Anomalía de Ebstein «like¼ en doble entrada ventricular. Una rara asociación / Ebstein's "like" anomaly ventricular double inlet. A rare association

Resumen: Introducción y objetivos: La asociación de corazones univentriculares con doble entrada y anomalía de Ebstein «like¼ de la válvula auriculoventricular común es extremadamente rara. Se describen 2 corazones con esta asociación. Métodos: Se utilizó el sistema secuencial segmentario que determina el situs auricular, los tipos y modos de conexión auriculoventricular y ventriculoarterial y las anomalías agregadas. Resultados: Ambos corazones presentaron situs solitus auricular, defecto septal auriculoventricular con foramen primum y doble entrada y doble salida con grandes arterias normalmente relacionadas en un ventrículo único. En el primero las 4 valvas auriculoventriculares estuvieron adosadas a la pared ventricular desde la unión auriculoventricular hasta el ápex con auricularización de la entrada y de la porción trabecular y presentó estenosis infundibular y valvular. En el segundo la porción proximal de la válvula auriculoventricular estuvo adosada a la pared ventricular con auricularización pequeña; en su porción distal mostró displasia con nodulaciones fibromixoides y cuerdas tendinosas pequeñas y gruesas y la arteria pulmonar estuvo dilatada. Conclusiones: Ambos corazones forman parte de la conexión auriculoventricular univentricular del sistema secuencial segmentario, cuya precisión diagnóstica muestra su eficacia. Las asociaciones de defectos congénitos en un solo corazón muestran el infinito espectro de las mismas, lo que expande nuestro conocimiento de la cardiología pediátrica.

Abstract: Introduction and objectives: The association of univentricular heart with double inlet and Ebstein's "like" anomaly of the common atrioventricular valve is extremely rare. Methods: Two hearts with this association are described with the segmental sequential system which determine the atrial situs, the types of atrioventricular and ventriculoarterial connections and associated anomalies. Results: Both hearts had atrial situs solitus, and a univentricular heart with common atrioventricular valve, a foramen primum and double outlet ventricle with normal crossed great arteries. In the fiefirst heart the four leaflets of the atrioventricular valve were displaced and fused to the ventricular walls, from the atrioventricular union roward the apex with atrialization of the inlet and trabecular zones and there was stenosis in the infundibulum and in the pulmonary valve. In the second heart the proximal segment of the atrioventricular valve was displaced and fused to the ventricular whith shot atrialization and the distal segment was dysplastic with fibromixoid nodules and tendinous cords short and thick; the pulmonary artery was dilate. Conclusions: Both hearts are grouped in the atrioventricular univentricular connection in the segmental sequential system. The application of this method in the diagnosis of congenital heart disease demonstrates its usefulness. The associations of complex anomalies in these hearts show us the infinite spectrum of presentation of congenital heart disease which expands our knowledge of pediatric cardiology.

Síndrome de la cimitarra. Correlación anatomoembriológica / Scimitar syndrome. Correlation anatomo-embryological

Resumen Objetivos Describir morfológicamente un bloque visceral toracoabdominal de un caso de síndrome de la cimitarra. Proponer una hipótesis patogenética que explique el desarrollo de las conexiones venosas pulmonares de este síndrome. Método El espécimen anatómico se describió con el sistema secuencial segmentario. Se determinó el situs, las conexiones entre los segmentos cardíacos y las anomalías agregadas. Se describió la anatomía de ambos pulmones, incluida la conexión venosa pulmonar. Se elaboró una hipótesis patogenética que explica la conexión venosa pulmonar a través de una correlación entre la patología del síndrome y el desarrollo normal de las venas pulmonares. Resultados El situs fue solitus, las conexiones entre los segmentos cardíacos fueron normales, existió hipoplasia y displasia del pulmón derecho con secuestro del lóbulo inferior; las venas pulmonares derechas drenaron a un colector curvo en la porción suprahepática de la vena cava inferior y las venas pulmonares izquierdas lo hicieron a la aurícula izquierda. El secuestro pulmonar recibió irrigación a través de una colateral aortopulmonar. Existió una comunicación interauricular. Conclusiones La hipótesis patogenética propone que las conexiones venosas pulmonares de este síndrome representan la persistencia del horizonte XIV de Streeter (28-30 días de desarrollo), período en el que el seno de las venas pulmonares presenta conexión venosa doble con la aurícula izquierda y con un colector de la conexión primitiva que desemboca en la vitelina derecha de la que se deriva la porción suprahepática de la vena cava inferior.

