38
Specific Conditions
4.1.3. Atrioventricular Septal Defect
COR LOE
Recommendations
Diagnostic
1 C-LD
1. Adults with an unrepaired AVSD or those with residual shunts
after prior repair should be assessed for the presence of PAH to
guide medical therapy and assess suitability for repair.
1 C-LD
2. Adults with an AVSD (repaired or unrepaired) and
PAH should be managed by clinicians with pulmonary
hypertension expertise to improve outcomes.
erapeutic
1 B-NR
3. In adults with repaired AVSD and LV outflow tract (LVOT)
obstruction with symptoms attributable to the obstruction,
or LV systolic dysfunction (LV ejection fraction <50%),
surgical repair is recommended to improve functional status
and preserve ventricular function.
1 B-NR
4. In adults with repaired AVSD with a) symptomatic severe left
atrioventricular valve regurgitation or b) asymptomatic severe
left atrioventricular valve regurgitation with LV dilation (LV
end-systolic diameter ≥40 mm) or LV systolic dysfunction
(LV ejection fraction <60%), valve surgery is recommended
to improve functional status and prevent worsening
ventricular function.
1 C-LD
5. In adults with an unrepaired AVSD or those with residual
shunts after previous repair, significant left-to-right shunt
(Qp:Qs ≥1.5), and/or significant or progressive atrial/
ventricular dilatation with no PAH (PVR ≤2 Wood units),
closure of the AVSD or residual shunt is recommended to
improve functional class and clinical outcomes.
2a C-EO
6. In adults with an unrepaired AVSD or those with residual shunts
after previous repair, significant left-to-right shunt (Qp:Qs
≥1.5), and/or significant or progressive atrial/ventricular
dilatation with mild PAH (PVR >2 to <5 Wood units), closure
is reasonable to improve functional class and clinical outcomes.
2b C-LD
7. In asymptomatic adults with AVSD, LVOT obstruction, and
progressive greater than mild aortic regurgitation, surgical
repair may be reasonable to prevent worsening ventricular
function and progressive aortic valve disease.
3: Harm C-LD
8. In adults with an unrepaired AVSD or those with residual
shunts after previous repair demonstrating Eisenmenger
physiology or PVR >10 Wood units, shunt closure should not
be performed to avoid additional morbidity and mortality.
