Images and Case Reports in Arrhythmia and Electrophysiology |
From the Cardiology Unit, Cardiovascular Department (P.D.F., R.B., A.B., F.C.), Ospedali Riuniti di Bergamo, Bergamo and Biosense Webster (S.C.), Milan, Italy.
Correspondence to Francesco Cantù, MD, Cardiology Unit, Cardiovascular Department, Ospedali Riuniti di Bergamo, Largo Barozzi 1, 24128 Bergamo, Italy. E-mail fcantu@ospedaliriuniti.bergamo.it
An extract of the first 250 words of the full text is provided, because this article has no abstract. |
A 49-year-old woman affected by the Turner syndrome with a history of repaired congenital cardiomyopathy and persistent atrial tachycardia was referred to our center for radiofrequency ablation. At 11 years of age, she had undergone a surgical closure of an ostium secundum atrial septal defect (direct suture) combined with ligation of a persistent left superior vena cava, the embryological precursor of the ligament of Marshall, at the level of its connection to the coronary sinus. At hospitalization, her electrocardiogram showed an atrial tachycardia (cycle length 375 ms) with long phases of 1:1 atrioventricular conduction. Before the procedure, a computed tomography (CT) scan was performed to merge the anatomic data with the electrophysiological findings during the procedure (CARTO-MERGE, Biosense Webster, Inc, Diamond Bar, Calif). The preacquired CT image, processed with CARTO-MERGE system, showed the presence of a giant left superior vena cava (isolated from the coronary sinus) and a sinus venosus defect (SVD) associated with a right-sided anomalous pulmonary venous connection. In detail, both right superior pulmonary vein and right inferior pulmonary vein drained into the superior caval atrial junction. Van Praagh et al1 reported that SVD appears to be a deficiency in the wall that normally separates the right pulmonary veins from the right sinus venosus. This deficiency unroofs the pulmonary vein, permitting it to drain into the right side of the heart (Figure 1).
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