Abstract Objectives To describe morphologically a toracoabdominal visceral block of a scimitar's syndrome case. We propose a pathogenetic theory wich explains the development of the pulmonary venous connection in this syndrome. Method The anatomic specimen was described with the segmental sequential system. The situs was solitus, the connections between the cardiac segments and the associated anomalies were determined. The anatomy of both lungs, including the venous pulmonary connection, was described. A pathogenetic hypothesis was made, which explains the pulmonary venous connection throw a correlation between the pathology of this syndrome and the normal development of the pulmonary veins. Results The situs was solitus, the connections of the cardiac chambers were normal; there were hypoplasia and dysplasia of the right lung with sequestration of the inferior lobe; the right pulmonary veins were connected with a curved collector which drainaged into the suprahepatic segment of the inferior vena cava; the left pulmonary veins were open into the left atrium. The sequestered inferior lobe of the right lung received irrigation throw a collateral aortopulmonary vessel. There was an atrial septal defect. Conclusions The pathogenetic hypothesis propose that the pulmonary venous connection in this syndrome represent the persistent of the Streeter's horizon XIV (28-30 days of development), period in which the sinus of the pulmonary veins has double connection, with the left atrium and with a primitive collector into the right viteline vein which forms the suprahepatic segment of the inferior vena cava.

Defecto de la tabicación atrioventricular con 2 válvulas asociado a ausencia del pericardio: Informe de un caso / Atrioventricular defect with 2 valves associated with absence of the pericardium: Report of a case

Presentamos el caso de una paciente con una malformación cardiaca que representa una forma de transición anatomoembriológica del defecto de la tabicación atrioventricular entre la forma de 2 válvulas y la que tiene una válvula común. Esta entidad además se asoció con ausencia de pericardio. A través de los diferentes estudios se ha establecido con precisión la secuencia diagnóstica, determinando cuál fue la aportación de cada método y aclarando además la nomenclatura del defecto de la tabicación atrioventricular.

We present a case of a patient with a cardiac malformation that represents a form of embryo-anatomical transition of an atrioventricular septal defect between a 2 valves form to a common valve form. This entity was associated with pericardium absence. Throughout several studies we have precisely established a diagnostic sequence by determining the adequate contribution of each method and we have been able to clear out the proper nomenclature of the atrioventricular cushion defect.

Doble salida de ventrículo derecho: Enfoque embriológico / Double outlet right ventricle: Embryological approach

Objetivo: Se presenta una explicación patogenética sobre la morfogénesis de la doble salida de ventrículo derecho que explica su origen. Método: Se describieron 35 corazones con doble salida de ventrículo derecho, 15 con grandes arterias ligeramente cruzadas, 10 con arterias lado a lado y 10 con aorta anterior y pulmonar posterior. Se comparó el plano de separación de las vías de salida y de las grandes arterias en los 3 tipos de esta cardiopatía, con el plano de la tabicación troncoconal normal del corazón embrionario. Se determinó el plano cefálico del tabique troncoconal y se comparó con la posición de su borde inferior, con lo cual se calculó el grado de torsión troncoconal en cada grupo de esta cardiopatía; esto permitió inferir el tipo de giro de ese tabique en las 3 formas anatómicas mencionadas. Resultados: En las grandes arterias ligeramente cruzadas el giro troncoconal fue de 135°,en las arterias lado a lado fue de 90°, en la aorta anterior derecha fue de 0° y en la aorta anterior izquierda fue de -90°. Conclusión: Embriológicamente esta cardiopatía se origina por persistencia de la continuidad entre el ventrículo derecho y el troncocono, que origina las vías de salida y las grandes arterias. Sus variantes anatómicas se originan por una detorsión progresiva del tabique troncoconal continuada con una torsión de -90°.

