Partial anomalous pulmonary venous connection surgery

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

Recommendations for Anomalous Pulmonary Venous Connections Surgical Treatment

Class I

1.Surgical repair is recommended for patients with partial anomalous pulmonary venous connection when functional capacity is impaired and RV enlargement is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (eg, Qp:Qs ≥1.5:1), PA systolic pressure is less than 50% systemic pressure, and pulmonary vascular resistance is less than one third of systemic resistance. (Level of Evidence: B-NR)

2.Repair of partial anomalous pulmonary venous connection is recommended at the time of closure of a sinus venosus defect or ASD.(Level of Evidence: C-LD)

3.Repair of a scimitar vein is recommended in adults when functional capacity is impaired, evidence of RV volume overload is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (eg, Qp:Qs ≥1.5:1), PA systolic pressure is less than 50% systemic pressure and pulmonary vascular resistance is less than one third systemic.(Level of Evidence: C-LD)

Class IIa

1.Surgery can be useful for right- or left- sided partial anomalous pulmonary venous connection in asymptomatic adults with RV volume overload, net left-to-right shunt sufficiently large to cause physiological sequelae (eg, Qp:Qs ≥1.5:1), pulmonary pressures less than 50% systemic and pulmonary vascular resistance less than one third systemic.(Level of Evidence: B-NR)

2. Surgery can be useful for repair of a scimitar vein in adults with evidence of RV volume overload, with Qp:Qs 1.5:1 or greater.