Mitral valve plasty in an adult patient without a right superior vena cava

Persistent left superior vena cava without a right superior vena cava is an extremely rare condition. We report the case of a 65-year-old woman with this condition who underwent mitral valve plasty. During cardiac catheterization, the asymptomatic patient with mitral valve prolapse syndrome was found to have a persistent left superior vena cava without a right superior vena cava. During mitral valve plasty, cardiopulmonary bypass was established using bicaval drainage through the persistent left superior vena cava and the right atrium. A cannula was inserted into the persistent left superior vena cava to provide a large surgical field in the left atrium. We selected a technique that involved direct insertion of an L-shaped cannula into the persistent left superior vena cava and obtained a clear view of the surgical field. Proper assessment of the right superior vena cava is necessary when a persistent left superior vena cava is suspected.     

Radical repair of cor triatriatum with partially unroofed coronary sinus and persistent left superior vena cava 35 years after operation for tetralogy of Fallot

We report a rare case of simultaneous repair for Cor triatriatum (CT), partially unroofed coronary sinus (CS) with persistent left superior vena cava (PLSVC), moderately severe tricuspid regurgitation, and atrial flutter 35 years after radical operation for tetralogy of Fallot (TOF). A 40-year-old woman underwent patch closure of atrial septal defect as to drain blood from left SVC to right atrium (RA), excision of diaphragm in left atrium (LA), tricuspid annuloplasty, and cryoablation. Postoperative course was uneventful with successful anatomical correction.     

Surgical management of persistent left superior vena cava associated with an absent right superior vena cava

A case of persistent left with absent right superior vena cava accompanied with atrial septal defect in a six-year-old girl is presented. This rare venous malformation was diagnosed incidentally during surgery when the pericardium was incised. An L-shape cannula was directly inserted into the left superior vena cava for cardiopulmonary bypass. The atrial septal defect was closed with a fresh autologous pericardial patch. The surgical management of this rare anomaly is the subject of this review.     

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.     

Lung cancer which accompanies anomalous venous connections, pulmonary and systemic-partial anomalous pulmonary venous connection and persistent left superior vena cava: report of 2 cases

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.     

Rupture of the inferior vena cava during open-heart surgery. Aetiology and surgical management

Bleeding during open-heart surgery is a not unusual complication. Rupture of the inferior vena cava during dissection of the heart from adhesions from a previous operation for mitral disease or congenital palliative surgery is a serious and sometimes fatal complication. Its treatment is a challenge even to the most experienced heart surgeon. In two of our three patients, vena cava rupture occurred during open-heart surgery for correction of mitral and aortic valvular disease. In one patient, congenital infundibular stricture of the right ventricle, associated with tricuspid valve insufficiency, was present. In this report, some relevant data were presented from each case and the aetiology of the rupture is discussed.     

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.     

A Gore-Tex 'new-innominate' vein: a surgical option for complicated bilateral cavopulmonary shunts.

A bilateral bidirectional cavopulmonary shunt was performed in a cyanotic 14-month-old girl who had tricuspid and pulmonary valve atresia, with right pulmonary artery (RPA) hypoplasia (3 mm), bilateral superior vena cavae and a ductus arteriosus-dependent pulmonary blood flow. Because of 62% postoperative arterial oxygen saturation and a right superior vena cava (RSVC) pressure of 30 mmHg, a 5 mm Gore-Tex tube was interposed to connect the two superior venae cavae. The creation of a 'new-innominate' vein allowed decompression of the right superior vena cava and an increase in arterial oxygen saturation to 86%.     

Bilateral absence of the superior vena cava

Congenital anomalies of the superior vena cava are rare and often coexist with other cardiac abnormalities. In this report, we present a case of absent bilateral superior vena cava with no associated cardiac anomaly. Blood from the arms, head, and upper torso returned to the right atrium through the azygos vein and the inferior vena cava, mimicking chronic superior vena cava obstruction.     

Right atrial surgery with unsnared inferior vena cava.

