Diagnóstico prenatal de dos casos de anomalías congénitas del desarrollo venoso sistémico: una entidad muy poco frecuente (España) / Prenatal diagnosis of two cases of congenital systemic venus return anomalies: a rare entity (Spain) / Diagnóstico pré-natal de dois casos de anormalidades congênitas do desenvolvimento venoso sistêmico: uma entidade muito rara (Espanha)

Introducción: las anomalías en el desarrollo del sistema venoso sistémico son una entidad poco frecuente, cuyo diagnóstico ecocardiográfico prenatal y posnatal puede suponer todo un reto. Por un lado, debido a su baja incidencia y por otro, a la dificultad en la correcta realización de los planos ecocardiográficos. No obstante, su diagnóstico es de vital importancia debido a la asociación con cardiopatías congénitas o cromosopatías. Objetivo: describir dos casos de una anomalía congénita cardiovascular poco frecuente. La persistencia de la vena cava superior izquierda con agenesia de la vena cava superior derecha es una de estas anomalías descritas cuya incidencia es muy baja cuando ambas variaciones se presentan conjuntamente. Casos clínicos: presentamos dos casos de recién nacidos sin antecedentes personales o familiares de interés, diagnosticados prenatalmente, cuyos hallazgos ecocardiográficos se confirman en el período posnatal. Conclusiones: ante el hallazgo aislado en el período fetal de esta variación anatómica que asocia dos anomalías del sistema venoso sistémico, cabe destacar la importancia de su confirmación ecocardiográfica posnatal para descartar cardiopatías congénitas de difícil diagnóstico durante la época prenatal. Así mismo, antes de la confirmación ecocardiográfica que será llevada a cabo por el cardiológico infantil, cabe destacar la importancia del pediatra en la primera exploración física y en la anamnesis a la familia para descartar posibles cardiopatías congénitas críticas o posibles síndromes asociados. El diagnóstico prenatal de persistencia de la vena cava superior izquierda con agenesia de la vena cava superior derecha le permitirá tener un alto grado de sospecha de estas patologías asociadas y por tanto llevar a cabo una actuación clínica precoz.

Introduction: anomalies in the development of the systemic venous system are a rare entity, and its prenatal and postnatal echocardiographic diagnosis can be challenging, due to its low incidence as well as to the difficulty in correctly performing echocardiographic imaging planes. However, its diagnosis is key because it is linked to congenital heart disease or chromosomal anomalies. Objective: describe two cases of a rare cardiovascular congenital anomaly. The persistence of the left superior vena cava with agenesis of the right superior vena cava is one of these described anomalies, with very low incidence when both variations occur together. Clinical cases: we present two cases of newborns with no relevant personal or family history, diagnosed prenatally with confirmed echocardiographic findings in the postnatal period. Conclusions: given the isolated finding in the fetal period of this anatomical variation that associates two anomalies of the systemic venous system, we should note the importance of its postnatal echocardiographic confirmation to rule out congenital heart disease that is difficult to diagnose during the prenatal period. Likewise, prior to the echocardiographic confirmation carried out by the pediatric cardiologist, we should stress the importance of the pediatrician diagnosis in the first physical examination and of the family history to rule out possible critical congenital heart disease or possible associated syndromes. The prenatal diagnosis of VCSIP with agenesis of the VCSD will lead to a high degree of suspicion of these associated pathologies and therefore may lead to early clinical action.

Introdução: as anomalias no desenvolvimento do sistema venoso sistêmico são uma entidade rara, cujo diagnóstico ecocardiográfico pré-natal e pós-natal pode ser um grande desafio. Por um lado, pela sua baixa incidência e, por outro, pela dificuldade em realizar corretamente os planos ecocardiográficos. No entanto, seu diagnóstico é vital devido à associação com cardiopatias congênitas ou anormalidades cromossômicas. Objetivo: descrever dois casos de rara anomalia congênita cardiovascular. A persistência da veia cava superior esquerda com agenesia da veia cava superior direita é uma dessas anomalias descritas cuja incidência é muito baixa quando ambas as variações ocorrem juntas. Casos clínicos: apresentamos dois casos de recém-nascidos sem antecedentes pessoais ou familiares significativos, diagnosticados no pré-natal e cujos achados ecocardiográficos foram confirmados no período pós-natal. Conclusões: dado o achado isolado no período fetal desta variação anatômica que associa duas anomalias do sistema venoso sistêmico, devemos ressaltar a importância de sua confirmação ecocardiográfica pós-natal para descartar cardiopatia congênita de difícil diagnóstico no pré-natal . Da mesma forma, antes da confirmação ecocardiográfica que será realizada pelo cardiologista pediátrico, ressaltamos a importância do pediatra no primeiro exame físico e na história familiar para afastar possíveis cardiopatias congênitas críticas ou possíveis síndromes associadas. O diagnóstico pré-natal de VCSIP com agenesia do VCSD permitirá ter um alto grau de suspeita dessas patologias associadas e, portanto, realizar uma ação clínica precoce.

