PubMed Health⌕ Search

Biomedical subjects

L Muñoz-Castellanos

Publications and source records attributed to L Muñoz-Castellanos.

At least 19 recordsLinked to original sources

Congenitally corrected transposition with atrioventricular septal defect.

We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement . All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ventriculography. In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the posterior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventricular valves.

Adolescent↗

Atrioventricular septal defect: quantitative anatomy of the right ventricle.

In 19 human fetal and newborn hearts with atrioventricular septal defect (AVSD), not associated with other anomalies, the two ventricles were studied morphometrically. A total of 17 different parameters were studied: nine in the right ventricle and eight in the left. In the right ventricle we analyzed ventricular wall thickness, length of right ventricular inflow and outflow tracts, and volume of right ventricular inflow and outflow tracts. The data for these parameters were compared with the patterns of normality published previously, and the volumetric data were compared with patterns of normality published previously by us. The ventricular inflow tract was shorter than the outflow tract, the difference being especially significant in the left ventricle. The length of the diaphragmatic wall of the heart in both the right and left ventricle was equal to the sum of the length of the inflow tract and the thickness of the ventricular wall at the apex.

Anthropometry↗

Congenitally corrected transposition with mirror-image atrial arrangement.

Congenitally corrected transposition (discordant atrioventricular and ventriculoarterial connexions) with mirror-image atrial arrangement is much less frequent than the same chamber combinations in the setting of usual atrial arrangement. This scarcity of cases has made their analysis difficult. In this study we have compared the anomalies found in 19 patients with congenitally corrected transposition in mirror-image arrangement with those in 39 patients having usual atrial arrangement. Absolute, relative and attributable risks were calculated for the presence of subvalvar pulmonary stenosis, ventricular septal defect, tricuspid regurgitation, atrioventricular block or the absence of these anomalies for each of the two groups. A greater absolute risk was found for both subvalvar pulmonary stenosis (68.4 vs. 43.6%, respectively) and ventricular septal defect (63.2 vs. 58.9%, respectively). The absolute risk for tricuspid regurgitation was 25.6% in those with usual arrangement as against 21.1% in those with mirror-image arrangement. Atrioventricular block was also more frequent in those with usual arrangement (25.6 vs. 10.5%) as was absence of associated cardiac defects (17.9 vs. 10.5%, respectively). The relative risks were greater for subvalvar pulmonary stenosis and ventricular septal defect in the patients with mirror-image as opposed to usual atrial arrangement (1.57: 1.00 and 1.07: 1.00, respectively), contrasting with greater relative risk for tricuspid regurgitation (1.26: 1.00), atrioventricular block (2.43: 1.00) and absence of associated anomalies (1.70: 1.00) in the patients with usual arrangement. The attributable risk for subvalvar pulmonary stenosis was estimated to be 24.8% and ventricular septal defect 4.3% for those having mirror-image atrial arrangement.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiomyopathy, Hypertrophic↗

Truncus arteriosus communis, an unusual anatomical variant of type I.

We present a case of type I truncus arteriosus communis characterized by the horizontal origin of the main pulmonary artery from the common trunk and by a winding course up to a total length of 11.5 cm to the point of bifurcation of the pulmonary artery. Other than cyanosis the patient had a normal clinical history, which was followed for 20 years. The length of the main pulmonary artery could be related to the lower site of the incomplete septation of the common trunk, and it seems that hemodynamic factors contributed to this unusual configuration.

Angiocardiography↗

Crossed atrioventricular connections.

Four cases of crossed atrioventricular connections are described. All of them were diagnosed at cardiac catheterization by angiocardiography and one was examined pathologically. Two possessed situs solitus, one with concordant connections and the other with discordant connections; the other had two situs inversus, both of them with concordant connections. Two had double-outlet right ventricle, one had transposition of the great arteries, and the other had normally related and connected great arteries. These cases have been interpreted as representing abnormal rotation of the ventricles following sepatation. A review of 36 cases previously reported on and our own cases, suggests that most patients have concordant atrioventricular connections. There are many types of ventriculo-arterial connections, the most frequent being transposition of the great arteries. There has not been any case reported with persistent truncus arteriosus. On the basis of atrioventriculo-arterial connections, we propose a classification for this malformation. We discuss the importance of the bulboventricular loop in the type of atrioventricular connections, some clinical implications for the diagnosis, and analyze the value of the rules to localize the ventricles by means of the position of the great arteries.

Adolescent↗

Angiographic studies of atrioventricular discordance.

The results and value of conventional and axial angiocardiography were studied in 26 cases of atrioventricular discordance (AVD). The angiographic anatomy of this malformation was analyzed to determine the precise characteristics of atrioventricular and ventriculoarterial connections. The left and right ventricles in atrial situs solitus and levocardia show angiographic features that differ from those seen in atrial situs solitus and dextrocardia. The same differences are found in atrial situs inversus. Regardless of the type of ventriculoarterial connections, the ventricles maintain the same angiographic characteristics. The ventricular septum in AVD in situs solitus and levocardia and situs inversus and dextrocardia has a peculiar orientation. The use of axial angiocardiography facilitates detection and localization of the ventricular septal defect. In cases of malposition of the heart, the conventional frontal view allows visualization of the ventricular septum in all of its length. The axial projection is useful in diagnosing left ventricular outflow tract obstruction.

