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.

Vena cava superior duplicada / Duplicate superior vena cava

Paciente de 71 años con antecedente de tuberculosis que ingresa en muy malas condiciones y con francas manifestaciones de insuficiencia cardiaca, arritmia completa por fibrilación auricularcon alta respuesta ventricular (160 Ipm), compromiso de las funciones hepática (Enzimas elevadas) y renal (creatinina elevada). Estudios complementarios demostraron trombos intracavitarios, pero además se detectó la presencia de una vena cava superior izquierda, desembocando en seno coronario, particularmente visible en la Angio - Tomografía Axial Computarizada (con reconstrucción anatómica y confirmada por ecocardiografía). El paciente fue dado de alta en aceptables condiciones.

Veia cava superior esquerda persistente: relato de caso / Persistent left superior vena cava: case report

O pleno conhecimento da anatomia vascular torácica é de suma importância para os profissionais envolvidos na realização de procedimentos invasivos como a punção de acesso venoso central. A persistência da veia cava superior esquerda é a malformação venosa torácica mais frequente, e seu diagnóstico costuma ser incidental. Apresentamos o caso de uma paciente de 14 anos em que o diagnóstico de veia cava superior esquerda persistente foi incidental em exame de imagem de controle após colocação de cateter venoso totalmente implantável. A paciente não apresentou dificuldade de infusão de quimioterapia pelo cateter e não houve complicações relacionadas ao cateter.

It is extremely important that health professionals involved in invasive procedures such as central venous access have a thorough knowledge of thoracic vascular anatomy. Persistent left superior vena cava is the most common congenital thoracic venous abnormality and is generally diagnosed as an incidental finding. We present the case of a 14-year-old female patient in whom a persistent left superior vena cava was diagnosed as an incidental finding of a control imaging exam after placement of a totally implantable venous catheter. The patient did not exhibit any difficulties with infusion of chemotherapy via the catheter and there were no complications related to the catheter.

Ivemark syndrome-a rare entity with specific anatomical features / Síndrome de Ivermark. Informe de un caso

Ivemark syndrome (IS) is a rare embryological disorder which results from failure of development of the left-right asymmetry of organs. It is often associated with cardiac and other organ abnormalities, which are the usual causes of death in early neonatal life. We report a 3 months old girl with IS with dextrocardia, transposition of the great vessels, atrio-ventricular connection, total anomalous pulmonary venous drainage, a right atrial and right pulmonary isomerism, a midline liver, a midline gallbladder, asplenia, intestinal malrotation and vena cava anomalies. To our knowledge, complete right heterotaxia syndrome has been rarely described in literature. Lateralization defects such as situs inversus, asplenia or polysplenia due to defective left-right axis development are considered as defects of the primary developmental field. Therefore, additional malformations in IS can be synchronic defects in the primary developmental field rather than causally independent malformations.

El síndrome de Ivermark es un desorden embriológico raro resultante de una falla en el desarrollo de la asimetría izquierda y derecha de los órganos. Usualmente se asocia con anomalías cardíacas y de otros órganos, que son la causa usual de muerte en la vida neonatal. Presentamos una niña de 3 meses con dextrocardia, trasposición de los grandes vasos, comunicación aurículo-ventricular, drenaje anómalo total de la vena pulmonar, isomerismo de la aurícula y pulmón derecho, hígado y vesícula en la línea media, asplenia, malrotación intestinal y anomalías de la vena cava. Una heterotaxia derecha completa ha sido raramente descrita en la literatura. Los defectos de lateralización como situs inverso, asplenia o poli esplenia causados por defectos en el desarrollo izquierda derecha son considerados como defectos del campo de desarrollo primario. Por lo tanto, las manifestaciones adicionales del síndrome de Ivemark pueden ser defectos sincrónicos del campo de desarrollo primario más que malformaciones causalmente independientes.

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

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

Terapia de resincronización cardiaca en paciente con vena cava superior izquierda persistente: Caso clínico / Placement of a biventricular resynchronization device in a patient with a persistent left superior vena cava: Report of one case

Persistent left superior vena cava is the most common venous congenital malformation and is usually asymptomatic. Its presence could increase the difficulty for transvenous lead implantation. We report a 71-year-old woman with an idiopathic dilated cardiomyopathy, atrial fibrillation and heart failure that required biventri-cular resynchronization therapy. During the placement of the device a persistent left superior vena cava was detected. The device was placed without problems and the patient had a satisfactory postoperative evolution.