The haemodynamic effects of umbilical cord milking in term infants: a randomised controlled trial

INTRODUCTION@#Umbilical cord milking (UCM) is a method that allows for postnatal placental transfusion. The benefits of UCM have been demonstrated in some studies, but knowledge about its haemodynamic effects in term infants is limited. The aim of this study was to evaluate the haemodynamic effects of UCM in term infants.@*METHODS@#In this prospective, randomised controlled study, 149 healthy term infants with a birth week of ≥37 weeks were randomly assigned to either the UCM or immediate cord clamping (ICC) group. Blinded echocardiographic evaluations were performed in all the neonates in the first 2-6 h.@*RESULTS@#Superior vena cava (SVC) flow measurements were higher in the UCM group compared to the ICC group (132.47 ± 37.0 vs. 126.62 ± 34.3 mL/kg/min), but this difference was not statistically significant. Left atrial diameter (12.23 ± 1.99 vs. 11.43 ± 1.78 mm) and left atrium-to-aorta diastolic diameter ratio (1.62 ± 0.24 vs. 1.51 ± 0.22) were significantly higher in the UCM group. There were no significant differences in other echocardiographic parameters between the two groups.@*CONCLUSION@#We found no significant difference in the SVC flow measurements in term infants who underwent UCM versus those who underwent ICC. This lack of significant difference in SVC flow may be explained by the mature cerebral autoregulation mechanism in term neonates.

Genetic analysis of a fetus with de novo 46,X,der(X)t(X;Y)(q26;q11) / 中华医学遗传学杂志

OBJECTIVE@#To carry out prenatal genetic testing for a fetus with de novo 46,X,der(X)t(X;Y)(q26;q11).@*METHODS@#A pregnant woman who had visited the Birth Health Clinic of Lianyungang Maternal and Child Health Care Hospital on May 22, 2021 was selected as the study subject. Clinical data of the woman was collected. Peripheral blood samples of the woman and her husband and umbilical cord blood of the fetus were collected and subjected to conventional G-banded chromosomal karyotyping analysis. Fetal DNA was also extracted from amniotic fluid sample and subjected to chromosomal microarray analysis (CMA).@*RESULTS@#For the pregnant women, ultrasonography at 25th gestational week had revealed permanent left superior vena cava and mild mitral and tricuspid regurgitation. G-banded karyotyping analysis showed that the pter-q11 segment of the fetal Y chromosome was connected to the Xq26 of the X chromosome, suggesting a Xq-Yq reciprocal translocation. No obvious chromosomal abnormality was found in the pregnant woman and her husband. The CMA results showed that there was approximately 21 Mb loss of heterozygosity at the end of the long arm of the fetal X chromosome [arr [hg19] Xq26.3q28(133912218_154941869)×1], and 42 Mb duplication at the end of the long arm of the Y chromosome [arr [hg19] Yq11.221qter(17405918_59032809)×1]. Combined with the search results of DGV, OMIM, DECIPHER, ClinGen and PubMed databases, and based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the deletion of arr[hg19] Xq26.3q28(133912218_154941869)×1 region was rated as pathogenic, and the duplication of arr[hg19] Yq11.221qter(17405918_59032809)×1 region was rated as variant of uncertain significance.@*CONCLUSION@#The Xq-Yq reciprocal translocation probably underlay the ultrasonographic anomalies in this fetus, and may lead to premature ovarian insufficiency and developmental delay after birth. Combined G-banded karyotyping analysis and CMA can determine the type and origin of fetal chromosomal structural abnormalities as well as distinguish balanced and unbalanced translocations, which has important reference value for the ongoing pregnancy.

Persistent left superior vena cava as an incidental finding in the introduction of a transient pacemaker: A case report / Vena cava superior izquierda persistente como hallazgo incidental en la introducción de un marcapasos transitorio: informe de un caso

The persistent left superior vena cava is the most common venous anomaly in the systemic drainage in adults and tends to be asymptomatic. The persistent left superior vena cava causes rhythm disorders such as tachyarrhythmias or bradyarrhythmias. We report a case of persistent left superior vena cava diagnosed in a 53-year-old female patient admitted due to an acute coronary syndrome associated with unstable bradycardia. A transvenous peacemaker impressed the left atrium; therefore, a transthoracic echocardiogram was required to diagnose persistent left superior vena cava. The patient needed management with percutaneous intervention; she had an adequate evolution and subsequent discharge from the intensive care unit

La vena cava superior izquierda persistente es la anomalía venosa más frecuente en el drenaje sistémico en adultos; tiende a ser asintomática, pero causa trastornos del ritmo como taquiarritmias o bradiarritmias. Se presenta un caso de vena cava superior izquierda persistente diagnosticada en una paciente de 53 años hospitalizada por un síndrome coronario agudo asociado a bradicardia inestable. Un marcapasos transvenoso dejó una impresión en la aurícula izquierda, por lo que se requirió un ecocardiograma transtorácico para diagnosticar la vena cava superior izquierda persistente. La paciente necesitó una intervención percutánea con una adecuada evolución y se le dio el alta de la unidad de cuidados intensivos.

