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一般在切口缝合前，可灌注透析液500～1000ml，观察液体出入是否通畅，以示透析管腹腔段位置良好。 | [
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在腹膜切口作荷包缝合，将腹透管周围的腹膜收紧，牢牢固定于透析管的深部涤纶套上，在观察局部无渗漏出后逐层关闭腹腔。 | [
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浅部涤纶套在皮下隧道内离出口处至少2cm，防止因牵拉或水肿解除后皮下涤纶套脱出导致皮下隧道感染。 | [
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临床观察结果显示流出口方向向上的病人更易患腹膜炎和流出口感染。 | [
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Sieniawskaet尝试在婴幼儿使用鹅颈导管并使导管流出口定位在胸前，据观察可降低婴幼儿导管相关感染的发生率。 | [
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在手术中应注意：①因儿童腹膜薄、脆、嫩，为降低腹膜透析液外漏应特别注意采用腹膜荷包缝合使深部涤纶套固定腹膜中，但切勿过分牵拉腹膜造成腹膜撕裂；②儿童大网膜相对较长，常常造成大网膜包裹腹透导管堵塞使管路扭曲异位，为避免其发生，有人主张在术中对大网膜较长者行部分切除。 | [
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在慢性肾衰竭时，腹透管置入术后不主张立即进行透析，最好在术后2～4周开始。 | [
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在此期间可用500～1000ml透析液冲洗腹腔后用肝素封闭透析管。 | [
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如病情需紧急透析，患儿应取作仰卧或半卧位，每次交换量15ml/kg为宜，这样可防止腹内压增加，有利于伤口组织愈合，避免发生透析液渗漏。 | [
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二、腺病毒肺炎腺病毒肺炎（adenoviralpneumonia）以腺病毒3型和7型为主。 | [
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起病大多急骤，先有上呼吸道感染症状，随后出现持续高热，咳嗽出现早，呈单声咳、频咳或阵咳，继而出现呼吸困难。 | [
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肺部体征出现迟，多在高热3～4天后出现湿啰音。 | [
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早期可出现中毒症状和多系统受累表现，如肝、脾肿大、嗜睡或烦躁不安，甚至中毒性脑病。 | [
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外周血白细胞计数大多轻度减少。 | [
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X线改变以肺实变阴影及病灶融合为特点，其范围不受肺叶的限制。 | [
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约1/6的病例可有胸膜炎。 | [
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根据上述临床表现，结合X线特点，诊断不难。 | [
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根据血清学和病毒学检查结果可确诊。 | [
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十、恶性神经鞘瘤恶性神经鞘瘤（malignantschwannoma）又称神经纤维肉瘤，占全部小儿软组织肉瘤的3%。 | [
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起源于周围神经或从神经纤维瘤病转化而来。 | [
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典型病变是在一较大的神经行径中，有一棱形肿块，脊神经的远、近端均匀变粗，表明肿瘤向神经外膜扩展。 | [
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肿瘤一般大于5cm，表面呈多肉状，可有出血或坏死区。 | [
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光镜下与纤维肉瘤相似，但本瘤的细胞有不规则的轮廓，细胞核呈波形、弯形或逗号形。 | [
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临床表现：30%～43%的儿童恶性神经鞘瘤来自神经纤维瘤病，而成人50%来自主要神经干。 | [
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对肿块突然增大或疼痛者，因其可能转为恶性，故要尽早做活检。 | [
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继发于神经纤维瘤病的患者，恶性程度更高。 | [
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由于肿瘤与主要神经干有关，一般局部完全切除肿瘤，还要截肢，不必做淋巴结清扫。 | [
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该肿瘤放疗无效，可按横纹肌肉瘤方案化疗，但效果不肯定。 | [
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第三节室间隔缺损室间隔缺损（ventricularseptaldefect，VSD）是最常见的先天性心血管畸形，可占先心病人的20%。 | [
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【病理解剖】在所有室间隔缺损的分类方法中，Soto等提出的分类法更有利于理解缺损的转归、累及的瓣膜和类似房室间通道的缺口大小。 | [
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从右心室面观察，根据缺损边界，可将室间隔缺损分为膜周部缺损、肌部缺损及双动脉下型缺损（图9-9）。 | [
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"id": 4,
"entity": "肌部缺损",
"start_offset": 30,
"end_offset": 34,
"label": "dis"
},
{
"id": 5,
"entity": "双动脉下型缺损",
"start_offset": 35,
"end_offset": 42,
"label": "dis"
}
] |
图9-18间隔缺损的部位（一）膜周部缺损占室间隔缺损的85%，缺损的边缘由纤维组织构成。 | [
{
"id": 0,
