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Biomedical subjects

J R Zuberbuhler

Publications and source records attributed to J R Zuberbuhler.

At least 73 records · Page 4Linked to original sources
PubMed1983

The anatomical heterogeneity of hearts lacking a patent communication between the left atrium and the ventricular mass ('mitral atresia') in presence of a patent aortic valve.

Thirty-five hearts were studied with no patent communication between the left atrium and the ventricular mass ('mitral atresia'). In ten, an imperforate and hypoplastic membrane separated the left atrium from a hypoplastic left ventricle. The imperforate valve ranged from a tiny membrane with no evidence of tension apparatus, through a larger membrane supported by muscular columns in the ventricular inlet portion, to an aneurysmal imperforate sac attached to a single hypoplastic papillary muscle. The ventriculo-arterial connection was concordant in nine of the ten hearts and double outlet from the right ventricle in one. In one further heart, there was double inlet left ventricle with imperforate left atrioventricular valve, a rudimentary right ventricle and ventriculo-arterial discordance. In the other 24 hearts the muscular floor of the left atrium was completely separated by the atrioventricular sulcus from the ventricular mass (absent left atrioventricular connection). In 11 of these hearts, the right atrium was connected to a right ventricle and there was a posterior and left-sided rudimentary left ventricular chamber which had no connection with the atrial chambers. The left ventricle supported the aorta in five of the 11 hearts, but in the other six it was simply a pouch of left ventricular morphology. In ten cases the right atrium was connected to a dominant left ventricle. An anterior rudimentary right ventricle was present in each but had no connection with the atrial chambers. It was to the left of the left ventricle in seven, directly anterior in one and to the right in two cases. The right ventricle supported the aorta in eight cases, the pulmonary trunk in one and neither great artery in the remaining case. Lastly, there were three cases in which a sole ventricular chamber of indeterminate morphology was present and gave rise to both great arteries.

Aortic Valve↗
PubMed1983

Morphology of ventricular septal defect associated with coarctation of aorta.

It is now well established that the morphology of the ventricular septal defect associated with interrupted aortic arch is such as to compromise blood flow to the ascending aorta. Though there is some evidence that a similar mechanism may be operative in coarctation with ventricular septal defect, we are unaware of any necropsy study of this hypothesis. We therefore studied 25 hearts from the Heart Museum of Children's Hospital of Pittsburgh with coarctation and ventricular septal defect. Four had a malalignment defect with left ventricular outflow tract obstruction such as is seen in cases with interruption. Twenty of the remaining cases had a particular form of perimembranous defect with aortic overriding. The defects were partially closed by tricuspid valve tissue, the left ventricular outflow tract being further narrowed by various anatomical lesions. In addition, mitral valve malformations were present in 14 cases. The anatomy of the group as a whole suggests a possible reduction of aortic blood flow during fetal development. The clinical implications of these findings remain to be evaluated.

Aortic Coarctation↗
PubMed1982

Coarctation of the aorta in infants and children: 25 years of experience.

Repair of coarctation of the aorta was performed in 334 patients ranging from less than a year to 16 years of age at Children's Hospital of Pittsburgh from 1953 to 1977. Resection and end-to-end anastomosis were performed in 310, interposition grafts in 7, and other procedures in 17. There were 41 operative deaths among the 95 infants less than 1 year old (43% mortality); all of these infants had associated cardiac anomalies. Only 1 operative death occurred in patients older than a year (0.4% mortality). No hospital deaths occurred in patients with isolated coarctation of the aorta. Eleven late deaths were due to associated anomalies (7) and unrelated or unknown causes (4). Postoperatively, hemorrhage occurred in 7, paradoxical or persistent hypertension in 128, postcoarctectomy syndrome in 32, neurological problems in 14, pulmonary complications in 53, and infections in 12 patients. Restenosis occurred in 26 patients (8%); only 10 have required reoperation. Residual hypertension was present in 28 of 264 patients (11%). Mortality from coarctation resection remains high in infants, but is minimal in children older than 1 year. Based on minimizing the risk of restenosis and residual hypertension, elective repair of coarctation is recommended when the patient is about 6 years of age.

Adolescent↗
PubMed1982

Blade atrial septostomy: collaborative study.