Abordaje quirúrgico del síndrome de vena cava superior asociado a neoplasia maligna: revisión narrativa / Surgical approach to superior vena cava syndrome associated with malignant neoplasm: A narrative review

Introducción: El síndrome de vena cava superior (SVCS) es una patología poco frecuente, asociado en la mayoría de los casos a neoplasias de origen maligno; la cual en estadios avanzados representa una urgencia médico-oncológica que compromete la vida del paciente. Propósito de la revisión: El objetivo de la revisión es delinear el rol de las diferentes alternativas quirúrgicas y percutáneas para el tratamiento del SVCS. Buscamos reportes en donde se incluye los resultados de mejor supervivencia para los distintos tratamientos actuales. Recientes hallazgos: La literatura médica describe tratamientos como la radioterapia, la quimioterapia, el bypass, la terapia endovascular y la reconstrucción vascular para el manejo del SVCS, sin embargo, no todos responden con la misma eficacia al momento de presentarse una urgencia vital por SVCS; esto producto de los factores intrínsecos y extrínsecos del paciente. Dentro de los factores extrínsecos destaca la condición social la cual, se convierte en un reto al momento de realizar un manejo integral del paciente en ciudades fronterizas, donde los datos que conoce el médico sobre el paciente son limitados o el paciente no tiene un diagnóstico previo que permita orientar el manejo. Conclusiones: El punto de partida del tratamiento del paciente con SVCS consiste en diferenciar la emergencia y la estabilidad mediante la tabla de clasificación de gravedad. En casos emergentes se tiene 2 alternativas: la terapia endovascular y la radioterapia. El abordaje quirúrgico con Baypass está contraindicado y el tratamiento definitivo con reconstrucción vascular con prótesis tiene ventajas y desventajas que deben definirse en forma individual considerando la etiología del cáncer asociado.

Introduction: Superior vena cava syndrome (SVCS) is a rare pathology, associated in most cases with neoplasms of malignant origin; which in advanced stages represents a medical-oncological emergency that compromises the patient's life. Purpose of the review: The objective of the review is to outline the role of the different surgi-cal and percutaneous alternatives for the treatment of SVCS. We look for reports that include the best survival results for the different current treatments. Recent findings: The medical literature describes treatments such as radiotherapy, chemo-therapy, bypass, endovascular therapy and vascular reconstruction for the management of SVCS, however, not all respond with the same efficacy at the time of a vital emergency. by SVCS; this product of the intrinsic and extrinsic factors of the patient. Among the extrinsic fac-tors, the social condition stands out, which becomes a challenge when carrying out an integral management of the patient in border cities, where the data that the doctor knows about the patient is limited or the patient does not have a previous diagnosis. to guide management. Conclusions: The starting point of the treatment of the patient with SVCS consists in differentiating the emergency and the stability by means of the severity classification table. In emergent cases there are 2 alternatives: endovascular therapy and radiotherapy. The surgical approach with Baypass is contraindicated and the definitive treatment with vascular reconstruc-tion with a prosthesis has advantages and disadvantages that must be defined individually considering the etiology of the associated cancer.

Effects of different routes of heparin on instant blood-mediated inflammatory reaction after portal vein islet transplantation / 中南大学学报(医学版)

