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（六）其他以下一些流速指标有助于我们了解小气道功能（图8-8）。 | [
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MEF75（FEF25）：75%肺活量时的呼气流速；MEF50（FEF50）：50%肺活量时的呼气流速；MEF25（FEF75）：75%肺活量时的呼气流速；及最大呼气中期流速（MMEF25～75）。 | [
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"entity": "MMEF25～75",
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随着肺活量的逐渐减少，MEF越能反映出小气道的情况。 | [
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参考文献1.BehrmanRE，etal.NelsonTextbookofPediatrics.16th</sup>ed，2000：15772.杨霁云，白克敏主编.小儿肾脏病临床与基础.北京：人民卫生出版社，2000：1983.林善锬主编.当代肾脏病学.上海：上海科技教育出版社，2001：313 | [
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第六节三尖瓣闭锁三尖瓣闭锁（tricuspidatresia）是一种不多见的心血管畸形，占所有先心病的1.3%～1.7%。 | [
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"entity": "心血管畸形",
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【病理解剖】右心房和右心室被闭锁的三尖瓣完全隔开，多为肌性闭锁，少数为纤维型。 | [
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"entity": "肌性闭锁",
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右心室常发育不良流出道组成，还包括发育幼稚的肌小梁成分。 | [
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"entity": "肌小梁",
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若室间隔完整，右心室发育极其不全甚至缺如，伴肺动脉瓣闭锁；反之，若存在大的室间隔缺损右心室则发育良好。 | [
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"entity": "右心室",
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本病心房间的交通必然存在，它是小儿存活必不可少的条件，通常属于非限制型，可以是卵圆孔未闭、继发性房间隔缺损或者是原发性房间隔缺损伴完全型房室隔缺损。 | [
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心室大动脉连接位置可以正常，亦可转位。 | [
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当存在大动脉转位心血管畸形左上腔静脉、水肿、动脉导管未闭肺动脉为非限制型，则可见到主动脉发育不良水肿。 | [
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"id": 10,
"entity": "水肿",
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其他伴随畸形尚有左右心耳并置永存动脉干、部分型房室间隔缺损。 | [
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肺血流量的多寡取决于肺动脉狭窄程度，从而产生相应的临床症状。 | [
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当动脉位置正常，室间隔缺损太小会引起肺血管梗阻大动脉转位梗阻常由于肺动脉下及肺动脉瓣狭窄造成。 | [
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"entity": "肺血管梗阻",
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"entity": "梗阻",
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若伴有肺动脉瓣闭锁肺血流量取决于动脉导管未闭和（或）主肺动脉间形成的侧支大小。 | [
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{
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"entity": "主肺动脉",
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【病理生理】本病心房间交通形成了血液被动性的右向左分流。 | [
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"entity": "血液",
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当房间隔缺损太小，血流受阻，右心房压力增高而使体静脉回血及肺血流量减少心搏出量减少青紫严重；若房间隔缺损大，其病理生理学改变主要依赖于肺血管流出道梗阻情况，即肺血流量的多少。 | [
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"entity": "心搏出量减少",
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{
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"entity": "青紫严重",
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"id": 8,
"entity": "房间隔",
"start_offset": 47,
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{
"id": 9,
"entity": "肺血管",
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"label": "bod"
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{
"id": 10,
"entity": "流出道",
"start_offset": 70,
"end_offset": 73,
"label": "bod"
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{
"id": 11,
"entity": "肺血流量",
"start_offset": 79,
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当肺动脉显著狭窄青紫，随着室间隔缺损不断变小、圆锥部渐进性变窄或动脉导管的变细，青紫可进一步加重，若肺血量极度减少时，可发生缺氧发作；相反，若肺血管流出道正常，肺血流量会增多，大多表现为心功能不全发绀不明显，这种患儿常伴有大动脉转位肺血管梗阻性疾病及肺高压。 | [
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"entity": "肺血流量",
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{
"id": 10,
"entity": "心",
"start_offset": 93,
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"id": 11,
"entity": "心功能不全",
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{
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"entity": "发绀不明显",
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"label": "sym"
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{
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"entity": "动脉",
"start_offset": 112,
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"label": "bod"
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{
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"entity": "大动脉转位",
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{
"id": 15,
"entity": "肺血管梗阻性疾病",
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{
"id": 16,
"entity": "肺高压",
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【临床表现】（一）肺血增多大动脉转位常会出现肺血流过多呼吸急促喂养困难、出汗多、活动量减少、反复肺部感染青紫较轻甚至无发绀。 | [
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{
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此类患儿在生后2～3个月可发展为充血性心力衰竭，主要表现为心脏扩大肝大呼吸困难听诊可及第三心音、舒张期隆隆样杂音，收缩期杂音多不明显。 | [
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"label": "sym"
},
{
"id": 7,
"entity": "听诊可及第三心音",
"start_offset": 39,
"end_offset": 47,
"label": "sym"
},
{
"id": 8,
"entity": "舒张期隆隆样杂音",
"start_offset": 48,
"end_offset": 56,
"label": "sym"
},
{
"id": 9,
"entity": "收缩期杂音多不明显",
"start_offset": 57,
"end_offset": 66,
"label": "sym"
}
] |
（二）肺血减少大多数患儿在新生儿期即可出现发绀或心脏杂音青紫最常见于中央型青紫，其严重程度与肺血管流出道梗阻程度有关，如肺血流进一步减少，则在婴儿时期就出现缺氧发作。 | [
{
"id": 0,
"entity": "肺血减少",
"start_offset": 3,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "心脏",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
},
{
"id": 2,
"entity": "发绀或心脏杂音",
"start_offset": 21,
"end_offset": 28,
"label": "sym"
},
{
"id": 3,
"entity": "青紫",
"start_offset": 28,
"end_offset": 30,
"label": "sym"
},
{
"id": 4,
"entity": "中央型青紫",
"start_offset": 34,
"end_offset": 39,
"label": "sym"
},
{
"id": 5,
"entity": "肺血管流出道梗阻",
"start_offset": 46,
"end_offset": 54,
"label": "sym"
},
{
"id": 6,
