儿童扩张型心肌病心脏无创性检查及其在鉴别诊断中的价值

目的　明确心脏无创性检查在儿童扩张型心肌病 (DCM )的变化及其在DCM与肥厚型心肌病 (HCM )、心内膜弹力纤维增生症 (EFE)鉴别中的价值。方法　对 14 6例DCM、4 1例HCM、5 9例EFE患儿 ,用心电图(EKG)、多普勒超声心动图 (ECHO)、彩色多普勒组织显像 (DTI)检查 ,并与 6 117名正常儿童EKG、2 86名正常儿童多普勒ECHO、14 3名正常儿童DTI检查对比。结果　EKG左室面电压增高者HCM、EFE显著多于DCM。多普勒ECHO检查 :DCM患儿心腔增大 ,左心室后壁 (LVPW )、室间隔 (IVS)不增厚 ;HCM患儿心腔不增大 ,LVPW、IVS增厚 ;EFE患儿心腔扩大 ,LVPW、IVS增厚。多普勒ECHO检查 :DCM和EFE患儿心脏收缩功能降低大于HCM患儿 ;DCM、HCM、EFE患儿心脏舒张功能下降差异无显著性。DTI检查 :HCM、DCM、EFE三者差异无显著性 ,DCM患儿心脏舒张功能降低重于收缩功能降低。DCM患儿MVRDeV <4cm/s者 ,预后极差。结论　EKG、多普勒ECHO、DTI对DCM的诊断和DCM与HCM、EFE的鉴别有重要价值。DTI对估计DCM预后有重要价值。     

Antenatal steroids are associated with a reduction in the incidence of cerebral white matter lesions in very low birthweight infants

AIMS: To investigate whether antenatal steroids reduce the incidence of cerebral white matter lesions in very low birthweight infants. METHODS: A total of 224 newborn infants of < 31 weeks gestational age and weighing < 1500 g was studied between January 1998 and June 2000. Obstetric and neonatal information was obtained from the case notes. The study population was subdivided into two groups according to antenatal steroid exposure. A complete course of treatment consisted of two doses of 12 mg each of betamethasone given at an interval of 12-24 hours. Infants in group 1 were born to mothers who had not received betamethasone, or were delivered within 24 hours of receiving the first dose of steroid. Infants in group 2 were born to mothers who had received one or more complete courses of betamethasone and were delivered > 24 hours after receiving the first dose of steroid. RESULTS: The two groups contained statistically similar proportions of boys and girls, and the infants had similar birth weights and survival rates. Those in group 2, compared with those in group 1, had a lower gestational age (p = 0.02) and a lower incidence of white matter lesions on cranial ultrasound scans (p = 0.03). Stepwise logistic regression analysis showed that gestational age (p = 0.0002) and a complete course of antenatal steroids (p = 0.02) had independent effects on cerebral white matter lesions. CONCLUSIONS: These observations suggest that a complete course of antenatal steroids may have a protective effect against cerebral white matter lesions in very low birthweight infants.     

Exposure to repeat doses of antenatal glucocorticoids is associated with altered cardiovascular status after birth

OBJECTIVE: To determine if exposure to more than one course of antenatal glucocorticoids is associated with changes in infant blood pressure and myocardial wall thickness in the first month after birth. DESIGN: Prospective cohort study. SETTING: Tertiary neonatal intensive care unit. PARTICIPANTS: Mothers who were eligible for but declined to enter a randomised trial of repeated doses of antenatal glucocorticoids (ACTORDS)-that is, who had a singleton, twin, or triplet pregnancy at <32 weeks gestation, had received an initial course of glucocorticoids seven or more days previously, and were considered to be at continued risk of preterm birth. MAIN OUTCOME MEASURES: Blood pressure daily for the first week then weekly until 4 weeks of age. End diastolic interventricular septal and left ventricular posterior wall (EDIVS and EDLVPW) thickness at 48-72 hours after birth. RESULTS: Thirty seven women were enrolled and delivered 50 infants. Thirty mothers (39 infants) were exposed to one course of glucocorticoids, and seven mothers (11 infants) to more than one course. Blood pressures were higher in the first week after birth in infants exposed to multiple courses of glucocorticoids, and in infants with a latency between last exposure and delivery of less than seven days. Systolic blood pressure on day 1 was >2SD above published normal ranges in 67% of babies exposed to multiple courses and 24% of babies exposed to a single course of glucocorticoids (p = 0.04). There was no difference between groups in thickness of the EDIVS or EDLVPW. However, 44/50 (88%) babies had EDIVS and 49/50 (98%) babies had EDLVPW thickness >2 SD above the expected mean for birth weight and gestation. EDIVS but not EDLVPW thickness increased with increasing latency (mean 0.02 mm/day, p = 0.03). CONCLUSION: Future randomised trials should assess the long term effects of exposure to antenatal glucocorticoids, particularly multiple courses, on the cardiovascular status of the infant.     

