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Biomedical subjects

L M Gerlis

Publications and source records attributed to L M Gerlis.

At least 37 records · Page 2Linked to original sources
PubMed1993

Morphology of the cardiovascular system in conjoined twins: spatial and sequential segmental arrangements in 36 cases.

Thirty-six pairs of conjoined twins are reviewed from the joint experience of the authors. The external forms of conjunctions were 18 thoracopagus (joined at chest), eight cephalothoracopagus (joined at head and chest), eight dicephalus (single trunk and two heads), one craniopagus (joined at head), and one omphalopagus (joined at abdomen). There was one case with a single conventional heart in association with isomerism of the left lungs and absence of the heart in the left-side twin. Six cases had two conventional hearts. All of them had associated lesions, which were more severe in three cases (50%) with abnormal laterality. All of the cephalothoracopagus (eight cases) had two shared hearts. Cardiac lesions were commonly seen in the posterior heart, but the arrangement of atrial appendages were normal in every heart. Twenty-one cases of either dicephalus or thoracopagus had a compound heart. Three cases (14%) had fusion only at the level of venous sinus; three others (14%) were fused at the atrial level only, but 15 cases (71%) had fusion at both atrial and ventricular levels. Abnormal laterality was an associated lesion in ten cases (48%) with a compound heart. The fusion at the atrial level always occurred between right atriums, but ventricular fusion was more commonly between two left ventricles. Four atrial or ventricular chambers, if fused, were arranged in a cruciate arrangement. Mode of conjunction was as important, in the formation of the cardiovascular system, as was the degree of fusion. Abnormal laterality, which is commonly associated in lateral or mixed lateral/facing conjunction, was the most important factor affecting the general morphology of the cardiovascular system.

Female↗
PubMed1993

Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia.

OBJECTIVE: Since 1905 there have been many reports of cases in which the right ventricle was deficient in myocardium. Several terms have been used to describe this condition. Of these, "Uhl's anomaly" and "arrhythmogenic right ventricular dysplasia" are most often used. Our study investigates the relation between these entities. METHOD: Five cases with a primary deficiency of the right ventricular musculature were examined. The findings were compared with those published reports to evaluate the similarities and differences between Uhl's anomaly and arrhythmogenic dysplasia. RESULTS: The five cases showed two patterns of myocardial deficiency in the right ventricle. On the one hand, the parietal wall was paper thin with complete absence of musculature and apposition of the endocardial and epicardial layers. On the other hand, patchy, localised fibrofatty tissue replacement was found within the parietal musculature. Evidence from our cases, combined with analysis of other publications, showed different modes and timing of clinical presentation of the patients with these two anatomical conditions, congestive heart failure or arrhythmia. CONCLUSIONS: The conditions variously described as Uhl's anomaly and arrhythmogenic dysplasia are separate and distinct morphological entities.

Adolescent↗
PubMed1993

Mitral valve anomalies associated with Ebstein's malformation of the tricuspid valve.

The original description of Ebstein's anomaly mentioned a mild deformity of the mitral valve. Reviews of large series of cases with Ebstein's malformation have, however, tended to omit reference to accompanying mitral valve malformations. We have compared the mitral valves in 19 specimens with Ebstein's malformation with those in 30 age-matched controls in order to determine the prevalence and nature of these anomalies. In the Ebstein's group the mitral valve was abnormal in 14 hearts with orificial anomalies in four, leaflet anomalies in 11, cord anomalies in 11 and papillary muscle anomalies in 13. In the control group, five cases showed mild shortening of the tendinous cords. Half of the changes found in the Ebstein's group were of moderate or significant degree suggesting a primary involvement of the mitral valve. In time, dynamic alterations within the left ventricle might become superimposed and contribute to the development of clinically significant lesions.

Adult↗
PubMed1993

Early ultrasound diagnosis of fetal congenital heart defects in high-risk and low-risk pregnancies.

OBJECTIVE: To evaluate the yield of early second-trimester transvaginal ultrasonography in the detection of congenital heart defects among patients with low or high risk for fetal anomalies. METHODS: During 5 years, we performed 12,793 transvaginal ultrasound examinations at 12-16 weeks' gestation, targeted for detection of fetal congenital anomalies. Three thousand four hundred fifty-three (27%) of these patients were considered to be at high risk for fetal congenital heart defects (because of family or medical history or teratogen exposure). The other 9340 patients were considered to be at low risk for fetal anomalies. The four-chamber view and the outflow tracts were evaluated systematically in all patients. RESULTS: Congenital heart malformations were observed in 47 cases, most of which (29 of 47) were diagnosed in the low-risk group. Additional extracardiac malformations were observed in 29 (62%) of the affected fetuses. Ten of 28 affected pregnancies that were karyotyped (36%) had abnormal chromosomes. Use of the four-chamber view alone would have failed to detect 11 (23%) of the abnormal fetuses. CONCLUSIONS: Transvaginal ultrasonography in the early second trimester is a useful tool for the detection of fetal cardiac structural defects, provided that both the four-chamber view and the outflow tracts are evaluated. When such an anomaly is suspected, additional fetal malformations should be sought and fetal karyotype should be determined.