Objective: It is proposed a pathogenetic explanation that explains the morphogenesis of the anatomic variants of double outlet right ventricle. Method: An anatomic embryological correlation was made in which the plane separating the outlets and great arteries in the types of this cardiopathy was compared with the normal truncoconal septum in the embryonic heart. Thirty five hearts with double outlet right ventricle were described, fifteen with great arteries slightly crossed, ten with side by side great arteries and ten with anterior aorta and posterior pulmonary artery. The cephalic border of the truncoconal septum was compared with its inferior border in each group. With this procedure we calculated the type of torsion of the truncoconal septum. Results: In the slightly crossed great arteries the truncoconal twist was of 135° in side by side great arteries the twist was of 90° and in anterior right aorta the truncoconal septum was straight with 0° of rotation, and with left anterior aorta the rotation was of -90°. Conclusion: Embryologically double outlet right ventricle is originated by the persisting continuity between the right ventricle with the truncus and conus which form the great arteries and their outlets. The anatomic variations are the consequence of progressive detortion of the truncoconal septum followed by a torsion of -90°.

Tetralogía de Fallot con atresia pulmonar: Morfopatología y anatomía quirúrgica / Tetralogy of Fallot with pulmonary atresia: Morphopathology and surgical anatomy

Se detalla la morfopatología de la tetralogía de Fallot con atresia pulmonar como un espectro de variaciones que sirve de fundamento para destacar la anatomía quirúrgica de esta cardiopatía y se muestra la base embriológica que determina su estructura. Se estudiaron 35 corazones con el sistema secuencial segmentario. Se determinó: situs atrial, conexiones entre los segmentos cardíacos y entre el ventrículo derecho y la vasculatura arterial pulmonar. El situs atrial fue solitus, predominó la conexión atrioventricular concordante (33), las conexiones ventriculoarteriales fueron concordantes (17), doble salida de ventrículo derecho (9) y única vía de salida (9). Todos presentaron atresia valvular pulmonar; el tronco pulmonar fue hipoplásico (20), atrésico proximal (6) y ausente (9), ramas pulmonares confluentes e hipoplásicas (25), ausencia de confluencia (5), ausencia total de ramas y de conductos arteriosos (5) y colaterales aortopulmonares (10). Los corazones mostraron un espectro morfopatológico de severidad que expresa la tendencia a la desaparición de la conexión entre el ventrículo derecho y la circulación arterial intrapulmonar. Se resaltan las formas de la irrigación arterial pulmonar como fundamento para unifocalizar el flujo hacia los pulmones y se hace hincapié en la utilidad de la clasificación anatomoquirúrgica de Barbero Marcial. El conocimiento embriológico es útil para entender las conexiones vasculares del ventrículo derecho con los derivados de los sextos arcos aórticos, los vasos arteriales intrapulmonares y las colaterales aortopulmonares.

