| syndroom van agenesie van pulmonalisklep, intact ventriculair septum en persisterende ductus arteriosus (aandoening) | | syndroom van agenesie van pulmonalisklep, intact ventriculair septum en persisterende ductus arteriosus | | syndroom van agenesie van pulmonaalklep, intact ventrikelseptum en persisterende ductus arteriosus Botalli
syndroom van agenesie van valva trunci pulmonalis, intact septum interventriculare en persisterende ductus arteriosus
| | Pulmonary valve agenesis, intact ventricular septum, persistent ductus arteriosus syndrome | | APV (agenesis pulmonary valve) PDA (patent ductus arteriosus) non-Fallot type
| | A rare, life-threatening, congenital, non-syndromic, conotruncal heart malformation disease characterized by absent or severely undeveloped pulmonary valve leaflets (with a restrictive ring of thickened tissue at the place of the pulmonary valve annulus), associated with an intact ventricular septum and a patent ductus arteriosus, manifesting with marked respiratory insufficiency. Additional features include dilated main pulmonary artery (with or without dilatation of pulmonary artery branches), to-and-fro flow at site of the dysplastic pulmonary valve, and systolic pressure gradient across narrowed pulmonary valve. Tricuspid atresia and variable extra-cardiac anomalies (e.g. diaphragmatic hernia or cleft lip/palate), may be present. |
| | Id | 784353002 | | Status | Primitive |
| SNOMED CT to Orphanet simple map |
| DHD Diagnosis thesaurus reference set |
| SNOMED CT to ICD-10 extended map | | Target | Q22.2 | | Rule | TRUE | | Advice | ALWAYS Q22.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | | Correlation | SNOMED CT source code to target map code correlation not specified |
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| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | Q22.2 | | Term | Congenitale pulmonalisklepinsufficiëntie |
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