Abstract
We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism
in association with tricuspid atresia. Several distinctive sonographic features of
isomerism of the right atrial appendages were present in this fetus: complex cardiac
abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior
vena cava to the right side, pulmonary atresia, and anomalous pulmonary venous return
to the morphological right atrium. Tricuspid atresia, which is an extremely rare lesion
within heterotaxy spectrum disorders, was present. Postnatal investigations confirmed
all prenatally diagnosed abnormalities, with additional findings of pulmonary atresia
with discontinuous pulmonary arteries and bilateral arterial ducts, asplenia, and
bilateral eparterial bronchi. To our knowledge, tricuspid atresia in the setting of
isomerism of the right atrial appendages has not previously been diagnosed or reported
prenatally. Because of the complexity of cardiac lesions that may be present in cases
of atrial isomerism, these disorders should be considered even if sonographic findings
are uncommon or atypical.
Keywords
fetus - heterotaxy - right atrial isomerism - tricuspid atresia - echocardiography
- ultrasound
