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Biomedical subjects

L M Gerlis

Publications and source records attributed to L M Gerlis.

At least 19 recordsLinked to original sources
PubMed2001

Unusual origin and course of the left pulmonary artery.

An infant with a cardiac murmur was found to have a patent arterial duct and an anomalous left pulmonary artery. The duct was surgically ligated at the age of 8 months, and she remained free of cardiac or respiratory symptoms up to her death at 6 years from an unrelated intestinal condition. An anomaly was discovered at post mortem examination; the left pulmonary artery arose from the right pulmonary artery and passed behind the trachea to enter the left lung. We describe and illustrate the anatomic features of this well-recognized entity, discuss the embryological substrate, and refer to the clinical implications.

Child↗
PubMed2001

Preductal aortic coarctation and patent ductus arteriosus in a Sumatran tiger (Panthera tigris sumatrae) cub.

Preductal aortic coarctation and patent ductus arteriosus are described in a neonatal Sumatran tiger, Panthera tigris sumatrae. Eight days postpartum, the cub appeared weak, and it was separated from the dam for hand rearing. On examination it was dehydrated and hypothermic. Despite treatment, the animal's condition worsened and the cub died 12 days postpartum. Gross postmortem and histologic examinations revealed a preductal aortic coarctation and patent ductus arteriosus with a patent foramen ovale and moderate dilatation of the right ventricle of the heart. Focal pneumonia and mild hepatitis were also present; however, diffuse pulmonary congestion and edema were considered to be the proximate cause of death.

Animals↗
PubMed2000

Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases.

We present two cases in which there was isomeric arrangement of the left atrial appendages in association with multiple spleens and complete heart block. In both of these, the venous connections were normal. In one case the arrangement of the bronchial tree and the lungs was normal, the liver was left-sided, and the stomach was right-sided. In the other case, there was left isomeric broncho-pulmonary morphology, and both liver and stomach were right-sided. These unusual combinations show the need for full description of the morphology of other organs, and the venoatrial connections, in cases with isomeric arrangement of the atrial appendages. They also demonstrate that connection of the inferior caval vein cannot be taken as a reliable marker of an atrium having a morphologically right appendage.

Abnormalities, Multiple↗
PubMed1999

Complex pulmonary atresia in an adult: natural history, unusual pathology and mode of death.

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistuious communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.

Aortic Dissection↗
PubMed1999

A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification.

This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.

Calcinosis↗
PubMed1999

Morphology of the elastic pulmonary arteries in pulmonary hypertension: a quantitative study.

Recent studies of intravascular ultrasound of the pulmonary arteries suggest that the technique can detect intimal and medial thickening in patients with pulmonary hypertension, potentially providing a method of assessing severity of pulmonary vascular disease in life. A major drawback of the technique is that only the elastic pulmonary arteries are accessible to current ultrasound catheters. The aim of this study was to determine whether morphological changes in vessels accessible to intravascular imaging reflect severity of pulmonary vascular disease and are of a sufficient degree to be detectable by current ultrasound catheters. Morphometric studies of the elastic pulmonary arteries were performed in specimens of lungs from 24 patients who had died with pulmonary hypertension (aged 3 weeks-9 years) and compared with measurements from infants who had died from sudden infant death syndrome. Morphological changes evident in the elastic pulmonary arteries in pulmonary hypertension included luminal dilation and medial thickening but these changes were too variable to be predictive of the severity of peripheral pulmonary vascular disease. Intimal thickening and atherosclerosis were present only in those with advanced pulmonary hypertensive disease. The changes of medial thickening and luminal dilation, nonetheless, are too variable to be predictive of the severity of peripheral pulmonary vascular disease. Thus, imaging by intravascular ultrasound may help confirm advanced pulmonary vascular disease, but is unlikely to differentiate less severe pulmonary vascular disease when the patient may still potentially be operable.

Child↗
PubMed1999

Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum.

An apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum. Histological examination showed a normal atrioventricular node and bundle, adjacent to the aneurysm. There was fibrous interruption at the commencement of the right bundle branch, which was considered the basis of the bundle branch block. It was also considered that the right bundle branch block was of the hereditary type and that this, rather than the aneurysm of the membranous septum, was responsible for the unexpected death.

Adult↗
PubMed1998

Anatomic conundrum in a case of complete transposition of the aorta and pulmonary trunk.

An unusual cord-like structure was found on postmortem examination of the heart of a 30-year-old woman who had complete transposition of the great arteries. This extended from the posterior aspect of the right side of the base of the aorta to the posterior wall of the distal part of the arch immediately proximal to the insertion of the arterial ligament. The nature and origin of this is discussed.

Adult↗
PubMed1998

Anatomical conundrum: unusual position of the aortic arch.

At autopsy on a 72-year-old woman, the aortic arch showed an unusual arrangement, passing behind the trachea and esophagus and descending on the left side. The heart was normal, and death was due to unrelated causes. The nature of this anomaly is considered in respect of embryological development.

Aged↗
PubMed1998

The Brock procedure (closed infundibular resection) for Fallot's tetralogy: 43 years later.

Innovative cardiac surgery ('blind' or indirect infundibular resection) for tetralogy of Fallot on a child of 4 years was followed by survival for 43 years without further surgery. The patient remained well until about one year before death, when he developed clinical features of progressive biventricular failure associated with pulmonary hypertension and incompetence of the pulmonary and tricuspid valves. Postmortem examination showed severe damage to one of the leaflets of the pulmonary valve, interpreted as due to inadvertent avulsion during the original surgical procedure. A large ventricular septal defect was present, but there was no residual subpulmonary infundibular obstruction.

Autopsy↗
PubMed1996

Fibrous matrix of ventricular myocardium in tricuspid atresia compared with normal heart. A quantitative analysis.

BACKGROUND: The collagen matrix is a small component of the myocardium, but it provides a supportive framework. An increase in collagen in the pressure-overloaded ventricle is known to cause myocardial stiffness. However, little is known about the collagen matrix in the volume-overloaded ventricle, particularly in relation to congenital heart disease. METHODS AND RESULTS: We examined a total of 53 hearts with tricuspid atresia and 58 normal hearts matched for age. Using a microscopic-morphometric method, we analyzed the percentage per field area occupied by interstitial fibrous tissue in four sites in the ventricular mass for each specimen. A comparison of sampling sites showed no significant variations between normal and malformed hearts. Results from a homogeneity of regression co-efficients analysis suggested that the two groups shared the same basic relation of proportion of fibrosis with age. The use of ANCOVA, however, revealed a clear separation between the extents of fibrous tissue in the two groups of hearts. CONCLUSIONS: The myocardium of hearts with tricuspid atresia is consistently more fibrotic than normal heart and is probably an inherent part of the malformation. This difference could explain, at least in part, the clinical observation that the left ventricle is frequently abnormal, even at an early age.

Adolescent↗
PubMed1996

Surgical anatomy of the coronary circulation in hearts with discordant atrioventricular connections.

We examined the arrangement of the coronary arterial and cardiac venous systems in 46 specimens with discordant atrioventricular connections so as to identify any structural abnormalities and to consider their surgical implications in terms of anatomical biventricular repair. Grossly abnormal arterial courses were seen in 11 hearts (24%). A substantial branch supplying the morphologically right ventricular outflow tract, which could restrict a ventriculotomy, was found in 61% of cases. The coronary sinus received all the morphologically right ventricular veins, as well as the posterior interventricular vein, in 40 hearts, this pattern being in contrast to the pattern in the normal heart. The morphologically left ventricular and anterior interventricular veins, all of which drain via the coronary sinus in the normal heart, were frequently connected independently to the morphologically right atrium in the specimens with discordant connections, the drainage occurring through the spaces between the pectinate muscles. These direct drainages are at risk of potential damage either by extensive intra-atrial maneuvers or by postoperative intraatrial thrombosis. It is predicted, therefore, that surgical results can be improved still further when account is taken of this vascular anatomy of the heart itself.

