Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies

Abstract

We report a case of a healthy, asymptomatic 6-year-old boy in whom an anomalous right pulmonary vein was noted to drain into both the inferior vena cava and left atrium in association with findings consistent with scimitar syndrome. The anomalous pulmonary vein took a very circuitous route through the lungs before draining into the left atrium, a condition previously termed "meandering pulmonary vein." To aid in the diagnosis, cardiovascular magnetic resonance imaging and magnetic resonance angiography were used to delineate this complex course and the connection of the anomalous pulmonary vein. To our knowledge, this is the 1st reported case of a meandering pulmonary vein with dual drainage to the inferior vena cava and left atrium in association with other anomalies.

Fig. 1 A) Posteroanterior chest radiograph shows mesocardia with a prominent right-heart border; a circular shadow (arrow) at the superolateral aspect of the upper right-heart border represents the meandering pulmonary vein as it courses back to the left atrium. B) This posteroanterior chest radiograph shows an anomalous vessel (arrow) coursing down the right-heart border. It resembles the Turkish sword called a scimitar.

Fig. 2 Sagittal frames from multislice cine MRI study of patient, reformatted with local maximal intensity projection. A) Rightward positioned section. White arrow indicates position of anomalous right lower pulmonary vein. B) Medially positioned section. White arrow indicates position of anomalous right lower pulmonary vein. Note that the right lower pulmonary vein has a small communication with the inferior vena cava. A = anterior; I = inferior; MRI = magnetic resonance imaging; P = posterior; S = superior

Fig. 3 Coronal frames from multislice cine MRI study of patient, reformatted with local maximal intensity projection. A) Posteriorly positioned section. White arrow indicates position of anomalous right lower pulmonary vein. B) Anteriorly positioned section. White arrow indicates position of anomalous right lower pulmonary vein. Note the common right pulmonary vein draining into the left atrium. I = inferior; L = left; MRI = magnetic resonance imaging; R = right; S = superior

Fig. 4 Maximal intensity projections of 3-dimensional contrast-enhanced MRA study of patient with sensivity encoding (SENSE) technique. A) White arrow indicates position of the anomalous right lower pulmonary vein. B) White arrow indicates position of the anomalous artery that originates from the abdominal aorta and supplies the right lower lobe. I = inferior; L = left; MRA = magnetic resonance angiography; R = right; S = superior

Fig. 5 Simultaneous angiograms are performed in the anteroposterior projection into the right pulmonary artery (RPA) and systemic collateral artery. A portion of the medial-basal and posterior-basal segment of the right lower lobe perfused by the collateral vessel does not have dual supply from the right pulmonary arterial system.