Double-sided superior vena cava: developmental considerations associated with the thymic veins / Vena cava superior de doble cara: consideraciones de desarrollo asociadas con las venas tímicas

SUMMARY: The superior vena cava is usually located only on the right side, but persistence of the left superior vena cavais observed in about 0.3 to 0.5 % of adults. A routine dissection of the cadaver of a 91-year-old Japanese female, whose cause of death was sepsis due to cholecystitis, was performed at Nagasaki University and revealed a double-sided superior vena cava. On the right side, the superior vena cava opened to the right atrium, while on the left, it opened into the extended coronary sinus. Veins in the left head, neck and upper limb regions joined to form the persistent left superior vena cava, with eventual drainage into the expanded coronary vein. An anastomosing branch occurred between each superior vena cava, and two thymic veins opened to the anastomosing branch. The azygos vein in the azygos venous system opened into the right superior vena cava, whereas a hemi-azygos vein opened into the azygos vein. The accessory hemi-azygos vein also opened into the azygos vein and opened cranially into the left superior vena cava. The left supreme intercostal vein also opened into the left superior vena cava. Several studies have reported a persistent left superior vena cava and the various considerations for its occurrence. Here, we propose a new hypothesis for the embryonic development of the persistent left superior vena cava with the thymic vein. This hypothesis essentially states that the left brachiocephalic vein fails to mature due to inadequate venous return from the thymic vein during the embryonic period, and the left superior vena cava then remains to maintain venous return from the left head, neck and upper limb. We also discuss the clinical significance of the persistent left superior vena cava.

RESUMEN: Usualmente la vena cava superior se localiza solo en el lado derecho, sin embargo en aproximadamente 0,3 a 0,5 % de los adultos se observa la persistencia de la vena cava superior izquierda. En la Universidad de Nagasaki se realizó una disección de rutina del cadáver de una mujer japonesa de 91 años, cuya causa de muerte fue sepsis debido a una colecistitis. El cuerpo presentaba una vena cava superior doble. En el lado derecho, la vena cava superior llegaba al atrio derecho, mientras que en el lado izquierdo drenaba al seno coronario. Las venas de las regiones de la cabeza, el cuello y del miembro superior izquierdo formaban la vena cava superior izquierda persistente, con drenaje hacia la vena coronaria. Se observó una rama anastomótica entre cada vena cava superior y dos venas tímicas drenaban a la rama anastomótica. La vena ácigos drenaba a la vena cava superior derecha, mientras que una vena hemiácigos drenaba a la vena ácigos. La vena hemiácigos accesoria también drnaba en la vena ácigos y cranealmente lo hacia la vena cava superior izquierda. La vena intercostal suprema izquierda drenaba en la vena cava superior izquierda. Varios estudios han informado una vena cava superior izquierda persistente y las diversas consideraciones para su aparición. Aquí, proponemos una nueva hipótesis para el desarrollo embrionario de la vena cava superior izquierda persistente con la vena tímica, que esencialmente establece que la vena braquiocefálica izquierda no se dearrolla debido a un retorno venoso inadecuado de la vena tímica durante el período embrionario, y se mantiene la vena cava superior izquierda para el retorno venoso de la cabeza, el cuello y el miembro superior izquierdo. Además se informa de la importancia clínica de la persistencia de la vena cava superior izquierda.

Two-Stage Intracardiac Repair for Total Anomalous Pulmonary Venous Return (Ib+Ib) with a Persistent Left Superior vena cava and an Enlarged Coronary Sinus / 日本心臓血管外科学会雑誌

This study reports the case of a 0-day-old girl who was transferred to our hospital for suspected total anomalous pulmonary venous return due to her postnatal cyanosis. After she was presented at our hospital, pulmonary vein stenosis was diagnosed and emergency surgery was planned. Preoperative computed tomography showed abnormal perfusion of the pulmonary veins into the left and right superior vena cava separately on the left and right sides. Given that the persistent left superior vena cava was refluxing into the coronary sinus, the coronary sinus was enlarged greatly, and the left atrial volume was small. In the neonatal period, the right pulmonary vein was anastomosed to the right atrium, and the left pulmonary vein was anastomosed to the unroofed coronary sinus. Thereafter, at 4 months of age, the patient underwent two-stage intracardiac repair with re-routing of the right pulmonary vein and extracardiac ligation of the left vena cava. The postoperative course was good. Intracardiac repair via a two-stage surgery was effective for total anomalous pulmonary venous return (Ib+Ib) with a persistent left superior vena cava and an enlarged coronary sinus.