Right atrial procedures require snaring the venous cannulas to prevent air entrapment in the venous line. In particular situations with complex congenital morphology and/or presence of severe pericardial adhesions the right atrial opening without the inferior vena cava cannula in the surgical field and without dissecting and snaring the inferior vena cava itself, might substantially facilitate the surgical technique, provided an adequate venous drainage is assured to avoid flow reduction or circulatory arrest. In several patients with congenital or acquired heart disease with potentially complicated venous drainage, like extracardiac Fontan procedure and tricuspid valve replacement, cardiopulmonary bypass was conducted either on normothermia (congenital lesions) or with mild hypothermia (acquired disease), with 3 l/min per m(2) flow index and venous drainage through femoral vein cannulation. The right atrium was opened without snaring the inferior vena cava, never provoking reduction of the venous drainage nor air locks in the venous line. This approach substantially enhanced the surgical exposure and therefore facilitated the operative technique without any negative consequence to the patients. Right atrial surgery on cardiopulmonary bypass without direct cannulation and snaring of both superior and inferior vena cava is feasible without flow reduction for surgeons taking care of both congenital and acquired cardiac lesions.     

Mitral valve replacement in dextrocardia and situs inversus

Cardiac surgery for acquired valvular diseases in patients with dextrocardia is extremely rare. We report a surgical case of mitral valve replacement and tricuspid annuloplasty in a patient with dextrocardia and situs inversus. A 74-year-old man with dextrocardia and situs inversus, who had undergone patch closure of atrial septal defect 25 years before, was referred for surgical treatment of severe mitral and tricuspid valve regurgitation. Preoperative computed tomography( CT) showed dextrocardia, situs inversus, interruption of the inferior vena cava with an azygos vein continuation, and drainage of the hepatic vein into the right atrium. Under redo-median sternotomoy, cardiopulmonary bypass was established by cannulating the ascending aorta, the superior vena cava, the right femoral and the hepatic veins. The surgeon operated from the left side of the operating table, and had an excellent exposure to the mitral and tricuspid valves during the operation. Mitral valve replacement and tricuspid annuloplasty were performed successfully. The postoperative course was uneventful.     

Accidental insertion of a percutaneous venovenous cannula into the persistent left superior vena cava of a patient undergoing liver transplantation

Purpose

Persistent left superior vena cava (PLSVC) is a rare congenital vascular abnormality found in 0.3% of the general population. We report herein a rare complication involving the accidental insertion of a large bore cannula into the PLSVC during liver transplantation (LT).

Clinical features

A 63-yr-old man with primary sclerosing cholangitis presented for LT. Given the existence of a tunnelled dialysis catheter in the right internal jugular vein (IJV) and a triple lumen catheter via the left IJV, insertion of an 18 French cannula for venovenous bypass (VVB) was performed via the left IJV using the existing triple lumen cannula as a conduit for a guidewire. Upon initiation of VVB, profound systemic hypotension occurred, and liver transplantation was completed without the further use of VVB. A chest x-ray confirmed a malposition of the VVB cannula with a large left hemothorax. A mini-sternotomy was performed for removal of the VVB cannula, which was found to be inserted in the PLSVC. Retrospectively, the presence of PLSVC was not anticipated due to a normal superior vena cava and a left innominate vein, as revealed by the course of a pre-existing left internal jugular vein triple lumen catheter on a preoperative chest x-ray, and due to a normal-sized coronary sinus on preoperative echocardiography.

Conclusion

Malpositioning of a venous cannula in a PLSVC should be anticipated as one of the potential complications of vascular access via the left internal jugular vein.     