Adolescent↗

Ventricular inversion without transposition of the great vessels in situs inversus.

A classification of one type of congenital malformation previously reported (de la Cruz et al., 1967) is based on the integration of an embryological theory for ventricular inversions with the embryological concepts of trunco-conal malformations (de la Cruz and da Rocha, 1956). In that classification we consider that in each situs, either solitus or inversus ventricular inversions may be associated with: (a) normally arranged great vessels (not transposed); (b) transposition of the great vessels; (c) persistent truncus arteriosus. The patients had ventricular inversion without transposition of the great vessels in situs inversus.THE CORRECT ANATOMICAL DIAGNOSIS WAS NOT FORESEEN BUT THE PHYSIOPATHOLOGICAL DIAGNOSIS WAS CORRECT: pulmonary ischaemia associated with septal defects. In one case these facts were substantiated by catheterization and by angiocardiographic findings which led us to advise surgery. Haemodynamically isolated inversion of the ventricles is as severe a malformation as complete (not corrected) transposition of the great vessels. However, an operation of the anastomotic type between a systemic vessel and the narrow pulmonary artery seemed justified in these cases, as a means to convey more blood to the lungs and improve the saturation of the arterial blood. It was unsuccessfully carried out in one patient.The anatomical, radiological, and electrocardiographic features which might aid in the diagnosis are analysed.

Adult↗

[Absence of right and left atrioventricular connexion].

Fifty seven hearts with absence of atrioventricular (A-V) connection were studied morphologically to specify their types of ventriculoarterial connection and their associated anomalies; the anatomic features of the hearts were correlated with their echocardiographic and cardioangiographic images in order to establish their mutual correspondence. Fifty six hearts had situs solitus; fifty specimens had right absent A-V connection and six had left absent A-V connection. One had situs inversus. All the specimens had: A deep A-V sulcus at the site of the absent A-V connection, a dimple in the muscular floor of the involved atrium connected with the dilated and hypertrophic left ventricle, incomplete right ventricle without inlet portion, ventricular septal defect of variable dimensions (it was obliterated in two), atrial septal defect, the ventricular septum deviated from the crux cordis. The left absent A-V connection had ventricular inversion and discordant ventriculoarterial connection. In the right absent A-V connection the ventriculoarterial connections were concordant in thirty eight hearts, from which thirty four had pulmonary stenosis both infundibular and valvular (five had the tetrad of Fallot), two had pulmonary valve atresia and two had a dilated pulmonary artery; discordant in nine hearts, one with aortic atresia; double outlet, from the right ventricle in two, (one with the tetrad of Fallot) and from the left ventricle in one. The heart in situs inversus had ventricular inversion, right absent A-V connection (left-sided), single (right) ventricle and atresia of the left ventricle. The correlations between cardiac morphology and imaging were precise. Developmentally, this cardiopathy is the result of an ectopic unequally lateralized septation of the common atrioventricular canal, which separates two canals, one stenotic leading to atresia and the other which develops too wide.

Heart Septal Defects, Atrial↗

[Pulmonary atresia with an intact interventricular septum and Ebstein's anomaly of the tricuspid valve].

Patients with pulmonary atresia and intact ventricular septum have a poor prognosis with or without conventional surgical treatment. The best results of surgical treatment are obtained in those cases with a mild underdeveloped right ventricle and minor sinusoidal communication in the absence of important dysfunction of the tricuspid valve. We present five cases of pulmonary atresia with intact ventricular septum associated with dysfunction of the tricuspid valve. On the basis of radiographic, electrocardiographic and hemodynamic findings, this group of patients could not be distinguished from others without dysplasia of the tricuspid valve. Echocardiographic and angiocardiographic studies are mandatory in the differential diagnosis. A combination of systemic-pulmonary artery anastomosis associated with pulmonary valvotomy, when possible, and reconstruction of the right ventricular outflow tract are indicated for surgical solution of these malformations. However, tricuspid valve replacement is indicated in some cases.

Angiocardiography↗

[Malposition of the great arteries. Is this term a valid one?].

We present four cases of a congenital heart malformation classically known as malposition of the great arteries. This term means there are concordant ventriculo-arterial connections with abnormal spatial relations of the aortic and the pulmonary arteries, in which the former is anterior relative to the latter, and either to the right or to the left, according to the position of the corresponding ventricle. This anomaly can be found in any type of atrial situs, notwithstanding the type or mode of atrioventricular connection, as in the cases we studied. If we consider that this anomaly depends on the associated lesions in order to produce physiological heart alterations, we think that the term of malposition of the great arteries is not valid, since in the anatomic diagnosis of congenital heart disease it is important to consider the connections between atria, ventricles and arteries, the spatial relations between them being of second importance.