OBJECTIVES@#Heparin is mainly used as an anticoagulant in clinic, and it also has a certain anti-inflammatory effect. At present, after portal vein islet transplantation in diabetic patients, heparin is mainly infused through the peripheral veins of the limbs to achieve the purpose of anticoagulation and protection of the graft, rather than through the portal vein. In this study, animal experiments were conducted to investigate the effect of heparin infusion via the portal vein and marginal ear vein on the instant blood-mediated inflammatory reaction (IBMIR) after portal vein islet transplantation, which is the choice of anticoagulation methods for clinical islet transplantation to provide a basis for decision-making.@*METHODS@#A total of 50 neonatal pigs (Xeno-1 type, 3-5 days) were selected. Islets were isolated and purified from the pancreas of neonatal pigs. Ten non-diabetic Landrace pigs (1.5-2.0 months) served as recipients, and 12 000 IEQ/kg neonatal porcine islets were transplanted into the liver through the portal vein. All recipients received bolus injection of 50 U/kg of heparin 10 minutes before transplantation. After the bolus injection of heparin, the experimental group received heparin via the portal vein [10 U/(kg·h), 5 recipients], and the control group received heparin via the marginal ear vein [10 U/(kg·h), 5 recipients]. The superior vena cava blood was collected from the 2 groups pre-operation at 1, 3, 24 h post-operation of the transplantation. The portal vein blood was collected from the experimental group at 1 and 3 h after the transplantation as well. The levels of complement C3a, C5a, thrombin-antithrombin complex (TAT), β-thromboglobulin (β-TG), and D-dimer as well as activated partial thromboplastin time (APTT) in superior vena cava blood from 1 and 3 h post-transplantation were detected in the 2 groups, and the levels of anti-Xa and anti-IIa in the portal vein and superior vena cava blood from 1 and 3 h post-transplantation in the experimental group were detected. Twenty four hours after the transplantation, the liver tissues in the 2 groups were collected for pathological examination to observe the inflammatory cell infiltration and peripheral thrombosis around the islets graft in liver.@*RESULTS@#Before transplantation, there was no statistically significant difference in C3a, C5a, TAT, β-TG, D-dimer levels and APTT between the 2 groups (all P>0.05). At 1 and 3 h after transplantation, the C3a, TAT, and D-dimer levels in the experimental group were significant decreased than those in the control groups (all P<0.05), and at 3 h after transplantation the C5a was significant decreased than that in the control group (P<0.05). At 1 and 3 h after transplantation, the anti-Xa and anti-IIa levels in the portal vein blood were significantly increased than those in the superior vena cava blood in the experimental group (all P<0.05). Pathological results showed the presence of islet cell clusters in the liver blood vessels. The thrombus formation and neutrophil infiltration around islet graft was not obvious in the experimental group, while massive thrombus formation and neutrophil infiltration in the control group.@*CONCLUSIONS@#Compared with marginal ear vein infusion of heparin, the direct infusion of heparin in the portal vein has a certain inhibitory effect on complement system, coagulation system activation and inflammatory cell infiltration in portal vein islet transplantation, which may attenuate the occurrence of IBMIR.

Value of bedside echocardiography in predicting persistent patency of the ductus arteriosus during the early postnatal period in very low birth weight infants / 中国当代儿科杂志

OBJECTIVES@#To study the value of bedside echocardiography in predicting persistent patency of the ductus arteriosus during the early postnatal period in very low birth weight (VLBW) infants.@*METHODS@#A retrospective analysis was performed for 51 VLBW infants who were admitted from March 2020 to June 2021, with an age of ≤3 days and a length of hospital stay of ≥14 days. According to the diameter of patent ductus arteriosus (PDA) on days 14 and 28 after birth, the infants were divided into three groups: large PDA group (PDA diameter ≥2 mm), small PDA group (PDA diameter <2 mm), and PDA closure group (PDA diameter =0 mm). The echocardiographic parameters measured at 72 hours after birth were compared among the three groups. The receiver operating characteristic (ROC) curve was used to evaluate the value of the echocardiographic parameters in predicting persistent patency of the ductus arteriosus (PDA≥2 mm) at the ages of 14 and 28 days.@*RESULTS@#On day 14 after birth, there were 17 infants in the large PDA group, 11 in the small PDA group, and 23 in the PDA closure group. On day 28 after birth, there were 14 infants in the large PDA group, 9 in the small PDA group, and 26 in the PDA closure group. There were significant differences in gestational age, birth weight, rate of pulmonary surfactant use, and incidence rate of hypotension among the three groups (P<0.05). PDA diameter, end-diastolic velocity of the left pulmonary artery, left ventricular output, and left ventricular output/superior vena cava flow ratio measured at 72 hours after birth were associated with persistent patency of the ductus arteriosus at the ages of 14 and 28 days (P<0.05), and the ratio of the left atrium to aorta diameter was associated with persistent patency of the ductus arteriosus at the age of 28 days (P<0.05). The ROC curve analysis showed that the area under the curve that the PDA diameter measured at 72 hours after birth predicting the persistent patency of the ductus arteriosus at the ages of 14 and 28 days was the largest (0.841 and 0.927 respectively), followed by end-diastolic velocity of the left pulmonary artery, with the area under the curve of 0.793 and 0.833 respectively.@*CONCLUSIONS@#The indicators obtained by beside echocardiography at 72 hours after birth, especially PDA diameter and end-diastolic velocity of the left pulmonary artery, can predict persistent patency of the ductus arteriosus at the ages of 14 and 28 days in VLBW infants, which provides a basis for the implementation of early targeted treatment strategy for PDA.

