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【临床表现】全身皮肤可出现出血点、瘀点、瘀斑、血肿,严重病例出现内脏出血,如颅内出血、消化道出血。
[ { "id": 0, "entity": "全身皮肤", "start_offset": 6, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "全身皮肤可出现出血点、瘀点、瘀斑、血肿", "start_offset": 6, "end_offset": 25, "label": "sym" }, { "id": 2, "entity": "颅内出血", "start_offset": 38, "end_offset": 42, "label": "sym" }, { "id": 3, "entity": "消化道出血", "start_offset": 43, "end_offset": 48, "label": "sym" } ]
【治疗】根据导致血小板减少的病因,进行病因治疗。
[ { "id": 0, "entity": "血小板", "start_offset": 8, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "血小板减少", "start_offset": 8, "end_offset": 13, "label": "sym" } ]
如血小板严重减少,应输新鲜血小板。
[ { "id": 0, "entity": "血小板", "start_offset": 1, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "血小板严重减少", "start_offset": 1, "end_offset": 8, "label": "sym" }, { "id": 2, "entity": "血小板", "start_offset": 13, "end_offset": 16, "label": "bod" } ]
第八节肺静脉异位连接肺静脉异位连接可分为完全性肺静脉异位连接及部分性肺静脉异位连接两类。
[ { "id": 0, "entity": "肺静脉异位连接", "start_offset": 3, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "肺静脉异位连接", "start_offset": 10, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "完全性肺静脉异位连接", "start_offset": 20, "end_offset": 30, "label": "dis" }, { "id": 3, "entity": "部分性肺静脉异位连接", "start_offset": 31, "end_offset": 41, "label": "dis" } ]
一、完全性肺静脉异位连接完全性肺静脉异位连接是指所有的肺静脉直接或借道体静脉间接入右心房,占所有心血管畸形的1.5%~2.6%。
[ { "id": 0, "entity": "完全性肺静脉异位连接", "start_offset": 2, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "完全性肺静脉异位连接", "start_offset": 12, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "肺静脉", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "借道体静脉", "start_offset": 33, "end_offset": 38, "label": "bod" }, { "id": 4, "entity": "右心房", "start_offset": 41, "end_offset": 44, "label": "bod" }, { "id": 5, "entity": "心血管畸形", "start_offset": 48, "end_offset": 53, "label": "dis" } ]
【病理解剖】2/3病人单独发生完全性肺静脉异位连接,不伴有其他畸形;1/3病例同时伴有其他心血管畸形,极少数伴内脏综合征。
[ { "id": 0, "entity": "完全性肺静脉异位连接", "start_offset": 15, "end_offset": 25, "label": "dis" }, { "id": 1, "entity": "畸形", "start_offset": 31, "end_offset": 33, "label": "dis" }, { "id": 2, "entity": "其他心血管畸形", "start_offset": 43, "end_offset": 50, "label": "dis" }, { "id": 3, "entity": "内脏综合征", "start_offset": 55, "end_offset": 60, "label": "dis" } ]
(一)心上型此类型最常见,占完全性肺静脉异位连接的1/2。
[ { "id": 0, "entity": "完全性肺静脉异位连接", "start_offset": 14, "end_offset": 24, "label": "dis" } ]
左、右肺静脉在左心房后面先汇合成静脉总干,然后再向上同无名静脉或上腔静脉相连。
[ { "id": 0, "entity": "左、右肺静脉", "start_offset": 0, "end_offset": 6, "label": "bod" }, { "id": 1, "entity": "左心房", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "静脉", "start_offset": 16, "end_offset": 18, "label": "bod" }, { "id": 3, "entity": "无名静脉", "start_offset": 27, "end_offset": 31, "label": "bod" }, { "id": 4, "entity": "上腔静脉", "start_offset": 32, "end_offset": 36, "label": "bod" } ]
大部分这类病儿的静脉总干通过垂直静脉与左无名静脉相通。
[ { "id": 0, "entity": "静脉总干", "start_offset": 8, "end_offset": 12, "label": "bod" }, { "id": 1, "entity": "垂直静脉", "start_offset": 14, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "左无名静脉", "start_offset": 19, "end_offset": 24, "label": "bod" } ]
少数病例连于奇静脉。
[ { "id": 0, "entity": "奇静脉", "start_offset": 6, "end_offset": 9, "label": "bod" } ]
(二)心内型静脉总干直接连于右心房或冠状静脉窦。
[ { "id": 0, "entity": "静脉总干", "start_offset": 6, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "右心房", "start_offset": 14, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "冠状静脉窦", "start_offset": 18, "end_offset": 23, "label": "bod" } ]
(三)心下型占完全性肺静脉异位连接的10%。
[ { "id": 0, "entity": "完全性肺静脉异位连接", "start_offset": 7, "end_offset": 17, "label": "dis" } ]
静脉总干向下穿过膈肌的食管裂孔注入静脉,常见的为门脉系统,其次为静脉导管、肝静脉、下腔静脉。
