text
stringlengths 4
500
| ents
list |
|---|---|
首选红霉素，30～50mg/（kg•d），14～21天，静脉滴注或口服分4次，疗程3周；或阿奇霉素10mg/（kg•d），3～5天，或联用利福平。 | [
{
"indices": [
2,
3,
4
],
"label": "dru",
"score": 1,
"text": "红霉素"
},
{
"indices": [
28,
29,
30,
31
],
"label": "pro",
"score": 1,
"text": "静脉滴注"
},
{
"indices": [
33,
34,
35,
36,
37,
38,
39,
40,
41,
42
],
"label": "pro",
"score": 1,
"text": "口服分4次，疗程3周"
},
{
"indices": [
45,
46,
47,
48
],
"label": "dru",
"score": 1,
"text": "阿奇霉素"
},
{
"indices": [
69,
70,
71
],
"label": "dru",
"score": 1,
"text": "利福平"
}
] |
危重病例必要时可考虑选用喹诺酮类抗生素。 | [
{
"indices": [
12,
13,
14,
15,
16,
17,
18
],
"label": "dru",
"score": 1,
"text": "喹诺酮类抗生素"
}
] |
重症军团菌病往往出现进行性呼吸困难致呼吸衰竭、肾衰竭、感染性休克、DIC和脑病。 | [
{
"indices": [
0,
1,
2,
3,
4,
5
],
"label": "dis",
"score": 1,
"text": "重症军团菌病"
},
{
"indices": [
10,
11,
12,
13,
14,
15,
16
],
"label": "sym",
"score": 1,
"text": "进行性呼吸困难"
},
{
"indices": [
18,
19,
20,
21
],
"label": "dis",
"score": 1,
"text": "呼吸衰竭"
},
{
"indices": [
23,
24,
25
],
"label": "dis",
"score": 1,
"text": "肾衰竭"
},
{
"indices": [
27,
28,
29,
30,
31
],
"label": "dis",
"score": 1,
"text": "感染性休克"
},
{
"indices": [
33,
34,
35
],
"label": "dis",
"score": 1,
"text": "DIC"
},
{
"indices": [
37,
38
],
"label": "dis",
"score": 1,
"text": "脑病"
}
] |
这些病往往是军团菌病死亡的重要原因，应积极防治。 | [
{
"indices": [
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "军团菌病"
}
] |
第四节气道反应性气道高反应性（BHR）是指气道对外界特异性或非特异性刺激过于强烈的反应，是儿童支气管哮喘的主要病理生理特征。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "ite",
"score": 1,
"text": "气道反应性"
},
{
"indices": [
8,
9,
10,
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "气道高反应性"
},
{
"indices": [
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "BHR"
},
{
"indices": [
21,
22
],
"label": "bod",
"score": 1,
"text": "气道"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42
],
"label": "sym",
"score": 1,
"text": "气道对外界特异性或非特异性刺激过于强烈的反应"
},
{
"indices": [
47,
48,
49,
50,
51
],
"label": "dis",
"score": 1,
"text": "支气管哮喘"
}
] |
气道反应性测定对儿童不典型哮喘、咳嗽变异型哮喘的诊断，以及支气管哮喘患儿用药治疗期间的疗效判断和何时停药显得格外重要。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6
],
"label": "pro",
"score": 1,
"text": "气道反应性测定"
},
{
"indices": [
10,
11,
12,
13,
14
],
"label": "dis",
"score": 1,
"text": "不典型哮喘"
},
{
"indices": [
16,
17,
18,
19,
20,
21,
22
],
"label": "dis",
"score": 1,
"text": "咳嗽变异型哮喘"
},
{
"indices": [
29,
30,
31,
32,
33
],
"label": "dis",
"score": 1,
"text": "支气管哮喘"
}
] |
一、支气管激发试验分为直接激发试验和间接激发试验，前者主要选用外源性非选择性直接激发剂，如组胺、乙酰甲胆碱。 | [
{
"indices": [
2,
3,
4,
5,
6,
7,
8
],
"label": "pro",
"score": 1,
"text": "支气管激发试验"
},
{
"indices": [
11,
12,
13,
14,
15,
16
],
"label": "pro",
"score": 1,
"text": "直接激发试验"
},
{
"indices": [
18,
19,
20,
21,
22,
23
],
"label": "pro",
"score": 1,
"text": "间接激发试验"
},
