Total Anomalous Systemic Venous Drainage

Viart, Pierre; Clerc, Jean-Louis Le; Primo, G; Polis, O

doi:10.1001/archpedi.1977.02120150077016

Cited by 9 publications

(6 citation statements)

References 17 publications

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“…Other reports suggest that these abnormal veins connect more commonly to the right-sided atrium. 1–5,8,9 Three cases described by Awasthy and colleagues 8 had left isomerism syndrome characteristics, similar to our case. Thus it is justifiable to suggest total anomalous systemic drainage as one of the indicators of left isomerism syndrome.…”

Section: Discussionsupporting

confidence: 88%

Left isomerism syndrome with total anomalous systemic connection

Vo¹,

Cao²,

Khoi³

et al. 2016

Asian Cardiovasc Thorac Ann

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We present a case of left isomerism with total anomalous systemic venous connection where the inferior vena cava was absent and all other systemic veins connected abnormally to the left atrium. The right atrium was hypoplastic with an intact atrial septum. Blood flow to the lungs was through a large ventricular septal defect. The diagnosis was made with echocardiography, angiography, and computed tomography. Complete repair was performed successfully, and the 7-year-old patient had an uneventful recovery.

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Section: Discussionsupporting

confidence: 88%

Left isomerism syndrome with total anomalous systemic connection

Vo¹,

Cao²,

Khoi³

et al. 2016

Asian Cardiovasc Thorac Ann

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“…Although one exceptional case was reported where all systemic venous blood returned to the coronary sinus,[1] the openings of total anomalous systemic veins are much more commonly connected to the left atrium. [2345678910]…”

Section: Discussionmentioning

confidence: 99%

Total anomalous systemic venous drainage to the left atrium: An entity reviewed and investigated

et al. 2014

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We present three unusual cases of total anomalous systemic venous drainage (TASVD) where all the systemic veins were abnormally connected to the atrium that drained into the left ventricle (LV). All three patients had features consistent with left atrial isomerism. Based on the available evidence and review of literature we propose that TASVD be included as a part of the syndrome of left atrial isomerism. A classification for TASVD is also proposed with surgical implications.

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“…Our review of the literature, together with our current experience, thus shows that TASVC, anatomically, can exist only in the setting of lateralized atrial arrangement [ Table 2 ]. [ 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 ] Hemodynamically, however, it can very well exist in the setting of isomeric atrial appendages. In our opinion, in the later setting, it is best to provide details of connections of systemic veins rather than clubbing under an ambiguous label of TASVC.…”

Section: Discussionmentioning

confidence: 99%

Clarifying the anatomy and physiology of totally anomalous systemic venous connection

et al. 2017

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The description of totally anomalous systemic venous connection is limited to case reports. In this review, we seek to clarify anatomic, physiologic, and hemodynamic aspects of this extremely rare anomaly. We also present findings of two patients in whom connection of all the systemic veins was anomalous. In the first patient, with usual atrial arrangement, all systemic veins, including the coronary sinus, were connected anomalously to the morphologically left atrium. Limited left-to-right shunt across an atrial septal defect provided the only source of blood flow to the lungs. The diagnosis was established by saline contrast echocardiography and cardiac catheterization. Extreme hypoplasia of the right ventricle precluded corrective surgery, so we performed a bidirectional Glenn operation, along with atrial septectomy. The second patient had isomerism of the left atrial appendages, which creates problems in the definition in anatomic terms since the connection of the systemic veins can never be normal anatomically when both atriums possess a morphologically left appendage. Our patient, nonetheless, had all the systemic and pulmonary veins, connected to the left-sided atrial chamber which then connected to the left ventricle, thus producing hemodynamics of totally anomalous systemic venous connection. We propose an algorithm for evaluation of this hemodynamic combination and discuss management options. We also intend to clarify the potential differences between connection and drainage, with particular attention to the arrangement of atrial appendages. Even though the hemodynamics may be comparable, in anatomic terms, both systemic and pulmonary venoatrial connection will always be anomalous with isomeric atrial appendages.

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Total Anomalous Systemic Venous Drainage

Cited by 9 publications

References 17 publications

Left isomerism syndrome with total anomalous systemic connection

Left isomerism syndrome with total anomalous systemic connection

Total anomalous systemic venous drainage to the left atrium: An entity reviewed and investigated

Clarifying the anatomy and physiology of totally anomalous systemic venous connection

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