Persistent left superior vena cava: case reports and clinical implications

We report four cases of persistent left superior vena cava (LSVC) incidentally recognized during device implantation. Persistent LSVC is the commonest venous anomaly of the thorax and drains into the right atrium via the coronary sinus. Persistent LSVC demonstrates several congenital variations including variable communication with right-sided superior vena cava (SVC), absence of right-sided SVC, drainage into the left atrium creating a right to left shunt, and association with other congenital anomalies of the heart and great vessels. Abnormal persistent fetal dispersion of specialized pacemaker and conduction tissue, which occurs in some individuals with persistent LSVC, may provide an arrhythmogenic substrate. In this article we discuss embryology, diagnostic approaches, clinical features, technical difficulties in accessing the right ventricle, and a review of literature related to persistent LSVC.     

Correction of anomalous drainage of right superior vena cava to left atrium

A 2-year-old boy with cyanosis was found to have normal situs and looping with anomalous drainage of a right-sided superior vena cava to the left atrium, and intact interatrial septum in association with anomalous drainage of the left pulmonary veins to the right superior vena cava. He underwent successful surgical repair of this rare congenital malformation.     

Total anomalous pulmonary venous draining to inferior vena cava

A 12-year-old girl complained of tachycardia and dyspnea after exertion but without cyanosis. Her echocardiogram showed an ASD and a widened inferior vena cava. A difference in PO2 between inferior and superior vena cava was evident. An anastomosis between left atrium and pulmonary vein, and a long oval pericardium to cover the opening of the descending vein and ASD into left atrium were performed during cardiopulmonary bypass. The patient recovered without complications.     

Abnormal drainage of inferior vena cava to left atrium together with a partial abnormal pulmonary venous drainage to right atrium in the presence of atrial septal defect

The direct abnormal drainage of the inferior vena cava (IVC), while rare, is well‐recognized anomaly of systemic venous drainage. It has been reported both in isolation and in association with other cardiac defects. This is a case of an abnormal drainage of IVC into left atrium (LA) together with partial abnormal pulmonary venous drainage to the right atrium (RA) and atrium septal defect (ASD).     

Right Superior Vena Cava Connected Left Atrium with Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum: An Unusual Cause of Paradoxical Embolism

A six-month-old girl presented with history of convulsion and cyanosis, was eventually found to have a right superior vena cava connected to left atrium with anomalous upper right pulmonary vein return and without atrial septal defect. Diagnosis was confirmed by cardiac magnetic resonance imaging. The patient underwent a successful cardiac surgery correction, routing SVC to right atrium and repair of right upper pulmonary vein to left atrium.     

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.     

Transcatheter occlusion of a persistent left superior vena cava to the left atrium using the transseptal approach

A persistent left superior vena cava (LSVC) is a common venous anomaly, occurring in up to 10% of patients with congenital heart defects. Usually, a LSVC drains into the coronary sinus, then to the right atrium. The LSVC can drain directly to the left atrium, resulting in a right‐to‐left shunt and systemic desaturation. Historically, surgery has been used to address this lesion. Transcatheter occlusion of the LSVC is an alternative to surgery. We report the novel use of the transseptal approach to access the LSVC, and device occlusion using the Amplatzer Vascular Plug‐II. © 2013 Wiley Periodicals, Inc.     

Use of contrast echocardiography in diagnosis of anomalous connection of right superior vena cava to left atrium.

A 4-month-old infant with cyanosis but without other abnormal cardiac findings is presented in whom the diagnosis of anomalous systemic venous connection to the left atrium was made by contrast echocardiography. The diagnosis was later confirmed by cardiac catheterisation and selective cineangiography. When saline was injected into a vein on the dorsum of each hand while echocardiographically recording the cardiac structures, the left atrium, left ventricle, and aorta were opacified without visualisation of the right ventricle. Similar study with injection into the right foot produced opacification of the right ventricle without visualisation of the left-sided structures. These data suggested normal drainage of the inferior vena cava with anomalous connection of the superior vena cava to the left atrium. A review of the previously reported cases of anomalous connection of the right superior vena cava to the left atrium is presented together with the possible embryological origin of this anomaly.     

Atrial septal defect and cyanosis. Apropos of 6 cases related to abnormal drainage of the inferior vena cava into the left atrium

The authors report six cases of atrial septal defect (ASD) associated with abnormal drainage of the inferior vena cava into the left atrium responsible for right-to-left shunting, without pulmonary hypertension. The abnormal drainage could be due either to an anatomical malposition of the inferior vena cava opening into the left atrium, or to an abnormal blood flow from this vein, normally located through a low ASD, under the influence of anatomical, mechanical and haemodynamic factors. Clinically, all patients presented with light cyanosis and with the usual signs of ASD. None of them had elevated pulmonary pressure. The lesion, suggested by clinical findings, was diagnosed either at angiography, which in four cases demonstrated an abnormal pulmonary venous return, or at colour-coded doppler echocardiogram, or at surgery. In every case, surgical correction consisted of closure of the often low-sited ADS by a patch which diverted the inferior vena cava into the right atrium and the abnormal venous return towards the left atrium. The short--and long-term results of surgery were excellent. The authors review the literature concerning this unusual association of ASD with an abnormal drainage of the inferior vena cava into the left atrium.     

Anesthetic implications of total anomalous systemic venous connection to left atrium with left isomerism

Total anomalous systemic venous connection (TASVC) to the left atrium (LA) is a rare congenital anomaly. An 11-year-old girl presented with complaints of palpitations and cyanosis. TASVC with left isomerism and noncompaction of LV was diagnosed after contrast echocardiogram and computed tomography angiogram. The knowledge of anatomy and pathophysiology is essential for the successful management of these cases. Anesthetic concerns in this case were polycythemia, paradoxical embolism and rhythm abnormalities. The patient was successfully operated by rerouting the systemic venous connection to the right atrium.     

