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Biomedical subjects

M Quero-Jiménez

Publications and source records attributed to M Quero-Jiménez.

16 recordsLinked to original sources
PubMed1989

Congenital mitral valve anomalies in transposition of the great arteries.

Seventy-nine specimens with transposition of the great arteries were reviewed. Among them were 45 with ventricular septal defect, including 6 with so-called "posterior aorta transposition". The morphology of the mitral valve was carefully studied. Mitral valve anomalies were found in 24 cases (30%). The anomalies involving the mitral valve were classified into 4 groups according to the different components of the valve: A) specimens with anomalies of the leaflets--2 cases with mitral cleft; B) those with anomalies of the commissures--1 case with a mitral gap; C) those with abnormalities of the chordae tendinae--2 cases of malattachment of the chordae tendinae to the ventricular septum and 2 with bridging chordae between the papillary muscles; and D) those with abnormalities of the papillary muscles--5 cases with a parachute valve, 10 with a "form fruste" of the parachute mitral valve (hypoplasia of one papillary muscle and short chordae tendinae) and 2 with abnormally placed papillary muscles, which reached the pulmonary annulus. In 14 cases, abnormalities of the tricuspid valve were also encountered, including 8 cases with a tricuspid gap and 6 with a malattachment of tricuspid chordae on the border of a ventricular septal defect. It can be concluded that mitral valve anomalies are not uncommon in transposition of the great arteries. This pathology must be carefully evaluated at the time of surgery, particularly in those patients for whom an anatomic correction is considered, for in those cases the left ventricle will be supplying the systemic circulation and therefore, a completely normal mitral valve will be required.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1982

A heart with three arterial trunks (tritruncal heart). Report of a case.

The case is described of a 4 1/2-year-old girl with a previously unreported malformation: a heart with three arterial trunks, aorta, main pulmonary artery, and an intermediate vessel, called "intermediate trunk" because it was situated between the aorta and the main pulmonary artery. Each of these three arteries had a semilunar valve at its origin, and their lumens were completely separated from each other. The pulmonary trunk arose from the right ventricle, while the aorta and the intermediate trunk arose from the left ventricle. The intermediate trunk continued as the right pulmonary artery. The pulmonary trunk continued as the left pulmonary artery. The outflow tracts of both ventricles were normal. There were therefore three arterial trunks arising from a heart with two outflow tracts. Our hypothesis on the embryopathogenesis of this case is that there was double septation of the primitive arterial trunk and the aortic sac, without involvement of the conus. In addition, there was a malalignment between the truncal and conal septa. This entity, however rare, should be considered in the differential diagnosis of absent or anomalous right pulmonary artery.

Aorta↗
PubMed1980

Wall thickness of ventricular chambers in transposition of the great arteries: surgical implications.

One hundred four specimens of complete transposition of the great arteries (TGA) were studied. The free wall thickness of the left (LV-T) and right (RV-T) ventricular chambers and the left ventricular/right ventricular (LV/RV) thickness ratio for all hearts were analyzed. A control group of 63 normal hearts was also studied. Specimens of TGA were divided into four groups: (1) TGA with intact ventricular septum (TGA + IVS); (2) TGA with ventricular septal defect (TGA + VSD); (3) TGA with a large patent ductus arteriosus (TGA + PDA); (4) TGA with pulmonary stenosis (TGA + PS). In Group I, LV-T was under the 95% confidence limits for normality after 8 months of age, and in Group II, the same was true after 18 months of age. For all groups the RV-T was above the 95% confidence limits for normality from the first month of age. The LV/RV ratio was under 1 in every group. Group I showed a significant decrease in the LV/RV ratio after the neonatal period (p less than 0.005). The potential failure of the left ventricle after anatomic correction of TGA is explained by a multifactorial hypothesis. In view of the ventricular wall thickness findings, we do not recommend the anatomic correction after 8 months of age in Group I or after 18 months in Group II.

Age Factors↗
PubMed1979

Nomenclature of the univentricular heart.

A simple system for the analysis and classification of the univentricular heart is described. The basis of this approach is the use of a step by step sequential nomenclature with attention paid to the morphology, connexions and relationships of each cardiac segment in turn. Three major morphological types of univentricular heart are identified and these may be distinguished, by their characteristic trabecular patterns, into hearts of left ventricular type, right ventricular type and indeterminate type. With the exception of the last it is usual for a rudimentary second chamber to be present upon the use of a clear definition of "ventricle", depending on readily ascertainable anatomical characteristics. Each morphological type of univentricular heart may be associated with a wide variety of different connexions and intracardiac relationships and it is neither possible nor desirable to describe these in terms of a rigidly structured classification. Adherence to the simple semantic framework outlined would resolve much of the confusion and controversy which exists in relation to the univentricular heart.

Heart Septal Defects, Ventricular↗
PubMed1979

Univentricular hearts: pathology of the atrioventricular valves.

