Left superior vena cava draining into the left atrium: Clinical entities, diagnosis and surgical treatment

Abstract

Left superior vena cava draining into the left atrium in the absence of coronary sinus is an anomaly that can appear in heterotaxy syndrome and unroofed coronary sinus syndrome. Regardless of the origin of these syndromes, biventricular repair can be done through rerouting by intracardiac procedures or through disconnection-reconnection of the left superior vena cava to the right atrium or right superior vena cava by extracardiac procedures. Different techniques can be used for this purpose, each of which has its own advantages and limitations. Therefore, appropriate selection is necessary to obtain the best results for each patient, and many factors, such as patient anatomy, age, associated cardiomyopathies, etc., have to be considered. In this review, we focus on heterotaxy and unroofed coronary sinus syndromes, associated cardiomyopathies, the state-of-the-art in their surgical treatment and our results in a sample of 10 patients. Our experience highlights the importance of accurate diagnosis and specific selection of surgical technique for the management of biventricular repair in patients with left superior vena cava draining into the left atrium in the absence of coronary sinus.

Case report of a left superior vena cava to left atrial connection treated with percutaneous covered stent placement

“…When associated with an unroofed CS, various techniques with intracardiac baffling and/or extracardiac re-routing of LSVC to the RA, right superior vena cava, or pulmonary artery have been reported, each with advantages and drawbacks. 4 With complex intra-atrial baffling, complications such as pulmonary vein stenosis, LSVC tunnel obstruction and supra-mitral stenosis are not uncommon. 4–6 In the case of direct LSVC to LA drainage, percutaneous closure or surgical ligation (without reconnection) of the distal segment of the LSVC is an alternative.…”

“… 4 With complex intra-atrial baffling, complications such as pulmonary vein stenosis, LSVC tunnel obstruction and supra-mitral stenosis are not uncommon. 4–6 In the case of direct LSVC to LA drainage, percutaneous closure or surgical ligation (without reconnection) of the distal segment of the LSVC is an alternative. 3 , 7–10 The caveat with this approach is that venous hypertension can develop with a risk of cerebral injury when venous collateralization is inadequate, particularly in patients without a bridging innominate vein.…”

“…The unique anatomic variant in our patient was amenable to re-routing and preservation of LSVC return to the RA by transcatheter placement of a covered stent; without sacrificing the systemic venous pathway by device occlusion of the LSVC in previous reports. 4 , 7 , 8 , 10 , 12 This strategy also avoids the risks of a re-do sternotomy, cardiopulmonary bypass, and attendant surgical morbidities. This concept is similar to the recently reported covered stent correction of sinus venosus atrial septal defect with encouraging early outcomes.…”

Persistent Left Superior Vena Cava—When a Left-Sided Central Line Does Not Make the Turn: A Case Report

“…3 There are associated vascular anomalies that are less benign, including an absent left brachiocephalic or innominate vein in 50% to 65% of cases, drainage into the azygos system or drainage into the left atrium causing a systemic emboli, abscesses, syncope, arrhythmias, or a right to left shunt. [3][4][5][6][7] "Isolated PLSVC" is the case where there is no right superior vena cava, and this anomaly is associated with a 50% incidence of atrial septal defect, endocardial cushion defect, tetralogy of Fallot, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, or cor triatriatum. [3][4][5][6][7][8] In our case, we had no index of suspicion that the patient had a PLSVC until the chest X-ray was taken.…”

“…[3][4][5][6][7] "Isolated PLSVC" is the case where there is no right superior vena cava, and this anomaly is associated with a 50% incidence of atrial septal defect, endocardial cushion defect, tetralogy of Fallot, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, or cor triatriatum. [3][4][5][6][7][8] In our case, we had no index of suspicion that the patient had a PLSVC until the chest X-ray was taken. We were certain that we were venous regardless of the tip projecting over the aorta given the normal if mildly dampened CVP waveform, manometry use, and ease of placement.…”