Surgical treatment of pediatric unroofed coronary sinus syndrome / 中华实用儿科临床杂志

Objective@#To analyze the clinical features and surgical treatment of unroofed coronary sinus syndrome(UCSS) in children, and to provide evidence for the diagnosis and treatment of such diseases during the perio-perative period.@*Methods@#A retrospective analysis was conducted based on the clinical data of 13 patients with UCSS, who underwent surgical treatment at Beijing Children′s Hospital Affiliated to Capital Medical University from January 2011 to December 2017.All 13 patients were complicated with persistent left superior vena cava(PLSVC) and other cardiac malformations; 7 patients were diagnosed preoperatively and 6 patients were diagnosed intraoperatively.Eleven cases were diagnosed type Ⅰ and 2 cases were type Ⅳ according to Kirklin′s classification.In all of 13 cases, atrial septal reconstruction was performed to drain the left superior vena cava(LSVC) to the right atrium, and other cardiac malformations were corrected at the same time.@*Results@#One patient died in this group, and the other 12 patients had early recovery after operation.Postoperative-ventilator-assisted time was 5-246 h(median 29 h) and hospital stay time was 8-40 d(median 16 d). The remaining 12 cases were followed up from 6 months to 7 years and 2 months.No death or complications occurred.No residual shunt or residual obstruction was found.@*Conclusions@#In the case of congenital heart disease with PLSVC, UCSS should be alerted.Atrial septal reconstruction and drainage of LSVC into right atrium in children with UCSS can achieve satisfactory surgical results.

Surgical treatment of pediatric unroofed coronary sinus syndrome / 中华实用儿科临床杂志

Objective To analyze the clinical features and surgical treatment of unroofed coronary sinus syn-drome(UCSS)in children,and to provide evidence for the diagnosis and treatment of such diseases during the perio-perative period. Methods A retrospective analysis was conducted based on the clinical data of 13 patients with UCSS, who underwent surgical treatment at Beijing Children＇s Hospital Affiliated to Capital Medical University from January 2011 to December 2017. All 13 patients were complicated with persistent left superior vena cava( PLSVC)and other cardiac malformations;7 patients were diagnosed preoperatively and 6 patients were diagnosed intraoperatively. Eleven cases were diagnosed typeⅠand 2 cases were typeⅣaccording to Kirklin＇s classification. In all of 13 cases,atrial sep-tal reconstruction was performed to drain the left superior vena cava(LSVC)to the right atrium,and other cardiac mal-formations were corrected at the same time. Results One patient died in this group,and the other 12 patients had early recovery after operation. Postoperative-ventilator-assisted time was 5-246 h( median 29 h)and hospital stay time was 8-40 d(median 16 d). The remaining 12 cases were followed up from 6 months to 7 years and 2 months. No death or complications occurred. No residual shunt or residual obstruction was found. Conclusions In the case of congenital heart disease with PLSVC,UCSS should be alerted. Atrial septal reconstruction and drainage of LSVC into right atrium in children with UCSS can achieve satisfactory surgical results.

