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成人及儿童心脏憩室临床分析
http://www.100md.com 《临床心血管病杂志》 2000年第4期
     作者:尤士杰 姚民 杨跃进 李琳 高润霖 乔树宾 秦学文 姚康宝 陈纪林 徐义枢 陈在嘉

    单位:中国医学科学院 中国协和医科大学 心血管病研究所 阜外心血管病医院冠心病研究室 北京,100037

    关键词:先天性心脏憩室;憩室缝合术;左心室造影

    临床心血管病杂志000414 摘要 目的:探讨成人与儿童心脏憩室的临床特点。方法:将经心脏造影或手术证实为心脏憩室的9例患者临床资料进行回顾性分析。结果:①4例成人患者(成人组)皆因并发冠心病在冠状动脉及左室造影检查时被偶然发现。5例儿童患者(儿童组)中4例因并存其他严重先天性心脏畸形而施行外科矫治手术时被确诊,另1例经左室造影证实。②成人组平均憩室瘤体径为20~48(36.3±11.8) mm,憩室颈径为10~17(14.0±2.9 )mm;儿童组平均憩室瘤体径25~80(42.0±22.0) mm,憩室颈径为4~12(8.6±3.9)mm。③成人组均为孤立性左室肌型憩室,无与憩室有关的并发症,未行手术治疗;儿童组中仅1例为孤立性左室肌型憩室,其余4例均根据其并存的心脏畸形类型分别接受了憩室缝合术和房、室间隔修补术、三尖瓣成形术、二尖瓣置换术或动脉导管结扎术等治疗。结论:成人与儿童心脏憩室的临床特点有许多不同之处。
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    Analysis of the clinical features of congenital cardiac diverticulum in adults and children

    YOU Shi-jie YAO Min YANG Yue-jin LI Ling GAO Run-lin QIAO Shu-bin

    QIN Xue-wen YAO Kang-bao CHENG Ji-lin XU Yi-shu CHEN Zai-jia

    (Cardiovascular Institute and Fuwai Heart Hospital, Chinese Academy of

    Medical Sciences and Peking Union Medical College, Beijing 100037)
, 百拇医药
    Abstract Objective:In order to elucidate different clinical features between adults and children with the congenital cardiac diverticulum.Method:The clinical characters of nine cases with congenital cardiac diverticulum confirmed by the ventriculography or surgical operation in our hospital were analyzed retrospectively.There were 4 adult cases,their average age were (59.3±7.6) years (50~68 years);and 5 children cases, their average age were (6.5±4.4) years (2.5~14years). Result:①Four cases with left ventricular diverticulum in adults were all incidentally discovered and confirmed by left ventriculography under went for differention coronary heart disease diagnosis.Five cases in children were confirmed by surgical operation during corrected operation for other severe congenital heart malformation.②The average diameter of the diverticulum were (36.3±11.8) mm(20~48 mm), the neck of diverticulum were (14.0±2.9) mm(10~17 mm) in 4 adults. The average diameter of the diverticulum were (42.0±22.0) mm(25~80 mm), the neck of diverticulum were (8.6±3.9) mm (4~12 mm) in 5 children.③All the diverticulum were isolateral left ventricular muscular diverticulum in 4 adults, and the patients had no complaines related to the diverticulum, and were not operated.Only 1 case with the diverticulum was isolateral left ventricular muscular diverticulum in children, other children with the diverticulum (1 case with bioverticular musucular diverticulum, 2 case with right ventricular muscular diverticulum, 1 case with left atrial fibrous diverticulum) were underwent surgery according to the types of congenital heart anomales their diverticulums were sutured at the same time.Conclusion:There were many different clinical features between the adults and the children with congenital cardiac diverticulum.
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    Key words Congenital cardiac diverticulum Diverticulosutura Left ventriculography

    心脏憩室是一种极为罕见的先天性心脏畸形,文献报道甚少。现将我院确诊的9例的临床特点回顾性分析如下。

    1 临床资料

    1.1 对象

    选择我院1983年3月~1999年3月期间收治的心脏憩室患者9例,分为成人组4例,均为男性,年龄50~68(59.3±7.6)岁;儿童组5例,其中男4例,女1例,年龄2.5~14(6.5±4.4)岁。

