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Biomedical subjects

A J Muster

Publications and source records attributed to A J Muster.

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

The role of the Glenn shunt in patients undergoing the Fontan operation.

Twenty-seven patients (3 to 22 years) underwent the Fontan operation. Seventeen had tricuspid atresia and 10 had other complex lesions. Sixteen patients had a Glenn shunt (12 prior to, three simultaneous with, and one following the Fontan operation). One early death (3.7%) occurred in a 4 1/2-year-old child with tricuspid atresia II-C, previous pulmonary artery banding, and a closing ventricular septal defect (80 mm Hg gradient). There were two late deaths (7.4%) from Candida sepsis, after 4 and 6 months, respectively. The 24 patients who survived the Fontan operation had postoperative hospital stays of 6 to 90 days (average 18). Patients with tricuspid atresia and an established Glenn shunt (nine patients, Group I) had postoperative hospital stays of 7 to 19 days (average 9.5), and none had significant pleural or pericardial effusions. Patients with tricuspid atresia without a Glenn shunt (seven patients, Group II) had postoperative hospital stays of 6 to 60 days (average 17.5), with three having significant effusions. Of the patients with other complex lesions, all without an established Glenn shunt, five had significant effusions. Four additional major complications (two tricuspid patch disruptions with ineffective pulmonary blood flow and two complete occlusions of a valved conduit) were encountered in which the Glenn shunt proved lifesaving. We believe that an established Glenn shunt played a major role in attaining minimal postoperative hemodynamic instability, effusions, renal failure, and mortality in our patients. The Glenn shunt should be considered in patients who are less than ideal candidates for the Fontan operation.

Adolescent↗
PubMed1983

Unusual mitral valve abnormalities complicating surgical repair of endocardial cushion defects.

A review of 155 cases of surgically repaired endocardial cushion defects revealed 16 patients (10%) with additional unusual mitral valve abnormalities that complicated the surgical procedure. Eight patients had accessory mitral valve tissue that connected the anterior and posterior leaflets to form a double-orifice valve (Group I). In four (50%), the lesion was associated with intermediate atrioventricular canal and small left ventricle; all four died following repair. In the other four, it was associated with ostium primum defect; all survived and are well. A single papillary muscle in the left ventricle was present in six patients (Group II). Two had intermediate atrioventricular canal and both died postoperatively. The other four had complete endocardial cushion defect and three are well following the operation. Perforation of the valve leaflets was present in two patients with ostium primum (Group III). Both patients are well postoperatively. Modification of the surgical technique is required to effect satisfactory repair. The bridge connecting the posterior and anterior leaflets of the mitral valve should be left undisturbed. Otherwise, severe regurgitation may result. In patients with single papillary muscle and complete atrioventricular canal, repair may be accomplished by borrowing from the tricuspid portion of the anterior leaflet, rotating that part posteriorly, and partially closing the cleft. Small perforations of the mitral leaflet do not require closure and do not result in regurgitation. Echocardiographic and angiographic delineation of these abnormalities and thorough intraoperative exploration are important in avoiding pitfalls at the time of repair.

Child, Preschool↗
PubMed1982

Unequal pressures in the central pulmonary arterial branches in patients with pulmonary stenosis. The influence of blood velocity and anatomy.

Significantly different pressures in the right (RPA) and left (LPA) pulmonary artery were observed at catheterization in patients with pulmonary valvar stenosis and no branch stenosis. The lower pressures in the RPA showed a "valley" during systole and were similar in contour and amplitude to the main pulmonary arterial (MPA) pressure; the LPA pressure, however, had a normal contour, and the peak systolic and mean pressures were higher than those in the MPA and RPA. Angiocardiograms, phonograms, and a simple analysis of fluid mechanics suggest that this pressure pattern is related to (1) the high-velocity jet in the MPA and (2) the anatomy of the central pulmonary arterial branches (bifurcation), the LPA originating more distally than the RPA. The high-velocity jet bypasses the origin of the RPA and breaks up in the distal MPA near the origin of the LPA. The kinetic energy is then reconverted into pressure, causing the higher LPA pressures. In patients with transposition of the great arteries and subvalvar pulmonary stenosis, the anatomy of the main pulmonary arterial bifurcation is different from normal, the RPA originating more distally than the LPA. The high-velocity jet may bypass the origin of the LPA and break up near the more distal origin of RPA, and the pressures in the RPA can be higher than those in the MPA and LPA.

Adolescent↗
PubMed1982

Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome.

The association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum and interruption or coarctation of the aortic isthmus has not previously been reported as a syndrome. This combination of anomalies was encountered in five new patients and was previously reported in three. Two patients have undergone surgery with successful results. In contrast to the sagittally oriented conventional proximal aortopulmonary septal defect, the patients in this series had a more distal type of defect, possibly representing a partial persistence of the common arterial trunk. The pulmonary arterial bifurcation may malattach to this undivided truncal segment and, as a result, the right pulmonary artery may be partially or completely shifted into the aorta. This abnormal right pulmonary arterial origin may lead to "steal" from the aortic flow during embryogenesis and to hypoplasia of the aortic arch. This concept is supported by the angiographic observation that the greater the rightward displacement of the right pulmonary artery, the greater the hypoplasia of the arch. The diagnostic angiographic sign is a strikingly high origin of the right pulmonary artery together with aortic arch hypoplasia or atresia. Closure of the aortopulmonary septal defect with implantation of the right pulmonary artery in the pulmonary trunk and repair of the aortic arch anomaly is the recommended surgical treatment.

