BB01.0 Pulmonary arterial hypertension International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01 Pulmonary arterial hypertension is a clinical condition characterised by the presence of pre-capillary pulmonary hypertension in the absence of other causes of pre-capillary pulmonary hypertension, such as due to lung diseases, chronic thromboembolic pulmonary hypertension, or other rare diseases. It includes different forms that share a similar clinical picture and virtually identical pathological changes of the lung microcirculation.
inclusions primary pulmonary hypertension Idiopathic pulmonary arterial hypertension postcoordination Add Stem and/or Extension codes to form a cluster code which adds detail to the condition.
Associated with - multiple selections are allowed [select] 1C60 Human immunodeficiency virus disease associated with tuberculosis 1C61 Human immunodeficiency virus disease associated with malaria 1C62 Human immunodeficiency virus disease without mention of tuberculosis or malaria 1C60.0 HIV disease clinical stage 1 associated with tuberculosis 1C60.1 HIV disease clinical stage 2 associated with tuberculosis 1C60.2 HIV disease clinical stage 3 associated with tuberculosis 1C60.3 HIV disease clinical stage 4 associated with tuberculosis 1C60.Z Human immunodeficiency virus disease associated with tuberculosis, clinical stage unspecified 1C61.0 HIV disease clinical stage 1 associated with malaria 1C61.1 HIV disease clinical stage 2 associated with malaria 1C61.2 HIV disease clinical stage 3 associated with malaria 1C61.3 HIV disease clinical stage 4 associated with malaria 1C61.Z Human immunodeficiency virus disease associated with malaria, clinical stage unspecified 1C62.0 HIV disease clinical stage 1 without mention of tuberculosis or malaria 1C62.1 HIV disease clinical stage 2 without mention of tuberculosis or malaria 1C62.2 HIV disease clinical stage 3 without mention of tuberculosis or malaria 1C62.3 HIV disease clinical stage 4 without mention of tuberculosis or malaria 1C62.Z Human immunodeficiency virus disease without mention of tuberculosis or malaria, clinical stage unspecified 1C60.30 Kaposi sarcoma associated with human immunodeficiency virus disease associated with tuberculosis 1C60.3Y Other specified HIV disease clinical stage 4 associated with tuberculosis 1C60.3Z HIV disease clinical stage 4 associated with tuberculosis, unspecified 1C61.30 Kaposi sarcoma associated with human immunodeficiency virus disease associated with malaria 1C61.3Y Other specified HIV disease clinical stage 4 associated with malaria 1C61.3Z HIV disease clinical stage 4 associated with malaria, unspecified 1C62.30 Kaposi sarcoma associated with human immunodeficiency virus disease without mention of tuberculosis or malaria 1C62.3Y Other specified HIV disease clinical stage 4 without mention of tuberculosis or malaria 1C62.3Z HIV disease clinical stage 4 without mention of tuberculosis or malaria, unspecified 1F86 Schistosomiasis 1F86.0 Schistosomiasis due to Schistosoma haematobium 1F86.1 Schistosomiasis due to Schistosoma mansoni 1F86.2 Schistosomiasis due to Schistosoma japonicum 1F86.3 Other schistosomiases 1F86.4 Cercarial dermatitis 1F86.5 Schistosomal pneumonitis 1F86.Z Schistosomiasis due to unspecified or unknown Schistosoma species 4A40 Lupus erythematosus 4A41 Idiopathic inflammatory myopathy 4A42 Systemic sclerosis 4A43 Overlap or undifferentiated nonorgan specific systemic autoimmune disease 4A44 Vasculitis 4A45 Antiphospholipid syndrome 4A4Y Other specified nonorgan specific systemic autoimmune disorders 4A4Z Nonorgan specific systemic autoimmune disorders, unspecified 4A40.0 Systemic lupus erythematosus 4A40.1 Drug-induced lupus erythematosus 4A40.Y Other specified lupus erythematosus 4A40.Z Lupus erythematosus, unspecified 4A41.0 Dermatomyositis 4A41.1 Polymyositis 4A41.2 Inclusion body myopathy 4A41.Y Other specified idiopathic inflammatory myopathy 4A41.Z Idiopathic inflammatory myopathy, unspecified 4A42.0 Paediatric onset systemic sclerosis 4A42.1 Diffuse systemic sclerosis 4A42.2 Limited systemic sclerosis 4A42.Z Systemic sclerosis, unspecified 4A43.0 IgG4 related disease 4A43.1 Mikulicz disease 