Meandering pulmonary vein syndrome

Clinical History

A 32-year-old male, who was a HIV seropositive patient, presented with a right pulmonar hilum tumefaction. Imaging investigations were performed to precisely evaluate.

Imaging Findings

This patient had a history of esophageal and anal congenital malformations. He was diagnosed with HIV seropositivity after a blood donation. He was a healthy man with no clinical complaints. A cardiopulmonary examination was found to be normal. His chest X-ray on admission showed an enlarged right hilum. A lymphadenopathy was suspected, and an enhanced CT-scan was performed. It demonstrated an abnormal pulmonary vascularity with a right superior vein (RSPV) coursing posterior to the bronchus intermedius, then joining the ipsilateral inferior vein, forming a common trunk. This one normally entered the left atrium. There was no abnormal lymph node present. A complementary MR scan with three dimensional contrast-enhanced magnetic resonance angiography was performed in order to eliminate the possibility of any associated cardiovascular malformation. The MR scan confirmed an isolated anomalous course of the right superior pulmonary vein.

Discussion

Anomalous venous pulmonary return (AVPR) is defined by the presence of one or more than one pulmonary veins not connected with the left atrium. Different variations have been described. Total AVPR is always associated with an atrioseptal defect and requires immediate surgery at birth. Various clinical manifestations can reveal partial AVPR; they depend on the degree of abnormal return and the presence or absence of an atrioseptal defect. In this case, there was no abnormal venous return. The pulmonary blood from the right upper lobe drains normally into the left atrium via a large trunk. This abnormal venous course is named "meandering pulmonary vein". It has been described in association with right pulmonary hypoplasia and the appearance of a scimitar radiological sign. The "meandering vein" can be considered a variant of the scimitar syndrome, even though in this case, there was no abnormal venous return at all. It can be included as part of a multiple malformation syndrome such as in this patient. Of course, no treatment was necessary in this patient. A gadolinium enhanced-MRA procedure provided a clear depiction of the abnormality, and a fine explanation of the abnormal X-ray.

Differential Diagnosis List

Final Diagnosis

Meandering vein syndrome.

URL:	https://www.eurorad.org/case/3129
DOI:	10.1594/EURORAD/CASE.3129
ISSN:	1563-4086