"entity": "间隔缺损",
"start_offset": 5,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "膜周部缺损",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 2,
"entity": "室间隔缺损",
"start_offset": 21,
"end_offset": 26,
"label": "dis"
},
{
"id": 3,
"entity": "缺损",
"start_offset": 31,
"end_offset": 33,
"label": "dis"
},
{
"id": 4,
"entity": "纤维组织",
"start_offset": 37,
"end_offset": 41,
"label": "bod"
}
] |
缺损可以存在于室间隔肌部、流入部或流出部。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "室间隔肌部",
"start_offset": 7,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "流入部",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 3,
"entity": "流出部",
"start_offset": 17,
"end_offset": 20,
"label": "bod"
}
] |
若缺损累及房室瓣叶与膜部室间隔之间的接合部，二尖瓣和三尖瓣间的纤维连接将会增强。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 1,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "房室瓣叶",
"start_offset": 5,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "膜部室间隔",
"start_offset": 10,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "二尖瓣",
"start_offset": 22,
"end_offset": 25,
"label": "bod"
},
{
"id": 4,
"entity": "三尖瓣",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
}
] |
正常情况下，流入部室间隔将右心室流入部和左心室流出部隔开，当此处的膜部室间隔缺损时，该处的间隔会变小，甚至出现左心室向右心房的分流。 | [
{
"id": 0,
"entity": "右心室流入部",
"start_offset": 13,
"end_offset": 19,
"label": "bod"
},
{
"id": 1,
"entity": "左心室流出部",
"start_offset": 20,
"end_offset": 26,
"label": "bod"
},
{
"id": 2,
"entity": "室间隔缺损",
"start_offset": 35,
"end_offset": 40,
"label": "dis"
},
{
"id": 3,
"entity": "左心室",
"start_offset": 55,
"end_offset": 58,
"label": "bod"
},
{
"id": 4,
"entity": "右心房",
"start_offset": 59,
"end_offset": 62,
"label": "bod"
}
] |
缺损部位可部分或全部被三尖瓣纤维组织覆盖，形成“假性室隔瘤”；主动脉瓣脱垂（aorticvalveprolapse）也会盖于缺损的室间隔上，使心室间的分流量减少。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "三尖瓣纤维组织",
"start_offset": 11,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "假性室隔瘤",
"start_offset": 24,
"end_offset": 29,
"label": "dis"
},
{
"id": 3,
"entity": "主动脉瓣脱垂",
"start_offset": 31,
"end_offset": 37,
"label": "dis"
},
{
"id": 4,
"entity": "aorticvalveprolapse",
"start_offset": 38,
"end_offset": 57,
"label": "dis"
},
{
"id": 5,
"entity": "缺损",
"start_offset": 62,
"end_offset": 64,
"label": "dis"
},
{
"id": 6,
"entity": "室间隔",
"start_offset": 65,
"end_offset": 68,
"label": "bod"
},
{
"id": 7,
"entity": "心室",
"start_offset": 71,
"end_offset": 73,
"label": "bod"
}
] |
（二）肌部缺损肌部缺损约占所有室间隔缺损的10%，边界全由肌性组织组成。 | [
{
"id": 0,
"entity": "肌部缺损",
"start_offset": 3,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肌部缺损",
"start_offset": 7,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "室间隔缺损",
"start_offset": 15,
"end_offset": 20,
"label": "dis"
},
{
"id": 3,
"entity": "肌性组织",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
}
] |
缺损可位于心尖部、流入道或流出道的肌部室间隔。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "心尖部",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "流入道",
"start_offset": 9,
"end_offset": 12,
"label": "bod"
},
{
"id": 3,
"entity": "流出道",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 4,
"entity": "肌部室间隔",
"start_offset": 17,
"end_offset": 22,
"label": "bod"
}
] |
它可以呈多发小孔，亦可伴有膜周部或双动脉下缺损。 | [
{
"id": 0,
"entity": "膜周部或双动脉下缺损",
"start_offset": 13,
"end_offset": 23,
"label": "dis"
}
] |
多发小孔的肌部缺损存在于心尖室间隔肌小梁之间，产生“Swiss-cheese”现象，它们可随年龄或肌小梁的肥厚而自行闭合；位于流出道部的肌部缺损也可随周围心肌的生长而自然闭合，此处分流量可为脱垂的主动脉瓣覆盖而减少；开口于流入道的肌部缺损可被三尖瓣瓣叶覆盖。 | [
{
"id": 0,