Vena cava superior izquierda persistente: Reporte de caso / Persistent left superior vena cava: A case report

La vena cava superior izquierda persistente es la anomalía venosa congénita más frecuente; se observa en 0,3 a 0,5% de la población general y 4 a 8% de los casos con cardiopatía congénita. El resultado perinatal es significativamente peor si se asocia con defectos cardiacos y el pronóstico es excelente en casos aislados. Se presenta un caso de vena cava superior izquierda persistente diagnosticado en un feto a las 30 semanas en una paciente de 21 años de edad referida para evaluación ecocardiográfica fetal prenatal. Durante la visualización de los tres vasos, se observó la presencia de una estructura circular vascular supernumeraria a la izquierda de la arteria pulmonar y que se extendía hasta llegar a la pared lateral de la aurícula izquierda, compatible con vena cava superior izquierda persistente. No se encontraron otras anomalías cardiacas. El embarazo fue sin complicaciones y se obtuvo recién nacido sano a término sin ninguna complicación. La ecocardiografía posnatal confirmó el diagnóstico. No fue necesaria ninguna intervención.

Persistent left superior vena cava is the most frequent congenital venous anomaly, observed in 0.3-0.5% of the general population and in 4-8% of patients with congenital heart disease. The perinatal outcome is significantly worse if it is associated with cardiac defects and the prognosis is excellent in isolated cases. We report a case of persistent left superior vena cava diagnosed in a 30-week fetus in a 21-year-old female patient referred for prenatal fetal echocardiographic evaluation. During the visualization of the three vessels, a supernumerary circular vascular structure was observed to the left of the pulmonary artery, extending into the lateral wall of the left atrium, consistent with persistent left superior vena cava. No other heart abnormalities were found. The pregnancy was uncomplicated and a healthy baby was born full-term without any complications. Postnatal echocardiography confirmed the diagnosis. No intervention was necessary.

Absent right superior vena cava and persistent left superior vena cava in a patient with bicuspid aortic valve with aortic stenosis

Persistent left superior vena cava (LSVC) with absent right SVC (RSVC) is a rare congenital anomaly. If undetected, the condition may pose difficulties in central venous catheter insertion, pacemaker electrode insertion, and cannulation during cardiopulmonary bypass. We describe a case of persistent LSVC with absent RSVC, who was diagnosed to have bicuspid aortic valve with aortic stenosis.

Prenatal ultrasonic diagnosis of fetal persistent left and absent right superior vena cava / 中国医学影像技术

Objective To investigate characteristics and value of prenatal ultrasound in diagnosing persistent left and absent right superior vena cava.Methods Ultrasonic data of 8 fetuses with persistent left and absent right superior vena cava were retrospectively analyzed.Ultrasonic findings of persistent left and absent right superior vena cava and other complicated anomalies were observed,and the outcomes were followed up.Results The ultrasonic characteristics of persistent left and absent right superior vena cava included a vessel which could be seen on the left of pulmonary artery on three vessel-trachea view draining into the dilated coronary sinus,and right superior vena cava was absent.With combined spatio-temporal image correlation (STIC) and high definition flow (HDF) technique,the spatial relationship of the left superior vena cava,aorta and pulmonary artery could be observed.Dilated coronary sinus was found in all 8 fetuses,other congenital heart defects were detected in 5 fetuses,and extracardiac anomaly was found in 1 fetus.Conclusion Persistent left and absent right superior vena cava and complicated anomalies can be accurately diagnosed with prenatal ultrasound.Dilated coronary sinus is an important clue for prenatal ultrasonic diagnosis.

Comparison of coronary sinus diameter Z-scores between normal fetuses and fetuses with persistent left superior vena cava / 中华超声影像学杂志

