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感染及肾瘢痕并不直接影响反流的消失,但肾瘢痕多见于严重反流的病例,反流自行消失机会少。
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原发性膀胱输尿管反流在青年和成人中的发展趋势尚有争论。
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【临床表现】原发性膀胱输尿管反流的症状主要从两方面表现,肾积水和尿路感染。
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反流导致上尿路内的尿液无法排空,到一定程度即会产生肾盂和输尿管的扩张尿路内的尿液无法排空,到一定程度即会产生肾盂和输尿管的扩张,在超声上反映出来。
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尿路感染在儿童中更多的表现是非特异性的,包括发热、嗜睡无力只允许尿液从输尿管流进膀胱,而不允许尿液从膀胱向输尿管的倒流。
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输尿管膀胱连接部抗反流机制在公元150年已被认识到,1883年Pozzi在动物实验和人体中观察到膀胱输尿管反流,但尚不能确定是否为异常现象。
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其后Bumpus和Gruber等发现反流与泌尿系感染之间的关系,但当时更多的认为膀胱输尿管反流是继发于膀胱颈部梗阻,故1950年前后,人们施行了许多不必要的手术如膀胱颈部成形去解除并不存在的膀胱颈梗阻。
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从20世纪60年代起,出现了大量关于膀胱输尿管反流与尿路感染和肾瘢痕之间关系的研究,并逐步认识到膀胱输尿管反流可导致高血压和肾衰竭。
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【发病率】原发性膀胱输尿管反流在正常婴儿和儿童的发生率很难知道,统计的发生率为1%~18.5%,但70%发生在尿路感染的患儿中。
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反流有一定的性别倾向,女孩占85%,男孩如有尿路感染其反流可能性大,包皮环切对其发生也有影响,环切者,反流率低。
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年龄与反流发生率成反比,4岁20%,12岁15%,成人5.2%,由此也提示随着膀胱及输尿管的发育(输尿管隧道延长),反流可自然缓解。
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图12-7膀胱输尿管反流分级反流对肾功能的影响与尿路不完全性梗阻对肾脏的影响是一样的。
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反流时上尿路内压增加,远端肾单位首受其害,因此肾小管功能受损早于肾小球。
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无菌反流影响肾小管的浓缩能力,且持续时间较长。
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肾小球功能在有肾实质损害时受影响,并与肾实质损害的程度成正比。
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反流可以影响肾脏的发育,如抑制其胚胎发生,导致肾发育不全或肾发育异常;而长期反流的患儿发生肾脏不生长等情况。
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反流病人发生高血压的机会较高。
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如反流未能有效控制,肾瘢痕进行性发展可导致肾衰竭。
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原发性膀胱输尿管反流一般随年龄增长逐渐好转,可能是因输尿管膀胱壁内段和膀胱三角区肌肉的生长和成熟之故。
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如静脉尿路造影显示输尿管口径正常,原发反流85%可自然消失。
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反流的自然消失与反流累及的范围也有关系,单侧反流自然消失率可达65%;双侧反流,输尿管无扩张者,自然消失率是50%,有输尿管扩张的仅10%左右。
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感染及肾瘢痕并不直接影响反流的消失,但肾瘢痕多见于严重反流的病例,反流自行消失机会少。
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原发性膀胱输尿管反流在青年和成人中的发展趋势尚有争论。
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【临床表现】原发性膀胱输尿管反流的症状主要从两方面表现,肾积水和尿路感染。
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反流导致上尿路内的尿液无法排空,到一定程度即会产生肾盂和输尿管的扩张,在超声上反映出来。
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在婴幼儿无菌反流可表现为肾绞痛,但表现不典型。
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大儿童可指出在膀胱充盈或排尿时肋部疼痛年长儿在并发急性肾盂肾炎时也有腰腹部疼痛和触痛。
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【诊断】排肾同位素尿道DMSA(VCUG)是确定膀胱输尿管反流诊断和分级的金标准瘢痕凡超声检查发现肾小球水和肾小管泌尿系感染发作的均应进行VCUG。
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由于小儿对检查的恐惧和不合作原发性膀胱输尿管反流结果,造影时可予镇静剂,适当时要重复进行。
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放射性核素膀胱造影,能准确确定有无反流,但对确定反流控制感染,保护肾功能,防止并发症究。
