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Biomedical subjects

J I Brenner

Publications and source records attributed to J I Brenner.

At least 37 records · Page 2Linked to original sources
PubMed1987

Early detection of congenital cardiovascular malformations in infancy.

In an ongoing population-based study of congenital cardiovascular malformations in the Baltimore-Washington, DC, area, 1527 affected infants were ascertained from multiple sources during the years 1981 to 1984. Ninety-eight percent were evaluated at a regional pediatric cardiology center. Among the unreferred cases, in which the cardiac defect was diagnosed only at autopsy, most infants died in the first week of life and had associated problems, such as low birth weight, major noncardiac malformations, or other life-threatening illnesses, but a few infants with potentially remediable heart disease escaped clinical detection. Until preventive measures become available, reduction of infant mortality due to congenital cardiovascular malformations will continue to depend on early recognition of signs of serious heart disease in infants and on effective community-wide use of specialized cardiac services.

Catchment Area, Health↗
PubMed1987

Cardiac and noncardiac malformations: observations in a population-based study.

A regional case-control study of congenital cardiovascular malformations (CCVMs) searches for all live-born infants in the community in whom the cardiac diagnosis has been confirmed by echocardiography, cardiac catheterization, surgery, or autopsy. Their families are studied in comparison to those of a representative sample of resident live-born infants. Detailed descriptions of noncardiac abnormalities are obtained from physician reports and maternal interviews expanded by medical record and death certificate data. Among 1,494 cases and 1,572 controls, chromosomal abnormalities, syndromes, heritable disorders, and suspect syndromes occurred with an overwhelming excess in cases (chromosomes, P less than 10(-4); syndromes/heritable disorders, P less than .005). Abnormalities affecting chromosomes 13, 18, and 21 constituted 93% of the cytogenetic defects. Syndromes and heritable disorders were of 39 types. Nonsyndromic abnormalities were three times more frequent in cases than in controls (P less than .005). Case excesses occurred for central nervous system malformations, eye disorders, major abdominal wall defects, and abnormalities of the alimentary and urinary tracts. Severe anomalies frequent among cases were those which also occur in certain recognized syndromes, and it is suggested that paired combinations of cardiac and other midline anomalies may represent "formes frustes" of syndromes with similar though variable phenotypic expressions. Cleft lip and palate, inguinal hernia, and lower limb anomalies occurred with equal frequency, suggesting their association with CCVMs by chance alone.

Chromosome Aberrations↗
PubMed1985

Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study.

The Baltimore-Washington Infant Study is a regional epidemiologic study of congenital heart disease. Among Infants born in the study area in 1981 and 1982, 664 had a diagnosis of congenital heart disease confirmed in the first year of life by echocardiography, cardiac catheterization, cardiac surgery, or autopsy. The prevalence rate was 3.7/1,000 livebirths for all cases and 2.4/1,000 livebirths for cases confirmed by invasive methods only. Diagnosis-specific prevalence rates of congenital heart disease are compared with those of eight previous case series. Changing diagnostic categorizations in the time span covered and methodological differences resulted in great variation of the data. However, the data of the New England Infant Cardiac Program which used the same case discovery methods showed similar occurrences of major morphologic abnormalities, suggesting that these are stable basic estimates in the eastern United States. For all case series, the rate of confirmed congenital heart disease was approximately 4/1,000 livebirths over the 40-year time span.

Cardiac Catheterization↗
PubMed1985

Classic versus modified Blalock-Taussig shunts in neonates and infants.

Between July 1979 and October 1984, 61 systemic-pulmonary arterial shunts were created in 45 patients. Clinical and angiographic results with 23 classic Blalock-Taussig shunts (BTSs) and 35 modified Blalock-Taussig shunts (MBTSs) with polytetrafluoroethylene grafts between the subclavian and pulmonary arteries were compared. Forty-three of the patients studied were infants: 28 were less than 1 month old, and 19 were less than 1 week old. Weights were 1.1 to 19 kg (mean 2.9 kg). Diagnoses were complex tetralogy of Fallot (18 patients), transposition of the great arteries with small left ventricle or left ventricular outflow tract obstruction (six patients) pulmonary atresia (seven patients) with intact ventricular septum (three) and ventricular septal defect (four), tricuspid atresia (four patients), univentricular heart (six patients), atrioventricular septal defect (canal) with pulmonary stenosis (three patients), and double-outlet right ventricle (one patient). Comparison groups were concurrent and were equivalent for age, weight, and complexity of anomaly. Patients were removed from the study population at the time of subsequent open heart surgery or at death. All patients were followed for a minimum of 6 months and for up to 5 1/2 years (BTS 1 1/2 to 5 1/2 years, mean 33 months; MBTS 6 months to 3 years, mean 20 months). There were no intraoperative deaths in either group. Among the 23 BTSs, three failed at 1, 3, and 19 days, resulting in two deaths (17%). One premature infant died despite a second shunt, one died during attempted intracardiac repair at 13 days of age, and the other was well after revision of the shunt. Eight patients underwent subsequent intracardiac repair 1 to 5 years (mean 34 months) after the initial procedure. Another four patients died from complex intracardiac anomalies with patent shunts. Three patients are alive 2 to 4 years after receiving shunts and have not undergone subsequent surgery. Late postoperative angiograms demonstrate a disturbing incidence (21%) of stenosis and right pulmonary arterial deformity despite satisfactory immediate postoperative studies and good clinical function. Eight patients required a second shunt, two for anatomic discontinuity of the pulmonary arteries and six because of inadequate blood flow through the first shunt. Among the 35 patients receiving the MBTS, two required early revision (technical error and ductal tissue at the anastomosis). There were no shunt-related deaths, but three (6%) occurred within 30 days of operation from left ventricular infarct, cerebral hemorrhage, sepsis, and severe aortic stenosis with arrhythmias.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed1984

Detection of cardiac dysrhythmias by continuous electrocardiographic recording in children undergoing cardiac surgery.

