Abstract

A 20-week fetus was referred by the Gynecology Service with the diagnosis of persistent left superior cava vein (PLSCV). The three-vessel echographic view of the left upper mediastinum showed from right to left, a right superior vena cava, the aorta, the pulmonary trunk and a vein structure compatible with PLSCV. The other eco views of the fetal heart were apparently normal and not recorded. Just after birth, the baby presented a mild cyanosis (satO2 85%). The echocardiogram revealed a non-obstructive total anomalous pulmonary venous drainage to the innominate vein. The vessel previously identified as a PLSCV turned out to be a vertical vein. No other anomaly was detected and the baby was successfully operated on. When the three-vessel view record was reviewed, we realized that the relative sizes of the vessels were anomalous, with the aorta and the right superior cava vein of similar size. In normal hearts with a PLSCV, the venous drainage from the upper body divides between both superior cava veins, both sizes in the 3-vessels view being clearly smaller than that of the aorta. This diagnostic clue was overlooked in the present case. This fact, along with the previous normal appearance of the other views of the fetal heart, led us to a wrong diagnosis. The total anomalous pulmonary venous drainage in an otherwise normal fetal heart remains a challenging diagnosis, in which a high degree of suspicion and a careful study of several views of the heart are needed. In a three-vessel view the presence of a vein structure (fourth vessel) left to pulmonary artery may be diagnosed as a PLSCV or a vertical vein in a total supracardiac pulmonary venous drainage. A right superior vena cava size smaller than that of the aorta points to a PLSCV diagnosis; otherwise it points to a vertical vein diagnosis.

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