Drenaje venoso anómalo. Vena cava superior izquierda persistente que drena en la aurícula derecha y vena cava superior derecha que drena en la aurícula izquierda: reporte de un caso / Anomalous Venous Drainage. Persistent Left Superior Vena Cava Draining into the Right Atrium and Right Superior Vena Cava Draining into the Left Atrium: Case Report

Introducción: Entre 0,3-0,5% de niños nacidos presentan una vena cava superior izquierda persistente, lo que lo hace una de las malformaciones congénitas más frecuentes del drenaje venoso. El drenaje de esta en la aurícula derecha, además del drenaje de la vena cava superior derecha en la aurícula izquierda, es extremadamente infrecuente. Caso Clínico: Se presenta el caso de un infante de 8 meses asintomático que es llevado a consulta por presentar desaturaciones. El examen físico es normal. El ecocardiograma de contraste con solución salina muestra una vena cava superior izquierda persistente que drena en la aurícula derecha y un retorno anómalo de la vena cava superior derecha. Se realiza una corrección quirúrgica y evoluciona de forma favorable. Conclusión: La presentación en simultáneo de una vena cava superior izquierda persistente que drena en la aurícula derecha y una vena cava derecha que drena en la aurícula izquierda es extremadamente rara. La mayoría de los casos registrados se diagnosticaron de manera incidental en personas adultas al no presentar síntomas.

Introduction: Between 0.3-0.5% of children born have a persistent left superior vena cava, which makes it one of the most frequent congenital malformations of venous drainage. Drainage of this vein into the right atrium, in addition to drainage of the right superior vena cava into the left atrium, is extremely rare. Clinical case: We present a case of an asymptomatic 8-month-old infant who was taken to the clinic for desaturations. Physical examination was normal. The contrast echocardiogram with saline solution showed a persistent left superior vena cava draining into the right atrium and an anomalous return of the right superior vena cava. Surgical correction was performed and the patient evolved favorably. Conclusion: The simultaneous presentation of a persistent left superior vena cava draining into the right atrium and a right superior vena cava draining into the left atrium is extremely rare. Most of the reported cases were diagnosed incidentally in adults in the absence of symptoms.

Coração em Criss-Cross: um relato de caso / Criss-Cross Heart: a case report

Coração em criss-cross (ou coração entrecruzado) foi descrito pela primeira vez em 1974. Trata-se de uma malformação cardíaca congênita, rara, ocorrendo 8 casos a cada 1.000.000 de crianças, e representando somente 0,1% das malformações congênitas. Os métodos diagnósticos de escolha são o ecocardiograma transtorácico, a ressonância magnética cardíaca (RMC), a angiotomografia (TC) e, eventualmente, o cateterismo cardíaco. Neste relato, descreve-se o caso de um recém-nascido com coração em criss-cross somado à dupla via de saída do ventrículo direito (VD), com vasos mal posicionados, além de comunicação interatrial (CIA), comunicação interventricular (CIV), displasia de valva tricúspide e veia cava superior esquerda persistente. Não se sabe a etiologia exata dessa malformação, mas parece ocorrer pela rotação dos ventrículos em seu eixo longitudinal, não acompanhada das rotações atrial e das valvas atrioventriculares (AV). Esse movimento produz uma alteração das vias de entrada dos ventrículos, determinando que o VD se posicione em plano superior e o esquerdo em plano inferior. Apesar de ainda não se saber a exata causa dessa anomalia, acredita-se que uma alteração genética possa estar levando a esses casos: a mutação do gene Cx43. O diagnóstico do caso em questão foi dado pela ecocardiografia transtorácica e da TC de aorta e artérias pulmonares, que mostraram, além do criss-cross, outras alterações, como dupla via de saída do VD, CIA e CIV amplas.(AU)

Criss-cross heart was first described in 1974. It is a rare congenital heart malformation that occurs in 8 cases per 1,000,000 children, and represents only 0.1% of congenital malformations. The diagnostic methods of choice are transthoracic echocardiography, cardiac magnetic resonance (CMR), computed tomography angiography (CT) and, sometimes, cardiac catheterization. This report describes the case of a newborn with a criss-cross heart in addition to double-outlet right ventricle (RV), with poorly positioned vessels, in addition to atrial septal defect (ASD), interventricular septal defect, tricuspid valve dysplasia and persistent left superior vena cava. The exact etiology of this malformation is not known, but it seems to occur due to rotation of the ventricles in their longitudinal axis, not accompanied by rotation of the atrial and atrioventricular (AV) valves. This movement produces abnormal ventricular inlets, determining that the RV be positioned on a superior plane and the left ventricle on an inferior plane. Although the exact cause of this anomaly is still unknown, it is believed that a genetic abnormality may be leading to these cases: mutation of the Cx43 gene. Diagnosis of the case concerned was given by transthoracic echocardiography and computed CT of the aorta and pulmonary arteries, which showed, in addition to the criss-cross heart, other abnormalities, such as double-outlet RV, large ASD and ventricular septal defect (VSD).(AU)

The combination of coronary sinus ostial atresia/abnormalities and a small persistent left superior vena cava-Opportunity for left ventricular lead implantation and unrecognized source of thromboembolic stroke.

