Left Vena Research Articles

Persistent left superior vena cava with patent left innominate vein: Ideal anatomy for mini invasive thoracoscopic ligature

Persistent left superior vena cava is the most common congenital anomaly of the thoracic venous system. In most cases, the left vena cava drains into the coronary sinus and remains asymptomatic. When draining in the left atrium, paradoxical embolization could be a severe complication of the right-to-left shunt and is an indication for surgery. We describe the thoracoscopic approach for ligation of a persistent left superior vena cava in a 9 years old girl with VACTERL association and a history of stroke. Bilateral superior vena cava connected by a bridging vein allowed simple ligature of the abnormal systemic venous return.

Adventitial Progenitor Cells of Human Great Saphenous Vein Enhance the Resolution of Venous Thrombosis via Neovascularization.

Background Vascular adventitia contains progenitor cells and is shown to participate in vascular remolding. Progenitor cells are recruited into the venous thrombi in mice to promote neovascularization. We hypothesized that the adventitial progenitor cells of human great saphenous vein (HGSV-AdPC) enhance the resolution of venous thrombosis via neovascularization. Methods Human great saphenous vein (HGSV) was harvested from the patients with great saphenous vein varicose and sectioned for immunohistochemistry, or minced for progenitor cell primary culture, or placed in sodium dodecyl sulfate solution for decellularization. Human venous thrombi were collected from patients with great saphenous vein varicose and superficial thrombophlebitis. Infrarenal abdominal aorta of New Zealand white rabbits was replaced with interposing decellularized vessel, and the patency of the grafts was confirmed by ultrasonic examination. Animal venous thrombi in the left infrarenal vena cava of mice were produced with Prolene suture ligation and ophthalmic force clipping of this portion. After HGSVs were digested by collagenase, the CD34+CD117+ HGSV-AdPC were isolated on FACS system, labelled with CM-Dil, and transplanted into the adventitia of infrarenal vena cava of nude mice. The percentage of thrombus organization area to the thrombus area was calculated as the organization rate. The thrombus cell, endothelial cells, and macrophages in the thrombi were counted in sections. Cell smears and frozen sections of human saphenous veins and venous thrombi were labeled with Sca1, CD34, CD117, Flk1, CD31, and F4/80 antibodies. The CD34+CD117+ HGSV-AdPC were cultured in endothelial growth medium with vascular endothelial growth factor (VEGF) to induce endothelial cell differentiation and analyzed with real time-PCR, Western blotting, and tube formation assays. Results Immunohistochemical staining showed that the CD34+CD117+ cells were located within the adventitia of HGSVs, and many CD34+ and CD117+ cells have emerged in the human venous thrombi. The number of progenitor cells within the marginal area of 7 days mice thrombi was shown to be Sca1+ ≈21%, CD34+ ≈12%, CD117+ ≈9%, and Flk1+ ≈5%. Many CD34+adventitial progenitor cells have migrated into the decellularized vessels. FACS showed that the number of CD34+CD117+ HGSV-AdPC in primary cultured cells as 1.2 ± 0.07%. After CD34+CD117+HGSV-AdPC were transplanted into the adventitia of nude mice vena cava with venous thrombi, the organization rate, nucleate cell count, endothelial cells, and macrophage cells of thrombi were shown to be significantly increased. The transplanted CD34+CD117+ HGSV-AdPC at the adventitia have crossed the vein wall, entered the venous thrombi, and differentiated into endothelial cells. The CD34+CD117+ HGSV-AdPC in the culture medium in the presence of VEGF-promoted gene and protein expression of endothelial cell markers in vitro and induced tube formation. Conclusions HGSV-AdPC could cross the vein wall and migrate from the adventitia into the venous thrombi. Increased HGSV-AdPC in the adventitia has enhanced the resolution of venous thrombi via differentiating into endothelial cells of neovascularization.

Phenotypic spectrum of the RBM10-mediated intellectual disability and congenital malformation syndrome beyond classic TARP syndrome features.

