DISEASE: Pulmonary atresia with intact ventricular septum
Entry
H01802 Disease
Name
Pulmonary atresia with intact ventricular septum
Description
Pulmonary atresia with intact ventricular septum (PAIVS) is a severe cardiac malformation characterized by variable right ventricular development and imperforate pulmonary valve. PAIVS has a wide spectrum of clinical presentation ranging from cyanosis to shock due to closure of ductus arteriosus. The treatment follows initial stabilization with prostaglandin and establishment of antegrade flow. Because of the morphologic heterogeneity, repair or palliation strategies vary widely, ranging from single ventricle palliation to complete biventricular repair. Overall, regardless of anatomical subtype, survival is poor, reported to be as low as 50% at 5 years.
Category
Congenital malformation
Brite
Human diseases in ICD-11 classification [BR:br08403]
20 Developmental anomalies
Structural developmental anomalies primarily affecting one body system
Structural developmental anomalies of the circulatory system
Structural developmental anomaly of heart or great vessels
LA8A Congenital anomaly of a ventriculo-arterial valve or adjacent regions
H01802 Pulmonary atresia with intact ventricular septum
