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Persistent left superior vena cava, absence of the innominate vein, and upper sinus venosus defect

A rare anomaly detected using bubbles

Persistierende V. cava superior sinistra, Fehlen der V. innominata und oberer Sinus-venosus-Defekt

Eine seltene, mittels Gasbläschen diagnostizierte Anomalie

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Abstract

Superior vena cava anomalies are rare malformations that are typically seen with other congenital cardiac defects. Although a persistent left superior vena cava is the most common anomaly of the systemic venous return in the thorax, its combination with an upper sinus venosus defect and absence of the innominate vein is extremely rare. Here, we report a patient diagnosed with these anomalies based on a bubble study and confirmed with magnetic resonance imaging.

Zusammenfassung

Anomalien der V. cava superior sind seltene Fehlbildungen, die gewöhnlich zusammen mit anderen angeborenen Herzfehlern festgestellt werden. Eine persistierende V. cava superior sinistra stellt zwar die häufigste Anomalie bei den Strukturen des systemischen Rückflusses in den Thorax dar, aber die Kombination mit einem oberen Sinus-venosus-Defekt und dem Fehlen der V. innominata ist äußerst selten. Hier wird ein Patient vorgestellt, bei dem diese Anomalien mithilfe einer Echokardiographie mit Gasbläschen als Kontrastmittel diagnostiziert und durch Magnetresonanztomographie bestätigt wurden.

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Conflict of interest

On behalf of all authors, the corresponding author states that there are no conflicts of interest.

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Correspondence to I. Akpinar MD.

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Additional material online: This article includes four additional Videos. This supplemental material is available at dx.doi.org/10.1007/s00059-012-3704-z.

Electronic supplementary material

Video 1: Parasternal long-axis view of the heart: When the agitated saline was administered via the left antecubital vein, bubbles were seen in right atrium and ventricle via the coronary sinus. Since there were no bubbles in the left atrium, the roof of the CS was intact. (QuickTime.mov 1,3MB)

Video 2: Parasternal long-axis view of the heart: When the agitated saline was given via the right antecubital vein, bubbles were detected throughout the heart, indicating a right to left shunt. (QuickTime.mov 1.1MB)

Video 3: Apical four-chamber view of the heart: When the agitated saline was administered via the left antecubital vein, bubbles were seen in right atrium and ventricle via the coronary sinus. Since there were no bubbles in the left atrium, the roof of the CS was intact. (QuickTime.mov 0,5MB)

Video 4: Apical four-chamber view of the heart: When the agitated saline was given via the right antecubital vein, bubbles were detected throughout the heart, indicating a right to left shunt. (QuickTime.mov 0,6MB)

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Akpinar, I., Sayin, M., Karabag, T. et al. Persistent left superior vena cava, absence of the innominate vein, and upper sinus venosus defect. Herz 38, 317–320 (2013). https://doi.org/10.1007/s00059-012-3704-z

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  • DOI: https://doi.org/10.1007/s00059-012-3704-z

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