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Biomedical subjects

A J Muster

Publications and source records attributed to A J Muster.

At least 91 records · Page 5Linked to original sources
PubMed1977

Angulation of the inferior vena cava-right atrial junction in children with repaired omphalocele.

Three children with repaired omphalocele underwent diagnostic cardiac catheterization. In addition to intracardiac shunts, each was found to have marked angulation at the junction of the inferior vena cava and the right atrium. After review of our autopsy material following omphalocele repair, it is suggested that the inferior vena cava - right atrial angulation is due to gradual abnormal fixation of this junction either in utero or related to the surgical repair. A change in current surgical therapy is NOT recommended. Angulation of the inferior vena cava - right atrial junction is potentially important because a) it may interfere with venous cardiac catheterization from "below", b) it poses a danger to inferior vena caval cannulation for cardio-pulmonary by-pass, and c) it may become obstructive with time producing portal hypertension.

Cardiac Catheterization↗
PubMed1977

Echocardiographic assessment of the relation between left ventricular wall and cavity dimensions and peak systolic pressure in children with aortic stenosis.

Echocardiographic measurements of the left ventricular end diastolic minor axis and posterior and septal wall thickness were obtained in 19 children with congenital aortic stenosis with left ventricular peak systolic pressures ranging from 110 to 225 mm Hg at cardiac catheterization. From these measurements were derived (1) the left ventricular peak circumferential wall stress, (2)the end-diastolic h/r ratio (that is, mean of septal and posterior wall thickness (h) to minor semiaxis (r) ratio), and (3) the LVM/LVV ratio (that is, left ventricular mass (LVM) to left ventricular end-diastolic volume (LVV) ratio). The peak stress was found to be within the normal range and independent of the left ventricular peak systolic pressure. The end-diastolic h/r and LVM/LVV ratios had highly significant linear relations to the left ventricular peak systolic pressure. It is concluded that these easily determined echocardiographic measurements provide a useful noninvasive means of assessing left ventricular peak systolic pressure in patients with aortic stenosis without myocardial decompensation.

Adolescent↗
PubMed1977

Transposition of the great arteries with intact ventricular septum and patent ductus arteriosus.

At the time of initial balloon atrial septostomy a patent ductus arteriosus was found with angiography in 39 of 81 infants with transposition of the great arteries with intact ventricular septum. By angiographic criteria the ductus shunt was considered small in 21 infants and large and significant in 18. In contrast to the usual clinical presentation of neonates with transposition and intact ventricular septum, 12 of these 18 infants with a significant patent ductus arteriosus had only slight cyanosis and 8 presented with tachypnea out of proportion to the degree of cyanosis. Ten of the 18 infants had no continuous murmur, bounding pulses, mid-diastolic rumble or differential cyanosis. Clinically occult narrowing or closure occurred, presumably gradually and relatively late, in six infants. Acute early narrowing or closure, spontaneous (six infants) or surgically produced (three infants), occurred usually within the 1st month of life and was associated with a marked decrease in arterial oxygen saturation in eight infants, often with a rapid clinical deterioration. Persistence of a large patent ductus arteriosus for several months appears to be associated with an increased incidence of early pulmonary vascular disease. Therapeutic considerations for the infant with a large patent ductus arteriosus after initial balloon atrial septostomy include: (1) careful initial follow-up of the infant in clinically stable condition in case the ductus arteriosus should acutely narrow or close and the patient require urgent palliative or corrective surgery; (2) urgent early closure of the ductus in the infant with overt left heart failure with concurrent atrial septectomy or preferably primary corrective surgery; and (3) elective closure of a persistent significant patent ductus arteriosus before age 4 months with concurrent corrective surgery in the infant in clinically stable condition.

Abnormalities, Multiple↗
PubMed1977

d-Transposition of the great vessels in the neonate. A clinical diagnosis.

We reviewed the records of 66 neonatal patients with d-transposition of the great vessels (d-TGV) admitted to our institution. The initial history, physical examination by the cardiologist, chest rotengenogram, ECG, hemoglobin level, and hematocrit value were analyzed. Although all patients all patients were cyanotic and 65% were tachypneic, other signs of a major congenital cardiac anomaly were usually absent. The number of clinical signs (poor general status, cyanosis, respiratory distress, S2 increased and single, murmur, classical chest roentgenogram, abnormal ECG) present in each patient was three or less in 41%, only 13.5% had more than five signs.

Aorta↗
PubMed1976

Discrete subvalvular aortic stenosis in childhood. Study of 51 patients.

Fifty-one children with discrete subvalvular aortic stenosis were studied between 1951 and 1974. The three anatomic types of obstruction found were the thin membranous type (43 cases), the fibromuscular collar type (5 cases) and the tunnel type (3 cases). The obstruction was usually severe, and the median left ventricular to aortic systolic pressure gradient was 90 mm Hg. Progressive obstruction with an increasing gradient was documented in 10 patients by serial cardiac catherizations. Significant associated cardiac defects, present in 57 percent of patients, often masked the typical clinical and cardiac catheterization features of subaortic stenosis. The stenosis was often not discovered until after surgery for the associated defect. Forty patients underwent surgical resection of the discrete subaortic obstruction. After surgery significant left ventricular to aortic pressure gradients can be found at postoperative cardiac catheterization. These gradients may reflect inadequate resection of the more complex discrete obstructions or represent proliferation and regrowth of the previously resected subvalvular fibrous tissue. The criteria for operability of discrete subaortic stenosis should be the angiographic demonstration of a discrete subvalvular diaphragm and the presence of a resting left ventricular to aortic systolic pressure gradient of 40 mm Hg or more.

