Fetal cardiac dextroposition in the absence of an intrathoracic mass: Signof significant right lung hypoplasia

We reviewed our experience of fetal cardiac dextroposition in the absence o f an intrathoracic mass. Ten cases were found by fetal echocardiography to have a normal cardiac axis, but the heart was shifted into the right chest and the amount of right lung tissue was reduced. At birth seven of the infa nts had confirmed structural heart disease (70%), including three with scim itar syndrome. Two infants had additional extracardiac anomalies (20%). Sev en infants born at term had clinical pulmonary hypertension with a diagnosi s of right lung hypoplasia in all of them. Two neonates died owing to signi ficant heart disease (one with scimitar syndrome and the other with hypopla stic left heart syndrome). Of the three pregnancies that were terminated, t he two fetuses with autopsies had severe right lung hypoplasia. Fetal cardi ac dextroposition and right pulmonary artery hypoplasia in the absence of a n intrathoracic mass are important signs of right lung hypoplasia, which ca n be associated with significant pathologic cardiac and extracardiac condit ions.