40
Specific Conditions
4.1.4. Anomalous Pulmonary Venous Connections
COR LOE
Recommendations
Diagnostic
1 B-NR
1. For adults with APVCs, cardiovascular magnetic resonance
(CMR) imaging is recommended to assess pulmonary venous
anatomy, to evaluate RV size and function, and to determine
shunt fraction (Qp:Qs).
1 B-NR
2. For adults with APVCs who do not have an indication for
repair, routine cardiac imaging is recommended for serial
evaluation of RV size and function and for assessment of
pulmonary hypertension.
1 B-NR
3. For adults with repaired APVCs and dyspnea on exertion
and/or reduced functional capacity, CMR or computed
tomography (CT) angiography is recommended to evaluate
for venous obstruction or residual shunt.
2a B-NR
4. For adults with unrepaired APVCs and noninvasive evidence
of, and/or risk factors for, PAH, cardiac catheterization can
be useful to evaluate hemodynamics and aid in determining
indication for repair.
erapeutic
1 B-NR
5. For adults with APVCs who have cardiac symptoms and/
or reduced functional capacity with RV volume overload,
and whose Qp:Qs is ≥1.5, surgical repair is recommended to
improve symptoms and clinical outcomes
1 B-NR
6. For adults with APVCs undergoing surgical repair for
an ASD, repair of the APVC(s) is recommended, when
technically feasible, to reduce left-to-right shunting and RV
volume overload.
2a B-NR
7. For adults with APVCs who are asymptomatic; whose Qp:Qs
is ≥1.5; and who have severe or progressive RV volume
overload, reduced RV systolic function, PAH by invasive
measurement, or objective exercise intolerance, surgical
repair* is reasonable in order to improve hemodynamics and/
or stabilize RV size and function.
3: No
Benefit
B-NR
8. For adults with APVCs who have a normal to mildly enlarged
right ventricle and whose Qp:Qs is <1.5, surgical repair is not
indicated, given the absence of data to support benefit.