"entity": "肌部缺损",
"start_offset": 5,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "心尖室间隔肌小梁",
"start_offset": 12,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "肌小梁",
"start_offset": 49,
"end_offset": 52,
"label": "bod"
},
{
"id": 3,
"entity": "流出道部",
"start_offset": 63,
"end_offset": 67,
"label": "bod"
},
{
"id": 4,
"entity": "肌部缺损",
"start_offset": 68,
"end_offset": 72,
"label": "dis"
},
{
"id": 5,
"entity": "心肌",
"start_offset": 77,
"end_offset": 79,
"label": "bod"
},
{
"id": 6,
"entity": "主动脉瓣",
"start_offset": 98,
"end_offset": 102,
"label": "bod"
},
{
"id": 7,
"entity": "肌部缺损",
"start_offset": 115,
"end_offset": 119,
"label": "dis"
},
{
"id": 8,
"entity": "三尖瓣瓣叶",
"start_offset": 121,
"end_offset": 126,
"label": "bod"
}
] |
这种类型缺损与膜周部缺损不同，其传导束位于缺损的前上方。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 4,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "膜周部缺损",
"start_offset": 7,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "缺损",
"start_offset": 21,
"end_offset": 23,
"label": "dis"
}
] |
（三）双动脉下型缺损此型在西方国家较少见，只占室缺的5%主动脉瓣在东方人中则有30%。 | [
{
"id": 0,
"entity": "双动脉下型缺损",
"start_offset": 3,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "室缺",
"start_offset": 23,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "主动脉瓣",
"start_offset": 28,
"end_offset": 32,
"label": "bod"
}
] |
其主要特征是在主动脉瓣和肺动脉瓣之间有纤维连接。 | [
{
"id": 0,
"entity": "主动脉瓣",
"start_offset": 7,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "肺动脉瓣",
"start_offset": 12,
"end_offset": 16,
"label": "bod"
}
] |
冠脉瓣脱垂可减少左向右分流，但却常引起主动脉反流。 | [
{
"id": 0,
"entity": "冠脉瓣脱垂",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "主动脉反流",
"start_offset": 19,
"end_offset": 24,
"label": "sym"
}
] |
此类型的传导束由缺损部位间接发出。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 8,
"end_offset": 10,
"label": "dis"
}
] |
【病理生理】室间隔缺损引起心脏左向右分流，其分流程度取决于缺损大小及肺循环阻力。 | [
{
"id": 0,
"entity": "室间隔缺损",
"start_offset": 6,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "心脏",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "缺损",
"start_offset": 29,
"end_offset": 31,
"label": "dis"
},
{
"id": 3,
"entity": "肺",
"start_offset": 34,
"end_offset": 35,
"label": "bod"
}
] |
出生早期因肺静脉阻力高，分流量小；而后肺小血管肌层逐渐舒张，肺血管阻力下降，分流量遂增多。 | [
{
"id": 0,
"entity": "肺静脉",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "肺小血管肌层",
"start_offset": 19,
"end_offset": 25,
"label": "bod"
},
{
"id": 2,
"entity": "肺血管",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
}
] |
大型缺损，因要避免肺血流过多，肺小血管收缩，这一过程往往延迟。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "肺",
"start_offset": 9,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "肺小血管",
"start_offset": 15,
"end_offset": 19,
"label": "bod"
}
] |
若肺静脉回流血增多，会使左心房、左心室负荷增加，心脏容量超负荷及继发性肺高压可最终导致充血性心力衰竭产生。 | [
{
"id": 0,
"entity": "肺静脉",
"start_offset": 1,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "左心房",
"start_offset": 12,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "左心室",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
},
{
"id": 3,
"entity": "心脏",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
},
{
"id": 4,
"entity": "继发性肺高压",
"start_offset": 32,
"end_offset": 38,
"label": "dis"
},
{
"id": 5,
"entity": "充血性心力衰竭",
"start_offset": 43,
"end_offset": 50,
"label": "dis"
}
] |
这种代偿机制包括Frank-Starling机制、交感兴奋及心肌肥厚。 | [
{
"id": 0,
"entity": "交感兴奋",
"start_offset": 25,
"end_offset": 29,
"label": "sym"
},
{
"id": 1,
"entity": "心肌肥厚",