Pathogenic variants in the RBM10 gene cause a rare X-linked disorder described as TARP (Talipes equinovarus, Atrial septal defect, Robin sequence, and Persistent left vena cava superior) syndrome. We report two novel patients with truncating RBM10 variants in view of the literature, presenting a total of 26 patients from 15 unrelated families. Our results illustrate the highly pleiotropic nature of RBM10 pathogenic variants, beyond the classic TARP syndrome features. Major clinical characteristics include severe developmental delay, failure to thrive, brain malformations, neurological symptoms, respiratory issues, and facial dysmorphism. Minor features are growth retardation, cardiac, gastrointestinal, limb, and skeletal abnormalities. Additional recurrent features include genital and renal abnormalities as well as hearing and visual impairment. Thus, RBM10 loss of function variants typically cause an intellectual disability and congenital malformation syndrome that requires assessment of multiple organ systems at diagnosis and for which provided clinical features might simplify diagnostic assessment. Furthermore, evidence for an RBM10-related genotype-phenotype correlation is emerging, which can be important for prognosis.

Surgical Ligation of Patent Ductus Arteriosus Using the Descending Aortic Approach in Two Dogs

Surgical ligation is the treatment of the choice in patients with patent ductus arteriosus (PDA). This case series presents two cases of PDA, one with and one without persistent left cranial vena cava (PLCVC), treated with surgical ligation through the descending aortic approach with mini-thoracotomy. There were no specific complications during the surgical procedures. The descending aortic approach would be an alternative method for dissection of the PDA.

Persistencia de vena cava superior izquierda: ¿marcador ecocardiográfico de malformaciones fetales cardiacas y extra-cardiacas?

Introducción: La vena cava superior izquierda persistente (VCSIP) es la malformación vascular congénita más frecuente, observada en el 0.3% de autopsias en la población general y en el 4 -8 % de pacientes con cardiopatías congénitas. Durante el desarrollo embriológico temprano el retorno venoso de la cabeza y los brazos normalmente drena en la aurícula derecha por las venas cardinales anteriores izquierda y derecha.
 Objetivo: Relacionar la Persistencia de Vena Cava Superior Izquierda (VCSIP) diagnosticada por estudio ecocardiográfico fetal, con la presencia de anomalías intra y extracardiacas.
 Metodología: Se realiza un estudio descriptivo, retrospectivo en 75 casos obtenidos de la base de datos del departamento de Medicina Perinatal del Hospital Universitario La Paz (Madrid –España), durante el periodo comprendido entre 2006 - 2017 con diagnóstico prenatal de (VCSIP). A los 75 pacientes se realiza estudio ecocardiográfico, a 32 amniocentesis y a 2 biopsia de velocidades coriónicas y, se establece su relación o no, con anomalías congénitas cardiacas y extracardiacas. El análisis estadístico se realiza mediante el programa STATA.
 Resultados: Se analizaron ecográficamente 75 fetos con diagnóstico de VCSIP, con una media de edad gestacional al diagnóstico de 26,6 semanas (DE: 5,87; IC: 95%). En 14 pacientes (18.67%) no se detectaron malformaciones; en 27 pacientes (36%) se evidenciaron alteraciones cardiacas; 15 pacientes (20%), presentaron malformaciones extra cardiacas y 19 pacientes (25.33%), presentaron malformaciones extra e intracardiacas.
 Conclusiones: La ecografía prenatal tiene un gran impacto para el diagnóstico temprano de afectaciones y variaciones anatómicas como la vena cava superior izquierda. El diagnóstico de esta alteración obliga a realizar despistaje y seguimiento de otras anomalías fetales asociadas ya sean intra y extra-cardiacas. Con un buen screening ecográfico prenatal no sería imperativo indicar a los pacientes con VCSIP aislada una prueba invasiva prenatal.

Complicated implant in a patient with suspected dextrocardia and persistence of upper left vena cava: physiological considerations and implant issue

Complicated implant in a patient with suspected dextrocardia and persistence of upper left vena cava: physiological considerations and implant issue