Aortic Coarctation↗
PubMed1982

Neointimal obstruction of Carpentier-Edwards valved conduit in two patients with modified Fontan procedure.

In 2 patients (single ventricle and dextrotransposition of the great arteries with hypoplastic right ventricle) who underwent a modified Fontan procedure, significant disruption of the tricuspid patch developed. The valved conduits remained patent until reoperation at two and six months, respectively. At the time of reclosure of the tricuspid patch, unavoidable manipulation of the conduits from dissection of adhesions led to detachment and collapse of the neointima, thereby producing complete obstruction of the conduit. The problem was detected immediately in the first patient and prompted clearance of the conduit of the collapsed neointima. In the second patient, the diagnosis was made six months later and conduit replacement was required. Both patients did well following reoperation. We suggest that in patients with woven valved conduits inserted for a Fontan procedure who require reoperation and extensive manipulation of the conduit, removal of the neointima or conduit replacement should be considered.

Adult↗
PubMed1981

Two dimensional echocardiographic evaluation of Mustard operation for d- transposition of the great arteries.

Two dimensional sector scan echocardiography was used to evaluate the morphologic characteristics of the surgically revised atria in 17 patients with d-transposition of the great arteries who had undergone the Mustard operation. Echocardiographic imaging of the atria was obtained from various planar projections. Dimensional measurements of various segments of the systemic and pulmonary venous atria were obtained in each patient. Correlative hemodynamic, angiographic, postmortem and echocardiographic data showed that seven patients (Group I) had no structural abnormalities of the atria. These 7 patients served as controls for 10 other patients with structural abnormalities of the surgically created atria. One patient (Group II) showed stenosis of the junction of the superior vena cava and systemic venous atrium compared with findings in the control group. Three patients (Group III) had significantly reduced echocardiographic dimensions of the junction of the anterior and posterior segments of the pulmonary venous atrium. Six patients (Group IV) had increased echocardiographic dimensions of all components of the pulmonary venous atrium due to tricuspid regurgitation. These data show that qualitative and quantitative two dimensional sector echocardiography can reliably detect structural abnormalities of the surgically revised atria after the Mustard operation.

Adolescent↗
PubMed1981

Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of the intracardiac repair?

Thirteen patients with complex of tetralogy of Fallot and absent pulmonary valve were treated. Three infants presented with severe cardiorespiratory distress (Group I) necessitating surgical intervention in two and intensive medical treatment in the third; there were no late survivals. The remaining 10 patients were operated upon electively at an older age (Group II); in six the ventricular septal defect was closed and the right ventricular outflow obstruction relieved (Group IIA), and in the other four a valve was inserted at the pulmonary anulus at the time of intracardiac repair (Group IIB). There was no hospital mortality in Group II. Five of the six patients in Group IIA remained symptomatic postoperatively, and three needed a subsequent pulmonary valve insertion. All patients in Group IIB were symptom free following operation; one patient began having a progressive decrease in exercise performance, decrease in pulmonary artery size, and cardiothoracic ratio were more marked in those patients who had primary insertion of a pulmonary valve. The clinical results and postoperative assessment of cardiac function tend to indicate that pulmonary valve insertion should be performed at the time of the intracardiac repair.

Adolescent↗
PubMed1980

Exercise performance in tetralogy of Fallot after intracardiac repair.

We evaluated 99 male and 36 female patients with tetralogy of Fallot 1 year or more after intracardiac repair (ICR) in 279 exercise studies between 1968 and 1979. Sixty tetralogy patients were studied serially two to nine times at 12 to 18 month intervals. Work performance and heart rate response to cycle exercise with stepwise loading (100 kg-m/min increments) were (1) related to clinical, pre- and post-ICR cardiac catheterization, surgical, and anatomic information and (2) compared to results obtained in 221 male and 157 female normal control volunteers. On the average, male tetralogy patients were shorter and weighed less than control subjects, but female tetralogy patients as a group showed no growth impairment. Many tetralogy patients had slower resting, submaximal, and final exercise heart rates than did control subjects. Work performance averaged 82% +/- 21% of predicted in male patients and 86% +/- 29% in female patients. Serial studies showed no significant changes in work performance in the majority of tetralogy patients for up to 10 years. Best work performance was found in nine varsity athletes (99% +/- 22%) and 21 patients with primar ICR (94% +/- 23%). Statistical analysis demonstrated a significant relationship between reduced work performance and residual disease, notably cardiac enlargement, increased peak systolic right ventricular pressure, pulmonic valve incompetence (PI), residual ventricular septal defect (VSD), pulmonary hypertension, and cardiac rhythm disturbances. However, in individuals work performance cannot be predicted reliably from the presence of these lesions and should therefore be measured. We conclude that exercise testing is a useful adjunct to the clinical and hemodynamic evaluation of post-ICR tetralogy.

