Pulmonic Valve Atresia:
Clinical:
In pulmonic atresia there is an imperforate pulmonary valve. If the ventricular septum is intact, there must be an obligatory right to left shunt at atrial level and pulmonary flow is dependent upon ductus arteriosus. Patients usually have an associated hypoplastic RV and tricuspid valve. Supra systemic RV pressure causes RV myocardial to coronary artery shunting. Patients present with intense cyanosis and early deathX-ray:
CXR demonstrates decreased pulmonary vascularity, a concave main pulmonary artery segment, and cardiomegaly.MR will demonstrate increased fat deposition in the region of the atretic valve ring. It can also be used to determine if the pulmonary arteries are continuous with each other to aid in surgical correction.
REFERENCES:
(1) Pediatric Clinics of North America 1999; Waldman JD, Wernly JA.
Cyanotic congenital heart disease with decreased pulmonary blood flow in
children. 46(2): 385-402