"start_offset": 30,
"end_offset": 34,
"label": "dis"
}
] |
肺动脉血流过多引起肺血管肌层肥厚，内膜增生，可导致肺小动脉结构破坏，产生不可逆的肺血管疾病，此时左向右的分流量可减少。 | [
{
"id": 0,
"entity": "肺动脉",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "肺血管肌层肥厚",
"start_offset": 9,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "内膜增生",
"start_offset": 17,
"end_offset": 21,
"label": "sym"
},
{
"id": 3,
"entity": "肺小动脉",
"start_offset": 25,
"end_offset": 29,
"label": "bod"
},
{
"id": 4,
"entity": "肺血管疾病",
"start_offset": 40,
"end_offset": 45,
"label": "dis"
}
] |
当肺血管破坏进一步发展，肺循环阻力进一步增高，右心室压力明显增加，大于左心室内压力，可以出现右向左分流，体循环缺氧；极少情况下，小儿出生后未有肺小血管平滑肌舒张，肺循环阻力高，左右心室压力相近，存在双向分流而没有充血性心衰的症状和体征。 | [
{
"id": 0,
"entity": "肺血管",
"start_offset": 1,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "肺",
"start_offset": 12,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "右心室",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
},
{
"id": 3,
"entity": "左心室",
"start_offset": 35,
"end_offset": 38,
"label": "bod"
},
{
"id": 4,
"entity": "肺小血管平滑肌",
"start_offset": 71,
"end_offset": 78,
"label": "bod"
},
{
"id": 5,
"entity": "左右心室",
"start_offset": 88,
"end_offset": 92,
"label": "bod"
},
{
"id": 6,
"entity": "充血性心衰",
"start_offset": 106,
"end_offset": 111,
"label": "dis"
}
] |
这两种情况，与Eisenmenger综合征晚期无多大区别。 | [
{
"id": 0,
"entity": "Eisenmenger综合征",
"start_offset": 7,
"end_offset": 21,
"label": "dis"
}
] |
除了肺血管疾病以外，其他导致左向右分流量减少的因素有：①右室圆锥部进行性肥厚造成狭窄，右心室流出道梗阻，临床上出现类似法洛四联症表现，而室缺本身症状被掩盖；②缺口由“瘤突”纤维或脱垂的主动脉瓣覆盖，而动脉下缺损常由脱垂的冠脉瓣覆盖，引起分流量的减少；③缺损可能自然缩小或完全关闭。 | [
{
"id": 0,
"entity": "肺血管疾病",
"start_offset": 2,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "右室圆锥部进行性肥厚",
"start_offset": 28,
"end_offset": 38,
"label": "dis"
},
{
"id": 2,
"entity": "右心室流出道梗阻",
"start_offset": 43,
"end_offset": 51,
"label": "dis"
},
{
"id": 3,
"entity": "法洛四联症",
"start_offset": 59,
"end_offset": 64,
"label": "dis"
},
{
"id": 4,
"entity": "室缺",
"start_offset": 68,
"end_offset": 70,
"label": "dis"
},
{
"id": 5,
"entity": "瘤突",
"start_offset": 83,
"end_offset": 85,
"label": "dis"
},
{
"id": 6,
"entity": "主动脉瓣",
"start_offset": 92,
"end_offset": 96,
"label": "bod"
},
{
"id": 7,
"entity": "动脉",
"start_offset": 100,
"end_offset": 102,
"label": "bod"
},
{
"id": 8,
"entity": "缺损",
"start_offset": 103,
"end_offset": 105,
"label": "dis"
},
{
"id": 9,
"entity": "冠脉瓣",
"start_offset": 110,
"end_offset": 113,
"label": "bod"
},
{
"id": 10,
"entity": "缺损",
"start_offset": 126,
"end_offset": 128,
"label": "dis"
}
] |
【临床表现】（一）小型缺损患儿无症状，通常是在体格检查时意外发现心脏杂音。 | [
{
"id": 0,
"entity": "小型缺损",
"start_offset": 9,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "体格检查",
"start_offset": 23,
"end_offset": 27,
"label": "pro"
},
{
"id": 2,
"entity": "心脏",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
}
] |
胸壁无畸形，左心室大小正常，外周血管搏动无异常。 | [
{
"id": 0,
"entity": "左心室",
"start_offset": 6,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "外周血管",
"start_offset": 14,
"end_offset": 18,
"label": "bod"
}
] |
主要体征为：胸骨左下缘有一响亮的收缩期杂音，常伴有震颤，杂音多为全收缩期；系动脉下缺损身体缩期杂音，常伴有震颤，杂音多为全收缩期；如系动脉下缺损，杂音和震颤则局限于胸骨左上缘。 | [
{
"id": 0,
"entity": "胸骨左下缘",
"start_offset": 6,
"end_offset": 11,
"label": "bod"
},
{
"id": 1,
"entity": "胸骨左下缘有一响亮的收缩期杂音，常伴有震颤，杂音多为全收缩期",
"start_offset": 6,
"end_offset": 36,
"label": "sym"
},
{
"id": 2,
"entity": "胸骨左上缘",
"start_offset": 82,
"end_offset": 87,
"label": "bod"
}
] |
对于小的肌部缺损，杂音特征为胸骨左下缘短促高亢的收缩期杂音，由于心肌收缩时肌小梁间的孔洞缩小或密闭，杂音于收缩中期终止。 | [
