Pulmonary atresia/critical stenosis with intact ventricular septum: prediction of outcome in the second trimester of pregnancy.

OBJECTIVES: To determine which cardiac parameters provide the best prediction of postnatal outcome--biventricular (BV) versus non-BV--in fetuses with pulmonary atresia/critical stenosis with intact ventricular septum (PA/CS-IVS).

METHODS: We searched our database for cases of PA/CS-IVS prenatally diagnosed in 2001-2009. Only fetuses diagnosed ≤28 weeks were included. Data of 16 fetuses (nine PA, seven CS) were retrieved and analyzed. Receiver-operating characteristics curves were constructed to assess the sensitivity and specificity of cardiac features for predicting postnatal outcome.

RESULTS: Twelve fetuses had a BV outcome and four had non-BV repair. Cut-off values yielding the best results for a non-BV outcome were a tricuspid valve/mitral valve ratio ≤0.83, a pulmonary valve/aortic valve ratio ≤0.75, tricuspid inflow duration/cardiac cycle length ≤36.5%, and a right ventricle/left ventricle length ratio ≤0.64. If 3/4 markers are present, this predicts a non-BV outcome with sensitivity of 100% and specificity of 92%, and both are 100% if all the four criteria are fulfilled.

CONCLUSIONS: The postnatal outcome of fetuses with PA-CS/IVS can be predicted in the second trimester at the first echocardiography by a four-criterion scoring system. This is clinically relevant since it allows early selection of candidates for fetal intervention and early and precise parental counseling.