[ { "id": 0, "entity": "静脉总干", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "膈肌", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "食管裂孔", "start_offset": 11, "end_offset": 15, "label": "bod" }, { "id": 3, "entity": "静脉", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 4, "entity": "静脉导管", "start_offset": 32, "end_offset": 36, "label": "bod" }, { "id": 5, "entity": "肝静脉", "start_offset": 37, "end_offset": 40, "label": "bod" }, { "id": 6, "entity": "下腔静脉", "start_offset": 41, "end_offset": 45, "label": "bod" } ]
图9-23完全性肺静脉异位连接A.心上型:通过上升的垂直静脉连接到无名静脉;B.心内型:通过下降的垂直静脉连接到门静脉;C.心下型:连接到冠状窦肺静脉梗阻可发生在各种类型的本病中,常见静脉总干自身狭窄,在心上型、心下型病例中亦可见外部压迫引起的梗阻。
[ { "id": 0, "entity": "完全性肺静脉异位连接", "start_offset": 5, "end_offset": 15, "label": "dis" }, { "id": 1, "entity": "垂直静脉", "start_offset": 26, "end_offset": 30, "label": "bod" }, { "id": 2, "entity": "无名静脉", "start_offset": 33, "end_offset": 37, "label": "bod" }, { "id": 3, "entity": "垂直静脉", "start_offset": 49, "end_offset": 53, "label": "bod" }, { "id": 4, "entity": "门静脉", "start_offset": 56, "end_offset": 59, "label": "bod" }, { "id": 5, "entity": "冠状窦", "start_offset": 69, "end_offset": 72, "label": "bod" }, { "id": 6, "entity": "肺静脉梗阻", "start_offset": 72, "end_offset": 77, "label": "dis" }, { "id": 7, "entity": "静脉总干", "start_offset": 92, "end_offset": 96, "label": "bod" }, { "id": 8, "entity": "梗阻", "start_offset": 122, "end_offset": 124, "label": "dis" } ]
如心下型,静脉回流通道穿过膈肌时可受外界压迫,狭窄的静脉导管及肝血窦也可使肺静脉血液回流受阻,这种类型梗阻较多见;心上型,垂直静脉上行时夹于左肺动脉与左支气管之间、或引流入右上腔静脉的静脉总干夹于右肺动脉与气管之间,均可受压变窄,大约半数病人会发生这种情况;心脏型患者梗阻最少见。
[ { "id": 0, "entity": "静脉", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "膈肌", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "静脉导管", "start_offset": 26, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "肝血窦", "start_offset": 31, "end_offset": 34, "label": "bod" }, { "id": 4, "entity": "肺静脉血液", "start_offset": 37, "end_offset": 42, "label": "bod" }, { "id": 5, "entity": "梗阻", "start_offset": 51, "end_offset": 53, "label": "dis" }, { "id": 6, "entity": "垂直静脉", "start_offset": 61, "end_offset": 65, "label": "bod" }, { "id": 7, "entity": "左肺动脉", "start_offset": 70, "end_offset": 74, "label": "bod" }, { "id": 8, "entity": "左支气管", "start_offset": 75, "end_offset": 79, "label": "bod" }, { "id": 9, "entity": "右上腔静脉", "start_offset": 86, "end_offset": 91, "label": "bod" }, { "id": 10, "entity": "静脉总干", "start_offset": 92, "end_offset": 96, "label": "bod" }, { "id": 11, "entity": "右肺动脉", "start_offset": 98, "end_offset": 102, "label": "bod" }, { "id": 12, "entity": "气管", "start_offset": 103, "end_offset": 105, "label": "bod" }, { "id": 13, "entity": "心脏", "start_offset": 129, "end_offset": 131, "label": "bod" }, { "id": 14, "entity": "梗阻", "start_offset": 134, "end_offset": 136, "label": "dis" } ]
梗阻还可发生在心房水平,限制性的房间交通使进入左心房的血流减少,右心房压力增高,继而影响肺静脉回流。
[ { "id": 0, "entity": "梗阻", "start_offset": 0, "end_offset": 2, "label": "dis" }, { "id": 1, "entity": "心房", "start_offset": 7, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "左心房", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "右心房", "start_offset": 32, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "肺静脉", "start_offset": 44, "end_offset": 47, "label": "bod" } ]
右心房、右心室、肺动脉因血流量增多而扩大,而左心房、左心室则因充盈减少,容积缩小。
[ { "id": 0, "entity": "右心房", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "右心室", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "肺动脉", "start_offset": 8, "end_offset": 11, "label": "bod" }, { "id": 3, "entity": "左心房", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "左心室", "start_offset": 26, "end_offset": 29, "label": "bod" } ]
【病理生理】本病体循环静脉的低氧血液和肺静脉含氧量多的动脉血在右心房汇合,其混合血液量的多少与心房间交通的大小有关。
[ { "id": 0, "entity": "静脉", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "肺静脉", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "动脉血", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "右心房", "start_offset": 31, "end_offset": 34, "label": "bod" }, { "id": 4, "entity": "心房", "start_offset": 47, "end_offset": 49, "label": "bod" } ]
