pulmonary atresia with ventricular septal defect
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.
MONDO:0003847
hereditary disease
is_a
MONDO:0016581
conotruncal heart malformations
is_a
pulmonary atresia with ventricular septal defect
exact
pulmonary valve atresia with ventricular septal defect
exact