During the past 4 years, five institutions have collaborated in evaluating the efficacy of blade atrial septostomy. The procedure was performed in 52 patients, including 31 with transposition of the great arteries, 10 with mitral atresia, five with tricuspid atresia and six with miscellaneous anomalies. The patient's ages ranged from 1 day to 12 years (mean 13 months). Improvement occurred in 41 of 52 patients (79%). Four patients had an intact interatrial septum, and blade atrial septostomy was successfully performed by a transseptal technique. One patient died from a lacerated left atrial wall; other complications occurred in four patients. Blade atrial septostomy is an effective palliative procedure, even when the interatrial septum is thickened or intact.

Arteries↗
PubMed1981

Surgical anatomy of tetralogy of Fallot.

On the basis of previous experience, we have analyzed the anatomy of a series of 53 hearts catalogued as having tetralogy of Fallot in the museum of the Children's Hospital of Pittsburgh. All hearts had a ventricular septal defect, aortic overriding, infundibular pulmonary obstruction, and right ventricular hypertrophy. None of the hearts had been operated upon during life. We paid particular attention to features of surgical importance. Thus the ventricular septal defect was found to be of variable form. It was perimembranous in 42 hearts. In 11 hearts it had entirely muscular rims and in the remaining heart it was roofed by the conjoined aortic and pulmonary valve rings (subarterial). From our previous histologic experience, it was evident that this varying morphology significantly affected the surgical anatomy of the atrioventricular conduction tissues. We endeavored to display this disposition as might be viewed by the surgeon. The degree of aortic override was variable, the aortic valve being connected by 15% to 95% to the right ventricle. In 17 hearts more than half the aortic valve was attached to right ventricular musculature. The nature of the infundibular obstruction also varied markedly. In all cases (except the one with absent infundibular septum) the infundibular septum was deviated in cephalad and anterior direction so as to produce obstruction. In the majority of hearts further anatomic structures also contributed to the obstruction. In some hearts there was hypertrophy of the infundibular septum, particularly at its junction with the trabecular septum. In others there was hypertrophy of the trabecula septomarginalis, and in many hearts there was hypertrophy of additional anterior infundibular trabeculations. The pulmonary valve was the narrowest point of the outflow tract in only six hearts, but in many of the others, it was abnormal. The results emphasize that while hearts may exhibit the classical features of tetralogy of Fallot, there is usually considerable individual variation in each of these features which is of major surgical significance.

Cardiomyopathy, Hypertrophic↗
PubMed1981

Cor triatriatum (subdivided left atrium).

Twenty-one patients (age 1 day to 156 months) with cor triatriatum sinister (subdivided left atrium) were treated over a 23 year period at two institutions. Anatomic classification of the hearts revealed 20 (95%) with classical cor triatriatum and normal pulmonary venous connections. One other patient had partial anomalous pulmonary venous connection. Three of these patients (14%) had other severe associated cardiac anomalies. Surgical correction of isolated cor triatriatum was performed in 13 patients of whom eight (62%) survived. Long-term results were excellent in seven (88%) patients; one late death occurred. A right atrial approach for infants and small children is recommended, while the left atrial approach appears satisfactory for larger children.

Adolescent↗
PubMed1980

Absent right superior vena cava with persistent left superior vena cava: implications and management.

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Children's Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 and in a 26 day old infant with multiple cogenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals s short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well, the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.

Adolescent↗
PubMed1980

Electrocardiogram in double chamber right ventricle.

Thirty patients with a double chamber right ventricle were seen during a 7 year period. The majority of patients had associated cardiac anomalies, most commonly a ventricular septal defect. In this series, as in others, the history, clinical examination, chest roentgenogram and echocardiogram were inconclusive for diagnosing double chamber right ventricle. Symptoms were generally related to the severity of the associated anomalies. In 40 percent of the patients reviewed, upright T waves were found in lead V3R as the only electrocardiographic finding suggestive of right ventricular hypertrophy. Because upright T waves in lead V3R in the absence of other evidence of right ventricular hypertrophy are not commonly seen in patients with an isolated ventricular septal defect or a defect associated with other forms of right ventricular outflow obstruction, this finding should alert the cardiologist to the possibility of the presence of double chamber right ventricle prior to cardiac catheterization.

Adolescent↗
PubMed1980

Morphological considerations pertaining to recognition of atrial isomerism. Consequences for sequential chamber localisation.

