[Atrial rhythms in heart abnormalities].
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Biomedical subjects
Publications and source records attributed to M Quero.
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Out of 1610 children's hearts with congenital malformations there were 27 specimens showing double outlet right ventricle. Cases with dextrocardia, situs inversus, or l-venticular loop were excluded. Anatomical examination was performed with particular reference to the infundibular region, the great vessels, and the ventricular septum. The commonest associated malformations were ventricular septal defect and pulmonary stenosis. Aortic stenosis was the predominant finding in those cases dying in the neonatal period. An aortic conus was associated with pulmonary stenosis, ventricular septal defect, and d-transposition, a pulmonary conus with ventricular septal defect and a double conus with stenosis of either great vessel. The anterior vessel always had a muscular conus and the posterior vessel was commonly stenotic.
An anatomical study of 54 specimens with endocardial cushion defect is described. The material was divided into two groups, according to the morphology and attachments of the anterior component of the anteroseptal mitral leaflet. We suggest that there is no divided or undivided anterior common leaflet. There are always two anterior leaflets, a mitral and a tricuspid one, separated by a commissure; depending on the degree of straddling of the mitral component over the interventricular septum, this will protrude into the interventricular septum or into the anterior papillary muscle of the right ventricle. Thus, the commissure separating the mitral and the tricuspid components will lie over the interventricular septum or will be quite separate in the right ventricle. In the latter case the straddling mitral component gives the false appearance of an undivided anterior common leaflet. The septal tricuspid leaflet was found to be underdeveloped or absent but we were not able to find any clefts or divisions in it. We suggest that this leaflet derives solely from the division of the posteroinferior endocardial cushion into two halves, right and left. The left half later becomes the posterior component of the normal anteroseptal mitral leaflet. Associated anamalies are also reviewed.
Eighteen cases of mitral atresia with normal aortic valve plus 68 cases from the literature are analyzed. A new classification based on anatomical findings is proposed. Pulmonary stenosis or atresia is frequent in the type with transposition of the great arteries. The rare instances of normal or large left ventricle are due to a large ventricular septal defect, or to straddling or displaced tricuspid valve. The clinical, radiologic and electrocardiographic findings have been summarized and correlated with the different physiopathological situations. In our catheterized cases an oxygen saturation step-up was found in the right atrium together with left atrial hypertension. In seven cases mitral atresia was demonstrated by selective left atrial contrast injection. The average age at death was six months. Among the cases surviving one year or more, the association of atrial septal defects or pulmonary stenosis was frequent. Enlargement of the interatrial communication, accompanied by other palliative measures, is the only available surgical procedure.
A case of aorticopulmonary window associated with tetralogy of Fallot is reported with its clinical, hemodynamic, angiocardiographic, surgical, and necropsy findings. The difficulty in diagnosing a tetralogy of Fallot in the presence of a large aorticopulmonary shunt is emphasized. It is also noted that the only means of discovering the infundibular stenosis of the right ventricle is by angiocardiographic study of the right ventricular outflow tract. The difficulty in distinguishing truncus arteriosus from an aorticopulmonary window coexisting with a ventricular septal defect is discussed, and it is pointed out that the only means of differentiating these two anomalies is the angiocardiographic finding of two sigmoid valves. An embryological hypothesis concerning the pathogenesis of this association is also discussed.
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The first well-documented case of Holmes heart associated with an atretic left atrioventricular orifice is reported. The Holmes heart may be considered as a single ventricle in which the main chamber has the morphologic characteristics of a left ventricle, the infundibular chamber is not inverted, and the great arteries are normally arranged. The importance of this report is greater than its simple description since it definitely establishes the coexistence of a true single ventricle and atresia of the atrioventricular orifice is possible.
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