Transvenous cardioverter-defibrillator implantation with a double coil lead via persistent left superior vena cava.

A dual-coil defibrillation lead was inserted in a 64-year-old male through a persistent left superior vena cava draining into the coronary sinus. The lead, connected to a cardioverter-defibrillator (ICD) implanted in the left pectoral area, was looped in the right atrium positioning the proximal and distal lead coils in the coronary sinus and right ventricular outflow track respectively and resulting in a low and stable defibrillation threshold. Because of its relative ease and effectiveness, this procedure may be recommended in patients with persistent left superior vena cava requiring an ICD implant.     

Unroofed coronary sinus and persistent left superior vena cava.

This report describes a case of unroofed coronary sinus and persistent left superior vena cava discovered during an echocardiographic investigation for dilated pulmonary artery. An unroofed coronary sinus is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava. The latter is a usual cause of a dilated coronary sinus. The detection of a dilated coronary sinus should therefore prompt the search for abnormal coronary sinus drainage and other cardiac abnormalities. The treatment of unroofed coronary sinus and persistent left superior vena cava is undertaken only after assessing the pre- and post-treatment haemodynamics of all co-existing abnormalities.     

Persistent left superior vena cava,absent right superior vena cava and coronary-bronchial fistula: The good,the bad and the ugly (case report and review of literature)

A 62-year-old Caucasian male presented with syncope during casual daily activity without preceding prodromes. During ECG Holter monitoring, we observed numerous asystolic pauses lasting >4 s due to sino-atrial blockade and sinus bradycardia. During pacemaker implantation, persistent left superior vena cava with agenesis of the right superior vena cava was diagnosed. Unproblematic placement of atrial lead was followed by challenging placement of the right ventricular lead. Anterior position with a sharp angulation to the right ventricular wall was achieved with excellent stimulation parameters. Transesophageal echocardiography confirmed the diagnosis of persistent left superior vena cava with agenesis of right superior vena cava. Moreover, selective coronary angiography showed connection between right coronary artery branch and bronchial vessel. To the best of our knowledge, we are the first to describe a combination of persistent left superior vena cava with absent right superior vena cava, coronary-bronchial fistula and conduction abnormality with the necessity of device implantation.     

Two cases of double superior vena cava

Two cases of double superior vena cava were found among 300 Japanese cadavers dissected at Kyoto University from 1980 to 1989. Case 1 was an 82-year-old male patient who died of cerebral infarction. Case 2 was a 39-year-old well-developed male patient who died of sepsis. They had no history of cardiovascular disorders. Common features in both cases are as follows: In addition to a right superior vena cava with normal appearance located in the normal position, on the left side, a normal-looking extra superior vena cava persisted and descended vertically in front of the aortic arch between the left pulmonary vein and the left auricle, traversed the coronary sulcus and finally drained into the right atrium. No differences in diameter were found between right and left venae cavae. No communicating vessels were present between them. Therefore, both cases, which had a persistent left superior vena cava draining into the coronary sinus, may be classified as McCotter's 1st type (1916) or Donadio's 3rd type (1925). Furthermore, in Case 2, the double azygos vein persisted symmetrically, suggesting the 4th type of Nandy and Blair (1965). In addition to venous anomalies, Case 2 had arterial anomalies such as the right subclavian artery arising as the last branch of the aortic arch similar to the G type of Adachi (1928), and the right vertebral artery arising from the right common carotid artery. The present cases are considered to be the 53rd and 54th cases of double superior vena cava found in Japanese cadavers, and the 18th and 19th cases of double superior vena cava without communicating vessels between them.     