The atrial morphology and venous connections were assessed "blind" in 51 necropsy specimens from patients with visceral heterotaxy. This was compared with bronchial morphology as established by dissection. Six specimens were found to have both atria and bronchi in situs solitus or inversus, and were rejected. In the remainder, atrial isomerism was diagnosed, though this required minor revision of the atrial assessment in two patients. Thirty-four patients had isomeric right atria and bronchi, while 11 had isomeric left atria and bronchi. In seven cases, splenic status was unknown, but in seven of the remaining 38 (18.4%) atrial isomerism was not associated with either asplenia or polysplenia. Nevertheless, right isomerism was strongly associated with total anomalous pulmonary venous drainage (as is asplenia) and left isomerism was likewise associated with interruption of the inferior vena cava (as is polysplenia). Bilateral superior venae cavae and hepatic veins, and absence of the coronary sinus, were frequent in both forms of isomerism (as they are in asplenia and polysplenia). These findings suggest that atrial situs can be defined as solitus inversus, right isomerism, and left isomerism. This determination of atrial situs is quite independent of any other abnormalities of visceral situs. The high incidence of anomalies of both venous return and common atrium resulted in presumed complete mixing of blood at atrial level in all but one patient (97.8%), making the haemodynamic connection between atria and ventricles almost always ambiguous. To describe this anatomical connection as ambiguous when there are two ventricles present is therefore no more than recognition of anatomical and haemodynamic reality.

Abnormalities, Multiple↗
PubMed1979

Iliac venous thrombosis in infants and children after cardiac catheterization.

Twenty-two patients developed thrombosis of the lower abdominal portion of the inferior vena cava, iliac or femoral vein (IVT) after cardiac catheterization. All patients has at least one previous study from 1 day to 11 months of age (mean, 2 months) and seven had two catheterizations before discovery of IVT. Transposition of the great arteries was the most common defect associated with IVT (12/22, 55%). From one to seven catheter changes were made during the time of venous cannulation (mean, 105 minutes). No clinical evidence of IVT was present immediately after the preceding cardiac catheterization. The discovery of significant IVT usually necessitated the use of a vein from the upper extremity to complete the cardiac catheterization. IVT was associated with previous balloon atrial septostomy using either a Fogarty or Rashkind septostomy catheter in 13/22 patients (59%). In addition, 86% of the patients had either of these balloon catheters or a Swan-Ganz catheter used during a previous study. The potential development of IVT should be considered especially in cyanotic infants and small children in whom balloon catheters are used.

Cardiac Catheterization↗
PubMed1979

Anomalous origin of the right coronary artery from the pulmonary artery.

Three patients with anomalous origin of the right coronary artery from the pulmonary artery are presented together with a review of the 14 patients with this anomaly previously reported. One of the current patients is the youngest to be diagnosed before operation and the youngest to undergo corrective operation. Although the lesion is usually asymptomatic, it may contribute to sudden death. The only suggestive physical finding is a continuous murmur with diastolic accentuation, which is present only in some patients. There are no diagnostic electrocardiographic or chest roentgenographic changes. Diagnosis is made best by selective left coronary arteriography showing retrograde filling of the right coronary artery from collateral vessels. Operative repair should consist either of direct reimplantation of the proximal right coronary artery into the aortic root or of saphenous vein bypass graft to the coronary artery with ligation of its origin. Operation is recommended when the lesion is recognized.

Aged↗
PubMed1979

Morphological variations in pulmonary atresia with intact ventricular septum.

The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The degree of right heart hypoplasia was quantified by measuring right and left heart dimensions and comparing them with 20 normal infant hearts. Right ventricular cavity size was usually smaller than normal but constituted a spectrum ranging from tiny to a dilated ventricle larger than normal. There was a positive correlation between triscuspid annular size and right ventricular size but no correlation between the size of the pulmonary artery and the right ventricle. Successful pulmonary valvotomy with subsequent adequate right ventricular function would have been precluded by a tiny right ventricular cavity or infundibular atresia in 14 specimens and by severe tricuspid stenosis or regurgitation in an additional 4. Severe right or left ventricular endocardial fibroelastosis may have adversely affected ventricular function in several others. Ten specimens displayed convex bulging of the left ventricular septal surface. If these these anatomical findings are representative for the condition as a whole, they provide a good explanation for its disastrous prognosis.

Ebstein Anomaly↗
PubMed1979

Morphologic spectrum of Ebstein's anomaly of the heart: a review.

The rare and curious congenital malformation of the tricuspid valve known as Ebstein's disease or Ebstein's anomaly has been a subject of considerable interest to the cardiologist and the morphologist since its first description by Wilhelm Ebstein in 1866. With the evolution of various surgical techniques for the correction of this anomaly, a thorough understanding of the pathologic anatomy of the malformation assumes greater practical importance. It is now clear that although all hearts with Ebstein's anomaly have in common displaced basal insertions of the septal and posterior leaflets of the tricuspid valve, each case may be pathologically and hemodynamically unique. The malformed tricuspid valve may be incompetent, stenotic, or rarely, imperforate. It may be found in a left-sided tricuspid valve with inversion of ventricles. It is important that these individual variations be recognized so that the operative approach can be tailored to the particular variant of the malformation at hand.

Coronary Vessels↗
PubMed1979

The spectrum of Ebstein's anomaly of the tricuspid valve.

The structure of the tricuspid valve in 14 cases of Ebstein's malformation is described and compared with that in the normal heart. The anomalous hearts showed a spectrum of malformation, varying from minimal displacement of the adjacent parts of the septal and inferior leaflets of the tricuspid valve to presence of an imperforate membrane or muscle shelf between the inlet and trabecular zones of the right ventricle. In "typical" Ebstein's anomaly the anterosuperior leaflet was abnormally attached so as to partially obstruct the route from right atrium to distal right ventricle, the communication between these chambers being between the abnormally attached leaflet and its malformed neighbors. In two hearts, a muscular shelf produced stenosis between inlet and trabecular portions, but a competent valve was produced at this site, the atrialized part of the right ventricle being of normal thickness. The significance of these findings is considered with regard to possible methods of repair. Description of the anomaly is facilitated by considering the right ventricle as possessing inlet, trabecular, and outlet portions. The essence of the anomaly is displacement of the tricuspid orifice to the junction of the inlet and trabecular ventricular zones.

Ebstein Anomaly↗
PubMed1979

Pneumonectomy for intractable left bronchial compression in d-transposition of the great arteries.

In infants with transposition of the great arteries (TGA) tracheobronchial obstruction may occur as a result of compression by a dilated posterior pulmonary artery, especially if there is a large interventricular septal defect with its attendant large pulmonary blood flow and high pressure. Banding of the pulmonary artery may give temporary relief. A patient with TGA had collapse of the left lung at 2 months of age, following atrial septectomy. Bronchoscopy and bronchography revealed extrinsic compression of the left main bronchus. Banding of the pulmonary artery at 6 months of age relieved the respiratory distress. At 8 years of age atelectasis of the left lung recurred following a Mustard procedure. Two additional procedures were attempted to decrease the pressure of the pulmonary artery on the bronchus but these were unsuccessful. At age 9 years, pneumonectomy, her sixth thoracotomy, was performed as a final attempt to relieve the respiratory problem caused by persistent atelectasis. She is now 17 years of age and has remained asymptomatic during the last 8 years.

Adolescent↗
PubMed1978

Echocardiographic and hemodynamic correlation in transposition of the great arteries.

Echocardiography was performed in 36 patients with transposition of the great arteries (TGA). Twenty patients were studied before a Mustard operation, 14 patients after operation and two patients both before and after operation. Right ventricular end-diastolic dimension (RVED) was larger than normal in each patient and tended to increase postoperatively. In contrast, the left ventricular end-diastolic dimension (LVED) was significantly reduced postoperatively in all patients. A linear relationship was demonstrated between the ratio of LVED/RVED and the ratio of peak systolic pressures in the left and right ventricles in studies both before and after Mustard operation. Systolic anterior motion of the mitral valve was observed in 18% of peroperative patients and increased to 44% postoperatively. The incidence of fluttering of the mitral valve increased from 50% to 94% after the operation. Abnormal septal motion was found in 39% of cases. Abnormal movement of the mitral valve and of the interventricular septum seems to be related to a reversed pressure relationship in the ventricles. Shifting of the ventricular septum toward the left ventricle and consequent distortion of the left ventricular cavity and mitral valve apparatus may be responsible for the abnormal echocardiographic findings. Serial echocardiographic studies may be useful as a noninvasive tool in the assessment of left ventricular pressure or the status of the pulmonary vascular bed in TGA.

Adolescent↗