Objective To establish normal reference ranges of Z-score of coronary sinus CS diameter in normal fetuses and to explore the diagnostic value of CS Z-score in fetuses with persistent left superior vena cava PLSVC Methods A retrospective cross-sectional study of 227 singleton normal fetuses and 30 fetuses with PLSVC were involved Non-cardiac biometrical parameters included biparietal diameter BPD femoral length FL heart area HA and an assessment of gestation age GA based on menstrual age GA Systolic diameter of CS CSDs and diastolic diameter of CS CSDd were measured at the end of systole and diastole CSDs and CSDd Z-score models were constructed by linear regression analysis with GA FL HA and BPD as independent variables Subsequently Z-scores between normal fetuses and fetuses with PLSVC were compared Results Correlations between fetal CSDs and CSDd and four independent variables BPD FL HA and GA were excellent GA had close correlation with CSDs and CSDd CSDd Z-score= the actual measurement of CSDd- prediction of CSDd based on GA BPD FL or HA SD forecast CSDd CSDs Z-score= actual measurement CSDs-prediction CSDs based on GA BPD FL or HA SD forecast CSDs Normal reference value of CSDd and CSDs in 227 normal fetuses could be predicted as followings with GA as independent variable CSDd and CSDs were 0 139 ~ 0 3 19 cm and 0 046~0 166 cm respectively For BPD CSDd and CSDs were 0 128 ~0 303 cm and 0 040~0 1 59 cm respectively For FL CSDd and CSDs were 0 135~0 307 cm and 0 046~0 164 cm respectively For HA CSDd 0 1 57~ 0 323 cm and CSDs 0 059 ~ 0 1 75 cm respectively With GA as independent variable CSDd Z-score and CSDs Z-score were -0 906~1 256 and -0 920~0 895 respectively For BPD CSDd Z-score and CSDs Z-score were -1 168~1 196 and -0 864~0 899 respectively For FL CSDd Z-score and CSDs Z-score were -1 478~ 1 546 and -1 297~ 1 3 10 respectively For HA CSDd Z-score and CSDs Z-score were -0 832 ~ 0 610 and -0 619 ~ 0 688 respectively CSD Z-scores of 30 PLSVC fetuses as followings with GA as independent variable CSDd Z-score and CSDs Z-score were 5 263 ~ 1 1 659 and 2 846~9 923 respectively For BPD CSDd Z-score and CSDs Z-score were 4 469 ~ 1 1 000 and 2 3 10 ~9 1 14 respectively For FL CSDd Z-score and CSDs Z-score were 5 473 ~ 13 056 and 3 234 ~ 13 135 respectively For HA CSDd Z-score and CSDs Z-score were 1 922~5 701 and 1 359~5 723 respectively Z-scores of PLSVC fetuses were far away from the normals Conclusions Development of normal fetal CSDs and CSDd Z-score reference ranges is realized The CSDs and CSDd Z-scores can provide quantitative evidence in prenatal diagnosis of PLSV.

Effects of isolated persistent left superior vena cava on fetal cardiac structure and hemodynamics / 中华超声影像学杂志

Objective To investigate the difference in cardiac structure and hemodynamics characteristics between fetuses with isolated persistent left superior vena cava (LSVC)and normal fetuses by using fetal echocardiography,in order to explore whether isolated PLSVC has some influence on the development of fetal heart structure development.Methods Twenty-two fetuses diagnosed with isolated PLSVC in the second and third trimester were enrolled in this study.The PLSVC fetuses were divided into two groups:group 1 ,twenty-two fetuses in the second trimester (23-27+6 W);group 2,twenty fetuses in the third trimester (28-39+6 W).Forty-nine fetuses without intra-cardiac and extra-cardiac anomalies with gestation age matched were involved as normal control groups:25 fetuses in the second trimester,and 24 fetuses in the third trimester.The parameters of cardiac structure and hemodynamics of the four groups were measured,including left and right sideheart size,the diameter of foramen ovale(FO),aorta(AO),aortic isthmus and pulmonary artery (PA).The ratio of RV/LV,RA/LA and PA/AO were calculated.The hemodynamic parameters included the flow velocity across mitral valve(MV),tricuspid valve(TV)and FO. All the parameters of PLSVC groups were compared with the normal groups respectively.Results In the second trimester group,the ratio of RV/LV and PA/AO of the PLSVC fetuses were significantly larger than normal,while the AO diameter,aortic isthmus diameter were smaller than normal group (P <0.05). However in the third trimester group,only the ratio of PA/AO of the PLSVC group was significantly greater,and the aortic isthmus diameter was still smaller than the normal group (P <0.05).Conclusions Isolated PLSVC connected with coronary sinus is associated with structural differences from normal.A dilated coronary sinus may have an influence on the development of fetal left heart structures.