Masked abnormal drainage of the inferior vena cava into the left atrium

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Morphological dilemma: Anomalous pulmonary venous confluence or cor triatriatum—does it matter?

Cardiac variant of total anomalous pulmonary venous return is a rare entity, whereby all the pulmonary veins drain directly to the right atrium or coronary sinus. The effective left heart blood flow channels through a small stretched patent foramen ovale and can often be confused with a variant of cor triatriatum. Cor triatriatum is a rare congenital cardiac anomaly where pulmonary veins drain to a persistent chamber above the left atrium with a membrane separating these two. There persists either a small aperture directly from the true to the accessory left atrium or none at all. Where there is no such aperture, it is often physiologically akin to the cardiac variant of total anomalous pulmonary venous return described above. Such morphological differentiation is often challenging in a clinical situation, but the effective treatment remains the same. It involves removal of the common wall between the two chambers and baffling the pulmonary veins effectively to the left atrium. We describe such a case where the pulmonary venous return is to the right atrium, managed recently in our centre, and discuss the morphological differences between these two.     

Temporary intraluminal bypass for superior vena cava reconstruction after cancer invasion

We report a case of right upper lobe bronchogenic cancer widely infiltrating the superior vena cava (SVC) in which right pneumonectomy was performed with partial resection of the SVC wall. The SVC was reconstructed by means of a pericardial patch; during reconstruction a temporary intraluminal bypass was set up to obtain a proper venous return to the right atrium.     

Isolated atrial inversion Visceral situs solitus, visceroatrial discordance, discordant ventricular d loop without transposition, dextrocardia: Diagnosis and surgical correction

After cardiac catheterization and angiocardiographic studies an infant with cyanosis from birth was found to have visceral situs solitus, visceroatrial discordance without venoatrial discordance, a discordant ventricular d loop without transposition, and dextrocardia. The circulatory pathway was from the venae cavae to right atrium to left ventricle to aorta, and from the pulmonary veins to left atrium to right ventricle to pulmonary artery. An atrial defect was present allowing some mixing between the pulmonary and systemic circulations, but this appeared inadequate and the defect was enlarged surgically. Subsequently, a corrective operation using an atrial baffle to direct the pulmonary venous return to the left ventricle and the systemic venous return to the right ventricle was successfully undertaken.     

Drenagem venosa pulmonar anômala em gestante identificada pela ressonância magnética cardíaca

Anomalous pulmonary venous return (APVR) is a rare cardiac anomaly defined as one or more pulmonary veins draining into a structure other than the left atrium, with venous return directly or indirectly to the right atrium. The most common form is partial APVR, in which one to three pulmonary veins drain into systemic veins or into the right atrium. We report the case of a woman diagnosed with partial APVR by magnetic resonance imaging during pregnancy.     

Bilateral absence of the superior vena cava presenting with superior vena cava syndrome: A case report

Bilateral absence of the superior vena cava (SVC) is a very rare congenital vascular anomaly that is mainly asymptomatic. In this report, we describe an adult male patient with bilateral absence of the SVC presenting with SVC syndrome. Blood from the upper body returned to the right atrium via the superficial thoracoepigastric veins, the great saphenous veins, the common femoral veins, and the inferior vena cava.     

完全性肺静脉异位引流的发病机制研究进展

完全性肺静脉异位引流（total anomalous pulmonary venous connection，TAPVC）是一类罕见而又严重的先天性心脏病。TAPVC是因肺静脉未能与左心房相连，而直接与右心房或体循环静脉系统连接，导致该类患儿生长发育明显受限，生后全身紫绀及缺氧表现逐渐加重，严重威胁患儿的生命，需尽早手术干预。TAPVC的发病原因和机制尚未完全明确，近年来随着现代分子遗传学的发展，对其的发病机制有了初步的认识，本文就TAPVC发病机制研究进展作一概述。     

Inferior vena caval return to the left atrium with intact interauricular septum. Apropos of 2 cases surgically treated with success

Two cases of abnormal inferior vena caval return to the left atrium are reported in a 9 year old boy and 13 year old girl. The diagnosis was suspected on the finding of severe cyanosis alone, and the anomalous systemic venous return was correctly identified by angiography of the inferior vena cava. There were three angiographic points of interest in these two cases: the normal position of the inferior vena cava, the absence of a left-to-right interatrial shunt and the presence of a vertical intra-left atrial "membrane" inserted behind the orifice of the inferior vena cava. Surgical correction consisted in resecting the inferior part of the interatrial septum and reconstructing a new septum behind the orifice of the inferior vena cava with a dacron patch in one case and by direct suture in the other. The good surgical result was controlled by angiography in both cases. In the second case, an intrapulmonary shunt due to multiple arteriovenous fistulae demonstrated by contrast echocardiography was responsible for persistent mild cyanosis for a few months after surgery. The long-term clinical results were excellent in both cases. The authors review the literature of this rare malformation due to persistence of part of the right valve of the sinus venosus.     

Persistent left superior vena cava communicating with the left atrium through a systemic-pulmonary venous malformation

A 14 year old white girl who presented with a brain abscess was discovered to have a left pulmonary vascular malformation on a chest roentgenogram. Angiograms revealed a left superior vena cava that drained into a venous malformation within the left lung, then communicated with the left atrium by way of the left superior pulmonary vein. The right superior vena cava was functionally absent and was anatomically an atretic cord. There was mild systemic arterial hemoglobin desaturation, but no evidence of cyanosis. The embryology, physiology and surgical repair of this rare lesion and the complication of a postoperative superior vena cava syndrome are discussed.