    1.2 临床特点及伴发其他心脏疾患情况

    成人组均未并发其他先天性心脏畸形,2例有陈旧性心肌梗死史,2例有心绞痛史,心电图可见相应导联异常Q波和(或)ST-T改变。
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    儿童组5例患者均可闻及心脏杂音和心电图异常(2例为完全性右束支传导阻滞,1例呈右房增大及左前分支阻滞,1例有异常Q波及ST-T改变,1例为窦性心动过速),2例并发预激综合征,4例并发其他先天性心脏畸形〔其中左、右双室憩室并发室间隔缺损(VSD)1例,右室憩室并发房间隔缺损(ASD)加三尖瓣关闭不全(TR)1例,右室憩室并发VSD加ASD加动脉导管未闭(PDA)1例,左房憩室并发二尖瓣关闭不全(MR)加TR1例〕,均伴有明显紫绀、胸闷和呼吸困难;另1例为孤立性左室憩室,伴轻度胸闷、心悸。

    1.3 影像学检查特点

    1.3.1 X线胸片:成人组肺血均正常,无肺动脉段突出,心胸比例正常,2例可见左室壁有局限性膨出。儿童组3例肺血增多,1例肺血减少,1例肺血正常;3例肺动脉段突出,4例心胸比例增大,心胸比值为0.55~0.72(0.63±0.07);左室扩大1例,左室、右房扩大1例,右房、右室、左房扩大1例,右房、右室扩大1例,无心脏扩大1例。
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    1.3.2 多普勒超声心动图:成人组各心腔大小均正常,其中3例可见左室壁呈局限性瘤样膨出,室壁变薄,运动幅度减低,憩室颈部明显小于扩张的瘤体与左室腔,颈部有酷似心脏破裂的室壁残端或局限性心肌肥厚改变,脉冲多普勒检测在憩室颈部均可探及与左室收缩和舒张同步的出入憩室瘤体的血流频谱,显示出肌型憩室的血流频谱特点,未发现其他心脏畸形和瓣膜病变;另1例多普勒超声心动图未发现异常。儿童组4例可见心腔扩大,并证实存在ASD、VSD或PDA等心脏畸形,1例并发严重MR和TR。2例可见心室壁膨出呈瘤样改变,分别位于左室心尖部和右室流出道,其中位于左室心尖部者可见憩室瘤壁主动收缩运动。

    1.3.3 心脏造影或手术所见:成人组左室造影均显示典型左室憩室改变,其中2例还经电子束扫描体层摄影(UFCT)和磁共振显像(MRI)进一步证实。左室憩室中位于心尖部2例,前侧壁1例,主动脉瓣下1例,其中3例可见与左室同步的收缩运动或瘤体造影剂排空现象。根据左室造影的记录胶片测量,憩室瘤体径20~48(36.3±11.8) mm,憩室颈径10~17(14.0±2.9) mm,憩室瘤体径与颈径比为2.6∶1。冠状动脉造影还显示,2例伴有左前降支中段或左回旋支近段50%~60%狭窄,2例(均为陈旧性心肌梗死患者)伴有左前降支近、中段>90%的严重狭窄。儿童组4例经手术分别证实为左室流出道和右室肺动脉瓣下双室肌型憩室、右室前壁肌型憩室、右室三尖瓣下肌型憩室和左房左侧纤维型憩室;1例经心脏造影证实为左室心尖部肌型憩室。根据术中实测或左室造影记录胶片测量,憩室瘤体径25~80(42.0±22.0)mm,憩室颈径4~12(8.6±3.9)mm,憩室瘤体径与颈径比为4.1∶1。
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    1.4 接受治疗情况

