Absent right superior vena cava with left superior vena cava draining into unroofed coronary sinus with common atrium in a child with an atrioventricular septal defect

The association of absent right superior vena cava and persistent left superior vena cava draining into unroofed coronary sinus with common atrium and the atrioventricular septal defect is an extremely rare form of the congenital cardiac disorder with only one case reported so far, hence, can be missed preoperatively if not carefully looked for. Failure to detect absent right superior vena cava beforehand may otherwise pose difficulties in carrying out invasive surgical or medical interventions.     

Surgical treatment in an infant with atrial septal defect and persistent left superior vena cava draining into the coronary sinus; report of a case

A 8-month-old boy with ostium secundum atrial septal defect and persistent left superior vena cava draining into the coronary sinus underwent surgical treatment because he had required long-term ventilator support due to congestive heart failure and respiratory distress. The left superior vena cava and dilated coronary sinus produce a ridge in the left atrium, causing a subdivided left atrium. In this case, the early symptom could be explained by an obligatory left to right shunt at the level of the left atrium resulting from a ridge produced by the left superior vena cava and the colonary sinus, as a subdivided left atrium.     

Persistent left superior vena cava draining to left atrium with normal coronary sinus

In embryology, a persistent connection of the left superior vena cava to the left atrium rarely coexists with a coronary sinus. We herein report an unusual case of persistent left superior vena cava terminating in a left atrium with normal coronary sinus, which was revealed at the time of permanent pacemaker implantation after a second operation for recurrent left atrioventricular valve regurgitation. Because this anomaly had gone undiagnosed at the first operation, we were unable to diagnose it prior to the second operation, because the preoperative coronary angiogram clearly demonstrated a coronary sinus that was not dilated. We would have repaired the anomaly using a patch or other procedure had it been diagnosed before the second operation in order to prevent cyanosis or brain complications secondary to right-to-left shunting. One should always be on guard for this rare condition.     

Persistent left superior vena cava draining to left atrium with normal coronary sinus

In embryology, a persistent connection of the left superior vena cava to the left atrium rarely coexists with a coronary sinus. We herein report an unusual case of persistent left superior vena cava terminating in a left atrium with normal coronary sinus, which was revealed at the time of permanent pacemaker implantation after a second operation for recurrent left atrioventricular valve regurgitation. Because this anomaly had gone undiagnosed at the first operation, we were unable to diagnose it prior to the second operation, because the preoperative coronary angiogram clearly demonstrated a coronary sinus that was not dilated. We would have repaired the anomaly using a patch or other procedure had it been diagnosed before the second operation in order to prevent cyanosis or brain complications secondary to right-to-left shunting. One should always be on guard for this rare condition.     

Right superior vena cava draining into the left atrium in a patient with sinus venosus defect

Right superior vena cava draining into the left atrium is an extremely rare anomaly of systemic venous return. It can be isolated or it can be associated with other congenital heart defects, thus clinical presentation may vary. A case of a 9-year-old asymptomatic patient with sinus venous defect and bilateral superior vena cava with the right-sided draining into the left atrium is described.     

Two cases of atrial septal defect with absence of right superior vena cava and persistent left superior vena cava]

Two women (16-year-old, 54-year-old) of atrial septal defect with absence of right superior vena cava and persistent left superior vena cava were successfully operated on. Electrocardiographic findings show coronary sinus rhythm and atrial fibrillation. When we closed atrial septal defect cannulated to persistent left superior vena cava via the coronary sinus directly, sick sinus syndrome was not appeared postoperatively.     

Atretic right superior vena cava with left superior vena cava draining directly into left atrium with absent coronary sinus in an adult with partial atrioventricular canal defect and complete heart block

The association of atretic right superior vena cava with persistent left superior vena cava draining directly into left atrium with absent coronary sinus in atrioventricular canal defect is virtually unknown in adults with no case reported so far. Though atretic right superior vena cava with persistent left superior vena cava is an extremely rare venous anomaly seen in congenital heart disease, it has important clinical implications in cardiac surgery and interventional cardiology. Atrial arrhythmias and right bundle branch block are common with advancing age in partial atrioventricualr canal defect but complete heart block has scarcely been reported in the medical literature.     

Surgical repair of common atrium associated with persistent left superior vena cava draining into the left atrium

A case of 54-year-old female of common atrium associated with persistent left superior vena cava (PLSVC) draining into the left atrium and absence of the coronary sinus was experienced. The corrective surgery was done by using a horseshoe-shaped autologous pericardial patch to make a new atrial septum to allow the PLSVC draining into the right atrium. Since the common atrium is frequently associated with maldrainage of the PLSVC, the surgical treatment should be determined according to existence of the PLSVC and its opening site.