4A43.2 Sjögren syndrome 4A43.3 Mixed connective tissue disease 4A43.4 Diffuse eosinophilic fasciitis 4A43.Y Other specified overlap non-organ specific systemic autoimmune disease 4A43.Z Undifferentiated non-organ specific systemic autoimmune disease 4A44.0 Rhizomelic pseudopolyarthritis 4A44.1 Aortic arch syndrome 4A44.2 Giant cell arteritis 4A44.3 Single organ vasculitis 4A44.4 Polyarteritis nodosa 4A44.5 Mucocutaneous lymph node syndrome 4A44.6 Sneddon syndrome 4A44.7 Primary angiitis of the central nervous system 4A44.8 Thromboangiitis obliterans 4A44.9 Immune complex small vessel vasculitis 4A44.A Antineutrophil cytoplasmic antibody-associated vasculitis 4A44.B Leukocytoclastic vasculitis 4A44.Y Other specified vasculitis 4A44.Z Vasculitis, unspecified 4A45.0 Primary antiphospholipid syndrome 4A45.1 Secondary antiphospholipid syndrome 4A45.2 Antiphospholipid syndrome in pregnancy 4A45.3 Lupus anticoagulant-hypoprothrombinaemia syndrome 4A45.Z Antiphospholipid syndrome, unspecified 4A40.00 Systemic lupus erythematosus with skin involvement 4A40.0Y Other specified systemic lupus erythematosus 4A40.0Z Systemic lupus erythematosus, unspecified 4A41.00 Adult dermatomyositis 4A41.01 Juvenile dermatomyositis 4A41.0Z Dermatomyositis, unspecified 4A41.10 Juvenile polymyositis 4A41.11 Paraneoplastic polymyositis 4A41.1Y Other specified polymyositis 4A41.1Z Polymyositis, unspecified 4A41.20 Inflammatory inclusion body myositis 4A41.21 Noninflammatory inclusion body myopathy 4A41.2Z Inclusion body myopathy, unspecified 4A43.20 Primary Sjögren syndrome 4A43.21 Secondary Sjögren syndrome 4A43.22 Paediatric onset Sjögren syndrome 4A43.2Y Other specified sjögren syndrome 4A43.2Z Sjögren syndrome, unspecified 4A44.90 Cryoglobulinaemic vasculitis 4A44.91 Hypocomplementaemic urticarial vasculitis 4A44.92 IgA vasculitis 4A44.9Y Other specified immune complex small vessel vasculitis 4A44.9Z Immune complex small vessel vasculitis, unspecified 4A44.A0 Microscopic polyangiitis 4A44.A1 Granulomatosis with polyangiitis 4A44.A2 Eosinophilic granulomatosis with polyangiitis 4A44.AY Other specified antineutrophil cytoplasmic antibody-associated vasculitis 4A44.AZ Antineutrophil cytoplasmic antibody-associated vasculitis, unspecified 4A44.B0 Cutaneous leukocytoclastic vasculitis 4A44.BY Other specified leukocytoclastic vasculitis 4A44.BZ Leukocytoclastic vasculitis, unspecified DB98.7 Portal hypertension DB98.70 Idiopathic portal hypertension DB98.71 Non-cirrhotic portal fibrosis DB98.72 Partial nodular transformation of liver DB98.73 Splanchnic arteriovenous fistula DB98.74 Secondary portal hypertension DB98.7Y Other specified portal hypertension DB98.7Z Portal hypertension, unspecified LA80 Anomalous position-orientation of heart LA81 Abnormal ventricular relationships LA82 Total mirror imagery LA83 Right isomerism LA84 Left isomerism LA85 Congenital anomaly of an atrioventricular or ventriculo-arterial connection LA86 Congenital anomaly of mediastinal vein LA87 Congenital anomaly of an atrioventricular valve or atrioventricular septum LA88 Congenital anomaly of a ventricle or the ventricular septum LA89 Functionally univentricular heart LA8A Congenital anomaly of a ventriculo-arterial valve or adjacent regions LA8B Congenital anomaly of great arteries including arterial duct LA8C Congenital anomaly of coronary artery LA8D Congenital pericardial anomaly LA8E Congenital anomaly of atrial septum LA8F Congenital anomaly of right atrium LA8G Congenital anomaly of left atrium LA8Y Other specified structural developmental anomaly of heart or great vessels LA8Z Structural developmental anomaly of heart or great vessels, unspecified LA80.0 Laevocardia LA80.1 Dextrocardia LA80.2 Mesocardia LA80.3 Extrathoracic heart LA80.Y Other specified anomalous position-orientation of heart LA80.Z Anomalous position-orientation of heart, unspecified LA85.0 Discordant atrioventricular connections LA85.1 Transposition of the great arteries LA85.2 Double outlet right ventricle LA85.3 Double outlet left ventricle LA85.4 Common arterial trunk LA85.Y Other specified congenital anomaly of an atrioventricular or ventriculo-arterial connection LA85.Z Congenital anomaly of an