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Biomedical subjects

J L Wilkinson

Publications and source records attributed to J L Wilkinson.

At least 127 records · Page 7Linked to original sources
PubMed1978

Conducting tissues in congenitally corrected transposition with situs inversus.

Three cases of congenitally corrected transposition in situs inversus individuals were characterised by visceroatrial situs inversus, atrioventricular discordance, and ventiruloarterial discordance: one case was studied clinically, and the other 2 were necropsy specimens. The disposition of the atrioventricular conducting tissues was established in each case, in the living patient by intraoperative mapping, and in the necropsy specimens by histopathological investigation. In all, the connecting atrioventricular bundle arose from a normally situated posterior atrioventricular node, normally related to the landmarks of the atrial septum. Though anterior nodes were identified in the necropsy specimens, as reported in congenitally corrected transposition in situs individuals, they differed from those in the latter situation in that they made no atrioventricular connection. These findings have obvious surgical importance. It is suggested that the posterior connection is related to the good septal alignment in these cases which lacked significant septal defects. Posterior connections are not necessarily present in all situs inversus individuals with corrected transposition, particularly when there are malalignment ventricular septal defects: further studies are required in such cases.

Aorta, Thoracic↗
PubMed1977

Pseudohypoparathyroidism. Variable manifestations within a family.

A wide variety of clinical and biochemical manifestations have been reported in association with pseudohypoparathyroidism and associated disorders. This is illustrated by a family study in which the affected members show widely differing characteristics.

Child, Preschool↗
PubMed1977

Atresia of the right atrioventricular orifice.

The results are reported of a study of 83 necropsied hearts with atresia of the right atrioventricular orifice. It is emphasised that right atrial or atrioventricular orificial atresia is a better term to describe this anomaly than "tricuspid atresia". Use of the latter term can be confusing when the morphologically tricuspid valve is located beneath the left atrium. It is accepted that the definition employed may include cases in which the mitral valve may be atretic, blocking normal exit from the right atrium, but it is argued that such cases would present clinically as "tricuspid atresia" and therefore are correctly designated as right atrial orificial atresia. The results show that the majority of hearts with right atrial orificial atresia have the ventricular morphology of primitive ventricle, most with, but a few without an outlet chamber. However, in a minority of hearts an imperforate membrane interposes between the right atrium and a formed but hypoplastic right ventricle. In two of the hearts, the imperforate membrane showed features of Ebstein's malformation. The hearts could be further subdivided according to the ventriculoarterial connection. Most had normally connected arteries (66 of 83), and all but 2 also had normal relations between the arteries; in these 2 hearts there was "anatomically corrected malposition". Twelve hearts showed transposition, one had double-outlet outlet chamber, and another persistent truncus arteriosus. The remaining 3 hearts, all without outlet chamber, had by definition a double outlet connection. A segmental approach provides the best way of classifying this anomaly, and an embryological explanation is offered for the variations in anatomy observed.

Aortic Valve Stenosis↗
PubMed1977

Surgical correction of a case of common atrium with anomalous systemic and pulmonary venous drainage.

A case of common atrium with anomalous systemic and pulmonary venous connections and pulmonary stenosis is described. Surgical correction was performed by pulmonary valvotomy and the insertion of an intra-atrial pericardial baffle. To our knowledge this is the first reported case of surgical correction of total anomalous connections of both systemic and pulmonary veins. The embryologic development of this condition is discussed.

Adolescent↗
PubMed1976

Congenitally corrected transposition of the great arteries: morphologic study of 32 cases.

The detailed anatomy of the heart is described in 32 autopsy cases of congenitally corrected transposition of the great arteries.Tis condition is defined as the combination of atrioventricular (A-V) discordance and transposition of the great arteries. Examples of primitive (single) ventricle with "inverted" (that is, left-sided in situs solitus) outlet chamber are excluded. Six hearts with A-V discordance and pulmonary atresia are described in an appendix. In 29 cases of corrected transposition the heart was in situs solitus; in 3 it was in situs inversus totalis. Only 5 of these 32 hearts had no potential for intracardiac shunting. Anomalies of the tricuspid valve (91 percent of cases), ventricular septal defect (78 percent) and pulmonary outflow tract obstruction (44 percent) occurred with sufficient frequency to be considered part of the basic malformation and are described in detail. The precise anatomy and disposition of the A-V valve tension apparatus, the coronary arteries and the conducting tissues are described with special reference to possible surgical approaches for repair of the anomalies. In two hearts with situs solitus the aortic valve was right-sided with respect to the pulmonary valve. This finding is important for both diagnosis and nomenclature.

Autopsy↗
PubMed1976

Morphogenesis of univentricular hearts.