Echocardiographic profile of infants of diabetic mothers

Echocardiographic M-mode examinations were performed in 30 infants of diabetic mothers (IDM). Twelve of them had severe symptoms with hyperbilirubinemia, respiratory distress syndrome, septicemia and hypoglycemia in the neonatal period. The gestational age ranged from the 34th week to the 40th week, the birth weight ranging between 2280 g and 5820 g (mean 3455 g). Fifteen patients were restudied at different ages. In group A (age 1-14 days) out of 23, in group B (age 14 days-6 months) 5 from 13 IDM and in group C (older than 6 months, (n=10) no IDM had an increased ratio of intraventricular septal thickness (IVS) to left ventricular posterior wall thickness (LVPW). One patient showed a systolic anterior motion of the mitral valve (SAM) without having other symptoms for a left ventricular outflow tract obstruction. All measured dimensions normalized within 6 months. It is concluded that a form of prematurity may be responsible for the transient, asymmetrical, septal hypertrophy.     

Antenatal glucocorticoids increase early total thyroxine levels in premature infants

BACKGROUND: Hypothyroxinemia is associated with adverse neonatal outcomes including white matter damage, cerebral palsy, poor neurodevelopment and death. It has become increasingly important to understand the natural history and modifiers of thyroid function in the premature infant. It is standard obstetrical practice to offer antenatal glucocorticoids to pregnant women with threatened preterm delivery. Few studies have investigated the effect of antenatal glucocorticoids on neonatal thyroid function. OBJECTIVE: To examine the association between antenatal exposure to glucocorticoids and early total thyroxine (T4) levels among extremely premature infants. METHODS: We studied 521 infants born at 4 medical centers. Entry criteria included a gestational age of 23-28 weeks and a serum thyroxine level obtained in the first postnatal week. Receipt of antenatal glucocorticoids was recorded as none, partial, or complete. A complete course consisted of two doses of betamethasone or four doses of dexamethasone within a 48-hour period between 2 and 7 days of delivery. Early total T4 levels were obtained from state-mandated newborn screening programs. RESULTS: Controlling for potential maternal, perinatal and neonatal confounding variables, infants exposed to a complete course of antenatal glucocorticoids had total T4 levels 0.8 microg/dl higher than their peers who were not exposed to a complete course of antenatal glucocorticoids (p = 0.03). CONCLUSIONS: Extremely premature infants who received a complete course of antenatal glucocorticoids had significantly higher total thyroxine levels in the first postnatal week. Maternal, perinatal, and early neonatal variables did not completely explain this association. We speculate that antenatal glucocorticoids influence early neonatal thyroid function.     

Echocardiographic studies in children with chronic kidney insufficiency and intermittent hemodialysis treatment

Cardiac function was measured in 10 children (mean age 12 years) undergoing chronic intermittent hemodialysis. Blood pressure, heart rate and body weight were checked three times before and after hemodialysis. Echocardiographic studies were performed according to the references of the American society of echocardiography. 6 patients were normotensive, 4 patients hypertensive. Cardiac function was normal before and after hemodialysis in all patients. Ultrafiltration reduced body weight on average by 1.2 kg. The mean systolic blood pressure remained unchanged, the mean diastolic blood pressure declined in normotensive patients from 66 mm Hg to 53 mm Hg and in hypertensive patients from 100 to 85 mm Hg on average; the systolic leftventricular diameter (LVES) was reduced by dialysis from 2.7 to 2.3 cm, the enddiastolic leftventricular diameter (LVED) from 4.42 to 4.02. Cardiac output declined from 62.04 ml to 56.68 ml/min, whereas heart rate, shortening fraction and mean circumferential fiber-shortening-time increased. In normotensive patients LVED shortened more (4.4 to 3.86 cm) than in hypertensive children (4.44 to cardiac output 4.18 cm), but there were no significant differences. Significantly decreased only in the normotensive children. In hypertensive children the shortening fraction increased significantly (35.54 to 42.48%) in contrast to normotensive children (40.84 to 38.44%). Cardiac preload (LVES) and (LVED) and afterload (diastolic pressure) was reduced significantly after volume elimination. These changes in cardiac function suggest, that cardiac alterations found before hemodialysis are only functional and not structural.     

