Discussion Author(s): Primary author: Jason Messinger
Contributing author: Eric Jones
Anomalous pulmonary venous return ("APVR") is a rare condition in which one to all pulmonary veins feed back into systemic venous circulation.
Different forms of APVR are classified as supracardiac, cardiac, infradiaphragmatic, or mixed.
In the supracardiac form, the aberrant vessel or vessels drain into one of the brachiocephalic veins or into the superior vena cava. In the cardiac form the drainage is into the coronary sinus or directly into the right atrium. In the subdiaphragmatic form the abnormal connection can be to the portal vein, one of the hepatic veins, or into the inferior vena cava.
When the right pulmonary vein drains into the inferior vena cava the condition is termed â€śscimitar syndromeâ€ť due to a characteristic scimitar-like appearance of the abnormal vein on conventional radiographs of the chest. In this syndrome, an abnormal systemic artery may also subserve the lung.
Total APVR can present in the posttenatal period and is lethal except when associated with a large atrial septal defect, patent foramen ovale, or patent ductus arteriosus. It can be diagnosed in the antenatal period with careful obstetrical ultrasound. Treatment is always surgical. Cardiac catheterization and echocardiography inform the surgeon.
Partial APVR can present later in life, usually with fatigue and dyspnea, and is most precisely diagnosed using the three dimensional imaging techniques of cardiac CT or cardiac MRI.
The pathophysiology of APVR is a complex topic beyond the scope of this factoid.
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