Intracardiac Eustachian Valve Cyst in an Adult Detected with Other Cardiac Anomalies: Usefulness of Multidetector CT in Diagnosis

We present an unusual case of an intracardiac Eustachian valve cyst observed concurrently with atresia of the coronary sinus ostium, a persistent left superior vena cava (LSVC) and a bicuspid aortic valve. There have been several echocardiographic reports of Eustachian valve cysts; however, there is no report of multidetector computed tomography (MDCT) findings related to a Eustachian valve cyst. Recently, we observed a Eustachian valve cyst diagnosed on MDCT showing a hypodense cyst at the characteristic location of the Eustachian valve (the junction of the right atrium and inferior vena cava). MDCT also demonstrated additional cardiovascular anomalies including atresia of the coronary sinus ostium and a persistent LSVC and bicuspid aortic valve.

Role of intraoperative echocardiography in surgical correction of the superior sinus venosus atrial septal defect.

Superior type of sinus venosus atrial septal defect (SVASD) is invariably associated with the unroofing of right upper pulmonary vein (RUPV). Warden procedure and pericardial patch repair with rerouting of the RUPV are commonly performed operations for the superior SVASD. Both operations involve the risk of obstruction to the flow of superior vena cava or rerouted pulmonary vein in the postoperative period. The sinus venosus defects are well visualized on the transesophageal echocardiography (TEE) because of the proximity of the TEE probe to these structures. We are reporting two cases operated for the superior SVASD with unroofed RUPV, highlighting the intraoperative echocardiographic features before and after the surgery.

Tratamento cirúrgico da conexão anômala parcial das veias pulmonares em veia cava superior / Surgical treatment of partial anomalous pulmonary venous connection to the superior vena cava

OBJETIVO: O tratamento cirúrgico da conexão anômala das veias pulmonares em veia cava superior, associada ao defeito septal atrial tipo seio venoso, é bem estabelecido e transcorre com baixa mortalidade e morbidade. Com a finalidade de diminuir a incidência de estenose ou oclusão da veia cava superior direita, especialmente quando associada à presença de veia cava superior esquerda, o apêndice atrial direito foi utilizado para ampliar a veia cava superior direita, após o desvio das veias pulmonares para o átrio esquerdo. MÉTODOS: No período entre junho de 1986 e setembro de 2008, foram operados 95 pacientes, consecutivos, portadores desta anomalia com drenagem em veia cava superior direita e porção alta do átrio direito. A idade variou de 6 meses a 68 anos e o sexo feminino predominou com 50 casos. RESULTADOS: No material apresentado, não ocorreu nenhum óbito na fase de pós-operatório imediato ou tardio. O ritmo cardíaco permaneceu sempre sinusal e não ocorreram complicações na evolução. CONCLUSÃO: O presente trabalho demonstra a aplicabilidade da técnica descrita, com resultados favoráveis em relação a mortalidade, distúrbios de ritmo e complicações na região da veia cava superior direita.

OBJECTIVE: Surgical treatment of anomalous pulmonary venous connection to the superior vena cava, associated with sinus venous atrial septal defect, is well established and correlates with low mortality and morbidity. In order to reduce the incidence of stenosis or occlusion of the right superior vena cava, especially when associated with the presence of left superior vena cava, the right atrial appendage was used to enlarge the right superior vena cava, after the diversion of the anomalous pulmonary veins for the left atrium. METHODS: Between June 1986 and September 2008, 95 consecutive patients were operated with anomalous drainage in the superior right vena cava and high right atrium. Ages ranged from 6 months to 68 years and females predominated with 50 cases. RESULTS: There was no death in the immediate or late post operative care. The sinus cardiac rhythm was preserved in all cases and there was no complications in the late follow up. CONCLUSION: This paper demonstrates the applicability of the technique described, with favorable results on mortality, rhythm disturbances and complications in the right superior vena cava.

Brain abscess with persistent left superior vena cava

Persistent left superior vena cava [PLSVC] is the most common congenital cardiac anomaly occurring in around 0.4% of the general population, and 1.3-4.5% in those with additional cardiac defects. Eighty-two percent of PLSVC coexists with a right superior vena cava. Usually PLSVC is asymptomatic, but can cause difficulties during central venous cannulation from the left side. There are a few cases of brain abscess in the literature associated with PLSVC. Here, we describe a patient of brain abscess with PLSVC in the intensive care unit of King Faisal Hospital, Makkah, Kingdom of Saudi Arabia

Double superior vena cava presenting with anomalous jugular and azygous venous systems.

