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Biomedical subjects

F Z Bierman

Publications and source records attributed to F Z Bierman.

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

Cardiovascular adaptations to transfusion/chelation therapy of homozygote sickle cell anemia.

The effect of transfusion/chelation therapy on the cardiovascular adaptations to chronic anemia in pediatric and young adult patients with homozygous sickle cell disease is uncertain. This study compares left ventricular (LV) function indexes and thoracoabdominal aortic systolic and diastolic blood flow in nontransfused and transfused patients with homozygous sickle cell disease. The study population consisted of 29 nontransfused patients with homozygous sickle cell disease, ages 0.4 to 20.9 years (group 1) and 11 chronically transfused/chelated patients, ages 4.0 to 21.8 years (group 2). The mean total hemoglobin concentration in group 2 was 28% greater than that in group 1. The mean duration of transfusion/chelation therapy in group 2 was 3.7 years. The percent of predicted LV end-diastolic and end-systolic dimensions were significantly greater than the respective controls in both groups. There was no significant difference in percent of predicted LV end-diastolic dimension (group 1, 120 +/- 12%; group 2, 120 +/- 12%) or percent of predicted LV end-systolic dimension (group 1, 120 +/- 12%; group 2, 117 +/- 8) between the groups. The percent of LV shortening fraction was similar in study groups and control subjects. Aortic systolic blood flow (cc/min/m2) for group 1 (2,426 +/- 841) and 2 (2,374 +/- 1.004) were significantly greater than corresponding control values (1,683 +/- 442, 1,736 +/- 430, respectively). Aortic diastolic blood flow was significantly greater than corresponding control values for both group 1 (699 +/- 313 vs 488 +/- 212) and group 2 (1,080 +/- 607 vs 588 +/- 219).(ABSTRACT TRUNCATED AT 250 WORDS)

Adaptation, Physiological↗
PubMed1987

Intrauterine therapy for presumptive fetal myocarditis with acquired heart block due to systemic lupus erythematosus. Experience in a mother with a predominance of SS-B (La) antibodies.

An experimental therapeutic regimen for congenital lupus erythematosus, which consists of treating the mother with high doses of dexamethasone and performing plasmapheresis (3 times per week), was developed. This regimen was administered to a woman whose pregnancy was complicated by the abrupt onset of fetal pericarditis, myocarditis, and complete heart block in the twenty-fourth week of gestation. The effectiveness of plasmapheresis was evidenced by a decrease in the very high titer of SS-B (La) antibodies in the mother. After 5 weeks of therapy, the cardiac disease was ameliorated. The infant tolerated delivery at week 31, and the heart block has persisted. The rationale for this therapy for fetal disease is discussed, with emphasis on 2 goals: to diminish the quantity of maternal antibody that is transmitted to the fetus, and to suppress the manifestations of fetal carditis with the use of glucocorticoids that are not inactivated by the placenta. Tentative recommendations for more intensive prenatal monitoring of maternal and fetal status for those at risk are outlined.

Adult↗
PubMed1987

Pulmonary venous obstruction and atrioventricular canal anomalies: role of cor triatriatum and double outlet right atrium.

Two-dimensional echocardiographic findings and surgical repair in three infants with atrioventricular (AV) canal and pulmonary venous obstruction are described. The AV canal was complicated by cor triatriatum in two patients and by double outlet right atrium in the other. In patients with AV canal, anatomic obstruction of pulmonary venous return should be excluded by detailed cardiac ultrasound examination.

Cor Triatriatum↗
PubMed1986

Hypoplastic left heart syndrome: report of a unique survivor.

A remarkable patient is described, a child who has survived until the age of 7 years with hypoplastic left heart syndrome (mitral and aortic atresia) without surgical intervention. The child has led an active, normal life and, aside from minimal cyanosis, has remained asymptomatic. The unique clinical course for this patient is the result of a number of favorable hemodynamic factors that have not been previously reported in an individual patient with hypoplastic left heart syndrome and intact ventricular septum widely patent ductus arteriosus, adequate retrograde coronary flow, unrestricted pulmonary venous return, and absence of significant vascular obstructive disease. This documentation of long-term survival in a child without surgical treatment for mitral and aortic atresia suggests that successful early palliative treatment for infants with this syndrome could also result in a favorable prognosis.

Angiocardiography↗
PubMed1985

Accuracy of prospective two-dimensional echocardiographic evaluation of left ventricular outflow tract in complete transposition of the great arteries.