Double superior vena cava with drainage of the right superior vena cava into the left auricle. Presentation as a cerebral abscess in an adult

A 44 year old man presented with a cerebral abscess, the location of which suggested a septicaemic origin. Although the patient was not cyanosed, a cardiological work-up was requested to exclude a right-to-left shunt. This showed a double abnormality of the systemic venous drainage: presence of an abnormal left superior vena cava draining into the coronary sinus and of a right superior vena cava draining into the left atrium. These two vena cava intercommunicated by anastomoses. Angiography in the right superior vena cava after occlusion by balloon catheter at its junction with the left atrium showed flow from the right to the left superior vena cava and to the azygos system. Simple ligature of the right superior vena cava was therefore performed to prevent recurrence of cerebral abscess. This case is rare and of interest because of the presence of two superior vena cavae, one on the right draining into the left atrium and the other on the left draining into the coronary sinus, with anastomoses between the two superior vena cavae. This double abnormality of systemic venous drainage explains the absence of cyanosis and therefore the relatively late detection of this malformation.     

Sinus node dysfunction associated with absence of right superior vena cava.

A 41-year-old man presented with dizziness associated with sinus bradycardia and sinus arrest. An attempt to implant a transvenous pacing lead was frustrated by absence of the right superior vena cava. The left superior vena cava persisted and drained via the coronary sinus into the right atrium. Absence of the right superior vena cava may present with symptomatic sinus node dysfunction and may require an epicardial demand pacing system.     

Sinus node dysfunction associated with absence of right superior vena cava.

A 41-year-old man presented with dizziness associated with sinus bradycardia and sinus arrest. An attempt to implant a transvenous pacing lead was frustrated by absence of the right superior vena cava. The left superior vena cava persisted and drained via the coronary sinus into the right atrium. Absence of the right superior vena cava may present with symptomatic sinus node dysfunction and may require an epicardial demand pacing system.     

Left superior vena cava connection to unroofed coronary sinus associated with positional cyanosis: successful transcatheter treatment using Gianturco-Grifka vascular occlusion device.

A persistent left superior vena cava connection to an unroofed coronary sinus is a rare cardiac anomaly that is associated with a variable degree of cyanosis. We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated to the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior vena cava and had an oxygen saturation 95%. When the patient's head was rotated to the right, the left internal jugular vein was widely patent and systemic oxygen saturation decreased to 87%. There was no right ventricular volume overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. Cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occlusion device. Cathet. Cardiovasc. Intervent. 48:369-373, 1999.     

体外循环中体循环血浆内皮素-1的变化

目的观察体外循环(CPB)手术中体循环血浆内皮素-1(ET-1)的变化,并探讨其变化的原因.方法16例心脏病患者,于主动脉阻断前即刻,主动脉开放后1、3、5分,分别取升主动脉根部血及冠状静脉窦血,于主动脉阻断前即刻,主动脉开放10分,停CPB时分别取上、下腔静脉血和升主动脉根部血,测定血浆ET-1含量.结果主动脉阻断前即刻,主动脉开放后1、3、5分,冠状静脉窦血和升主动脉根部血血浆ET-1水平无显著差异;停CPB时下腔静脉血浆ET-1水平显著高于主动脉阻断前即刻(P＜0.05),也显著高于同时点升主动脉根部血血浆ET-1水平(P＜0.05).主动脉阻断前即刻,主动脉开放后10分,两者无显著差异;CPB期间上腔静脉血和主动脉根部血血浆ET-1水平无显著升高,两者也无显著差异.结论CPB手术中体循环血浆中升高的ET-1主要来源于腹部脏器,而不是心肌组织.     

Anomalous Left Anterior Descending Coronary Artery from the Pulmonary Artery,Unroofed Coronary Sinus,Patent Foramen Ovale,and a Persistent Left‐sided SVC in a Single Patient: A Harmonious Quartet of Defects

Unroofing of the coronary sinus without complex structural heart defects is a rare congenital defect often seen in conjunction with a persistent left‐sided superior vena cava. Anomalous origin of the left anterior descending artery from the pulmonary artery with normal origin of the left circumflex coronary artery is an even rarer congenital cardiac defect. We report a case of a 54‐year‐old woman presenting with mild dyspnea on exertion who was found on invasive and noninvasive evaluations to have a unique combination of defects—unroofed coronary sinus, persistent left‐sided superior vena cava, patent foramen ovale, and anomalous origin of the left anterior descending artery from the pulmonary artery without evidence of previous coronary ischemia.     

