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Diseases of the circulatory system (I00-I99)

Excludes 2:: 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.-)

Pulmonary heart disease and diseases of pulmonary circulation (I26-I28)

I26

Pulmonary 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)

I26.0

Pulmonary embolism with acute cor pulmonale [+]

I26.9

Pulmonary embolism without acute cor pulmonale [+]

I27

Other pulmonary heart diseases [+]

I27.0

Primary 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)

I27.1

Kyphoscoliotic heart disease

I27.2

Other secondary pulmonary hypertension [+]

Code also	associated underlying condition
Excludes 1:	Eisenmenger's syndrome (I27.83)

I27.20

Pulmonary hypertension, unspecified
Pulmonary hypertension NOS

I27.21

Secondary 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.-)

I27.22

Pulmonary 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.-)

I27.23

Pulmonary 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-)

I27.24

Chronic thromboembolic pulmonary hypertension
Group 4 pulmonary hypertension

Code also

associated pulmonary embolism, if applicable (I26.-, I27.82)

I27.29

Other 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.-)

I27.8

Other specified pulmonary heart diseases [+]

I27.9

Pulmonary heart disease, unspecified
Chronic cardiopulmonary disease

I28

Other diseases of pulmonary vessels [+]