CTICasesPartial Anomalous Pulmonary Venous Connection (PAPVC) with Sinus Venosus ASD
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Imaging findings
A patient with a history of bronchogenic cyst resection in childhood showed a generous pulmonary artery on radiograph, confirmed by MRA to be enlarged. The right pulmonary artery was also enlarged. An anomalous drainage of a pulmonary vein into the SVC was identified, along with a communication between the SVC and the left atrium, consistent with a sinus venosus ASD. The shunt (QP/QS) was 1.29, with mildly abnormal RV function and normal LV function.
Key takeaways
Partial anomalous pulmonary venous connection (PAPVC), particularly of a right pulmonary vein into the SVC, is frequently associated with a sinus venosus atrial septal defect (ASD). This combination creates a left-to-right shunt, leading to right heart and pulmonary artery enlargement. While often managed conservatively for smaller shunts, it represents a significant congenital anomaly.
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