hereditary disease pulmonary atresia-intact ventricular septum syndrome https://www.malacards.org/card/pulmonary_atresia_with_intact_ventricular_septum 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. SCTID:253590009 pulmonary atresia with intact ventricular septum MONDO:0009931 NANDO:2200253 Orphanet:1208 OMIM:265150 NANDO:1200707 MEDGEN:87491 UMLS:C0344975 pulmonary valve atresia with intact ventricular septum GARD:0004600 icd11.foundation:131289265 MESH:C562832 NCIT:C99032 hypoplastic right heart syndrome