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A 52-year-old man was diagnosed with dextrocardia at the age of 1 year and was asymptomatic until 1 year before admission. He was transferred to our hospital for management of atrial fibrillation. A transthoracic echocardiogram showed dextrocardia with atrial septal defect;moderate tricuspid valve regurgitation; and a large, persistent left superior vena cava. A cardiac catheterization study revealed that pulmonary flow/systemic flow (Qp/Qs) was 3.6 and that pulmonary vascular resistance was 2.5 Wood U·m². Intracardiac repair with tricuspid annuloplasty and a maze procedure was scheduled. When establishing cardiopulmonary bypass, venous drainage was initially obtained from the inferior vena cava and the left superior vena cava, and the small superior vena cava was then directly cannulated after opening the right atrium. The patient's postoperative course was uneventful, and serial electrocardiograms have demonstrated maintenance of normal sinus rhythm for 3.5 years after the operation.

A 78-year-old man was referred to our hospital with syncope and palpitation. A Holter electrocardiography (ECG) revealed sick sinus syndrome (SSS), and an enhanced chest computed tomography (CT) scan showed persistent left superior vena cava (PLSVC) and absent right superior vena cava. Myocardial leads and a pacemaker implantation were peformed through left anterior thoracotomy approach. There were only 10 reports of pacemaker implantation in a patient with SSS complicated with PLSVC and absent right superior vena cava in Japan. Open thoracotomy approach was thought to be usuful for these patients.

A 3-year-old boy suffered from the poor body weight gain under the diagnosis of partial anomalous pulmonary venous connection (PAPVC) to the high portion of the superior vena cava (SVC) associated with ventricular septal defect (VSD) and intact atrial septum (IAS). Preoperative cardiac catheterization revealed the pulmonary to systemic flow ratio 2.6 and mean pulmonary arterial pressure of 21 mmHg. He successfully underwent surgical repair with Williams method using right atrial (RA) flap out and VSD patch closure. Postoperative course was uneventful and echocardiography demonstrated no obstruction at the both routes of neo-SVC and neo-right upper pulmonary venous return.

We report the cases of a left partial anomalous pulmonary venous connection (PAPVC) and a persistent left superior vena cava (PLSVC), combined with primary lung cancer. Our case of PAPVC, the anomalous pulmonary vein originated from the hilum of the left upper lobe flowed into the left brachiocephalic vein. A left lower lobectomy was performed uneventfully without correcting the anomalous vein. And a case of PLSVC, the left superior vena cava flowed into the right superior vena cava, running under the aortic arch. A left upper lobectomy and mediastinal lymph node dissection was performed in safety. Although PLSVC was detected by chest computed tomography (CT) before operation, PAPVC was noticed intraoperatively in our case. We should keep in mind the possibility of variations of pulmonary vessel distribution, especially PAPVC located in a different lobe for resection, when undertaking lung resection.

A 10-year-old boy with partial anomalous pulmonary venous connection to the high superior vena cava (SVC) underwent surgical repair by Williams method. The SVC was divided above the orifice of the anomalous pulmonary vein. The proximal end of the SVC was closed and the distal end of the SVC was anastomosed to the right atrial appendage (RAA). The anomalous pulmonary vein was rerouted to the left atrium via the SVC and the surgically enlarged central type atrial septal defect (ASD). The postoperative course was uneventful except transient sinus bradycardia and catheter study showed no stenosis of the SVC and the RAA.

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.

A patient was a 65-year-old female who had a complaint of palpitation was diagnosed aortic valve stenosis due to congenital bicuspid aortic valve with pseudotendon by the echocardiographic examination. We suspected left single coronary artery by the aortography and the coronary artery angiography. Aortic valve replacement and resection of pseudotendon was performed with Carbomedics supra-annular aortic valve (21 A). During surgery, persistent left superior vena cava was detected. High-posterior take-off right coronary artery was casually detected at aortic closure. Ventricular fibrillation due to insufficient supply of cardioplegic solution at right coronary area frequently occurred after cardio-pulmonary bypass and percutaneous cardiopulmonary support was required. The patient was discharged 32 days after the operation. Preoperative and intraoperative evaluation was important in the case of aortic valvular disease.

A two and a half year-old boy with partial anomalous pulmonary venous connection (PAPVC) to the high superior vena cava (SVC) underwent a surgical repair by Vargas' procedure. The SVC was divided above the orifice of the anomalous pulmonary vein and the cephalad end of the SVC was anastomosed to the right atrial (RA) appendage. The anomalous pulmonary venous flow was diverted to the left atrium through the enlarged atrial septal defect using the RA wall only. Post-operative angiography showed no stenosis of pulmonary venous pathway, however, the SVC was compressed by the ascending aorta. Vargas' procedure could be useful for repair of PAPVC to the high SVC, however, care must be taken not to compress the rerouted SVC by the ascending aorta.