The morphopathology of tetralogy of Fallot with pulmonary atresia is detailed as a spectrum of variations which is the foundation to highlight the surgical anatomy of this cardiopathy and it is shown the embryological basis which determines its structure. Thirty five hearts were studied with the methodology of the segmental sequential system. The atrial situs, the connections between the cardiac chambers and between the right ventricle and the arterial pulmonary vasculature were determined. The atrial situs was solitus, the concordant atrioventricular connection was the most frequent (33), the ventriculoarterial connections were concordant (17), double outlet right ventricle (9) and single outlet (9). All hearts had atresia of the pulmonary valve; the pulmonary trunk was hypoplastic (20), atretic proximally (6) and completely absent (9), confluent and hypoplastic pulmonary branches (25), absence of confluence (5), complete absence of pulmonary branches and arterial ducts (5) and presence of aortopulmonary collaterals (10). The hearts show a morphopathologic spectrum of severity which documents the tendency in disappearing the connection between the right ventricle and the intrapulmonary arterial circulation. The determination of the arterial supply to the lungs is highlighted to unifocalize the blood flow toward the lungs. The usefulness of Barbero Marcial's surgical classification is emphasized. The embryologic knowledge is basic in understanding the vascular connections between right ventricle and the derivatives of embryonic sixths aortic arches, the intrapulmonary arterial vessels and the aortopulmonary collaterals.

Anatomic diagnosis of congenital heart disease. A practical approach based on the sequentiality principle / Diagnóstico anatómico de las cardiopatías congénitas

Based on the sequentiality principle, this review proposes a practical method that allows the systematization of the anatomic diagnosis of congenital heart disease. We emphasize the need to use sequential connection between the different cardiac segments: atria, ventricles and great arteries. Five ordered steps are defined, which include determination of atrial situs and of the connection features between the ventricles and the great arteries. Related lesions and some additional special features are a second stage in the sequential analysis of congenital heart disease, which is also important for the integral diagnosis.

En esta revisión se propone, en base al principio de secuencialidad, un método práctico que permite sistematizar el diagnóstico anatómico de las cardiopatías congénitas. Se enfatiza la necesidad de utilizar la conexión secuencial entre los distintos segmentos cardiacos que son los atrios, los ventrículos y las grandes arterias. Se definen cinco pasos ordenados que incluyen, en primera instancia, la determinación del situs atrial y de las características de la conexión entre los ventrículos y las grandes arterias. Las lesiones asociadas y algunas particularidades adicionales ocupan una segunda etapa en el análisis secuencial de las cardiopatías congénitas, que también tienen importancia en el diagnóstico integral.

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.

Anomalía de Ebstein y comunicación interventricular: una asociación poco frecuente / Ebstein's anomaly and interventricular communication, a rare association

The Ebstein's anomaly is a malformation of the tricuspid valve, in which the septal and posterior leaflets are attached to the wall of the right ventricle. The usual association is with an atrial septal defect, followed by pulmonary stenosis and pulmonary atresia; the ventricular septal defect is unusual. We present three cases with diagnosis of Ebstein's anomaly and ventricular septal defect. The initial presentation of the first two was an acyanotic heart defect with congestive heart failure and increased pulmonary flow, whereas the third patient was cyanotic and functionally impaired. The three patients underwent surgery. In the first one, the ventricular septal defect was corrected with a valvular patch, a pleat of the atrialized portion, and a tricuspid valvuloplasty. The outcome was aberrant; the patient had multiple arrhythmia episodes and died five days after surgery. In the second case, only the ventricular septal defect was corrected, the patient remains under treatment and is in functional class II. In the third patient, a one and a half ventricular surgery with a tricuspid prosthesis was performed; the outcome was favorable, the patient is in functional class II. The association of Ebstein's anomaly and ventricular septal defect is unusual. The increase of the pulmonary flow is due to the size and location of the ventricular defect. A good clinical judgment is needed to make a correct diagnosis and timely treatment.

