Anomalous Connection of the Right Superior Vena Cava to the Left Atrium in a Child with Bilateral Superior Vena Cavae: An Unusual Cause of Cyanosis

Abstract

Anomalous drainage of the right superior vena cava into the left atrium (LA) has been previously reported in the medical literature. We present a unique case of a child who had two superior vena cavae, a left superior vena cava that drained normally through the coronary sinus to the right atrium, and a right superior vena cava that drained anomalously to the LA.

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Acknowledgements

The author deeply thanks Dr Syed Aitizaz Uddin, Consultant Cardiac Surgeon, and Dr. Mohamed Mofeed Morsy, Professor of Pediatrics and Pediatric Cardiology Madinah Cardiac Center in editing and improving the linguistics of this scientific paper.

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Correspondence to Saad Khoshhal.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Video S1—Subcostal short-axis transthoracic echocardiographic view showing the right superior vena cava (RSVC) connected and draining to the left atrium (LA) with absence of a normal RSVC connection and drainage to the right atrium (RA) (MP4 7815 KB)

Video S2—Subcostal short-axis transthoracic (color Doppler) echocardiographic view showing the RSVC connected and draining to the LA with absence of a normal RSVC connection and drainage to the RA (abbreviations as in video 1) (MP4 5153 KB)

Video S3—Transesophageal bicaval echocardiographic view after a saline injection (bubble test) in the right arm shows opacification of the LA initially rather than the RA, confirming an RSVC connection and drainage to the LA (RA opacified later through a patent foramen ovale (PFO) (abbreviations as in video 1) (MP4 11575 KB)

Video S4—Transesophageal bicaval echocardiographic (zoomed view) after a saline injection (bubble test) in the right arm shows opacification of the LA initially rather than the RA, confirming an RSVC connection and drainage to the LA (RA opacified later through (PFO) (abbreviations as in videos 1 and 3) (MP4 15106 KB)

Video S5—RSVC angiography (using 4 Fr access to the right jugular vein) showing that the RSVC is connected to the LA, which is connected to the left ventricle. Another catheter is placed in the left SVC, which drains into the RA through a dilated coronary sinus (abbreviations as in video 1) (MP4 7131 KB)

Video S6—Left superior vena cava angiography (LSVC) showing the LSVC draining in the right atrium through a dilated coronary sinus (MP4 6921 KB)

Video S7—Surgical view showed RSVC connected and draining to the LA before surgical correction (abbreviations as in video 1) (MP4 1378 KB)

Video S8—Surgical view showed RSVC connected and draining normally to the RA after surgical rerouting of RSVC to RA (abbreviations as in video 1) (MP4 898 KB)

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Khoshhal, S. Anomalous Connection of the Right Superior Vena Cava to the Left Atrium in a Child with Bilateral Superior Vena Cavae: An Unusual Cause of Cyanosis. Pediatr Cardiol 40, 226–229 (2019). https://doi.org/10.1007/s00246-018-2013-6

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Keywords

  • Abnormal systemic venous return
  • Cyanosis
  • Congenital heart disease