{
"id": 0,
"entity": "肌部缺损",
"start_offset": 4,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "胸骨左下缘",
"start_offset": 14,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "心肌",
"start_offset": 32,
"end_offset": 34,
"label": "bod"
},
{
"id": 3,
"entity": "肌小梁",
"start_offset": 37,
"end_offset": 40,
"label": "bod"
}
] |
心脏杂音的强弱与室间隔缺损的大小无直接关系。 | [
{
"id": 0,
"entity": "心脏",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "室间隔缺损",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
}
] |
（二）中型至大型缺损患儿常在生后1～2个月肺循环阻力下降时出现临床表现。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 8,
"end_offset": 10,
"label": "dis"
}
] |
由于肺循环流量大产生肺水肿，肺静脉压力增高，肺顺应性下降，出现吮乳困难，喂养时易疲劳、大量出汗，体重减轻，后渐出现身高发育延迟，呼吸急促，易反复呼吸道感染，进一步加剧心力衰竭形成。 | [
{
"id": 0,
"entity": "肺水肿",
"start_offset": 10,
"end_offset": 13,
"label": "sym"
},
{
"id": 1,
"entity": "肺静脉压力增高",
"start_offset": 14,
"end_offset": 21,
"label": "sym"
},
{
"id": 2,
"entity": "肺顺应性下降",
"start_offset": 22,
"end_offset": 28,
"label": "sym"
},
{
"id": 3,
"entity": "吮乳困难",
"start_offset": 31,
"end_offset": 35,
"label": "sym"
},
{
"id": 4,
"entity": "喂养时易疲劳",
"start_offset": 36,
"end_offset": 42,
"label": "sym"
},
{
"id": 5,
"entity": "大量出汗",
"start_offset": 43,
"end_offset": 47,
"label": "sym"
},
{
"id": 6,
"entity": "体重减轻",
"start_offset": 48,
"end_offset": 52,
"label": "sym"
},
{
"id": 7,
"entity": "后渐出现身高发育延迟",
"start_offset": 53,
"end_offset": 63,
"label": "sym"
},
{
"id": 8,
"entity": "呼吸急促",
"start_offset": 64,
"end_offset": 68,
"label": "sym"
},
{
"id": 9,
"entity": "易反复呼吸道感染",
"start_offset": 69,
"end_offset": 77,
"label": "sym"
},
{
"id": 10,
"entity": "进一步加剧心力衰竭形成",
"start_offset": 78,
"end_offset": 89,
"label": "sym"
}
] |
体格检查：小儿面色红润，反应稍差，脉率增快强弱正常，但当有严重心力衰竭或有很大的左向右分流时，脉搏减弱。 | [
{
"id": 0,
"entity": "体格检查",
"start_offset": 0,
"end_offset": 4,
"label": "ite"
},
{
"id": 1,
"entity": "脉率",
"start_offset": 17,
"end_offset": 19,
"label": "ite"
},
{
"id": 2,
"entity": "严重心力衰竭",
"start_offset": 29,
"end_offset": 35,
"label": "dis"
},
{
"id": 3,
"entity": "脉搏",
"start_offset": 47,
"end_offset": 49,
"label": "ite"
}
] |
患儿呼吸困难出现呼吸急促、肋间隙内陷。 | [
{
"id": 0,
"entity": "呼吸急促",
"start_offset": 8,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "肋间隙内陷",
"start_offset": 13,
"end_offset": 18,
"label": "dis"
}
] |
触诊，心尖搏动外移，有左心室抬举感，胸骨左下缘常可触及收缩期震颤。 | [
{
"id": 0,
"entity": "触诊",
"start_offset": 0,
"end_offset": 2,
"label": "pro"
},
{
"id": 1,
"entity": "心尖搏动外移",
"start_offset": 3,
"end_offset": 9,
"label": "sym"
},
{
"id": 2,
"entity": "左心室",
"start_offset": 11,
"end_offset": 14,
"label": "bod"
},
{
"id": 3,
"entity": "胸骨左下缘",
"start_offset": 18,
"end_offset": 23,
"label": "bod"
}
] |
听诊第二心音响亮，如有肺高压时，胸骨左下缘可闻及典型的全收缩期杂音。 | [
{
"id": 0,
"entity": "听诊",
"start_offset": 0,
"end_offset": 2,
"label": "pro"
},
{
"id": 1,
"entity": "第二心音响亮",
"start_offset": 2,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "肺高压",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
},
{
"id": 3,
"entity": "胸骨左下缘",
"start_offset": 16,
"end_offset": 21,
"label": "bod"
}
] |
如系动脉下缺损型，杂音通常以胸骨左缘第二肋间隙最为明显，当有大的左向右分流时，在心尖部可闻及第三心音及舒张中期隆隆样杂音。 | [
{
"id": 0,
"entity": "系动脉下缺损型",
"start_offset": 1,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "胸骨左缘第二肋间隙",
"start_offset": 14,
"end_offset": 23,
"label": "bod"
},
{
"id": 2,
"entity": "心尖部",
"start_offset": 40,
"end_offset": 43,
"label": "bod"
}
] |
与之相比，当小儿长至6月～2岁，心力衰竭比例反而可以下降。 | [
{
"id": 0,
"entity": "心力衰竭",
"start_offset": 16,
"end_offset": 20,
"label": "dis"
}
] |
这可能由于缺损自然闭合、瓣膜纤维组织及脱垂的瓣叶覆盖缺口、右室圆锥部狭窄或肺循环阻力增高使左向右分流减少的缘故。 | [
{
"id": 0,
"entity": "缺损",
"start_offset": 5,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "瓣膜纤维组织",
"start_offset": 12,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "脱垂的瓣叶",
"start_offset": 19,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "右室圆锥部狭窄",
"start_offset": 29,
"end_offset": 36,
"label": "bod"
}
] |
随着肺血管压力增高，分流量的减少，心前区搏动逐渐减弱而仅出现严重的肺高压表现：第二心音亢进、单一，收缩期杂音短促最终消失。 | [
{
"id": 0,
"entity": "肺血管",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "肺高压",
"start_offset": 33,
"end_offset": 36,
"label": "sym"
}
] |
若有肺动脉反流，在胸骨左缘尚可闻及舒张期杂音；如出现三尖瓣相对关闭不全，有严重三尖瓣反流，则于胸骨左下缘可及全收缩期杂音。 | [
{
"id": 0,
"entity": "肺动脉",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "胸骨左缘",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "三尖瓣",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
},
{
"id": 3,
"entity": "三尖瓣反流",
"start_offset": 39,
"end_offset": 44,
"label": "dis"
},
{
"id": 4,
"entity": "胸骨左下缘",
"start_offset": 47,
"end_offset": 52,
"label": "bod"
}
] |
在十几岁的小儿中，更常见因出现右向左分流而引起的发绀。 | [
{
"id": 0,
"entity": "发绀",
"start_offset": 24,
"end_offset": 26,
"label": "dis"
}
] |
少数患儿，出生后肺循环压力未降，其主要表现为肺动脉高压，而心力衰竭症状不明显。 | [
{
"id": 0,
"entity": "肺动脉高压",
"start_offset": 22,
"end_offset": 27,
"label": "dis"
},
{
"id": 1,
"entity": "心力衰竭",
"start_offset": 29,
"end_offset": 33,
"label": "dis"
}
] |
当右室圆锥部进行性肥厚，右心室增大的体征可较左心室更明显。 | [
{
"id": 0,
"entity": "右室圆锥部",
"start_offset": 1,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "右心室增大",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "左心室",
"start_offset": 22,
"end_offset": 25,
"label": "bod"
}
] |
如出现右心室流出道梗阻时，第二心音变弱。 | [
{
"id": 0,
"entity": "右心室流出道梗阻",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
}
] |
若狭窄进一步加重，左右心室收缩期压力平衡，全收缩期杂音减弱甚至消失，于胸骨左上缘可及响亮的收缩期喷射性杂音。 | [
{
"id": 0,
"entity": "左右心室",
"start_offset": 9,
"end_offset": 13,
"label": "bod"
},
{
"id": 1,
"entity": "全收缩期杂音减弱甚至消失",
"start_offset": 21,
"end_offset": 33,
"label": "sym"
}
] |
主动脉瓣脱垂可引起主动脉反流，因左心室舒张末期容量增加，可出现洪脉，心尖搏动外移及特征性的胸骨左缘高亢的舒张期吹风样杂音。 | [
{
"id": 0,
"entity": "主动脉瓣",
"start_offset": 0,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "左心室",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "洪脉",
"start_offset": 31,
"end_offset": 33,
"label": "dis"
},
{
"id": 3,
"entity": "心尖",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
}
] |
【辅助检查】（一）X线胸片小型室缺小儿X线胸片常完全正常。 | [
{
"id": 0,
"entity": "X线",
"start_offset": 9,
"end_offset": 11,
"label": "pro"
},
{
"id": 1,
"entity": "胸片",
"start_offset": 11,
"end_offset": 13,
"label": "pro"
},
{
"id": 2,
"entity": "小型室缺",
"start_offset": 13,
"end_offset": 17,
"label": "dis"
},
{
"id": 3,
"entity": "X线",
"start_offset": 19,
"end_offset": 21,
"label": "pro"
},
{
"id": 4,
"entity": "胸片",
"start_offset": 21,
"end_offset": 23,
"label": "pro"
}
] |
有大型缺损、分流量大、左心室超容的小儿，胸片表现为心影向左下扩大、左心房扩大、肺野淤血；如出现肺动脉高压，肺动脉干突出，右室肥厚，心尖上翘。 | [
{
"id": 0,
"entity": "左心室超容",
"start_offset": 11,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "胸片",
"start_offset": 20,
"end_offset": 22,
"label": "pro"
},
{
"id": 2,
"entity": "心影向左下扩大",
"start_offset": 25,
"end_offset": 32,
"label": "sym"
},
{
"id": 3,
"entity": "左心房",
"start_offset": 33,
"end_offset": 36,
"label": "bod"
},
{
"id": 4,
"entity": "左心房扩大",