Conexión anómala de venas pulmonares. Correlación anatomoembriológica / Anomalous pulmonary venous connection

An anatomical-embryological correlation of anomalous venous pulmonary connection is presented to gain an insight of its pathogenesis. The basic publications on embryonic development of the pulmonary veins were analyzed in its two stages: the primary connection of these veins (collectors) with the embryonic systemic veins from which the definitive veins arise. These channels normally disappear once the pulmonary veins sinus is connected with the roof of the left atrium (secondary definitive connection); when the latter does not exist, any of the primitive channels persist and originates the anatomic sites of the anomalous venous pulmonary connection. The embryonic systemic veins are the right cardinal (superior vena cava and azygos vein), the left cardinal (venous coronary sinus and vertical vein) and the umbilical-vitelline (portal vein and ductus venosus). Other mechanisms are discussed such as ectopic origin of the primitive pulmonary vein in the roof of the right atrium and misplaced left of the atrial septum. The atrial septal defect is conditioned by the hemodynamics of this malformation. The knowledge of the pulmonary venous development is of great value in understanding the structure and the anatomic variants of this cardiovascular malformation in its total and partial forms.

Tronco arterioso común en adultos / Common truncus arteriosus in adults

The purpose of this study is to describe the factors that allow for the survival of patients with persistent truncus arteriosus until adult life and cared for at the National Institute of Cardiology [quot ]Ignacio Chávez[quot ]. From May 2003 to June 2007, six adult patients with clinical suspicion of truncus arteriosus were studied. All patients were subjected to clinical history, electrocardiogram of twelve leads, echocardiography, and cardiac catheterism. In these patients, the clinical symptoms began early of life. The ecocardiographic findings demonstrated the presence of truncus arteriosus type I in all cases and the most frequent associated anomalies were: hypoplasia of pulmonary branches, isolated coronary ostium, persistent ductus arteriosus and right aortic arch. Almost all patients had pulmonary hypertension. Based on this work, we conclude that until now we do not known the actual survival time of adult patients with complex congenital heart disease, such as truncus arteriosus. There are factors, such as pulmonary hypertension and hypoplasia of the pulmonary branches that are deleterious for life in some cardiovascular diseases, but in others allow for the survival of patients, as occurred in these patients with truncus arteriosus.

Espectro anatómico entre el defecto de la tabicación atrioventricular completo y parcial. Evaluación con ecocardiografía bi y tridimensional / Anatomic spectrum between complete and partial atrioventricular septal defect. Two and three-dimensional echocardiography approach

INTRODUCTION: It has been postulated that there is a morphogenetic relation between the atrioventricular septal defect (AVSD) type A of Rastelli and the type of two separated orifices, this so called partial forms, existent between both types a spectrum of anatomical forms in which interchordal spaces determinate the ventricular septal defects (VSD) size to forms in which the VSD is closed by fusion of the left septal valves to the crest of ventricular septum. METHODS: We present five patients which illustrates the variability of the atrioventricular defect by means of two dimensional and three-dimensional echocardiography. In each case was made a transesophagic echocardiogram using three-dimensional reconstruction with an Echo-Scan system (4.0 TomTec Gmb version, Munich, Germany). RESULTS: It was observed the following spectrum of atrioventricular defect: one patient had a complete closure of the VSD by the insertion of the left septal valves to the interventricular septal crest. One patient has a partially closed VSD. The last 3 patients had a large VSD with a large shunt and high pulmonary pressure. In those patients in whom the VSD was completely or partially closed, the hemodynamic behavior depended of the interatrial shunt and the regurgitation of the atrioventricular valve. They didn't present pulmonary hypertension, what allowed them to be less symptomatic. CONCLUSIONS: The three-dimensional echocardiographic study of the spectrum of AVSD type A of Rastelli, defines accurately the valve components and septal structures, so we can understand the transition between complete and partial forms. This difference determines the clinical evolution of the patients.

Sindrome de ventrículo izquierdo hipoplásico: reporte de una enferma en edad adulta / Hypoplastic left ventricle syndrome: report of a case in an adult woman

Patients with hypoplastic left heart syndrome without surgery dye during the first year of life. In the literature there is only one report from our institution of a patient who survived to the second decade of life without surgery. The purpose of this work is to describe a case of a 24 years old female with hypoplastic left heart syndrome, who has survived until now in functional class I of New York Heart Association without surgery. In our patient, the survival is probably related with the presence of a ductus arteriosus, an interatrial septal defect and the secondary pulmonary arterial hypertension.

