Pulmonary atresia with ventricular septal defect
Pulmonary atresia with ventricular septal defect
Definition
Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot.
Also known as pulmonary atresia with ventricular septal defect, pulmonary valve atresia with ventricular septal defect — per MONDO
Also identified as
- MESH C562833 per MONDO
- NCIT C99033 per MONDO
- OMIM 178370 per MONDO
- Orphanet 1207 per MONDO
- SCTID 253591008 per MONDO
- UMLS C0344976 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Cardiovascular system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Cardiovascular system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Heart | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Heart | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Respiratory system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |