新生儿单心室伴永存动脉干1例

1 病史患儿,男,6 d,因“少吃、少动2 d,呻吟1 d”入院。母亲系G1P1,患儿胎龄38 6周,于2005年8月30日剖宫产分娩,Apgar评分1 min内8-9分,5 min内10分,脐带绕颈1 周,羊水、胎盘无异常,无窒息抢救史,出生体重3 100 g。母亲在孕早期曾有病毒感染,口服阿莫西林胶囊1-2粒。父母非近亲婚配,无家族遗传性疾病史。入院查体:反应     

新生儿永存动脉干伴单心室一例

永存动脉干（persistent truncus arteriosus，PTA）是极为罕见的复杂先天性心血管畸形，发病率约0．5％～1％左右，约占婴儿先天性心脏病的0．4％。在先天性心血管的尸解中约占1％-3％，分娩活婴者约3／10000，是围产儿死亡的原因之一。由于严重的病例在宫内可能难以存活到分娩以及超声诊断学的发展，活产婴幼儿永存动脉干非常罕见。其中约1／4病例合并有单心室。现将临床所见一例新生儿永存动脉干合并单心室，共同房室瓣，房间隔缺损（多孔型）病例报道如下。     

新生儿永存动脉干1例

患儿 ,男 ,57ｈ ,因“全身皮肤发灰 ,呻吟样呼吸2 0ｍｉｎ”由产科转入。系第 3胎第 1产 ,孕 4 1+2 周 ,因“胎儿宫内窘迫”剖宫产 ,出生体重 2 950 ｇ ,Ａｐｇａｒ评分 1分钟及 5分钟均为 10分 ,羊水Ⅲ°污染。查体 :Ｔ 36℃ ,Ｒ 80次 /ｍｉｎ ,刺激无反应 ,全身皮肤苍白发灰、下腹部散在数个出血点 ,指压皮肤充盈 >3ｓ,前囟平坦 ,双目凝视 ,瞳孔等大等圆 ,对光反应迟钝。唇绀 ,鼻扇 ,两肺呼吸音粗 ,心率 140次 /ｍｉｎ ,心音有力、无杂音。肝右肋下 4ｃｍ ,质中 ,脾未及 ,四肢瘫软 ,肢端厥冷 ,原始反应消失。辅检 :血ＷＢＣ12 .3× 10 9/…     

婴儿肥厚型心肌病伴第二孔房间隔缺损早期死亡一例

患儿 女,出生28d,因纳呆伴出汗1d,剧烈哭吵数分钟急诊入院。来院时面色发绀,呼吸心跳停止,经抢救无效死亡。否认家族遗传病史,母亲系孕1产1,否认孕期感染及服用药物史,定期产前检查,空腹血糖无异常。患儿系足月顺产,出生时体重3．5kg,出生后1min阿普加（Apgar）评分10分。     

先天性风疹综合征致动脉导管未闭合并房间隔缺损一例

患儿女,21天。因“发现心脏杂音20天”入院。患儿系孕38”周,因羊水过少行剖宫产出生,手术顺利,Apgar评分1分钟为8分,5分钟为9分。出生体重2000g,羊水清,70ml,胎盘轻度钙化,脐带无异常。出生第2天发现心脏杂音。生后不久有多汗、气急,吃奶及哭闹后紫绀,而且气急多汗加重,喂养困难,进行性消瘦。出生后1周患过肺炎。     

新生儿三尖瓣闭锁并房室间隔缺损、肺动脉狭窄1例

1病历摘要 患者，男，20天，因“突发性呼吸暂停2次（每次约10s左右）伴颜面口唇紫绀10余小时”于2005年4月13日5：00am急诊入院，患儿系第1胎，足月顺产，新法接生，产后无紫绀及窒息，Apgar评分不详，6h开奶，12h排小便，18h排胎粪，未进行预防接种，其父母非近亲婚配，否认有家族遗传病史，其母孕期无异常，查体：T37．2℃，P148次／min，R45次／min，w3．0kg。     

新生儿先天性心脏病单心室一例

患儿男,9 h,以"发现全身皮肤黏膜青紫4 h"入院.系第1胎第1产,足月顺产,否认有宫内窘迫和产后窒息史.阿氏评分:1 min 10分,5 min 10分.出生体重3 500 g.     

单心室合并单心房、大动脉转位、共同房室瓣裂1例

患儿男 ,1小时 ,因生后皮肤青紫 ,气促伴口吐沫收入院。患儿Ｇ1Ｐ1足月阴道顺产 ,阿氏评分 1分钟 9分 ,5分钟、1 0分钟均 9分 ,肤色青紫扣 1分 ,胎膜早破 2天 ,羊水Ⅲ°污染 ,脐带绕颈 2周 ,呈麻花样 ,胎盘无异常。无家族遗传性疾病史 ,父母非近亲婚配 ,身体健康 ,母孕期无疾病史 ,无放射线及化学毒品接触史。体检 :体温 37℃ ,吸呼 6 6次 /分 ,体重 2 6 0 0ｇ ,营养中等 ,神志清楚 ,全身皮肤青紫 ,前囟平软 ,呼吸促 ,可见三凹征 ,双肺呼吸音粗 ,闻及痰鸣音 ,心率 1 30次”分 ,律齐 ,心音有力 ,各瓣膜听诊区未闻及杂音 ,腹部未异常 ,左侧睾…     