The effect of antenatal betamethasone on cord blood concentrations of retinol-binding protein, transthyretin, transferrin, retinol, and vitamin E

We assessed the effect of short-term (less than or equal to 1 week) and prolonged (greater than 1 week) exposure to antenatal betamethasone on umbilical cord serum concentrations of retinol-binding protein (serum t 1/2 = 12 h), transthyretin (t 1/2 = 2 days), transferrin (t 1/2 = 8 days), retinol (vitamin A), and vitamin E in appropriate-for-gestational-age preterm newborn infants of less than 36 weeks' gestation. A group of 30 infants whose mothers received a single course of betamethasone less than or equal to 1 week prior to delivery had significantly elevated mean retinol-binding protein and transthyretin but not transferrin concentrations when compared with a group of 30 gestational age- and birth weight-matched infants with no exposure to antenatal betamethasone. A group of eight infants whose mothers received multiple (more than two) weekly courses of betamethasone prior to delivery had significantly elevated mean serum concentrations of all three proteins when compared with eight gestational age- and weight-matched control infants with no betamethasone exposure. Serum retinol and vitamin E concentrations were measured in a group of 21 infants exposed to short-term prenatal betamethasone and were significantly greater than in a group of 21 control infants without steroid exposure. We conclude that antenatal steroids increase the umbilical cord serum concentrations of retinol-binding protein, transthyretin, transferrin, retinol, and vitamin E. The effect on the various serum proteins is dependent on the duration of exposure to steroids.     

Blood glucose level in preterm infants after antenatal exposure to glucocorticoid

AIM: To determine the impact of antenatal glucocorticoid on neonatal glucose homeostasis. METHODS: This is a retrospective gestationally paired survey followed by a randomized study. On the basis of the interval between last antenatal dexamethasone and birth, 228 preterm infants born before 34 weeks were divided into Short (< 24 h), Intermediate (1-6 days), and Long (> or = 7 days) exposure groups and compared their gestationally paired controls. After a single course of betamethasone, the parturients remaining undelivered for one week were randomized to receive either one dose of betamethasone (n = 52) or placebo (n = 53). Glucose values were recorded at 11 time points in the first 3 days of life. Hypoglycaemic and hyperglycaemic values were counted. RESULTS: There were no overall differences in mean glucose levels between the antenatal glucocorticoid and the control groups. However, the long exposure time to antenatal glucocorticoid was associated with increased risk of hyperglycaemia (OR 4.1; 2.2-7.6). CONCLUSION: Antenatal glucocorticoid administration was associated with subtle disturbances of glucose homeostasis in preterm infants. These differences were dependent on the length of drug-delivery interval so that long exposition time seemed to increase the incidence of hyperglycaemia during the first days of life.     

Echocardiographic characteristics of premature infants with patient ductus arteriosus

As part of a multicenter collaborative study, M-mode echocardiograms were obtained shortly after birth on 3,559 premature infants with birth weight less than or equal to 1,750 g. Of these infants, 1,496 did not develop a cardiac murmur or other signs of a "hemodynamically significant" patent ductus arteriosus (PDA). Echocardiographic parameters from this "normal" group were compared with results obtained from 415 infants in whom PDA was diagnosed on the basis of clinical findings alone, irrespective of the echocardiogram (PDA group). The best discrimination between the two groups, when considering a single parameter, was provided by a left atrial to aortic root ratio (LA/AO) of 1.40, a left ventricular to aortic root ratio (LV/AO) of 2.10, or a left ventricular systolic time interval ratio (LPEP/LVET) of 0.27. Multivariate analysis demonstrated that better separation between the two groups occurred when the left atrial to aortic root ratio and the left ventricular systolic time interval ratio were considered jointly. Because of a large degree of overlap of all echocardiographic variables between the normal group and the group with PDA, the echocardiogram alone was not a good indicator of PDA. However, when used in conjunction with a priori estimates of the probability of PDA (based, for example, on birth weight and degree of respiratory disease), use of echocardiographic data was found to improve the detection of hemodynamically significant patent ductus arteriosus in premature infants.     