Ultrasound-guided percutaneous cannulation for extracorporeal membrane oxygenation in children / 中华儿科杂志

Objective: To evaluate the effectiveness and safety of ultrasound-guided percutaneous cannulation for extracorporeal membrane oxygenation (ECMO) in children. Methods: In this retrospective observational study, 66 cases who underwent non-cardiac surgery ECMO in pediatric intensive care unit (PICU) of Shanghai Children's Hospital from May 2016 to April 2021 were collected. The demographics, model of ECMO support, type and size of arteriovenous cannulas, way of catheterization and complications were recorded and summarized. Patients were divided into percutaneous cannulation group and surgical cannulation group according to catheterization strategies. The demographics, duration of cannulation and ECMO support, ECMO weaning rate and hospital survival rate were compared among two groups. χ2 and nonparametric rank sum test were used for comparison. Results: Among the 66 patients who received ECMO, 38 were male and 28 were female, with age 44.5 (12.0, 83.5) months and weight 15.0 (10.0, 25.0) kg; 21 patients underwent percutaneous cannulation, with a success rate of 95% (20 cases). Point-of-care ultrasound was performed for all percutaneous cannulation cases. The duration of percutaneous cannulation was significantly shorter than that of surgical cannulation (26.0 (23.3, 30.3) vs. 57.0 (53.8, 64.0) min, Z=6.31, P<0.001). Successful percutaneous cannulation cases were aged 70.5 (23.8, 109.5) months, and their weight was 23.2 (13.6, 37.0) kg. Ten cases were initially given veno-venous (VV) ECMO support, and 10 cases were given veno-arterial (VA) ECMO support. ECMO arterial cannulas were sized from 8 F to 17 F, and venous cannulas sized from 10 F to 19 F. For VV-ECMO, the right internal jugular and femoral veins were used as vascular access, while VA-ECMO used right internal jugular vein-femoral artery or right femoral vein-left femoral artery approach. Only one patient suffered severe complication (superior vena cava perforation). There was no catheter-related bloodstream infection. Conclusion: Ultrasound-guided percutaneous cannulation for ECMO can be performed with a high rate of success and safety in children.

Medium and long-term follow-up of the Pul-Stent in treating postoperative branch pulmonary artery stenosis in children with congenital heart disease / 中华儿科杂志

Objective: To evaluate the effectiveness and safety of Pul-Stent as the treatment of postoperative branch pulmonary artery stenosis in children with congenital heart disease. Methods: This was a retrospective study. Thirty-three patients who underwent Pul-Stent implantation in Shanghai Children's Medical Center due to postoperative residual pulmonary artery stenosis from August 2014 to June 2015 were included. The immediate curative effect, follow-up and complications of Pul-Stent implantation were assessed. Comparisons between groups were performed with unpaired Student t test. Results: Pul-Stent implantation of 33 patients (19 males and 14 females) were performed successfully. Thirty-one patients underwent percutaneous stenting, and 2 patients underwent hybrid stenting. A total of 35 Pul-Stents were implanted (19 of model small, 15 of model medium and one of model large), 23 stents were planted in the proximal left pulmonary artery and 12 stents were in the proximal right pulmonary artery. The initial diameter of dilation balloon ranged from 6 to 16 mm, and the long sheath of percutaneous implantation ranged from 8 to 10 F in 29 patients (29/31, 94%). After stenting, the diameter of the narrowest segment of pulmonary artery increased from (4.0±1.7) mm to (9.1±2.1) mm in all patients (t=-21.60, P<0.001). The pressure gradient at the stenosis in 26 patients after biventricular correction decreased from (30.5±12.3) mmHg (1 mmHg=0.133 kPa) to (9.9±9.6) mmHg (t=12.92, P<0.001), and the right ventricular to aortic pressure ratio decreased from 0.57±0.14 to 0.44±0.12 (t=7.44, P<0.001). The pressure of the superior vena cava after stenting in 5 patients after cavopulmonary anastomosis decreased from (17.0±1.9) mmHg to (14.0±0.7) mmHg (t=2.86, P=0.046). Two patients died during reoperation for repairing other cardiac malformations. The remaining 31 patients were clinically stable during the follow-up period of (5.3±1.6) years, and one stent fracture was found on chest X-ray. Cardiac catheterization reexaminations in 16 patients showed that restenosis was found in one stent, while stent position and patency were satisfactory in the remaining stents. Nine children underwent post-dilation without stent fracture, displacement or aneurysm formation. Cardiac tomography showed no stent stenosis, fracture observed, or significant change in diameter of the stent in 8 patients. The inner diameter and pulmonary blood perfusion could not be accurately evaluated due to artifacts by cardiac magnetic resonance imaging in 4 patients. Conclusions: Pul-Stent has good compliance and adequate radial strength, and can dilate further over time to accommodate for somatic growth. It performs safely and effectively in treating post-operative branch pulmonary artery stenosis in children.

