[Anatomic spectrum between complete and partial atrioventricular septal defect. Two and three-dimensional...

Area of Science:

• Cardiology
• Medical Imaging
• Pediatric Heart Disease

Background:

• A morphogenetic link is proposed between Rastelli type A atrioventricular septal defect (AVSD) and partial forms with two separate orifices.
• A spectrum of anatomical forms exists, ranging from VSD size determined by interchordal spaces to VSD closure by septal valve fusion.

Purpose of the Study:

• To illustrate the anatomical variability of atrioventricular septal defects using advanced echocardiography.
• To correlate echocardiographic findings with clinical presentation and hemodynamic status.

Main Methods:

• Utilized two-dimensional and three-dimensional echocardiography in five patients.
• Performed transesophageal echocardiograms with 3D reconstruction using an Echo-Scan system.

Main Results:

• Observed a spectrum of AVSD, including complete VSD closure, partial VSD closure, and large VSD with significant shunting and pulmonary hypertension.
• Hemodynamic behavior in partially or completely closed VSD depended on interatrial shunt and AV valve regurgitation.
• Patients without pulmonary hypertension were less symptomatic.

Conclusions:

• Three-dimensional echocardiography accurately defines valve components and septal structures in AVSD type A.
• This imaging modality enhances understanding of the transition between complete and partial forms of AVSD.
• The specific anatomical form significantly influences the clinical evolution and patient prognosis.