Impaired left ventricular diastolic function in newborn infants of mothers with pregestational or gestational diabetes with good glycemic control

THE AIM OF THE STUDY: We assessed by echocardiography the left ventricular systolic and diastolic function in newborn infants of mothers with well-controlled pregestational type 1 or gestational diabetes (IDM) in comparison to normal term neonates. SUBJECTS AND METHODS: Two-dimensional/M-mode and Doppler transmitral flow velocity measurements were performed in 18 IDM and 26 control infants of non-diabetic mothers (gestational ages 36-40 and 36-41 weeks, respectively) between days 2 and 5 after birth. In the IDM, there were nine mothers with pregestational (White class C or D) and nine mothers with gestational diabetes (White class A or A/B). Peak early and atrial filling velocity, early deceleration time, early acceleration time, early, atrial and total time velocity integrals were used to examine the left ventricular diastolic performance. We also calculated the early/atrial velocity ratio, early/atrial integral ratio and early/total integral ratio. The fractional shortening, fractional shortening area, midwall fractional shortening (mFS), left ventricular mass and indexed left ventricular mass for body surface area (BSA) and birth weight were used in assessment of left ventricular systolic performance. RESULTS: The early deceleration time was longer, resulting in higher early integral and early filling fraction (EFF) in the IDM than in the control infants (p<0.01). In the IDM, the fractional shortening was somewhat greater and the left ventricular mass/body surface area ratio was higher than in the control group (p<0.05), although the measures of systolic performance were within the normal range. There were no significant differences in the systolic or diastolic function parameters between the gestational and pregestational groups. CONCLUSION: In the infants of mothers with well-controlled pregestational or gestational diabetes, we found prolonged deceleration time of early left ventricular diastolic filling, probably reflecting an impaired left ventricular relaxation rather than compliance. The mechanism for the findings may be maternal hyperglycemia during the third trimester and subsequent fetal hyperinsulinaemia leading to neonatal cardiac hypertrophy.     

Cardiovascular Manifestations in Fabry's Disease — Age-Related Changes in Hemizygotes and Heterozygotes —

Fabry's disease is an X-linked recessive disorder of glycosphingolipid metabolism. Accumulation of neutral sphingolipid in vascular endothelium is known to cause various clinical manifestations, including cardiac problems. Female heterozygotes, however, may be asymptomatic or mildly affected. Cardiovascular manifestations in Fabry's disease were studied noninvasively in 10 male hemizygotes and 10 female heterozygotes from seven families. Mitral valve prolapse was found in both hemizygotes and heterozygotes. Measured echocardiographic variables were normalized by dividing them by normal values calculated from basal surface area (BSA) and age. Normalized thickness of the interventricular septum [IVS(n)] and left ventricular posterior wall thickness [LVPW(n)] increased significantly with age both in hemi- and heterozygotes. [IVS(n): Y=0.94+0.014X (age in years), r=0.68, LVPW(n): Y= 1.03+0.015X (age in years), r=0.64]. The incidence of ST-T changes in the ECG was also observed in hemi- and heterozygotes. A short PR interval was recorded in one hemizygote and one heterozygote from the same family. Thus, heterozygotes appear not to be free from cardiac manifestations, as previously believed. It is possible that the cardiac changes in heterozygotes occur regardless of the amount of sphingolipid accumulation.     