Malformación de Dandy-Walker asociada a malformaciones extracraneales en un neonato / Dandy-Walker malformation associated with extracranial abnormalities in a newborn

La hidrocefalia es una condición clínica que consiste en un cúmulo de líquido cefalorraquídeo a nivel encefálico. Una de las causas, poco frecuente, es el síndrome de Dandy-Walker. Se presenta el caso de un recién nacido con diagnóstico prenatal de hidrocefalia secundaria a una malformación de Dandy-Walker y sospecha de genitales ambiguos. Tras el nacimiento, se confirma el diagnóstico prenatal de malformación de Dandy-Walker asociado a manifestaciones extracraneales poco frecuentes como hipospadias interescrotal y dilatación del seno coronario secundario a persistencia de la vena cava superior izquierda. Con este caso clínico queremos exponer la complejidad del síndrome de Dandy-Walker debido a sus múltiples asociaciones, que marcarán el pronóstico del paciente y la necesidad de tratamiento multidisciplinar.

Hydrocephalus is a clinical condition that consists of an accumulation of cerebrospinal fluid around the brain; Dandy-Walker syndrome is a rare cause of it. We present the case of a newborn with prenatal diagnose of hydrocephalus due to a Dandy-Walker malformation, as well as ambiguous genitalia. After birth, diagnosis of Dandy-Walker malformation associated with uncommon extracranial manifestations is confirmed. Specifically, the baby presents interscrotal hypospadias and coronary sinus dilatation due to the persistence of the left superior vena cava. With the exposition of this case, we bring out the complexity of the Dandy-Walker syndrome due to the malformations associated with it; the ones that will determine the prognosis and the need of a multidisciplinary treatment

Heterotopic heart transplantation as a left ventricular biological assistance: a new two-stage method proposal

Abstract Since Barnard's first heterotopic heart transplant in 1974, Copeland's method has been the greatest contribution to heterotopic transplants but has the drawback of donor's right ventricular atrophy. This new method proposes a modification in the anastomosis of the superior vena cava aiming to pre-serve donor's right ventricular function by decompressing the pulmonary territory and reducing the pulmonary arterial pressure, as a biological ventricular assist device. Finally, a second intervention is proposed, where a "twist" is performed to place the donor's heart in an orthotopic position after re-moval of the native heart. A pioneering research on this method received approval from the ethics committee of the Heart Institute of São Paulo. We believe that this method has the potential to im-prove quality of life in a selected group of patients.

The new surface landmarks for blind axillary vein puncture

Abstract Objective: To compare the efficacy of blind axillary vein puncture utilizing the new surface landmarks for the subclavian method. Methods: This prospective and randomized study was performed at two cardiology medical centers in East China. Five hundred thirty-eight patients indicated to undergo left-sided pacemaker or implantable cardioverter defibrillator implantation were enrolled, 272 patients under the axillary access and 266 patients under the subclavian approach. A new superficial landmark was used for the axillary venous approach, whereas conventional landmarks were used for the subclavian venous approach. We measured lead placement time and X-ray time from vein puncture until all leads were placed in superior vena cava. Meanwhile, the rate of success of lead placement and the type and incidence of complications were compared between the two groups. Results: There were no significant differences between the two groups in baseline characteristics or number of leads implanted. There were high success rates for both strategies (98.6% [494/501] vs. 98.4% [479/487], P=0.752) and similar complication rates (14% [38/272] vs. 15% [40/266], P=0.702). Six cases in the control group developed subclavian venous crush syndrome and five had pneumothorax, while neither pneumothorax nor subclavian venous crush syndrome was observed in the experimental group. Conclusion: We have developed a new blind approach to cannulate the axillary vein, which is as effective as the subclavian access, safer than that, and also allows to get this vein without the guidance of fluoroscopy, contrast, or echography.

Diagnóstico prenatal de la vena cava superior izquierda persistente / Prenatal diagnosis of persistent left superior vena cava

RESUMEN Introducción: La persistencia de la vena cava superior izquierda (VCSIP) es la anomalía más común del sistema venoso torácico y puede estar asociadas a otras malformaciones cardiacas. Objetivo: Describir las características ecocardiográficas de la VCSIP y su relación con los factores de riesgo y anomalías asociadas. Método: Se realizó un estudio descriptivo, longitudinal y retrospectivo, con el propósito de contribuir al estudio del diagnóstico prenatal de la VCSIP mediante el análisis de diferentes indicadores. La muestra estuvo conformada por 62 casos con diagnóstico prenatal de VCSIP, en fetos entre 22 y 35 semanas, examinados en el Servicio de Ecocardiografía Fetal del Cardiocentro Pediátrico William Soler, durante el período comprendido desde enero de 2008 hasta mayo de 2012. Resultados: La VCSIP se presentó asociada a cardiopatías congénitas en 33 de los 62 casos. Dentro de estas, las más frecuentes fueron las anomalías troncoconales (38,46%). Conclusiones: La VCSIP es de fácil reconocimiento mediante el ultrasonido de pesquisa y constituye un hallazgo importante para el diagnóstico de las cardiopatías congénitas; por lo cual, ante su sospecha -debido a su frecuente asociación con cardiopatías congénitas- se sugiere ofrecer a la gestante una evaluación detallada del corazón fetal.

