Prenatal diagnosis of absent pulmonary valve syndrome: results of a single-center experience in Beijing

Abstract

Objective: The purpose of this study was to analyze the fetal echocardiographic features of absent pulmonary valve syndrome (APVS). Materials and Methods: Echocardiographic findings were retrospectively analyzed and summarized in nine fetuses with APVS. Results: The right ventricle was dilated in six cases (66.7%). The cardiac axis was abnormally deviated to the left in four cases (44.4%). The pulmonary valve was either absent or rudimentary in all cases. The main pulmonary artery or its branches were significantly dilated. Color Doppler flow imaging showed severe pulmonary regurgitation. Spectral Doppler imaging showed stenosis of the pulmonary annulus in seven cases. Seven cases were associated with tetralogy of Fallot, two with right aortic arch, two with double outlet right ventricle, and one with mitral valve atresia and single ventricle of right ventricular type. Conclusions: Presence of a markedly dilated right ventricle and pulmonary arteries, combined with stenosis and severe regurgitation of the pulmonary annulus should be considered as APVS.