Thirty-two consecutive infants with transposition of the great arteries (TGA) and ventricular septal defect underwent subxiphoid 2-dimensional echocardiography (2-D echo). Two independent observers prospectively evaluated each echocardiogram for the presence or absence of left ventricular (LV) outflow tract obstruction, whether outflow obstruction was dynamic or fixed, or both, and the precise anatomic type of fixed obstruction. Compared with the LV-to-pulmonary artery gradient determined at cardiac catheterization, 2-D echo yielded low false-negative (7 to 13%) and false-positive (0 to 6%) rates for diagnosing the presence or absence of LV outflow tract obstruction. Moreover, the false-negative cases were only minor errors, because the measured LV-pulmonary artery gradients proved to be less than 25 mm Hg. Compared with the long-axial oblique LV angiogram, 2-D echo yielded no false-negative results in detection of outflow tract obstruction, which was at least partly fixed. Compared with autopsy/surgical observation, 2-D echo made no significant errors in delineating the exact anatomic type of fixed obstruction. The diagnostic accuracy of 2-D echo in detecting and characterizing LV outflow tract obstruction limits the need for "routine" cardiac catheterization before repair in infants with TGA and intact ventricular septum. Furthermore, because certain types of fixed LV outflow tract obstruction are difficult for the surgeon to visualize and alleviate, precise knowledge of the anatomic type of fixed obstruction influences the choice among Rastelli, intraatrial baffle and arterial switch procedures in patients with TGA and ventricular septal defect.

Cardiac Catheterization↗
PubMed1985

"Bedside" balloon septostomy in infants with transposition of the great arteries: new concepts using two-dimensional echocardiographic techniques.

We performed balloon atrial septostomies in six successive infants with transposition of the great arteries, using echocardiographic guidance at the bedside in the neonatal intensive care unit. In all cases adequate septostomy was obtained and instantaneously assessable. PaO2 values increased as expected (mean before septostomy 28 mm Hg; after, 42 mm Hg). In cases of transposition clearly diagnosed echocardiographically, this intervention need not be done in the catheterization laboratory. The need for a full hemodynamic study in the neonatal period is arguable.

Cardiac Catheterization↗
PubMed1985

Two-dimensional echocardiography in the older child.

The role of two-dimensional echocardiography in the management of any congenital cardiovascular anomaly depends on the lesion's natural history and surgical alternatives. Diagnostic assessment of subaortic stenosis and postoperative follow-up of Fontan and Mustard/Senning procedures and tetralogy of Fallot reconstruction illustrate the application of two-dimensional echocardiography to the management of these problems in the older child. Two-dimensional echocardiography displays the static anatomic as well as dynamic functional sequels of left ventricular outflow obstruction. Detailed anatomic display of Fontan reconstruction is compromised by its somewhat unpredictable spatial organization. Combining two-dimensional imaging with gated pulsed Doppler techniques, however, supplements limited anatomic definition with flow profile data specific to the varied surgical interpretations of the Fontan principle. In a similar manner, combined imaging after Senning/Mustard procedures permits detailed anatomic and hemodynamic interrogation of pulmonary and systemic venous return to the neo-left and neo-right atria. The limitations of cardiac ultrasound imaging of the older child after tetralogy of Fallot reconstruction offer some insight into the future directions that are necessary in the application of this imaging technique. Imaging of these patients is compromised by the prosthetic material, which compromises the assessment of right ventricular outflow pulmonary artery reconstruction and ventricular septal closure. Although gated pulsed Doppler echocardiography overcomes some of these limitations, better tissue characterization in the presence of bioprosthetic material is necessary.

Aortic Valve Stenosis↗
PubMed1984

Two-dimensional echocardiography and its influence on cardiac catheterization.

Successful management of congenital heart disease requires complete anatomic definition and timely surgical intervention. Preoperative anatomic detail is enhanced by coordination of different imaging disciplines. Precatheterization cardiac ultrasound avoids unnecessary catheter manipulation, excess radiation and reduces contrast volume. Detailed noninvasive imaging, coupled with an understanding of the natural history and surgical alternatives, obviate the need for preoperative cardiac catheterization in selected lesions. Cardiovascular anomalies which, in a well defined set of circumstances, may be exclusively managed by two-dimensional echocardiography include sinus venosus and secundum type interatrial septal defects, severe aortic stenosis in infancy, and hypoplastic left heart syndrome. The "well defined set of circumstances" are particular to each lesion and must include the patient's history, physical examination, electrocardiogram, and chest X-ray. Definitive management of a cardiovascular anomaly entails consideration of the relative superiority and cost benefit advantages of the different imaging disciplines.

Aortic Valve↗
PubMed1984

Two-dimensional echocardiographic diagnosis of intracardiac masses in infancy.