Anomalies of the atrio-ventricular valves are frequent in cases of univentricular heart with severe symptomatology during the first year of life and in necropsy material. This suggests that they are important determinants of the natural history of the malformation. Radical "corrective" surgery, in the presence of these anomalies, may be less successful than palliative programs based on sound physiological grounds. Under such circumstances, enlargement or creation of an atrial septal defect may be necessary. A careful assessment of the state of the atrio-ventricular orifices has to be included in the cardiac catheterization protocol of cases of univentricular hearts. From a morphogenetic viewpoint, a single ventricular cavity is not the appropriate substrate for two normal atrio-ventricular valves to develop.

Cardiac Catheterization↗
PubMed1979

Ventricular morphology in the univentricular heart.

The ventricle morphology is described in hearts in which the entire atrial inputs are committed to a single chamber in the ventricular mass, this being considered the criterion for definition of the univentricular heart. Such a definition does not exclude from the univentricular category hearts with two chambers in their ventricular mass providing that the second chamber does not receive a direct atrial input. Three types of ventricular morphology were identified: hearts with a main chamber of left ventricular pattern and a rudimentary chamber of right ventricular pattern; hearts with a main chamber of right ventricular pattern and a rudimentary chamber of left ventricular pattern and hearts with a sole chamber in their ventricular mass of indeterminate pattern. Hearts may also exist of right or left ventricular type without rudimentary chambers. Variation was noted in type and position of the rudimentary chambers. Chambers of either right or left ventricular type were found supporting either or both great arteries (outlet chambers), and also without a direct outlet (trabecular pouches). Right ventricular rudimentary chambers were usually anterior but could be right or left sided; left ventricular chambers were posterior but again could be right or left sided. Hearts with these ventricular morphologies possessed either double inlet atrioventricular connexions, or absence of the right or left atrioventriuclar connexion. In double inlet ventricles, cases were observed with two valves, a common valve, one imperforate valve, and with straddling atrioventricular valves. A straddling valve was also found in hearts with absence of one atrioventricular connexion. Further variation was found in the pattern of arterial connexions. It is concluded that ventricular morphology is an important feature of the iniventricular heart but must be considered in the context of other important and variable features.

Heart Septal Defects, Ventricular↗
PubMed1979

Problems in the nomenclature of the univentricular heart.

There are three main reasons why there are problems concerning the nomenclature of the univentricular heart. The first is that different authorities have used the same term to describe different varieties of the univentricular heart. The second is that many of the terms used in categorisation of these anomalies are derived from embryological concepts which are more or less speculative. The final reason, perhaps the major one, is that there is a lack of definition of a ventricle. Consequently there is no consensus as to precisely what constitutes a "univentricular" heart. Many of the heart described as "single" or "common" ventricle possess two chambers within their ventricular mass. For such hearts to be univentricular it is evident that one of these chambers cannot be a ventricle. Yet definitions have yet to be provided to distinguish ventricles from rudimentary chambers. The solutions to these problems can be produced firstly by using descriptive terms for categorisation of univentricular hearts which do not have multiple meanings. Secondly, the problems concerning usage of embryologic terms can be easily circumvented by eschewing all embryologic considerations in naming these malformations. The problem of definition of a ventricle is best solved by considering normal ventricles as possessing three portions -- an inlet portion, a trabecular portion and an outlet portion. It may then be proposed that the minimal requirements for a ventricle are that it possesses at least an inlet and a trabecular portion. Chambers without inlet portions are rudimentary chambers. Either ventricles or rudimentary chambers may possess a trabecular pattern of right or left ventricular type. Tue univentricular heart may then be categorised as composing all anomalies which have a sole chamber in their ventricular mass together with all anomalies which possess a rudimentary chamber.

Heart Septal Defects, Ventricular↗
PubMed1979

Familial hypertrophic cardiomyopathy with unusual involvement of the right ventricle.

4 cases of familial hypertrophic cardiomyopathy are reported. In all of them, the onset of symptoms occurred before the 8th mth of life, and were characterized by severe right cardiac failure. In 3 cases the catheterization revealed significant decrease in right ventricular compliance and severe reduction of its cavity. The pathological studies were consistent with hypertrophic cardiomyopathy with predominant involvement of the right ventricle. The pedigree disclosed a Mendelian autosomal recessive type of inheritance.

Cardiomyopathy, Hypertrophic↗
PubMed1975

Isolated ventricular inversion with situs solitus.

The clinical and anatomical findings in two patients with isolated ventricular inversion and situs solitus are described. The other 4 previously published cases are reviewed. The 6 patients with this malformation, all without pulmonary stenosis, presented a clinical picture of cyanotic congenital heart disease, associated with increased pulmonary blood flow (hypoxaemia and cardiac failure). The importance of different diagnostic tests is discussed and it is concluded that angiocardiography is the only definitive means of establishing the dianosis. Because the physiopathological disturbance is the same as in transposition of the great arteries, both malformations should be similarly considered with respect to diagnosis and treatment. Nevertheless, the high incidence of certain associated malformations in cases of isolated ventricular inversion adds to difficulty in diagnosis, and makes a good result from the Mustard procedure less likely than in transposition of the great arteries.

Angiocardiography↗
PubMedDate not supplied

[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↗