Left Isomerism of the Atrial Appendages With Sinus Venosus Defect and Anomalous Systemic Venous Drainage

Talwar, Sachin; Ramakrishnan, Pradeep; Anderson, Robert H.; Choudhary, Sandeep; Makhija, Neeti; Kumar, Sanjeev; Airan, Balram

doi:10.1177/2150135115610283

Cited by 2 publications

(6 citation statements)

References 12 publications

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“…In a similar fashion, the systemic veins can never be connected in anatomically normal fashion in the setting of isomeric left atrial appendages. [ 18 ] It follows, therefore, that it is illogical, from the anatomic stance, to diagnose TASVC in patients with so-called “heterotaxy,” as was the case in our second patient. The hemodynamics, nonetheless, was identical to that in our first patient.…”

Section: Discussionmentioning

confidence: 60%

“…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%

“…In the majority, the left-to-right shunt is at atrial level although it can be across a VSD[ 6 9 13 21 22 27 ] or a persistently patent arterial duct. [ 6 7 18 ] It is the amount of left-to-right shunt that determines the arterial saturation, and hence, the likelihood of survival. In addition, the amount of left-to-right shunt determines the intrauterine growth of RV.…”

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%

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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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Section: Discussionmentioning

confidence: 60%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Clarifying the anatomy and physiology of totally anomalous systemic venous connection

et al. 2017

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New Insights into Sinus Venosus Defects from Cross-Sectional Imaging

Yoo¹,

Lee²,

Mora³

et al. 2022

Congenital Heart Disease

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Sinus venosus defects include two varieties, superior and inferior sinus venosus defects. The superior sinus venosus defect is characterized by abnormal communication between two closely related venoatrial structures: 1) the normally positioned superior vena cava-right atrium complex and 2) the right pulmonary vein-left atrium complex that is displaced leftward, forward and upward. Inferior sinus venosus defects primarily involve the inferior vena cava-right atrial junction while the right pulmonary vein-left atrial junction can also be affected. Because of the rarity and wide variation of the defects, the morphological characterization of sinus venosus defects is inconsistent among investigators and often inaccurate. Modern imaging technologies with high spatial and temporal resolutions have allowed accurate and detailed assessment of the pathological anatomy in larger numbers of cases. In this pictorial essay, we revisit the established and controversial features of the sinus venous defects using twodimensional (2D) and three-dimensional (3D) images obtained by magnetic resonance (MR) or computed tomography (CT) with brief discussion on imaging and treatment strategies.

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Left Isomerism of the Atrial Appendages With Sinus Venosus Defect and Anomalous Systemic Venous Drainage

Cited by 2 publications

References 12 publications

Clarifying the anatomy and physiology of totally anomalous systemic venous connection

Clarifying the anatomy and physiology of totally anomalous systemic venous connection

New Insights into Sinus Venosus Defects from Cross-Sectional Imaging

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