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Biomedical subjects

C S Kleinman

Publications and source records attributed to C S Kleinman.

At least 37 records · Page 2Linked to original sources
PubMed1994

Ectopic atrial tachycardia in utero.

BACKGROUND: The antenatal diagnosis of fetal arrhythmias helps direct medical management, which may include intrauterine therapy. Ectopic atrial tachycardia is an unusual arrhythmia in children, and we know of no previous reports of antenatal diagnosis of this particular arrhythmia. CASE: The diagnosis of fetal ectopic atrial tachycardia was suggested by the monitor tracing during labor and was subsequently confirmed by the postpartum behavior of the arrhythmia and its electrocardiographic characteristics. CONCLUSION: Ectopic atrial tachycardia, although uncommon, should be considered in the differential diagnosis of fetal tachycardias. The fetal monitor tracing may be useful in making this diagnosis antenatally, which may help to direct management both before and after birth.

Adult↗
PubMed1993

Fetal echocardiography and fetal cardiology: indications, diagnosis and management.

Over the last 15 years, the ultrasound examination of the fetal heart has become increasingly sophisticated. Fetal echocardiography is now paramount in making an early and accurate assessment of cardiovascular structure as well as diagnosing and treating fetal arrhythmias. For the fetal sonographer, the increasing challenge of fetal evaluation has been paralleled by advancing technology. Today, a complete fetal echocardiographic study includes the structural and rhythm analysis using a combination of two-dimensional imaging, M-mode scanning, pulsed- and continuous-wave Doppler measurements, and color-flow mapping. When the data obtained from the fetal echocardiogram suggest normal cardiac structure, the reassurance provided to the family at risk is immeasurable. When a prenatal diagnosis of structural or rhythm abnormalities is obtained, the health care team can outline a management strategy to optimize the care and support given to the fetus, mother, and family.

Arrhythmias, Cardiac↗
PubMed1992

Outcome of prenatally detected cardiac malformations.

Reliable, prenatal detection of congenital heart disease has become possible over the past decade with the evolution of fetal echocardiography. We have documented the outcome of 170 cardiac defects diagnosed prenatally since 1984. Of 170 cases, 55 (32%) had major extracardiac malformations and 45 (28%) chromosomal abnormalities (16 had both). Elective termination was chosen in 77 (45%) pregnancies. Of 93 continuing pregnancies 15 were stillborn and 43 died postnatally (48% of these fetuses and infants had extracardiac or chromosomal anomalies, or both). Thirty-five patients survive at 1 to 80 months (mean 36). Aneuploidy or extracardiac defects are present in 20% of survivors. Nonimmune hydrops secondary to cardiac failure was present in 7 continuing pregnancies and none of these patients survived. The prognosis of prenatally diagnosed cardiac lesions is negatively influenced by the presence of cardiac failure, aneuploidy or extracardiac malformations, or a combination of these. Optimal counseling and management requires the identification of these conditions when present.

Abortion, Eugenic↗
PubMed1992

Fetal cardiovascular physiology and therapy.

The ability to diagnose fetal cardiovascular malformations prenatally has been established during the past 14 years. This has naturally led to an increased interest in the possibility of providing timely therapy in the neonatal period and has raised the prospects for prenatal intervention. Before medical, catheter, and/or surgical interventions are performed, however, it is important to understand the normal and abnormal physiology of the fetal cardiovascular system. Significant insights have been gained into human fetal cardiovascular physiology utilizing data previously gleaned from fetal lamb models and correlating anatomic, pulsed Doppler, and color flow Doppler observations that have been made echocardiographically during the second and third trimesters of human fetal cardiac development. Regional blood flow distribution studies in the human have demonstrated a relative right-ventricular volume dominance in the human fetus of a somewhat lesser magnitude than previously described in the lamb. Observations of ventricular filling characteristics suggest a relative paucity of diastolic 'reserve' in the human fetal heart implying a relative sensitivity to acute volume overloading. The human fetal heart, like that of the fetal lamb, also appears to have a relatively modest amount of 'systolic reserve', making the heart particularly susceptible to acute ventricular afterload. Further studies have demonstrated the ability to recognize altered intrauterine shunt flow across the fetal ductus arteriosus and foramen ovale. As predicted in fetal lamb models, alterations in shunt flow accompany major malformation complexes including ventricular hypoplasia. Studies to date suggest that attempts at surgical intervention should be made very cautiously due to the inability to ascertain whether altered shunt flow patterns precede (cause?) or follow the development of structural abnormalities.

