Superior vena cava obstruction after the Glenn procedure

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Mapeo y ablación por radiofrecuencia a cielo abierto: un caso de taquicardia ventricular refractaria en anomalía de Ebstein / Open-heart mapping and radiofrequency ablation: a case of refractory ventricular tachycardia in Ebstein's anomaly

Resumen La anomalía de Ebstein es una enfermedad congénita, caracterizada por el desplazamiento y la adherencia de la válvula tricúspide. Las valvas septal y posterior se implantan en posiciones más inferiores, causando insuficiencia tricuspídea, esta condición de forma progresiva genera dilatación y deterioro de la función sistólica ventricular derecha. Comúnmente se asocia a otras entidades, principalmente, a la comunicación interauricular y las arritmias mediadas por vías accesorias. La muerte súbita ocurre en estadios avanzados, usualmente relacionados con episodios de taquicardia ventricular. Este reporte describe un caso de anomalía de Ebstein y tormenta arrítmica, que se sometió a ablación del sustrato arrítmico endo y epicárdico en la porción atrializada del ventrículo derecho, que presentó como complicación intraprocedimiento la perforación del ventrículo, el taponamiento cardiaco y la necesidad urgente de la reparación quirúrgica, realizándose en el intraoperatorio una segunda ablación por radiofrecuencia a cielo abierto con el objetivo de eliminar los potenciales tardíos y los fragmentados remanentes en el área de bajo voltaje de la escara. Veintidós semanas después de la ablación se implantó un cardiodesfibrilador subcutáneo para prevención secundaria de la muerte súbita; en el seguimiento a 14 meses no hubo terapias apropiadas del dispositivo.

Abstract Ebstein's anomaly is a congenital disease characterised by the displacement and adherence of the tricuspid valve. Septal and posterior valves are implanted in lower positions, thus causing tricuspid insufficiency. This condition gradually generates dilation and deterioration of the right ventricular systolic function. It is commonly associated to other conditions, mainly atrial septal defect and accessory pathway arrhythmias. Sudden death occurs in the advanced stages, usually related to episodes of ventricular tachycardia. This report describes a case of Ebstein's anomaly and arrhythmic storm who underwent ablation of the arrhythmic endo- and epicardial substract in the atrialized portion of the right ventricle, that developed as an intrasurgical complication the perforation of the ventricle, cardiac tamponade and urgent need of surgical repair. During the surgery a second open-heart radiofrequency ablation was conducted with the goal of eliminating potentially late and fragmented remnants in the low voltage area of the scars. Twenty two weeks after the ablation a subcutaneous cardioverterdefibrillator was implanted for secondary prevention of sudden death; in the follow-up after 14 months there were no appropriate therapies of the device.

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...

Cirugía de Fontan: complicaciones posquirúrgicas: Medellín, Colombia / Fontan surgery: complications postsurgical: Medellin, Colombia

El manejo del corazón univentricular ha mejorado laexpectativa de vida de los pacientes con la cirugía deFontan.Objetivo: identificar las complicacionesposquirúrgicas más frecuentes de la cirugía de Fontanen pacientes de una institución privada de la ciudadde Medellín.Metodología: estudio descriptivo del tipo de seriesclínicas. La población estuvo compuesta porpacientes sometidos a la cirugía de Fontan. Lainformación sobre las variables sociodemográficas yclínicas fue tomada directamente de las historiasclínicas. La investigación contó con la aprobación delComité de ética. Se empleó el programa SPSS®versión 17.0 (SPSS Inc; Chicago, Illinois, EE.UU.) parael procesamiento y análisis de la información. Para elmanejo estadístico de los datos se empleó laestadística descriptiva.Resultados: participaron 21 pacientes, la mediana deedad fue de 5 años (mínimo 3 y máximo 10); el 66,7%fueron de sexo masculino. El 100% de los niños tenían antecedentes de cirugía paliativa previa, la másfrecuente fue el Glenn bidireccional. En cuanto a lascomplicaciones más frecuentes fueron derramepleural (52,4%), disfunción miocárdica e infección enherida (19%).Conclusiones: las complicaciones que sepresentaron coinciden con lo reportado en la literatura, cabe resaltar que ningún paciente refirió estenosissubaórtica ni hemorragias.

