

  • I27.1

    Kyphoscoliotic heart disease

  • I27.2

    Other secondary pulmonary hypertension

  • I27.20

    Pulmonary hypertension, unspecified

  • I27.21

    Secondary pulmonary arterial hypertension

  • I27.22

    Pulmonary hypertension due to left heart disease

  • I27.23

    Pulmonary hypertension due to lung diseases and hypoxia

  • I27.24

    Chronic thromboembolic pulmonary hypertension

  • I27.29

    Other secondary pulmonary hypertension

  • I27.8

    Other specified pulmonary heart diseases

  • I27.81

    Cor pulmonale (chronic)

  • I27.82

    Chronic pulmonary embolism

  • I27.83

    Eisenmenger's syndrome

  • I27.84

    Fontan related circulation

  • I27.840

    Fontan-associated liver disease [FALD]

  • I27.841

    Fontan-associated lymphatic dysfunction

  • I27.848

    Other Fontan-associated condition

  • I27.849

    Fontan related circulation, unspecified

  • I27.89

    Other specified pulmonary heart diseases

  • I27.9

    Pulmonary heart disease, unspecified

  • I28

    Other diseases of pulmonary vessels

  • I28.0

    Arteriovenous fistula of pulmonary vessels

  • I28.1

    Aneurysm of pulmonary artery

  • I28.8

    Other diseases of pulmonary vessels

  • I28.9

    Disease of pulmonary vessels, unspecified

  • I30

    Acute pericarditis

  • I30.0

    Acute nonspecific idiopathic pericarditis

  • I30.1

    Infective pericarditis

  • I30.8

    Other forms of acute pericarditis

  • I30.9

    Acute pericarditis, unspecified

  • I31

    Other diseases of pericardium

  • I31.0

    Chronic adhesive pericarditis

  • I31.1

    Chronic constrictive pericarditis

  • I31.2

    Hemopericardium, not elsewhere classified

  • I31.3

    Pericardial effusion (noninflammatory)

  • I31.31

    Malignant pericardial effusion in diseases classified elsewhere

  • I31.39

    Other pericardial effusion (noninflammatory)

  • I31.4

    Cardiac tamponade

  • I31.8

    Other specified diseases of pericardium

  • I31.9

    Disease of pericardium, unspecified

  • I32

    Pericarditis in diseases classified elsewhere

  • I33

    Acute and subacute endocarditis

  • I33.0

    Acute and subacute infective endocarditis

  • I33.9

    Acute and subacute endocarditis, unspecified

  • I34

    Nonrheumatic mitral valve disorders

  • I34.0

    Nonrheumatic mitral (valve) insufficiency

  • I34.1

    Nonrheumatic mitral (valve) prolapse

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