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L M Gerlis

Publications and source records attributed to L M Gerlis.

72 records · Page 4Linked to original sources
PubMed1982

Left-sided obstructive lesions in atrioventricular septal defects: an anatomic study.

Actual and potential factors that create an obstruction to the flow within the systemic side of the heart have been analyzed in 114 specimens with atrioventricular septal defects. Hearts with separate right and left atrioventricular orifices and with common atrioventricular orifice were included. Potential inlet obstruction was due to tethering of the "cleft" of the left atrioventricular valve to the septum in 17.5%, incomplete "cleft" in 8%, and deformed valve leaflets in 70% of cases. Potential factors predisposing to left ventricular outflow tract obstruction were seen in 70% of cases, 21% being due to abnormal position of the papillary muscle and the remainder due to intrinsic hypoplasia of the outflow tract. Anatomic factors producing unequivocal obstruction were seen in 29% of cases. Twenty-two percent had obstructions at the inlet level, of which 13% were due to malformations of the left atrioventricular valve or the left component of a common valve. The other 9% had overall inlet obstruction due to severe hypoplasia of the left ventricle itself. Unequivocal outlet obstruction was observed in 7% of cases, 5% due to abnormal disposition of papillary muscles in the outflow tract and 2% to aneurysm of the interventricular septal remnant. Although anatomic obstruction occurs in the minority of cases (29%), this report shows that potential obstruction must be taken into account when considering surgical repairs.

Heart Septal Defects↗
PubMed1981

Two chambered left ventricle. Three new varieties.

A short review of two chambered left ventricle is given, and this rare condition is briefly compared and contrasted with the more common condition involving the right ventricle. Three cases are described, each of which presents a new variety of subdivision of the left ventricle; in two of these the condition was first shown by angiocardiography. The first case had left heart hypoplasia with dysplastic stenosis of the mitral and aortic valves and severe endocardial fibroelastosis of the main left ventricular cavity. The second case also showed dysplasia of the left sided valves with pronounced endocardial fibroelastosis, and in addition there was a congenital malformation of the left lung. The third case showed a bizarre ventricular subdivision without other congenital malformations. The role of the intertrabecular myocardial sinusoids in the pathogenesis of some types of two chambered ventricle is discussed and this is considered to have been of significance in the first two cases. The third case possibly represented an intramyocardial aneurysm of obscure aetiology.

Female↗
PubMed1979

Morphology and classification of atrioventricular defects.

Anatomical studies were made on 114 necropsy specimens of atrioventricular defects with atrioventricular concordance. The malformation is characterised by disproportion between the ventricular inlet and outlet dimensions and a malorientation of the aortic valve relative to the atrioventricular valve or valves. Associated with this there is a characteristic 'scopped-out' appearance of the muscular ventricular septum, gross abnormalities of the membranous components of the septum as compared with the normal heart, and narrowing of the aortic outflow tract. Hearts with these anatomical features can be divided into partial and complete forms depending on the morphology of the atrioventricular annuli. In the partial form the septal leaflets are conjoined to give separate mitral and tricuspid orifices, the conjoined leaflets being displaced into the ventricles and usually attached to the crest of the septum. In the complete form, anterior and posterior components of the 'septal' leaflets are separate, so that a single valve orifice connects the atrial to the ventricular chambers. Further subdivision of the complete form, apart from the morphology of the anterior leaflet, is dependent upon the presence or absence of an ostium primum atrial septal defect.

Aorta↗
PubMed1979

Morphology and classification of complete atrioventricular defects.

Anatomical studies were made on 70 necropsied hearts with atrioventricular defects from patients with situs solitus and atrioventricular concordance, all having a common atrioventricular orifice. The arterial connections were concordant in 68 and were double outlet right ventricle in two; cases with arterial discordance (transposition) or single outlet of the heart were excluded. It proved possible to subdivide the hearts, depending on the morphology of the valve leaflets. Five leaflets were distinguished by the commissural pattern and their insertion to major papillary muscles. They were a posterior bridging leaflet, right and left lateral leaflets, and right and left anterior leaflets. Subdivision was made on the basis of the disposition of the anterior leaflets. In six hearts the left anterior leaflet was committed to the left ventricle and the right anterior leaflet to the right ventricle, the commissure between them being on the crest of the ventricular septum. In 39 hearts there was minimal bridging of the left anterior leaflet so that it extended between the anterior papillary muscle of the left ventricle and the medial papillary complex of the right ventricle. In eight hearts the right margin of the left anterior leaflet was attached to an apical papillary muscle, while in 17 hearts it was attached to the anterolateral papillary muscle of the right ventricle. As the bridging of the left anterior leaflet increased, so the size of the right anterior leaflet decreased, but in all hearts both leaflets were identified. These findings were compared with previous classifications of complete atrioventricular defects.

Heart Atria↗
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↗
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

Cor triatriatum dexter with imperforate Ebstein's anomaly.

A case of cor triatriatum dexter is described in which the anomalous right atrial partition is identified as the right venous valve; there is an associated anomaly of the right atrioventricular valve which combines features of Ebstein's anomaly, and an atypical, imperforate, tricuspid atresia.

Abnormalities, Multiple↗
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↗
PubMed1975

Anatomically corrected malposition of great arteries.