Unusual paradoxical embolic stroke in a patient with persistent left superior vena cava / 영남의대학술지

Cardiogenic embolic stroke accounts for approximately 20% of ischemic strokes and the likelihood of its recurrence is high. Paradoxical embolism may be an important cause of cardioembolic stroke, which can be evaluated through multiple diagnostic modalities including transesophageal echocardiography (TTE) or transcranial Doppler. A persistent left superior vena cava (PLSVC) is a rare congenital vascular anomaly, which mainly drains to the right atrium via the coronary sinus. Although rare, PLSVC draining into the left heart predisposes the patient to paradoxical embolism through a right-to-left shunt. We report on a 78-year-old female patient with an ischemic stroke associated with PLSVC draining into the left atrium through the pulmonary vein, which was investigated via TTE with an agitated saline test and computed tomography.

Persistent left superior vena cava: a case report and review of literature.

Persistent Left Superior Vena Cava (PLSVC) is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities. The presence of PLSVC can render access to the right side of heart challenging via the left subclavian approach, which is a common site of access utilized. Incidental notation of a dilated coronary sinus on echocardiography should raise the suspicion of PLSVC. Here we present a case of PLSVC that was discovered in an infant accidently after central line insertion.

When the Left is left.

Persistent left superior vena cava is an uncommon vascular anomaly; however it is the most common anomaly of the thoracic venous system. It may be stand alone or associated with other congenital heart diseases and even other extracardiac anomalies. It is due to a lack of regression and adsorption of the left anterior cardinal vein. The persistence of this vessel renders a left subclavian approach for interventions on the right heart a challenge. It may be responsible for arrthymiias. We present a report of a persistent left superior vena cava draining into the coronary sinus with a coexisting normal right superior vena cava. Keeping in mind its widespread implications on cardiac procedures and a causative factor of cardiac disturbances we have considered its course, embryological source and clinical significance.

Correlación anátomo tomográfica de la vena cava superior izquierda persistente / Anatomy and CT correlation of persistent left superior vena cava

Introducción: La vena cava superior izquierda es un hallazgo incidental en la mayoría de los casos y representa la persistencia de la vena cardinal anterior izquierda embriológica. Es la anomalía venosa intratorácica más común. Se halla entre el 0,1 y el 0,3% de las personas normales y hasta en el 10% de los que presentan alguna malformación cardíaca congénita. Habitualmente, desemboca en la aurícula derecha a través del seno coronario y en alrededor del 70% de los casos está comunicada con la vena cava superior derecha por el tronco venoso innominado. Objetivo: Correlacionar anatomía y tomografía computada de la vena cava superior izquierda persistente. Materiales y métodos: Para la realización de la presente comunicación, se utilizaron un cadáver adulto, conservado en formaldehído al 10% en el que, durante la disección del bloque cardiopulmonar, se reconoció una vena cava superior izquierda; y un conjunto de imágenes tomográficas en las que también aparece dicha anomalía. Se realizó la correlación anátomoimagenológica correspondiente. Discusión: Mediante imágenes de tomografía computada se logró reconocer claramente la vena cava superior izquierda. Además, pudo lograrse una excelente correlación anátomoimagenológica de la estructura considerada. Conclusiones: La presencia de una doble vena cava se podría sospechar ante la aparición de un arco rectificado de la arteria pulmonar izquierda en la radiografía de tórax, ó también, de manera incidental al realizarse una tomografía o unecocardiograma. Cabe destacar la importancia de este hallazgo tanto desde el punto de vista de la imagenología como desde su implicancia clínica.

