BB01.0 Pulmonary arterial hypertension International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01 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 – 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 –– 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 Human immunodeficiency virus disease associated with malaria – 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 –– 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 Human immunodeficiency virus disease without mention of tuberculosis or malaria – 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 associated disease or condition, clinical stage 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 – 4A40.0 Systemic lupus erythematosus – 4A40.1 Drug-induced lupus erythematosus – 4A40.Y Other specified lupus erythematosus – 4A40.Z Lupus erythematosus, unspecified –– 4A40.00 Systemic lupus erythematosus with skin involvement –– 4A40.0Y Other specified systemic lupus erythematosus –– 4A40.0Z Systemic lupus erythematosus, unspecified 4A41 Idiopathic inflammatory myopathy – 4A41.0 Dermatomyositis – 4A41.1 Polymyositis – 4A41.2 Inclusion body myopathy – 4A41.Y Other specified idiopathic inflammatory myopathy – 4A41.Z Idiopathic inflammatory myopathy, 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 4A42 Systemic sclerosis – 4A42.0 Paediatric onset systemic sclerosis – 4A42.1 Diffuse systemic sclerosis – 4A42.2 Limited systemic sclerosis – 4A42.Z Systemic sclerosis, unspecified 4A43 Overlap or undifferentiated nonorgan specific systemic autoimmune disease – 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 –– 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 Vasculitis – 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 –– 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 4A45 Antiphospholipid syndrome – 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 4A4Y Other specified nonorgan specific systemic autoimmune disorders 4A4Z Nonorgan specific systemic autoimmune disorders, 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 ––– 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 –– LA81 Abnormal ventricular relationships –– LA82 Total mirror imagery –– LA83 Right isomerism –– LA84 Left isomerism –– LA85 Congenital anomaly of an atrioventricular or ventriculo-arterial connection ––– 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 –––– 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 Congenital anomaly of mediastinal vein ––– 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 –––– 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 Congenital anomaly of an atrioventricular valve or atrioventricular septum ––– 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 –––– 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 Congenital anomaly of a ventricle or the ventricular septum ––– 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 –––– 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 –– LA89 Functionally univentricular heart ––– 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 Congenital anomaly of a ventriculo-arterial valve or adjacent regions ––– 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 –––– 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 Congenital anomaly of great arteries including arterial duct ––– 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 –––– 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 –– LA8C Congenital anomaly of coronary artery ––– 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 –– LA8D Congenital pericardial anomaly –– LA8E Congenital anomaly of atrial septum ––– 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 –– LA8F Congenital anomaly of right atrium –– LA8G Congenital anomaly of left atrium ––– LA8G.0 Divided left atrium ––– LA8G.Y Other specified congenital anomaly of left atrium ––– LA8G.Z Congenital anomaly of left atrium, unspecified –– LA8Y Other specified structural developmental anomaly of heart or great vessels –– LA8Z Structural developmental anomaly of heart or great vessels, unspecified Has severity - only one may be selected [select] XS5W Mild XS0T Moderate XS25 Severe
synonyms Pulmonary arterial hypertension primary pulmonary hypertension PAH - [pulmonary arterial hypertension] Arrillaga Ayerza syndrome 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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