Anomalous origin of the left coronary artery from the pulmonary artery. Variability of clinical aspects, echocardiography and angiography findings]

OBJECTIVES: Analysis of symptoms, diagnostical difficulties and follow-up in infants and children with anomalous origin of the left coronary artery from the pulmonary artery. METHODS: Retrospective study; 12 children between 3 weeks and 2 years old; time period: 1980-1991. RESULTS: Three infants were detected on routine examination because of a new cardiac murmur, the others presented with signs of cardiac failure. Cross sectional echocardiography and color Doppler flow mapping allowed to verify the suspected diagnosis. However, in one infant a false-negative cross sectional echocardiographic result was obtained. In this case nuclear magnetic resonance imaging was able to delineate the exact anatomy. Mean preoperative left ventricular ejection fraction: 33 +/- 4%; percentage of infants below the age of 6 months: 92%; surgery related mortality: 66%; mean follow-up of the remaining 4 patients being in good clinical condition: 2.9 +/- 1 years. CONCLUSIONS: 1. An anomalous origin of the left coronary artery should be included into the differential diagnosis when a new cardiac murmur is detected. 2. Possibility of false-negative echocardiographic results is emphasized. 3. With early symptoms and highly reduced left ventricular function, the mortality is still high.     

扩张型心肌病的心功能变化:多普勒组织显像的敏感性与可靠性

探讨扩张型心肌病（DCM）的心功能变化,比较多普勒组织显像（DTI）与传统心功能指标的敏感性及可靠性。方法对25例DCM患儿分别测量其心腔大小、EPSS（Epoint－septalseparation）、左室心肌重量指数（LVMI）、二大瓣血流E峰及A峰、左室射血分数（LVEF）、每搏指数（SI）及DTI测量其二尖瓣环收缩期及舒张早期运动速度（MVR－SV、MVR－DeV）,在室后壁心内膜收缩期及舒张期运动速度（LVPW－EnSV、LVPW－EnDV）,并设正常对照组143名。结果DCM患儿心脏收缩及舒张功能均显著下降,心肌运动速度显著降低．MVR－DeV与LVMI、EPSS是显著负相关（r=-0．84,-0．83P＜0．01）,与LVEF、SI是显著正相关（r=0．844,0．77P＜0．01）,同时其敏感性显著高于LVEF及SI（P＜0．05）;DTI测量心肌的舒张功能其敏感性、可靠性均高于二失瓣血流E峰与A峰,而E／A敏感性最差,且多数DCM呈假性正常,MVR－DeV与E峰、A峰及E／A均无显著直线相关性（P均＞0．05）,与病情一致。结论DCM的收缩及舒张功能均显著下降,DTI指标较传统心功能指标更敏感而可靠。     

Serial evaluation for myocardial performance in fetuses and neonates using a new Doppler index

BACKGROUND: Fetal echocardiography has been used for non-invasive evaluation of human fetal cardiac anatomy, function and hemodynamics. The Tei index, a new Doppler index known to be independent of both ventricular geometry and heart rate, has recently been applied to the evaluation of myocardial performance. METHODS: In the present study, the Tei index was prospectively and longitudinally determined in 50 normal fetuses, 35 fetuses with intrauterine growth retardation (IUGR), 30 fetuses of diabetic mothers (DM) and 20 normal infants. The Tei index of both left and right ventricles was calculated from a Doppler ventricular inflow and outflow trace using the following formula: Tei index = (ICT + IRT)/ET, where ICT is isovolumetric contraction time; IRT, isovolumetric relaxation time; and ET, ejection time). RESULTS: The Tei index of the left ventricle decreased linearly with advancing gestational age during 18-33 weeks and decreased acceleratively with increasing gestational age after 34 weeks. The index of the right ventricle decreased slightly and linearly with advancing gestational age during 18-41 weeks. In neonates, the Tci index of the left and right ventricle increased immediately and transitorily after birth and decreased and stabilized after 24 h of life. From 18 to 26 weeks of gestation, the Tei indices in fetuses with IUGR and of DM were not significantly different from controls. However, from 27 to 40 weeks of gestation, the Tei indices in both fetuses with IUGR and of DM were significantly greater than controls. CONCLUSIONS: This gradual decrease in the Tei index during gestation may represent the maturational or developmental alternation of myocardial performance in utero. Fetuses with IUGR and of DM may have abnormal myocardial performance in later gestation.     