"entity": "肺",
"start_offset": 60,
"end_offset": 61,
"label": "bod"
},
{
"id": 7,
"entity": "缺氧",
"start_offset": 78,
"end_offset": 80,
"label": "sym"
}
] |
年长儿中可表现为呼吸急促活动后蹲踞。 | [
{
"id": 0,
"entity": "呼吸",
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"end_offset": 10,
"label": "ite"
},
{
"id": 1,
"entity": "呼吸急促",
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"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "活动后蹲踞",
"start_offset": 12,
"end_offset": 17,
"label": "sym"
}
] |
严重青紫及有红细胞增多症脑血管栓塞，尤其在铁质缺乏时更是如此。 | [
{
"id": 0,
"entity": "青紫",
"start_offset": 2,
"end_offset": 4,
"label": "sym"
},
{
"id": 1,
"entity": "红细胞",
"start_offset": 6,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "红细胞增多症",
"start_offset": 6,
"end_offset": 12,
"label": "sym"
},
{
"id": 3,
"entity": "脑血管栓塞",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
}
] |
体格检查：小儿出现中至重度中央型青紫，年长儿杵状指（趾）明显心前区隆起心尖搏动增强听诊第一心音单一肺动脉狭窄、肺动脉瓣闭锁或伴大动脉转位，则第二心音单一喀喇音偶可于胸骨左缘闻及肺动脉流出道狭窄或限制性室间隔缺损而产生的收缩期喷射性杂音。 | [
{
"id": 0,
"entity": "体格检查",
"start_offset": 0,
"end_offset": 4,
"label": "ite"
},
{
"id": 1,
"entity": "中至重度中央型青紫",
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"end_offset": 18,
"label": "sym"
},
{
"id": 2,
"entity": "趾",
"start_offset": 26,
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"label": "bod"
},
{
"id": 3,
"entity": "杵状指（趾）明显",
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"label": "sym"
},
{
"id": 4,
"entity": "心前区",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
},
{
"id": 5,
"entity": "心前区隆起",
"start_offset": 30,
"end_offset": 35,
"label": "sym"
},
{
"id": 6,
"entity": "心尖",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
},
{
"id": 7,
"entity": "搏动",
"start_offset": 37,
"end_offset": 39,
"label": "ite"
},
{
"id": 8,
"entity": "心尖搏动增强",
"start_offset": 35,
"end_offset": 41,
"label": "sym"
},
{
"id": 9,
"entity": "听诊",
"start_offset": 41,
"end_offset": 43,
"label": "ite"
},
{
"id": 10,
"entity": "听诊第一心音单一",
"start_offset": 41,
"end_offset": 49,
"label": "sym"
},
{
"id": 11,
"entity": "肺动脉狭窄",
"start_offset": 49,
"end_offset": 54,
"label": "dis"
},
{
"id": 12,
"entity": "肺动脉瓣闭锁",
"start_offset": 55,
"end_offset": 61,
"label": "dis"
},
{
"id": 13,
"entity": "大动脉转位",
"start_offset": 63,
"end_offset": 68,
"label": "dis"
},
{
"id": 14,
"entity": "心音",
"start_offset": 72,
"end_offset": 74,
"label": "ite"
},
{
"id": 15,
"entity": "第二心音单一",
"start_offset": 70,
"end_offset": 76,
"label": "sym"
},
{
"id": 16,
"entity": "胸骨",
"start_offset": 82,
"end_offset": 84,
"label": "bod"
},
{
"id": 17,
"entity": "喀喇音偶可于胸骨左缘闻及",
"start_offset": 76,
"end_offset": 88,
"label": "sym"
},
{
"id": 18,
"entity": "肺动脉流出道狭窄",
"start_offset": 88,
"end_offset": 96,
"label": "dis"
},
{
"id": 19,
"entity": "限制性室间隔缺损",
"start_offset": 97,
"end_offset": 105,
"label": "dis"
},
{
"id": 20,
"entity": "收缩期喷射性杂音",
"start_offset": 109,
"end_offset": 117,
"label": "sym"
}
] |
随着肺动脉流出道梗阻加重或室间隔缺损的缩小，杂音强度可逐渐降低。 | [
{
"id": 0,
"entity": "杂音强度可逐渐降低",
"start_offset": 22,
"end_offset": 31,
"label": "sym"
}
] |
如颈静脉搏动有明显“a”波、肝脏收缩前心房间存在限制性分流。 | [
{
"id": 0,
"entity": "肝脏",
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"end_offset": 16,
"label": "bod"
},
{
"id": 1,
"entity": "肝脏收缩",
"start_offset": 14,
"end_offset": 18,
"label": "sym"
},
{
"id": 2,
"entity": "心房",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
}
] |
【辅助检查】（一）胸部X线肺血流量少者，肺野肺血减少，肺动脉干凹陷心胸比值正常或轻度增大右房缘凸出明显肺血流量增加、有心力衰竭的小儿，其显著特征为心脏扩大、肺充血。 | [
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"id": 0,
"entity": "胸部X线",
"start_offset": 9,
"end_offset": 13,
"label": "pro"
},
{
"id": 1,
"entity": "肺血流量",
"start_offset": 13,
"end_offset": 17,
"label": "ite"
},
{
"id": 2,
"entity": "肺动脉干",
"start_offset": 27,
"end_offset": 31,
"label": "bod"
},
{
"id": 3,
"entity": "肺动脉干凹陷",
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"end_offset": 33,
"label": "sym"
},
{
"id": 4,
"entity": "心",
"start_offset": 33,
"end_offset": 34,
"label": "bod"
},
{
"id": 5,
"entity": "胸",
"start_offset": 34,
"end_offset": 35,
"label": "bod"
},
{
"id": 6,
"entity": "心胸比值正常或轻度增大",
"start_offset": 33,
"end_offset": 44,
"label": "sym"
},
{
"id": 7,
"entity": "右房缘",
"start_offset": 44,
"end_offset": 47,
"label": "bod"
},
{
"id": 8,
"entity": "右房缘凸出明显",
"start_offset": 44,
"end_offset": 51,
"label": "sym"
},
{
"id": 9,
"entity": "肺血流量",
"start_offset": 51,
"end_offset": 55,
"label": "ite"
},
{
"id": 10,
"entity": "心力衰竭",
"start_offset": 59,
"end_offset": 63,
"label": "dis"
},
{
"id": 11,
"entity": "肺",
"start_offset": 78,
"end_offset": 79,
"label": "bod"
},
{
"id": 12,
"entity": "肺充血",
"start_offset": 78,
"end_offset": 81,
"label": "sym"
}
] |
（二）心电图该病心电图特点是：QRS额面电轴偏左上，右心房肥大，右心室电势减小或消失（图9-9）。 | [
{
"id": 0,
"entity": "心电图",
"start_offset": 3,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "心电图",
"start_offset": 8,
"end_offset": 11,
"label": "pro"
},
{
"id": 2,
"entity": "右心房",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
},
{
"id": 3,
"entity": "右心室",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
}
] |
如有大型室间隔缺损，右心室发育较好，右心室电压可变得明显。 | [
{
"id": 0,
"entity": "室间隔缺损",
"start_offset": 4,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "右心室",
"start_offset": 10,
"end_offset": 13,
"label": "bod"
},
{
"id": 2,
"entity": "右心室",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
}
] |
当肺血流量多，左心室容量负荷增加，左胸导联示ST段压低，T波改变，并可见V6深Q波、R波高尖。 | [
{
"id": 0,
"entity": "肺血流量",
"start_offset": 1,
"end_offset": 5,
"label": "ite"
},
{
"id": 1,
"entity": "左心室",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
}
] |
图9-35三尖瓣闭锁患儿（2月）典型心电图显示：电轴左偏，右房肥大和右、心室低电压。 | [
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"id": 0,
"entity": "三尖瓣闭锁",
"start_offset": 5,
"end_offset": 10,
"label": "dis"
