Total anomalous systemic with partial anomalous pulmonary venous connections

Vallath, Gopakumar; Gajjar, Trushar; Desai, Nisarg

doi:10.1177/0218492312463567

Cited by 1 publication

(6 citation statements)

References 7 publications

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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%

“…Patients with large ASD, on the other hand, can have RV sufficiently large to allow corrective surgery. [ 8 9 10 11 12 14 15 17 19 ] A coexisting left-to-right shunt at the level of VSD[ 6 ] and or patent arterial duct,[ 7 ] however, can also reduce the interatrial shunt, and result in hypoplasia of RV. Irrespective of the amount of left-to-right shunt, systemic arterial saturation is expected to be less than normal.…”

Section: Discussionmentioning

confidence: 99%

“…ECG is often abnormal showing poor right ventricular forces and sinus node dysfunction. In cases providing the details of the ECG, the finding of an ectopic atrial rhythm[ 4 5 6 14 15 18 ] junctional rhythm,[ 12 ] or right ventricular hypoplasia is common. [ 32 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 2 3 4 5 6 7 8 9 ] On the other hand, the presence of a large shunt allows good oxygenation, together with the growth of RV, thus permitting complete surgical repair. [ 9 10 11 12 13 14 15 16 17 18 19 ] The presence of cyanosis despite left-to-right shunt across an ASD, in the setting of a hypoplastic RV in hearts with concordant atrioventricular and ventriculoarterial connections, is almost diagnostic of totally anomalous systemic venous connection (TASVC). Unfortunately, even in the current era of modern echocardiography, despite many clues, the diagnosis is often delayed.…”

Section: Introductionmentioning

confidence: 99%

“…Unfortunately, even in the current era of modern echocardiography, despite many clues, the diagnosis is often delayed. [ 5 8 11 12 14 15 20 ] Not uncommonly, the diagnosis is missed completely,[ 10 ] and the arrangement is misinterpreted as functionally single ventricle. It is also not uncommon to find that anomalous connection of only one caval vein, generally the superior caval vein (SCV), is correctly identified mainly because saline contrast echocardiography is generally performed only from the upper limb.…”

Section: Introductionmentioning

confidence: 99%

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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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