The incidence of dysrhythmias was studied using 24-hour ambulatory electrocardiographic monitoring in 65 consecutive cases of children undergoing cardiac surgery. Recordings were made prior to surgery and in the early (1-10 days) and late (3-12 months) post operative periods. Prior to surgery, 25% of the patients displayed supraventricular dysrhythmias (SVD) and 39% ventricular dysrhythmias (VD). Immediately following surgery a dramatic increase in incidence occurred in both SVD and VD. On late follow-up the frequency of ectopy decreased but not to pre-operative levels. Standard 12-lead ECG was much less sensitive than Holter monitoring, detecting less than 25% of all dysrhythmias. Assessment of preoperative rhythm status is necessary to properly interpret postoperative dysrhythmias. Twenty-four hour ambulatory ECG recording is essential for the detection and evaluation of dysrhythmias in children undergoing cardiac surgery.

Adolescent↗
PubMed1984

Cardiologic perspectives of chest pain in childhood: a referral problem? To whom?

Discussed are the diagnostic and therapeutic issues of cardiac disease in the pediatric age group that have the potential to result in ischemic ventricular dysfunction. The discussion then turns to the nontraumatic thoracic, gastrointestinal, and psychogenic disturbances that are likely to produce symptoms of chest pain. In this context, the question of when to refer, when to reassure, when to begin a diagnostic evaluation, and when to institute longitudinal follow-up are addressed.

Arrhythmias, Cardiac↗
PubMed1984

Echocardiographic evidence of left ventricular bands in infants and children.

M-mode and two-dimensional echocardiographic studies were carried out on 100 consecutive neonates, infants and children to evaluate the incidence of left ventricular chordae or bands. These fibromuscular structures were noted in 61 subjects and were seen to traverse both the long and short axes of the left ventricle. Although a single left ventricular band was usually noted, several subjects had multiple bands. These bands were seen in normal subjects and in patients with a wide variety of congenital cardiac lesions. Distortion of left ventricular geometry was noted in one subject. Identification of these linear echoes is essential to allow differentiation of left ventricular bands from true anatomic left ventricular outflow tract obstruction or septal thickening.

Adolescent↗
PubMed1984

Hematologic disorders and congenital cardiovascular malformations: converging lines of research.

In a population-based study on congenital cardiovascular malformations (CCVM), the occurrence of heritable coagulopathies among case parents and not among controls raised the possibility of an etiologic association of CCVM with blood disorders. The literature was searched for evidence that such an association could be biologically plausible. Reported embryologic and clinical data provided confirmatory findings. The heart and blood arise from common angiogenic cells; endothelial cells, the first components of the primitive heart, synthesize coagulation factors; resultant osmotic alterations of embryonic fluids could alter early cardiac morphogenesis. Bleeding diatheses are common in cyanotic and acyanotic patients with CCVM and hemostatic disorders have been reported in some families. CCVM and blood disorders are joint components of several malformation syndromes. The hypothesis of an etiologic relationship between HBD and CCVM needs to be tested in multiple research areas. Future experimental studies should be based on current theories of cardiac morphogenesis to include investigations of embryonic blood in genetic blood disorders. Clinical studies should clarify hematologic alterations in CCVM probands and their families.

Anemia↗
PubMed1984

Subclavian flap repair of coarctation of the aorta in neonates. Realization of growth potential?

The subclavian flap repair for coarctation of the aorta allows potential for growth by utilizing autogenous tissue. Although well documented in young children, its promise in the tiny neonate warrants further evaluation. Since August, 1979, 29 patients, including 24 infants, have undergone subclavian flap repair at the University of Maryland Hospital. Weights ranged from 1.4 to 5 kg (mean 3.2 kg). All patients less than 6 months old had associated intracardiac defects and were in severe congestive failure. Fifteen responded to preoperative prostaglandin infusions. The overall early mortality was 14%; among the neonates it was 21%; and among those operated upon within the first week of life, 33%. There was one intraoperative death among the eight patients who underwent simultaneous pulmonary artery banding. There were no deaths among patients older than 5 days at operation. Four of the five neonates who died had some variant of hypoplastic left heart syndrome, with severe stenosis or atresia of the systemic atrioventricular valve, critical aortic stenosis, or hypoplastic left ventricle. Twenty-two survivors continue to do well up to 3.7 years postoperatively (mean follow-up 26 months). At follow-up all patients are normotensive with brisk lower extremity pulses. Patients now weigh 1.3 to 6.9 (mean 2.3) times their operative weight, and only one patient has a measured arm-to-leg gradient greater than 10 mm Hg (mean gradient 3.7 mm Hg). Seven of the neonates have undergone repeat catheterization, and all had satisfactory growth of the subclavian flap segment of repair and no gradient. Two older patients (3 and 4 years old at operation) have undergone exercise testing 3.7 years after repair, with peak exercise gradients of only 7 and 15 mm Hg. We therefore continue to utilize this technique for the treatment of coarctation even in tiny neonates.

Aortic Coarctation↗