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. 2012:2012:461040.
doi: 10.1155/2012/461040. Epub 2012 Jun 20.

Bilateral absence of the superior vena cava

Kaisa Ylänen  1 , Tuija Poutanen  2 , Päivi Savikurki-Heikkilä  3 , Jukka Uotila  4 , Matti Korppi  1 , Anneli Eerola  2
Affiliations

Bilateral absence of the superior vena cava

Kaisa Ylänen et al. Case Rep Cardiol. 2012.

Abstract

Bilateral absence of the superior vena cava (SVC) is a very rarely detected, mainly asymptomatic congenital vascular anomaly. Though usually innocent, this anomaly may complicate cardiothoracic surgery and certain procedures like central venous catheter insertion. This SVC anomaly is poorly known, and we assume that its incidence in the general population may be higher than detected. In this paper, we summarize current knowledge on this anomaly and its clinical implications. In addition, we present a neonatal case with bilateral absence of the SVC associated with a fetal cystic hygroma. Conclusion. Totally absent SVC can cause unexpected problems during cardiothoracic surgery. Suspicion of SVC absence should arise in basic echocardiography. Our paper suggests that, like other congenital anomalies, bilateral absent SVC may be associated with a fetal cyctic hygroma.

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Figures

Figure 1
Figure 1
Ultrasound images prenatally and postnatally. (a) Fetal scan at the 12th week of pregnancy demonstrating moderate nuchal edema and cystic hygroma. (b) Pathological flow of the venous duct at the 20th week. During atrial contractions, a reverse flow is noticeable. (c) Two-dimensional echocardiogram of a newborn with absent SVC. The azygos and the hemiazygos veins have fused, and the venous duct formed is draining into the IVC.
Figure 2
Figure 2
(a) Schematic drawing of azygos vein and hemiazygos vein fusion in the thoracic cavity. The venous duct formed is draining into the IVC. (b) Sagittal CT scan showing venous duct draining into the IVC (asterix).
Figure 3
Figure 3
3D MRA construction of bilateral SVC absence from a posterior view showing the aorta and venous structures around the diaphragm. *Right azygos vein. **Left hemiazygos vein. ***Fused azygos and hemiazygos veins joining the IVC. Liver (L).
See this image and copyright information in PMC

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