A case of persistent left and absent right superior caval vein: An anatomical and embryological perspective.

The relatively common persistent left superior caval vein (LSCV) is in most cases associated with doubling of the superior caval vein. A persistent LSCV with absent right superior caval vein (RSCV)-a rather rare event-was found during our course of gross anatomy. The LSCV drained into an enlarged coronary sinus, which was partly accompanied by an apparent "double" sinus of normal size draining into this enlarged coronary sinus. Histological and immunofluorescence studies using antibodies against smooth and cardiac muscle actins were performed. The terminal part of the LSCV near the opening into the right atrium contained cardiac actin as expected for a normal derivative of the left sinus horn. Previously only one case of doubled coronary sinus with LSCV has been reported and this abnormality was explained by splitting of the sinus. In our case, the partly doubled coronary sinus had the structure of coronary veins. Mechanical forces have been invoked for the obliteration of the LSCV. Therefore, we examined thirteen human embryos from 15 mm to 32 mm crown-rump length. In one embryo, we found a persistent LSCV together with an enormously enlarged left atrium. Contrary to previous suggestions our data indicate that during normal development a compression of the left anterior cardinal vein does not sufficiently explain the obliteration of the left and the persistence of the right vein. We therefore believe that beside a left dominated blood flow of head and arm, genes for left-right signaling may have to be taken into consideration. 2010 Wiley-Liss, Inc.