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静脉尿路造影可很好地显示肾脏形态,通过所显示的肾轮廓,可计算肾实质的厚度和肾的生长情况,但后一方面,超声更加简单易行。
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肾同位素(DMSA)扫描可清晰显示肾瘢痕情况,用于随访患儿有无新瘢痕形成,并可评价肾小球和肾小管的功能,确定分肾功能,比较手术前后的肾功能等。
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【治疗】由于原发性膀胱输尿管反流在许多小儿随生长发育可自然消失,而且无菌尿的反流不引起肾损害,原发性膀胱输尿管反流的治疗原则是控制尿液染,保护肾功能,防止并发症。
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即使是严重的反流,如患儿年龄小,肾功能好,无生长发育障碍预防性抗生素虑药物治疗。
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(一)药物治疗所选择的药物应当是抗菌谱广、易服用、价廉、对患儿毒身高小、体重内血压高及对体内正常尿常规影响血红蛋白的抗白细胞计数应以其最小剂量肾功能测定制感染为宜。
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感染发作时使用治疗量,感染被控制尿液用预防量,预防量应为治疗量的1/2~1/3,这样很少引起不良反应。
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预超声前服用,是肾脏夜间尿液在肾盂内输尿管间最长,更易排尿性膀胱尿道造影间一直持续到反流消失为止。
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反流程度明显减轻的大龄儿是否应继续服用预防性抗生素,目前放射性同位素膀胱造影治疗期间,患儿应定期随访手术治疗个月做一次体格检查,记录身高、体重及血压。
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实验室检查包括尿常规、血红蛋白及白细胞计数等,每年做一次肾功进行性肾瘢痕扩展查也要根据患儿的病情随时调整。
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为膀胱输尿管反流和梗阻并存、异位输尿管开口、或伴有较大的输尿管旁憩室、或输尿管开口于膀胱憩室内肾脏的发育手术肾盂抗反流的输尿管膀胱再吻合术膀胱尿道输尿管膀胱再植术个月重复检查,以后每经膀胱外12经膀胱内一膀胱内外联合操作查也可改用放射性同位素膀胱造影。
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Cohen输尿管膀胱再吻合术疗Politano-Leadbetter输尿管膀胱再吻合术不是Glenn-Anderson输尿管膀胱再吻合术或不能防止感染复发;有进行性肾瘢痕扩展或新瘢痕形成时要膀胱镜术治疗。
[ { "id": 0, "entity": "Cohen输尿管膀胱再吻合术", "start_offset": 0, "end_offset": 14, "label": "pro" }, { "id": 1, "entity": "Politano-Leadbetter输尿管膀胱再吻合术", "start_offset": 15, "end_offset": 43, "label": "pro" }, { "id": 2, "entity": "Glenn-Anderson输尿管膀胱再吻合术", "start_offset": 45, "end_offset": 68, "label": "pro" }, { "id": 3, "entity": "膀胱镜", "start_offset": 95, "end_offset": 98, "label": "equ" } ]
膀胱输尿管反流和梗阻并存Teflon管胶原蛋白或伴有较大的输尿管旁憩室、或输尿管开口于膀胱憩室内时首先考虑手术。
[ { "id": 0, "entity": "Teflon", "start_offset": 12, "end_offset": 18, "label": "dru" }, { "id": 1, "entity": "胶原蛋白", "start_offset": 19, "end_offset": 23, "label": "dru" }, { "id": 2, "entity": "伴有较大的输尿管旁憩室、或输尿管开口于膀胱憩室内", "start_offset": 24, "end_offset": 48, "label": "sym" } ]
抗反流的输尿管膀胱再吻合术(或称输尿管膀胱再Teflon多种,术式分为经膀胱外肺经脑胱内和膀胱内外联合操作三大类。
[ { "id": 0, "entity": "抗反流的输尿管膀胱再吻合术", "start_offset": 0, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "输尿管膀胱", "start_offset": 16, "end_offset": 21, "label": "bod" }, { "id": 2, "entity": "Teflon", "start_offset": 22, "end_offset": 28, "label": "dru" }, { "id": 3, "entity": "膀胱", "start_offset": 36, "end_offset": 38, "label": "bod" }, { "id": 4, "entity": "肺", "start_offset": 39, "end_offset": 40, "label": "bod" }, { "id": 5, "entity": "脑", "start_offset": 41, "end_offset": 42, "label": "bod" }, { "id": 6, "entity": "膀胱", "start_offset": 45, "end_offset": 47, "label": "bod" } ]
目前较肉芽肿的术式有,Cohen输尿管膀胱再吻合术、Politano-LeDefluxer输尿管葡聚糖颗粒合术及Gle高分子透明质酸钠on输尿管膀胱再吻合术等。
[ { "id": 0, "entity": "肉芽肿", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "Cohen输尿管膀胱再吻合术", "start_offset": 11, "end_offset": 25, "label": "dis" }, { "id": 2, "entity": "Deflux", "start_offset": 37, "end_offset": 43, "label": "dru" }, { "id": 3, "entity": "葡聚糖颗粒", "start_offset": 48, "end_offset": 53, "label": "dru" }, { "id": 4, "entity": "高分子透明质酸钠", "start_offset": 59, "end_offset": 67, "label": "dru" } ]