若房缺很小,血液流入左心房受限,引起体循环灌注量不足,同时右心房压力增高,体静脉及肺静脉回流受阻;若房缺够大,两心房间血流通畅,自右心房分别向右心室或左心房的血流量则取决于心房、心室的顺应性和体肺循环的阻力。
[ { "id": 0, "entity": "血液", "start_offset": 6, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "左心房", "start_offset": 10, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "右心房", "start_offset": 29, "end_offset": 32, "label": "bod" }, { "id": 3, "entity": "体静脉", "start_offset": 37, "end_offset": 40, "label": "bod" }, { "id": 4, "entity": "肺静脉", "start_offset": 41, "end_offset": 44, "label": "bod" }, { "id": 5, "entity": "心房", "start_offset": 56, "end_offset": 58, "label": "bod" }, { "id": 6, "entity": "右心房", "start_offset": 65, "end_offset": 68, "label": "bod" }, { "id": 7, "entity": "右心室", "start_offset": 71, "end_offset": 74, "label": "bod" }, { "id": 8, "entity": "左心房", "start_offset": 75, "end_offset": 78, "label": "bod" }, { "id": 9, "entity": "心房", "start_offset": 86, "end_offset": 88, "label": "bod" }, { "id": 10, "entity": "心室", "start_offset": 89, "end_offset": 91, "label": "bod" } ]
本病的病理生理与是否存在肺静脉梗阻密切相关。
[ { "id": 0, "entity": "肺静脉梗阻", "start_offset": 12, "end_offset": 17, "label": "dis" } ]
(一)无梗阻型随着生后肺血管阻力逐渐下降,动静脉混合血液进入肺循环的流量相应增加,其血流动力学与大型房间隔缺损类似。
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右心室容量负荷过大,引起右室扩大、心肌肥厚,肺动脉压力明显升高,静脉总干梗阻部位和肺小静脉中膜肥厚、内膜增生,肺部淋巴液增多,淋巴管扩张,引起肺动脉反射性收缩,中膜肥厚,肺水肿。
[ { "id": 0, "entity": "右心室", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "右室", "start_offset": 12, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "心肌", "start_offset": 17, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "肺动脉", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "右室扩大、心肌肥厚,肺动脉压力明显升高", "start_offset": 12, "end_offset": 31, "label": "sym" }, { "id": 5, "entity": "静脉总干", "start_offset": 32, "end_offset": 36, "label": "bod" }, { "id": 6, "entity": "肺小静脉中膜", "start_offset": 41, "end_offset": 47, "label": "bod" }, { "id": 7, "entity": "内膜", "start_offset": 50, "end_offset": 52, "label": "bod" }, { "id": 8, "entity": "肺部淋巴液", "start_offset": 55, "end_offset": 60, "label": "bod" }, { "id": 9, "entity": "淋巴管", "start_offset": 63, "end_offset": 66, "label": "bod" }, { "id": 10, "entity": "肺动脉", "start_offset": 71, "end_offset": 74, "label": "bod" }, { "id": 11, "entity": "中膜", "start_offset": 80, "end_offset": 82, "label": "bod" }, { "id": 12, "entity": "肺", "start_offset": 85, "end_offset": 86, "label": "bod" } ]
肺动脉收缩又可引起继发性肺动脉高压,右心室压力增高,最终导致右心衰竭。
[ { "id": 0, "entity": "肺动脉", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "继发性肺动脉高压", "start_offset": 9, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "右心室", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 3, "entity": "右心衰竭", "start_offset": 30, "end_offset": 34, "label": "dis" } ]
右心室搏动明显。
[ { "id": 0, "entity": "右心室搏动明显", "start_offset": 0, "end_offset": 7, "label": "sym" } ]
肺动脉瓣区第二心音亢进,呈宽的固定分裂。
[ { "id": 0, "entity": "肺动脉瓣", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "肺动脉瓣区第二心音亢进,呈宽的固定分裂", "start_offset": 0, "end_offset": 19, "label": "sym" } ]
第三心音可及。
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右心房、右心室血流量增多,引起肺动脉瓣相对狭窄,在胸骨左上缘可闻及柔和的收缩期喷射样杂音。
[ { "id": 0, "entity": "右心房", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "右心室", "start_offset": 4, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "肺动脉瓣", "start_offset": 15, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "胸骨左上缘", "start_offset": 25, "end_offset": 30, "label": "bod" }, { "id": 4, "entity": "右心房、右心室血流量增多,引起肺动脉瓣相对狭窄,在胸骨左上缘可闻及柔和的收缩期喷射样杂音", "start_offset": 0, "end_offset": 44, "label": "sym" } ]
胸骨左下缘常可闻及三尖瓣相对狭窄时出现的舒张期杂音。
