Diagnóstico prenatal de atresia tricuspidea / Prenatal diagnosis of tricuspid atresia

La atresia tricuspidea es una cardiopatía rara caracterizada por oclusión de la válvula tricúspide y asociada a hipoplasia de ventrículo derecho y estenosis pulmonar. Clásicamente se presenta con un defecto en septun ventricular. El diagnóstico puede establecerse con ecocardiografía fetal y usualmente se sospecha ante una imagen de cuatro cámaras anormal.

Terapia de ressincronização cardíaca em portador de coração univentricular para correção de dissincroniomiopatia causada por mudança do sítio de estimulação / Cardiac resynchronization therapy in severe systolic dysfunction in a univentricular heart patient to correct dyssynchrony cardiomyopathy caused by change of stimulation site

Paciente com 6 anos de idade, do sexo masculino, portador de coração univentricular decorrente deatresia tricúspide com hipoplasia de ventrículo direito tipo IIC e submetido a implante de marcapasso por bloqueio atrioventricular total no pós-operatório de cirurgia de Fontan, em 2012. Houve necessidade de troca do sistema de estimulação em decorrência de infecção de loja logo após a troca do marcapasso, em 2014. Seis meses após amudança do sítio de estimulação, começou a apresentar quadro de insuficiência cardíaca refratária e disfunção sistólica grave, e o transplante cardíaco foi indicado. O paciente foi submetido a terapia de ressincronização cardíaca orientada por eletrocardiografia triaxial como ponte para transplante e apresentou melhora clínico-estrutural após10 semanas, recebendo alta hospitalar...

We report the case of a 6-year-old male patient with univentricular heart due to tricuspid atresia with right ventricular hypoplasia (IIc), who had a pacemaker implanted for complete heart block after a Fontan surgery in 2012. The stimulation system had to be exchanged due to a pocket infection soon after the pacemaker was exchanged in 2014. Six months after the stimulation site was exchanged he presented refractory heart failure and severe systolic dysfunction and a heart transplantation was indicated. He was submitted to cardiac resynchronization therapy guided by triaxial electrocardiography as a bridge for transplantation. The patient presented clinical and structural improvement after 10 weeks and was discharged...

Diagnóstico pré-natal de atresia tricúspide tipo IIC com atresia aórtica / Prenatal diagnosis of tricuspid atresia IIC associated with aortic atresia

Introdução: A atresia tricúspide é uma cardiopatia rara, sendo sua associação com discordância atrioventricular e atresia aórtica ainda mais rara. Objetivo: Relatar caso de associação cardíaca rara, reiterando o papel do ecocardiograma fetal no diagnóstico precoce das malformações cardíacas complexas. Relato: Recém-nascido com diagnóstico pré-natal de ausência de conexão direita, ventrículo direito hipoplásico, via de saída única pulmonar emergindo do ventrículo esquerdo e arco aórtico hipoplásico foi submetido à cirurgia de Norwood-Sano, com 48 horas de vida, com boa evolução. Conclusão: Os achados ecocardiográficos no feto predisseram a necessidade de intervenção neonatal precoce e asseguraram o manejo pós-natal adequado.

Atresia of the coronary sinus ostium to the right atrium with a persistent left superior vena cava / Atresia del ostium del seno coronario en el atrio derecho con una vena cava superior izquierda persistente

The coronary sinus has lately assumed an important role in the cardiologic clinic once it has been widely used in invasive procedures of the heart. Commonly, it is used during the electrodes implants for the epimiocardic monitoring of the cardiac rhythm, through a biventricular pace maker. These invasive procedures are not possible in hearts with an atresic coronary sinus ostium. In the presence of this anomaly, another may occur: the development of the "Marchal" vein which is a remaining of the left superior vena cava (LSVC). This happens so that the venous blood from the heart can drain into the right atrium, by a communication between the LSVC and the left brachiocephalic vein. The presence of a LSVC brings difficulties when performing an invasive procedure in order to access the right atrium through the superior vena cava, usually done in the cardiologic clinic. Moreover, the LSVC crossing over the left atrium is vulnerable to cardiovascular surgical interventions, confirmed by clinical reports. In the present study, 400 formalin fixed hearts from male cadavers, aged between 35 and 80 years, were investigated, particularly for the anatomy of the coronary sinus. The obliterated ostium of the coronary sinus to the right atrium associated with a persistent LSVC was present in only one (0.25 percent). We performed a diameter study of these structures since they were dilated due to the venous blood from the heart draining into the right atrium, by a communication between the LSVC and the left brachiocephalic vein. We also perform a literature review of these cases and discuss our finding in relation to its clinical importance.