PB2437 THROMBOLYTIC THERAPY IN PEDIATRIC PATIENTS; OUTCOME AND COMPLICATIONS OF TISSUE PLASMINOGEN ACTIVATOR

Background:Although tissue plasminogen activator (tPA), is not indicated in patients under 18 years, off‐label administration is increasingly reported in life or limb‐threatening thrombi.Aims:To present experience with tPA administration in pediatric patients.Methods:Between March 2012 and January 2018, hospital records of patients who received thrombolytic therapy were reviewed. Written informed consent forms signed by legal guardians of the patients for off‐lable use of tPA were present in patients’ hospital files. Major bleeding was defined as any intracranial or retroperitoneal bleeding, or bleeding requiring prolonged admission, transfusion or bleeding leading to death. All other bleeding events were classified as minor. Dose modifications were performed with monitorization of clinical findings, imaging studies and bleeding complications.Results:Seven children, 1 day‐17 years underwent thrombolysis with tPA. First patient had acute lymphoblastic leukemia, fungal pneumonia; she had also central venous catheter and developed superior vena cava syndrome due to right juguler vein thrombosis. Second patient had nephrotic syndrome and diffuse pulmonary thrombosis causing hypoxia. Third patient with Kawasaki disease had coronary aneurism and thrombosis. Fourth patient was a newborn with 37 week‐gestation born with left arm cyanosis and had subclavian artery thrombosis. This baby had methylen tethra hydropholate reductase plus phrothrombin 20210 heterozygous mutations. Fifth patient with homozygous antithrombin deficiency had adolescent pregnancy and vaginal delivery; she presented on the post‐partum 10th day with left renal vein, artery and vena cava inferior (VCI) thrombosis. Sixth patient had hypertension and right kidney atrophy developed left renal artery thrombosis. Seventh patient had Behçet's disease and developed right atrium, pulmonary arteries, VCI, vena‐porta, vena hepatica, bilateral iliac vein thrombosis. Administration of tPA was started median 2 days (1‐12) after onset of symptoms. Median initial tPA dose was 0.1 mg/g/h (0.03‐0.4) and the median maximum dose was 0.15 mg/kg/h (0.06‐0.4). Dose escalation or de‐escalation was performed according to monitorization by imaging findings, resolution of clinical findings, and bleeding complication. Median tPA duration was 46 h (12‐72 h). Concomitant heparin was administered in all patients except patient 2 and 3. Outcome was complete resolution was achieved in patients 1,2,6,7, partial resolution in patient 3 and no resolution in patients 4 and 5. There was one organ (kidney in patient 4) and one limp loss (patient 5). There was one major bleeding in patient 7; he had melena, hematemesis, epistaxis that required transfusion and withdrawal of concomitant heparin. Three of the patients had minor bleedings (epistaxis, gingival bleeding, venipuncture site bleeding) that could be easily controlled.Summary/Conclusion:Thrombolysis with tPA seems safe and effective in pediatric patients. A dose between 0.06‐0.1 mg/kg/h seems safer regarding bleeding complications. Doses between‐ 0.15‐0.4 mg/kg/h may be used if necessary with close monitorization.

Accessory polar renal artery not pre-operatively visualized at extra-peritoneal para-aortic lymphadenectomy

The objective of this video is to describe the technique of extra-peritoneal para-aortic laparoscopic lymphadenectomy and emphasize potential vascular risks that should be taken into account during the procedure.The procedure...

Slc:Hartley guinea pigs frequently possess duplication of the caudal vena cava.

The formation of the caudal vena cava is a complex process involving development, regression, and anastomosis. In mammals, the normal caudal vena cava runs to the right side of the abdominal aorta, while duplication of the caudal vena cava has been identified as a congenital abnormality in both companion animals and humans. The present study demonstrates that Slc:Hartley guinea pigs frequently possess asymptomatic duplicated caudal vena cava. The prevalence was 30% and 24% for males and females, respectively, with no sex-related differences. In accordance with Saad et al. (2012)’s criteria, duplicated caudal vena cava were classified into two distinct variations. The dominant variation was a complete duplication without iliac anastomosis where the left caudal vena cava continued from the left common iliac vein and joined the left renal vein; the left renal vein ran to the right to join the right caudal vena cava. The alternative variation was an incomplete duplication where the left caudal vena cava joined the right infrarenal caudal vena cava at a more cranial point than in normal cases; the renal segment was unchanged. Iliac anastomosis was not found in any cases. Duplicated caudal vena cava neither affected the body weight nor the kidney weight. In conclusion, Slc:Hartley guinea pigs frequently possess asymptomatic duplicated caudal vena cava in the absence of iliac anastomosis and appear to be a novel and useful animal model for duplicated caudal vena cava in animals and humans.