Univentricular heart management has improved thelife expectancy of patients with Fontan surgery.Objective: identify the most common postoperative complications of the Fontan surgery in patientsfrom a private institution from Medellin.Methodology: descriptive clinical series. Thepopulation was patients undergoing the Fontansurgery. Information about sociodemographic andclinical variables was taken directly from themedical records. The research was approved bythe Ethics Committee. We used SPSS ® version17.0 (SPSS Inc, Chicago, Illinois, USA) forprocessing and analysis of information. For statistical handling of data is used for descriptiv estatistics. Results: 21 patients participated, the median agewas 5 years (minimum 3, maximum 10), 66.7% were male. 100% of the children had a history of priorpalliative surgery, the most common was the bidirectional Glenn. As for the most common complications were pleural effusion (52.4%), myocardial dysfunction and wound infection (19%). Conclusions: the complications presented agree with those reported in the literature, it is notable that no patient reported sub aortic stenosis and bleeding.

Morbilidad y mortalidad posoperatoria en niños con corazón univentricular según diagnóstico morfológico / Postoperative mortality and morbidity found in children with univentricular heart according to the morphological diagnosis

Introducción: la atresia tricuspídea es la cardiopatía congénita cianótica que motiva mayor indicación de cirugía univentricular. El tratamiento quirúrgico consiste en separar las circulaciones sistémica y pulmonar a través de una derivación cavopulmonar total (proceder de Fontan). Objetivo: comparar la evolución posoperatoria del proceder de Fontan entre enero de 1990 y diciembre de 2010 en relación con el diagnóstico morfológico. Métodos: se ordenaron en 2 grupos de estudio (AT: atresia tricuspídea y No AT: con diagnóstico diferente a atresia tricuspídea). Las variables cualitativas y cuantitativas se analizaron a través de las pruebas chi cuadrado y t de student para un nivel de significación £ 0,05. Resultados: presentaron atresia tricuspídea 31 pacientes y 61 no atresia tricuspídea. En el posoperatorio 68 pacientes presentaron complicaciones, 23 con atresia tricúspidea (74,2 por ciento) y 45 con diagnóstico diferente a atresia tricuspídea (73,7 por ciento). Treinta pacientes presentaron efusiones pleurales persistentes durante el posoperatorio, 11 (35,5 por ciento) y 19 (31,4 por ciento) para la atresia tricúspidea y con diagnóstico diferente a atresia tricuspídea respectivamente. Fallecieron 18 enfermos, sin diferencia significativa entre ambos grupos. Durante el seguimiento, la causa de morbilidad más frecuentes fue por capacidad funcional disminuida de forma moderada o grave, en 13 pacientes con atresia tricúspidea (44,8 `por ciento) y en 20 (44,4 por ciento) en el otro grupo (p= 1). Conclusiones: el proceder de Fontan es una opción quirúrgica que permite la supervivencia de los pacientes con corazón univentricular asociada a morbilidad posoperatoria a largo plazo. El diagnóstico morfológico de atresia tricuspídea no se relaciona con la morbilidad y mortalidad

Introduction: tricuspid atresia is the cyanotic congenital heart disease that more frequently involves the indication of univentricular heart surgery. The surgical treatment consists of separating the systemic circulation from the pulmonary one through total cavopulmonary shunt (Fontan procedure). Objective: to compare the evolution of Fontan procedure postoperatively from January 1990 to December 2010 with the morphological diagnosis. Methods: the children were divided into 2 study groups (Triscupid atresia and Triscupid atresia-free with diagnosis different from that of tricuspid atresia). Chi square and Student's tests helped to analyze qualitative and quantitative variables for significance level £ 0.05. Results: tricuspid atresia was observed in 31 patients and 61 tricuspid atresia-free. In the postoperative period, 68 patients suffered complications, 23 with tricuspid atresia (74.2 percent) and 45 diagnosed with a disease other than tricuspid atresia (73.7 percent). Thirty patients had persistent pleural effusions during the postoperative period, 11 (35.5 percent) and 19 (31.4) in the tricuspid atresia group and in the tricuspid atresia-free group respectively. Eighteen patients died and no significant association was found in neither of the groups. During the follow-up, the most frequent cause of morbidity was reduced functional capacity either moderate or severe in 13 patients with tricuspid atresia (44.8 percent) and in 20 children (44.4 percent)from the other group (p= 1). Conclusions: Fontan procedure is a surgical option that allows the survival of patients with univentricular heart associated to long-term postoperative morbidity. The morphological diagnosis of tricuspid atresia is not related to morbility and mortality

