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Unilateral total anomalous pulmonary venous connection from a unilateral lung is extremely rare. A 6-year-old patient with anomalous pulmonary venous connection from the entire left lung to the left innominate vein, with an intact atrial septum, diagnosed by two-dimensional and Doppler echocardiography is reported. The combination of two-dimensional and Doppler echocardiography is very useful not only for anatomical diagnosis, but also for evaluation of its hemodynamics, despite the wide anatomical variability of the pulmonary venous connection. This is the first report of a case of left total anomalous pulmonary venous connection diagnosed noninvasively.

A case of total left anomalous pulmonary venous connection with intact atrial septum diagnosed by two-dimensional and Doppler echocardiography.