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{
"id": 1,
"entity": "心电图",
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"label": "pro"
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{
"id": 2,
"entity": "右房",
"start_offset": 29,
"end_offset": 31,
"label": "bod"
},
{
"id": 3,
"entity": "右、心室",
"start_offset": 34,
"end_offset": 38,
"label": "bod"
},
{
"id": 4,
"entity": "右房肥大和右、心室低电压",
"start_offset": 29,
"end_offset": 41,
"label": "sym"
}
] |
（三）超声心动图剑突下及心尖四腔位可清楚捕捉到三尖瓣缺如和右心室发育不良心房间交通的大小，结合Doppler显像估计左右心房间的压力阶差，从而了解心房间的分流程度。 | [
{
"id": 0,
"entity": "超声心动图",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "心尖四腔位",
"start_offset": 12,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "右心室",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 3,
"entity": "右心室发育不良",
"start_offset": 29,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "心房",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
},
{
"id": 5,
"entity": "Doppler显像",
"start_offset": 47,
"end_offset": 56,
"label": "pro"
},
{
"id": 6,
"entity": "心房",
"start_offset": 60,
"end_offset": 62,
"label": "bod"
},
{
"id": 7,
"entity": "心房",
"start_offset": 73,
"end_offset": 75,
"label": "bod"
}
] |
若房间隔向左房膨出则表明左右心房分流受限。 | [
{
"id": 0,
"entity": "房间隔",
"start_offset": 1,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "左房",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "心房",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
}
] |
二维超声心动图同时尚能看出室间隔缺损的大小、心室大动脉连接和肺动脉流出道梗阻的情况。 | [
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"id": 0,
"entity": "二维超声心动图",
"start_offset": 0,
"end_offset": 7,
"label": "pro"
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{
"id": 1,
"entity": "室间隔缺损",
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"label": "dis"
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{
"id": 2,
"entity": "心室",
"start_offset": 22,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "动脉",
"start_offset": 25,
"end_offset": 27,
"label": "bod"
}
] |
脉冲及连续Doppler还能明确肺动脉流出道收缩期压力阶差。 | [
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"id": 0,
"entity": "脉冲",
"start_offset": 0,
"end_offset": 2,
"label": "pro"
},
{
"id": 1,
"entity": "连续Doppler",
"start_offset": 3,
"end_offset": 12,
"label": "pro"
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{
"id": 2,
"entity": "肺动脉流出道收缩期",
"start_offset": 16,
"end_offset": 25,
"label": "dis"
}
] |
（四）心导管三尖瓣闭锁通常可通过超声心动图明确诊断，而在行Fontan手术之前，诊断性心导管术及心导管造影则有利于显示肺动脉解剖结构、肺动脉压力和阻力、左心室功能、二尖瓣活动情况和体肺静脉形态。 | [
{
"id": 0,
"entity": "心导管",
"start_offset": 3,
"end_offset": 6,
"label": "equ"
},
{
"id": 1,
"entity": "三尖瓣闭锁",
"start_offset": 6,
"end_offset": 11,
"label": "dis"
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{
"id": 2,
"entity": "超声心动图",
"start_offset": 16,
"end_offset": 21,
"label": "pro"
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{
"id": 3,
"entity": "Fontan手术",
"start_offset": 29,
"end_offset": 37,
"label": "pro"
},
{
"id": 4,
"entity": "心导管造影",
"start_offset": 48,
"end_offset": 53,
"label": "pro"
},
{
"id": 5,
"entity": "肺动脉解剖",
"start_offset": 59,
"end_offset": 64,
"label": "pro"
},
{
"id": 6,
"entity": "肺动脉",
"start_offset": 67,
"end_offset": 70,
"label": "bod"
},
{
"id": 7,
"entity": "左心室",
"start_offset": 76,
"end_offset": 79,
"label": "bod"
},
{
"id": 8,
"entity": "二尖瓣",
"start_offset": 82,
"end_offset": 85,
"label": "bod"
},
{
"id": 9,
"entity": "体肺",
"start_offset": 90,
"end_offset": 92,
"label": "bod"
},
{
"id": 10,
"entity": "静脉",
"start_offset": 92,
"end_offset": 94,
"label": "bod"
}
] |
心导管术还能看到大动脉转位、主动脉下狭窄及主动脉弓畸形。 | [
{
"id": 0,
"entity": "心导管术",
"start_offset": 0,
"end_offset": 4,
"label": "pro"
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{
"id": 1,
"entity": "大动脉转位",
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{
"id": 2,
"entity": "主动脉下狭窄",
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{
"id": 3,
"entity": "主动脉弓畸形",
"start_offset": 21,
"end_offset": 27,
"label": "dis"
}
] |
行顺行静脉插管到达右心房。 | [
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"id": 0,
"entity": "插管",
"start_offset": 5,
"end_offset": 7,
"label": "equ"
},
{
"id": 1,
"entity": "右心房",
"start_offset": 9,
"end_offset": 12,
"label": "bod"
}
] |
若为限制性卵圆孔或房间隔缺损，右心房压力增高，两房间压力阶差显著，＞3mm时记录右房压力曲线可见大的“a”波。 | [
{
"id": 0,
"entity": "卵圆孔",
"start_offset": 5,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "房间隔",
"start_offset": 9,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "右心房",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "右房",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
}
] |
利用漂浮导管，经心房间交通进入左心房、左心室。 | [
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"id": 0,
"entity": "漂浮导管",
"start_offset": 2,
"end_offset": 6,
"label": "equ"
},
{
"id": 1,
"entity": "心房",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "左心房",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "左心室",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
}
] |
在大动脉转位且无肺血管梗阻病例中，导管通常可经左心室插入肺动脉，如大血管连接正常，导管偶尔也可通过缺损的室间隔入发育不全的右心室，再直接进入肺动脉。 | [
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"id": 0,
"entity": "大动脉转位",
"start_offset": 1,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "导管",
"start_offset": 17,
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"label": "equ"
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{
"id": 2,
"entity": "左心室",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
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{
"id": 3,
"entity": "肺动脉",
"start_offset": 28,
"end_offset": 31,
"label": "bod"
},
{
"id": 4,
"entity": "血管",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
},
{
"id": 5,
"entity": "室间",
"start_offset": 52,
"end_offset": 54,
"label": "bod"
},
{
"id": 6,
"entity": "右心室",
"start_offset": 61,
"end_offset": 64,