Cadaver↗
PubMed1995

Pathologic elucidation of the echocardiographic features of Ebstein's malformation of the morphologically tricuspid valve in discordant atrioventricular connections.

We defined the morphology of the left atrioventricular valve in Ebstein's malformation associated with congenitally corrected transposition to elucidate the approach to diagnosis by echocardiography. We found 14 unequivocal cases out of a total of 3,720 specimens. We noted the atrial arrangement, displacement, and nature of the tricuspid leaflets, and axis of the plane of the tricuspid orifice. We constructed ratios of volume of the atrialized to the functional right ventricle, and of the right to left ventricle, as well as distances of the septal attachments of the atrioventricular junctions to the respective ventricular apices. Three specimens had abnormalities of cardiac position, and 8 had ventricular septal defect. The tricuspid valve plane was rotated 47 +/- 21 degrees from its usual position into the ventricle. The tricuspid valvar tissue was variably attached to the underlying myocardium, with the most severely affected lesion being the mural leaflet followed by the septal leaflet, and the anterior leaflet attachment the least affected. Abnormalities of the tendinous cords and the effective valvar orifice occurred in 3 specimens. The ratio of the atrialized to the functional right ventricular volume was 0.74 +/- 0.49; the ratio of the fetal right to left ventricular volume was 1.18:1 +/- 0.70:1. These data suggest that plastic repairs of the right ventricle would leave a small functional right ventricle, but that valve replacement could restore the volume of the ventricle. Thus, the plane of displacement of the valve in corrected transposition appears less amenable to 4-chamber echocardiography than other forms of Ebstein's malformation. Changes in the echocardiographic planes should display the morphology and also provide some hemodynamic information.

Adult↗
PubMed1995

Fibrous obstruction within the left ventricular outflow tract associated with ventricular septal defect: a pathologic study.

OBJECTIVES: We examined the nature of ridges within the left ventricular outflow tract associated with ventricular septal defects that might be found by echocardiography. BACKGROUND: Echocardiography displays even small ridges well. Surgical removal of such ridges at the time of defect closure is recommended. METHODS: We examined 37 heart specimens with ventricular septal defects with a ridge, noting its nature and relation to the defect and adjacent valves. We excluded left ventricular outflow tract obstruction associated with complex lesions. RESULTS: Defects were perimembranous in 25 specimens, muscular in 8 and part of an atrioventricular septal defect in 5. Some hearts had multiple defects. Many of the original reports had not mentioned ridges. Three distinct ridge patterns were found. The first (n = 18) was a fold of endocardial tissue related to the membranous septum. The second (n = 12) was a defect of a fibrous nature; in 8 this was a discrete, protuberant fibrous ridge, and in 4 the obstruction was diffuse, which we termed keloidal. The third pattern (n = 7) lay circumferentially around the ventricular septal defect, seemingly associated with the defect's attempted spontaneous diminution in size. Endocardial folds were not found in specimens from patients > 5 years old. Fibrous and keloidal lesions, which may represent a continuum of progression, generally were found in specimens from older patients. Histologic studies of 17 specimens confirmed the morphologic findings. The endocardial folds were endothelial tissue, whereas the fibrous and keloidal ridges were of fibrous tissue, as were circumferential lesions. All specimens had mitral-semilunar valvular continuity. CONCLUSIONS: Endocardial fold and circumferential lesions appear to be benign. The endocardial folds arose from the membranous ventricular septum, were not protuberant and usually were found in younger patients. The fibrous ridges, in contrast, were protuberant and were always associated with the underlying muscle of the outlet septum. These pathologic distinctions may facilitate echocardiographic diagnosis and prognosis.

Adolescent↗