[ { "id": 0, "entity": "三尖瓣", "start_offset": 9, "end_offset": 12, "label": "bod" }, { "id": 1, "entity": "胸骨左下缘常可闻及三尖瓣相对狭窄时出现的舒张期杂音", "start_offset": 0, "end_offset": 25, "label": "sym" } ]
如果异位引流静脉连于左无名静脉,则在心底部两侧可听到连续性静脉杂音静脉杂音。
[ { "id": 0, "entity": "异位引流静脉", "start_offset": 2, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "左无名静脉", "start_offset": 10, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "心底部", "start_offset": 18, "end_offset": 21, "label": "bod" }, { "id": 3, "entity": "心底部两侧可听到连续性静脉杂音静脉杂音", "start_offset": 18, "end_offset": 37, "label": "sym" } ]
肝脏增大亦可作为右心衰竭的一个凭据。
[ { "id": 0, "entity": "肝脏", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "肝脏增大", "start_offset": 0, "end_offset": 4, "label": "sym" }, { "id": 2, "entity": "右心衰竭", "start_offset": 8, "end_offset": 12, "label": "dis" } ]
(二)梗阻型这类患儿出生后数天内即可出现临床症状:迅速加重的呼吸困难、发绀、喂养困难。
[ { "id": 0, "entity": "迅速加重的呼吸困难、发绀、喂养困难", "start_offset": 25, "end_offset": 42, "label": "sym" } ]
与无梗阻型患儿相比,此类型小儿心血管体征不显著。
[ { "id": 0, "entity": "心血管", "start_offset": 15, "end_offset": 18, "label": "bod" } ]
由于心排出量下降,外周血管搏动减弱。
[ { "id": 0, "entity": "心", "start_offset": 2, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "外周血管", "start_offset": 9, "end_offset": 13, "label": "bod" } ]
心脏不扩大,右心室搏动不明显,杂音通常缺。
[ { "id": 0, "entity": "心脏", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "心脏不扩大", "start_offset": 0, "end_offset": 5, "label": "sym" }, { "id": 2, "entity": "右心室", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 3, "entity": "右心室搏动不明显", "start_offset": 6, "end_offset": 14, "label": "sym" }, { "id": 4, "entity": "杂音通常缺", "start_offset": 15, "end_offset": 20, "label": "sym" } ]
肺动脉瓣区第二心音明显,存在分裂,但分裂时限不宽。
[ { "id": 0, "entity": "肺动脉瓣", "start_offset": 0, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "肺动脉瓣区第二心音明显,存在分裂,但分裂时限不宽", "start_offset": 0, "end_offset": 24, "label": "sym" } ]
如有严重呼吸窘迫时,由于肺水肿,肺部可及湿啰音。
[ { "id": 0, "entity": "肺水肿", "start_offset": 12, "end_offset": 15, "label": "sym" }, { "id": 1, "entity": "肺部可及湿啰音", "start_offset": 16, "end_offset": 23, "label": "sym" } ]
腹部检查可触及肝脏肿大。
[ { "id": 0, "entity": "腹部检查", "start_offset": 0, "end_offset": 4, "label": "ite" }, { "id": 1, "entity": "肝脏", "start_offset": 7, "end_offset": 9, "label": "bod" }, { "id": 2, "entity": "腹部检查可触及肝脏肿大", "start_offset": 0, "end_offset": 11, "label": "sym" } ]
【辅助检查】(一)胸部X线1.无梗阻型右心房、右心室增大,肺血增多。
[ { "id": 0, "entity": "胸部X线", "start_offset": 9, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "右心房", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "右心室", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "肺血", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 4, "entity": "右心房、右心室增大,肺血增多", "start_offset": 19, "end_offset": 33, "label": "sym" } ]
如连于左无名静脉,左上心缘可见扩张的垂直静脉和左无名静脉,右侧可见上腔静脉,使心影呈典型的8字形或雪人样,这种表现多出现在年长儿,出生数月的小儿这种影像不明显。
[ { "id": 0, "entity": "左无名静脉", "start_offset": 3, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "左上心缘", "start_offset": 9, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "垂直静脉", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 3, "entity": "左无名静脉", "start_offset": 23, "end_offset": 28, "label": "bod" }, { "id": 4, "entity": "左上心缘可见扩张的垂直静脉和左无名静脉", "start_offset": 9, "end_offset": 28, "label": "sym" }, { "id": 5, "entity": "上腔静脉", "start_offset": 33, "end_offset": 37, "label": "bod" }, { "id": 6, "entity": "右侧可见上腔静脉", "start_offset": 29, "end_offset": 37, "label": "sym" }, { "id": 7, "entity": "使心影呈典型的8字形或雪人样", "start_offset": 38, "end_offset": 52, "label": "sym" } ]
2.梗阻型其胸片特征为肺间质弥漫性斑点网状阴影,由肺门向周围放射。
