 I27 |
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Other pulmonary heart diseases  |
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 I27.0 |
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Primary pulmonary hypertension |
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| 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)
pulmonary hypertension NOS (I27.20)
secondary pulmonary arterial hypertension (I27.21)
secondary pulmonary hypertension (I27.29)
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| I27.1 |
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Kyphoscoliotic heart disease |
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| I27.2 |
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Other secondary pulmonary hypertension |
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| CodeAlso |
associated underlying condition
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| Excludes1 |
Eisenmenger's syndrome (I27.83)
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| I27.20 |
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Pulmonary hypertension, unspecified |
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| Pulmonary hypertension NOS |
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| I27.21 |
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Secondary pulmonary arterial hypertension |
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| (Associated) (drug-induced) (toxin-induced) pulmonary arterial hypertension NOS | | (Associated) (drug-induced) (toxin-induced) (secondary) group 1 pulmonary hypertension |
| CodeAlso |
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.-)
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| I27.22 |
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Pulmonary hypertension due to left heart disease |
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| Group 2 pulmonary hypertension |
| CodeAlso |
associated left heart disease, if known, such as:
multiple valve disease (I08.-)
rheumatic mitral valve diseases (I05.-)
rheumatic aortic valve diseases (I06.-)
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| I27.23 |
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Pulmonary hypertension due to lung diseases and hypoxia |
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| Group 3 pulmonary hypertension |
| CodeAlso |
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-)
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| I27.24 |
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Chronic thromboembolic pulmonary hypertension |
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| Group 4 pulmonary hypertension |
| CodeAlso |
associated pulmonary embolism, if applicable (I26.-, I27.82)
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| I27.29 |
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Other secondary pulmonary hypertension |
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| 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 |
| CodeAlso |
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.-)
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| I27.8 |
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Other specified pulmonary heart diseases |
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| I27.81 |
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Cor pulmonale (chronic) |
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| Cor pulmonale NOS |
| CodeAlso |
, if applicable, right heart failure (I50.81-)
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| Excludes1 |
acute cor pulmonale (I26.0-)
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| I27.82 |
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Chronic pulmonary embolism |
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| UseAdditionalCode |
code, if applicable, for associated long-term (current) use of anticoagulants (Z79.01)
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| Excludes1 |
personal history of pulmonary embolism (Z86.711)
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| I27.83 |
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Eisenmenger's syndrome |
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| Eisenmenger's complex | | (Irreversible) Eisenmenger's disease | | Pulmonary hypertension with right to left shunt related to congenital heart disease |
| CodeAlso |
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)
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| I27.84 |
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Fontan related circulation |
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| I27.840 |
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Fontan-associated liver disease [FALD] |
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| I27.841 |
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Fontan-associated lymphatic dysfunction |
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| CodeAlso |
associated conditions such as:
chylothorax (J94.0)
Fontan associated protein-losing enteropathy (K90.89)
plastic (obstructive) bronchitis (J44.89)
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| I27.848 |
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Other Fontan-associated condition |
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| UseAdditionalCode |
code to specify the Fontan associated condition
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| I27.849 |
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Fontan related circulation, unspecified |
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| I27.89 |
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Other specified pulmonary heart diseases |
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| I27.9 |
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Pulmonary heart disease, unspecified |
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| Chronic cardiopulmonary disease |
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