Analysis and Prediction of Collateral Channels from Left Superior Vena Cava after Bilateral Bidirectional Cavopulmonary Anastomosis

Objectives: To analyze and predict collateral channels in bilateral bidirectional cavopulmonary anastomosisfocused on that from left superior vena cava. Methods: 41 consecutive patients who all underwent bilateral bidirectional cavopulmonary anastomosis were enrolled the study. The number, shape, size, origin and entry, and course of collateral vessels from left superior vena cava were referred for evaluation based on 41 cardiac angiographies. The influential factors on the formation of collateral vessels were identified by binary logistic regression analysis. Results: The number of the left superior vena cava with collateral vessels was larger than right one, 22 versus 8 (X2 = 10.303, P = 0.001). The medium-distal parts of left systemic vena were prone to development of collateral channel. The logic function with respect to the probability of left systemic venous collateral channel could be expressed as the linear expression of left systemic venous pressure x: 0.418x ?7.111 (X2 = 23.095, P Setting 17.0 mmHg of left systemic venous pressure as prediction cut-point to discriminate the occurrence of left collateral vessels, the predictive accuracy reached 85.7%. Conclusions: The left systemic venous pressure was an independent factor associated with left systemic venous collateral channel after bilateral bidirectional cavopulmonary shunt. It might be practically feasible to predict the development of collateral vessels according to systemic venous pressure.

Influence of persistent left cranial vena cava on mitral valve repair in two dogs

Influence of persistent left cranial vena cava on mitral valve repair in two dogs

Neurological Symptoms as a Showcase of the Inborn Anomaly in a Development of the Arterial and Venous Vessels (Case Report)

Inborn specific anomalies in the development of the cardio-vascular system require strong attention from physicians of many specialties, especially during diagnosing process. The results of all available diagnostic methods of patient examination determine the further treatment tactic. This tactic implying an individual approach to a specific pathology. There are some cases of combined defects of both, the arterial as well as the venous systems, which appealing special interest. This article describes case report of such anomalies in the structure of the vascular system. This case shown in the form of congenital inflection of the aortic arch with true coarctation and the persistent left vena cava superior.

Persistent left cranial vena cava with congenital heart defect in two dogs

Persistent left cranial vena cava with congenital heart defect in two dogs

Ultrasonographic features of the persistence of superior left vena cava and pathological cardiac associations in fetus. Case series.

The persistence of superior left vena cava (PLSVC) is a pathological condition in fetus with risk of association with abnormalities like heterotaxy, cardiac abnormalities - atrioventricular septum defect, and conotruncal anomalies. In this paper we report 23 cases of fetuses with PLSVCs, reviewing their diagnosis, co-morbidities, and evolution in the newborns.

Imaging characteristics of persistent left cranial vena cava incidentally diagnosed with computed tomography in dogs.

Persistent left cranial vena cava (PLCVC) is an uncommon congenital thoracic venous anomaly in dogs. This study examines the clinical and CT findings of dogs diagnosed with PLCVC incidentally. In this study, complete type of PLCVC was diagnosed in 26 dogs with CT angiography. Shih tzu (17 cases) and Pekingese dogs (3 cases) were overrepresented. There was no gender predisposition, and the average age at presentation was 10.3 years. Of 26 dogs, one dog had a bridging vein connecting right and left cranial vena cavae, and another dog showed azygos vein terminating PLCVC. On the thoracic CT images in the third dog, the right cranial vena cava was absent so that right brachiocephalic vein ended to PLCVC. However, the right costocervical vein drained another vein coursing caudally to the right atrium with azygos vein. In conclusion, CT angiography is a very useful method to diagnose PLCVC and variations of related thoracic vein anomalies in dogs.