Estudio morfopatológico de la conexión anómala total de venas pulmonares / Morphopathologic study of total anomalous pulmonary venous connection

To know the morphological features and the frequency of pulmonary anomalous connection types, 106 hearts from the collection of the Instituto Nacional de Cardiologia Ignacio Chávez were studied with the segmental sequential system. The atrial situs, connections of cardiac segments, morphology of cardiac chambers, interatrial septum, venous collector, sites of anomalous pulmonary venous connection and associated anomalies were determined. Darling's classification was used. The atrial situs was: solitus (73), dextroisomerism (30) and inversus (3). In the supradiaphragmatic level the supracardiac connection (44) was distributed into superior vena cava (24) and vertical vein (20). The cardiac level (39) was divided into venous coronary sinus (22) and to the right atrium (17). The infradiaphragmatic level was to the portal vein (10) and the mixed group (13) had different combinations. 100 hearts had atrial septal defect and 6 had patent foramen ovale. The hearts with dextroisomerism had several intracardiac malformations; 14 specimens had obstructed venous collectors. Three important aspects in diagnosis are: site of anomalous connection, size of atrial septal defect and obstruction in the venous collector; this information is valuable to understand the physiopathology, to establish the diagnosis and to help the surgeon to chose the best surgical strategy.

Hipoplasia ventricular derecha: estudios morfométrico y morfológico / Morphometric and morphologic studies in right ventricular hypoplasia

In hypoplastic right ventricle the dysplasia of the tricuspid valve (TV) has repercussions on the development of the right ventricle. This paper documents the results of an anatomopathologic and morphometric studies of the tricuspid valve and the right ventricle in 25 hearts with this cardiopathy, which were morphologically analyzed using the segmentary sequential system. The following measurements were made: the annulus of the tricuspid valve, the thickness of the walls of the right ventricle, the ventricular septum and the distances from the atrioventricular-apex (AV-A) and from the apex-pulmonary valve (A-PV). The values obtained were compared with those of normal hearts of equivalent ages; the morphologic features of the tricuspid valve and the right ventricle were described. The thickness of the anterior and posterior walls of righ ventricle was between 2 and 10 times greater than normal. The tickness of the ventricular septum was 1 to 3 times greater than normal and the anterior wall of the infundibulum from 1 to 4 times greater than normal; the AV-A and A-PV distances were reduced in a half of the normal length. The great reduction of the right ventricular cavity was found to be related to the great hypertrophy of the right ventricular walls. The TV was dysplastic with Ebstein's anomaly (72%). The size of the tricuspid valve was small and it was a good indicator for the size of the right ventricle. Morphometric studies are useful because they quantitatively document alterations in the dimensions of valves, walls and diameters of the cardiac chambers in congenital heart disease.

Aspectos clínicos de criss-cross: A propósito de un caso / Clinic aspect of criss-cross: a case report

A 1-month-old girl was referred at our Institution with a history of heart failure and cyanosis. We established diagnosis of multiple congenital heart disease with criss-crossed atrioventricular connections but concordant. This type of anomaly has a variety of presentations of high diagnostic complexity. The multiple lesions included double outlet of the right ventricle, right subaortic stenosis, ventricular septal defect, coarctation of the aorta with hypoplasia of the arch, and persistent patency of the arterial duct, established through echocardiography, angiocardiography and magnetic resonance. These findings prompted palliative surgery and correction of the aortic arch.

Tejido valvular mitral accesorio como causa de obstrucción subaórtica. A propósito de un caso / Accessory mitral valve tissue as a cause of subaortic obstruction. A case report

It is a case of a 28-year-female with severe subaortic stenosis due to accesory mitral valve tissue of the type of an accesory medial valve belove the normal anterior one. The diagnosis was performed by echocardiography.