Intracardiac masses in infancy are uncommon. Tumors, thrombi and vegetations of bacterial endocarditis are exceedingly rare in this age group. These masses are seldom diagnosed before the infant's death. In a review of two-dimensional echocardiograms performed from May 1979 to January 1981 on 741 infants less than 2 years of age, intracardiac masses were prospectively identified in 6 patients. All six patients presented with unexplained murmurs associated with either hemodynamic instability, arrhythmias or systemic emboli. One patient had a vegetation from bacterial endocarditis, one had an intracardiac thrombus associated with myocarditis, three had rhabdomyomas (two patients with tuberous sclerosis) and one had a fibro-fatty nodule attached to the eustachian valve. Two-dimensional echocardiography not only was important in the diagnosis, but also provided guidance in the medical and surgical treatment of these patients.

Echocardiography↗
PubMed1984

Absence of the aortic valve: antenatal and postnatal two-dimensional and Doppler echocardiographic features.

Antenatal and postnatal two-dimensional and gated pulsed Doppler echocardiography, beginning at 32 weeks' gestation, were used to evaluate the cardiovascular contribution to nonimmunologic hydrops in a gravida 1, para 0 nineteen year old woman. Antenatal two-dimensional imaging demonstrated a hypoplastic left heart variant of complete common atrioventricular (AV) canal. Antenatal gated pulsed Doppler examination of the fetal thoracoabdominal aorta revealed atypical pandiastolic retrograde flow. Postnatal noninvasive examination at 36 weeks' gestational age was unchanged from the prenatal study. Postmortem examination confirmed the noninvasive anatomic findings and revealed polysplenia. The mechanism of pandiastolic retrograde flow was attributable to "absence of the aortic valve," a previously unreported congenital cardiac anomaly, which resulted in severe antenatal and postnatal aortic regurgitation.

Abnormalities, Multiple↗
PubMed1983

2-dimensional echocardiographic appearance of complete left-sided juxtaposition of the atrial appendages.

This report describes the 2-dimensional (2-D) echocardiographic appearance of left-sided juxtaposition of the atrial appendages (JAA). From January 1, 1978, to June 30, 1979, 2 infants had the diagnosis of left-sided JAA at autopsy. Both patients had previously been examined by subxiphoid 2-D echocardiography. On review of these studies, the septum secundum was found to be oriented posteriorly in the transverse view of the atria. From July 1979 to June 1981, prospective evaluation of all infants for the presence of this finding revealed 2 more patients. Left-sided JAA was confirmed at surgery in 1 case and by selective right atrial angiography in the other. The diagnosis of left-sided JAA has important implications for both atrial baffle operations and for the Fontan procedure.

Echocardiography↗
PubMed1983

Subxyphoid 2-dimensional echocardiographic identification of left ventricular papillary muscle anomalies in complete common atrioventricular canal.

Mitral valve dysfunction is probably the major cause of operative mortality from total repair of complete common atrioventricular (AV) canal in infancy. The presence of a solitary left ventricular (LV) papillary muscle appears to be 1 anatomic factor influencing the success of mitral reconstruction because suturing of the cleft between the superior and inferior components of the anterior mitral leaflet creates a parachute mitral valve deformity, which may result in stenosis or in unduly high tension on the components of the repair. This study reports on (1) the 2-dimensional (2-D) echocardiographic appearance of the LV papillary muscle architecture in patients with complete common AV canal compared with that in normal subjects, and (2) the incidence of solitary LV papillary muscle in patients with complete common AV canal. Two-dimensional echocardiography was performed in 31 infants with complete common AV canal, 14 normal infants, and 9 infants with a large ventricular septal defect not involving the AV canal region. Of 31 infants with complete common AV canal, 26 (80%) had 2 LV papillary muscles on 2-D echocardiography, 3 (10%) had 3 LV papillary muscles, and 3 (10%) had 1 LV papillary muscle. In patients with 2 LV papillary muscles, the anterolateral papillary muscle was displaced posteriorly compared with that in normal subjects and in patients with ventricular septal defect, whereas the posteromedial papillary muscle was in its normal location. Among the 25 patients with complete common AV canal with 2 LV papillary muscles, there was 1 operative death. Among the 6 infants with complete common AV canal with LV papillary muscle anomalies, 5 underwent surgical repair with 4 early deaths. Subxyphoid 2-D echocardiography is a useful technique for evaluating LV papillary muscle architecture in complete common AV canal and permits identification of patients who may be at higher risk for unsuccessful mitral reconstruction.

Echocardiography↗