"label": "bod"
},
{
"id": 7,
"entity": "肺动脉",
"start_offset": 70,
"end_offset": 73,
"label": "bod"
}
] |
但是，如肺血管流出道严重梗阻，这种方法可引起缺氧发作，操作需十分谨慎。 | [
{
"id": 0,
"entity": "肺血管流出道严重梗阻",
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},
{
"id": 1,
"entity": "缺氧",
"start_offset": 22,
"end_offset": 24,
"label": "sym"
}
] |
当导管无法插入肺动脉，平均肺动脉压可通过测肺静脉楔压得出。 | [
{
"id": 0,
"entity": "肺动脉",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "肺动脉压",
"start_offset": 13,
"end_offset": 17,
"label": "ite"
},
{
"id": 2,
"entity": "肺静脉楔压",
"start_offset": 21,
"end_offset": 26,
"label": "ite"
}
] |
肺动脉中度狭窄，其压力常正常；而肺动脉无狭窄，肺血流增多，肺血管阻力增高，肺高压形成，此种情况多出现在伴大动脉转位的该病中。 | [
{
"id": 0,
"entity": "肺动脉",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "肺动脉",
"start_offset": 16,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "肺血流",
"start_offset": 23,
"end_offset": 26,
"label": "ite"
},
{
"id": 3,
"entity": "肺血管阻力",
"start_offset": 29,
"end_offset": 34,
"label": "ite"
},
{
"id": 4,
"entity": "肺高压",
"start_offset": 37,
"end_offset": 40,
"label": "sym"
},
{
"id": 5,
"entity": "大动脉转位",
"start_offset": 52,
"end_offset": 57,
"label": "sym"
}
] |
心室大血管连接不一致，可行逆行动脉插管，直接越过主动脉瓣进入右心室；如有主-肺动脉分流，肺动脉分流，肺动脉还可通过主动脉或其分支进入。 | [
{
"id": 0,
"entity": "心室",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "血管",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 2,
"entity": "逆行动脉插管",
"start_offset": 13,
"end_offset": 19,
"label": "pro"
},
{
"id": 3,
"entity": "主动脉瓣",
"start_offset": 24,
"end_offset": 28,
"label": "bod"
},
{
"id": 4,
"entity": "右心室",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
},
{
"id": 5,
"entity": "主-肺动脉",
"start_offset": 36,
"end_offset": 41,
"label": "bod"
},
{
"id": 6,
"entity": "肺动脉",
"start_offset": 44,
"end_offset": 47,
"label": "bod"
},
{
"id": 7,
"entity": "主动脉",
"start_offset": 57,
"end_offset": 60,
"label": "bod"
}
] |
如同时伴有限制性室间隔缺损，右心室收缩期压力下降，低于左心室压力，左心室和升主动脉间存在压力阶差。 | [
{
"id": 0,
"entity": "限制性室间隔缺损",
"start_offset": 5,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "右心室",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "左心室",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "主动脉",
"start_offset": 38,
"end_offset": 41,
"label": "bod"
}
] |
但超声心动图诊断三尖瓣闭锁效果良好，一般很少行选择性右心房造影；左心室造影可见发育程度不同的右心室，像附着于前侧壁居中的一个憩室，还可清楚描绘出室间隔缺损、大动脉位置关系、肺血管流出道梗阻及肺动脉解剖结构（图9-9），左室舒张末期容积增加。 | [
{
"id": 0,
"entity": "超声心动图",
"start_offset": 1,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "三尖瓣闭锁",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "右心房造影",
"start_offset": 26,
"end_offset": 31,
"label": "pro"
},
{
"id": 3,
"entity": "左心室造影",
"start_offset": 32,
"end_offset": 37,
"label": "pro"
},
{
"id": 4,
"entity": "右心室",
"start_offset": 46,
"end_offset": 49,
"label": "bod"
},
{
"id": 5,
"entity": "室间隔缺损",
"start_offset": 72,
"end_offset": 77,
"label": "dis"
},
{
"id": 6,
"entity": "动脉",
"start_offset": 79,
"end_offset": 81,
"label": "bod"
},
{
"id": 7,
"entity": "肺血管流出道梗",
"start_offset": 86,
"end_offset": 93,
"label": "dis"
},
{
"id": 8,
"entity": "肺血管流出道梗",
"start_offset": 86,
"end_offset": 93,
"label": "pro"
},
{
"id": 9,
"entity": "道梗阻",
"start_offset": 91,
"end_offset": 94,
"label": "bod"
},
{
"id": 10,
"entity": "-9",
"start_offset": 105,
"end_offset": 107,
"label": "bod"
}
] |
若伴有大动脉转位、限制性室间隔缺损，则需行主动脉造影，估计主动脉弓发育程度及水肿情况。 | [
{
"id": 0,
"entity": "限制性室间隔缺损",
"start_offset": 9,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "主动脉造影",
"start_offset": 21,
"end_offset": 26,
"label": "pro"
},
{
"id": 2,
"entity": "主动脉弓",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "水肿",
"start_offset": 38,
"end_offset": 40,
"label": "sym"
}
] |
图9-36左心室造影（左前斜位）显示：右心室发育不良和心室-大血管连接不一致，肺动脉已环缩Ao：主动脉LV：左心室PA：肺动脉RV：右心室【治疗】早期治疗原则为：保持心房间交通的通畅；保证一定量的肺血流。 | [
{
"id": 0,
"entity": "左心室造影",
"start_offset": 5,
"end_offset": 10,
"label": "pro"
},
{
"id": 1,
"entity": "右心室",
"start_offset": 19,
"end_offset": 22,
"label": "bod"
},
{
"id": 2,
"entity": "心室-大血管",
"start_offset": 27,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "肺动脉",
"start_offset": 39,
"end_offset": 42,
"label": "bod"
},
{
"id": 4,
"entity": "主动脉",
"start_offset": 48,
"end_offset": 51,
"label": "bod"
},
{
"id": 5,
"entity": "左心室",
"start_offset": 54,
"end_offset": 57,
"label": "bod"
},
{
"id": 6,
"entity": "肺动脉",
"start_offset": 60,
"end_offset": 63,
"label": "bod"
},
{
"id": 7,
"entity": "右心室",
"start_offset": 66,
"end_offset": 69,
"label": "bod"
},
{
"id": 8,
"entity": "心房",
"start_offset": 83,
"end_offset": 85,
"label": "bod"
}
] |
有严重低氧血症的新生儿，因其肺动脉血主要来源于未闭的动脉导管，故应静脉注射前列腺素E1或E2以维持动脉导管的开放，肺血流增加，体循环氧饱和度提高。 | [
{
"id": 0,
"entity": "低氧血症",
"start_offset": 3,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "肺动脉血",
"start_offset": 14,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "动脉导管",
"start_offset": 26,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "静脉注射前列腺素E1或E2",
"start_offset": 33,
"end_offset": 46,
"label": "pro"
},
{
"id": 4,
"entity": "动脉导管",
"start_offset": 49,
"end_offset": 53,
"label": "bod"
},
{
"id": 5,
"entity": "肺血流",
"start_offset": 57,
"end_offset": 60,
"label": "ite"
},
{
"id": 6,
"entity": "体循环氧饱和度",
"start_offset": 63,
"end_offset": 70,
"label": "ite"
}
] |
改良的Blalock-Taussig分流术避免了Waterston分流术（升主动脉-右肺动脉吻合）或Potts分流术（降主动脉-左肺动脉吻合）术后肺血流量过多肺动脉高压、心力衰竭、肺动脉扭曲等并发症。 | [
{
"id": 0,
"entity": "Blalock-Taussig分流术",
"start_offset": 3,
"end_offset": 21,
"label": "pro"
},
{
"id": 1,
"entity": "Waterston分流术",
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"end_offset": 36,
"label": "pro"
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{
"id": 2,
"entity": "主动脉-右肺动脉",
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},
{
"id": 3,
"entity": "Potts分流术",
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"end_offset": 58,
"label": "pro"
},
