Echocardiographic and anatomic findings in atrioventricular discordance with ventriculoarterial concordance

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Atrioventricular (AV) discordance with ventriculoarterial (VA) concordance is a rare congenital heart disease with 5 known anatomic types.
• Anatomic correction is achievable via an atrial switch procedure, addressing associated septal defects.

Purpose of the Study:

• To present three cases of AV discordance with VA concordance.
• To emphasize the diagnostic utility of 2-dimensional echocardiography and surgical management strategies.
• To document a previously undescribed form of congenital heart disease involving ventricular inversion.

Main Methods:

• Comprehensive segmental analysis using multiple-plane 2-dimensional echocardiography.
• Determination of visceroatrial situs, ventricular loop, and great arterial position/alignment.
• Surgical intervention including atrial switch and ventricular septal defect closure.

Main Results:

• Two-dimensional echocardiography proved highly reliable for complex defect anatomy assessment.
• A novel case of ventricular inversion with inverted normally related great arteries in situs solitus was identified.
• Surgical closure of a ventricular septal defect via the left-sided infundibulum in isolated ventricular inversion provided excellent exposure and avoided AV conduction block.

Conclusions:

• A segmental approach to 2-dimensional echocardiography is crucial for diagnosing complex AV discordance with VA concordance.
• Atrial switch procedures are effective for anatomic correction.
• Specific surgical techniques, like left-sided infundibular VSD closure, can optimize outcomes in rare presentations.