The patient was a 22-year-old woman and suffered from atrial septal defect (ASD) complicated by persistent left superior vena cava (PLSVT). The patient underwent a patch closure using calf pericardium. Despite favorable progress immediately after the operation, fever, enlarged cervical lymph nodes, and leukocytopenia appeared beginning day 8 after the operation. Based on the results of cytodiagnosis of myeloaspiration specimen, the diagnosis of hemophagocytic syndrome (HPS) was made. Since an elevation of 2-5 oligo A synthetase (2-5 AS) levels was observed, the patient was diagnosed to suffer from virus-associated hemophagocytic syndrome (VAHS), and steroid was administered. The patient exhibited a good response to the treatment. The fever declined 3 days after the start of administration. Leukocyte counts were also increased gradually. The patient condition was improved and she was discharged from the hospital on day 28. At present, seven months after the operation, there is no evidence of recurrence and the patient's condition is still monitored.

Two cases of sinus venosus atrial septal defect with partial anomalous of right upper pulmonary venous drainage into superior vena cava treated by the Vargas' procedure were reported. These patients were 5 and 6 years old. Post operative courses were uneventful, and results of postoperative hemodynamic and clinical evaluation were encouraging. The Vargas' procedure is useful method for a reconstraction of the anomaly in childhood because of use of the autologus tissues. Though long-term fate of this procedure is still uncertain. Then, we consider that a long-term follow up is necessary.

A 7-year-old boy with partial anomalous pulmonary venous drainage to the high superior vena cava (SVC) underwent surgical repair by Williams procedure. Right upper pulmonary vein was drained to the left atrium via the SVC and the surgically enlarged sinus venosus type ASD. SVC was ligated and divided. SVC was then anastomosed to RA appendage pedicle directly. Autologous pericardium was patched anteriorly to create a new SVC pathway. The postoperative course was uneventful without arrhythmia and cardiac catheterization 1 month postoperation revealed no stenosis of the SVC and right upper pulmonary venous return.

Twelve years ago, we reported a method of extrapericardial approach to the L-SVC. Since then we have used this approach and performed simple occlusion of L-SVC during cardiopulmonary bypass without any cerebral complications. We have been expecting for a long time and have believed that this method will be very valuable especially in repeat open heart surgery. A case of 49-year-old man successfully underwent a redo operation for mitral and tricuspid valve replacement using extrapericardial occlusion of the L-SVC and retrograde cardioplegia. Although there was a pitfall for retrograde cardioplegia in the persistent L-SVC and a possibility of steal of cardioplegic fluid through a branch of the accessory hemiazygos vein during retrograde cardioplegia for a case with persistent L-SVC, sufficient cardiac protection was obtained in the case presented.

A 56-year-old woman was admitted to our department because of congestive heart failure. Chest X-ray showed dextrocardia with situs inversus. The echocardiography revealed severe mitral regurgitation due to prolapse of posterior leaflet and secondary Venography demonstrated the bilateral superior vena cava, huge coronary sinus and hemiazygos continuation with hypoplastic inferior vena cava. Mitral valve regurgitation was repaired by Carpentier's and Burr's technique, and tricuspid valve regurgitation was repaired by Kay's technique. Her postoperative course was uneventful. Valve surgery for acquired valvular lesion in dextrocardia with situs inversus is rare. Although valve repair is easily performed even in mirror-image heart as well as in normal, levo-position heart, preoperative evaluation of systemic venous return is important in the case of dextrocardia with systemic venous anomalies.

Successful repair was performed for a 7-year-old male with a diagnosis of partial anomalous pulmonary venous connection (PAPVC) to superior vena cava (SVC) and superior sinus venosus atrial septal defect (ASD). The SVC was divided above the orifice of the anomalous pulmonary vein and the cephalad end of the SVC was anastomosed directly to the right atrial appendage. A patch was used to divert pulmonary venous flow from the orifice of the SVC through superior sinus venosus ASD into the left atrium. Postoperative course was uneventful with normal sinus rhythm. There was no evidence of vena caval or pulmonary venous obstruction. At 3-month after surgery, sinus node function was confirmed to be normal by electrophysiological study. This is useful alternative method for repair of PAPVC to high or middle SVC.

A 44-year-old female with left to right shunt and persistent left superior vena cava (PLSVC) suspected of unroofed coronary sinus defect with patent right SVC and innominate vein was treated surgically. PLSVC was ligated and coronary sinus ostium was closed with a EPTFE patch. Roof defect of coronary sinus was left open and allowed the coronary venous blood to drain into the left atrium. Patient recovered well and post-operative cardiac catheterization revealed no significant right to left shunt nor desaturation of arterial blood. In the correction of unroofed coronary sinus defect and PLSVC and no other intra-cardiac defect, ligation of PLSVC and closure of coronary sinus ostium was simple and effective, although it was not anatomically corrected.

We reported a 8-year-old girl having discrete type of subpulmonary stenosis. This patient was associated with atrial septal defect (ASD), valvular pulmonary stenosis, and persistent left superior vena cava (PLSVC). The incidence of this type of subpulmonary stenosis is less frequent than that of subaortic lesion. ASD was closed by the autologous pericardium and a transannular patch was used for enlargement of the right ventricular outflow tract followed by commissurotomy of the pulmonary valve and resection of subpulmonary discrete lesion. She was uneventful and postoperative pulsed doppler echocardiogram showed good results.