{
"id": 4,
"entity": "主动脉-左肺动脉",
"start_offset": 60,
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"label": "bod"
},
{
"id": 5,
"entity": "肺血流量",
"start_offset": 73,
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},
{
"id": 6,
"entity": "肺血流量过多",
"start_offset": 73,
"end_offset": 79,
"label": "sym"
},
{
"id": 7,
"entity": "肺动脉高压",
"start_offset": 79,
"end_offset": 84,
"label": "dis"
},
{
"id": 8,
"entity": "心力衰竭",
"start_offset": 85,
"end_offset": 89,
"label": "dis"
},
{
"id": 9,
"entity": "肺动脉扭曲",
"start_offset": 90,
"end_offset": 95,
"label": "dis"
}
] |
患儿长至6个月以上，可行腔静脉-肺动脉双向分流术：切断右上腔静脉，远心端与右肺动脉上缘作端侧吻合，缝合近心端。 | [
{
"id": 0,
"entity": "腔静脉-肺动脉双向分流术",
"start_offset": 12,
"end_offset": 24,
"label": "pro"
},
{
"id": 1,
"entity": "右上腔静脉",
"start_offset": 27,
"end_offset": 32,
"label": "bod"
},
{
"id": 2,
"entity": "心端",
"start_offset": 34,
"end_offset": 36,
"label": "bod"
},
{
"id": 3,
"entity": "右肺动脉",
"start_offset": 37,
"end_offset": 41,
"label": "bod"
},
{
"id": 4,
"entity": "心端",
"start_offset": 52,
"end_offset": 54,
"label": "bod"
}
] |
此方案上腔静脉将体循环1/3血流送至左右肺动脉，而不增加左心室容量负荷，较改良的Blalock-Taussig分流术更符合生理学纠正标准，且可作为全腔静脉肺动脉连接术前的一期手术。 | [
{
"id": 0,
"entity": "上腔静脉",
"start_offset": 3,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "体循环",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "肺动脉",
"start_offset": 20,
"end_offset": 23,
"label": "bod"
},
{
"id": 3,
"entity": "左心室",
"start_offset": 28,
"end_offset": 31,
"label": "bod"
},
{
"id": 4,
"entity": "Blalock-Taussig分流术",
"start_offset": 40,
"end_offset": 58,
"label": "pro"
},
{
"id": 5,
"entity": "全腔静脉肺动脉连接术",
"start_offset": 73,
"end_offset": 83,
"label": "pro"
}
] |
如伴左上腔静脉残存，若左无名静脉粗大，为防止血液流入冠状窦及右心房，术中需结扎左上腔静脉；若连接静脉很细或缺如，则应将上腔静脉两侧与肺动脉作双向分流。 | [
{
"id": 0,
"entity": "左上腔静脉",
"start_offset": 2,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "静脉",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "冠状窦",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
},
{
"id": 3,
"entity": "右心房",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
},
{
"id": 4,
"entity": "左上腔静脉",
"start_offset": 39,
"end_offset": 44,
"label": "bod"
},
{
"id": 5,
"entity": "静脉",
"start_offset": 48,
"end_offset": 50,
"label": "bod"
},
{
"id": 6,
"entity": "上腔静脉",
"start_offset": 59,
"end_offset": 63,
"label": "bod"
},
{
"id": 7,
"entity": "肺动脉",
"start_offset": 66,
"end_offset": 69,
"label": "bod"
}
] |
但在小于6个月的婴儿中，因其肺动脉细小，肺血管阻力尚高，此类手术不宜进行。 | [
{
"id": 0,
"entity": "肺动脉",
"start_offset": 14,
"end_offset": 17,
"label": "bod"
},
{
"id": 1,
"entity": "肺血管阻力",
"start_offset": 20,
"end_offset": 25,
"label": "ite"
}
] |
上腔静脉-肺动脉连接术术后并发症包括静脉间侧支形成、肺动-静脉侧支形成，但其发生率大大少于标准Glenn手术（Glenn手术是将右肺动脉末端与上腔静脉作部分或完全性端侧吻合）。 | [
{
"id": 0,
"entity": "上腔静脉-肺动脉连接术",
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"end_offset": 11,
"label": "pro"
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{
"id": 1,
"entity": "肺动-静脉侧支",
"start_offset": 26,
"end_offset": 33,
"label": "bod"
},
{
"id": 2,
"entity": "Glenn手术",
"start_offset": 47,
"end_offset": 54,
"label": "pro"
},
{
"id": 3,
"entity": "Glenn手术",
"start_offset": 55,
"end_offset": 62,
"label": "pro"
},
{
"id": 4,
"entity": "右肺动脉末端",
"start_offset": 64,
"end_offset": 70,
"label": "bod"
},
{
"id": 5,
"entity": "上腔静脉",
"start_offset": 71,
"end_offset": 75,
"label": "bod"
}
] |
半Fontan手术与上腔静脉-肺动脉双向分流术相似，它是将上腔静脉两个断端均与右肺动脉相连，右心房上腔静脉入口处以补片关闭，因而二期Fontan手术仅需移动该补片。 | [
{
"id": 0,
"entity": "上腔静脉-肺动脉双向分流术",
"start_offset": 10,
"end_offset": 23,
"label": "pro"
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{
"id": 1,
"entity": "上腔静脉",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
},
{
"id": 2,
"entity": "右肺动脉",
"start_offset": 39,
"end_offset": 43,
"label": "bod"
},
{
"id": 3,
"entity": "右心房上腔静脉",
"start_offset": 46,
"end_offset": 53,
"label": "bod"
},
{
"id": 4,
"entity": "补片",
"start_offset": 57,
"end_offset": 59,
"label": "equ"
},
{
"id": 5,
"entity": "Fontan手术",
"start_offset": 66,
"end_offset": 74,
"label": "pro"
},
{
"id": 6,
"entity": "补片",
"start_offset": 79,
"end_offset": 81,
"label": "equ"
}
] |
对于存在肺血流增加，出现充血性心力衰竭的患儿，出生后3个月内以抗心力衰竭药物治疗，同时行肺动脉环缩术，防止肺高压、肺血管疾病的发展，控制心力衰竭症状。 | [
{
"id": 0,
"entity": "肺血流",
"start_offset": 4,
"end_offset": 7,
"label": "ite"
},
{
"id": 1,
"entity": "充血性心力衰竭",
"start_offset": 12,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "心力衰竭",
"start_offset": 32,
"end_offset": 36,
"label": "dis"
},
{
"id": 3,
"entity": "肺动脉环缩术",
"start_offset": 44,
"end_offset": 50,
"label": "pro"
},
{
"id": 4,
"entity": "肺高压",
"start_offset": 53,
"end_offset": 56,
"label": "dis"
},
{
"id": 5,
"entity": "心力衰竭",
"start_offset": 68,
"end_offset": 72,
"label": "sym"
}
] |
伴有完全性大动脉转位的小儿，可有限制性室间隔缺损、主动脉瓣下狭窄，应切除主动脉瓣下组织，或实行Damus-Kaye-Stansel改良术或Norwood手术，建立肺动脉干-升主动脉吻合。 | [
{
"id": 0,
"entity": "完全性大动脉转位",
"start_offset": 2,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "限制性室间隔缺损",
"start_offset": 16,
"end_offset": 24,
"label": "dis"
},
{
"id": 2,
"entity": "主动脉瓣下狭窄",
"start_offset": 25,
"end_offset": 32,
"label": "dis"
},
{
"id": 3,
"entity": "切除主动脉瓣下组织",
"start_offset": 34,
"end_offset": 43,
"label": "pro"
},
{
"id": 4,
"entity": "Damus-Kaye-Stansel改良术",
"start_offset": 47,
"end_offset": 68,
"label": "pro"
},
{
"id": 5,
"entity": "Norwood手术",
"start_offset": 69,
"end_offset": 78,
"label": "pro"
},
{
"id": 6,
"entity": "肺动脉干-升主动脉",
"start_offset": 81,
"end_offset": 90,
"label": "bod"
}
] |
Fontan手术：主要用于早期姑息性手术后仍存在低氧血症、出现活动量明显减少的患儿。 | [
{
"id": 0,
"entity": "Fontan手术",
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"label": "pro"
},
{
"id": 1,
"entity": "姑息性手术",
"start_offset": 15,
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"label": "pro"
},
{
"id": 2,
"entity": "低氧血症",
"start_offset": 24,
"end_offset": 28,
