This is a prospective, international, multi-institutional study.

Inclusion criteria: patients with diagnosis of right dominant uAVSD defined as mAVVI < 0.4.

Data on each patient must be submitted through this website, including echocardiographic preoperative assessment, surgical protocol and inpatient postoperative follow-up. Updates must be submitted at 3 months, 6 months and 1 year after surgery.

The echocardiographic protocol is simple. Six echocardiographic measurements must be taken in apical 4-chamber view, end-diastole, one frame before AVV closure. Average of three different measures in three different images of the 4C view must be calculated.

Figure 1

Measurements:

  1. The diameter of the atrioventricular valve (AVV) annulus (cm), between left and right hingepoints of the AVV annulus (Figure 1).
  2. The diameter of the ventricular septal defect (VSD) (cm), measured perpendicularly to the AVV. Trace a line from the crest of the ventricular septum to the previously drawn line of the AVV annulus, achieving a 90-degree angle between the two lines (Figure 1).
  3. The diameter of the left component (LC) of the AVV (cm), from the left hingepoint of the AVV to the point of intersection of the VSD line with the AVV (Figure 1).
  4. The diameter of the secondary annulus (SA) (cm), at the narrowest diameter of color Doppler at LV inflow, as a line extended between the crest of the interventricular septum and the LV wall (Figure 1).
  5. The diameter of the ostium primum atrial septal defect (ASD) (cm), measured perpendicularly to the AVV. Trace a line from the end point of the interatrial septum to the previously drawn line of the AVV annulus, achieving a 90-degree angle between the two lines (Figure 2).
  6. The diameter of the left atrial outflow (LAO) (cm), measured from the left hingepoint of the AVV to the point of intersection of the ASD line with the AVV (Figure 2).
  7. Figure 2

    Then, the following indexes are calculated:

    1. mAVVI: ratio between size of the LC of the AVV and total AVV diameter.
    2. inVSD: ratio between VSD size and total AVV diameter.
    3. inSA: ratio between size of the SA and total AVV diameter. This value must be compared to its critical value, using the following Table

    Figure 3

    Finally, selection of the surgical strategy to be followed is made using the proposed algorithm.

    Figure 4