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Biomedical subjects

L Muñoz Castellanos

Publications and source records attributed to L Muñoz Castellanos.

At least 19 recordsLinked to original sources

[Persistent common trunk. Anatamopathological study of 25 specimen].

In order to inform the pathologic features and their associated anomalies twenty five hearts with common trunk were studied with the segmental sequential system. An anatomico-embryological correlation was made to understand the pathological complex of this malformation. The results were: type I truncus (96%), infundibular ventricular septal defect (96%), displastic truncal valve (28%), ventriculo infundibular fold (92%), left coronary artery arising from the posterior wall of the truncus (75%), right coronary artery arising from their anterior wall (96%), coronary arteries arising from opposite Valsalva sinuses in the tetracuspid valves; the biventricular conection of the truncus was balanced in 60%, prevailing on the right (16%) or on the left (16%) ventricles. Among the associated anomalies there were right aortic arch, interruption of the aortic arch, anomalous origin of the left subclavian artery, vascular ring, aneurysm of the sinus of Valsalva, and absence of the left branch of the pulmonary artery. Developmentally common trunk is explained as a failure of truncoconal septation in the embryonic heart; a migration arrest of neural crest cells is implicated in the Di George syndrome. Knowledge of the anatomic features of common trunk and their associated anomalies, provides the morphological basis to interpret correctly the clinical diagnostic imagenology.

Aorta, Thoracic↗

[Anatomopathologic-embryologic correlation in atrioventricular connection absence].

In order to offer a pathogenetic explanation for the absence of atrioventricular connexion, a correlation was made between the pathologic anatomy of this cardiac malformation and the embryonic processes which take part in the septation of the atrioventricular canal and the development of atrioventricular connections. The correspondence was made between the development of the canal's atrioventricular cushions, the septation of the common atrioventricular canal, the right and left atrioventricular canals and the morphogenesis of the mitral valve, all these processes were correlated with the anatomic elements derived from them. This led to infer that the malposition of the atrioventricular cushions divide the common atrioventricular canal unequally, giving rise to a narrow canal becoming atretic and a big canal where the mitral valve is evolved. The extreme lateralization of the atrioventricular septum to the right side would led to the absence of the right atrioventricular connection and the same process but to the left side, would give rise to the left absence of the atrioventricular connection. This ectopic septation process is supported by similar ones which can occur in other segments of the heart such as in tetralogy of Fallot and the transposition of the great arteries. This hypothesis explains sufficiently the pathologic anatomy of this type of congenital heart disease.

Heart Septal Defects↗

[Double inlet left ventricle. Morphopathology and surgical anatomy].

Because of the possibility of surgical treatment of double inlet left ventricle, its basic morphologic features of surgical and imaging importance are analyzed. Seventeen hearts were studied with the segmental sequential system. The situs was solitus in thirteen; dextroisomerism in three and levoisomerism in one. The atrioventricular valves were separated in nine; there was a common atrioventricular valve in eight; straddling of the atrioventricular valve in seven and stenosis in two. The rudimentary right ventricle was to the right side in twelve hearts and to the left side in five. The discordant ventriculoarterial connection was the most frequent (seven), followed by the concordant one (five) and double outlet right ventricle (five). There was pulmonary stenosis in five. The ventricular septum did not reach the crux cordis. The ventricular septal defect was bigger in straddling atrioventricular valves than in valves completely open into the left ventricle, being restrictive in ventriculoarterial concordance. Surgical treatment varies as the complexity of this cardiopathy does, from cardiac septation to palliation procedures. The morphologic knowledge of this cardiac malformation is basic to interpret correctly the diagnostic imaging.

Cardiac Surgical Procedures↗

[Absence of the tricuspid valve. A case report].

An anatomical embryological and histological study of a malformed heart is presented in which the right concordant atrioventricular connexion is unguarded by the tricuspid valve, due to absence of the three leaflets, chordae tendinae and papillary muscles; only the fibrous ring is present in the atrioventricular junction. This very rare malformation is associated with atresia of the valve connected with the right ventricle generally being the pulmonary valve; the ventricular septum is intact. This congenital lesion always present fibrosis in the free wall of the right ventricle. We believe a primary pathogenetic step occurs in the wall of the right ventricle which prevents the morphogenesis of the tricuspid valve from this ventricle. This malformation must be distinguish from Ebstein's disease and Uhl's disease, although they are pathogenetically related.

Diagnosis, Differential↗

[Bilateral infundibula in congenital cardiopathies].