Defecto del canal auriculoventricular, aurícula única y atresia tricuspídea como parte de un caso de síndrome de Ellis-Van Creveld / Atrioventricular canal defect, single atrium and tricuspid atresia as part of a case of Ellis-Van Creveld syndrome

El síndrome de Ellis-Van Creveld (SEVC) o displasia condroectodérmica se debe a una mutación de transmisión autosómica recesiva en el brazo corto del cromosoma 4 y afecta múltiples órganos. Descrito como una tétrada clásica de condrodisplasia, displasia ectodérmica, polidactilia y defectos cardíacos congénitos, sólo se conoce a partir de informes y series de casos. Se describe el caso de un varón de 3 meses, sin antecedentes familiares de importancia, que presentó un cuadro de condrodisplasia, labio superior fusionado al paladar, simpolidactilia posaxial bilateral en las manos, displasia del desarrollo de las caderas, tórax estrecho con costillas cortas y compromiso cardíaco. El presente caso sería la primera comunicación del SEVC en el Perú.

Ellis-Van Creveld Syndrome or chondrectodermal dysplasia is produced by an autosomal recessive inheritance secondary to mutation in the short arm of chromosome 4. The syndrome affects multiple organs. It is described as a clinical tetrad that involves chondrodysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. It is only known from reports and case series. We present a three months old male, without relevant family history, who presented chondrodysplasia, upper lip merged to palate, bilateral sinpolydactyly in the hands, developmental dysplasia of the hip, narrow chest with short ribs, and heart defects. This case is the first report of EVC in Peruvian literature.

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 unilateral del orificio atrioventricular atresia mitral (ATRM). Estudio de 44 especímenes / Unilateral atresia of the hole mitral atresia atrioventricular (ATRM). Study of 44 specimens

La atresia mitral (ATRM) forma un amplio espectro de gran complejidad, varias entidades se incluyen en este espectro, interrelacionadas embriológica, morfológica, fisiológica y quirúrgicamente. Clasificaciones basadas en estructuras alejadas del orificio atrésico llevan a desconocer integralmente esta patología. El objetivo de este estudio es mostrar la existencia de un amplio espectro en nuestra serie de 44 casos de ATRM y sus implicancias quirúrgicas. Material y métodos. El estudio se basa en un material de autopsia de 44 especímenes de ATRM entre 1 500 especímenes de la colección de cardiopatías congénitas del Instituto de Salud del Niño Servicio de Patología. Lima, Perú. Definición.- Se considera que la ATRM está presente cuando la salida atrial atrésica no se comunica directamente con el ventrículo subyacente morfológicamente izquierdo. Se incluyen algunas formas de doble entrada. Clasificación. Criterios: 1) combinación segmental; 2) estado de la salida atrial atrésica (muscular o membranosa, usual o no usual, alineamiento A-V normal o anormal); 3) relación V-A; 4) anomalías asociadas. Resultados. Grupos establecidos de acuerdo a la combinación segmental y tipos de acuerdo al estado de la salida atrial atrésica. Grupo 1: (S, D, X) 31 casos. Sexo en 29: 17 masculino y 12 femenino. Edad en 28: entre 1 día y 5 años. Levocardia en 29 y dextrocardia en 2 casos. Situs solitus. D-asa. Relación V-A variable. ATRM muscular, usual, alineamiento A-V anormal 28/31: (S, D, S) ATRA (con atresia aórtica y conus subpulmonar) en 10/28, (S, D, D) ATRP (con atresia pulmonar y conus subaórtico) en 3/28, (S, D, SR) en 6/28 con doble salida ventricular derecha y conus subpulmonar, (S, D, DR) en 9/28 con doble salida ventricular derecha D-aorta y conus subaórtico. ATRM membranosa, usual (S,D,S) ATRA con alineamiento A-V normal en 1/31 casos.