"start_offset": 33,
"end_offset": 38,
"label": "sym"
},
{
"id": 5,
"entity": "肺野淤血",
"start_offset": 39,
"end_offset": 43,
"label": "sym"
},
{
"id": 6,
"entity": "肺动脉",
"start_offset": 47,
"end_offset": 50,
"label": "bod"
},
{
"id": 7,
"entity": "肺动脉高压",
"start_offset": 47,
"end_offset": 52,
"label": "sym"
},
{
"id": 8,
"entity": "肺动脉干",
"start_offset": 53,
"end_offset": 57,
"label": "bod"
},
{
"id": 9,
"entity": "右室",
"start_offset": 60,
"end_offset": 62,
"label": "bod"
},
{
"id": 10,
"entity": "右室肥厚",
"start_offset": 60,
"end_offset": 64,
"label": "sym"
},
{
"id": 11,
"entity": "心尖",
"start_offset": 65,
"end_offset": 67,
"label": "bod"
},
{
"id": 12,
"entity": "心尖上翘",
"start_offset": 65,
"end_offset": 69,
"label": "sym"
}
] |
若系双动脉下型缺损，由于大量快速分流的血流直接撞击肺动脉，肺动脉干也突出。 | [
{
"id": 0,
"entity": "双动脉下型缺损",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "肺动脉",
"start_offset": 25,
"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "肺动脉干",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
}
] |
肺血管疾病的特征表现为肺动脉干及其主支很粗，但周围血管影不粗甚至变细。 | [
{
"id": 0,
"entity": "肺血管疾病",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "肺动脉干",
"start_offset": 11,
"end_offset": 15,
"label": "bod"
}
] |
而因肺循环阻力很高，左向右分流量减少，所以心影可正常。 | [
{
"id": 0,
"entity": "心影",
"start_offset": 21,
"end_offset": 23,
"label": "pro"
}
] |
（二）心电图缺损小的儿童心电图类似完全正常。 | [
{
"id": 0,
"entity": "心电图",
"start_offset": 3,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "缺损",
"start_offset": 6,
"end_offset": 8,
"label": "dis"
},
{
"id": 2,
"entity": "心电图",
"start_offset": 12,
"end_offset": 15,
"label": "pro"
}
] |
大型缺损儿童可出现左室肥厚表现：Ⅱ、Ⅲ、aVF、V<sub>5</sub>、V<sub>6</sub>深Q波，R波高大，T波高尖；左心房大，P波变宽。 | [
{
"id": 0,
"entity": "左室",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 1,
"entity": "左室肥厚",
"start_offset": 9,
"end_offset": 13,
"label": "sym"
},
{
"id": 2,
"entity": "左心房",
"start_offset": 65,
"end_offset": 68,
"label": "bod"
},
{
"id": 3,
"entity": "Ⅱ、Ⅲ、aVF、V<sub>5</sub>、V<sub>6</sub>深Q波，R波高大，T波高尖；左心房大，P波变宽",
"start_offset": 16,
"end_offset": 74,
"label": "sym"
}
] |
流入部室间隔缺损可出现电轴左偏。 | [
{
"id": 0,
"entity": "室间隔缺损",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
}
] |
有肺高压、右心室增大时，V<sub>1</sub>呈rsR。 | [
{
"id": 0,
"entity": "肺高压",
"start_offset": 1,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "右心室增大",
"start_offset": 5,
"end_offset": 10,
"label": "dis"
}
] |
右心室压力增高时，右胸导联R波高电压、T波直立。 | [
{
"id": 0,
"entity": "右心室",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "右胸",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
}
] |
当有严重右心室流出道梗阻或肺血管病变时，心电图呈右心室占优势的图形。 | [
{
"id": 0,
"entity": "右心室流出道梗阻",
"start_offset": 4,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "肺血管病变",
"start_offset": 13,
"end_offset": 18,
"label": "dis"
},
{
"id": 2,
"entity": "心电图",
"start_offset": 20,
"end_offset": 23,
"label": "pro"
},
{
"id": 3,
"entity": "右心室",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
}
] |
（三）超声心动图二维超声可直接显示缺损的位置。 | [
{
"id": 0,
"entity": "超声心动图",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "二维超声",
"start_offset": 8,
"end_offset": 12,
"label": "pro"
},
{
"id": 2,
"entity": "缺损",
"start_offset": 17,
"end_offset": 19,
"label": "dis"
}
] |
流入道缺损可由心尖和肋下四腔位看到；稍向前移，所取的平面即可看到膜周部的缺损。 | [
{
"id": 0,
"entity": "流入道缺损",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "心尖",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "肋下四腔位",
"start_offset": 10,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "缺损",
"start_offset": 36,
"end_offset": 38,
"label": "dis"
}
] |
以这些平面，还可看到来源于三尖瓣瓣叶的“瘤突”。 | [
{
"id": 0,
"entity": "三尖瓣瓣叶",
"start_offset": 13,
"end_offset": 18,
"label": "bod"
},
{
"id": 1,
"entity": "瘤突",
"start_offset": 20,
"end_offset": 22,
"label": "dis"
}
] |
胸骨旁短轴平面时，若存在膜周部缺损和“瘤突”，则其位于10点位；漏斗部肥厚也可在此时看到。 | [
{
"id": 0,
"entity": "胸骨",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "缺损",
"start_offset": 15,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "瘤突",
"start_offset": 19,
"end_offset": 21,
"label": "dis"
},
{
"id": 3,
"entity": "漏斗部肥厚",
"start_offset": 32,
"end_offset": 37,
"label": "dis"
}
] |
另外，动脉下缺损于1点位可看到主肺动脉瓣联合部纤维。 | [
{
"id": 0,
"entity": "动脉下缺损",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "主肺动脉瓣联合部纤维",
"start_offset": 15,
"end_offset": 25,
"label": "bod"
}
] |
如有主动脉瓣脱垂，可通过胸骨旁长、短轴清楚看到（图9-9）。 | [
{
"id": 0,
"entity": "主动脉瓣脱垂",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "胸骨",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
}
] |
前肌部缺损可通过长轴探察。 | [
{
"id": 0,
"entity": "前肌部缺损",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
}
] |
心尖部的多发小孔可从心尖、剑突下、短轴到达二尖瓣、心尖等邻近部位观察。 | [
{
"id": 0,
"entity": "心尖部",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "心尖",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "剑突下",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 3,
"entity": "短轴",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 4,
"entity": "二尖瓣",
"start_offset": 21,
"end_offset": 24,
"label": "bod"
},
{
"id": 5,
"entity": "心尖",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
}
] |
彩色血流显像对上述缺损的定位更有帮助。 | [
{
"id": 0,
"entity": "彩色血流显像",
"start_offset": 0,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "缺损",
"start_offset": 9,
"end_offset": 11,
"label": "dis"
}
] |
图9-19经食管超声显示：贯穿动脉下室间隔缺损的右冠状动脉瓣脱垂（RCC）（箭头所示）LV：左心室RV：右心室通过无创的Doppler超声，运用Bernoulli校正公式可估计肺动脉压力。 | [
{
"id": 0,
"entity": "食管超声",
"start_offset": 6,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "贯穿动脉下室间隔缺损",
"start_offset": 13,
"end_offset": 23,
"label": "dis"
},
{
"id": 2,
"entity": "右冠状动脉瓣脱垂",
"start_offset": 24,
"end_offset": 32,
"label": "dis"
},
{
"id": 3,
"entity": "RCC",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
},
{
"id": 4,
"entity": "左心室",
"start_offset": 46,
"end_offset": 49,
"label": "bod"
},
{
"id": 5,
"entity": "右心室",
"start_offset": 52,
"end_offset": 55,
"label": "bod"
},
{
"id": 6,
"entity": "Doppler超声",
"start_offset": 60,
"end_offset": 69,
"label": "pro"
},
{
"id": 7,
"entity": "肺动脉压力",
"start_offset": 88,
"end_offset": 93,
"label": "ite"
}
] |
心室间的压力阶差可由通过缺损处血流速度推算。 | [
{
"id": 0,
"entity": "心室",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "缺损",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
}
] |
收缩期肺动脉压力通过测收缩期体循环压力及心室间压力阶差后计算得出（假设无右心室流出道梗阻）。 | [
{
"id": 0,
"entity": "肺动脉",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "收缩期体循环压力",
"start_offset": 11,
"end_offset": 19,
"label": "ite"
},
{
"id": 2,
"entity": "心室间压力",
"start_offset": 20,
"end_offset": 25,
"label": "ite"
},
{
"id": 3,
"entity": "右心室流出道梗阻",
"start_offset": 36,
"end_offset": 44,
"label": "dis"
}
] |
同样，左房、左室的方位为左向右分流的大小提供了足够的信息。 | [
{
"id": 0,
"entity": "左房",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "左室",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
}
] |
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