Teflon微粒由于可渗入血流,引起肺、脑等关键脏器的栓塞,或在注射局部形成肉芽肿,至今尚未获得美国食品和药品监督局(FDA)的批准。
[ { "id": 0, "entity": "Teflon微粒", "start_offset": 0, "end_offset": 8, "label": "dru" }, { "id": 1, "entity": "肺", "start_offset": 18, "end_offset": 19, "label": "bod" }, { "id": 2, "entity": "脑", "start_offset": 20, "end_offset": 21, "label": "bod" } ]
Deflux,一种葡聚糖颗粒和1%的高分子透明质酸钠各半混合而成的悬液,作为注射材料已有产品供应。
[ { "id": 0, "entity": "Deflux", "start_offset": 0, "end_offset": 6, "label": "dru" }, { "id": 1, "entity": "葡聚糖颗粒", "start_offset": 9, "end_offset": 14, "label": "dru" }, { "id": 2, "entity": "高分子透明质酸钠", "start_offset": 18, "end_offset": 26, "label": "dru" } ]
第二节房间隔缺损房间隔缺损(atrialseptaldefect,ASD)是指心房间隔任何部位出现缺损造成心房水平的交通。
[ { "id": 0, "entity": "房间隔缺损", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "房间隔缺损", "start_offset": 8, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "atrialseptaldefect", "start_offset": 14, "end_offset": 32, "label": "dis" }, { "id": 3, "entity": "ASD", "start_offset": 33, "end_offset": 36, "label": "dis" }, { "id": 4, "entity": "心房间隔", "start_offset": 39, "end_offset": 43, "label": "bod" }, { "id": 5, "entity": "心房", "start_offset": 53, "end_offset": 55, "label": "bod" } ]
【病理解剖】在胚胎发育达4mm时,原始心房内相继长出第一及第二房间隔,经与中心心内膜垫会合后,将单腔的原始心房一分为二。
[ { "id": 0, "entity": "心房", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 1, "entity": "第一及第二房间隔", "start_offset": 26, "end_offset": 34, "label": "bod" }, { "id": 2, "entity": "中心心内膜垫", "start_offset": 37, "end_offset": 43, "label": "bod" }, { "id": 3, "entity": "原始心房", "start_offset": 51, "end_offset": 55, "label": "bod" } ]
在房间隔发育的同时,静脉窦也不断发育和移位,静脉窦移至右心房并扩大成为右心房的主要部分,使上腔静脉、下腔静脉、冠状静脉窦分别开口于右心房内,构成右心房的静脉窦部,而原始的右心房侧发育成为右心耳及右心房外侧壁,构成右心房的体部。
[ { "id": 0, "entity": "房间隔", "start_offset": 1, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "静脉窦", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "静脉窦", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "右心房", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 4, "entity": "右心房", "start_offset": 35, "end_offset": 38, "label": "bod" }, { "id": 5, "entity": "上腔静脉", "start_offset": 45, "end_offset": 49, "label": "bod" }, { "id": 6, "entity": "下腔静脉", "start_offset": 50, "end_offset": 54, "label": "bod" }, { "id": 7, "entity": "冠状静脉窦", "start_offset": 55, "end_offset": 60, "label": "bod" }, { "id": 8, "entity": "右心房", "start_offset": 65, "end_offset": 68, "label": "bod" }, { "id": 9, "entity": "右心房的静脉窦部", "start_offset": 72, "end_offset": 80, "label": "bod" }, { "id": 10, "entity": "右心房", "start_offset": 85, "end_offset": 88, "label": "bod" }, { "id": 11, "entity": "右心耳", "start_offset": 93, "end_offset": 96, "label": "bod" }, { "id": 12, "entity": "右心房外侧壁", "start_offset": 97, "end_offset": 103, "label": "bod" }, { "id": 13, "entity": "右心房", "start_offset": 106, "end_offset": 109, "label": "bod" } ]
心房形成及分隔过程出现异常,就可出现相应的畸形,根据胚胎发生,将房间隔缺损房间隔缺损分为四个类型:(一)原发孔型房间隔缺损房室瓣未被累及,少见。