El seno coronario recientemente ha asumido un papel importante en la clínica cardiológico, siendo ampliamente utilizado en procedimientos invasivos del corazón. Comúnmente, se utiliza en los implantes de los electrodos para el monitoreo epimiocárdico del ritmo cardiaco, a través de un ritmo biventricular establecido. Estos procedimientos invasivos no son posibles en los corazones con una atresia del ostium del seno coronario. En presencia de esta condición, se puede producir otra anomalía: el desarrollo de la vena de "Marchal" la cual es un vestigio de la vena cava superior izquierda (VCSI). Esto provoca que la sangre venosa del corazón pueda drenar en el atrio derecho, por una comunicación entre la VCSI y la vena braquicefálica izquierda. La presencia de una VCSI trae dificultades a la hora de realizar un procedimiento invasivo con el fin de acceder al atrio derecho a través de la vena cava superior, usualmente hecho en la clínica cardiológica. Por otra parte, el cruzamiento de la VCSI sobre el atrio izquierdo es vulnerable en las intervenciones quirúrgicas cardiovasculares, confirmado por informes clínicos. En el presente estudio, 400 corazones fijados en formalina provenientes a cadáveres de sexo masculino, con edades comprendidas entre los 35 y 80 años, fueron investigados, en particular por la anatomía del seno coronario. El ostium obliterado del seno coronario al atrio derecho asociado con una VCSI persistente estuvo presente en sólo una muestra (0,25 por ciento). Se realizó un estudio del diámetro de estas estructuras dilatadas debido a que la sangre venosa drena desde el corazón hacia el atrio derecho, por una comunicación entre la VCSI y la vena braquicefálica izquierda. También se realiza una revisión de la literatura de estos casos y se discuten nuestros hallazgos en relación con su importancia clínica.

Diagnosis and management of cyanotic congenital heart disease: part I.

Most commonly encountered cyanotic cardiac lesions in children, namely, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia are reviewed. Pathology, pathophysiology, clinical features, non-invasive and invasive laboratory studies and management are discussed. The clinical and non-invasive laboratory features are sufficiently characteristic for making the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required and are needed either to define features, not clearly defined by non-invasive studies or as a part of catheter-based intervention. Surgical correction or effective palliation can be undertaken with relatively low risk. However, residual defects, some requiring repeat catheter or surgical intervention, are present in a significant percentage of patients and therefore, continued follow-up after surgery is mandatory.

conducta quirurugica en la insuficiencia tricuspidea funcional / Procedure surgical in the insuficiency tricuspidea funtional

Los avances en la cirugia valvular cardiaca son enormes y estan directamente vinculados a mejoras en las tecnicas de circulacion extracorporea y en la proteccion del miocardio, en la introduccion de imaginativas tecnicas de construccion valvular y en la utilizacion de ecograafia transesofagica para valorar intraoperatoriamente el resultado de la actitud quirurgica en las valvulopatias. A pesar de ello, todavia hay la cirugia de la valvula tricuspide y sobre todo cuando la lesion es de tipo insuficiencia funcional se discute decide y efectua en el contexto de la lesion valvular asociada en la evaluacion de cada caso en particular y con criterio compartido entre clinicos y cirujanos. En el prresente trabajo, se revisa la literatura con referencia a la mejor actitud quirurgica frente a la insuficiencia tricuspidea funcional y se presenta los resultados del Departamento de cirugia del Instituto Nacional de Torax, en un periodo de diez años, reportandose 47 casos consecutivos de cirugia conservadora, fundamentalmente con tecnica de De Vega. Se discuten las ventajas de esta tecnica y los resultados obtenido, concluyendose que aun existen respecto de lacirugia de la insuficiencia tricuspidea,controversias ne la indicacion quirurugica que todavia los procedimientos disponibles estan en perfeccion y que los resultados de la cirugia estan en funcion deltiempo de enfermedad del estado de contraccion ventricular del grado de hipertension arterial pulmonar y del tipo y grado de las lesiones valvulares concomitantes

The Fontan operation: experience at Siriraj Hospital.

Seventy five patients underwent modified Fontan operation at Siriraj Hospital from October 1987 to December 1998. Cardiology data was analyzed retrospectively. Four patients' data was unavailable. Median age at operation was 9.7 (1.8-34) years old. Tricuspid atresia accounted for 38 per cent of the patients. Ten patients (14.1%) died in the acute post operative period due to consequence of low cardiac output. Another 3 patients (4.2%) expired in the intermediate and late post operative period. Age at operation, pulmonary artery size, pre-operative oxygen saturation, and mean pre-operative pulmonary artery pressure were not different between those who survived and those who died. Abnormal pulmonary vein, atrioventricular valve regurgitation, and underlying ventricular morphology statistically affected the acute survival of modified Fontan operation. Intraoperative aortic cross clamp time, and post operative mean pulmonary artery pressure on day 0, 1 and 2 post operation were found statistically shorter and lower in the survival group. Survival rate at 5 years was 83 per cent. Modified Fontan operation is the final palliative operation of choice for low risk single ventricle physiology in our institution with acceptable outcome. Thorough pre-operative hemodynamic and anatomic studies and staging modified Fontan procedure may include a higher number of candidates and improve the outcome of the operation.