Two main theories exist for the explanation of univentricular hearts. One states that the bulboventricular septum becomes realigned to form the interventricular septum, and that univentricular hearts are a consequence of failure of this realignment. The other states that bulboventricular and interventricular septa are different structures, and that the univentricular heart results from failure of formation of the posterior interventricular septum. Four hearts are described in which both the posterior septum and an anterior bulboventricular septum are present. In each heart, therefore, the right ventricular sinus is separated both from the left ventricular sinus and from a discrete outlet chamber which supports the pulmonary artery. It is argued that these findings militate strongly against theories proposing reorientation of the bulboventricular septum to form the definitive interventricular septum. They support strongly the concept that the definitive right ventricle is formed in part from the bulbus and in part from the primitive ventricle. On the basis of these findings, it is suggested that the distinctive feature of the univentricular heart is absence of the posterior septum. Such hearts can properly be termed 'primitive ventricle'. It is also suggested that hearts with atretic or straddling valves should be included within this category.

Heart Defects, Congenital↗
PubMed1976

The conducting tissues in primitive ventricular hearts without an outlet chamber.

We have studied the disposition of the cardiac conducting tissues in four hearts from situs solitus individuals possessing primitive ventricles without outlet chambers. These hearts correspond to the type of univentricular heart defined as common ventricle by Lev. All the hearts studied possessed normally positioned great arteries. Two groups, each consisting of two hearts, could be distinguished. The first type possessed a small posterior ridge which divided the posterior portion of the primitive ventricle into right and left ventricular sinuses. The papillary muscles to the atrioventricular valves were separate structures and arose on each side of this posterior ridge. The conducting tissues in these hearts arose from an atrioventricular node situated in the atrial septum but deviated posteriorly. The atrioventricular bundle pierced the fibrous annulus posteriorly and descended on the posterior ridge, lying to its left side. A bifurcation was not identified, and bundle branches were not present. The other two hearts had no posterior ridge. A common posterior papillary muscle supported both atrioventricular valves, and in one a marked anterior muscle bar produced obstruction of the pulmonary outflow tract. The connecting atrioventricular node was situated laterally in the right atrioventricular valve orifice, and the atrioventricular bundle descended into the right parietal wall of the primitive ventricle. A bifurcation and bundle branches were not observed. The disposition of conducting tissue in these hearts differs from that found in "primitive ventricle with outlet chamber" in that the connecting atrioventricular node and bundle are situated anteriorly and are intimately related to the transposed pulmonary artery outflow tract in the latter anomaly. The surgical significance of these findings is emphasized.

Child, Preschool↗
PubMed1976

Right juxtaposition of the auricular appendages.

3 cases are described exhibiting the rare malformation of right juxtaposition of the auricular appendages. 1 case was encountered unexpectedly during surgical repair of an atrial septal defect and the patient survived. In this instance the segmental arrangement was solitus-concordant-normally connected arteries and the only associated defects in the heart were the atrial septal defect and a persistent left superior vena cava. In contrast, the other cases were discovered at autopsy and in both the juxtaposition accompanied severe additional anomalies. Common atrioventricular canal deformities were present in ambiguus 1-loop hearts and the ventriculo-arterial connections were double outlet right ventricle with d-malposition and normal connections with d-malposition respectively. Histological study in one of the hearts confirmed the presence of 1-bulboventricular looping, and showed an unusual arrangement of conducting tissues. Two atrioventricular nodes were present, and each communicated via separate atrioventricular bundles with the ventricular conducting tissues. The cases are compared with other reported examples of right juxtaposition, and it is indicated that whereas right juxtaposition is frequently an isolated finding this is rarely the case in the more common left juxtaposition.

Child, Preschool↗
PubMed1975

Double outlet right ventricle with 1-malposition of the aorta.

Four patients are described with a recently recognized variant of double outlet right ventricle. Clinical examination favoured tetralogy of Fallot, but the chest X-ray suggested corrected transposition. Catheterization and angiocardiography showed that the aorta was to the left of the main pulmonary artery, and both arose from a normally positioned morphological right ventricle. Egress of blood from the left ventricle was through a subaortic ventricular septal defect. In all patients severe pulmonary stenosis was present and the right coronary artery ran an anomalous course anterior to the pulmonary valve ring. Two children had successful total correction, and one a palliative Blalock-Taussing shunt. Necropsy material from the fourth patient allowed confirmation of the ventricular morphology and the conducting tissued was examined. In corrective surgery, blood from the left ventricle was rerouted into the aorta by an intraventricular baffle. Pulmonary stenosis was relived by infundibulectomy and outflow tract patch.

Adolescent↗
PubMed1975

'Posterior' transposition reconsidered.

Seven cases of transposition with an unusually posterior aorta and mitral-aortic fibrous continuity are described. The cases are similar to four cases published by Van Praagh et al. in 1971. The use of the term 'p' transposition is suggested to distinguish this unusual form of transposition from cases with a subaortic conus ('d' and 'l' transposition). The use of the term 'transposition' is considered justifiable on both semantic and morphogenetic grounds and a possible common embryological mechanism for all forms of transposition is discussed.

Aorta↗