Animals↗
PubMed1992

Hemodynamic evaluation before and after closure of fenestrated Fontan. An acute study of changes in oxygen delivery.

BACKGROUND: Acute changes in hemodynamics and oxygen delivery accompanying temporary occlusion of atrial defects in 14 patients after a fenestrated Fontan procedure were evaluated at a median interval of 32 days after surgery to identify candidates for permanent transcatheter closure of their defects. METHODS AND RESULTS: Patients ranged in age from 9 months to 33 years and in weight from 7.9 to 69 kg. Right atrial (RA), left atrial (LA), and aortic pressures, mixed venous (SmvO2) and aortic (SaO2) oxygen saturation, and whole-body oxygen consumption (VO2) were measured, and systemic blood flow (Qs), systemic oxygen transport (SOT), and oxygen extraction were calculated before and after occlusion. SmvO2, VO2, and RA pressures did not change, but SaO2 increased from 84 +/- 6% to 95 +/- 3% (p less than 0.05), and LA pressures fell from 5.1 +/- 3.6 to 3.7 +/- 2.2 mm Hg (p less than 0.05). Qs fell from 2.4 +/- 0.7 to 1.8 +/- 0.41.min-1.m-2 (p less than 0.05), SOT fell from 425 +/- 154 to 366 +/- 112 ml.O2.min-1.m-2 (p less than 0.05), and oxygen extraction increased from 0.40 +/- 0.12 to 0.46 +/- 0.13 (p less than 0.05). Only one patient did not undergo definitive closure of his defect because of a marked decrease in Qs and SOT with a significant rise in RA pressure. CONCLUSIONS: Although delayed closure of an atrial defect in these patients improved morbidity and mortality, the complete separation of the venous and systemic circulations was accomplished at the expense of decreased Qs and oxygen delivery despite the improved level of arterial oxygenation. The resting levels of oxygen extraction are elevated and will be associated with decreased exercise capability. The long-term benefits of closure of these fenestrations are yet to be shown.

Adolescent↗
PubMed1992

Fenestrated Fontan operation with delayed transcatheter closure of atrial septal defect. Improved results in high-risk patients.

Ten patients, each with two or more risk factors for morbidity and death, underwent a fenestrated Fontan procedure in which a 4 to 6 mm circular fenestration was left between the systemic and pulmonary venous chambers. None died; a similar group of high-risk patients without fenestration had a mortality rate of 2 of 8. Patients with fenestration had significantly less drainage from the chest tube, less need for inotropic support, and shorter intensive care and hospital stays than did patients without fenestration. Comparison with a group of low-risk patients undergoing the Fontan operation showed no statistical difference in these postoperative parameters. Fenestrations were closed in all 10 patients at from 9 days to 6 months after operation by means of the transcatheter clamshell occluder device. Two patients had left pulmonary artery balloon angioplasty and three patients had other atrial communications closed with additional clamshell devices. During short-term follow-up periods averaging 18 months, all patients were clinically well; however, one patient with mitral atresia required reoperation for obstruction between the left atrium and the tricuspid valve, not related to the clamshell device. These data indicate that fenestration may be one method of achieving lower morbidity and mortality rates among high-risk patients undergoing the Fontan procedure.

Adolescent↗
PubMed1991

Foramen ovale size in the normal and abnormal human fetal heart: an indicator of transatrial flow physiology.

We used fetal echocardiography to measure the sizes of the foramen ovale and atrial septum in 80 human fetuses from 17.5 to 38 weeks gestation. Forty-six fetuses had normal cardiac anatomy, 19 had left heart obstructive lesions, and 15 had right heart obstructive lesions. Pulsed and color flow Doppler studies were incorporated when available. We found that normal fetuses had a foramen ovale/atrial septum size ratio of 0.33 +/- 0.04 (mean +/- SD). In all (52%) fetuses in which it was available, pulsed Doppler study revealed bidirectional, but predominantly right-to-left, flow through the foramen ovale. Fetuses with left heart obstructive lesions had a foramen ovale/atrial septum size ratio smaller than normal (0.28 +/- 0.05; p < 0.001). Seven of nine fetuses with left heart obstructive lesions who had color Doppler studies demonstrated reversal of the normal flow pattern, exhibiting unidirectional left-to-right transforamenal flow. Those with right heart obstructive lesions had a larger than normal foramen ovale/atrial septum size ratio (0.47 +/- 0.04; p < 0.001). Nine fetuses with right heart obstructive lesions had color Doppler studies which demonstrated almost exclusive right-to-left transforamenal flow. We conclude that foramen ovale/atrial septum size ratio and Doppler interrogation of transatrial flow are helpful adjuncts in determining the presence of congenital heart disease in utero. In addition, early detection of abnormal foramen ovale/atrial septum size ratio may predict whether left or right ventricular development will be impaired throughout gestation, even before gross disparity of ventricular size is apparent.

