新生儿先天性心脏病145例分析

目的　了解新生儿先天性心脏病（简称先心病）的临床特点和预后。　方法　对１９９１年～１９９７年新生儿病房所收治的１４５例先心病进行分析,并随访２４例非青紫型先心病患者３个月～７年。　结果　非青紫型先心病９３例（６４．１％ ）,青紫型５２例（３５．９％ ）,最常见者依次为室间隔缺损（４４．１）％ 、动脉导管未闭１３．８％ 、大动脉转位（９．７％ ）。１３．１％ 合并有心外畸形,以染色体畸形居首位３１．６％ 。比较青紫型与非青紫型的临床表现（青紫型％ 、非青紫型％ ）：生后２４小时内出现青紫（４２．０％ 、０％ ）、气促（７０．０％ 、５９．０％ ）、心率快（４０．０％ 、２３．０％ ）、心力衰竭（４８．０％ 、１４．０％ ）、心脏杂音（７６．０％ 、８９．０％ ）、低氧血症（８２．６％ 、９．８％ ）、代酸（２１．７％ 、８．５％ ）、住院时的病死率（３６．５％ 、０％ ）、２例青紫型先心病在新生儿期作了手术,放弃治疗（５１．９％ 、１１．８％ ）。随访非青紫型者２４例,２例死亡、５例手术、１２例自愈、５例未愈无合并症。　结论　先心病是婴幼儿死亡的主要原因。现国外已用胎儿超声心动图作产前诊断并对重症终止妊娠,国内应在产科中广泛开展此项检查     

3个月内婴儿先天性心脏病193例回顾性分析

目的总结3个月内婴儿先天性心脏病的临床特点,提高早期诊断率。方法对193例经彩色多普勒超声心动图诊断为先天性心脏病患儿资料进行回顾性分析,其中新生儿145例,~3个月婴儿48例。结果新生儿期先天性心脏病复合畸形占21.4%（31/145）,较~3个月婴儿8.3%（4/48）明显增多,其先天性心脏病主要类型为房间隔缺损合并室间隔缺损;~3个月婴儿中以单纯畸形为主,占91.7%（44/48）,其先天性心脏病主要类型为室间隔缺损。临床表现多种、无特异性,以青紫、呼吸急促、喂养困难、心脏杂音多见,易反复呼吸道感染。结论 3个月内婴儿先天性心脏病类型多,临床表现复杂,对可疑者应尽早行超声心动图检查,早期确诊及时治疗。     

早期新生儿先天性心脏病的筛查和随访

目的降低先天性心脏病（简称先心病）的发病及减少病死率,探索在基层医院的管理模式.方法2002至2005年对北京市郊区两所妇幼保健院（大兴、门头沟）出生的足月活产儿5640例,在生后7 d内查体筛查先心病,可疑者用彩色多普勒超声心动图进行诊断,出院后定期随访.结果足月活产儿中共检出先心病65例,检出率11.52‰.65例先心病中非青紫型占76.9%,以室间隔缺损（简称室缺）为主（60.1%）,青紫型23.1%,以肺动脉狭窄（9.1%）及法洛四联症（6.2%）为主.随访12例重症先心病,10例家长放弃治疗（其中6例已死亡）,仅2例手术治愈.先心病占同期婴儿死因的首位35.3%（6/17）.室缺37例随访中缺损自行闭合（自闭）、扩大及无明显变化者约各占1/3.缺损直径＜5 mm者23例,12例（52%）自闭,缺损≥5 mm者14例中,自闭1例（7%）,8例（57%）缺损继续扩大（x^2=9.4,P＜0.01）,其中2例死亡,3例已手术矫治.结论防治先心病应自宫内筛查作起,对重症难治的先心病建议及早终止妊娠,经定期随访不能自闭的病例,选择最佳时期手术矫治,降低病死率.     

