Pulmonary heart disease and diseases of pulmonary circulation (I26-I28)
I26Pulmonary embolism
Includes: pulmonary (acute) (artery)(vein) infarction
› pulmonary (acute) (artery)(vein) thromboembolism
› pulmonary (acute) (artery)(vein) thrombosis
Excludes 1: cor pulmonale without embolism (I27.81)
Excludes 2: chronic pulmonary embolism (I27.82)
› personal history of pulmonary embolism (Z86.711)
› pulmonary embolism complicating abortion, ectopic or molar pregnancy (O00-O07, O08.2)
› pulmonary embolism complicating pregnancy, childbirth and the puerperium (O88.-)
› pulmonary embolism due to trauma (T79.0, T79.1)
› pulmonary embolism due to complications of surgical and medical care (T80.0, T81.7-, T82.8-)
› septic (non-pulmonary) arterial embolism (I76)
I26.0Pulmonary embolism with acute cor pulmonale
I26.01Septic pulmonary embolism with acute cor pulmonale
Code First: underlying infection
I26.02Saddle embolus of pulmonary artery with acute cor pulmonale
I26.03Cement embolism of pulmonary artery with acute cor pulmonale
Code First: complication of other artery following a procedure (T81.718)
I26.04Fat embolism of pulmonary artery with acute cor pulmonale
Code First: , if applicable:
› complication of other artery following a procedure (T81.718)
› traumatic fat embolism (T79.1)
I26.09Other pulmonary embolism with acute cor pulmonale
› Acute cor pulmonale NOS
› Other thrombotic pulmonary embolism with acute cor pulmonale
I26.9Pulmonary embolism without acute cor pulmonale
I26.90Septic pulmonary embolism without acute cor pulmonale
Code First: underlying infection
I26.92Saddle embolus of pulmonary artery without acute cor pulmonale
I26.93Single subsegmental thrombotic pulmonary embolism without acute cor pulmonale
› Subsegmental pulmonary embolism NOS
I26.94Multiple subsegmental thrombotic pulmonary emboli without acute cor pulmonale
I26.95Cement embolism of pulmonary artery without acute cor pulmonale
Code First: complication of other artery following a procedure (T81.718)
I26.96Fat embolism of pulmonary artery without acute cor pulmonale
Code First: , if applicable:
› complication of other artery following a procedure (T81.718)
› traumatic fat embolism (T79.1)
I26.99Other pulmonary embolism without acute cor pulmonale
› Acute pulmonary embolism NOS
› Other thrombotic pulmonary embolism without acute cor pulmonale
› Pulmonary embolism NOS
I27Other pulmonary heart diseases
I27.0Primary pulmonary hypertension
› Heritable pulmonary arterial hypertension
› Idiopathic pulmonary arterial hypertension
› Primary group 1 pulmonary hypertension
› Primary pulmonary arterial hypertension
Excludes 1: persistent pulmonary hypertension of newborn (P29.30)
› pulmonary hypertension NOS (I27.20)
› secondary pulmonary arterial hypertension (I27.21)
› secondary pulmonary hypertension (I27.29)
I27.1Kyphoscoliotic heart disease
I27.2Other secondary pulmonary hypertension
Code Also: associated underlying condition
Excludes 1: Eisenmenger's syndrome (I27.83)
I27.20Pulmonary hypertension, unspecified
› Pulmonary hypertension NOS
I27.21Secondary pulmonary arterial hypertension
› (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)
› congenital heart disease (Q20-Q28)
› human immunodeficiency virus [HIV] disease (B20)
› polymyositis (M33.2-)
› portal hypertension (K76.6)
› rheumatoid arthritis (M05.-)
› schistosomiasis (B65.-)
› Sjögren syndrome (M35.0-)
› systemic sclerosis (M34.-)
I27.22Pulmonary hypertension due to left heart disease
› Group 2 pulmonary hypertension
Code Also: associated left heart disease, if known, such as:
› multiple valve disease (I08.-)
› rheumatic mitral valve diseases (I05.-)
› rheumatic aortic valve diseases (I06.-)
I27.23Pulmonary hypertension due to lung diseases and hypoxia
› Group 3 pulmonary hypertension
Code Also: associated lung disease, if known, such as:
› bronchiectasis (J47.-)
› cystic fibrosis with pulmonary manifestations (E84.0)
› interstitial lung disease (J84.-)
› pleural effusion (J90)
› sleep apnea (G47.3-)
I27.24Chronic thromboembolic pulmonary hypertension
› Group 4 pulmonary hypertension
Code Also: associated pulmonary embolism, if applicable (I26.-, I27.82)
I27.29Other secondary pulmonary hypertension
› 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)
› essential thrombocythemia (D47.3)
› Gaucher disease (E75.22)
› hypertensive chronic kidney disease with end stage renal disease (I12.0, I13.11, I13.2)
› hyperthyroidism (E05.-)
› hypothyroidism (E00-E03)
› polycythemia vera (D45)
› sarcoidosis (D86.-)
I27.8Other specified pulmonary heart diseases
I27.81Cor pulmonale (chronic)
› Cor pulmonale NOS
Code Also: , if applicable, right heart failure (I50.81-)
Excludes 1: acute cor pulmonale (I26.0-)
I27.82Chronic pulmonary embolism
Use Additional Code: code, if applicable, for associated long-term (current) use of anticoagulants (Z79.01)
Excludes 1: personal history of pulmonary embolism (Z86.711)
I27.83Eisenmenger's syndrome
› 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-)
› Eisenmenger's defect (Q21.8)
› patent ductus arteriosus (Q25.0)
› ventricular septal defect (Q21.0)
I27.84Fontan related circulation
I27.840Fontan-associated liver disease [FALD]
I27.841Fontan-associated lymphatic dysfunction
Code Also: associated conditions such as:
› chylothorax (J94.0)
› Fontan associated protein-losing enteropathy (K90.49)
› plastic (obstructive) bronchitis (J44.89)
I27.848Other Fontan-associated condition
Use Additional Code: code to specify the Fontan associated condition
I27.849Fontan related circulation, unspecified
I27.89Other specified pulmonary heart diseases
I27.9Pulmonary heart disease, unspecified
› Chronic cardiopulmonary disease
I28Other diseases of pulmonary vessels
I28.0Arteriovenous fistula of pulmonary vessels
Excludes 1: congenital arteriovenous fistula (Q25.72)
I28.1Aneurysm of pulmonary artery
Excludes 1: congenital aneurysm (Q25.79)
› congenital arteriovenous aneurysm (Q25.72)
I28.8Other diseases of pulmonary vessels
› Pulmonary arteritis
› Pulmonary endarteritis
› Rupture of pulmonary vessels
› Stenosis of pulmonary vessels
› Stricture of pulmonary vessels
I28.9Disease of pulmonary vessels, unspecified