[ { "id": 0, "entity": "心房", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "房间隔缺损", "start_offset": 32, "end_offset": 37, "label": "dis" }, { "id": 2, "entity": "房间隔缺损", "start_offset": 37, "end_offset": 42, "label": "dis" }, { "id": 3, "entity": "原发孔型房间隔缺损", "start_offset": 52, "end_offset": 61, "label": "dis" }, { "id": 4, "entity": "房室瓣", "start_offset": 61, "end_offset": 64, "label": "bod" } ]
缺损位于冠状静脉窦开口的前方,缺损的下缘即为左右房室环的接合部,前方接近主动脉壁,后缘接近房室结。
[ { "id": 0, "entity": "冠状静脉窦开口", "start_offset": 4, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "左右房室环的接合部", "start_offset": 22, "end_offset": 31, "label": "bod" }, { "id": 2, "entity": "主动脉壁", "start_offset": 36, "end_offset": 40, "label": "bod" }, { "id": 3, "entity": "房室结", "start_offset": 45, "end_offset": 48, "label": "bod" } ]
(二)继发孔型房间隔缺损(中央型)占总数约70%,可以呈单孔,少数为多发型,也有筛孔状者。
[ { "id": 0, "entity": "继发孔型房间隔缺损", "start_offset": 3, "end_offset": 12, "label": "dis" } ]
(三)静脉窦型房间隔缺损占4%,其上方为上腔静脉开口,下缘为房间隔,卵圆窝和冠状静脉窦口均存在。
[ { "id": 0, "entity": "静脉窦型房间隔缺损", "start_offset": 3, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "上腔静脉", "start_offset": 20, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "房间隔", "start_offset": 30, "end_offset": 33, "label": "bod" }, { "id": 3, "entity": "卵圆窝", "start_offset": 34, "end_offset": 37, "label": "bod" }, { "id": 4, "entity": "冠状静脉窦口", "start_offset": 38, "end_offset": 44, "label": "bod" } ]
几乎均伴有右上肺静脉异位引流。
[ { "id": 0, "entity": "右上肺静脉异位引流", "start_offset": 5, "end_offset": 14, "label": "dis" } ]
可分为三种亚型:①上腔静脉窦型房间隔缺损:位于上腔静脉入口处,多数伴有1支或数支右上肺静脉或右肺上、中叶静脉向上移位,进入上腔静脉根部;②下腔静脉窦型房间隔缺损:此型罕见。
[ { "id": 0, "entity": "上腔静脉窦型房间隔缺损", "start_offset": 9, "end_offset": 20, "label": "dis" }, { "id": 1, "entity": "上腔静脉", "start_offset": 23, "end_offset": 27, "label": "bod" }, { "id": 2, "entity": "上肺静脉", "start_offset": 41, "end_offset": 45, "label": "bod" }, { "id": 3, "entity": "右肺上、中叶静脉", "start_offset": 46, "end_offset": 54, "label": "bod" }, { "id": 4, "entity": "上腔静脉根部", "start_offset": 61, "end_offset": 67, "label": "bod" }, { "id": 5, "entity": "下腔静脉窦型房间隔缺损", "start_offset": 69, "end_offset": 80, "label": "dis" } ]
在卵圆窝后下方腔静脉入口处出现裂隙状小缺损,Kirklin等称之为后房间隔缺损,常伴有右下肺静脉1支或数支向下移位进入下腔静脉中。
[ { "id": 0, "entity": "卵圆窝", "start_offset": 1, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "腔静脉", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "裂隙状小缺损", "start_offset": 15, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "后房间隔缺损", "start_offset": 33, "end_offset": 39, "label": "dis" }, { "id": 4, "entity": "右下肺静脉", "start_offset": 43, "end_offset": 48, "label": "bod" }, { "id": 5, "entity": "下腔静脉", "start_offset": 59, "end_offset": 63, "label": "bod" } ]
因右下肺静脉造影时右心下缘呈弯刀状放射影,也称为弯刀综合征(scimitarsyndrome)。
[ { "id": 0, "entity": "右下肺静脉造影", "start_offset": 1, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "右心下缘", "start_offset": 9, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "弯刀状放射影", "start_offset": 14, "end_offset": 20, "label": "sym" }, { "id": 3, "entity": "弯刀综合征", "start_offset": 24, "end_offset": 29, "label": "dis" }, { "id": 4, "entity": "scimitarsyndrome", "start_offset": 30, "end_offset": 46, "label": "dis" } ]