新生儿先天性鱼鳞病1例报告

<正>先天性鱼鳞病是一组常染色体遗传性皮肤脱屑性疾病,临床表现为皮肤发硬和脱屑。本病病因不明,除遗传因素外,脂质代谢异常、维生素A不足及表皮增生和脱落不平衡也可能是致病原因。多数患儿在婴幼儿时期即开始发病。笔者于2011-10收治新生儿先天性鱼鳞病1例,现报道如下。     

新生儿近端肾小管酸中毒合并先天性心脏病一例

患儿男，生后18h，因皮肤黄染伴反应差4h入院。患儿系第4胎第2产，胎龄38周。因其母合并妊娠高血压综合征行剖宫产，术前曾使用硫酸镁、能量合剂及白蛋白等。术中发现羊水量少，胎盘及脐带均无异常，生后置暖箱中吸氧。生后18h皮肤黄染及心脏杂音、呼吸急促、呻吟、反应差收入院。患儿之母孕32周血压160／120mmHg     

先天性梅毒新生儿15例护理体会

目的总结先天性梅毒新生儿的临床表现及护理,提高护理水平。方法对先天性梅毒新生儿采取合理的、有针对性的护理措施。主要护理措施包括：做好皮肤护理、消毒隔离,防止交叉感染、认真观察病情变化等。结果治愈12例,放弃治疗3例。结论密切观察病情,采取有效的护理措施,对先天性梅毒新生儿的治疗起着重要作用。     

妊娠合并先天性心脏病对新生儿的影响

妊娠合并心脏病是高危妊娠之一，随着风湿性心脏病的减少及心脏外科手术的迅速发展，妊娠合并先天性心脏病已跃居妊娠合并心脏病首位。母亲患不同类型的先天性心脏病对新生儿的影响显著，母亲的心功能状态也与新生儿疾病发生有关。为保证母儿安全，明确先天性心脏病患者的妊娠风险，现对我院收治的妊娠合并先天性心脏病患者45例进行分析。     

双胎妊娠156例新生儿结局分析

双胎妊娠属高危妊娠范畴，早产发生率及同生儿死亡率较单胎妊娠高，而且，分娩期的处胛足影响双胎妊娠新生儿预后的重要环节。降低新生儿死亡率是产科工作者十分关注的问题，现对在我院分娩的156例双胎妊娠资料总结如下。     

新生儿先天性喉囊肿2例并文献复习

先天性喉囊肿是导致新生儿气道阻塞的原因之一，发病率为1.82/10万～3.49/10万，其中50%是在窒息死亡患儿的尸检中发现的。对本病认识不足可导致病情延误，甚至导致新生儿死亡。报告中国人民武装警察部队特色医学中心分娩的2例新生儿先天性喉囊肿病例。新生儿出生后发生原因不明的窒息、低Apgar评分且复苏效果欠佳时，除外产科因素的同时，还应考虑先天性喉囊肿致新生儿呼吸道阻塞的可能，建议行喉镜检查，紧急情况下可采取囊肿穿刺抽液以在最短时间内缩小囊肿体积，缓解呼吸困难的危重情况，为下一步治疗赢得时机。     

妊娠合并先天性心脏病165例临床分析

目的 探讨妊事并先天性心脏病（先心病）对母儿预后的影响和围生期处理原则。方法 对１６５例妊娠合并先心病患者的临床资料进行回顾性分析。结果 妊合并先心病孕产妇死亡２例,围生儿死亡９例,小于胎龄儿（ＳＧＡ）３１例,早产儿２０例,均明显高于同期非心脏病孕产妇死亡数,围生儿死亡数,ＳＧＡ及早产儿,Ｐ均〈０．００５。４６例妊娠前行心脏矫治手术组１例巨大儿,无ＳＧＡ、早产儿、孕产妇及围生儿死亡。结论 多数先心     

Perinatal outcomes of congenital heart disease after emergent neonatal cardiac procedures

Purpose: We compared outcome of neonates with prenatal and post-natal diagnosis of congenital heart disease presenting in our paediatric heart centre between March 2005 and May 2015 who underwent an emergent intervention within 48?h post-partum.