Four anomalous hearts are described in which the great arteries arise in unusual fashion from their morphologically appropriate ventricles. This malformation, previously termed anatomically corrected transposition, is now termed anatomically corrected malposition. This is because, following the precedent of Van Praagh and his associates, we now reserve the term 'transposition' to describe the situation in which both great arteries arise from separate morphologically inappropriate ventricles. All the hearts examined exhibited atrioventricular concordance, I with viscero-atrial situs inversus, and 3 with situs solitus. However, there were considerable variations in ventricular morphology between the cases. Thus, 2 cases exhibited atresia of the right atrioventricular valve, and in the remaining 2 cases right and levt ventricular sinuses were both identified. Two of the cases also had pulmonary atresia, and coronary artery anomalies were present in all 4. The cases emphasize the fact that the term anatomically corrected malposition describes not a discrete anomaly but only a ventriculo-arterial relation, which is one of ventriculo-arterial concordance. Doubt has previously been cast upon the existence of this as an anatomical entity. It is concluded that the relation does indeed exist, and furthermore can coexist with all varieties of atrioventricular relations. It is suggested that the differing atrioventricular relations can be distinguished by usage of the terms 'concordant' or 'discordant' anatomically corrected malposition. Finally, it is emphasized that it is necessary to distinguish this anomaly, which in most cases presents with left-sided anterior aorta, from the left-sided anterior aorta more frequently encountered in classically corrected transposition'.

Aorta, Thoracic↗
PubMed1975

Complete heart block as a consequence of atriondal discontinuity.

We have recently studied a case of complete heart block in which there was considerable difficulty in deciding whether it was of congenital or acquired origin. This was because the heart block was first discovered at the age of 2 years in the course of acute diphtheria. Though diphtheritic infections are known to affect the cardiac conduction system, authenticated cases of post-diphtheritic block persisting after the infection are rare, and, furthermore, histopathological study in this case revealed discontinuity between the atrial tissues and the more peripheral parts of the atrioventricular conduction tissues. This has been more commonly observed in congenital cases of complete heart block and it has been postulated on theoretical grounds that this could be thebasis for congenital heart block; on the other hand, it has been noted in a single casethought to be of acquired origin. Review of the evidence available failed to allow accurate classification of the case into either congenital or acquired categories. Thenormal, segmental development of the atrioventricular node, each segment being of different embryological origin, is discussed and the case presented is understandable in thelight of this.

Adult↗
PubMed1975

The pulmonary outflow tract in classically corrected transposition.

Twenty-one specimens of classically corrected transposition have been studied in order to elucidate the morphology of the pulmonary outflow tract and the nature and origin of structures which obstruct it. The tract is an oblique channel wedged deeply between the inverted mitral and tricuspid valves. As a consequence of septal malalignment, the pulmonary valve ring overrides the muscular ventricular septum. In most specimens, the left wall of the tract is patent owing to incomplete formation of the interventricular portion of the membranous septum. This permits communication of the pulmonary artery with the left-sided morphologic right ventricle. Obstruction to the pulmonary outflow tract may be valvular or muscular or may result from the presence of fibrous tissue tags. Obstruction or atresia was present in 12 specimens (57 per cent). Fibrous tags were the most common cause, and these originated from the intact or perforated membranous septum, the inverted tricuspid valve, or the pulmonary valve. The unusual anterior relationship of the cardiac conducting tissue to the outflow tract in the anomaly is emphasized.

Heart Septal Defects, Ventricular↗
PubMed1969

Aortic hypoplasia.

Explore the source record for details and available documents.

Anthropometry↗
PubMedDate not supplied

Total anomalous pulmonary venous drainage associated with tetralogy of Fallot: report of a case.

The association of total anomalous pulmonary venous drainage and Tetralogy of Fallot is a rare occurrence; only six cases have been reported and only in one of these was the pulmonary drainage by the infracardiac route. A further such case is reported in a girl twin, with cleft palate and umbilical hernia and a normal spleen, who died at the age of three weeks and the clinical and post mortem features are described. The masking effect of the pulmonary outflow stenosis on pulmonary venous obstruction is discussed together with the relevance to clinical diagnosis and surgical intervention as "palliative" surgery may be dangerous.

Abnormalities, Multiple↗
PubMedDate not supplied

The anatomy of a collection.

An old collection of 81 congenitally malformed hearts was examined in detail using the system of sequential segmental analysis. The specimens were almost entirely from infants and young children who had died during the two decades between 1954 and 1973. There had been surgical intervention in 26 cases, and most of these patients died during or shortly after the operation. The various anomalies are listed, and the pattern of prevalence is contrasted to that of present-day collections. Among the many interesting specimens there was a fine example of one of the rarest of cardiac anomalies, a criss-cross arrangement of atrioventricular connections. The collection, as an entity, gives an interesting historical insight of the state of congenital heart disease during the early years of the development of cardiac surgery.

Adolescent↗
PubMedDate not supplied

A postmortem review of congenital cardiac malformations in a series of 180 adults, over the age of 16 years, born between 1865 and 1980.

The evolution of diagnosis and treatment of congenital heart malformations can be traced through patients surviving into adulthood. We reviewed the heart specimens from 180 patients aged 16 to 86 years and considered the morphological features, the nature of any interventional procedures, and the events leading to death. Based on the mode of clinical presentation, 33 cases were considered covert, and the remaining 147 cases were known or suspected to have a cardiac abnormality during life. Of the symptomatic cases, 60 had no surgical intervention, whereas 167 surgical procedures had been performed in the remaining 87 cases. Acquired heart disease was noted in 7 of the covert cases and in 16 of the symptomatic cases. Overall, there were only 3 instances of errors in clinical identification of significant morphological abnormalities, and 2 cases related to surgical procedures. This review emphasizes the value of autopsy examination for clinicopathologic correlations and the case for retention of cardiac specimens for teaching purposes.

Adolescent↗