Adolescent↗
PubMed1979

Clinical manifestations of dynamic left ventricular outflow tract stenosis in infants with d-transposition of the great arteries with intact ventricular septum.

Four infants with d-transposition of the great arteries and intact ventricular septum who manifested early clinical symptoms and deterioration due to dynamic left ventricular outflow stenosis are presented. All four had an anatomically adequate atrial septal defect, made at the initial balloon atrial septostomy, that was later confirmed intraoperatively. Two infants continued to have a low arterial oxygen saturation level because of inadequate interatrial mixing, and one of these had severe persistent cyanosis and was treated with the Mustard operation at age 4 days. The other two infants subsequently presented with hypercyanotic spells at age 3 months. All four infants had features of dynamic left ventricular outflow stenosis on hemodynamic, angiocardiographic and echocardiographic studies. The left ventricular outflow pressure gradient was shown to increase after administration of isoproterenol in one infant, and relief of a cyanotic spell with reduction of left ventricular systolic pressure was achieved in another after intravenous administration of propranolol. The Mustard operation relieved symptoms in all infants. The effect of left ventricular outflow tract stenosis on the mechanisms responsible for interatrial mixing in d-transposition of the great arteries with intact ventricular septum is discussed.

Cardiac Catheterization↗
PubMed1979

Pulmonary vascular disease in transposition of the great vessels and intact ventricular septum.

Eight of 135 (6%) children with d-transposition of the great vessels and with intact ventricular septum and no patent ductus arteriosus had evidence of progressive pulmonary vascular disease. Seven of 101 (7%) patients for whom histologic data was available, had Heath-Edwards grades IV or V pulmonary vascular disease, six had grade II, and 88 had either normal or grade I findings. One of 34 patients for whom histologic data was not available had hemodynamic evidence of pulmonary vascular disease at cardiac catheterization after the Mustard operation. When infants younger than 3 months old were excluded, eight of 85 (9%) had advanced pulmonary vascular disease. Twenty-three patients had microthrombi in their pulmonary arteries and arterioles, and in one patient thrombi were observed before the development of pulmonary vascular disease. Clinically unrecognized pulmonary microthrombi are suggested as a possible etiologic agent in the development of pulmonary vascular disease in patients with transposition of the great vessels. Progressive pulmonary vascular disease can first be discovered after the Mustard operation, even in patients without preoperative evidence of pulmonary hypertension or elevated pulmonary vascular resistance.

Adolescent↗
PubMed1979

Taussig-Bing anomaly with straddling mitral valve.

Five patients with the left-sided or intermediate type (Lev) of the Taussig-Bing anomaly were found to have an associated straddling of the mitral valve. In four patients the anomaly of the mitral valve was not recognized preoperatively, and all four died postoperatively. The persistent subpulmonic obstruction caused by the abnormal attachment of the anterior mitral leaflet is considered a significant factor in the poor operative outcome. Retrospective study of angiograms in these four patients revealed diagnostic clues of straddling mitral valve which enabled us to diagnose a fifth patient angiographically and confirm the diagnosis by cross-sectional echocardiography. A surgical approach to correct this association of abnormalities is proposed which avoids operating upon the mitral valve. Its function is thereby preserved, yet the hemodynamic problem caused by the straddling is bypassed. The fifth patient in this series was successfully treated by the proposed operative method.

Adolescent↗
PubMed1977

Pulmonary vascular disease after systemic-pulmonary arterial shunt operations.

More than 1,100 patients underwent a systemic-pulmonary arterial shunt operation at the Children's Memorial Hospital, Chicago, from 1946 to 1974. Of these patients, 294 were studied to assess the presence of pulmonary vascular disease and the time of its occurrence after surgery. They were selected either because lung tissue was available for histologic grading of pulmonary vascular disease (114 patients) or because hemodynamic studies were performed after the shunt operation (180 patients). The shunts, all patent at the time of the study, ranged in duration from 1 month to 23 years. After a subclavan-pulmonary arterial shunt (75 patients) there was little evidence of advatients) pulmonary vascular disease in either histologic C2 of 25 patients) or hemodynamic (1 of 50 patients) studies. After an aortic-pulmonary arterial shunt (44 Waterston, 175 Potts) there was small likelikhood of advanced pulmonary vascular disease with a shunt duration of less than 5 years (1 of 35 histologic, 0 of 27 hemodynamic studies). With a shunt duration of more than 5 years the incidence of advanced pulmonary vascular disease increased significantly (15 of 54 histologic, 17 of 103 hemodynamic studies). A mean pulmonary arterial pressure of 50 mm Hg or greater strongly suggests the presence of advanced pulmonary vascular disease. When a systemic-pulmonary arterial shunt has provided effective palliation with decreased pulmonary pressure there is small likelihood that advanced pulmonary vascular disease will develop, even with a long shunt duration of 20 to 25 years.

Aorta↗