Diagnosis of persistent left superior vena cava in a child using transesophageal echocardiography

An 11-year-old boy with X-linked alpha-thalassaemia/ mental retardation symptom underwent gastrostomy. After the surgical procedure, insertion of a central venous (CV) catheter via the left subclavian vein was performed under X-ray radioscopy. However, the catheter did not move toward the right side of the heart shadow but descended caudally through the left side of the heart shadow. Transesophageal echocardiography (TEE) showed thickening of the coronary sinus, suggesting the existence of a persistent left superior vena cava (PLSVC). Contrast echocardiography using infusion of agitated saline into the CV catheter was performed. Microbubbles flowing from the coronary sinus into the right atrium were observed, and a diagnosis of PLSVC was made. TEE is useful for diagnosis of PLSVC when passage of a CV catheter via the left subclavian vein or left internal jugular vein is incorrect in a pediatric patient.     

Single (left) superior vena cava draining into the left atrium. Surgical repair

Anomalous drainage of a single left superior vena cava into the left atrium is rare, and correction presents a technical problem. This report describes a patient with absent right superior vena cava, a persistent large left superior vena cava draining to the left atrium, and an intact interatrial septum.Surgical correction was accomplished by removing the entire septum and placing a cloth baffle to cover the orifices of the pulmonary veins and mitral valve, thus partitioning pulmonary venous blood into the left ventricle. This technique of septal excision and replacement is applicable to other complicated anomalies involving the systemic and pulmonary venous channels and their relationship to the two atria.The surgical management of related anomalies of the superior vena cava is also discussed.     

Bypass of superior vena cava. Fifteen years' experience with spiral vein graft for obstruction of superior vena cava caused by benign disease

Nine patients had operations for obstruction of the superior vena cava with superior vena caval syndrome caused by benign disease. Three patients had fibrosing mediastinitis, four had fibrosing mediastinitis with caseous necrosis, one had thrombosis of the superior vena cava around a pacemaker electrode, and one had spontaneous thrombosis of the superior vena cava. Patients ranged in age from 25 to 68 years. All bypass operations were performed with a composite spiral vein graft constructed from the patient's own saphenous vein, split longitudinally and wrapped around a stent in spiral fashion. The edges of the vein were sutured together to form a large conduit ranging in diameter from 9.5 to 15.0 mm. Six grafts were from the left innominate vein and three grafts were from the internal jugular vein. The grafts were placed into the right atrial appendage in all except one case, in which the graft was to the distal superior vena cava. Follow-up extends from 1 to 15 years. One patient required reoperation at 4 days for thrombosis at the innominate vein-graft anastomosis. Resection of the anastomosis and reconstruction of the graft rendered the patient symptom free. Two grafts closed during the first year after operation. One patient had advancing fibrosing mediastinitis, and a second bypass graft from the external jugular veins remain patent. Another patient had recurrence of spontaneous venous thrombosis. Thus seven of nine grafts remain patent for up to nearly 15 years and all but one patient is free of superior vena caval syndrome. These data show that bypass of the obstructed superior vena cava with a spiral vein graft relieves superior vena caval syndrome and demonstrate long-term patency of the graft.     

A case of completely unroofed coronary sinus with persistent left superior vena cava

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.     

Pacemaker implantation in a patient with sick sinus syndrome, complicated with persistent left superior vena cava and absent right superior vena cava; report of a case

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.     

右腋下小切口心内直视术中永存左上腔静脉的处理

目的总结右腋下小切口心内直视术中对永存左上腔静脉的处理方法及临床经验。方法自2009年9月～2014年1月,我们分别采用了3种方法对17例合并PLSVC异位连接冠状静脉窦的患者实施了右腋下小切口心内直视术,包括简单先心病16例,二尖瓣病变1例。其中心外套带直接临时阻断3例;右心吸引管置于冠状静脉窦口吸引显露5例;经冠状静脉窦置管引流9例。结果全组患者无住院死亡,无心律失常及其他严重并发症。随访3-6个月,均恢复良好。结论右腋下小切口心内直视术采用合理的方法处理永存左上腔静脉可取得良好效果,扩大应用范围。     

Coronary artery fistula into a persistent left superior vena cava: Report of a case

We herein report the rare case of a patient with coronary artery fistula (CAF) between the left circumflex coronary artery and persistent left superior vena cava (PLSVC) with a complete absence of the right superior vena cava (SVC).