Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot—Four Cases of a Rare Association

Abstract

<jats:p> Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully. </jats:p>

Left atrial appendage occlusion using hydrogel coils in a child

“…LSVC draining into the LA (when the coronary sinus is absent or unroofed coronary sinus) commonly presents with desaturation 3,4 . It is usually treated surgically by intra‐ or extracardiac routing of the LSVC to the RA.…”

“…It is usually treated surgically by intra‐ or extracardiac routing of the LSVC to the RA. The alternative is ligating the LSVC above its drainage into the LA, usually preferred when the bridging vein is well‐developed 3,5 . In unique scenarios, transcatheter treatment can be an option 4,6,7 .…”

“…In unique scenarios, transcatheter treatment can be an option 4,6,7 . LSVC baffle leak presentation and management are not well described in the literature 3 . However, in patients with D transposition of the great arteries who underwent arterial switch, baffle leak is a well‐recognized cause of desaturation, which can be closed using occluder devices or covered stents 8,9 …”

Low Oxygen Saturation Following Total Correction in a Patient with Tetralogy of Fallot and Persistant Left Superior Caval Vein - How Did We Diagnose and Manage?

“…3 Ramman et al found UCS in a patient with operated TOF and PLSVC who developed post-operative hypoxia. 4 Persistent left superior vena cava is frequently diagnosed in centers concentrated on congenital heart diseases. However, coronary sinus pathologies can be missed out and found later either as a result of complications or through post-operative desaturation.…”

“…PLSVC cases accompanying TOF are known to exist, although rarely seen. [3][4][5] A 4-month-old girl was admitted to our institution with the diagnosis of TOF and PLSVC. Unroofed coronary sinus was detected during intraoperative echocardiography which was performed due to persisting low oxygen saturation despite total correction.…”