Chromosome Aberrations↗
PubMed1992

Right ventricular outflow obstruction by anomalies of the tricuspid valve: report of a windsock diverticulum.

A 9-month-old infant with a clinical diagnosis of double outlet from the right ventricle, subaortic ventricular septal defect, and muscular subpulmonary obstruction underwent surgical repair. The defect was corrected and the right ventricular infundibulum enlarged with a patch. Eight hours after the operation there was a sudden collapse which rapidly progressed to cardiac arrest and death. Postmortem examination of the heart confirmed the clinical diagnosis but noted that the subpulmonary obstruction was caused by a "windsock" deformity of the tricuspid valve which was not identified at operation. The variants of malformations of the tricuspid valve causing subpulmonary obstruction are reviewed.

Heart Septal Defects, Ventricular↗
PubMed1992

Morphology of the posterior junctional area in atrioventricular septal defects.

The location and size of the coronary sinus in hearts with atrioventricular septal defect were investigated in relation to the known disposition of the atrioventricular conduction axis. We examined the morphology in 40 hearts and supplemented this series with two other hearts that had been serially sectioned previously. The coronary sinus received drainage from a persistent left superior caval vein in 5 hearts. Six cases of 40 had malalignment of the septal structures relative to the crux of the heart. In these, the conduction axis was anticipated to course in the position where the inlet ventricular septum met the atrioventricular junction. The coronary sinus terminated in the left atrium in 4 hearts: 2 in the morphological series and 2 that were sectioned for histological studies. The sectioned hearts showed the atrioventricular conduction axis in the usual position for the defect, unrelated to the coronary sinus. The principle that the node and penetrating bundle are located at the intersection of the ventricular septum with the atrioventricular junction holds good despite the variability of the coronary sinus.

Adolescent↗
PubMed1990

Atrioventricular septal defect with intact septal structures in a 74-year-old.

We report the incidental finding at post mortem of deficient atrioventricular septation in the heart of a 74-year-old woman. A review of her clinical history showed little indication of this lesion. The heart had intact septal structures but exhibited other features pathognomonic of atrioventricular septal defect.

Aged↗
PubMed1990

Three anomalies of the coronary arteries co-existing in a case of pulmonary atresia with intact ventricular septum.

A female infant, who died 17 hours after spontaneous birth, was found to have three distinct coronary arterial anomalies in association with pulmonary atresia with intact ventricular septum. These anomalies were first, an aneurysmal left coronary artery with a fistulous communication into the right ventricle; second, an anomalous origin of the right coronary artery from the pulmonary artery; and third, a supernumerary coronary artery arising from the right ventricle. Although the coronary arterial system was entirely connected to the right ventricle, the perfusing blood originated from the left ventricle and there was no clinical or histological evidence of significant myocardial ischaemia.

Coronary Vessel Anomalies↗
PubMed1990

The site of origin of nonconfluent pulmonary arteries from a common arterial trunk or from the ascending aorta: its morphological significance.

There are three possible embryological derivatives for nonconfluent pulmonary arteries which arise from a common arterial trunk or from the ascending aorta. We considered the feasibility of identifying these derivatives on the basis of the site of origin. We examined 15 specimens, in which both pulmonary arteries arose by separate orifices from a common arterial trunk (persistent truncus arteriosus), and 3 specimens, in which one pulmonary artery arose from the ascending aorta, the other being connected to the morphologically right ventricle (hemitruncus). Measurements were made for both the upper and lower margins of the orifices of the pulmonary arteries and then expressed as a percentage of the length of the ascending common trunk or aorta. The position of origin ranged from 39 to 100% for the upper margin and from 17 to 90% for the lower margin, without significant difference between left and right arteries. Since the range varied so widely, we contend that it is not possible to make a morphological identification of the pulmonary artery under consideration simply on the basis of the location of the site of origin.

Aorta↗
PubMed1989

Persistent 5th aortic arch--a great pretender: three new covert cases.