ABSTRACT Introduction: The persistence of the left superior vena cava (PLSVC) is the most common anomaly of the thoracic venous system and may be associated with other cardiac malformations. Objective: To describe the echocardiographic characteristics of PLSVC and its relationship with risk factors and associated abnormalities. Method: A descriptive, longitudinal and retrospective study was carried out, in order to contribute to the study of the prenatal diagnosis of PLSVC through the analysis of different indicators. The sample consisted of 62 cases with a prenatal diagnosis of PLSVC, in fetuses between 22 and 35 weeks, examined in the Fetal Echocardiography Department of the Cardiocentro Pediátrico William Soler, during the period from January 2008 to May 2012. Results: The PLSVC was associated with congenital heart disease in 33 of the 62 cases. Among these, the most frequent were conotruncal heart defects (38.46%). Conclusions: The PLSVC is easily recognized by screening ultrasound and represents an important finding for the diagnosis of congenital heart disease; therefore, given its suspicion -due to its frequent association with congenital heart disease- it is suggested to offer the pregnant woman a detailed evaluation of the fetal heart.

Ablación de reentrada nodal en vena cava superior izquierda persistente y ausencia de vena cava superior derecha / Nodal re-entry in patient with persistent left superior vena cava and absent right superior vena cava

Resumen La taquicardia por reentrada nodal es la arritmia más frecuente entre las taquicardias supraventriculares paroxísticas; la variedad lenta-rápida es la más común. En muchos casos la ablación de la vía lenta es el tratamiento definitivo y puede llegar a tener alta tasa de éxito y baja frecuencia de complicaciones. La presencia de una vena cava superior izquierda y la ausencia de vena cava superior derecha con drenaje venoso sistémico superior al seno coronario es una malformación congénita poco frecuente, la cual genera alteraciones anatómicas que dificultan el abordaje tradicional para la ablación de esta arritmia. Se presenta el caso de una paciente con esta condición en la que fue exitosa la ablación mediante ecocardiografía intracardiaca y reconstrucción tridimensional.

Abstract Nodal re-entrant tachycardia is the most common arrhythmia among the paroxysmal atrioventricular tachycardias, with the slow-fast variant being the most common. In many cases, the ablation of the slow pathway is the definitive treatment in many cases, often with a high rate of success and low frequency of complications. The presence of a left superior vena cava and the absence of a right superior vena cava with systematic venous drainage above the coronary sinus is a rare congenital malformation that leads to anatomical anomalies. These make it difficult to use the traditional approach for the ablation of this arrhythmia. The case is presented of a patient with this condition in which the ablation was successful using intracardiac echocardiography and three-dimensional reconstruction.

Vena cava superior izquierda persistente, agenesia de vena cava superior derecha e insuficiencia tricuspídea / Persistent left superior vena cava, absence of the right superior vena cava and tricuspid regurgitation

Resumen La persistencia de una vena cava superior izquierda en la población general representa una de las malformaciones del retorno venoso torácico más frecuentes, sin embargo su asociación con agenesia de la vena cava superior derecha e insuficiencia tricuspídea son hallazgos extremadamente infrecuentes.

Abstract The persistence of a left superior vena cava in the general population represents one of the most frequent thoracic venous return malformations, however its association with abscence of the right superior vena cava and tricuspid regurgitation are extremely rare findings.

Tratamiento intervencionista con stent para el síndrome de vena cava superior con complicación por el uso de marcapasos en un lactante / Stent interventional treatment of superior vena cava syndrome with a pacemaker placement complication in an infant

Resumen Introducción: El marcapasos epicárdico es el método preferido en recién nacidos y niños que pesan menos de 10 kg. Sin embargo, su colocación cuenta con una alta incidencia de fracaso prematuro. Caso clínico: Se presenta el caso de un lactante que requirió la colocación de un marcapasos por bloqueo atrioventricular congénito diagnosticado prenatalmente. El paciente presentaba estenosis crítica de la vena cava superior como complicación secundaria a los cables del marcapasos. La resolución intervencionista mediante un stent en el sitio de estenosis corrigió el síndrome de vena cava superior de forma efectiva. Conclusiones: Este caso representa una alternativa clínicamente viable en el tratamiento del síndrome de vena cava superior, que puede emplearse después de otras terapias convencionales que hayan fallado.

