Right atrial isomerism diagnosed by STIC-HD live flow and autopsy: A case report

Right atrial isomerism (RAI) is one of the most severe forms of congenital heart disease. This case of RAI was so complex that it incorporated 7 heart defects. It can be challenging to display the spatial relationship between different anatomical structures using conventional two-dimensional and color ultrasound (2D-Doppler imaging); therefore, we used spatio-temporal image correlation (STIC) and high definition live flow imaging technology to vividly display this case of RAI in a stereoscopic mode.

A 24-year-old woman was referred to our tertiary center at 24 weeks of gestation. The woman had difficult conceiving. Once pregnant, she was opposed to abortion, even if there was a possibility of deformity.

The fetus presented with an atrioventricular septal defect, persistent left superior vena cava, supra-cardiac total anomalous pulmonary venous connection (TAPVC), double outlet right ventricle, right ductus arteriosus, right aortic arch (RAA) with mirror image branching, and aortic arch dysplasia.

After consulting a pediatric cardiologist, the woman requested an abortion and consented to an autopsy.

Autopsy supported the echocardiographic findings.

Accurate diagnosis of RAI is essential for clinical and parent decision making. 2D-Doppler imaging combined with STIC-HD live flow can be used to visualize the spatial morphology of blood vessels, including the cardiac chambers and great vessels of the fetal heart, and smaller peripheral vessels.