Anomalous connection of the right superior caval vein to the morphologically left atrium

Gorenflo, Matthias; Sebening, Christian; Ulmer, Herbert E.

doi:10.1017/s1047951106000151

Cited by 12 publications

(13 citation statements)

References 6 publications

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“…Drainage of the right SVC to the LA is an exceedingly rare congenital malformation of systemic veins [1]. In a previously published case report, Van Praagh and colleagues were able to identify 18 cases of LA drainage of the right SVC, and an additional seven cases of biatrial drainage of right SVC in viscera-atrial situs solitus [2].…”

Section: Discussionmentioning

confidence: 99%

Bilateral superior vena cava with right superior vena cava draining into left atrium

Alghamdi

Elfaki

Alhabshan

et al. 2015

Journal of the Saudi Heart Association

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Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2 years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88-90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.

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Section: Discussionmentioning

confidence: 99%

Bilateral superior vena cava with right superior vena cava draining into left atrium

Alghamdi

Elfaki

Alhabshan

et al. 2015

Journal of the Saudi Heart Association

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“…There are several cases of this defect in association with other congenital heart defects and heterotaxy, but through our review only 42 cases of this as an isolated anomaly have been reported in the English literature to date 1,3‐43 . Table 1 summarizes the reported cases as case number, year of publication, first author, age and gender of patient, presentation, method of diagnosis, and the outcome.…”

Section: Discussionmentioning

confidence: 99%

Isolated right superior vena cava drainage into the left atrium: An uncommon cause of persistent hypoxia in a neonate

et al. 2020

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This report describes the case of a 13-day-old African American female with persistent hypoxia found to have an isolated right superior vena cava (RSVC) draining into the left atrium (LA) confirmed with contrast echocardiography. K E Y W O R D S hypoxia, left atrium, neonate, right superior vena cava How to cite this article: Gulati R, Smith T, Siow M, Batten L. Isolated right superior vena cava drainage into the left atrium: An uncommon cause of persistent hypoxia in a neonate.

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“…As far as we know just twenty cases were described [5], while the persistent left superior vena cava draining into the coronary sinus is relatively common. The concomitance of these two anomalies of systemic venous return is almost anecdotal, but while the persistent left superior vena cava is quite common and its morphogenesis is well known, for the right superior vena cava draining into the left atrium there are different theories about the embryogenesis.…”

Section: Discussionmentioning

confidence: 99%

Double superior vena cava: Right connected to left atrium and left to coronary sinus

Miraldi¹,

Carbone

Ascarelli

et al. 2009

International Journal of Cardiology

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Anomalous connection of the right superior caval vein to the morphologically left atrium

Cited by 12 publications

References 6 publications

Bilateral superior vena cava with right superior vena cava draining into left atrium

Bilateral superior vena cava with right superior vena cava draining into left atrium

Isolated right superior vena cava drainage into the left atrium: An uncommon cause of persistent hypoxia in a neonate

Double superior vena cava: Right connected to left atrium and left to coronary sinus

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