atrioventricular or ventriculo-arterial connection, unspecified LA86.0 Left superior caval vein LA86.1 Unroofed coronary sinus LA86.2 Anomalous pulmonary venous connection LA86.3 Congenital pulmonary venous stenosis or hypoplasia LA86.Y Other specified congenital anomaly of mediastinal vein LA86.Z Congenital anomaly of mediastinal vein, unspecified LA87.0 Congenital anomaly of tricuspid valve LA87.1 Congenital anomaly of mitral valve LA87.3 Common atrioventricular junction without an atrioventricular septal defect LA87.4 Common atrioventricular junction with atrioventricular septal defect LA87.Y Other specified congenital anomaly of an atrioventricular valve or atrioventricular septum LA87.Z Congenital anomaly of an atrioventricular valve or atrioventricular septum, unspecified LA88.0 Congenital right ventricular outflow tract obstruction LA88.1 Double chambered right ventricle LA88.2 Tetralogy of Fallot LA88.3 Congenital left ventricular outflow tract obstruction LA88.4 Ventricular septal defect LA88.Y Other specified congenital anomaly of a ventricle or the ventricular septum LA88.Z Congenital anomaly of a ventricle or the ventricular septum, unspecified LA89.0 Double inlet atrioventricular connection LA89.1 Tricuspid atresia LA89.2 Mitral atresia LA89.3 Hypoplastic left heart syndrome LA89.Y Other specified functionally univentricular heart LA89.Z Functionally univentricular heart, unspecified LA8A.0 Congenital anomaly of pulmonary valve LA8A.1 Congenital pulmonary atresia LA8A.2 Congenital anomaly of aortic valve LA8A.3 Congenital supravalvar aortic stenosis LA8A.4 Aneurysm of aortic sinus of Valsalva LA8A.5 Congenital subaortic stenosis LA8A.6 Congenital subpulmonary stenosis LA8A.Y Other specified congenital anomaly of a ventriculo-arterial valve or adjacent regions LA8A.Z Congenital anomaly of a ventriculo-arterial valve or adjacent regions, unspecified LA8B.0 Congenital aortopulmonary window LA8B.1 Congenital anomaly of pulmonary arterial tree LA8B.2 Congenital anomaly of aorta or its branches LA8B.3 Tracheo-oesophageal compressive syndrome LA8B.4 Patent arterial duct LA8B.Y Other specified congenital anomaly of great arteries including arterial duct LA8B.Z Congenital anomaly of great arteries including arterial duct, unspecified LA8C.0 Anomalous origin of coronary artery from pulmonary arterial tree LA8C.1 Anomalous aortic origin or course of coronary artery LA8C.2 Congenital coronary arterial fistula LA8C.Y Other specified congenital anomaly of coronary artery LA8C.Z Congenital anomaly of coronary artery, unspecified LA8E.0 Patent oval foramen LA8E.1 Atrial septal defect within oval fossa LA8E.2 Sinus venosus defect LA8E.3 Interatrial communication through coronary sinus orifice LA8E.Y Other specified congenital anomaly of atrial septum LA8E.Z Congenital anomaly of atrial septum, unspecified LA8G.0 Divided left atrium LA8G.Y Other specified congenital anomaly of left atrium LA8G.Z Congenital anomaly of left atrium, unspecified LA85.00 Congenitally corrected transposition of great arteries LA85.0Y Other specified discordant atrioventricular connections LA85.0Z Discordant atrioventricular connections, unspecified LA85.20 Double outlet right ventricle with subpulmonary ventricular septal defect, transposition type LA85.21 Double outlet right ventricle with non-committed ventricular septal defect LA85.22 Double outlet right ventricle with subaortic or doubly committed ventricular septal defect without pulmonary stenosis, ventricular septal defect type LA85.23 Double outlet right ventricle with subaortic or doubly committed ventricular septal defect and pulmonary stenosis, Fallot type LA85.2Y Other specified double outlet right ventricle LA85.2Z Double outlet right ventricle, unspecified LA85.40 Common arterial trunk with aortic dominance LA85.41 Common arterial trunk with pulmonary dominance and interrupted aortic arch LA85.4Y Other specified common arterial trunk LA85.4Z Common arterial trunk, unspecified LA86.20 Total anomalous pulmonary venous connection LA86.21 Partial anomalous pulmonary venous connection LA86.22 Scimitar syndrome LA86.2Y Other specified anomalous pulmonary venous connection LA86.2Z Anomalous