The anatomic spectrum of mitral atresia is wide, there are considerable variations in the anatomic type of the orifice atresia per se, in ventricular morphology and in ventriculoarterial relationships. The variations are so marked that the conventional nomenclature æwith or without transpositionÆ, hardly cover all of these variations. The best adequate classification and description of each anatomic type is only possible by using the concept of the spectrum of unilateral A-V orifice atresia together with the segmental approach. The objective of this study is to show the existence of a broad spectrum in our series of 44 cases of mitral atresia. His knowledge has surgical involvement. Materials and Methods. This study is based on an autopsy material, from 44 specimens of mitral atresia (ATRM) between 1500 specimens belonging to the collection of congenital heart disease from the National Child Health Institute. Lima, Perú. By definition, mitral orifice atresia is present when one of the atrial outlets is atretic without direct communication with the morphologically left ventricle. Even if the ventricular anatomy indicates the presence of a single ventricle or a double inlet ventricle, The classification is based on the following criteria: 1) segmental approach; 2) the status of mitral atresia, a) muscular or membranous, b) usual or unusual in regard to size of the homologous ventricle to the atretic valve, c) type of A-V alignment normal or abnormal; 3) ventriculoarterial (V-A) relationship; 4) associated anomalies. Findings Groups are classified according to segmental approach (segmental set) and types according to the state of the exit atrial atresia. Group 1. (S, D, X) 31 cases. Solitus of atria, D-loop (D-bulboventricular loop), X (several kinds of ventriculoarterial relationships can occur) Sex was obtained in 29/31 cases, 17 male and 12 female cases. Age was obtained in 28/31 cases. Age ranged from 1 day to 5 yearsà.

Soporte anatómico en el diagnóstico ecocardiográfico de la ausencia de conexión atrioventricular derecha / Anatomic support in the echocardiographic diagnosis of right atrioventricular connection

OBJECTIVE: To show the close relationship between the anatomic features and the echocardiographic image in the absence of right atrioventricular connection. MATERIAL AND METHOD: Echocardiographic study of a patient with right atrioventricular connection and a corresponding anatomic specimen from the Museum of Embryology of the INC "lgnacio Chávez". RESULTS: A close correlation between an anatomic specimen with absence of right atrioventricular connection and an echocardiographic image of a patient with the same cardiopathy was established. CONCLUSION: The correlation between the anatomic features and the echocardiographic image, supports the imagenologic diagnostic precision of this method.

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.

Atresia tricuspídea asociada a tronco común: reporte de un caso / Tricuspid atresia associated with common trunk: a case report

The tricuspid atresia associated with persistent truncus arteriosus is a rare anomaly. A case is presented of one male patient of three months age in which was detected a cardiac murmur. The diagnosis was made by echocardiography and confirmed by cardiac catheterization. The first step of the palliation was done with disconnection of the trunk of the pulmonary artery combined with a systemic- to- pulmonary shunt of 4 mm. The patient died 24 hours later for persistent metabolic acidosis.

El método de Fick subestima el cálculo deflujo pulmonar en los enfermos operados de Glenn bidireccional / The Fick method underestimates pulmonary flow calculation in patients subjected to bidirectional Glenn procedure

In patients with Bidirectional Glenn who have undergone surgery, the superior caval venous flow provides the only pulmonary blood supply. This is the effective pulmonary flow and at the same time its volume is not enough to overflow the single ventricle. The unsaturated, inferior vena cava flow is not oxygenated, since it goes across the interatrial septal communication and gets mixed in the left ventricle with the pulmonary venous blood. In this work, a bidirectional Glenn case is analyzed. The hemodynamic data before and after the operation are shown. It was evident from this case that the use of the Fick method to measure pulmonary flow in patients with bidirectional Glenn operation is not appropriate. Alternative methods, such as Doppler echocardiography and Magnetic Resonance Imaging, are recommended. A literature review on this subject was carefully done.

Atresia unilateral del orificio atrioventricular. Atresia Tricúspide (ATRT) - atresia mitral (ATRM) / Unilateral Atresia of the orifice atrioventricular. Atresia Tricúspide (ATRT) - atresia mitral (ATRM)

