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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 valva trunci pulmonalis, intact septum interventriculare en persisterende ductus arteriosus
syndroom van agenesie van pulmonaalklep, intact ventrikelseptum en persisterende ductus arteriosus Botalli
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 with characteristics of 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 (for example diaphragmatic hernia or cleft lip/palate) may be present.
Id784353002
StatusPrimitive
Associated morphologyagenesie
Finding sitestructuur van valva trunci pulmonalis
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
referentieset met complexe 'mapping' naar ICD-10
TargetQ22.2
RuleTRUE
AdviceALWAYS Q22.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified