[Double outlet right ventricle. Embryological approach]. / Doble salida de ventrículo derecho. Enfoque embriológico.

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°.

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

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.

[Ebstein's anomaly and interventricular communication, a rare association]. / Anomalía de Ebstein y comunicación interventricular. Una asociación poco frecuente.

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.

Understanding atrioventricular septal defect: anatomoechocardiographic correlation.

OBJECTIVE: Correlate the anatomic features of atrioventricular septal defect with echocardiographic images. MATERIALS AND METHODS: Sixty specimen hearts were studied by sequential segmental analysis. Echocardiograms were performed on 34 patients. Specimen hearts with findings equivalent to those of echocardiographic images were selected in order to establish an anatomo-echocardiographic correlation. RESULTS: Thirty-three specimen hearts were in situs solitus, 19 showed dextroisomerism, 6 were in situs inversus and 2 levoisomerism. Fifty-eight had a common atrioventricular valve and 2 had two atrioventricular valves. Rastelli types were determined in 21 hearts. Nine were type A, 2 intermediate between A and B, 1 mixed between A and B, 4 type B and 5 type C. Associated anomalies included pulmonary stenosis, pulmonary atresia atrial septal defect, patent ductus arteriosus and anomalous connection of pulmonary veins. Echocardiograms revealed dextroisomerism in 12 patients, situs solitus in 11, levoisomerism in 7 and situs inversus in 4. Thirty-one patients had common atrioventricular valves and three two atrioventricular valves. Rastelli types were established in all cases with common atrioventricular valves; 17 had type A canal defects, 10 type B, 3 intermediate between A and B, 1 mixed between A and B and 3 type C. Associated anomalies included regurgitation of the atrioventricular valve, pulmonary stenosis, anomalous connection of pulmonary veins, pulmonary hypertension and pulmonary atresia. CONCLUSION: Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography.

[Common truncus arteriosus in adults]. / Tronco arterioso común en adultos.

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 "Ignacio Chávez". 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.

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

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.

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

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.

Ebstein's Anomaly: anatomo-echocardiographic correlation.

OBJECTIVE: The aim of this investigation is to demonstrate that in Ebstein's Anomaly (EA) the right ventricle (RV) is affected in its three portions and to establish an anatomoechocardiographic correlation between the anatomic features and the equivalent echocardiographic images. METHODS: Thirty hearts with EA were studied. The alterations of each portions of the RV were described. Fifty adult patients with this anomaly were studied by echocardiography. RESULTS: Anatomy: All hearts had atrial situs solitus, 27 had concordant atrioventricular connection and 3 discordant, of these 2 had transposition of the great arteries (TGA) and one double outlet right ventricle (DORV). The degree of tricuspid valve (TV) displacement showed a spectrum from I to III. The inlet of the RV was markedly thin in 27. The trabecular portion had multiples muscular bands in all. The outlet portion was dilated in 20 and stenotic in 5. In 25 atrial septal defects were found. Echocardiography: All patients had atrial situs solitus, 42 with concordant atrioventricular connection and 8 with discordant, of these last patients 5 had TGA and 3 DORV. The degree of TV displacement varied from I to III. The inlet of RV was markedly thin in 42. The trabecular portion had muscular bands in 45. The outlet portion was dilated in 31 and stenotic in 11. In 30 atrial septal defects were found. CONCLUSION: The EA affects the whole RV and the anatomoechocardiographic correlation provides an appropriate understanding of echocardiographic images in terms of a precise diagnosis, therapeutic decisions and prognosis.

[Morphometric and morphologic studies in right ventricular hypoplasia]. / Hipoplasia ventricular derecha. Estudios morfométrico y morfológico.

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.

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

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.

[Hypoplastic left ventricle syndrome. Report of a case in an adult woman]. / Sindrome de ventrículo izquierdo hipoplásico. Reporte de una enferma en edad adulta.

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.

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

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.

Aortic obstruction: anatomy and echocardiography.

Echocardiography is a valuable non-invasive technique for identifying the site and type of aortic obstruction. Knowledge of the morphological details of each type of obstruction is the basis for correct interpretation of the diagnostic images and clinical decisions. This study was undertaken to correlate the echocardiographic images with anatomic specimens of equivalent valvular and supravalvular aortic obstruction. Specimens were part of the collection of the Department of Embryology. Fifty six patients were studied, and forty specimens with aortic obstruction were analyzed. Echocardiographic characteristics: Thirty one (55.3%) patients were women and twenty five (44.7%) men. Valvular aortic obstruction was found in Thirty six patients (64.3 %) and supravalvular aortic obstruction in twenty (35.7%). Anatomic characteristics: Of the forty specimens examined, twenty one (52.5%) had valvular aortic obstruction and nineteen (47.5%) supravalvular aortic obstruction. The anatomoechocardiographic correlation clearly showed that the anatomic findings of the specimen hearts and aortas corresponded to echocardiographic images of valvular and supravalvular aortic obstruction and provided solid corroboration of echocardiographic diagnoses.

[Clinical and echocardiographic characteristics of scimitar syndrome]. / Aspectos clínicos y ecocardiográficos del síndrome de la cimitarra.

Seven patients with scimitar syndrome underwent retrospective clinical and echocardiographic examination. The findings were: respiratory infection with dyspnea on moderate exercise in 90%, scimitar sign in four (57%), dextrocardia in five (71%), and interatrial septal defect in five (71%), one of whom had patent ductus arteriosus. Overall, two patients had patent ductus arteriosus: one also had aortic coarctation and the other, a bicuspid aortic valve. Dilation of the right cavities was found in five (71%) and blunt edge in five (71%). In two patients, anomalous drainage was into the right atrium; in another two, into the inferior vena cava; and in three, towards the junction of the right atrium and the inferior vena cava. In three patients, drainage was obstructed. Six patients with cardiac abnormalities proceeded to surgery. Scimitar syndrome is a rare entity. In the large majority of patients (86%), diagnosis and follow-up can be performed echocardiographically.

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

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.

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

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.

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

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.