Pulmonary atresia-intact ventricular septum syndrome
Pulmonary atresia-intact ventricular septum syndrome
Definition
Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare form of cyanotic congenital heart malformation characterized by severe cyanosis and tachypnea. PA-IVS presents significant morphologic diversity: at the end of the spectrum are patients with a mildly hypoplastic and tripartite right ventricle (RV) and mild tricuspid valve (TV) hypoplasia, and at the other end are patients with severe RV and TV hypoplasia, often with RV-dependent coronary circulation.
Also known as pulmonary valve atresia with intact ventricular septum — per MONDO
Also identified as
- MESH C562832 per MONDO
- NCIT C99032 per MONDO
- OMIM 265150 per MONDO
- Orphanet 1208 per MONDO
- SCTID 253590009 per MONDO
- UMLS C0344975 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 |