The goal of this paper has been to count and describe congenital heart disease associated with double conus. We studied 196 pathologic specimens from to the Pathologic Collection of the Department of Embryology of the National Institute of Cardiology "Ignacio Chávez" of México. We found 3% of double conus in classic tricuspid atresia with concordant ventriculoarterial connection, 53% with double outlet right ventricle, 16% with discordant ventriculoarterial connection, 91% with double outlet single ventricle, 8% with one outlet right ventricle with pulmonary atresia, 20% with one outlet left ventricle and 25% with one outlet single ventricle. The morphological data of the associated anomalies were gathered in an informatized sheet designed according the segmental and sequential approach to diagnose congenital heart disease. From our results we conclude that double conus cannot be considered pathognomonic of any congenital cardiopathy, although they appear associated more frequently with truncoconal anomalies and single ventricle. It is frequent the association between double conus and stenosis of one of them, specially the subpulmonary one. We found great association between left juxtaposition of the atrial appendages and double conus.

Electronic Data Processing↗

[The pathological remnants of the right sinus venosus valve. A presentation of 6 hearts of the cor tritriatum dexter type].

Six hearts specimens of cor triatriatum dextrum, eight with the Chiari's network, and 3 with a membranous remnant of the crista terminalis are studied. These anomalies are interpreted as varying degrees of persistence of the right valve of the sinus venous which reflect different stages of their morphogenesis. The most frequent congenital heart defects associated with these sinus remnants are the absence of a right atrioventricular connexion and pulmonary atresia with intact ventricular septum. There may be some haemodynamic factors in the right atrium such as partial of complete obstruction between the atrium and the right ventricle which explain the fact that the valve is not reabsorbed. In our specimens the persistent right valve divided the right atrium into two compartments; one is located in the medial sinusal portion where both cavae veins and coronary sinus are connected; the other is lateral compartment which represents the primitive right atrium. It must be noted that the persistent right valve favors blood flow to the left atrium through the patent foramen ovale or through an atrial septal defect deviating the course of the blood away from the right ventricle. This could cause the hypoplasia of the right ventricle. When the valve bulges it obstructs the blood flowing into the right ventricle. The diagnosis of these malformations can be made by echocardiographic, angiocardiographic procedures and nuclear magnetic resonance.

Cor Triatriatum↗

[Juxtaposition of the atria].

Left juxtaposition of atrial appendages is an anomaly of their origin and position which is associated with a high incidence of complex congenital heart defects. We studied six cases of juxtaposed right atrial appendages. They emerged from the anterior wall adjacent to the atrial septum and were situated to the left of the origin of the great arteries. Associated congenital heart defects were: single outlet, right ventricular hypoplasia, absent right atrioventricular connection, ventricular and atrial septal defects and pulmonary outflow obstruction. Other abnormal morphological features present were anatomically corrected malposition of the great arteries, bilateral infundibulum and right aortic arch. Although the association of these malformations with juxtaposed atrial appendages does not per se constitute a syndrome, its presence is an ominous sign of severe cyanotic congenital heart disease which should alert both clinicians and surgeons. There are various possible embryological explanations for the genesis of this malformation. We believe that juxtaposition is the consequence of the ectopic origin of the atrial appendage rudiment, which in the presence of truncal alterations grows behind and to the left of the great arteries.

Heart Atria↗

[Aortic atresia. Presentation of 7 cases].

The clinical and anatomic findings of 7 patients with aortic atresia were studied. All cases had usual atrial arrangement, atrioventricular and ventriculoarterial concordance. All but one had an intact ventricular septum. All cases presented a hypoplastic left ventricle, and in one, a mitral atresia was found. The clinical diagnosis was made by means of cardiac catheterization and angiocardiography. The presence of a patent ductus arteriosus, the size of the atrial and ventricular septal defects, the diameter of the ascending aorta, pulmonary vascular resistances and right ventricular function are all factors that influence the survival in the patients with aortic atresia.

Abnormalities, Multiple↗

[Scimitar syndrome].

We studied 15 cases of the scimitar syndrome. The diagnosis was suspected in 87% of the patients by x-rays studies. Bronchography is useful in order to detect bronchial anomalies, such as hypoplasia or agenesis of the medial lobe which is responsible for the left isomerism. The hemodynamic study is important in symptomatic patients in order to demonstrate associated congenital heart disease as well as the degree of pulmonary hypertension. Angiography is fundamental in determining the anomalous venous drainage of the right lung, whether complete or partial, and in demonstrating the exact site of the connection which may be to the right atrium or more frequently to the inferior vena cava above or below the diaphragm. There are variant forms in so far as the pathway, extension and connection of the anomalous venous trunk which gives rise to the scimitar sign. This syndrome is due to a persistent connection of the right pulmonary veins to the proximal portion of the right vitelin vein which normally forms the suprahepatic segment of the inferior vena cava. Asymptomatic patients should have medical treatment symptomatic ones with an associated congenital heart disease and moderate pulmonary hypertension must be subject to surgery.