BACKGROUND: Coronary sinus (CS) ostial atresia/abnormalities prevent access to the CS from the right atrium (RA) for left ventricular (LV) lead implantation. Some patients with CS ostial abnormalities also have a small persistent left superior vena cava (sPLSVC). OBJECTIVE: The purpose of this study was to describe CS ostial abnormalities and sPLSVC as an opportunity for LV lead implantation and unrecognized source of stroke. METHODS: Twenty patients with CS ostial abnormalities and sPLSVC were identified. Clinical information, imaging methods, LV lead implantation techniques, and complications were summarized. RESULTS: Forty percent had at least 1 previously unsuccessful LV lead placement. In 70%, sPLSVC was identified by catheter manipulation and contrast injection in the left brachiocephalic vein, and in 30% by levophase CS venography. In 30%, sPLSVC was associated with drainage from the CS into the left atrium (LA). When associated with CS ostial abnormalities, the sPLSVC diameter averaged 5.6 ± 3 mm. sPLSVC was used for successful LV lead implantation in 90% of cases. In 80%, the LV lead was implanted down sPLSVC, and in 20%, sPLSVC was used to access the CS from the RA. Presumably because of unrecognized drainage from the CS to the LA, 1 patient had a stroke during implantation via sPLSVC. CONCLUSION: When CS ostial abnormalities prevent access to the CS from the RA, sPLSVC can be used to successfully implant LV leads. In some, the CS partially drains into the LA and stroke can occur spontaneously or during lead intervention. It is important to distinguish sPLSVC associated with CS ostial abnormalities from isolated PLSVC.

Prenatal diagnosis of persistent left superior vena cava, polyhydramnios and a small gastric bubble in a fetus with VACTERL association.

OBJECTIVE: We reported a fetus that presenting with persistent left superior vena cava (PLSVC), polyhydramnios, and a small gastric bubble during prenatal examination and identified VACTERL association after birth. CASE REPORT: A 34-year-old woman underwent amniocentesis at 18 weeks of gestation because of advanced maternal age and the result was normal. Subsequently, an ultrasound revealed single umbilical artery (SUA) at 21 weeks of gestation. She received a detailed fetal anatomy survey that presented the same findings and PLSVC. A small visible gastric bubble was noted at that time, and the other organs were unremarkable. Polyhydramnios was identified at 30 weeks of gestation and amnioreduction was subsequently performed at 32 weeks of gestation. However, polyhydramnios was persisted despite amnioreduction and intrauterine growth restriction was also detected. A cesarean section was performed because of fetal distress at 36 + 2 weeks, and a 1832-g female baby was delivered. Pre-axial polydactyly at left thumb, SUA and esophageal atresia with distal tracheoesophageal fistula (TEF) were identified after birth. The neonate died at age of 4 days because of surgical complication following esophageal anastomosis. CONCLUSION: Prenatal diagnosis of PLSVC associated with polyhydramnios and a small gastric bubble may indicate esophageal atresia with TEF, and further examination for associated syndromes such as VACTERL association is warranted.

Left upper lung cancer with persistent left superior vena cava and left azygos vein: a case report.

BACKGROUND: With the popularization of thoracoscopic surgery, more and more macrovascular malformations have been reported. Understanding some vascular malformations with relatively fixed anatomical site and their range of drainage could avoid severe complications during the surgery. Persistent left superior vena cava (PLSVC) is a common thoracic vascular malformation, and is always combined with other cardiovascular dysplasia. As for the patient with upper left lung cancer in this case, he had PLSVC and left azygos vein, and non-metastatic enlargement of the lymph nodes at the same time, which had influenced the decisions on surgery and treatment. We made a summary of experience regarding this. CASE PRESENTATION: A 46-years-old male patient, his CT found a space-occupying lesion in the superior lobe of the left lung. The chest CT showed that the patient had PLSVC and left azygos vein, and multiple enlarged lymph nodes in the mediastinum. The patient received thoracoscopic upper left lung lobectomy and lymph node dissection. It was discovered that the left azygos vein had a concealed form, which influenced the lymph node dissection. The post-surgery pathology showed that there was squamous cell carcinoma in the upper left lung (pT2bN0M0 p Phase IIA) and no cancer metastasis with the lymph nodes. The patient had a good post-surgery recovery. CONCLUSIONS: PLSVC is not rare, and is always combined with other vascular malformations. If discovering PLSVC before surgery, we suggest completing chest enhanced CT and vascular reconstruction, to find out other cardiovascular malformations that may exist. Left azygos vein is a rare vascular malformation, but it has a relatively fixed anatomical site, and always co-exists with PLSVC, therefore, understanding anatomy of left azygos vein is good for preventing accidental damage. Especially when performing surgery above the left pulmonary artery trunk, attention shall be paid to preventing damage to the left azygos vein. In addition, as for the patient with the diagnosis of lung cancer before surgery, it is not reliable to judge whether there is metastasis or not merely according to the size of the lymph nodes, instead, PET-CT or needle biopsy is recommended.

Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot-Four Cases of a Rare Association.

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.

A Case of Persistent Left Superior Vena Cava and Left Pulmonary Venous Drainage to the Coronary Sinus.

We report a case of a persistent left superior vena cava draining to the right atrium via the coronary sinus in conjunction with partial anomalous pulmonary venous return of the left pulmonary veins to the coronary sinus. Although a persistent left superior vena cava is typically of little clinical consequence, in this case, it complicated surgical repair of the congenital heart disease. Successful repair of this unusual combination of systemic and pulmonary venous anomalies required a combination of two well-described surgical techniques.

Partially Anomalous Pulmonary Venous Connection to Solitary and Persistent Left Superior Caval Vein in Usual Visceroatrial Arrangement.

Although the occurrence of bilateral superior caval veins (SCVs) is not unusual, persistence of the left SCV with atretic right SCV is extremely uncommon in the setting of normal visceroatrial arrangement. We report such a case that was also associated with anomalous pulmonary venous connection of the right pulmonary veins to the solitary left SCV.