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Biomedical subjects

J R Zuberbuhler

Publications and source records attributed to J R Zuberbuhler.

At least 37 records · Page 2Linked to original sources
PubMed1988

Cineangiographic spectrum of Ebstein's malformation: its relevance to clinical presentation and outcome.

Eight heart specimens were examined that had concordant connections of the cardiac segments and exhibited Ebstein's malformation. The displacement of the leaflets of the tricuspid valve from the atrioventricular junction varied from minimal and isolated involvement of the septal leaflet to involvement of the mural and anterosuperior leaflets as well. This resulted in a wide range of physiologic "atrialization" of the right ventricle. The distal insertion of the valve leaflets also exhibited a spectrum of malformation. At one end the insertion was the normal focal variety, allowing free communication between the atrialized and functional parts of the right ventricle. At the other end there was abnormal linear attachment of the anterosuperior and mural leaflets to an anomalous muscular shelf at the junction between the inlet and apical trabecular portions of the right ventricle. The anteromedial commissure between the anterosuperior and the displaced septal leaflets provided a "keyhole" communication between the two ventricular compartments. Between these extremes were cases in which hyphenations along a locus of linear attachment allowed additional communications between the ventricular compartments. In light of these anatomic observations, cineangiograms of 26 patients with Ebstein's malformation were reviewed retrospectively. It was possible to classify the patients into three groups with focal, hyphenated and linear attachment, respectively. Size, ejection and displacement indexes of the functional right ventricle measured from the angiograms suggested that the severity of the malformation increased from focal attachment through hyphenated to linear attachment. Clinical observations relative to symptoms (cyanosis at rest, reduced exercise tolerance) and outcome supported this morphologic-angiographic grading.

Child↗
PubMed1988

Prevalence of congenital cardiac anomalies at high altitude.

The effect of high altitude on the prevalence of congenital heart disease was tested by examining 1,116 school children at four study sites in the People's Republic of China. Sites ranged in altitude from sea level to 4,500 m above sea level. Children were screened by physical examination, and an echocardiogram and electrocardiogram were performed on each child suspected of having a cardiac anomaly. A high prevalence of patent ductus arteriosus and atrial septal defect was found at the three high altitude sites and the effect of altitude was progressive. Both anomalies were postulated to be the result of the lower atmospheric oxygen tension present at high altitude. Failure of lower oxygen tension to constrict the ductus is thought to be the mechanism in patent ductus arteriosus. It is theorized that the persistence of high pulmonary vascular resistance and high right heart pressures at high altitude inhibits early closure of the foramen ovale. Subsequent growth may result in stretching of the fossa ovalis and incompetence of the flap and may produce an atrial septal defect. The high prevalence of atrial septal defect in tetralogy of Fallot is cited as a possible analogy because right ventricular pressure is high and right ventricular compliance is low from birth.

Adolescent↗
PubMed1988

The cross sectional anatomy of ventricular septal defects: a reappraisal.

The cross sectional echocardiographic description of holes in the ventricular septum has been unsatisfactory, chiefly because there are so many classifications of this defect. The accurate description of the anatomy of individual defects, from cross sectional images, is more important than attempts to fit them into a preconceived classification. One hundred specimens of hearts with a ventricular septal defect were reviewed to identify those features that are of value in the interpretation of cross sectional images. Three groups of defect were identified: those which abutted the central fibrous body, those with a margin partly formed by an area of fibrous continuity between the leaflets of the aortic and pulmonary valves, and those with entirely muscular margins. Each group had features that were readily discernible in cross section. Other features of the defects seen in the cross sectional images identified defects that opened between the two ventricular inlets, defects that opened between the two subarterial outlets, and those that extended solely into the trabecular septum. The criteria for describing the anatomy of defects were established in hearts with normal connections, but they were found to be equally applicable in hearts with discordant atrioventricular connection, discordant ventriculoarterial connection, common arterial trunk, and double outlet from the morphologically right ventricle. Cross sectional imaging, by echocardiography and potentially by other techniques, provided a uniquely detailed and precise description of the morphology of ventricular septal defects.

Abnormalities, Multiple↗
PubMed1988

The determination of atrial arrangement by examination of appendage morphology in 1842 heart specimens.

The morphology of the atrial appendages was examined in 1842 specimen hearts from patients with congenital lesions. The external and internal features that permitted the identification of the right and left appendages were studied in detail in one tenth of the hearts. These results were compared with a similar analysis of 25 normal hearts. This study showed that criteria for identification of right and left appendages were reliable. Application of these criteria to the overall collection identified the usual arrangement in 1776 (97%) hearts, a mirror image arrangement in eight (0.4%); left atrial isomerism in 22 (1.2%); and right atrial isomerism in 36 (1.9%). Fourteen (0.81%) had juxtaposed atrial appendages (13 with usual arrangement and one with left isomerism). This did not interfere with identification of the left and right atria on the basis of appendage morphology. In only two cases did the determination by atrial morphology produce a result that was inconsistent with the arrangement of the other thoracoabdominal organs. Further examination of the atria in these showed a mistake had been made in the initial assessment. The atrial arrangement can be accurately determined by the morphology of the atrial appendages.