Dilated coronary sinus due to persistent left superior vena cava in a healthy athlete: A case report with brief review

The left superior vena cava is a persistent embryo-genetic variant (PLSVC), in which the venous blood of the upper half of the body is drained through a left vena cava into the coronary sinus and/or through the atrio-ventricular groove in the right atrium. PLSVC is an uncommon but clinically relevant venous congenital abnormality which is encountered by non-invasive cardiovascular imaging. Its recognition is important when an enlarged coronary sinus occurs and consequently a differential diagnosis must be made. PLSVC should always be suspected in presence of a dilated coronary sinus, incidentally discovered by echocardiography. Here the author describes the incidental discovery of a dilated coronary sinus due to persistent left superior vena cava in a healthy 46-year-old male athlete during sports pre-participation screening, and discuss the importance and usefulness of the combination of non-invasive cardiovascular examinations, i.e. echocardiography and multi-slice computed tomography, to make an optimal diagnosis and illustration of congenital cardiovascular anomalies.

Advances of Echocardiography in Equines Practice - A Review

Echocardiography has become routinely procedure used in the diagnosis, management, and follow-up of patients with any suspected or known heart diseases. It is one of the most widely used diagnostic modality in cardiology. It can provide a wealth of helpful information, including the size and shape of the heart, pumping capacity, and the location and extent of any tissue damage of heart. An Echocardiogram on other hand gives physician’s estimates of heart function such as a calculation of the cardiac output, ejection fraction, and diastolic function. It also helps to evaluate cardiac defects such as, atrial septal defects, AV valve stenoses, coronary artery defects, occult DCM, coronary artery disease, feline heartworm disease, persistent left cranial vena cava, canine hypertrophic cardiomyopathy and feline diastolic dysfunction may be identified. This Review details principle, uses, technique, types and limitations of Echocardiography in equines.

Primary cranial mediastinal hemangiosarcoma in a young dog.

Primary cranial mediastinal hemangiosarcomas are uncommon tumors. A 30-kg, 2-year-old, intact female German shepherd was presented for evaluation of cachexia and respiratory distress of a few days’ duration. Lateral radiographic projection of the thorax revealed significant pleural effusion. Computed tomography revealed a cranial mediastinal mass effect adjacent to the heart. On surgical exploration, a pedunculated mass attached to the esophagus, trachea, brachiocephalic trunk, left subclavian artery and cranial vena cava without attachment to the right atrium and auricular appendage was removed and debrided by use of blunt dissection and dry gauzes, respectively. Histopathology results described the cranial mediastinal mass as hemangiosarcoma. At 8 months and 5 days post-operatively, the patient died. Primary cranial mediastinal hemangiosarcomas, although a seemingly rare cause of thoracic pathology in young dogs, should be considered in the differential diagnosis for pleural effusion and soft tissue mass effect in the cranial mediastinum. This is the first case report in a dog to describe primary cranial mediastinal hemangiosarcoma.

Whole-exome resequencing reveals recessive mutations in TRAP1 in individuals with CAKUT and VACTERL association

Congenital abnormalities of the kidney and urinary tract (CAKUT) account for approximately half of children with chronic kidney disease and they are the most frequent cause of end-stage renal disease in children in the US. However, its genetic etiology remains mostly elusive. VACTERL association is a rare disorder that involves congenital abnormalities in multiple organs including the kidney and urinary tract in up to 60% of the cases. By homozygosity mapping and whole exome resequencing combined with high-throughput mutation analysis by array-based multiplex PCR and next-generation sequencing, we identified recessive mutations in the gene TNF receptor-associated protein 1 (TRAP1) in two families with isolated CAKUT and three families with VACTERL association. TRAP1 is a heat shock protein 90-related mitochondrial chaperone possibly involved in antiapoptotic and endoplasmic reticulum-stress signaling. Trap1 is expressed in renal epithelia of developing mouse kidney E13.5 and in the kidney of adult rats, most prominently in proximal tubules and in thick medullary ascending limbs of Henle’s loop. Thus, we identified mutations in TRAP1 as highly likely causing CAKUT or CAKUT in VACTERL association.

Persistent left cranial vena cava draining into the left atrium associated with pulmonary stenosis in a French bulldog

A 5-month-old female French bulldog was evaluated for the presence of a heart murmur. Through clinical and echocardiographic evaluations, a severe Type A pulmonary stenosis was diagnosed. Angiography during right ventricular catheterization for valvuloplasty revealed drainage from a persistent left cranial vena cava (PLCVC) into the left atrium; this was confirmed later by contrast echocardiography. This report is the first to describe this anatomical variant of a PLCVC in a dog.