Journal Article↗
PubMed1991

Hypoplastic left heart syndrome: prenatal diagnosis, clinical profile, and management.

We diagnosed hypoplastic left heart syndrome, generally regarded as a lethal congenital heart defect, by fetal echocardiography in 20 pregnancies in the last 5 years. The clinical profile, management, and outcome of these pregnancies were reviewed. We found a 40% association of karyotype and extracardiac malformations. Elective abortion was performed in nine pregnancies. Two of seven live-born babies had early neonatal assessment and intervention as a result of in utero diagnosis and counseling. Prolonged survival was achieved in both infants. We conclude that prenatal diagnosis of the hypoplastic left heart syndrome necessitates complete evaluation of the fetus for associated genetic and extracardiac malformations. Prenatal diagnosis of this defect provides opportunities for in depth counseling of parents and obtaining informed consent for either postnatal intervention or nonintervention before the medical and emotional complexities associated with the neonatal intensive care setting are encountered.

Chromosome Aberrations↗
PubMed1991

Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome.

The echocardiographic studies and clinical course of 27 fetuses (mean gestational age 26.9 weeks) diagnosed in utero with tricuspid valve disease and significant tricuspid regurgitation were reviewed. The diagnosis of Ebstein's anomaly was made in 17 of the fetuses, 7 had tricuspid valve dysplasia with poorly developed but normally attached leaflets and 2 had an unguarded tricuspid valve orifice with little or no identifiable tricuspid tissue. One fetus was excluded from data analysis because a more complex heart lesion was documented at autopsy. All fetuses had massive right atrial dilation and most who were serially studied had progressive right-sided cardiomegaly. Hydrops fetalis was found in six cases and atrial flutter in five. Associated cardiac lesions included pulmonary stenosis in five cases and pulmonary atresia in six. Four fetuses with normal forward pulmonary artery flow at the initial examination were found at subsequent study to have retrograde pulmonary artery and ductal flow in association with the development of pulmonary stenosis (n = 1) and pulmonary atresia (n = 3). On review of the clinical course of the 23 fetuses (excluding 3 with elective abortion), 48% of the fetuses died in utero and 35% who were liveborn died despite vigorous medical and, when necessary, surgical management, many of whom had severe congestive heart failure. Of the four infants who survived the neonatal period, three had a benign neonatal course, all of whom were diagnosed with mild to moderate Ebstein's anomaly; only one had pulmonary outflow obstruction. An additional finding at autopsy was significant lung hypoplasia documented in 10 of 19 autopsy reports.(ABSTRACT TRUNCATED AT 250 WORDS)

Ebstein Anomaly↗
PubMed1991

Cardiac growth in fetuses of diabetic mothers with good metabolic control.

To evaluate cardiac growth in fetuses of those diabetic mothers with good metabolic control, we examined M-mode echocardiographic measurements obtained from 24 fetuses of diabetic mothers (FODM) and compared these with measurements from 31 normal fetuses of similar gestational age. Fetuses were grouped into three gestational periods: 20 to 26 weeks, 27 to 33 weeks, and 34 to 40 weeks. The mothers were believed to have good metabolic control on the basis of mean daily glucose profiles and glycosylated hemoglobin A (HbA1C) values of approximately 110 mg/dl (610 mumol/L) and 7.5%, respectively, before fetal scanning, and estimated fetal weight similar to that of normal fetuses during all three gestational periods. Both FODM and normal fetuses had significant increases in M-mode measurements from period 1 to period 3, but in FODM, cardiac hypertrophy developed by late gestation (period 3). This involved the interventricular septum (6.1 +/- 0.7 vs 4.9 +/- 0.3 mm, p less than 0.05), right ventricular free wall (5.7 +/- 0.8 vs 3.2 +/- 0.3 mm, p less than 0.01), and left ventricular free wall (6.4 +/- 0.6 vs 3.3 +/- 0.4 mm p less than 0.01). The interventricular septum/right ventricular free wall ratio was similar, whereas the interventricular septum/left ventricular free wall ratio in FODM was smaller by period 3 (1.0 +/- 0.1 vs 1.6 +/- 0.1, p less than 0.05). The right ventricular diastolic dimension was similar, but the left ventricular diastolic dimension was significantly smaller in FODM during periods 2 and 3 (8.2 +/- 1.2 vs 12.2 +/- 0.7 mm, p less than 0.05). Strict metabolic control did not prevent FODM from having abnormal cardiac growth. We conclude that good metabolic control results in normal estimated fetal weight but that FODM remain at risk for mild global cardiac hypertrophy and altered diastolic dimensions.