OBJECTIVE: To report on the double superior vena cava occurring with anomalous azygous vein and abnormal termination of the cardiac veins, which has not been reported before in one individual. RESULTS: Examination of the heart revealed the presence of both the right and left superior vena cava. The right superior vena cava terminated into the right atrium and received the right root of the azygous vein, and the left superior vena cava received the left root of the azygous vein before entering the dilated coronary sinus. The azygous vein ascended in the right chest, and at the level of the third thoracic vertebra, it divided into the right and left roots, which joined the respective superior vena cava. Observation of the left lung revealed the presence of both the oblique and horizontal fissures that demarcated the upper, middle, and lower lobes. Dissection of the neck revealed abnormal connection of the superficial veins. The left external and anterior jugular veins opened at the confluence of veins that was drained by the venous arch that passed to the right side of the neck to open into the right external jugular vein. CONCLUSION: The coexistence of double superior vena cava with azygous and superficial jugular venous anomalies is considered to be incidental finding. However, continued documentation of such anomalies is clinically important, and it remains to be important in medical science.

Drenagem anômala parcial de quatro veias pulmonares com septo interatrial íntegro: relato de um caso raro / Partial anomalous return of four pulmonary veins with intact interatrial septum defect: a rare case report

É relatado caso raro de drenagem anômala parcial de quatro veias pulmonares no átrio direito e veia cava superior, com septo interatrial íntegro em criança de cinco anos de idade. Havia poucos sintomas, em contraste com débito ventricular esquerdo dependente do fluxo da veia lobar superior esquerda e da língula. Complacência diminuída à esquerda motivou quadro acentuado de hipertensão venocapilar pulmonar no pós-operatório imediato, aliviado por feitura de comunicação interatrial de 8 mm. A evolução posterior foi boa.

We report on the rare case of partial anomalous return of four pulmonary veins in the right atrium and superior vena cava with intact interatrial septum in a five-year-old child. There were few symptoms in contrast with the left ventricular output dependent on the flow of the left upper lobe vein and from the lingula. Reduced compliance to the left led to a severe picture of pulmonary venocapillary hypertension in the immediate postoperative period, mitigated by an 8-mm interatrial septal defect. The patient progressed well after the intervention.

Agenesia de cava superior associada a bloqueio atrioventricular de 3º grau / Agenesis of the right superior vena cava associated with total heart block

A persistência de veia cava superior esquerda com ausência da veia cava superior é uma anomalia rara, principalmente quando associada a bloqueio atrioventricular de 3º grau. Relatamos o caso de uma paciente, na qual durante implante de marca-passo definitivo, para a correção de bloqueio atrioventricular total, foi detectada presença de veia cava superior esquerda com suspeição de ausência de veia cava superior, o que levou ao emprego de técnica diferenciada para fixação do eletrodo ventricular. Para confirmação da provável agenesia, foram realizados diversos exames complementares de imagem, demonstrando-se a dificuldade no diagnóstico da síndrome aqui descrita.

The superior left vena cava with the absent superior vena cava is a rare abnormality, especially when associated with total heart block. We report a case of a patient in which the presence of superior left vena cava and the absence of the superior vena cava was detected during the implantation of a pacemaker for the correction of a total heart block, which led us to use a different technique for the fixation of the ventricular electrode. To confirm the supposed absence, several image exams were made showing the difficulty on the diagnosis of the described syndrome.

Anomalous connection of superior vena cava to the left atrium masquerading as epilepsy: a case report with review of literature.

An 11-year-old girl who presented with recurrent epileptic fits was eventually found to have a superior vena cava draining into the left atrium. There was no atrial septal defect. The patient underwent a successful surgical correction.

Holt-Oram syndrome with double outlet right ventricle with valvular and subvalvular pulmonary stenosis

The Holt-Oram syndrome is an autosomal dominant condition characterized by skeletal abnormalities that are frequently accompanied by congenital cardiac defects, most commonly an atrial septal defect. We report a rare case of sporadic Holt-Oram syndrome with double outlet right ventricle [DORV], valvular and subvalvular pulmonary stenosis, persistent left superior vena cava draining to the left atrium, and an ectopic right kidney