Although persistence of the embryonic fifth aortic arch is considered a rare congenital malformation, analysis of the records of the cardiopathological collections of the Brompton and Killingbeck Hospitals reveals 6 examples in approximately 2000 specimens, an incidence amongst our material of 1 in 330. Since our review of the literature reveals only a further 13 described cases, we wonder if the condition may go unrecognized rather than being exceedingly rare. This possibility is further supported by the fact that 3 of our cases were incorrectly interpreted when initially described. In this report, we describe the details of 2 of these specimens and one other case that was not included in our earlier descriptions of this malformation. The first case had the persistent fifth arch as a conduit between the pulmonary and systemic circulations in the setting of aortic atresia with interruption of the aortic arch. The second case demonstrated a double lumen aortic arch, the fifth arch in this instance being an accessory systemic-to-systemic conduit. In the final case, the persistent arch was initially considered to represent an aorto-pulmonary window but review revealed an aortic to pulmonary conduit more in keeping with the presence of a fifth arch. We conclude that the powers of mimicry of this enigmatic structure may account for its apparent rarity.

Aorta, Thoracic↗
PubMed1989

Aortic root triangulation as a cause of sudden death.

We have reviewed an autopsy series of thirty-six patients who died suddenly after receiving a Starr--Edwards aortic valve prosthesis. In fifteen of the thirty-six explanted valves, the aortic outlet was narrowed with the three-legged prosthetic cage causing a triangular distortion of the wall. In the remaining twenty-one patients, the cause of sudden death was not related to failure of the mechanical valve. Although aortic root triangulation would appear to be an uncommon complication with the Starr--Edwards prosthesis, these findings emphasise the need to carefully size the aortic root diameter at the time of surgery, whilst the known problems of morbidity and mortality with mechanical heart valves should continue to stimulate those developing conservative valve reconstruction.

Adult↗
PubMed1989

Anomalous subaortic position of the brachiocephalic (innominate) vein: a review of published reports and report of three new cases.

Anomalous courses of the left innominate vein have rarely been described in anatomical specimens. Investigative techniques such as angiography and echocardiography have brought to light more instances of this anomaly. Three more cases identified by anatomical study are described. Earlier cases were reviewed to assess the type of associated cardiac malformations. Clinically, the abnormality is regarded as benign. When it is recognised during investigation it should alert the clinician to the possibility of associated malformations. Features commonly seen in tetralogy of Fallot--right aortic arch, ventricular septal defect, and right ventricular outflow obstruction--were common in patients with anomalous subaortic innominate veins.

Brachiocephalic Veins↗
PubMed1989

Anomalous origin of the left coronary artery from the pulmonary trunk. Anatomic findings in relation to pathophysiology and surgical repair.

Anomalous origin of the left coronary artery from the pulmonary trunk, though a discrete anatomic malformation, manifests a spectrum of clinical and pathologic consequences. The objectives of this study were to characterize the primary anatomic findings in a group of specimens with anomalous left coronary artery and the extent of secondary morphologic and pathologic changes. Although the cases studied probably represent the least favorable end of the spectrum, the observed pathogenesis and evolution of secondary changes suggest that reconstruction of a two-coronary arterial system supplied through two coronary arteries would be advantageous to most patients. A high origin of the right coronary artery or location of the left coronary artery adjacent to a pulmonary cusp or branch may complicate the tunnel-type repair. In these cases, transfer of the left coronary artery to the aorta may be preferable.

Abnormalities, Multiple↗
PubMed1987

Aortic valve damage caused by operative balloon dilatation of critical aortic valve stenosis.

Operative balloon dilatation of the aortic valve was performed in seven neonates with critical stenosis of the aortic valve. The procedure was followed by the development of severe aortic regurgitation in four patients. Necropsy was performed in three and revealed partial detachment of the right coronary cusp of the aortic valve. Damage to the valve leaflet caused by balloon dilatation was probably the result of using a balloon with a diameter that was too large in relation to the aortic valve ring diameter and of shearing forces created in the aortic wall by the contracting ventricle. The diameter of the inflated balloon should not be larger than the diameter of the aortic valve ring.

Angioplasty, Balloon↗
PubMed1986

Maternal antibodies against fetal cardiac antigens in congenital complete heart block.

An immunologic basis for congenital heart block has been proposed previously. To investigate the association between congenital heart block and maternal antibodies capable of crossing the placenta, we used immunofluorescence to examine serum samples from 41 mothers and 8 affected children, together with serum from controls, for antibodies to fetal cardiac tissue. Twenty-one mothers (51 percent) had IgG antibody reactive with fetal heart tissue, as compared with only 9 of 94 controls (10 percent; P less than 0.001). Three of 8 affected babies, but none of 50 healthy babies, had similar antibodies. The antibodies reacted with all myocardial tissue and were not directed specifically to the conduction system. They also reacted with other fetal tissues and could be distinguished from nuclear and smooth-muscle autoantibodies. We also observed a higher occurrence of antibodies to cytomegalovirus, but not to Epstein-Barr virus, in these mothers. Autopsy specimens from babies with congenital heart block examined by immunoperoxidase staining showed deposition of immunoglobulin and complement components in all cardiac tissues. These findings strengthen the case implicating immune reactivity related to maternal antibody in the development of some but not all cases of congenital heart block.

Antibodies, Antinuclear↗