Child↗
PubMed1988

Divided right atrium (prominence of the eustachian and thebesian valves).

Division of the morphologically left atrium (cor triatriatum) is a recognized clinical and surgical entity. Division of the right atrium (prominence of the eustachian and thebesian valves) is recognized pathologically, but is rare. It is unusual for this entity to be diagnosed during life. Stimulated by our experience with two patients seen at operation, one with an obstructive spinnaker-like formation and the other with a partitioned right atrium in the setting of pulmonary atresia, we reviewed the specimens in the heart museum of Children's Hospital of Pittsburgh that had prominence of the eustachian and thebesian valves. We identified 14 such hearts, which could be divided into two groups. In the first group, comprising six hearts, the valves were prominent in the form of Chiari networks and were of no functional significance. The valves were more extensive in the other eight hearts and partitioned the right atrium. In two of these, the valves themselves were the impediment to flow through the right side of the heart. In the other six, there was either atresia or severe stenosis along the right-sided pathways so that, after birth, the prominent valves retained their role during fetal life; namely, to deflect inferior caval venous return across the atrial septum to the left atrium. The partitions in these latter hearts would be of functional significance only if it were necessary to perform a Fontan procedure, when they might obstruct flow through an atriopulmonary (or atrioventricular) anastomosis.

Female↗
PubMed1987

Identification and analysis of left atrial isomerism.

The veno-atrial connections, atrial morphology, atrioventricular (AV) junction, ventricular mass, ventriculoarterial (VA) connection and great arteries in 22 autopsied hearts, diagnosed as having bilateral left-sidedness because of the morphology of the atrial appendages, were studied. The findings were correlated with the arrangement of the thoracic-abdominal organs. A solitary spleen was found in 3 and double spleens in 2 hearts (the remaining 17 hearts had multiple spleens) but left bronchial isomerism existed in all hearts in which bronchial arrangement could be determined. The heart was in the left chest in 14 cases, in the right chest in 5 and midline in 3. The apex pointed to the left in 18 hearts while in 4 hearts it pointed to the right. Fifteen hearts had a biventricular and ambiguous AV connection, 3 hearts had an absent left AV connection and 4 had double-inlet connection via a common valve (to the left ventricle in 3 and the right ventricle in 1). The VA connection was concordant in 14 hearts, discordant in 1, double outlet from the right ventricle in 4, double outlet from a solitary indeterminate ventricle in 1 and single outlet from the right ventricle through a pulmonary trunk with aortic atresia in 2 hearts. Superior caval veins were present bilaterally in 13 hearts. There was interruption of the infrahepatic inferior caval vein with azygos or hemiazygos continuation in 19 hearts while in 3 hearts the inferior caval vein continued upwards to drain into the right-sided morphologically left atrium.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗
PubMed1987

Long-term follow-up after aortic valvuloplasty and defect closure in ventricular septal defect with aortic regurgitation.

The clinical course was studied in 25 patients with ventricular septal defect (VSD) and aortic regurgitation (AR) who had undergone aortic valvuloplasty and VSD closure. Twelve patients had a doubly committed subarterial VSD and 13 had a perimembranous type of VSD. Preoperatively, progressive hemodynamic disturbance after the onset of AR occurred in 11 patients (44%). The follow-up period extended from 6 months to 23 years (mean 10 years). Four patients required prosthetic valve replacement after valvuloplasty. One of these had had initial improvement of the AR but required valve replacement 20 years later; the condition of the other 3 did not improve initially and their valves were replaced 1 month to 8.5 years later. Four other patients had no initial improvement as a result of valvular reconstructive surgery, but the AR did not progress and remained hemodynamically well tolerated. Hence, the overall success rate of the valvuloplasty, defined as improving or preventing progressive AR, was 21 of 25 (84%). Since initial and long-term improvement in aortic valve function can be expected in most patients after valvuloplasty and closure of the VSD, early surgical intervention is recommended in patients with a VSD and AR.

Aortic Valve↗
PubMed1987

The surgical anatomy of the Taussig-Bing malformation.

There is no consensus as to the most appropriate definition of the use of the term Taussig-Bing malformation. In this study, we endorse the suggestion that the lesion be considered a spectrum of anomalies, unified by a juxtapulmonary ventricular septal defect with malalignment of the infundibular septum. The ends of the spectrum are hearts with, on the one hand, double-outlet right ventricle and, on the other hand, a discordant ventriculoarterial connection. We studied 10 hearts within this spectrum, eight with a double outlet and two with discordant ventriculoarterial connection. We found that the same basic morphology could exist with a bilaterally complete infundibulum or in the presence of fibrous continuity between the mitral and the pulmonary valves. Features of most significance for surgical correction were the arrangement of the outlet septum (particularly the presence or absence of chordal attachments from the atrioventricular valves), the morphology of the coronary arteries, and the state of the subaortic infundibulum. The options for surgical correction are determined by the internal morphology of the lesions rather than by the precise ventriculoarterial connection.

