Prenatal diagnosis of criss-cross heart: sonographical and pathological features of five cases

Abstract

Objective:

To describe the sonographical and pathological features of fetal criss-cross heart (CCH).

Study Design:

All cases of fetal CCH diagnosed by fetal echocardiogram from May 2003–May 2011 were identified at a single referral center using an established perinatal database. Demographic and genetic information, sonographical images and autopsy reports were reviewed. Sonographical and pathological features are described.

Result:

Five cases of fetal CCH were identified, all of which were confirmed by autopsy. Characteristic sonographical findings include: (1) the inability to obtain four-chamber view at standard transverse plane through the fetal chest; (2) appreciation of the misaligned spatial atrial–ventricle connection with the interventricular septum in a ‘spiraling’ orientation; (3) orientation of the two ventricular inlets in a superior–inferior and crossing position; and (4) a four-chamber-like view seen in the sagittal plane of the fetal chest. Doppler ultrasound demonstrates the ‘criss-cross’ arrangement of the inflow tracts into the two ventricles simultaneously in the transverse plane of the fetal chest.

Conclusion:

CCH is a rare developmental disorder that can be accurately diagnosed prenatally. Early diagnosis will allow for more targeted counseling and early intervention.