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Published on: May 5, 2018
Prenatal diagnosis of criss-cross heart: sonographical and pathological features of five cases
S Li1, G Luo, E R Norwitz
1Department of Ultrasound, Shenzhen Maternity and Child Healthcare Hospital, Affiliated to Southern Medical University, Shenzhen, China. lishengli63@126.com
Insights
Fetal criss-cross heart (CCH) is a rare developmental disorder. Sonography can accurately diagnose CCH prenatally, enabling targeted counseling and early intervention.
Area of Science:
- Cardiology
- Developmental Biology
- Medical Imaging
Background:
- Fetal criss-cross heart (CCH) is a rare congenital heart anomaly.
- Accurate prenatal diagnosis of CCH is crucial for management.
Purpose of the Study:
- To delineate the sonographical and pathological characteristics of fetal CCH.
- To establish diagnostic criteria for prenatal identification of CCH.
Main Methods:
- Retrospective review of fetal echocardiograms and autopsy reports for CCH cases (May 2003-May 2011).
- Analysis of demographic, genetic, sonographical, and pathological data.
- Description of characteristic sonographical findings using standard and Doppler ultrasound.
Main Results:
- Five cases of fetal CCH were identified and confirmed by autopsy.
- Key sonographical findings include difficulty obtaining the four-chamber view, spiraling interventricular septum, superior-inferior crossing ventricular inlets, and a sagittal four-chamber-like view.
- Doppler ultrasound revealed the criss-cross arrangement of ventricular inflow tracts.
Conclusions:
- Fetal criss-cross heart (CCH) is a rare developmental disorder with distinct sonographical features.
- Prenatal diagnosis of CCH is achievable through fetal echocardiography.
- Early identification facilitates genetic counseling and timely intervention strategies.
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.
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