Introduction: The left superior vena cava is an incidental finding in most cases and represents the persistence of the embryologic left anterior cardinal vein. It is the most common intrathoracic venous anomaly. It lies between 0.1 and 0.3 % of normal people and up to 10 % of those with a congenital heart defect. Usually empties into the right atrium through the coronary sinus and in about 70 % of cases are reported to the right superior vena cava by the brachiocephalic vein. Objective: Correlate anatomy and computed tomography persistent left superior vena cava. Materials and methods: To achieve this communication, we used adult corpse, preserved in 10% formaldehyde in which, during dissection of the heart lung block was recognized the left superior vena cava, and a set of tomographic images in the it alsoappears that anomaly. Correlation was performed for anatomic imagenological. Discussion: Using tomographic images were also recognized clearly the left superior vena cava. Furthermore, an excellent correlation was achieved anatomic imagenological considered structure. Conclusions: The presence of a double vena cava could be suspected in the occurrence of an arc grinding of the left pulmonary artery in the chest radiograph, or also, incidentally when performing a tomographic or echocardiogram. Note the importance of this finding from the point of view of the imaging and from its clinical implication.

Successful Implantation of an Implantable Cardioverter-Defibrillator Through a Persistent Left Superior Vena Cava / 대한내과학회지

The prevalence of persistent left superior vena cava (PLSVC) in the general population has been estimated to be approximately 0.3%, as determined by autopsy. PLSVC is hemodynamically insignificant if it is not associated with other congenital cardiac anomalies, and usually goes unrecognized until a left superior approach to the heart is required. Here, we report a 60-year-old male diagnosed with dilated cardiomyopathy, who had survived an episode of sudden cardiac arrest with documented ventricular fibrillation. PLSVC was recognized incidentally while implanting an implantable cardioverter-defibrillator (ICD). The ICD was successfully implanted through the left superior vena cava via the coronary sinus using a straight stylet and an active fixation device.

Radiofrequency cather ablation of right sided accessory atrioventricular pathway with persistent left superi-or vena cava in 7 patients / 实用医学杂志

Objective To summarize radiofrequency cather ablation (RFCA)of tachycardias with right sid-ed accessory atrioventricular pathway in 7 patients with persistent left superior vena cava (PLSVC). Methods RF-CA for right sided accessory atrioventricular pathway in 7 patients with PLSVC was carried out . Coronary sinus ac-cess was performed through the left subclavian and the left femoral vein respectively. Swartz sheath was used during ablation if neccesary. Results There was no siginificant differences in fluoroscopy time between the left subclavian approach and the left femoral vein approach[(2.8 ± 0.6)min vs (3.2 ± 0.3)min, P>0.05)]. No recurrence of ac-cessory pathway conduction was found during a mean follow-up of (23 ± 11) months. The mean discharging time and fluoroscopy time were (312 ± 67)s and (17 ± 5)min respectively. Conclusions Although RFCA of right sided accessory atrioventricular pathway in patients with PLSVC shows specialities in cather manipulation , mapping and ablation, high successful results can be achieved by experienced physician.

Partially unroofed coronary sinus with persistent left superior vena cava: the utility of two and three-dimensional transesophageal echocardiography: a case report / 대한마취과학회지

Unroofed coronary sinus (URCS) is a rare cardiac anomaly, in which communication occurs between the coronary sinus (CS) and the left atrium (LA) because of partial or complete absence of the CS roof. A 30-year-old woman was scheduled for surgical closure of atrial septal defect, mitral valve repair and tricuspid annuloplasty. The intraoperative transesophageal echocardiography (TEE) revealed left-to-right shunt between the CS and the LA. The three-dimensional (3D) TEE confirmed the diagnosis of partially URCS. This defect was repaired with a pericardial patch. In this case, the 3D images of URCS, which were a helpful supplement to the 2D images, providing better visualization of the wall defect and more information regarding the size and location of the defect. The combined use of 2D and 3D images provides valuable information to aid in understanding the anatomy and morphology of this rare anomaly.