    因成人组均未并发其他先天性心脏畸形,且无与憩室有关的并发症,故对左室憩室本身未行外科手术治疗,对其中2例伴冠状动脉左前降支近、中段90%以上严重狭窄者,施行经皮腔内冠状动脉成形术(PTCA)及冠状动脉内支架植入术(ICS),另2例伴冠状动脉左前降支或左回旋支50%~60%狭窄者,仅给予内科保守治疗。儿童组除1例孤立性左室肌型憩室给予内科保守治疗外,其余4例均根据其伴发的心脏畸形的类型分别接受了憩室缝合术和房、室间隔修补术、三尖瓣成形术、二尖瓣置换术或动脉导管结扎术等治疗。

    2 讨论

    据文献报道心脏憩室绝大多数病例为婴幼儿和儿童,成人少见〔1〕。通常在X线胸片、超声心动图检查时被发现,经心血管造影、手术或尸检证实,UFCT、MRI等检查有助于确定诊断。

    大多数心脏憩室伴有其他类型心脏畸形,少数不伴者称为孤立性心脏憩室〔1,2〕。本文4例成人心脏憩室全部为孤立性左室憩室,而5例儿童心脏憩室中,仅1例为孤立性左室憩室,其余4例均并存其他类型的心脏畸形。提示孤立性心脏憩室多为左室憩室,且常在成年人群中被发现〔2,3〕,而幼儿和儿童期的心脏憩室患者多同时并发其他类型的心脏畸形。
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    心脏憩室在组织学上可分为肌型和纤维型两种类型〔4,5〕。多普勒超声血流频谱对鉴别肌型憩室抑或纤维型憩室及真、假性室壁瘤有重要参考价值。文献报道肌型憩室以位于左室者最多见〔2,3〕,本文4例成人心脏憩室与上述结论相似。5例儿童肌型心脏憩室多见于左室以外的心腔〔2,3〕

    本文资料表明,儿童组与成人组心脏憩室的临床特点有明显不同。儿童组常因有其他先天性心脏病引起的临床症状和心脏杂音,在X线胸片和超声心动图检查时发现心脏畸形,在施行心脏畸形矫正手术中发现心脏憩室。而成人组往往由于患有冠心病,在冠状动脉及左室造影检查时被偶然发现,除有与冠状动脉狭窄病变有关的心肌缺血症状外,并无其他临床症状。多数作者认为若不同时并发其他心脏畸形或无明显临床症状时,不宜选择外科手术治疗〔1,2〕。本文4例并发其他先天性心脏畸形的儿童在行心脏畸形矫治手术中发现心脏憩室,并同时行憩室缝合术。而对患左室孤立性憩室的4例成人和1例幼儿因考虑上述原因未行外科手术治疗,仅针对其中2例成人患者的冠状动脉严重狭窄病变施行了PTCA和ICS等介入性治疗。
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    临床上心脏憩室患者常可发生某些由憩室本身引起或与其有关的并发症〔1〕。本文儿童心脏憩室患者中均可见二尖瓣或三尖瓣关闭不全、心力衰竭、预激综合征及其他心律失常发生,而4例成人左室憩室患者均无上述并发症发生,但其中2例有前壁或前壁加高侧壁心肌梗死史,冠状动脉造影显示左前降支存在≥90%的严重狭窄病变,但左室造影除显示与左室心尖部憩室部位一致的前壁心肌梗死相应室壁节段运动明显减低外,并无真、假性室壁瘤形成,其原因尚不清楚,可能与发生急性心肌梗死时冠状动脉早期再灌注有关。

    参考文献

    1,Ichikava K, Makino K, Futagami Y, et al. Isolated congenital left ventricular diverticulum in an adult. Angiology, 1994, 45: 743~747

    2,Mady C. Left ventricular diverticulum: Analysis of two operated cases and review of the literature. Angiology, 1982, 33: 280~286
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    3,Okereke O U J, Cooley D A, Frazier O H, et al. Congenital diverticulum of left ventricle. Thorac Cardiovasc J Surg, 1986, 91: 208~214

    4,Treistman B, Cooley D A, Luftschanowski R, et al. Diverticulum or aneurysm of left ventricle.Am J Cardiol, 1973, 32: 119~123

    5,Gueron M, Higsch M, Opschitzer I, et al. Left ventricular diverticulum and mitral incompetence in asymptomatic children.Circulation,1975, 22:181~186

    (收稿 1999-07-12), 百拇医药