"label": "dis"
}
] |
手术适应证为：年龄大于2岁，平均肺动脉压力等于或小于15mmHg，肺血管阻力低（＜2Wood单位/平方米），肺动脉粗无狭窄，左室收缩舒张功能良好，没有明显二尖瓣反流体静脉回血直接引入肺部，使静脉血不再汇入动脉血中，从而解决了体循环低氧血症的问题。 | [
{
"id": 0,
"entity": "肺动脉压力",
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"end_offset": 21,
"label": "ite"
},
{
"id": 1,
"entity": "肺血管阻力",
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"label": "ite"
},
{
"id": 2,
"entity": "肺动脉",
"start_offset": 54,
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"label": "bod"
},
{
"id": 3,
"entity": "左室",
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"end_offset": 64,
"label": "bod"
},
{
"id": 4,
"entity": "二尖瓣",
"start_offset": 77,
"end_offset": 80,
"label": "bod"
},
{
"id": 5,
"entity": "二尖瓣反流",
"start_offset": 77,
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"label": "sym"
},
{
"id": 6,
"entity": "体静脉",
"start_offset": 82,
"end_offset": 85,
"label": "bod"
},
{
"id": 7,
"entity": "肺部",
"start_offset": 91,
"end_offset": 93,
"label": "bod"
},
{
"id": 8,
"entity": "静脉血",
"start_offset": 95,
"end_offset": 98,
"label": "bod"
},
{
"id": 9,
"entity": "动脉血",
"start_offset": 102,
"end_offset": 105,
"label": "bod"
}
] |
早期的手术方案有右心房-肺动脉直接吻合和右心房-右心室导管连接加室间隔缺损修补术两种。 | [
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"entity": "室间隔缺损修补术",
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] |
但这两种术式都会引起渐进性右心房扩大，导致体循环回流房性心律失常肺静脉回流潜在梗阻静-肺动脉双向分流；②右房内置膜片直接将下腔静脉与右肺动脉下壁连接。 | [
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"id": 5,
"entity": "肺静脉回流潜在梗阻",
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"id": 6,
"entity": "静-肺动脉",
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{
"id": 7,
"entity": "右房",
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{
"id": 8,
"entity": "膜片",
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},
{
"id": 9,
"entity": "右肺动脉下壁",
"start_offset": 66,
"end_offset": 72,
"label": "bod"
}
] |
这避免了右心房扩大血液湍流减少心外将下腔静脉远心端与右肺动脉远心端相接，这种方法无须在心脏和肺动脉间建立旁道，可减少房性心律失常的发生。 | [
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"id": 0,
"entity": "右心房",
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"id": 1,
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{
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{
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{
"id": 4,
"entity": "心外",
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{
"id": 5,
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{
"id": 6,
"entity": "右肺动脉远心端",
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{
"id": 7,
"entity": "心脏",
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"entity": "肺动脉",
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{
"id": 9,
"entity": "心律",
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"label": "ite"
},
{
"id": 10,
"entity": "房性心律失常",
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"end_offset": 64,
"label": "sym"
}
] |
对于一些高危病人，应在右心房膜片或心外导管上作一开窗（fenestration），降低静脉系统压力及右向左分流，使体循环血液回流通畅，心排出量提高，明显改善患儿胸膜渗出、右心衰竭、心低排血量状况，以便病人度过术后危险期。 | [
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"id": 1,
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"entity": "心排出量",
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{
"id": 4,
"entity": "胸膜",
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"entity": "右心衰竭",
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{
"id": 6,
"entity": "心低排血量",
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"label": "sym"
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] |
术后小儿可有轻度发绀。 | [
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"entity": "发绀",
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] |
待心功能好转后，可于监护病房内缝合窗口或在心导管室用各种装置填塞（图9-9）。 | [
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"entity": "心导管室",
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"label": "bod"
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] |
图9-37Fontan手术后，心房间交通被Amplatzer房间隔缺损闭塞器堵塞术后应给予阿司匹林或其他抗凝剂防止静脉血栓形成。 | [
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"id": 0,
"entity": "Fontan手术",
"start_offset": 5,
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"label": "pro"
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{
"id": 1,
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"entity": "Amplatzer房间隔缺损闭塞器",
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{
"id": 3,
"entity": "阿司匹林",
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{
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"entity": "抗凝剂",
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},
{
"id": 5,
"entity": "静脉血栓",
"start_offset": 57,
"end_offset": 61,
"label": "sym"
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] |
多数病人还应服用利尿剂、ACEI、地高辛。 | [
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"entity": "地高辛",
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血管扩张剂能降低外周血管阻力，减轻左心室后负荷，使肺血流量和心排出量增加。 | [
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{
"id": 4,
"entity": "心排出量",
"start_offset": 30,
"end_offset": 34,
"label": "ite"
}
] |
近年来，已证明NO可降低肺血管阻力，这提示在Fontan术后可能有内源性NO生成减少，对于这些病人，应通过治疗促进肺动脉内皮细胞释放NO。 | [
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"id": 1,
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【预后】本病的自然病程取决于肺动脉流出道梗阻情况、是否存在左室梗阻、有无左心室功能不全等因素。 | [
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若体肺循环血流量平衡，患儿早期可存活，但如不治疗，90%的病人在10岁以前死亡，一些患者可活至20～30岁，不过长期左心室超负荷必然会引起左心室功能不全二尖瓣功能障碍心排出量减少心室大动脉连接不一致者，其病情变化较连接一致者更严重，部分患者同时伴有水肿或主动脉断离，不经治疗常在1岁以内夭折。 | [
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{
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{
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{
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"entity": "心排出量",
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{