El espectro de la atresia unilateral de los orificios atrioventriculares es grande. Clasificaciones en base a estructuras alejadas del orificio atrésico y clasificaciones simples llevan a desconocer el espectro integralmente, quedando fuera muchas variantes importantes. El objetivo de este estudio es mostrar la existencia de un amplio espectro de nuestra serie de 47 casos de Atresia Tricúspide. Su conocimiento es de importancia quirúrgica. Material y Métodos. Este estudio se basa en un material de autopsia de 47 especímenes de atresia tricúspide (ATRT) entre1500 especímenes que pertenecen a la colección de cardiopatías congénitas del Instituto de Salud del Niño. Definición.- La Atresia unilateral de un orificio A-V está presente cuando una de las salidas atriales es atrésica y sin directa comunicación entre la aurícula y el ventrículo. Incluimos casos con ventrículo único izquierdo (VUI) y coexistencia de ATRT. La clasificación se realiza en base a los siguientes criterios: 1.- Combinación segmental (simbología) Es el método más apropiado y esencial para este estudio 2. Estado de la salida atrial atrésica derecha o izquierda: Atresia tricúspide ATRT o mitral ATRM.a).- Muscular o Membranosa. Muscular cuando la cavidad atrial está separada del ventrículo topográficamente homólogo por un piso muscular, Membranosa cuando un diafragma fibroso se interpone entre la cavidad atrial y la cavidad ventricular topográficamente homóloga, el tejido valvular está fusionado. b) Usual o No Usual. Usual: con ventrículo topográficamente homólogo hipoplásico, incompleto o ausente. No Usual: con ventrículo topográficamente homólogo normal o grande. c) Alineamiento A-V: Anormal: El piso atrial ciego no está sobre la cavidad ventricular homóloga sino sobre el septum interventricular (SIV) o sobre la pared libre ventricular no homóloga. Normal: cuando la cavidad atrial ciega está sobre el ventrículo homólogo.3.- Relación ventrículoarterial: (V-A)4.- Anomalías asociadas...

The anatomic spectrum of unilateral atrioventricular(A-V) orifice atresia is wide, there are considerable variations in the anatomic type of the orifice atresia perse, in ventricular morphology and in ventriculo arterial relationships. The variations are so marked that the conventional nomemclatures mainly by õwith or without transpositionõ, hardly cover all of these variations. To theour present knowledge, the best adequate classification and description of each anatomic types is only possible by using the concept of the spectrum of unilateral A-Vorifice atresia together with the segmental method. The objective of these study is to show the existence of abroad spectrum in our series of 47 cases of Tricuspid atresia. His knowledge has surgical implicance. This study is based on an autopsy material, from 47 specimens of tricuspid atresia (ATRT) between 1500 specimens belonging to the collection of congenital heart disease from the Institute of Child Health. By definition unilateral A-V orifice atresia, tricúspid ormitral orifice atresia is present when one of the atrial outlets is atretic without direct communication with the ventricle. Even if the ventricular anatomy indicates the presence of a single ventricle or a double inlet ventricle, those cases with coexistence of atretic right or left A-Vorifice are included as a form of unilateral A-V orifice atresia.The classification is based on the following criteria: 1. Segmental approach 2. The status of right or left atrial outlet atresia. Tricuspid or Mitral. a). Muscular o Membranosa. b). Usual and Unusual in regard to size of the homologous ventricle to the atretic valve. c). Type of A-V alignment Normal or abnormal. 3. Ventriculoarterial (V-A) relationship. 4. Associated anomalies...

Aplicación del análisis secuencial segmentario en 100 especímenes cardiopulmonares / Sequential segmental analysis in 100 cardiopulmonary specimens

OBJECTIVE: To evaluate the feasibility and accuracy of the sequential segmental analysis (SSA) to diagnose congenital heart diseases. MATERIAL AND METHODS: 100 cardiopulmonar specimens were analyzed according with the sequential segmental analysis. The atrial situs, atrioventricular (AVC) and ventriculoarterial connections (VAC) were defined, finally associated lesions were observed. RESULTS: From 100 specimens, 91 had situs solitus (SS), 6 inversus (SI) and 3 isomerism (2 right isomerism and 1 left isomerism). The AVC in these hearts were: 89 biventricular, 80 (90%) concordant, 6 (6.7%) discordant and 3 (3.3%) ambiguous (2 right topology an 1 left topology). The univentricular AVC were 11, 4 (36%) had absent left AVC (mitral atresia), 3 (27%) absent right AVC (tricuspid atresia), 2 (18%) double inlet right ventricle (DIRV), 1 double inlet left ventricle (DILV) and 1 (9%) double inlet undetermined ventricle. The VAC in SS were: 52 (57%) concordant, 13 (14%) discordant, 6 (6.6%) double outlet right ventricle (DORV), 20 (22%) with single outlet (13 pulmonary atresia , 5 aortic atresia and 2 common arterial trunk). The VAC in SI were: concordant in one, (16.7%), discordant 3 (50%) and DORV in 2 (33.3%). In the hearts with isomerism, the VAC were concordant in one, DORV in one and single outlet in another (pulmonary atresia). The associated anomalies were ASD (67%), PAD (51%) and VSD (53%). CONCLUSIONS: The SSA was and effective and accurate tool to codify clear and completely the congenital heart disease. It is suggested that if the SSA is normal to describe this situation as a Normal Basic Heart Organization and then to describe the associated anomalies.