Child↗
PubMed1986

Atrioventricular septal defects: cross-sectional echocardiographic and morphologic comparisons.

We examined the cross-sectional echocardiographic findings of 171 patients with atrioventricular septal defects. The echocardiographic findings were confirmed by angiography, surgery and/or autopsy. The echocardiographic findings determined whether the common atrioventricular junction was guarded by a common valve or separate right and left valves. In addition, we were able to judge whether the bridging leaflets were related to the septal structures so as to permit both interatrial and interventricular communications [127 cases] or whether the interatrial communication ("ostium primum atrial septal defect") [43 cases] or an interventricular communication [1 case] existed in isolation. Defects existing with a common atrioventricular valve could be further classified as having minimal bridging of the antero-superior leaflet (Rastelli Type A [113 cases]); intermediate bridging (Rastelli Type B [3 cases]); or extreme bridging (Rastelli Type C [11 cases]). Of the patients with Down's syndrome, 9 had separate right and left valves while 66 had a common valve, all the latter existing in the setting of minimal bridging of the antero-superior leaflet. In the overall group, there were 9 cases having an unbalanced ventricular mass, 5 with right ventricular dominance and 4 with left dominance. Other associated defects were common. The echocardiographic findings were supplemented by pulsed Doppler examination. Atrioventricular valve insufficiency, when mild, was frequently demonstrated only in the right atrium just above the leaflets of the atrioventricular valve. When there was more severe valve insufficiency the regurgitant jet could be detected for greater distances behind the atrioventricular valve and in either or both atria, but more frequently in the left atrium. As might be anticipated in view of the complexity of the lesion, a combined imaging approach yielded the most accurate results.

Adolescent↗
PubMed1986

Venous systolic thrill and murmur in the neck: a consequence of severe tricuspid insufficiency.

A palpable venous systolic thrill and murmur at the base of the neck are described as new physical findings in five patients with severe tricuspid regurgitation. In two of these patients, the tricuspid valve had been resected as treatment for infective endocarditis related to intravenous drug abuse. The third patient had severe chronic pulmonary disease with right heart failure. The fourth patient had a complex congenital defect in which the mitral valve served as the venous atrioventricular valve and was severely incompetent. The fifth patient suffered from long-standing rheumatic mitral and tricuspid disease with pulmonary hypertension 10 years after placement of a mitral prosthesis. From these observations, it is apparent that pulsatile retrograde flow in the cervical veins resulting from severe right-sided atrioventricular valve incompetence can produce a palpable systolic thrill and murmur at the base of the neck.

Adolescent↗
PubMed1985

Variable morphology of ventricular septal defect in double inlet left ventricle.

The morphologic characteristics of ventricular septal defect (VSD) relative to the disposition of the atrioventricular conduction axis were studied (as far as was possible from gross observation) in 33 hearts with double inlet left ventricle. The hearts were separated into 4 groups. The hearts had either right-sided or left-sided rudimentary right ventricles (RV) coexisting with either ventriculoarterial (VA) concordance or discordance, respectively. Significant differences were noted in the morphology of the VSD according to these patterns. With right-sided rudimentary RV and VA concordance the anatomy was similar to that of classic tricuspid atresia, with an extensive outlet septum in the roof of the defect. With left-sided rudimentary RV and VA concordance, 2 further patterns were seen. In 1 pattern the arrangement was similar to that found with VA discordance. In the other, there was a pinhole VSD, a grossly hypoplastic right ventricle and a complete subaortic infundibulum in the dominant left ventricle. With VA discordance the morphology was broadly similar be the rudimentary RV right-sided or left-sided. The outlet septum was much less extensive than in the arrangement with right-sided RV and VA concordance. Minimal differences related to the formation of the right margin of the defect, which always carried the conduction axis on its left ventricular aspect. These differences dictated the "safest margins" of the defect should its excision be required during life. Basically, the left margin of the apical trabecular septum can most safely be excised.

Child↗
PubMed1985

Echocardiographic findings before and after liver transplantation.

Echocardiographic studies were performed in 73 patients with various types of chronic liver disease. They were 0.5 to 19 years old (mean 5). Thirteen patients underwent follow-up echocardiography 1 to 13 months (mean 6) after liver transplantation. Preoperatively 60 patients (82%) showed evidence of high cardiac output (cardiac index greater than 4 liters/min/m2); these patients manifested increased left ventricular (LV) and left atrial dimensions and a thickened LV posterior wall. Transvenous contrast echocardiographic study confirmed the presence of intrapulmonary arteriovenous shunting in 4 patients. Studies after liver transplantation revealed a reduced LV end-diastolic dimension in 12 patients. Cardiac index was reduced a mean of 35% after transplantation (p less than 0.001). This study suggests that liver transplantation improves common hemodynamic abnormalities in chronic liver disease.

Adolescent↗