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"label": "bod"
},
{
"id": 10,
"entity": "水肿",
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"label": "sym"
}
] |
随着病源选择的严谨及手术技术的提高，Fontan术后的死亡率已低于10%，手术预后较功能性单心室更好。 | [
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对复杂型畸形患儿，行腔静脉-肺动脉双向分流加右心房开窗术更能降低术后罹病率及死亡率，但术后早期仍可出现一些并发症，如胸腔积液、腹水、心包积液室上性心律失常心脏传导阻滞体循环静脉血栓形成心排出量减少等。 | [
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"entity": "心包积液",
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{
"id": 5,
"entity": "心律",
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{
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"entity": "室上性心律失常",
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{
"id": 7,
"entity": "心脏",
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{
"id": 8,
"entity": "心脏传导阻滞",
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{
"id": 9,
"entity": "体循环",
"start_offset": 83,
"end_offset": 86,
"label": "bod"
},
{
"id": 10,
"entity": "体循环静脉血栓形成",
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"label": "sym"
},
{
"id": 11,
"entity": "心排出量减少",
"start_offset": 92,
"end_offset": 98,
"label": "sym"
}
] |
这可能与术后心脏结构重建、左心室功能变化引起心肌休整功能紊乱有关，术后早期使用血管扩张剂可有助于提高心排出量。 | [
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大多数手术病人临床症状及运动量均有明显改善，体循环氧饱和度恢复正常，血栓栓塞、脑脓疡的发生率也减少。 | [
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{
"id": 2,
"entity": "脑脓疡",
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但是，即使在一些没有临床症状的患儿中，也存在心排出量低于正常范围的现象，活动时体静脉压可进一步升高。 | [
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{
"id": 1,
"entity": "体静脉压",
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"label": "ite"
}
] |
长期的体静脉高压左室功能不全心房-肺动脉直接相连必然会使右心房逐渐扩大，易产生房性心律失常肺静脉梗阻血栓形成。 | [
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"id": 0,
"entity": "静脉",
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"id": 1,
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{
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{
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},
{
"id": 10,
"entity": "血栓",
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}
] |
除了饮食摄取及定期静脉滴注白蛋白外，其他根治性治疗方法效果仍不理想。 | [
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] |
排除肺动脉狭窄球囊扩张术或先置入stent补片，再行Fontan术，疗效颇佳；心房开窗术也可降低体静脉压力，但会加重青紫现象；还可选择腔静脉-肺动脉双向分流术或心脏移植手术。 | [
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{
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{
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{
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{
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{
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{
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},
{
"id": 9,
"entity": "心脏移植手术",
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"end_offset": 86,
"label": "pro"
}
] |
最近，在尚未产生明显体静脉高压肾上腺皮质激素和高分子肝素已获得成功，其作用机制目前仍不清楚。 | [
{
"id": 0,
"entity": "体静脉",
"start_offset": 10,
"end_offset": 13,
"label": "bod"
},
{
"id": 1,
"entity": "体静脉高压",
"start_offset": 10,
"end_offset": 15,
"label": "sym"
},
{
"id": 2,
"entity": "肾上腺皮质激素",
"start_offset": 15,
"end_offset": 22,
"label": "dru"
},
{
"id": 3,
"entity": "高分子肝素",
"start_offset": 23,
"end_offset": 28,
"label": "dru"
}
] |
此外，其他晚期并发症有肝酶轻度升高、肝硬化凝血因子缺乏等，C蛋白、S蛋白及凝血因子Ⅶ的缺乏还可引起血栓栓塞。 | [
{
"id": 0,
"entity": "肝酶轻度升高",
"start_offset": 11,
"end_offset": 17,
"label": "sym"
},
{
"id": 1,
"entity": "肝",
"start_offset": 18,
"end_offset": 19,
"label": "bod"
},
{
"id": 2,
"entity": "肝硬化",
"start_offset": 18,
"end_offset": 21,
"label": "sym"
},
{
"id": 3,
"entity": "凝血因子缺乏",
"start_offset": 21,
"end_offset": 27,
"label": "sym"
},
{
"id": 4,
"entity": "C蛋白、S蛋白及凝血因子Ⅶ的缺乏",
"start_offset": 29,
"end_offset": 45,
"label": "sym"
},
{
"id": 5,
"entity": "血栓栓塞",
"start_offset": 49,
"end_offset": 53,
"label": "sym"
}
] |
在Fontan术后长期随访的病人中，有些人出现心功能逐渐衰退的情况，有报道称术后早中期患者心功能不全以舒张功能不全为主。 | [
{
"id": 0,
"entity": "Fontan术",
"start_offset": 1,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "心功能逐渐衰退",
"start_offset": 23,
"end_offset": 30,
"label": "sym"
},
{
"id": 2,
"entity": "心功能不全",
"start_offset": 45,
"end_offset": 50,
"label": "sym"
},
{
"id": 3,
"entity": "舒张功能不全",
"start_offset": 51,
"end_offset": 57,
"label": "sym"
}
] |
一份最新研究进一步证实，术后心肌持续工作能导致左室顺应性降低，从而引起肺动脉压力升高、肺淤血心排出量下降心功能衰退的机制之一。 | [
{
"id": 0,
"entity": "心肌",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 1,
"entity": "左室",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 2,
"entity": "肺动脉压力升高",
"start_offset": 35,
"end_offset": 42,
"label": "sym"
},
{
"id": 3,
"entity": "肺",
"start_offset": 43,
"end_offset": 44,
"label": "bod"
},
{
"id": 4,
"entity": "肺淤血",
"start_offset": 43,
"end_offset": 46,
"label": "sym"
},
{
"id": 5,
"entity": "心排出量",
"start_offset": 46,
"end_offset": 50,
"label": "ite"
},
{
"id": 6,
"entity": "心排出量下降",
"start_offset": 46,
"end_offset": 52,
"label": "sym"
},
{
"id": 7,
"entity": "心功能衰退",
"start_offset": 52,
"end_offset": 57,
"label": "sym"
}
] |
总而言之，从后期并发症来看，Fontan手术仍是目前最佳的治疗手段。 | [
{
"id": 0,
"entity": "Fontan手术",
"start_offset": 14,
"end_offset": 22,
"label": "pro"
}
] |
有20%～25%的病人可通过后期手术或心导管干预而好转。 | [
{
"id": 0,
"entity": "心导管",
"start_offset": 19,
"end_offset": 22,
"label": "equ"
}
] |
第二节病毒性疾病的治疗病毒性疾病的发生和发展取决于病毒和机体两方面诸多因素，其治疗措施应包括针对病毒的抗病毒疗法，以及针对机体调节和提高其免疫功能，以清除细胞内病毒。 | [
{
"id": 0,
"entity": "病毒性疾病",
"start_offset": 3,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "病毒性疾病",
"start_offset": 11,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "病毒",
"start_offset": 25,
"end_offset": 27,
"label": "mic"
},
{
"id": 3,
"entity": "机体",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
},
{
"id": 4,
"entity": "病毒",
"start_offset": 48,
"end_offset": 50,
"label": "mic"
},
{
"id": 5,
"entity": "抗病毒疗法",
"start_offset": 51,