Effect of single and multiple courses of prenatal corticosteroids on 17-hydroxyprogesterone levels: implication for neonatal screening of congenital adrenal hyperplasia

Measurement of 17-hydroxyprogesterone (17-OHP) from filter-paper blood is widely used to screen for congenital adrenal hyperplasia (CAH). However, in pregnancies with an expected preterm delivery, prenatal treatment with steroids to induce pulmonary maturation could suppress the fetal adrenals and interfere with this screening. In 160 infants who were born between 25 and 35 wk of gestation, we measured 17-OHP in filter-paper blood at 72-96 h and compared the values between those who had not received antenatal steroids (n=50) and those who had (n=110). A single course of steroids was two 12-mg injections of betamethasone given within a 24-h interval: 30 infants received a half single course, 45 received a full single course, and 35 received multiple courses. Results are expressed as medians (25th percentile; 75th percentile). Blood 17-OHP differed significantly among groups: 23.7 (14.2; 30.7) nmol/L, 26.1 (15.0; 50.1) nmol/L, 20.1 (13.8; 29.1) nmol/L, and 14.9 (9.5; 26.2) nmol/L (for, respectively, no steroid, half a single course, a full single course, and multiple courses; p <0.05, multiple comparisons with the Kruskal-Wallis test). However, only infants who were treated with multiple antenatal courses of steroids had lower blood 17-OHP than those who were untreated (p <0.05 with the Mann-Whitney U test). In multiple regression analysis, steroid treatment and intrauterine growth retardation were significant negative predictors of blood 17-OHP, whereas respiratory distress syndrome was a significant positive predictor (multiple R=0.50, p <0.001). Multiple courses of steroids in preterm infants decrease 17-OHP values by approximately 30% in filter-paper blood, thus raising the risk of false-negative results in screening programs for CAH.     

Cardiac troponin I levels and its relation to echocardiographic findings in infants of diabetic mothers

ABSTRACT: BACKGROUND: Cardiomyopathy is noted in up to 40% of infants of diabetic mothers, and the exact mechanisms responsible for it are unknown. The aim of this study was to compare between infants of diabetic mothers (IDM) and infants of non- diabetic mothers (INDM) as regards cardiac troponin I (cTnI) levels (as a marker of cardiac dysfunction) and to examine the relationship between this marker and neonatal echocardiographic changes (cardiac structure and function). METHODS: A prospective, comparative study included eighty full term neonates during the first three days of life, during the period from April 2008 to June 2011. They were divided into 2 groups, group I: included 40 infants of diabetic mothers (IDMs)and group II: included 40 infants of non diabetic healthy mothers as a control group. RESULTS: 37.5% of the IDMs were large for gestational age and demonstrated a significantly lower blood glucose level than the control group (34.6 +/- 11.3mg/dl Vs 77.2 +/- 19.8 mg/dl respectively) , respiratory distress and cyanosis were the presenting signs in 67.5% of IDMs. Cardiac TnI on the second day of life increased significantly in infants of diabetic mothers in comparison to INDM (p < 0.006) . IDMs had a significant increase in left atrial thickness ( 11.5 +/- 1.8mm in IDM Vs 10.5 +/- 0.9mm in INDM P < 0.002*) and a significant increase in septal thickness (5.0 +/- 1.2mm in IDM Vs 4.0 +/- 0.5mm in INDM P < 0.001*). CTnI correlated positively with interventricular septum thickness (P-value = 0.002*). Cardiac TnI was significantly increased in IDMs with respiratory distress (P -value < 0.05). CONCLUSIONS: This study demonstrated a highly significant positive correlation between cTnI level on the second day of life and the left ventricular end diastolic diameter (LVED) and interventricular septum diameter (IVSD). Cardiac troponin I (cTnI) is a useful biochemical marker for monitoring myocardial injury in infants of diabetic mothers. An elevated cTnI concentration in infants of diabetic mothers with respiratory distress was a good predictor for hypertrophic cardiomyopathy and/or left ventricular dysfunction.     

The antenatal use of betamethasone in the prevention of respiratory distress syndrome: a controlled double-blind study.