孤立性单脐动脉新生儿78例临床分析

目的 探讨孤立性单脐动脉（isolated single umbilical artery,ISUA）新生儿出生时的健康情况.方法 2006年1月至2012年12月于我院出生的ISUA新生儿78例为ISUA组,同期于我院出生的脐带发育正常新生儿78例为对照组,回顾性分析两组新生儿出生后的Apgar评分、出生体重、转入新生儿重症监护室发生率、新生儿脐动脉血pH值及新生儿母亲产前进行羊膜腔穿刺进行染色体检查情况.结果 ISUA组新生儿体重（3246±75）g,低于对照组（3565±58）g,差异有统计学意义（P＜0.05）,而两组新生儿的出生后Apgar评分、转入新生儿重症监护室的发生率、新生儿脐动脉血pH值的差异均没有统计学意义（P＞0.05）.ISUA组新生儿母亲产前26例行羊膜腔穿刺,对照组仅l例,胎儿染色体检查均正常.结论 ISUA新生儿出生时与正常新生儿相比健康状况无明显差异,不需特别监护.     

先天性食管闭锁合并右位心1例

患儿,男,6．5h,因出生后口吐白沫、发绀人院。体查：呼吸急促,唇周稍发绀,双肺呼吸音粗,可闻及湿哕音,心尖搏动最响处位于右胸侧,腹胀,叩诊呈鼓音,听诊可闻及胃泡呼吸音,其余未见异常。胃管不能顺利插入,消化道造影提示食管闭锁,闭锁端位于第四胸椎下缘,胃泡及肠管充气明显。心脏彩超提示右位心、房间隔缺损（继发孔型）、左向右分流型;二尖瓣及三尖瓣轻度反流;大血管位置及连接关系正常。诊断为：①食道闭锁（3b／C型）;②新生儿肺炎;     

Truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle

Summary The association of truncus arteriosus with congenital left heart abnormalities is rare. We describe a case of truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle. In 11 other patients diagnosed with truncus arteriosus, none had left heart hypoplasia. Despite the truncal root being primarily or entirely committed to the right ventricle, more right than left heart abnormalities have been found. This is in contrast to double-outlet right ventricle in which left heart abnormalities are more often associated.     

“Absent” pulmonary valve with atrial septal defect and patent ductus arteriosus

Summary The case of a newborn with absent pulmonary valve, atrial septal defect, and patent ductus arteriosus is reported. He underwent surgical repair at five weeks of age, with closure of the patent ductus arteriosus and porcine pulmonary valve insertion. The clinical course leads us to suggest that reduction of pulmonary artery pressure and pulmonary valve competence will lead to prompt improvement in tracheobronchial compression and respiratory symptoms; no pulmonary arterioplasty is needed.     

Transcatheter occlusion of atrial septal defect and patent ductus arteriosus: Now a reality in India

Conclusions Recent developments have resulted in availability of a safe and effective method for transcatheter occlusion of atrial septal defects and patent ductus arteriosus. Furthermore, this technique has now been brought to India. The secundum atrial septal defects can be closed effectively with buttoned devices that can be delivered via 8-French sheaths. Patent ductus arteriosus can also be closed with adjustable buttoned devices delivered via 7-French sheaths. Patient selection, meticulous attention to the details of the technique, and careful follow-up remain germane to the continued success of this technique in occluding cardiac septal defects. This study is supported in part by a NIH Grant No. RR. 03186 from the Division of Research Resources to the University of Wisconsin Medical School, Madison, Wisconsin.     

国产房间隔缺损堵塞器关闭猪房间隔缺损的实验研究

目的 评价国产房间隔缺损（ASD）堵塞器关闭猪ASD的可行性、有效性和安全性。方法 选用3-4周乳猪6头，体重5-10kg（平均7.5kg)；应用房隔穿刺和Rashkind球囊房隔造口术，建立ASD动物模型；在X线和超声引导下，用测量球囊测定ASD最大伸展直径并结合经胸超声（TEE），选择适当大小的国产ASD封堵器进行堵塞。结果 6头猪完全封猪，仅1例术后即刻有微量的残余分流，24h消失；随访1-6个月，无产发症发生。结论 国产ASD堵塞器关闭猪ASD成功率高、并发症少、创伤小，是一种安全、有效、可行的方法。     

Three-dimensional echocardiography in children with atrial septal defect

The selection of patients for transcatheter or surgical closure of a secundum atrial septal defect requires accurate information regarding the anatomy of the defect such as its maximal diameter and the amount of circumferential tissue rim. Two-dimensional echocardiography is wanting as a means to define selection criteria for atrial septal defect closure. Since the defect is visualised from multiple orthogonal planes, maximal atrial septal defect diameter is widely under-estimated. The transcatheter approach measures the stretched diameter, but cannot be applied alone for patient selection since it does not provide information on the tissue rim. Three-dimensional echocardiography allows unique en face views of the atrial septum. Previous studies have shown the ability of 3-dimensional echocardiography to depict information regarding the shape, the maximal diameter and the rims surrounding the defect. Transthoracic 3-dimensional echocardiography is accurate to measure atrial septal defect maximal diameter and rims surrounding the defect. Such a non-invasive method could be applied to children selection for atrial septal defect closure. The transcatheter balloon method provides additional information such as the resistance of the septum. Device sizing and placement should take into account both 3-dimensional echocardiography and transcatheter findings.