位于正常冠状窦口处,缺损后缘为心房壁。
[ { "id": 0, "entity": "冠状窦口", "start_offset": 4, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "心房壁", "start_offset": 15, "end_offset": 18, "label": "bod" } ]
有两种亚型:冠状静脉窦顶盖部分或全部缺如(unroofedcoronarysinus),伴残存左上腔静脉入冠状静脉窦或左房者占90%;异位肺静脉入冠状静脉窦(三房心的一种),不伴左上腔静脉。
[ { "id": 0, "entity": "冠状静脉窦顶盖部分或全部缺如", "start_offset": 6, "end_offset": 20, "label": "dis" }, { "id": 1, "entity": "unroofedcoronarysinus", "start_offset": 21, "end_offset": 42, "label": "dis" }, { "id": 2, "entity": "残存左上腔静脉入冠状静脉窦", "start_offset": 45, "end_offset": 58, "label": "dis" }, { "id": 3, "entity": "异位肺静脉入冠状静脉窦", "start_offset": 67, "end_offset": 78, "label": "dis" }, { "id": 4, "entity": "左上腔静脉", "start_offset": 89, "end_offset": 94, "label": "dis" } ]
(四)单心房此型多并发其他复杂性先天性心脏病。
[ { "id": 0, "entity": "单心房", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "复杂性先天性心脏病", "start_offset": 13, "end_offset": 22, "label": "dis" } ]
【病理生理】除非缺损较小,通常通过房间隔缺损分流方向及分流量取决于两个下游心室的相对顺应性,与房间隔缺损的大小无关。
[ { "id": 0, "entity": "缺损", "start_offset": 8, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "房间隔缺损", "start_offset": 17, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "下游心室", "start_offset": 35, "end_offset": 39, "label": "bod" }, { "id": 3, "entity": "房间隔缺损", "start_offset": 47, "end_offset": 52, "label": "dis" } ]
通常右心室顺应性较左心室佳,因此,多数情况下为左向右分流。
[ { "id": 0, "entity": "右心室", "start_offset": 2, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "左心室", "start_offset": 9, "end_offset": 12, "label": "bod" } ]
在婴儿期,由于右心室肥厚、顺应性不佳,心房水平的左向右分流少。
[ { "id": 0, "entity": "右心室肥厚", "start_offset": 7, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "心房", "start_offset": 19, "end_offset": 21, "label": "bod" } ]
在出生后第一周,随着肺血管阻力下降,右心室顺应性改善,左向右分流增加。
[ { "id": 0, "entity": "肺血管", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "右心室", "start_offset": 18, "end_offset": 21, "label": "bod" } ]
绝大多数的单纯房间隔缺损婴儿无临床症状,亦有出现心功能衰竭的报道,但此类患儿心导管检查除心房水平左向右分流外,多无其他异常发现,心力衰竭的发病机制尚不明了,且易伴发心外畸形、生长发育迟缓。
[ { "id": 0, "entity": "单纯房间隔缺损", "start_offset": 5, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "心功能衰竭", "start_offset": 24, "end_offset": 29, "label": "dis" }, { "id": 2, "entity": "心导管检查", "start_offset": 38, "end_offset": 43, "label": "pro" }, { "id": 3, "entity": "心房", "start_offset": 44, "end_offset": 46, "label": "bod" }, { "id": 4, "entity": "心力衰竭", "start_offset": 64, "end_offset": 68, "label": "dis" }, { "id": 5, "entity": "心外畸形", "start_offset": 82, "end_offset": 86, "label": "dis" }, { "id": 6, "entity": "生长发育迟缓", "start_offset": 87, "end_offset": 93, "label": "dis" } ]
后者即使在房隔缺损关闭后亦不改善。
[ { "id": 0, "entity": "房隔缺损", "start_offset": 5, "end_offset": 9, "label": "dis" } ]
通常情况下,患儿肺动脉血流量较正常高3~4倍,而肺动脉压力仅轻度升高,肺血管阻力维持正常范围。
[ { "id": 0, "entity": "肺动脉", "start_offset": 8, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "肺动脉", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 2, "entity": "肺血管", "start_offset": 35, "end_offset": 38, "label": "bod" } ]