Transcatheter Treatment of Patent Foramen Ovale Combined with Abnormal Drainage of Left Superior Vena Cava to Left Upper Pulmonary Vein

Patent foramen ovale (PFO) has been known to be the cause of transient ischemic attacks or stroke, and transcatheter device closure has been the treatment of choice for these defects. Combined defect of abnormal drainage of left superior vena cava (LSVC) to left superior pulmonary vein (LSPV) in PFO patients is an uncommon combination, and both can act as a pathway for paradoxical embolism. We report a successful closure of PFO, using Amplatzer(R) PFO occluder (St. Jude Medical, St. Paul, MN, USA) and persistent LSVC connected to LSPV using an Amplatzer(R) Vascular Plug II (St. Jude Medical, St. Paul, MN, USA). Because this combined anomaly of PFO and persistent LSVC can be treated by a single transcatheter intervention, if clinically suspected, a complete evaluation for this anomaly should be considered.

Anomalías venosas sistémicas más frecuentes en el feto: embriología, diagnóstico prenatal y asesoramiento cardiogenético / Most common systemic venous abnormalities found in the fetus: embryology, prenatal diagnosis and cardiogenetic advice

Se hace una extensa revisión de la embriología del sistema venoso torácico y su relación con la anomalía venosa más frecuente a este nivel: la vena cava superior izquierda persistente. Se destaca la importancia de su conocimiento en la interpretación de estas malformaciones, y otra menos frecuente, como la interrupción de la vena cava inferior. Si bien es cierto que su hallazgo per se no afecta significativamente al portador de esta anomalía, su asociación a otras cardiopatías muy graves -que a manera de marcadores anatómicos la acompañan- hacen que su estudio sea necesario para todos aquellos interesados en la evaluación de las cardiopatías congénitas, pediatras, ultrasonografistas y asesores cardiogenéticos.

An extensive review of the embryology of thoracic venous system, and its relation with the most common venous abnormality, that is, persistent left superior vena cava, was made. The review underlined the importance of knowledge in the interpretation of such malformations, and of other less frequent like the interruption of the inferior vena cava. Although it is true that finding this abnormality does not significantly affect the patient, its association with other very severe cardiopathies, which accompany it as a sort of anatomical markers, makes the study necessary for all those people interested in the evaluation of congenital cardiopathies, pediatricians, ultrasonography technicians and cardiogenetic advisers.

Permanent Pacemaker for Sick Sinus Syndrome with Absent Right Superior Vena Cava – A Case Report.

A 42 year old patient, admitted into our hospital for permanent implantation. Implantation of a permanent pacemaker was attempted by the transvenous approach via venous puncture of cephalic vein & subclavian vein on both sides but guide wire failed to pass. Coronary angiogram with cardiac catheterization was done & revealed absence of right superior vena cava and presence of persistant left superior vena cava. Under endotracheal general anesthesia (GA) with limited left thoracotomy epicardial pacemaker was implanted.

Reestablishment the roof of coronary sinus in treating unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava / 中华胸心血管外科杂志

Objective To summarize the surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava.Methods From 2000 to 2008,753 cases of endocardial cushion defect underwent surgical repair.Among them 15 cages(2.0%)were associated with persistent left superior vena cava and unroofed coronary sinus syndrome.The drainage of the persistent left superior vena cava and coronary vein were corrected simultaneously during the operation by reestablishing the roof of the coronary sinus.The persistent left superior vena cava were drained to right atrium by intra-atrial tunnel with pericardial patch or left atrium folding in 14 cases and it was ligated in the remain one.Most of the coronary veins were drained into right atrium after the correction of cardiac defect.Results One patient died of severe pulmonary infection early after operation.Echocardiogram indicated that the velocity of left superior vena cava increased in the site of the roof of left atrium in one patient after operation.No obstruction of vena cava and pulmonary vein and remnant shunt between the atriums were found in the other 14 cases.There were no death and other complications during3 months to 6 years of follow-up.Conclusion The surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava is difficult and good result can be expected by reestablishing the roof of the coronary sinus.