Adolescent↗

[Double inlet left ventricle].

Twenty-five cases of double-inlet left ventricle, 23 in atrial situs solitus and 2 with dextroisomerism are described. Twenty had both atrioventricular valves and in 3 a common atrioventricular valve connected to the left ventricle. In rest, the left atrioventricular valve was straddling over the left-sided right ventricle in about 15%. The position of the rudimentary right ventricle varied from superior, anterior and to the right of left ventricle (6 with transposition and 9 with concordant ventriculo arterial connection), to superior anterior and to the left of left ventricle (10 cases, all with transposition). Five cases had stenosis of the left atrioventricular valve and 2 stenosis of the right one. In 11, the clinical presentation was dominated by cyanosis, reduced pulmonary blood flow and right to left shunt. The rest had cyanosis and congestive heart failure. Five patients with right ventricle on the left had complete atrioventricular block, 9 right AQRS orientation and all of them findings of left ventricle hypertrophy. Three cases showed initial Q wave in VI and one left bundle branch block. Cases with right ventricle on the right, had left QRS orientation, left ventricle hypertrophy and 2 complete atrioventricular block. Cross-sectional echoes showed in 13, two atrioventricular valves committed to the left ventricle; in 3 of them the right ventricle was visualized. In 22 cases angiography determined the type of atrioventricular connection. Double inlet left ventricle is one of the most challenging diagnosis in congenital heart disease. Considering its lack of clinical specificity and according to our results, the electrocardiogram, echocardiogram and angiographic findings are of great help for its identification.

Adolescent↗

[Echocardiographic diagnosis of the right tri-atrial heart].

In this report we describe the two-dimensional echocardiographic features of a case of cor triatriatum dexter. In this situation the right atrium is divided by a membrane in two chambers. Considering the anatomic characteristics of this case we added a new type in the classical classification proposed by Doucette et al.

Cardiac Catheterization↗

[Diagnostic sequence for congenital cardiopathies].

Nomenclature for congenital heart malformations has always been confusing. Efforts of classifying these malformations according to the relations and morphology of the cardiac segments have been attempted but have risen confusion because there many terms for the same malformations. In this paper we describe a well defined sequencial diagnosis that links the cardiac segments, and besides, permits the identification of the associated defects. The connections between the cardiac segments are considered has the most important step in the classification of heart malformations while, relations are left as a secondary stage in this study. In some congenital hearts malformations concordant or discordant atrioventricular connections do not apply as the univentricular hearts or ambiguus atrial situs, but we also stablish the parameters for their classification. In our opinion, this nomenclature describe in a complete and objective way the congenital heart malformation.

Heart↗

[Anatomy of atrioventricular discordances].

Nineteen anatomic specimens were revised, 10 in situs solitus and 9 in situs inversus. The type of atrioventricular connection, atrioventricular relation, the characteristics of atrioventricular valves and ventricular cavities, the atrial and ventricular septa, the conducting tissue, the type of arterio-ventricular connections, the anatomy of the coronary arteries and associated defects were studied. The anatomic features of the ventricular cavities differ from those in the normal heart with respect to the orientation of the atrio-ventricular valve rings, the implantation of the papillary muscles in both ventricles and the extention of the membranous septum. The most frequently found type of arterioventricular connection was discordant, followed by double-outlet right ventricle, concordant connections and finally single outlet. We did not find any case of double-outlet left ventricle. It is important to know the anatomy of the conducting tissue that is in close contact with the ventricular septal defects, the most common associated anomaly in our series, and with infundibular pulmonary stenosis.

Heart Atria↗

[Morphofunctional correlation in congenital anomalies of the coronary arteries. II. The ectopic origin of the coronary arteries].

The authors describe the morphogenesis and functional alterations of the coronary arterial net in the ectopic coronary arteries: a) with origin in the aorta or its branches and b) with origin in the pulmonary artery. The coronary arteries are developed from: 1) endothelial sprouts localized in the great arteries walls at the level of the sigmoidal values, 2) right and left subepicardial vascular network and 3) the intramyocardial sinusoids. Most of the ectopic coronary arteries result from alterations in the connection between these three embryonic elements. The deviation of one of the subepicardial vascular network in a wrong way (in direction of pulmonary artery or the opposite Valsalva sinus) will stimulate the development of endothelial sprouts which will connect such network originating abnormal connections and anomalous origin of the coronary arteries. The origin of both coronary arteries from the pulmonary artery is in compatible with life. Myocardial ischemia is absent in patients with type I (infant) or type II (adult) anomalous origin of one coronary artery from the pulmonary artery, only in the transitional phase between both types (I and II) there is myocardial ischemia previous to the formation of the collateral coronary circulation. The ectopic origin of the coronary artery from the aortic Valsalva sinus have very little hemodynamic repercussion in the patient. Although there are cases with postexercise sudden dead. These anomalies associated to atherosclerotic coronary stenosis have an impact on the evolution and prognosis of ischemic heart disease.

Adult↗