Unusual venacaval anomalies in a patient with atrial septal defect

An unusual systemic venous drainage pattern was found in a 30-year-old man with ostium secundum atrial septal defect and pulmonary stenosis. He had the rare association of absent right superior vena cava, persistent left superior vena cava draining into the coronary sinus, and a left-sided inferior vena cava draining into a left superior vena cava through the hemiazygous vein.     

A case of absent right superior vena cava with persistent left superior vena cava: Cross-sectional echocardiographic diagnosis

Summary We report a case of absence of the right superior vena cava. Hitherto, the findings in this condition have only been obtained using invasive methods; this report is the first in which the diagnosis was made by means of echocardiography. The findings include a markedly enlarged coronary sinus draining into the right atrium and a specific finding of contrast echocardiography in which contrast material injected into the right antecubital vein can be seen in the enlarged coronary sinus. The contrast echo flows into the right atrium from the coronary sinusonly. The size of the coronary sinus was far beyond the range (91±38 mm²/m²) in patients with persistent left superior vena cava with the presence of the right superior vena cava.     

Absent right superior vena cava with left superior vena cava draining to an unroofed coronary sinus

We describe the case of a 1-month-old infant with a complete atrioventricular septal defect with right dominance, situs solitus, and drainage from the persistent left superior vena cava to the coronary sinus. Corrective surgery was carried out without previous cardiac catheterization. During the operation, the right superior vena cava was found to be absent. Cyanosis and head-and-neck edema were observed in the immediate postoperative period. Transthoracic echocardiography carried out after injection of a small volume of stirred saline into an epicranial vein demonstrated the presence of microbubbles in the left cardiac cavities. A second operation was performed to prevent drainage from the left superior vena cava to the left atrium (via the unroofed coronary sinus) and to insert a PTFE conduit between the innominate vein and the right atrial appendage. The outcome was excellent. In this report, the embryological, clinical, diagnostic and therapeutic characteristics of this entity are discussed.     

Coronary arteriovenous fistula

Congenital coronary arteriovenous fistulas represent abnormal communication between coronary arteries and any cardiac chambers, pulmonary artery, coronary sinus, superior vena cava or pulmonary vein. We reported an interesting case of very long and tortuous coronary arteriovenous fistulas originated from the left circumflex coronary artery, which drained into bronchial arteries in right lower lung field diagnosed by coronary angiography and multidetector computed tomography.     

Dual left anterior descending coronary artery: Angiographic description of important variants and surgical implications

Twenty-three cases of an anatomic variant of the left anterior descending artery (LAD) are described. This variant is termed “dual LAD” and consists of two branches which supply the usual distribution of the LAD. One branch (short LAD) terminates in the proximal aspect of the anterior interventricular sulcus (AIVS). A second, longer branch has a variable course outside the AIVS and returns to the AIVS distally. The long LAD arose from the LAD proper in 21 cases and from the RCA in two cases. The initial course of the long LAD was on the epicardial surface of the left ventricle (17 cases), right ventricle (three cases), or within the interventricular septum (three cases). Recognition of these variants is important for correct surgical identification of the short and long LADs.     

Absent Right Superior Vena Cava with Persistent Left Superior Vena Cava Which Drains to Unroofed Coronary Sinus in a Child with Atrioventricular Septal Defect and Cor Triatriatum Sinister: Preop Correct Diagnosis and Successful Surgery in a Single Session

We report a unique case of a 4‐year‐old boy with intermediate‐type atrioventricular septal defect, cor triatriatum sinister, persistent left superior vena cava, unroofed coronary sinus, and absent right superior vena cava. Persistent left vena cava draining into the unroofed coronary sinus was demonstrated easily using the agitated saline‐contrast echocardiography. After conformation with angiographic evaluation, surgery was performed at a single session. Roofing of the coronary sinus with polytetrafluoroethylene patch, mitral cleft repair, tricuspid annuloplasty, atrioventricular defect repair with pericardial patch, and resection of the membrane in the left atrium was succeeded without complication.     