[ { "id": 0, "entity": "胸片", "start_offset": 6, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "肺间质", "start_offset": 11, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "肺间质弥漫性斑点网状阴影", "start_offset": 11, "end_offset": 23, "label": "sym" }, { "id": 3, "entity": "肺门", "start_offset": 25, "end_offset": 27, "label": "bod" }, { "id": 4, "entity": "由肺门向周围放射", "start_offset": 24, "end_offset": 32, "label": "sym" } ]
肺间质及肺泡水肿严重时,可产生毛玻璃样改变。
[ { "id": 0, "entity": "肺间质及肺泡水肿", "start_offset": 0, "end_offset": 8, "label": "dis" } ]
心缘不清晰,但心脏多不增大(图9-9)。
[ { "id": 0, "entity": "心脏", "start_offset": 7, "end_offset": 9, "label": "bod" } ]
X线只有在新生儿期即出现心衰但心影不大的患儿中,才具诊断意义。
[ { "id": 0, "entity": "X线", "start_offset": 0, "end_offset": 2, "label": "pro" }, { "id": 1, "entity": "心衰", "start_offset": 12, "end_offset": 14, "label": "sym" }, { "id": 2, "entity": "心影不大", "start_offset": 15, "end_offset": 19, "label": "sym" } ]
图9-24胸部X片显示:心下型完全性肺静脉异位引流由于肺静脉阻塞导致两肺野模糊(二)心电图电轴右偏,右心室肥大。
[ { "id": 0, "entity": "胸部X片", "start_offset": 5, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "心下型完全性肺静脉异位", "start_offset": 12, "end_offset": 23, "label": "dis" }, { "id": 2, "entity": "肺静脉", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "肺野", "start_offset": 35, "end_offset": 37, "label": "bod" }, { "id": 4, "entity": "心电图", "start_offset": 42, "end_offset": 45, "label": "pro" }, { "id": 5, "entity": "右心室", "start_offset": 50, "end_offset": 53, "label": "bod" }, { "id": 6, "entity": "右心室肥大", "start_offset": 50, "end_offset": 55, "label": "sym" } ]
没有肺静脉梗阻的患儿,可伴右心房增大,表现为Ⅱ导联及右胸导联P波高尖。
[ { "id": 0, "entity": "肺静脉梗阻", "start_offset": 2, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "右心房", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "右心房增大", "start_offset": 13, "end_offset": 18, "label": "sym" }, { "id": 3, "entity": "右胸", "start_offset": 26, "end_offset": 28, "label": "bod" } ]
(三)超声心动图当检查时发现右心房容量负荷过重、卵圆孔或房缺处有右向左分流、左心房内没有看到正常回流的肺静脉、左心房、左心室小、房间隔向左膨出时,需高度怀疑完全性肺静脉异位连接。
[ { "id": 0, "entity": "超声心动图", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "右心房", "start_offset": 14, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "卵圆孔", "start_offset": 24, "end_offset": 27, "label": "bod" }, { "id": 3, "entity": "左心房", "start_offset": 38, "end_offset": 41, "label": "bod" }, { "id": 4, "entity": "肺静脉", "start_offset": 51, "end_offset": 54, "label": "bod" }, { "id": 5, "entity": "左心房", "start_offset": 55, "end_offset": 58, "label": "bod" }, { "id": 6, "entity": "左心室", "start_offset": 59, "end_offset": 62, "label": "bod" }, { "id": 7, "entity": "房间隔", "start_offset": 64, "end_offset": 67, "label": "bod" }, { "id": 8, "entity": "完全性肺静脉异位连接", "start_offset": 78, "end_offset": 88, "label": "dis" } ]
心脏超声检查的目的在于明确异位肺静脉的数量、梗阻部位和肺动脉压力。
[ { "id": 0, "entity": "心脏超声检查", "start_offset": 0, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "异位肺静脉", "start_offset": 13, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "梗阻", "start_offset": 22, "end_offset": 24, "label": "dis" }, { "id": 3, "entity": "肺动脉", "start_offset": 27, "end_offset": 30, "label": "bod" } ]
当肺动脉流速高时可见肺动脉干扩张。
[ { "id": 0, "entity": "肺动脉", "start_offset": 1, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "肺动脉干", "start_offset": 10, "end_offset": 14, "label": "bod" } ]
1.心上型剑突下冠状切面可见左右肺静脉汇集腔与左房相隔。
[ { "id": 0, "entity": "左右肺静脉汇集腔", "start_offset": 14, "end_offset": 22, "label": "bod" }, { "id": 1, "entity": "左房", "start_offset": 23, "end_offset": 25, "label": "bod" } ]
向上行走的垂直静脉在胸骨上窝区最易看到(图9-9a),脉冲Doppler可探测到垂直静脉内正向血流频谱及上腔静脉内负向血流。
[ { "id": 0, "entity": "垂直静脉", "start_offset": 5, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "胸骨上窝区", "start_offset": 10, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "脉冲Doppler", "start_offset": 27, "end_offset": 36, "label": "pro" }, { "id": 3, "entity": "垂直静脉", "start_offset": 40, "end_offset": 44, "label": "bod" }, { "id": 4, "entity": "上腔静脉", "start_offset": 52, "end_offset": 56, "label": "bod" } ]
