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Biomedical subjects

A J Rein

Publications and source records attributed to A J Rein.

At least 19 recordsLinked to original sources
PubMed1995

Left heart obstructive lesions and left ventricular growth in the midtrimester fetus. A longitudinal study.

BACKGROUND: Isolated case reports that suggest the potential for development of left heart hypoplasia late in gestation provide the only information about the in utero natural history of left heart obstructive lesions. METHODS AND RESULTS: We reviewed the prenatal and postnatal echocardiograms of 21 fetuses with left heart obstructive lesions, including 15 with serial antenatal study, to elucidate the antenatal natural history of this spectrum of disease and to identify features indicative of postnatal disease severity. Ventricular, atrioventricular valve, and great artery dimensions were measured and growth curves were developed with comparisons to data from 47 normal fetuses. Fetuses were divided into groups according to whether postnatally the left heart was capable (group 1, n = 10) or incapable (group 2, n = 7) of supporting the systemic circulation in the presence of a patent aortic valve. Group 3 (n = 4) included fetuses with aortic atresia. At the initial examination (21.7 +/- 3.4 weeks' gestation), left heart dimensions were normal or reduced, with the most diminutive measurements in group 3. Three fetuses in group 2 and most in group 1 had normal initial left heart dimensions. Subsequent growth of left heart structures either paralleled normal growth or was reduced, the latter resulting in the development or progression of left heart hypoplasia. All left heart dimensions grew more slowly in group 2 and group 3 than in group 1 (P < .05). Other prenatal features observed only in groups 2 and 3 included reversed (n = 10) or bidirectional (n = 1) foramen ovale flow and retrograde distal arch flow (n = 9). Initial midtrimester mitral valve and ascending aorta z scores and the growth rates of all left heart structures correlated strongly with postnatal left ventricular end-diastolic dimension (P = .0007 to .03, r = .57 to .82) and could be additional indicators of postnatal disease severity. One group 1 fetus developed severe aortic stenosis late in gestation. CONCLUSIONS: The potential for the in utero development or progression in severity of left heart obstruction and hypoplasia in left heart obstructive lesions necessitates serial prenatal study in affected fetuses carried to term.

Echocardiography↗
PubMed1992

Prenatal diagnosis of tetralogy of Fallot with absence of the leaflets of the pulmonic valve.

Tetralogy of Fallot with absence of the leaflets of the pulmonic valve is relatively rare. We describe the prenatal diagnosis of this malformation in two fetuses. The association between the agenesis of the arterial duct and absence of the valvar leaflets is discussed. The diagnosis of the syndrome in the presence of dilated and pulsatile pulmonic arteries is easy to make. It should allow early counselling and planning of early care after delivery.

Abnormalities, Multiple↗
PubMed1992

Recombinant interleukin-2 and interferon alpha immunotherapy following autologous bone marrow transplantation. A case report of cardiovascular toxicity with serial echocardiographic evaluation.

The most serious side effects of recombinant interleukin-2 (rIL-2) and recombinant interferon-alpha (rIFN alpha) immunotherapy are cardiovascular disturbances, including systemic hypotension, left-ventricular dysfunction and pulmonary edema. We present a 25-year-old female who developed reversible cardiogenic shock during intermediate dose rIL-2 and low dose rIFN alpha therapy. Rapid clinical improvement occurred after intravenous fluid and dopamine support. A serial echocardiographic evaluation, which has not been described previously in this setting, is reported.

Adult↗
PubMed1990

Genetics of conotruncal malformations: review of the literature and report of a consanguineous kindred with various conotruncal malformations.

Genetic predisposition in congenital heart disease is considered to be a component of multifactorial inheritance. Recently, monogenic inheritance in conotruncal malformations has been suggested. We describe a consanguineous kindred with various conotruncal malformations, the presence of which lends support to the idea that this spectrum of malformation is monogenically inherited. Theoretical background and experimental and clinical data are reviewed and discussed.

Consanguinity↗
PubMed1989

Right ventricular outflow tract obstruction due to extracardiac tumors. A report of three cases diagnosed and followed up by echocardiographic studies.

Echocardiography has become a valuable diagnostic tool in various clinical conditions. Its use for the detection of extracardiac tumors has seldom been reported. The majority of these descriptions are of single case reports. We have recently encountered three patients (two with lymphomas and one with seminoma) who presented with signs and symptoms suggestive of right ventricular outflow tract obstruction. Two-dimensional echocardiography enabled the prompt diagnosis of extracardiac tumors compressing the heart. Moreover, echocardiography proved to be an excellent noninvasive tool for assessing the success of therapy for mediastinal tumors.

Adult↗
PubMed1988

Intracardiac causes of superior vena cava obstruction.

Most causes of superior vena cava (SVC) obstruction are extracardiac. In rare instances, an intracardiac process may obstruct the venous return from the SVC. This is illustrated by a report of a patient with a congenital coronary artery fistula to the right atrium obstructing the SVC return. We propose a classification and clinical approach to the differential diagnosis of SVC obstruction.

Child, Preschool↗
PubMed1988

An unusual presentation of right coronary artery fistula.

A four year old girl with infective endocarditis had unexplained facial swelling. Cross sectional echocardiography showed that a large right coronary artery fistula to the right atrium was obstructing the distal superior vena cava. The diagnosis was confirmed by cardiac catheterisation and at operation. The child was symptom free one year after operation.

Child, Preschool↗
PubMed1987

Regional and global left ventricular function in infants with anomalous origin of the left coronary artery from the pulmonary trunk: preoperative and postoperative assessment.