Patterns of anomalous pulmonary venous drainage

All of our cases of abnormal pulmonary venous connections collected to the middle of 1965 and verified at surgery or autopsy have been reviewed by means of diagrams and tabulations, using a specially devised code to facilitate the survey. The material consisted of 52 autopsy cases (half of them obtained after surgery) and the cases of 72 patients who survived operation. The postmortem group was much younger than the surgical group and differed also from the latter by showing male preponderance as well as relatively many instances of total abnormal pulmonary venous connection and frequently associated cardiac anomalies. Partial anomalous connection of right pulmonary veins was 10 times more frequent than that of the left pulmonary veins. This was caused by (1) the frequent drainage of some of the right pulmonary veins into the junctional area between right atrium and superior vena cava in the presence of normal left pulmonary veins, and (2) the complete absence of isolated left pulmonary venous connection to the right atrium. Abnormal connection of solitary pulmonary veins was always effected to the most proximal venous structure among the four possible ones which are derived from the main embryonic channels (superior vena cava and inferior vena cava on the right side, and left superior vena cava and coronary sinus on the left side). Common pulmonary veins from one lung also drained in accordance with this proximity rule, if this may be taken to apply also to the drainage of right pulmonary veins into the right atrium. The one exception in our material was the drainage of all right pulmonary veins into the portal venous system. Total abnormal pulmonary venous connection may be found with all structures mentioned, but most frequently with the left superior vena cava, or coronary sinus, or both, usually by way of a common pulmonary vein. In a few cases however, drainage into different sites, all of them abnormal, did occur. Then again the proximity rule seemed to apply. A tentative embryological explanation is given for the patterns described.     

Sinus Node Dysfunction Associated with Isolated Left Superior Vena Cava

The absence of the right superior vena cava with persistance of the left is a rare venous anomaly, which occurs in 0.05% of general population. This anomaly has been associated with other congenital cardiac defects but rarely with sinus node dysfunction. We report the case of a 52-year-old woman who suffered from symptomatic bradycardia that was associated with supraventricular arrhythmias, and refractory to drugs. Implantation of a pacemaker was necessary and an AAI mode possible because of normal atrioventricular conduction. Insertion of the PM lead was performed via the right cephalic vein, then conducted through the innominate vein into the left persistent superior vena cava, leading to the right atrium via the coronary sinus. The electrode was fixed to the right atrial lateral wall; stimulation threshold was within normal limits. Digital angiography of superior venous inflow confirmed complete absence of the right superior vena cava and described a dilated coronary sinus. No other structural heart disease was present. In accordance with reports in the literature, we found the absence of right superior vena cava complicated by sick sinus syndrome and suggest an etiologic link between these two afflictions. Sinus node function should be studied whenever this venous anomaly is discovered in presence of evocated symptoms.     

Unruptured congenital aneurysm of the left sinus of Valsalva in an adult with complex left heart malformations

A 26 year old man who presented with the first signs of right heart failure was found to have a large congenital aneurysm of the aortic sinus of Valsalva and of the left coronary sinus. These were combined with left heart anomalies in the form of a bicuspid aortic valve, a rare variant of a persistent left superior vena cava with blood flow from the left atrium through the brachiocephalic vein into the superior vena cava and a kink in the aortic arch. An aortic coarctation had been corrected with a patch 12 years earlier. Although the aneurysm was not perforated and there were no clinical signs of infarction, the aneurysm was resected prophylactically and the left coronary artery was reinserted through a bypass with a Gore-Tex conduit. The case is noteworthy because this diagnosis is very rare and its early treatment may prevent several complications. The clinical features, treatment, and outcome are discussed.     

Two cases of left superior vena cava draining directly to a left atrium with a normal coronary sinus.

The most common variation in the thoracic systemic venous system is a persistent left superior vena cava draining to a coronary sinus. A rare anomaly is a persistent left superior vena cava connecting directly to the left atrium. In this situation it is believed that the coronary sinus must be absent. This report describes two cases of a persistent left superior vena cava draining to a left atrium with a normal coronary sinus.