若垂直静脉行走于左肺动脉与左支气管间,可通过剑突下矢状切面观察。
[ { "id": 0, "entity": "静脉", "start_offset": 3, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "左肺动脉", "start_offset": 8, "end_offset": 12, "label": "bod" }, { "id": 2, "entity": "左支气管", "start_offset": 13, "end_offset": 17, "label": "bod" } ]
2.心内型剑突下冠状切面可看到肺静脉各自或汇总后进入右心房。
[ { "id": 0, "entity": "肺静脉", "start_offset": 15, "end_offset": 18, "label": "bod" }, { "id": 1, "entity": "右心房", "start_offset": 26, "end_offset": 29, "label": "bod" } ]
如肺静脉异位引流入冠状静脉窦,该切面还可看到冠状窦膨大(图9-9b),这需要与大型房间隔缺损鉴别(原发房间隔缺损位于更靠前的一个切面)。
[ { "id": 0, "entity": "肺静脉", "start_offset": 1, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "冠状静脉窦", "start_offset": 9, "end_offset": 14, "label": "bod" }, { "id": 2, "entity": "冠状窦", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "大型房间隔缺损", "start_offset": 39, "end_offset": 46, "label": "dis" }, { "id": 4, "entity": "房间隔缺损", "start_offset": 51, "end_offset": 56, "label": "dis" } ]
3.心下型左右肺静脉汇集腔同样在通过剑突下冠状切面看到,即而以剑突下矢状切面可见一静脉向下穿过膈肌。
[ { "id": 0, "entity": "左右肺静脉汇集腔", "start_offset": 5, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "静脉", "start_offset": 41, "end_offset": 43, "label": "bod" }, { "id": 2, "entity": "膈肌", "start_offset": 47, "end_offset": 49, "label": "bod" } ]
通过彩色Doppler显像示静脉总干在穿行食管裂孔处狭窄,追踪血流见汇入门静脉或肝静脉(图9-9c)。
[ { "id": 0, "entity": "彩色Doppler显像", "start_offset": 2, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "静脉总干", "start_offset": 14, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "食管裂孔处", "start_offset": 21, "end_offset": 26, "label": "bod" }, { "id": 3, "entity": "门静脉", "start_offset": 36, "end_offset": 39, "label": "bod" }, { "id": 4, "entity": "肝静脉", "start_offset": 40, "end_offset": 43, "label": "bod" } ]
门静脉常扩张。
[ { "id": 0, "entity": "门静脉", "start_offset": 0, "end_offset": 3, "label": "bod" } ]
最常见的混合型完全性肺静脉异位连接为右肺静脉直接入右心房,左肺静脉通过垂直静脉与左无名静脉连接。
[ { "id": 0, "entity": "混合型完全性肺静脉异位连接", "start_offset": 4, "end_offset": 17, "label": "dis" }, { "id": 1, "entity": "右肺静脉", "start_offset": 18, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "右心房", "start_offset": 25, "end_offset": 28, "label": "bod" }, { "id": 3, "entity": "左肺静脉", "start_offset": 29, "end_offset": 33, "label": "bod" }, { "id": 4, "entity": "垂直静脉", "start_offset": 35, "end_offset": 39, "label": "bod" }, { "id": 5, "entity": "左无名静脉", "start_offset": 40, "end_offset": 45, "label": "bod" } ]
肺静脉梗阻情况可通过连续Doppler及彩色Doppler显像来估计。
[ { "id": 0, "entity": "肺静脉梗阻", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "连续Doppler", "start_offset": 10, "end_offset": 19, "label": "pro" }, { "id": 2, "entity": "彩色Doppler显像", "start_offset": 20, "end_offset": 31, "label": "pro" } ]
梗阻部位血流流速增快,无时相性血流图谱(图9-9d)。
[ { "id": 0, "entity": "梗阻", "start_offset": 0, "end_offset": 2, "label": "dis" } ]
但如果右心室流出道梗阻,肺静脉血流量不足,上述现象可能会被掩盖,常见于复杂型青紫性心脏病。
[ { "id": 0, "entity": "右心室流出道梗阻", "start_offset": 3, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "肺静脉", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "复杂型青紫性心脏病", "start_offset": 35, "end_offset": 44, "label": "dis" } ]
给予前列腺素E可予以鉴别。
[ { "id": 0, "entity": "前列腺素E", "start_offset": 2, "end_offset": 7, "label": "dru" } ]
用三尖瓣反流检测仪可测得三尖瓣两侧压力阶差,进而明确肺动脉压力。
[ { "id": 0, "entity": "三尖瓣反流检测仪", "start_offset": 1, "end_offset": 9, "label": "equ" }, { "id": 1, "entity": "三尖瓣", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 2, "entity": "肺动脉", "start_offset": 26, "end_offset": 29, "label": "bod" } ]
(四)心导管造影检查对大多数病人,二维心脏超声和Doppler技术可了解肺静脉解剖结构及梗阻部位等细节问题,通常很少再行心导管检查。