Blood Glucose↗
PubMed1991

Perinatal outcome of fetal complete atrioventricular block: a multicenter experience.

The clinical course and outcome of 55 fetuses with complete atrioventricular (AV) block detected prenatally were studied to identify factors that affect the natural history of this lesion. In 29 fetuses (53%) complete AV block was associated with complex structural heart defects, usually left atrial isomerism (n = 17) or discordant AV connection (n = 7). The other 26 fetuses had normal cardiac anatomy; in 19 cases the mother had connective tissue disease or tested positive for antinuclear antibodies. Six fetuses showed progression from sinus rhythm or second degree block to complete AV block. Of the 55 pregnancies, 5 were terminated and 24 fetuses or neonates died; at the end of the neonatal period 26 fetuses were still alive. Fetal or neonatal death correlated significantly with the presence of structural heart defects (4 of 29 surviving, p less than 0.001), hydrops (0 of 22 surviving, p less than 0.001), an atrial rate less than or equal to 120 beats/min (1 of 12 surviving, p less than 0.005) or a ventricular rate less than or equal to 55 beats/min (3 of 21 surviving, p less than 0.001). Mean atrial and ventricular rates were higher in surviving than in nonsurviving fetuses (142 +/- 8 vs. 127 +/- 21 beats/min, p less than 0.002; 64 +/- 8 vs. 52 +/- 8 beats/min, p less than 0.001, respectively). A slow atrial rate, however, was frequently associated with left atrial isomerism.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Pacing, Artificial↗
PubMed1991

Congenital heart disease and fetal thoracoabdominal anomalies: associations in utero and the importance of cytogenetic analysis.

We examined the frequency with which congenital heart disease (CHD) and cytogenetic abnormalities were found associated with omphalocele, gastroschisis, duodenal atresia and posterior diaphragmatic hernias. We performed fetal echocardiograms on 80 patients with these diagnoses and found congenital heart disease in 13 of 37 with omphalocele (35%), 2 of 17 with gastroschisis (12%), 4 of 15 with duodenal atresia (27%), and 2 of 11 with posterior diaphragmatic hernia (18%). Karyotypes were obtained in 74 and were abnormal in 24 (32%). Although most fetuses with these extracardiac malformations and abnormal karyotypes had associated CHD, many did not. Normal karyotypes were found in 69% of fetuses with CHD and omphalocele, and 50% of fetuses with CHD and duodenal atresia. We conclude that CHD may be present in fetuses with extracardiac malformations whether or not the karyotype is normal and that the prenatal evaluation of fetuses with these lesions should include both karyotype and fetal echocardiography. Although karyotypes play an important role in prenatal diagnosis, they are not predictive of normal cardiac structure when normal in the abnormalities studied. Even when the karyotype is normal in the presence of these abnormalities, fetal echocardiography is indicated.

Abdominal Muscles↗
PubMed1991

Sudden death in children receiving Norpramin: a review of three reported cases and commentary.

The 1990 "package insert" for Norpramin, the Merrell Dow Pharmaceuticals Inc., brand of desipramine, was changed to include the following statement in the Adverse Reactions section, "There has been a report of an 'acute collapse' and 'sudden death' in an eight-year-old (18 kg) male, treated for two years for hyperactivity. There have been additional reports of sudden death in children." The purpose of this commentary is to review what is known about the three reported cases of sudden death and to discuss the implications of these tragedies for children receiving treatment with tricyclic drugs.

Attention Deficit Disorder with Hyperactivity↗