Estudio morfopatológico de 101 corazones portadores de defecto septal interventricular / Morphopathological study of 101 hearts with interventricular septal defect

Las diversas denominaciones y clasificaciones de los defectos septales ventriculares (DSV) motivó la realización de este estudio con el propósito de contribuir a la adopción de una terminología común y de una clasificación en la que se integren criterios morfológicos, topográficos y quirúrgicos. Se estudiaron 101 defectos septales ventriculares con el método secuencial segmentario de la colección patológica del Instituto Nacional de Cardiología "Ignacio Chávez". El septum ventricular (SV) se dividió en cuatro áreas, tres musculares (entrada, trabecular y salida) y una perimembranosa. Los DSV se describieron y agruparon según su localización y el tejido de sus bordes y se relacionaron con las válvulas cardíacas y el sistema de conducción. Los DSV fueron: de tipo perimembranoso (77 = 77.23%) con los subtipos de acuerdo con las extensiones musculares: de entrada (31 = 39.75%), entrada y trabecular (25 = 32.05%) con las tres extensiones (17 = 21.80%), entrada y salida (2 = 2.56%), salida (1 = 1.28%), trabecular (1=1.28%) y a las porciones trabecular y de salida (1 = 1.28%). DSV trabeculares (9 = 8.91%), de la porción de salida (7 = 6.93%), de entrada (3 = 3.97%) y el mixto (4 = 3.96%). La clasificación topográfica estructural permite ubicar el sitio del defecto septal, determinar el tejido de sus bordes y sus relaciones con las válvulas cardíacas y el sistema de conducción. El conocimiento de la anatomía quirúrgica de esta cardiopatía es de gran utilidad para el cirujano y constituye la base para la interpretación precisa de la imagenología diagnóstica. El retardo en el crecimiento de los esbozos embrionarios que conforman el tabique ventricular y la falta de compactación septal son mecanismos patogenéticos de estos defectos.

The various terms and classifications of ventricular septal defects (VSD) stimulated us to perform the present study with the purpose of adopting a common terminology and classification in which morphologic, topographic and surgical criteria concur. One hundred and one VSD where studied by means of the sequential segmental approach from the pathologic collection of the Instituto Nacional de Cardiología "Ignacio Chávez". The ventricular septum (VS) was divided in four areas, three muscular (inlet, trabecular and outlet) and one perimembranous. The VSD where described and cathegorized according to their tissue borders, localization and relationship with the cardiac valves and the conduction tissue. The results were: Perimembranous type (77 = 77.23%), with the subtypes according to the muscular extensions: inlet (31 = 39.75%), inlet and trabecular (25 = 32.05%), with three extensions (17 = 21.80%), inlet and outlet (2 = 2.56%), outlet (1 = 1.28%), trabecular (1 =1.28%), trabecular and outlet (1 = 1.28%); trabecular (9 = 8.91%), outlet (7 = 6.93%), inlet (3 = 3.97%) and mixed types (4 = 3.96%). The topographic-structural classification permits the localization of VSD, as well as to specify their border's tissue and their relationship with the cardiac valves and conduction system. Knowledge of the surgical anatomy of VSD is useful to the surgeon, and constitutes the basis to interpret with precision the diagnostic imaging studies. Delay in the development of the embryonic primordia of the VS and the absence of septal consolidation are the pathogenic mechanisms involved.