Materials and methods: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized foetal echocardiography, diagnosis was made post-natally. In 98/111 (88.2%), 30-day outcome was known.

Results: Regarding the entire cohort, 30-day survival did not differ significantly in prenatal and post-natal diagnosis group (71.2 vs. 72.9%; p?>?.1). Infants with prenatal diagnosis were more likely to be born by caesarean section (59.6% vs. 33.9%, p?=?.01). Those with post-natal diagnosis had a higher need for intubation (32.7% vs. 52.5%; p?p?=?.075).

Conclusion: For newborns who require emergent neonatal cardiac procedures, our results point towards a lower death rate after prenatal diagnosis of HLH/HLHC.     

Routine screening with fetal echocardiography for prenatal diagnosis of congenital heart disease

Congenital cardiac anomalies are the most common congenital anomalies, occurring in approximately eight of 1000 live births. Proper perinatal and neonatal management is dependent upon accurate prenatal diagnosis. Approximately 10% of fetuses with cardiac abnormalities have identified risk factors; hence, most of the anomalies occur in pregnancies without prenatal risk factors. The application of detailed fetal echocardiography for prenatal screening, at present reserved mainly for high-risk cases, requires further evaluation before being recommended for the general population.

This article presents our experience of evaluating the accuracy of fetal echocardiography as a screening method in detecting cardiac anomalies in the general population of Singapore. We reviewed data from 39 808 pregnant women who received antenatal care at the National University Hospital, Singapore, between January 1986 and December 1994, and who underwent routine fetal echocardiography at 21-22 weeks of gestation. We identified 294 cases of congenital heart defects by fetal echocardiography. We obtained a sensitivity of 85.4% for the detection of congenital heart disease, and a specificity of 99.9% to rule out such anomalies. Our positive and negative predictive rates were 87.7% and 99.9%, respectively.

We recommend routine screening by echocardiography of all pregnancies at 21-22 weeks of gestation, irrespective of risk stratifcation, for the prenatal detection of cardiac anomalies, in order to improve perinatal management.     

Smith-Magenis syndrome and cyanotic congenital heart disease: a case report

We report a male newborn infant of Chinese descent with the Smith-Magenis (SMS) syndrome who presented with a severe cyanotic congenital heart disease. This report adds pulmonary atresia and ventricular septal defect to the spectrum of cardiac defects seen in SMS.     

新生儿脑梗死早期诊断方法的探讨

目的 探讨早期诊断新生儿脑梗死(NCI)的相对可行的方法.方法 有目的 地收集在我院分娩的NCI高危儿和同顾分析转诊患儿的围产期病史;记录发病或确诊前后患儿临床表现;比较颅脑超声、CT及MRI三种影像学检查对NCI诊断的敏感性及各自特点并分析NCI发病高危因素、临床和影像学特点.结果 (1)与NCI相关的高危因素包括:母亲妊娠期高血压疾病和宫内感染、各种原因所致胎儿窘迫和(或)生后窒息、脑血管发育畸形和新生儿红细胞增多症等.(2)主要临床表现:55.3%(21/38)出现惊厥.其中57.1%(12/21)为与病灶部位相吻合的局灶性惊厥.23.7%(9/38)未出现惊厥,而有不同程度意识改变、肌张力异常和(或)原始反射异常等非特异性神经系统表现.21.1%(8/38)患儿没有显著临床表现.(3)发病后2～3 d内颅脑超声检查的阳性率达85.0%～90.9%,MRI的阳性率为(5/7～9/9),均高于CT的阳性率(2/4～5/9)(P=0.043).三种方法对晚期脑梗死病灶均能有效地做出诊断,阳性率分别为:颅脑超声94.7%、头颅CT 93.8 %,MRI为100%,差异没有统计学意义(P=0.59).结论 MRI尤其是弥散加权MRI和颅脑超声均能对NCI做出早期诊断;早期诊断的程序包括:关注具有高危围产因素的新生儿;生后密切观察神经系统的症状和体征、并给予常规颅脑超声筛查;必要时进行MRI确诊.     

