Extract

A 27-year-old asymptomatic man presented for evaluation of pulmonary emphysema found during a routine examination. Contrasted computed tomography showed localized pulmonary emphysema in the right inferior lobe (Panel A, black arrow heads), and revealed an aberrant shunt connecting the right inferior pulmonary vein (RIPV) and portal vein (PoV), forming an oesophageal (Eo) varix (Panels B–D, white arrow heads). Four pulmonary veins typically converged at the left atrium (LA) (Panels C–D); thus, the anomalous pulmonary venous connection was denied. Gadolinium-enhanced magnetic resonance imaging demonstrated that blood flow in the shunt drained from RIPV into PoV (Panel E, white arrow heads, Supplementary data online, Video S1). Cardiac catheterization revealed normal portal venous pressure (8 mmHg), as well as normal pulmonary arterial and left atrial pressure. The shunt was suspected not to be secondary to portal hypertension; instead, it was considered a congenital malformation.

The shunt was large (maximally >20 mm); however, the volume of this left-to-right shunt was considered to be minimal for the following reasons: (i) right heart size and pressure were normal. Pathophysiologically, blood traveled from PoV to the pulmonary circulation through the liver circulation, leading to volume overload in the right heart, depending on the shunt volume and (ii) the shunt exhibited low flow because RIPV-PoV pressure gradient was minimal.

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