"end_offset": 56,
"label": "pro"
},
{
"id": 6,
"entity": "细胞",
"start_offset": 77,
"end_offset": 79,
"label": "bod"
},
{
"id": 7,
"entity": "病毒",
"start_offset": 80,
"end_offset": 82,
"label": "mic"
}
] |
由于很多急性病毒性疾病呈自限性，并缺乏特效的抗病毒药物，故综合对症处理常为其主要疗法。 | [
{
"id": 0,
"entity": "急性病毒性疾病",
"start_offset": 4,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "抗病毒药物",
"start_offset": 22,
"end_offset": 27,
"label": "dru"
}
] |
一、综合对症治疗主要包括：①一般处理：适宜休息，合理饮食，补充营养素，中枢神经系统感染时生命体征监护和呼吸管理等。 | [
{
"id": 0,
"entity": "综合对症治疗",
"start_offset": 2,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "适宜休息",
"start_offset": 19,
"end_offset": 23,
"label": "pro"
},
{
"id": 2,
"entity": "合理饮食",
"start_offset": 24,
"end_offset": 28,
"label": "pro"
},
{
"id": 3,
"entity": "补充营养素",
"start_offset": 29,
"end_offset": 34,
"label": "pro"
},
{
"id": 4,
"entity": "中枢神经系统感染",
"start_offset": 35,
"end_offset": 43,
"label": "dis"
},
{
"id": 5,
"entity": "生命体征监护",
"start_offset": 44,
"end_offset": 50,
"label": "pro"
},
{
"id": 6,
"entity": "呼吸管理",
"start_offset": 51,
"end_offset": 55,
"label": "pro"
}
] |
②对症治疗：如高温时退热，颅高压症时降低颅内压和止惊，止咳祛痰，降酶退黄等。 | [
{
"id": 0,
"entity": "对症治疗",
"start_offset": 1,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "高温",
"start_offset": 7,
"end_offset": 9,
"label": "sym"
},
{
"id": 2,
"entity": "退热",
"start_offset": 10,
"end_offset": 12,
"label": "pro"
},
{
"id": 3,
"entity": "颅高压症",
"start_offset": 13,
"end_offset": 17,
"label": "dis"
},
{
"id": 4,
"entity": "降低颅内压",
"start_offset": 18,
"end_offset": 23,
"label": "pro"
},
{
"id": 5,
"entity": "止惊",
"start_offset": 24,
"end_offset": 26,
"label": "pro"
},
{
"id": 6,
"entity": "止咳祛痰",
"start_offset": 27,
"end_offset": 31,
"label": "pro"
},
{
"id": 7,
"entity": "降酶退黄",
"start_offset": 32,
"end_offset": 36,
"label": "pro"
}
] |
③保护脏器功能：如肝炎时的护肝治疗，脑炎时保护脑细胞处理。 | [
{
"id": 0,
"entity": "脏器",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "肝炎",
"start_offset": 9,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "护肝治疗",
"start_offset": 13,
"end_offset": 17,
"label": "pro"
},
{
"id": 3,
"entity": "脑炎",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
},
{
"id": 4,
"entity": "保护脑细胞处理",
"start_offset": 21,
"end_offset": 28,
"label": "pro"
}
] |
④预防继发感染：有些病毒感染可抑制机体免疫功能，易继发细菌等其他病原感染如麻疹时，应注意预防。 | [
{
"id": 0,
"entity": "继发感染",
"start_offset": 3,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "病毒感染",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "细菌",
"start_offset": 27,
"end_offset": 29,
"label": "mic"
},
{
"id": 3,
"entity": "感染",
"start_offset": 34,
"end_offset": 36,
"label": "sym"
},
{
"id": 4,
"entity": "麻疹",
"start_offset": 37,
"end_offset": 39,
"label": "dis"
}
] |
一般不主张预防性使用抗生素。 | [
{
"id": 0,
"entity": "抗生素",
"start_offset": 10,
"end_offset": 13,
"label": "dru"
}
] |
⑤恢复期康复治疗：主要在中枢神经系统感染有明显神经性损伤者如脑炎、脊髓炎时，应加强康复治疗，以减少或减轻后遗症。 | [
{
"id": 0,
"entity": "中枢神经系统感染",
"start_offset": 12,
"end_offset": 20,
"label": "dis"
},
{
"id": 1,
"entity": "神经性损伤",
"start_offset": 23,
"end_offset": 28,
"label": "dis"
},
{
"id": 2,
"entity": "脑炎",
"start_offset": 30,
"end_offset": 32,
"label": "dis"
},
{
"id": 3,
"entity": "脊髓炎",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
},
{
"id": 4,
"entity": "康复治疗",
"start_offset": 41,
"end_offset": 45,
"label": "pro"
}
] |
三、真菌感染性口炎鹅口疮（thrush）：念珠菌感染引起的口炎中以白色念珠菌致病力最强，儿童期感染常称之为鹅口疮。 | [
{
"id": 0,
"entity": "真菌感染性口炎",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "鹅口疮",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "thrush",
"start_offset": 13,
"end_offset": 19,
"label": "dis"
},
{
"id": 3,
"entity": "念珠菌感染",
"start_offset": 21,
"end_offset": 26,
"label": "dis"
},
{
"id": 4,
"entity": "口炎",
"start_offset": 29,
"end_offset": 31,
"label": "dis"
},
{
"id": 5,
"entity": "白色念珠菌",
"start_offset": 33,
"end_offset": 38,
"label": "mic"
},
{
"id": 6,
"entity": "鹅口疮",
"start_offset": 53,
"end_offset": 56,
"label": "dis"
}
] |
念珠菌是人体常见的寄生菌，其致病力弱，仅在一定条件下感染致病，故为条件致病菌，近年来随着抗生素及肾上腺皮质激素的广泛应用，使念珠菌感染日益增多。 | [
{
"id": 0,
"entity": "念珠菌",
"start_offset": 0,
"end_offset": 3,
"label": "mic"
},
{
"id": 1,
"entity": "寄生菌",
"start_offset": 9,
"end_offset": 12,
"label": "mic"
},
{
"id": 2,
"entity": "条件致病菌",
"start_offset": 33,
"end_offset": 38,
"label": "mic"
},
{
"id": 3,
"entity": "抗生素",
"start_offset": 44,
"end_offset": 47,
"label": "dru"
},
{
"id": 4,
"entity": "肾上腺皮质激素",
"start_offset": 48,
"end_offset": 55,
"label": "dru"
},
{
"id": 5,
"entity": "念珠菌感染",
"start_offset": 62,
"end_offset": 67,
"label": "dis"
}
] |
【病因】为白色念珠菌感染。 | [
{
"id": 0,
"entity": "白色念珠菌感染",
"start_offset": 5,
"end_offset": 12,
"label": "dis"
}
] |
诱因有营养不良、腹泻及长期使用抗生素、肾上腺皮质激素等，这些诱因加上乳具污染，便可引起鹅口疮。 | [
{
"id": 0,
"entity": "营养不良",
"start_offset": 3,
"end_offset": 7,
"label": "sym"
},
{
"id": 1,
"entity": "腹泻",
"start_offset": 8,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "抗生素",
"start_offset": 15,
"end_offset": 18,
"label": "dru"
},
{
"id": 3,
"entity": "肾上腺皮质激素",
"start_offset": 19,
"end_offset": 26,
"label": "dru"
},
{
"id": 4,
"entity": "鹅口疮",
"start_offset": 43,
"end_offset": 46,
"label": "dis"
}
] |
【临床表现及诊断】鹅口疮的特点是口腔黏膜上出现白色乳凝块样物，分布于颊黏膜、舌、齿龈和上腭表面。 | [
{
"id": 0,
"entity": "鹅口疮",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "口腔黏膜",
"start_offset": 16,
"end_offset": 20,
"label": "bod"
},
{
"id": 2,
"entity": "口腔黏膜上出现白色乳凝块样物",
"start_offset": 16,
"end_offset": 30,
"label": "sym"
},
{
"id": 3,
"entity": "颊黏膜",
"start_offset": 34,
"end_offset": 37,
"label": "bod"
},
{
"id": 4,
"entity": "舌",
"start_offset": 38,
"end_offset": 39,
"label": "bod"
},
{
"id": 5,
"entity": "齿龈",
"start_offset": 40,
"end_offset": 42,
"label": "bod"
},
{
"id": 6,
"entity": "上腭",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
}
] |
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