[ { "id": 0, "entity": "心导管造影检查", "start_offset": 3, "end_offset": 10, "label": "pro" }, { "id": 1, "entity": "二维心脏超声", "start_offset": 17, "end_offset": 23, "label": "pro" }, { "id": 2, "entity": "Doppler技术", "start_offset": 24, "end_offset": 33, "label": "pro" }, { "id": 3, "entity": "肺静脉", "start_offset": 36, "end_offset": 39, "label": "bod" }, { "id": 4, "entity": "梗阻", "start_offset": 44, "end_offset": 46, "label": "dis" }, { "id": 5, "entity": "心导管检查", "start_offset": 60, "end_offset": 65, "label": "pro" } ]
心导管术仅用于复杂病例和狭窄血管的精确定位上。
[ { "id": 0, "entity": "心导管术", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "血管", "start_offset": 14, "end_offset": 16, "label": "bod" } ]
1.血氧饱和度肺静脉异位入口处血氧饱和度明显升高。
[ { "id": 0, "entity": "血氧饱和度", "start_offset": 2, "end_offset": 7, "label": "ite" }, { "id": 1, "entity": "肺静脉", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "血氧饱和度", "start_offset": 15, "end_offset": 20, "label": "ite" } ]
2.压力当心房间交通很小时,右心房压力常高于左心房,不过如没有此压力上的区别,也不能排除限制性心房内交通的存在。
[ { "id": 0, "entity": "心房", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "右心房", "start_offset": 14, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "左心房", "start_offset": 22, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "心房", "start_offset": 47, "end_offset": 49, "label": "bod" } ]
右心室及肺动脉压力稍高于体循环。
[ { "id": 0, "entity": "右心室", "start_offset": 0, "end_offset": 3, "label": "bod" }, { "id": 1, "entity": "肺动脉", "start_offset": 4, "end_offset": 7, "label": "bod" } ]
3.造影选择性肺静脉造影对明确肺静脉异常开口位置具有诊断意义。
[ { "id": 0, "entity": "造影", "start_offset": 2, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "选择性肺静脉造影", "start_offset": 4, "end_offset": 12, "label": "pro" }, { "id": 2, "entity": "肺静脉", "start_offset": 15, "end_offset": 18, "label": "bod" } ]
在梗阻型患儿中,可显示血管狭窄,造影剂排空时间延长。
[ { "id": 0, "entity": "梗阻", "start_offset": 1, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "血管", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "造影剂", "start_offset": 16, "end_offset": 19, "label": "dru" } ]
但是,由于在肺静脉回流通道中放置心导管,可进一步增加梗阻的程度,此检查在肺静脉梗阻同时有肺动脉高压的患婴中危险性较大。
[ { "id": 0, "entity": "肺静脉", "start_offset": 6, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "心导管", "start_offset": 16, "end_offset": 19, "label": "equ" }, { "id": 2, "entity": "梗阻", "start_offset": 26, "end_offset": 28, "label": "dis" }, { "id": 3, "entity": "肺静脉梗阻", "start_offset": 36, "end_offset": 41, "label": "dis" }, { "id": 4, "entity": "肺动脉高压", "start_offset": 44, "end_offset": 49, "label": "dis" } ]
(五)磁共振成像异位连接的肺静脉和回流静脉狭窄部位还可以通过磁共振来显示。
[ { "id": 0, "entity": "磁共振成像", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "肺静脉", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "回流静脉", "start_offset": 17, "end_offset": 21, "label": "bod" }, { "id": 3, "entity": "磁共振", "start_offset": 30, "end_offset": 33, "label": "pro" } ]
但是,将患婴置于磁共振仪器上有一定的危险性,且目前尚无研究表明磁共振显像优于上述诊断方法。
[ { "id": 0, "entity": "磁共振仪器", "start_offset": 8, "end_offset": 13, "label": "equ" }, { "id": 1, "entity": "磁共振", "start_offset": 31, "end_offset": 34, "label": "pro" } ]
【治疗】婴幼儿一旦病情稳定,即应该实施根治性手术。
[ { "id": 0, "entity": "手术", "start_offset": 22, "end_offset": 24, "label": "pro" } ]
有严重肺水肿、低氧血症、低心排量的患婴,早期应予正压通气插管、强心剂、利尿剂及纠正代谢性酸中毒以稳定病情,等待手术时机。
[ { "id": 0, "entity": "严重肺水肿", "start_offset": 1, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "低氧血症", "start_offset": 7, "end_offset": 11, "label": "dis" }, { "id": 2, "entity": "低心排量", "start_offset": 12, "end_offset": 16, "label": "dis" }, { "id": 3, "entity": "正压通气插管", "start_offset": 24, "end_offset": 30, "label": "pro" }, { "id": 4, "entity": "强心剂", "start_offset": 31, "end_offset": 34, "label": "dru" }, { "id": 5, "entity": "利尿剂", "start_offset": 35, "end_offset": 38, "label": "dru" }, { "id": 6, "entity": "纠正代谢性酸中毒", "start_offset": 39, "end_offset": 47, "label": "pro" }, { "id": 7, "entity": "手术", "start_offset": 55, "end_offset": 57, "label": "pro" } ]
如果可能,则尽量避免行心导管术,以防耽误手术时间、出现其他并发症。
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现已不再认为球囊房隔造口术是必要的姑息性手术。