Adolescent↗

[Ebstein's anomaly in infants].

Ebstein's disease is a congenital malformation of the tricuspid valve that causes various hemodynamic alterations depending on the anatomic changes in the valve, presence or absence of atrial septal defect and reduction in the ventricular function. We present 19 patients with Ebstein's malformation of the tricuspid valve younger than 2 years of age. The long-term follow-up was from 1 week to 16 years with a mean of 40 months. Eight children died, 6 were lost in the follow-up and 5 survived. Early cyanosis with associated lesions give a bad prognosis. If the child survives the first year of life he has a better long term prognosis.

Arrhythmias, Cardiac↗

[Surgical anatomy of tetralogy of Fallot].

We analyzed 131 hearts with tetralogy of Fallot for their prominent surgical anatomical features. Special attention was paid to the different elements of the right ventricular outflow tract obstruction, the supraventricular bands and types of septal defect and their relations with the conducting tissue and coronary supply. The infundibular region proved to be the core of the malformation in tetralogy, the left and anterior septal deviation being the origin of pulmonary stenosis and the rest of the embryopathological elements that characterize this malformation. Our results support the notion that each case has, besides the general features of the malformation, particular aspects which must be analyzed. This conjunction of general and particular features provides the clinician with a morphological basis for the interpretation of the diverse diagnostic procedures and the surgeon with the general scope of tetralogy and the individual features of each case which must be taken into account in the choice of the best possible surgical procedure.

Humans↗

[Pulmonary atresia with interventricular communication. Review of concepts].

The purpose of this paper is to clarify the definition and anatomic characteristics of pulmonary atresia with ventricular septal defect. This complex congenital cardiac malformation produces great controversy in the literature. We analyze some embryological aspects, the right ventricular outflow tract anatomy and pulmonary arterial pattern with the various patterns of collateral blood supply. A good understanding of these anatomic characteristics is of great importance in the hemodynamic and angiographic diagnostic procedures and in the planning of surgical correction. The differential diagnosis with other congenital heart diseases with single outlet from the heart is discussed.

Collateral Circulation↗

[Axial angiography in tetralogy of Fallot].

We studied 15 patients with the diagnosis of tetralogy of Fallot by means of conventional and axial angiocardiography. Axial studies were performed in the four chamber view (vertical and horizontal X-ray beams) and in the sitting-up view (vertical X-ray beam). With the right ventriculogram we analyzed the anatomic characteristics of this chamber, infundibular stenosis and the pulmonary branches. Left ventriculograms were performed in order to rule out other less common associated defects. In the sitting-up view we analyzed the characteristics of the right ventricular infundibulum and the pulmonary valve, artery and branches. None of our patients underwent aortography or coronary artery angiography. We conclude that utilization of axial angiocardiography is useful in tetralogy of Fallot because it permits a better appreciation of the anatomy of this malformation, the presence or absence of stenosis of the pulmonary branches and other less frequent associated defects. Aortography or coronary artery angiography must be performed in children in which the anatomy of the coronary arteries cannot be determined by either left or right ventriculograms.

Angiocardiography↗

[Spectrum of biventricular aortic connection and double outlet chamber of the right ventricle produced experimentally in the chicken heart by hypothermia (34.5 degrees C)].

In this experimental work, fertilized chicken eggs were subjected to hypothermia (34.5 degrees) as to inhibit the incorporation of the aortic infundibulum into the left ventricle. This produced a spectrum of biventricular connection of the aorta including double outlet right ventricle (DORV), a cardiopathy seen naturally in man and in chicken. It represents the persistence in the postnatal heart of the spectrum of embryonic aortic dextroposition, a fact which allowed us to establish a precise anatomoembryologic correlation. Pathogenetically, the failure in the connection of the aortic infundibulum with the left ventricle is due to inhibition, in different degrees, of the leftward morphogenetic movement of the infundibular segment, an embryologic process which is discussed in relation to different theories on the origin of DORV. The role played by cell death in normal and pathologic morphogenesis, is emphasized and some methodological aspects on experimental teratogeneses are mentioned.

Animals↗