Anomalous origin of the left coronary artery from the pulmonary trunk (ALCA) remains a diagnostic as well as a therapeutic problem. The purposes of this study were: (1) to analyze left ventricular mechanics, including regional wall motion, in infants with ALCA, (2) to determine if the pattern of wall motion in infants with ALCA distinguishes these patients from those with congestive cardiomyopathy of other causes, and (3) to evaluate the potential for recovery of left ventricular function after successful restoration of a dual coronary artery system. Left ventricular mechanics were studied before and serially after surgery in six infants (2 to 13 months old) with ALCA. Fifteen age-matched normal subjects and seven age-matched patients with idiopathic congestive cardiomyopathy were also studied for comparison. Preoperatively, the end-diastolic volume in infants with ALCA was about four times larger than normal and did not differ from that in infants with CM; the myocardial volume was also about three times larger than normal, similar to that in the patients with CM. The myocardial volume/end-diastolic volume ratio was extremely low in patients with ALCA and in those with CM. The infants with ALCA did not exhibit specific segmental wall motion abnormalities but rather had global hypokinesis indistinguishable from that in the patients with CM. After successful repair, end-diastolic volume index rapidly decreased, reaching near normal values by 7 to 22 months after surgery, while myocardial volume index decreased at a slower rate, leading to an early phase of "overshoot hypertrophy," but reaching normal values by 7 to 22 months after surgery.+

Cardiomyopathy, Dilated↗
PubMed1987

Left ventricular mechanics in the normal newborn.

The transition from fetal to neonatal circulatory status is accompanied by marked alteration in relative right and left ventricular systolic and diastolic pressure. These alterations would be expected to influence both global and regional performance of the left ventricle. To address this issue, sequential two-dimensional echocardiographic studies were performed in normal newborns during the first days of life. Global and regional left ventricular wall motion were quantified by computer digitization with the use of an automated edge detection algorithm and a floating-center-of-mass model. Comparison was made with a control group of normal infants and young children and the sequential change over the first 5 days of life was assessed. Newborns were found to have a circular left ventricular configuration at end-diastole beginning on day 1. At end-systole, however, there was significant left ventricular distortion due to septal flattening, which persisted until day 3 and resolved entirely by day 5 of life. Regional wall motion analysis demonstrated a corresponding augmentation of septal and contralateral left ventricular free wall systolic movement during the first days of life, with a normal pattern attained by day 4. Due to the nonhomogeneity of the left ventricular wall motion in the first few days of life, standard single-dimension shortening fraction provided an unreliable measure of global left ventricular performance before day 4. Thus, systolic right ventricular hypertension at a level sufficient to distort the left ventricular configuration is present until day 4 or 5 of life, resulting in altered left ventricular regional wall motion. As a result, usual M mode echocardiographic assessment of left ventricular function is unreliable in this age group.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1987

Anomalous origin of left coronary artery from pulmonary artery. Ligation versus establishment of a two coronary artery system.

Between 1959 and 1985, 24 patients (mean age 38 months, range 15 days to 13 years) with anomalous origin of the left coronary artery from the pulmonary artery as an isolated lesion were treated surgically at Children's Hospital, Boston. In 11 cases a left coronary-to-aortic tunnel was created with a pulmonary artery baffle (Takeuchi) with no deaths either early or late over a mean follow-up period of 18 1/2 months. Late complications of this procedure include moderate aortic regurgitation (one), supravalvular pulmonary stenosis (two-one required a second operation), obstructed baffle (one-asymptomatic). In 11 cases of coronary ligation or ostial closure there was a 27% early mortality and a 25% late mortality over a mean follow-up period of 10 1/2 years. Late complications include residual shunt (three-two required a second operation), severe mitral regurgitation (one), and recurrence of angina (one). Two patients had other procedures. Both early and late deaths occurred in the group who had congestive heart failure and who had simple ligation. Five infants who had profoundly depressed ventricular function and moderate to severe mitral regurgitation, together with widespread Q waves on electrocardiogram, showed a dramatic improvement in ventricular function after the Takeuchi procedure. The Takeuchi procedure is a simple and effective means of establishing a two coronary artery system in the child with anomalous origin of the left coronary artery from the pulmonary artery. This procedure is particularly recommended over coronary ligation in patients in congestive heart failure.

Adolescent↗
PubMed1986

Severe hydrops fetalis due to congenital supraventricular tachycardia.

Intrauterine supraventricular tachycardia is one of the main causes of non-immunological intrauterine hydrops fetalis. Without early diagnosis and treatment it may terminate in fetal death or delivery of a baby with severe hydrops and extreme heart failure. With the improvement in non-invasive imaging techniques in prenatal medicine, this condition can be diagnosed early and treated successfully. A case is described of such a newborn diagnosed in utero and treated before and after delivery, and the different therapeutic approaches to congenital supraventricular tachycardia are discussed.

Adult↗
PubMed1986

Cardiac output estimates in the pediatric intensive care unit using a continuous-wave Doppler computer: validation and limitations of the technique.

We compared the cardiac index obtained by means of a continuous-wave Doppler computer with simultaneous thermodilution measurements in 25 children in the pediatric intensive care unit (40 observations). The aortic diameter was measured at various levels to determine which provided the best measure of cardiac index. The Doppler measurements were performed independently by a physician trained in Doppler cardiography and by a nurse with no experience in echocardiography. Both the nurse and physician obtained high-quality flow velocity recordings in all patients in a mean time of 5 minutes or less. Cardiac index and total systemic vascular resistance measured by means of Doppler and thermodilution techniques were highly correlated (r = 0.86 and r = 0.93, respectively). The highest correlation was obtained when Doppler cardiac index was computed by means of the internal diameter measured at the aortic anulus. There was no significant difference between the nurse's and physician's measurements. We conclude that cardiac index can be accurately determined in the pediatric intensive care unit by means of continuous-wave Doppler computer, even when operated by personnel not trained in Doppler cardiography.

Cardiac Output↗