Abstract Background: Epicardial pacemaker is the preferred method for newborns and children under 10 kg of weight. However, its placement presents a high rate of premature failure. Case report: The case of an infant requiring placement of pacemaker by congenital atrioventricular block with prenatal diagnosis is described. The patient showed critical stenosis of the superior vena cava as a complication. Interventional resolution using a stent at the site of stenosis corrected the superior vena cava syndrome effectively. Conclusions: This case represents a clinically viable alternative for the treatment of superior vena cava syndrome, which can be used after other conventional therapies have failed.

Unroofed coronary sinus syndrome: A case report / 中南大学学报(医学版)

Unroofed coronary sinus syndrome (UCSS), also named coronary sinus septal defect, is a rare type of atrial septal defect with the incidence less than 1% of the total number of atrial septal defects. It is caused by incomplete formation of left atrial venous folds during embryonic development. Here we reported a patient with UCSS, who was treated in the Second Xiangya Hospital of Central South University. The patient was 50 years old and the main clinical manifestations were fatigue and shortness of breath after repeated exercise. Color Doppler echocardiography showed coronary sinus dilatation (17 mm×14 mm), indicating the possibility of permanent left superior vena cava. Pulmonary angiography showed that the left ventricle and coronary sinus were developed at the same time while the atrial septum was intact after the development of the left atrium, followed by the right atrium and right ventricle, indicating a partial anomalous pulmonary venous drainage (intracardiac type). Finally, the cardiac computed tomograhic angiography showed that 4 pulmonary veins and permanent left superior vena cava (PLSVC) went into the left atrium and the coronary sinus, respectively, while the coronary sinus septum was absent and the PLSVC was connected with the left atrium. The patient was later treated with the correction of non-parietal sinus syndrome in the Cardiovascular Surgery Department of our hospital.

Advances in the Application of Peripheral Central Venous Catheter Tip Positioning Technology / 中国医疗器械杂志

The best tip position of PICC is located in the inferior 1/3 of superior vena cava to the junction between superior vena cava and right atrium. Ensuring the best tip position of PICC is very important for the treatment of patients. In this paper, the applications of X-ray positioning, electrocardiograph, ultrasound, electrocardiograph Doppler ultrasound guidance, and electromagnetic navigation system in PICC tip positioning technology are reviewed. The future development of PICC tip positioning technology is prospected.

Utilidad de la ecocardiografía de contraste con solución salina agitada en el diagnóstico de la persistencia de vena cava superior izquierda / Usefulness of contrast echocardiography with agitated saline at diagnosis of persistence of the left superior vena cava

Resumen La ecocardiografía contrastada con solución salina agitada es una modalidad de imagen establecida. Este método es usualmente utilizado para el diagnóstico de foramen oval permeable, shunts intracardiacos, anomalías del drenaje venoso y shunts intrapulmonares. En este artículo se revisarán aspectos generales de la ecocardiografía contrastada con solución salina, así como métodos diagnósticos enfocados principalmente a la detección de la persistencia de vena cava superior izquierda y sus variantes.

Abstract Echocardiography contrasted with agitated saline solution is an established imaging modality. This method is usually used for the diagnosis of patent foramen ovale, intracardiac shunts and venous drainage abnormalities. In this article, general aspects of the echocardiography contrasted with saline solution will be reviewed, as well as diagnostic methods focused mainly on the detection of the persistence of the left superior vena cava and its variants.

Fibrosis mediastínica post-irradiación: un factor a tener en cuenta en cirugía cardiaca / Post-radiation mediastinal fibrosis: a factor to take into account in cardiac surgery

Resumen La radioterapia mediastínica es uno de los tratamientos fundamentales de las enfermedades malignas torácicas, pero también representa una causa potencial de complicaciones, tanto a corto como a largo plazo. Se presenta una serie de casos de rotura de vena innominada durante esternotomía media en pacientes con fibrosis mediastínica. Los tres casos descritos corresponden a pacientes intervenidos de cirugía cardiaca con antecedentes de radioterapia mediastínica, entre 15-30 años antes de la cirugía, por tres diferentes enfermedades malignas (linfoma de Hodgkin, timoma y cáncer de mama). En los tres casos se reporta rotura de la vena innominada, con desinserción de su origen en la vena cava superior debido a fibrosis mediastínica intensa.

Abstract Although mediastinal radiotherapy is one of the basic treatments of malignant thoracic diseases, it is also a potential cause of short and long-term complications. A series of cases of rupture of the innominate vein during sternotomy are presented in patients with mediastinal fibrosis. The three cases described correspond to patients intervened by cardiac surgery, with a history of mediastinal radiotherapy between 15 to 30 years before the surgery, due to three different malignant diseases (Hodgkin lymphoma, thymoma, and breast cancer). In the three cases, a rupture of the innominate vein is reported; with de-insertion of its origin in the superior vena cava sue to intense mediastinal fibrosis.