但亦有在出生后3个月即发现有肺动脉阻塞性疾病的报道。
[ { "id": 0, "entity": "肺动脉阻塞性疾病", "start_offset": 14, "end_offset": 22, "label": "dis" } ]
房间隔缺损伴有由肺动脉阻塞性疾病所致的严重青紫少见。
[ { "id": 0, "entity": "房间隔缺损", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "肺动脉阻塞性疾病", "start_offset": 8, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "严重青紫", "start_offset": 19, "end_offset": 23, "label": "sym" } ]
继发孔型房间隔缺损患儿出现青紫的另一种原因是较大的冠状窦静脉瓣、瓯氏瓣或塞氏瓣(Thebesianvalve)直接将血流从下腔静脉导入房间隔缺损。
[ { "id": 0, "entity": "继发孔型房间隔缺损", "start_offset": 0, "end_offset": 9, "label": "dis" }, { "id": 1, "entity": "青紫", "start_offset": 13, "end_offset": 15, "label": "sym" }, { "id": 2, "entity": "冠状窦静脉瓣", "start_offset": 25, "end_offset": 31, "label": "bod" }, { "id": 3, "entity": "瓯氏瓣", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "塞氏瓣", "start_offset": 36, "end_offset": 39, "label": "bod" }, { "id": 5, "entity": "Thebesianvalve", "start_offset": 40, "end_offset": 54, "label": "bod" }, { "id": 6, "entity": "血流", "start_offset": 58, "end_offset": 60, "label": "bod" }, { "id": 7, "entity": "下腔静脉", "start_offset": 61, "end_offset": 65, "label": "bod" }, { "id": 8, "entity": "房间隔缺损", "start_offset": 67, "end_offset": 72, "label": "bod" } ]
此时,必须手术关闭房间隔缺损。
[ { "id": 0, "entity": "手术关闭房间隔缺损", "start_offset": 5, "end_offset": 14, "label": "pro" } ]
【临床表现】多数房间隔缺损婴儿因无症状而被忽略,少数可有生长发育迟缓、反复上呼吸道感染甚至心衰。
[ { "id": 0, "entity": "房间隔缺损", "start_offset": 8, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "生长发育迟缓", "start_offset": 28, "end_offset": 34, "label": "sym" }, { "id": 2, "entity": "反复上呼吸道感染", "start_offset": 35, "end_offset": 43, "label": "sym" }, { "id": 3, "entity": "心衰", "start_offset": 45, "end_offset": 47, "label": "sym" } ]
只有大分流量的患儿才出现明显的气促和乏力并随年龄的增长逐年加重。
[ { "id": 0, "entity": "明显的气促", "start_offset": 12, "end_offset": 17, "label": "sym" }, { "id": 1, "entity": "乏力", "start_offset": 18, "end_offset": 20, "label": "sym" } ]
体格检查可见心前区隆起心房水平左向右分流明显时可见心尖搏动明显。
[ { "id": 0, "entity": "体格检查", "start_offset": 0, "end_offset": 4, "label": "ite" }, { "id": 1, "entity": "心前区", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "心前区隆起", "start_offset": 6, "end_offset": 11, "label": "sym" }, { "id": 3, "entity": "心房", "start_offset": 11, "end_offset": 13, "label": "bod" } ]
听诊可及三种特征:①典型的第二音固定分裂;②在左侧胸骨旁第二肋间可及柔和的收缩期杂音在左侧胸骨旁下缘可及早-中期舒张期杂音。
[ { "id": 0, "entity": "听诊", "start_offset": 0, "end_offset": 2, "label": "pro" }, { "id": 1, "entity": "典型的第二音固定分裂", "start_offset": 10, "end_offset": 20, "label": "sym" }, { "id": 2, "entity": "左侧胸骨旁第二肋间", "start_offset": 23, "end_offset": 32, "label": "bod" }, { "id": 3, "entity": "在左侧胸骨旁第二肋间可及柔和的收缩期杂音", "start_offset": 22, "end_offset": 42, "label": "sym" }, { "id": 4, "entity": "左侧胸骨", "start_offset": 43, "end_offset": 47, "label": "bod" }, { "id": 5, "entity": "在左侧胸骨旁下缘可及早-中期舒张期杂音", "start_offset": 42, "end_offset": 61, "label": "sym" } ]
第二心音分裂的原因与以下两个原因有关:①由于在房缺时右心室收缩期搏出血量增多而使肺动脉瓣第二音出现延迟;②由于肺动脉明显扩张,造成肺动脉关闭的动脉内张力上升延迟,而使肺动脉瓣关闭滞后。