Adolescent↗
PubMed1976

Echocardiographic localization of interatrial baffle after Mustard operation for dextrotransposition of the great arteries.

The position of the interatrial baffle was determined by echocardiography in 11 patients with complete transposition of the great arteries who had undergone the Mustard operation. The location of the interatrial baffle and the newly created systemic venous atrium and the pulmonary venous atrium behind the pulmonary arterial root was established by echocardiographic contrast studies during cardiac catherization. Angiographic analysis indicated that the segments of the systemic and pulmonary venous atria seen behind the pulmonary arterial root in the echocardiogram were, respectively, the superior limb of the systemic venous atrium and the posterior segment of the pulmonary venous atrium. The area of mitral-pulmonary valve continuity in the echocardiogram was used as a reference point for dimensional measurements of the systemic and pulmonary venous atria. It is suggested that longitudinal measurements of these structures by echocardiogram may help evaluate their growth in size. Fine to coarse fibrillatory movements of the atrioventricular valves and, more frequently, of the mitral valve were seen in the majority of patients.

Cardiac Catheterization↗
PubMed1976

Asymmetric distribution of the pulmonary blood flow between the right and left lungs in d-transposition of the great arteries.

Pulmonary angiograms, radionuclide lung images and chest roentgenograms were evaluated regarding the incidence, magnitude and natural evolution of maldistribution of the pulmonary blood flow between the lungs in 63 patients with dextrotransposition of the great arteries. Approximately half of these patients had some degree of greater perfusion of the right relative to the left lung. A significant correlation was demonstrated between the incidence of this maldistribution of blood flow and the angulation between the main and the right pulmonary arteries. For any given angulation between these vessels, additional pulmonary stenosis increased the incidence of disparity in perfusion. Our observations suggest the following developmental mechanisms: The maldistribution in flow results from the abnormal rightward inclination of the main pulmonary artery in the transposition malformation which straightens the flow axis from the main to the right pulmonary artery. Under these circumstances the momentum of the blood in the main pulmonary artery carries the blood preferentially into the right pulmonary artery. This momentum is increased when there is stenosis of the left ventricular outflow tract. Consequent differences in the mechanical properties of the two pulmonary vascular beds can increase this maldistribution. The disparity in perfusion between the lungs is not present in newborns with d-transposition, appears to be progressive in severity and in time may result in almost complete cessation of effective perfusion of the left lung. The effect of the Mustard operation on this abnormality of flow is discussed.

Angiocardiography↗
PubMed1976

Ascending aorta-right pulmonary artery shunt.

Seventy-seven patients with ascending aorta-right pulmonary artery shunt were reviewed; 48 had tetralogy of Fallot, 9 had pulmonary atresia, 11 had transposition of the great vessels with pulmonary stenosis, 4 had tricuspid atresia, and 5 had miscellaneous complex lesions. Their ages ranged from one day to 13 years. The over-all mortality rate was 17.8 per cent. Ten patients underwent total repair and takedown of the shunt with no deaths. The problems of increased pulmonary flow, pulmonary hypertension, preferential flow to one lung, kinking and distortion of the pulmonary artery, and the technical difficulties at the time of takedown were reviewed and discussed.

Aorta↗
PubMed1975

Cor pulmonale secondary to upper airway obstruction. Cardiac catheterization, immunologic, and psychometric evaluation in nine patients.

Previous reports of cor pulmonale due to upper airway obstruction have not noted that chronic hypoxemia due to alveolar hypoventilation can lead to left as well as right ventricular failure in these patients. We found elevated left ventricular end-diastolic pressure in four of five patients. Although these patients have frequent respiratory infections, no investigation of their immune status has previously been reported. We performed such investigations but could not demonstrate an immunologic deficiency. Although nine of 44 patients previously reported were mentally retarded, no psychometric data were available. We studied five patients but could not determine the role of mental subnormality in this syndrome. Diagnosis of this syndrome should be suspected when clinical and roentgenographic findings of biventricular heart failure are associated with right ventricular hypertrophy on electrocardiogram. The diagnosis of alveolar hypoventilation is confirmed by blood gas findings and examination of the upper airways. Surgical removal of obstructive tissue reverses the cardiac involvement.

Adenoidectomy↗
PubMed1975

Persistence of the fetal cardiopulmonary circulatory pathway: survival of an infant after a prolonged course;.

The first case of "idiopathic" persistence of the fetal cardiopulmonary circulatory pathway with survival after a prolonged course is documented by serial cardiac catheterizations. All previously reported infants have either markedly improved within the first week of life or subsequently died. This entity has been described in term infants with prenatal or perinatal distress who present with tachypnea and cyanosis from birth. The chest radiograph is remarkable for the absence of pulmonary parenchymal pathology and the hemoglobin and hematocrit are normal. Blood gas determinations indicate hypoxia and acidosis with or without hypercarbia. Cardiac catheterization and angiography reveal an anatomically normal heart with severe pulmonary hypertension, left ventricular pressure lower than right ventricular pressure, and right-to-left venoarterial shunting through the fetal channels (atrial and or ductal levels). It is postulated that this entity may result from antenatal factors that affect the pulmonary vasculature and its subsequent adjustment to extrauterine life. These factors may include abnormal intrauterine stress resulting in excessive hypertrophy of the pulmonary arteriolar smooth muscle, or abnormal pulmonary vascular responsiveness to the usual vasoactive stimuli before or after birth, or an immature or dysfunctioning enzyme system necessary for the normal decrease in pulmonary artery pressure.

Angiography↗