A 2-year-old girl of heterotaxia associated with complex anomalies was reported. Major cardiac anomaly was tetralogy of Fallot (TOF), which associated with the interruption of the inferior vena cava, persistent left superior vena cava (PLSVC), common atrium and partial anomalous pulmonary venous drainage (PAPVD) to the coronary sinus. The two dimensional echocardiogram displayed the characteristic views of TOF and the additional interesting abnormalities near the mitral ring. The markedly enlarged and protruded coronary sinus by the association of PLSVC and PAPVD overlay along the posterior mitral leaflet and the abnormal muscular septum surrounded immediately above the anterior mitral leaflet. These structures created a sort of supramitral ring structure, although this was not important hemodynamically because of the association of single atrium and TOF preoperatively. At the operation the coronary sinus roof was resected along the posterior mitral leaflet as well as the abnormal muscular flange. The atrial septum was created with a porcine pericardium and the left superior vena cava was ligated. The postoperative course was not eventful.

A 10-year-old boy with tricuspid atresia (type I-b), bilateral superior venae cavae and left pulmonary arterial branch stenosis was treated successfully by a two-staged Fontan procedure. At 7 years of age, left Glenn shunt was performed after creation of a communicating vessel between the right and left superior vena cava, using a GORE-TEX graft of 10 mm of diameter. Three years later, after having confirmed graft patency, Fontan procedure was achieved without any problems. We think the innominate vein creation by an artificial material was a key design for the following successful two-staged Fontan procedure in this case.

Successful surgical repair of a 1-year-old girl of complete endocardial cushion defect associated with single atrium, persistent left superior vena cava draining into the left atrium, hemiazygos connection and double orifice mitral valve was reported. We repaired the endocardial cushion defect by using an endocardial cushion prosthesis and a new atrial septum was created to allow the left superior vena cava draining into the right atrium. We made no treatment for the accessory mitral valve orifice because of no regurgitation.

Complete type of thoracic ectopia cordis was diagnosed with prenatal ultrasound study. Just after birth of female baby with 2,750 g body weight, a silastic mesh was selected for covering the heart because of no space for putting the heart into the mediastinum. Protrusion of the liver towards the mediastinum was one of main causes of lifting the heart outside the chest wall. The baby died 7 days after her birth due to heart failure. Soft silastic mesh was not good enough to sustain the ectopic heart constantly and caused paradoxical respiratory movement of mediastinal side of the lung. It is concluded that hard shell coverage of the extremely ectopic heart is necessary to sustain it constantly and to prevent paradoxical respiratory movement of mediastinal side of the lung.

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.

A 36-year-old women with pure unroofed coronary sinus syndrome was reported. The anomalies of the cardiovascular system were absence of the coronary sinus, coronary sinus ASD and persistent left superior vena cava (PLSVC) draining into the left atrium. In this case, the PLSVC was ligated in the pericardium and the atrial septal defect was closed with a Dacron patch. There was no cerebral complication due to the increased intracranial pressure after ligation of the PLSVC. Postoperative coronary arteriography revealed absence of the coronary sinus in the venous phase.

A successfully treated case of one-year-old girl of common atrium associated with bilateral superior vena cava and an interrupted inferior vena cava with hemiazygos continuation is reported. In addition to these anomalies of systemic venous connection, both atrial appendages show morphological characteristics of left type, and the final diagnosis of left atrial isomerism was made. In the corrective surgery, atrial septation was reconstructed using trimmed autogenous pericardial patch to allow the left superior vena cava to drain into the right side of the common atrium. The patient had been pointed out bradycardia (50-100/min) since her birth. Postoperative electrocardiogram also showed bradycardia (78-100/min). Especially in sleeping, her heart rate was 50-60/min, resulting in A-V junctional escaped rhythm and A-V dissociation. This fact suggests that her sinus node is absent or hypoplastic, one of the characteristics of left atrial isomerism. Since left atrial isomerism is reported to cause a decrease of atrial rhythm with aging, careful postoperative observation on specific arrhythmia is necessary.

A 34-year-old female presented with exertional dyspnea. Investigation by echocardiography and cardiac catheterization showed completely unroofed coronary sinus with persistent left superior vena cava (PLSVC) (coronary sinus atrial septal defect, absence of the coronary sinus, and PLSVC-left atrium connection) combined with tricuspid valve regurgitation. Angiocardiography made by injection into the PLSVC demonstrated that the PLSVC was connected to the hemiazygos vein before it drained into the left atrium and the left innominate vein was absent. Although jugular vein pressure rose up to 18 mmHg when the PLSVC was temporarily occluded, it remained unchanged. Therefore, simple ligation of the PLSVC was selected for therapy. Patch closure of the atrial septal defect, tricuspid valve repair, and ligation of the PLSVC was performed successfully.

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.