One hundred forty-six pregnant women were enrolled in a prospective double-blind study to assess the effectiveness and side-effects of antenatal administration of betamethasone in the prevention of respiratory distress syndrome (RDS) in potentially premature infants. On admission to the study, the women were given, at random, either 12 mg of betamethasone or placebo. The same dose was repeated 24 hours later and then weekly up to 34 weeks of gestation. Gestational age of the infants ranged from 25 to 34 weeks, and birth weights ranged between 730 and 2,650 gm. Statistically significant differences in favor of the infants in the betamethasone group were found in the incidence of RDS, 20.7% in the betamethasone group compared with 59.5% in the control group (P less than .005); in the severity of RDS (P less than .05); and in the death rate (P less than .05). A higher incidence of hypoglycemia was found among infants in the betamethasone group (P less than .05). Prolonged rupture of the membranes played no protective role against RDS, and the incidence of infection was similar in both groups.     

实时三维超声心动图对先天性心室肌致密化不全的诊断价值

目的 应用实时三维超声心动图（RT3DE）诊断儿童先天性心室肌致密化不全（NCVM），初步评价其应用价值。方法 2006年7至12月应用二维超声心动图诊断为NCVM的患儿4例，其中女性3例，男性1例，年龄2~11个月。采用RT3DE观察心脏肌小梁和隐窝的形态，计算左室射血分数，根据时间 容量曲线参数对左室同步性进行评价。结果 ①RT3DE对NCVM的形态观察：1例累及整个左室，3例累及左室中段和心尖部。②RT3DE对NCVM的量化诊断：收缩末期最大肌小梁顶部至心外膜表面距离与隐窝底部至心外膜表面距离的比值（X′s/Y′s）为3.3~5.0；舒张末期最大肌小梁顶部至心外膜表面距离与隐窝底部至心外膜表面距离的比值（X′d/Y′d）为2.6~4.4。③RT3DE对NCVM的心功能评价：左室射血分数为21.2%～56.4%。④RT3DE对NCVM的左室同步性评价：所有16个心肌节段到达每一个节段收缩期最小容量时间的标准差在心动周期中所占的比例为2.65%～19.30%，最大差值在心动周期中所占比例为8.75%～78.20%。结论 RT3DE不仅能够观察NCVM累及的范围，而且可以定量评价其严重程度，测定心功能以及评价同步化运动。     

Primary cardiac tumours in a paediatric population

OBJECTIVE: To review the presentation, diagnosis, histology and outcome of primary cardiac tumours presenting to a paediatric cardiac unit over a 20-year period. METHODS: Hospital records and data bases were searched for the years 1980-2000. RESULTS: There were 12 patients with four histological tumour types including a predominance of rhabdomyoma, as well as myxoma, fibroma and myocardial hamartoma. Diagnosis was made in utero, by ultrasound in five cases and in the neonatal period in a further three cases. Six cases (50%) required surgical intervention and there were three tumour-related deaths. Two infants with large left ventricular tumours diagnosed in utero developed univentricular physiology, acting like hypoplastic left heart syndrome at birth. CONCLUSIONS: Despite an absence of malignant histology there was significant mortality and morbidity among the patients reviewed. The development of univentricular physiology in infants with large left ventricular tumours is rare and is a difficult management problem.     

Two sensitive echocardiographic techniques for detecting doxorubicin toxicity

This investigation was designed to evaluate echoes of patients who received a course of doxorubicin (225-550 mg/m²) by two sensitive indicators of myocardial fibrosis: (1) M-mode scans of thickening-thinning curves of the left ventricular posterior wall (LVPW), and (2) two-dimensional qualitative evaluation of LVPW contraction at three levels of the LV short axis (leaflet, chordal, and papillary). These were compared to standard M-mode shortening fraction Δ. Eighteen children with cancer were evaluated; 11 had received doxorubicin and 7 were treated with other agents. Echocardiographers were unaware of the treatment category. All controls and 10 of 11 doxorubicin patients had normal. An M-mode echocardiogram of the expanded LVPW was digitized and wall thickness was evaluated by determining if diastolic relaxation had the normal two phases or only one; six of ten doxorubicin patients and no controls had abnormal relaxation. Qualitative evaluation of LVPW and septal contraction toward the center of the ventricle showed that seven of eleven patients who received doxorubicin and one control (a postthoracotomy patient) had contraction deficits. Six of seven with contraction deficit were the same patients with slowed relaxation. The greatest contraction deficit occurred in the LVPW behind the posterior mitral leaflet. Patients with more extensive involvement had an additional contraction deficit extending to the apex. These tests are more sensitive for detection of doxorubicin toxicity than Δ.