Echocardiometric evaluation of cardiovascular abnormalities in Marfan syndrome / Valoración ecocardiométrica de las anomalías cardiovasculares en el síndrome de marfán

Marfan syndrome is an inherited disorder of connective tissue with manifestations in various organ-systems including cardiovascular system. The aim of this study was to characterize and determine the frequency of cardiovascular alterations by echocardiography in 2 age cohorts of Mexican patients with Marfan syndrome and their comparisons with control groups. Material and methods: Sixty six with Marfan syndrome and 33 control patients were evaluated by echocardiography. Segments of the aorta and pulmonary artery were measured at different levels, cardiac valves were examined for prolapse and the interatrial septum was assessed for septal aneurysm. Numeric values were corrected forthe body surface area and compared with the control group. Results: Mean significant values between group I (children) and Group II (adults) were as follows: aortic annulus 16.62 ± 4.57 mm/m² vs 12.81 ± 1.95 (p< 0.001), aortic root 23.30 ±7.49 mm/m²vs 18.36 ± 2.97 (p < 0.001), sinuses of Valsalva 24.14 ± 7.29 mm/m² vs 19.84 ± 3.59 (p < 0.001), ascending aorta 18.43 ± 5.90 mm/m² vs 17.02 ± 4.79 (p < 0.001), aortic arch 16.12 ± 4.73 mm/m² vs 14.20 ± 2.68 (p < 0.001). Pulmonary valve prolapse was seen in 10/22 (45.5%) vs 7/44 (15.9%), p < 0.03. Interatrial septal aneurysm was found in 3/22 (13.6%) vs 20/44 (45.5%), p < 0.03. There was a significative diference in the presence of atrial septal aneurysm between the adult group and control group (p < 0.001). Conclusions: The incidence of cardiovascular abnormalities in our series is similar to that in the literature with the exception of the very high incidence of pulmonary valve prolapse vs control groups, then it suggests that the clinical manifestations of MFS are strikingly severe in the Mexican population. Also a high incidence of interatrial septal aneurysm (34.9%) in comparison to control groups (18.2%) was found.

El síndrome de Marfán es una enfermedad hereditaria del tejido conectivo con manifestaciones en varios órganos incluyendo el sistema cardiovascular. El objetivo del estudio fue caracterizar y determinar la frecuencia de las alteraciones cardiovasculares mediante ecocardiografía en 2 grupos de pacientes mexicanos con síndrome de Marfán en comparación con los grupos controles. Material y métodos: Se estudiaron mediante ecocardiografía a 66 pacientes con síndrome de Marfán y 33 pacientes del grupo control. Se midieron los segmentos de la aorta y de la arteria pulmonar a diferentes niveles, se valoró prolapso valvular y presencia de aneurisma del septum interatrial. Los valores numéricos fueron corregidos por área de superficie corporal y comparados con el grupo control. Resultados: Los variables con valor significativo entre el grupo I (niños) y el grupo II (adultos) fueron: Anillo aórtico: 16.62 ± 4.57 mm/m² vs 12.81 ± 1.95 (p< 0.001), raíz aórtica 23.30 ±7.49 mm/m²vs 18.36 ± 2.97 (p < 0.001), senos de Valsalva 24.14 ± 7.29 mm/m² vs 19.84 ± 3.59 (p < 0.001), aorta ascendente 18.43 ± 5.90 mm/m² vs 17.02 ± 4.79 (p < 0.001), arco aórtico 16.12 ± 4.73 mm/m² vs 14.20 ± 2.68 (p < 0.001). Se encontró prolapso valvular pulmonar en 10/22 (45.5%) vs 7/44 (15.9%), p < 0.03. Aneurisma del septum interatrial en 3/22 (13.6%) vs 20/44 (45.5%), p < 0.03. Hubo diferencia significativa para la presencia de aneurisma del septum interatrial entre el grupo de adultos y el grupo control (p < 0.001). Conclusiones: La incidencia de anormalidades cardiovasculares en nuestra serie es similar a la reportada en la literatura excepto la alta incidencia de prolapso valvular pulmonar en relación al grupo control, lo que sugiere que las manifestaciones clínicas cardiovasculares del síndrome de Marfán son más severas en la población mexicana. Se encontró también una alta incidencia de aneurisma del septum interatrial (34.9%) en comparación al grupo control (18.2%). (Arch Cardiol Mex 2005; 75: 133-140).