2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines[1]
Recommendations for Anomalous Pulmonary Venous Connections
| Class I
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| 1."CMR or CTA is recommended for evaluation of partial anomalous pulmonary venous connection. (Level of Evidence: B-NR)"
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| Class IIa
|
| 1."Cardiac catheterization can be useful in adults with partial anomalous pulmonary venous connection to further define hemodynamics (Level of Evidence: B-NR)"
|
Volume overload of the right heart caused by an abnormal link between a pulmonary vein and a systemic vein will have physiological effects resembling those of an ASD. However, anomalous pulmonary venous connection differs from related ASD in that there is no chance for right-to-left shunting, and the severity of the left-to-right shunt is not increased by the onset of acquired left heart disease. The right upper pulmonary vein's connection to the superior vena cava, which may be accompanied by a sinus venosus defect, is the most frequent aberrant pulmonary venous connection.
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