250. ACHD: Partial Anomalous Pulmonary Venous Return (PAPVR) with Dr. Ian Harris

Описание: Partial anomalous pulmonary venous return refers to anomalies in which one or more (but not all) of the pulmonary veins connects to a location other than the left atrium. This causes left to right shunting which may have hemodynamic and therefore clinical significance, warranting repair in some patients.



Join CardioNerds (  / cardionerds  ) to learn about partial anomalous pulmonary venous return! Dr. Dan Ambinder (  / dr_danmd  ) (CardioNerds co-founder), Dr. Josh Saef (  / jsaef1  ) (ACHD FIT at the University of Pennsylvania and ACHD Series co-chair), and Dr. Tripti Gupta (  / t_guptamd  ) (ACHD FIT at Vanderbilt University and episode lead) learn from Dr. Ian Harris (https://www.ucsfhealth.org/providers/...) (Director of the Adult Congenital Heart Disease program at University of California, San Francisco). Audio editing by CardioNerds Academy Intern (https://www.cardionerds.com/academy/) , student doctor Shivani Reddy (  / shivanireddy_1  ) .



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Disclosures: None



Pearls (#pearls) • Notes (#notes) • References (#references) • Guest Profiles (#guests) • Production Team (#production)








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Pearls – Partial Anomalous Pulmonary Venous Return (PAPVR)




1. What is partial anomalous pulmonary venous return (PAPVR)?



• PAPVR refers to anomalies in which one or more (but not all) of the pulmonary veins connects to a location other than the left atrium. Often, this means one or more pulmonary veins empty into the right atrium or a systemic vein such as the superior vena cava or inferior vena cava. Physiologically, this produces a left-to-right shunt, allowing for already-oxygenated blood to recirculate into the lungs and result in excessive pulmonary blood flow. 





2. What are the clinical features of PAPVR?



• Diagnosis is usually incidental on a cross sectional imaging such as CTA or CMR.





• The most common associated lesion is an atrial-level defect.





• It is unusual for a single anomalous pulmonary venous connection of only 1 pulmonary lobe to result in significant shunting.





• Patients with a significant degree of left to right shunting may have right heart dilatation or symptoms of dyspnea on exertion.





3. When are some strategies for managing patients with PAPVR?



• A surgical correction is recommended for patients with PAPVR when functional capacity is impaired and RV enlargement is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (aka: ratio of pulmonary flow (Qp) to systemic flow (Qs) is ＞ 1.5:1), PA systolic pressure is less than 50% systemic pressure and pulmonary venous resistance is less than one third of systemic venous resistance.





• Surgical repair involves intracaval baffling of the left atrium (Warden procedure) or direct reimplantation o...