[ { "id": 0, "entity": "第二心音分裂", "start_offset": 0, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "房缺", "start_offset": 23, "end_offset": 25, "label": "dis" }, { "id": 2, "entity": "右心室", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "肺动脉瓣", "start_offset": 40, "end_offset": 44, "label": "bod" }, { "id": 4, "entity": "肺动脉", "start_offset": 55, "end_offset": 58, "label": "bod" }, { "id": 5, "entity": "肺动脉", "start_offset": 65, "end_offset": 68, "label": "bod" }, { "id": 6, "entity": "动脉", "start_offset": 71, "end_offset": 73, "label": "bod" }, { "id": 7, "entity": "肺动脉瓣", "start_offset": 83, "end_offset": 87, "label": "bod" } ]
由于通过肺动脉瓣的血流量明显增加,在左侧胸骨旁上缘可及喷射性收缩期杂音,并向肺部传导。
[ { "id": 0, "entity": "肺动脉瓣", "start_offset": 4, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "左侧胸骨旁上缘", "start_offset": 18, "end_offset": 25, "label": "bod" }, { "id": 2, "entity": "喷射性收缩期杂音", "start_offset": 27, "end_offset": 35, "label": "sym" }, { "id": 3, "entity": "肺部", "start_offset": 38, "end_offset": 40, "label": "bod" } ]
心房水平左向右分流使舒张期通过三尖瓣的血流量增加,造成三尖瓣区舒张早中期杂音。
[ { "id": 0, "entity": "心房", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "三尖瓣", "start_offset": 15, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "三尖瓣", "start_offset": 27, "end_offset": 30, "label": "bod" } ]
【实验室检查】(一)心电图通常为正常窦性心律,在年长儿可有交界性心律和室上性心动过速。
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由于心房内及希氏束心室肌间传导延缓,年长儿可见PR间期延长,出现Ⅰ°房室传导阻滞。
[ { "id": 0, "entity": "心房", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "希氏束心室肌", "start_offset": 6, "end_offset": 12, "label": "bod" } ]
近半数患者可有P波改变,几乎所有的病例存在不同程度的V<sub>1</sub>导联rsR'或RSR'的不完全性右束支传导阻滞的表现,并伴有右心室大胸部X线心脏通常扩大,心胸比例>0.5,肺血管影随着年龄增长及左向右分流量的增加而增加。
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当出现肺血管梗阻性疾病时,主肺动脉明显扩大而外周肺野血管影稀少。
[ { "id": 0, "entity": "肺血管梗阻性疾病", "start_offset": 3, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "主肺动脉", "start_offset": 13, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "外周肺野血管影", "start_offset": 22, "end_offset": 29, "label": "pro" } ]
(三)超声心动图1.二维超声心动图(1)直接征象:①在心尖四腔切面时因为超声束与房间隔几乎平行易产生回声失落现象。
[ { "id": 0, "entity": "超声心动图", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "二维超声心动图", "start_offset": 10, "end_offset": 17, "label": "pro" } ]
剑下两腔切面、四腔切面为最佳切面,因房间隔束与房间隔几乎垂直,再结合胸骨旁四腔切面及大动脉短轴切面帮助检出,且要多个切面结合起来诊断。
[ { "id": 0, "entity": "房间隔", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 1, "entity": "房间隔", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 2, "entity": "胸骨旁四腔", "start_offset": 34, "end_offset": 39, "label": "bod" }, { "id": 3, "entity": "大动脉短轴", "start_offset": 42, "end_offset": 47, "label": "bod" } ]
房间隔缺损的游离端呈球状增厚,形如火柴头,又称“T”字征,以此特征明确缺损的位置、大小及数目比较可靠。
[ { "id": 0, "entity": "房间隔缺损", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "缺损", "start_offset": 35, "end_offset": 37, "label": "dis" } ]
②明确所有肺静脉与左房的关系,以排除肺静脉异位引流。
[ { "id": 0, "entity": "肺静脉", "start_offset": 5, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "左房", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "肺静脉异位引流", "start_offset": 18, "end_offset": 25, "label": "dis" } ]
(2)间接征象:右心房、右心室增大、肺动脉增宽、室间隔运动平坦或与左心室后壁呈同向运动2.脉冲多普勒超声将取样容积定位于分流的右心房侧,注意让血流方向与声束夹角尽可能小,一般可以得到舒张期1~3个正向波和1个收缩早期负向波,其最大流速一般在1.3m/s以下。