pulmonary venous connection, unspecified LA87.00 Congenital tricuspid regurgitation LA87.01 Congenital tricuspid valvar stenosis LA87.02 Dysplasia of tricuspid valve LA87.03 Ebstein malformation of tricuspid valve LA87.0Y Other specified congenital anomaly of tricuspid valve LA87.0Z Congenital anomaly of tricuspid valve, unspecified LA87.10 Congenital mitral regurgitation LA87.11 Congenital mitral valvar stenosis LA87.12 Dysplasia of mitral valve LA87.13 Congenital anomaly of mitral subvalvar apparatus LA87.1Y Other specified congenital anomaly of mitral valve LA87.1Z Congenital anomaly of mitral valve, unspecified LA87.40 Atrioventricular septal defect with communication at the atrial level only LA87.41 Atrioventricular septal defect with communication at the ventricular level only LA87.42 Atrioventricular septal defect with communication at atrial level and restrictive communication at ventricular level LA87.43 Atrioventricular septal defect with communication at atrial level and unrestrictive communication at ventricular level LA87.44 Atrioventricular septal defect with ventricular imbalance LA87.45 Atrioventricular septal defect and tetralogy of Fallot LA87.4Y Other specified common atrioventricular junction with atrioventricular septal defect LA87.4Z Common atrioventricular junction with atrioventricular septal defect, unspecified LA88.20 Tetralogy of Fallot with absent pulmonary valve syndrome LA88.21 Tetralogy of Fallot with pulmonary atresia LA88.22 Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral artery LA88.2Y Other specified tetralogy of Fallot LA88.2Z Tetralogy of Fallot, unspecified LA88.40 Trabecular muscular ventricular septal defect LA88.41 Perimembranous central ventricular septal defect LA88.42 Ventricular septal defect haemodynamically insignificant LA88.4Y Other specified ventricular septal defect LA88.4Z Ventricular septal defect, unspecified LA8A.00 Congenital pulmonary valvar stenosis LA8A.01 Congenital pulmonary regurgitation LA8A.0Y Other specified congenital anomaly of pulmonary valve LA8A.0Z Congenital anomaly of pulmonary valve, unspecified LA8A.10 Pulmonary atresia with intact ventricular septum LA8A.1Y Other specified congenital pulmonary atresia LA8A.1Z Congenital pulmonary atresia, unspecified LA8A.20 Congenital aortic valvar stenosis LA8A.21 Congenital aortic regurgitation LA8A.22 Bicuspid aortic valve LA8A.23 Aortic valvar atresia LA8A.24 Unicuspid aortic valve LA8A.2Y Other specified congenital anomaly of aortic valve LA8A.2Z Congenital anomaly of aortic valve, unspecified LA8B.21 Coarctation of aorta LA8B.22 Interrupted aortic arch LA8B.23 Congenital anomaly of descending thoracic or abdominal aorta LA8B.24 Congenital anomaly of aortic arch branch LA8B.2Y Other specified congenital anomaly of aorta or its branches LA8B.2Z Congenital anomaly of aorta or its branches, unspecified Has severity - only one may be selected [select] XS5W Mild XS0T Moderate XS25 Severe
synonyms Pulmonary arterial hypertension Arrillaga Ayerza syndrome PAH - [pulmonary arterial hypertension] primary pulmonary hypertension Idiopathic pulmonary arterial hypertension Familial pulmonary arterial hypertension heritable pulmonary arterial hypertension Familial pulmonary arterial hypertension due to BMPR2 mutations Familial pulmonary arterial hypertension due to ALK1 or endoglin mutations Familial pulmonary arterial hypertension of unknown origin Pulmonary arterial hypertension induced by drugs or toxins Pulmonary arterial hypertension associated with other conditions APAH - [Associated pulmonary arterial hypertension] Pulmonary arterial hypertension associated with connective tissue diseases Pulmonary arterial hypertension associated with HIV infection Pulmonary arterial hypertension associated with portal hypertension Pulmonary arterial hypertension associated with congenital heart disease Eisenmenger syndrome Pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunt Pulmonary arterial hypertension associated with schistosomiasis Pulmonary arterial hypertension associated with other aetiologies Pulmonary veno-occlusive disease Pulmonary capillary haemangiomatosis
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