| syndroom van agenesie van pulmonalisklep, tetralogie van Fallot en afwezigheid van ductus arteriosus (aandoening) | | syndroom van agenesie van pulmonalisklep, tetralogie van Fallot en afwezigheid van ductus arteriosus | | syndroom van agenesie van valva trunci pulmonalis, tetralogie van Fallot en afwezigheid van ductus arteriosus Botalli
| | Pulmonary valve agenesis, tetralogy of Fallot, absence of ductus arteriosus syndrome | | Absence of pulmonary valve, Fallot tetralogy, absence of ductus arteriosus syndrome
| | A rare congenital heart malformation with characteristics of tetralogy of Fallot (pulmonary stenosis, overriding aorta, ventricular septal defect and right ventricular hypertrophy), complete absence or rudimentary pulmonary valve that is both stenotic and regurgitant and an absence of the ductus arteriosus. It presents prenatally with cardiomegaly, polyhydramnios, fetal heart failure, hydrops fetalis and fetal demise or postnatally with cyanosis and respiratory failure due to bronchomalacia secondary to bronchial compression from dilated pulmonary arteries. It is frequently associated with 22q11 deletion. |
| | Id | 766976003 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | Q22.2 | | Term | Congenitale pulmonalisklepinsufficiëntie |
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| SNOMED CT to Orphanet simple map | 101206 |
| 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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