[ { "id": 0, "entity": "右心房", "start_offset": 8, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "右心室", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "右心房、右心室增大", "start_offset": 8, "end_offset": 17, "label": "sym" }, { "id": 3, "entity": "肺动脉", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 4, "entity": "肺动脉增宽", "start_offset": 18, "end_offset": 23, "label": "sym" }, { "id": 5, "entity": "室间隔", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 6, "entity": "左心室后壁", "start_offset": 33, "end_offset": 38, "label": "bod" }, { "id": 7, "entity": "室间隔运动平坦或与左心室后壁呈同向运动", "start_offset": 24, "end_offset": 43, "label": "sym" }, { "id": 8, "entity": "脉冲多普勒超声", "start_offset": 45, "end_offset": 52, "label": "pro" }, { "id": 9, "entity": "右心房", "start_offset": 63, "end_offset": 66, "label": "bod" } ]
三尖瓣流速增快,跨肺动脉血流流速加快,但一般很少超过2.5m/s,如超过要注意合并肺动脉瓣狭窄。
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3.彩色多普勒血流显像通常左心房压高于右心房,故能显示由左心房入右心房的穿隔血流束,血流位于房隔的中部,上部或多条分流束,以此判断缺损的类型,也可以估计流量的大小,缺损的大小。
[ { "id": 0, "entity": "彩色多普勒血流显像", "start_offset": 2, "end_offset": 11, "label": "pro" }, { "id": 1, "entity": "左心房", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "右心房", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "左心房", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 4, "entity": "右心房", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 5, "entity": "房隔", "start_offset": 46, "end_offset": 48, "label": "bod" }, { "id": 6, "entity": "缺损", "start_offset": 65, "end_offset": 67, "label": "dis" }, { "id": 7, "entity": "缺损", "start_offset": 82, "end_offset": 84, "label": "dis" } ]
注意分流程度并不完全取决于缺损的大小,重要的是取决于右心室的顺应性。
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值得注意的是左上腔残存的患者易与冠状静脉窦型房缺并存可结合彩色多普勒和临床其他检查以免漏诊。
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4.三维超声心动图二维超声只能从平面结构上显示房间隔缺损病变及分流束的方向与大小。
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需观察多个不同方位上二维切面图像来想象出房间隔缺损整体形态及其毗邻结构的立体解剖结构关系,这种想象通常十分困难且不准确。
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三维超声心动图则能以三维视角观察房间隔缺损的特征、空间位置及其与周围结构的空间关系,可从右心侧(L2a)或左心侧(L1a)直接观察缺损部位的整体形态、面积、大小及与上腔静脉、下腔静脉、冠状窦等的毗邻结构关系,还能观察二维超声心动图所不能显示的面积随心动周期对称收缩的动态变化特征,从而对房间隔缺损全面病理解剖诊断,进行正确的分型及准确测量缺损大小(图9-9)。
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早在1993年Belohlavek等就报道了三维超声对正常和异常房间隔能获良好显示,此后有关研究更加深入。
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Marx等的研究中,16例房缺病人中有13例进行了成功的动态三维重建,并能以三维视角观察缺损的特征、空间位置及其与周围结构的空间关系,如主动脉瓣与房间隔的关系,正常连接的肺静脉入口处等;Dall’Agata等对23例要外科修补的Ⅱ孔型房缺进行经胸和经食管动态三维重建,发现与手术的相关性高达0.90以上,还发现Ⅱ孔型房缺并非是单纯的两房之间的孔洞,从右房侧看,它存在于房间隔上一个形状相对独立的折叠区域内,也具有三维的结构。
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许多研究表明,三维超声可提供心脏解剖结构更为详细的空间活动信息,从而提高房间隔缺损的诊断正确性。
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图9-17三维超声心动图L2a剖视面,正对房间隔缺损观察,显示房间隔缺损大小、形态及与周围结构的关系(四)心导管及心血管造影通常对于继发孔型房间隔缺损的诊断,不必进行心导管检查。
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只有怀疑合并有肺动脉阻塞性疾病或其他并发畸形时才进行。
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心导管时,如果右心房的氧饱和度明显高于上、下腔静脉(>10%),应考虑有房间隔缺损的存在。
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