Prostaglandin induced cortical hyperostosis in neonates with cyanotic heart disease

OBJECTIVE: To study the side effects of prolonged prostaglandin therapy especially hyperostosis and other musculoskeletal changes. METHODS: Case files of the neonates, with cyanotic heart disease, who had received prostaglandin infusion from early days of life, were reviewed. Patients with periosteal changes were identified. Their radiographs, serum alkaline phosphatase activity, duration and dose of prostaglandin and other side effects related to the prostaglandin were studied. RESULTS: Ten patients developed hyperostosis, who had received PGE1 infusion for a period of 9 to 195 days. Two babies developed coarse facial features, hypertrichosis, and edema of extremities and digital swelling. Serum alkaline phosphatase activity was significantly raised in the patients, with hyperostosis. Besides long bones, ribs and scapulae, the clavicles were also involved. The involvement of clavicles has not been previously reported. CONCLUSION: Hyperostosis is a common side effect of prolonged prostaglandin therapy. Abnormal facial features, hypertrichosis and coarse skin are additional adverse effects of long term therapy. Serum alkaline phosphatase activity can be used as a marker of the hyperostosis. In addition to other bones clavicles can also be involved. The above effects seem to be both dose and duration dependent.     

Effectiveness of pulse-oximetry in addition to routine neonatal examination in detection of congenital heart disease in asymptomatic newborns

Objective: To assess the feasibility and effectiveness of pulse-oximetry as a screening tool in the detection of critical congenital heart disease (CCHD) in newborns.

Methods: Post-natal babies born between 01/01/2007–31/12/2009 were eligible. Post-ductal pulse-oximetry was performed using Nellcor® NPB 40 pulse oximeter with reusable OXI-A/N saturation probe. Saturations ≥95% were deemed normal. If saturations were <95%, an echocardiogram was done. The regional paediatric cardiology database and death records identified babies later diagnosed with CCHD.

Results: 6329/9613 eligible babies were studied and pulse-oximetry was performed at a mean age of 28 hours (range 6–72 hours). Fourteen babies had saturations <95%. CCHD was diagnosed in 7/14 babies; 4/7 had no clinical signs. Of the remaining 7 babies, 3 had non-critical but significant CHD and 4 had an undiagnosed respiratory illness or sepsis. All babies with low saturations had identifiable pathologies. One baby with normal saturations was later diagnosed with transposition of the great arteries. The sensitivity and specificity of identifying an unwell baby was 93.3% and 100% respectively; the sensitivity and specificity of identifying CCHD was 87.5% and 99.8% respectively. Clinical examination alone would have missed 4/7 (57%) of these.

Conclusion: Pulse-oximetry is safe, acceptable, non-invasive and effective. Our study supports the routine use of pulse oximetry as part of the newborn check.     

Early diagnosis of fetal distress and neonatal asphyxia

122 cases of high risk pregnancies, consisting of mainly pregnancy-induced hypertension (PIH), medical complications, postmaturity and suspected distress were periodically monitored with 11 biophysical and biochemical assays. Positive prediction accuracy rate was analyzed with a neonatal UA pH less than 7.20 as the criterion of fetal hypoxia and ischemia. A number of predicting items together, namely: NST, the other Manning's 4 items, internal monitoring with abnormal Cardiotocography, meconium deeply stained amniotic fluid, FBS pH less than 7.2, uterine contraction time lasting greater than 37 sec and interval shorter than 70 sec, gave the best sensitivity, specificity, positive and negative prediction rates and total accuracy rate of 71.69%, 84.06%, 77.55%, 79.45% and 78.68% respectively. The causes for false positive and false negative cases were discussed. Special attention must be paid to those cases with low UA pH al though normal Apgar scores during the neonatal period.