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如异位肺静脉直接入右心房,可打开右心房,切开房间隔,用补片将左右肺静脉全部隔入左心房;如异位开口于冠状窦,切开左心房与冠状窦之间的房间隔组织,随即缝合冠状窦开口以及房间隔缺损,使冠状窦直接引流入左心房;对于心上型或心下型肺静脉异位连接者,将肺静脉总干与左心房之间做大的侧-侧吻合,关闭房间隔缺损,而是否结扎肺静脉回流通道仍存在争议。
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一些主张开放回流静脉者认为,开放垂直静脉可预防术后早期左心偏小、肺静脉回流受阻情况。
[ { "id": 0, "entity": "回流静脉", "start_offset": 6, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "垂直静脉", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "左心", "start_offset": 27, "end_offset": 29, "label": "bod" }, { "id": 3, "entity": "肺静脉", "start_offset": 32, "end_offset": 35, "label": "bod" } ]
且有报道称,结扎下行的静脉通道后,会引起急性肝细胞坏死,故一些外科医生建议不缝合该静脉。
[ { "id": 0, "entity": "静脉", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "急性肝细胞坏死", "start_offset": 20, "end_offset": 27, "label": "dis" }, { "id": 2, "entity": "外科", "start_offset": 31, "end_offset": 33, "label": "dep" }, { "id": 3, "entity": "静脉", "start_offset": 41, "end_offset": 43, "label": "bod" } ]
图9-25超声心动图显示:肺静脉异常引流入(箭头所示为血流方向)CS:冠状窦PV:肺静脉SVC:上腔静脉VV:垂直静脉【预后】若不行手术治疗,本病预后差。
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手术治疗的效果已有明显改善,患儿死亡率从20世纪70年代末期的30%降至80年代末90年代初的不到10%。
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手术死亡率与患儿术前病情状况密切相关。
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心下型患儿、术前需用呼吸机、有代谢性酸中毒患儿术后死亡率较高。
[ { "id": 0, "entity": "呼吸机", "start_offset": 10, "end_offset": 13, "label": "equ" }, { "id": 1, "entity": "代谢性酸中毒", "start_offset": 15, "end_offset": 21, "label": "dis" } ]
肺静脉梗阻小儿术后早期可出现肺高压,故对于这种患儿,吸入NO有助于扩张肺动脉,降低死亡率。
[ { "id": 0, "entity": "肺静脉梗阻", "start_offset": 0, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "肺高压", "start_offset": 14, "end_offset": 17, "label": "dis" }, { "id": 2, "entity": "肺动脉", "start_offset": 35, "end_offset": 38, "label": "bod" } ]
偶尔也可发生晚期肺静脉梗阻,可能与术后组织反应或吻合口狭窄有关,但再次手术、球囊扩张术或用stent补片却易造成该处血液反流。
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少数患儿后期可出现心律失常,包括窦性心动过缓、房扑及室上性心动过速,其中房性心律失常最为常见。
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第九章功能性消化不良功能性消化不良(functionaldyspepsia,FD)是指有持续存在或反复发作的上腹痛腹胀早饱、嗳气、厌食、胃灼热泛酸、恶心及呕吐等消化功能障碍症状,经各项检查排除器质性疾病的一组小儿消化内科最常见的临床综合征。
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功能性消化不良的患儿主诉各异,又缺乏肯定的特异病理生理基础,因此,对这一部分患者,曾有许多命名,主要有功能性消化不良、非溃疡性消化不良(nonulcerdyspepsia,NUD)、特发性消化不良(idiopathicdyspepsia)、原发性消化不良(essentialdyspepsia)、胀气性消化不良(flatulentdyspepsia)以及上腹不适综合征(epigastricdistresssyndrome)等。
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目前国际上多采用前三种命名,而“功能性消化不良”尤为大多数学者所接受。
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【流行病学】FD发病十分普遍,美国东北部郊区507名社区青少年调查发现,5%~10%的受调查者具有典型的消化不良症状。
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意大利北部校园儿童研究表明3.5%存在溃疡样消化不良的表现,3.7%存在动力障碍样消化不良,但本研究中未纳入12岁以上的青少年,所以患病率低。
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一项在儿科消化专科门诊进行的研究表明,4~9岁功能性胃肠病患儿中,13.5%被诊断为消化不良,10~18岁中有10.2%有消化不良。
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在我国此病有逐年上升的趋势,以消化不良为主诉的成人患者约占普通内科门诊的11%、占消化专科门诊的53%。
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国内儿科患者中功能性消化不良的发病率尚无规范的统计。
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【病因及发病机制】FD的病因不明,其发病机制亦不清楚。
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这些因素包括了饮食和环境、胃酸分泌、幽门螺旋杆菌感染、消化道运动功能异常、心理因素以及一些其他胃肠功能紊乱性疾病,如胃食管反流性疾病(GERD)、吞气症及肠易激综合征等。
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(一)饮食与环境因素FD患者的症状往往与饮食有关,许多患者常常主诉一些含气饮料、咖啡、柠檬或其他水果以及油炸类食物会加重消化不良。
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虽然双盲法食物诱发试验对食物诱因的意义提出了质疑,但许多患儿仍在避免上述食物并平衡了膳食结构后感到症状有所减轻。
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(二)胃酸部分FD的患者会出现溃疡样症状,如饥饿痛,在进食后渐缓解,腹部有指点压痛制酸剂或抑酸药物症状可在短期内缓解。
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这些都提示这类患者的发病与胃酸有关。
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