Right-Dominant Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical Decision Making

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Echocardiography

Background:

• Right-dominant atrioventricular septal defect (AVSD) presents management challenges due to varying ventricular hypoplasia.
• Accurate assessment of echocardiographic indices is crucial for surgical planning in right-dominant AVSD.

Purpose of the Study:

• To evaluate if specific echocardiographic indices and novel measurements correlate with the chosen surgical approach in right-dominant AVSD.
• To identify reliable echocardiographic markers for guiding operative strategy in complex congenital heart defects.

Main Methods:

• Retrospective analysis of preoperative echocardiograms from 46 patients with right-dominant AVSD.
• Measurement of ventricular dimensions, atrioventricular valve index (AVVI), and right ventricular/left ventricular (RV/LV) inflow angle.
• Correlation analysis to assess the relationship between ventricular septal defect size and RV/LV inflow angle; validation in a separate cohort.

Main Results:

• Patients undergoing single-ventricle palliation had smaller left ventricles and lower AVVI and RV/LV inflow angles compared to those with biventricular operations.
• A strong negative correlation was found between ventricular septal defect size and RV/LV inflow angle in systole (r = -0.7, P < .001).
• In the validation cohort, RV/LV inflow angle ≤ 114° and AVVI ≤ 0.70 showed high sensitivity for predicting single-ventricle palliation.

Conclusions:

• Mortality remains significant in right-dominant AVSD cases.
• RV/LV inflow angle in systole and AVVI are reproducible echocardiographic measurements.
• These indices, along with other parameters, can aid in determining suitability for biventricular repair in right-dominant AVSD.