{
"indices": [
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42
],
"label": "dru",
"score": 1,
"text": "外源性非选择性直接激发剂"
},
{
"indices": [
45,
46
],
"label": "dru",
"score": 1,
"text": "组胺"
},
{
"indices": [
48,
49,
50,
51,
52
],
"label": "dru",
"score": 1,
"text": "乙酰甲胆碱"
}
] |
间接激发试验主要通过刺激支气管内炎性细胞使其释放多种能间接引起支气管狭窄的介质，作用于支气管平滑肌上特异性受体而引起气道收缩。 | [
{
"indices": [
0,
1,
2,
3,
4,
5
],
"label": "pro",
"score": 1,
"text": "间接激发试验"
},
{
"indices": [
12,
13,
14,
15,
16,
17,
18,
19
],
"label": "bod",
"score": 1,
"text": "支气管内炎性细胞"
},
{
"indices": [
31,
32,
33,
34,
35
],
"label": "dis",
"score": 1,
"text": "支气管狭窄"
},
{
"indices": [
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54
],
"label": "bod",
"score": 1,
"text": "支气管平滑肌上特异性受体"
},
{
"indices": [
58,
59
],
"label": "bod",
"score": 1,
"text": "气道"
},
{
"indices": [
58,
59,
60,
61
],
"label": "sym",
"score": 1,
"text": "气道收缩"
}
] |
目前间接激发试验不论在成人还是儿童应用都很少，尤其是儿童尚没有规范的量化标准。 | [
{
"indices": [
2,
3,
4,
5,
6,
7
],
"label": "pro",
"score": 1,
"text": "间接激发试验"
}
] |
而且特异性抗原刺激危险性较大，可诱发严重的哮喘。 | [
{
"indices": [
2,
3,
4,
5,
6,
7,
8
],
"label": "pro",
"score": 1,
"text": "特异性抗原刺激"
},
{
"indices": [
21,
22
],
"label": "dis",
"score": 1,
"text": "哮喘"
}
] |
目前大多数支气管激发试验选用前者。 | [
{
"indices": [
5,
6,
7,
8,
9,
10,
11
],
"label": "pro",
"score": 1,
"text": "支气管激发试验"
}
] |
直接支气管激发试验检测方法目前主要有两种：1.用常规通气方法以吸药前的FEV<sub>1</sub>做对照，间歇吸入不同浓度（或相同浓度）的乙酰甲胆碱或组胺，每吸入1次，检测当时的FEV<sub>1</sub>。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8
],
"label": "pro",
"score": 1,
"text": "直接支气管激发试验"
},
{
"indices": [
35,
36,
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47,
48,
49
],
"label": "ite",
"score": 1,
"text": "FEV<sub>1</sub>"
},
{
"indices": [
70,
71,
72,
73,
74
],
"label": "dru",
"score": 1,
"text": "乙酰甲胆碱"
},
{
"indices": [
76,
77
],
"label": "dru",
"score": 1,
"text": "组胺"
},
{
"indices": [
90,
91,
92,
93,
94,
95,
96,
97,
98,
99,
100,
101,
102,
103,
104
],
"label": "ite",
"score": 1,
"text": "FEV<sub>1</sub>"
}
] |
一般以FEV<sub>1</sub>较对照值下降20%的最低累积剂量（PD20-FEV<sub>1</sub>）或最低累积浓度（PC20-FEV<sub>1</sub>）为试验的反应阈值，当FEV<sub>1</sub>下降大于20%对照值或基础值时为激发试验阳性。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17
],
"label": "ite",
"score": 1,
"text": "FEV<sub>1</sub>"
},
{
"indices": [
35,
36,
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54
],
"label": "ite",
"score": 1,
"text": "PD20-FEV<sub>1</sub>"
},
{
"indices": [
64,
65,
66,
67,
68,
69,
70,
71,
72,
73,
74,
75,
76,
77,
78,
79,
80,
81,
82,
83
],
"label": "ite",
"score": 1,
"text": "PC20-FEV<sub>1</sub>"
},
{
"indices": [
95,
96,
97,
98,
99,
100,
101,
102,
103,
104,
105,
106,
107,
108,
109
],
"label": "ite",
"score": 1,
"text": "FEV<sub>1</sub>"
},
{
"indices": [
126,
127,
128,
129
],
"label": "pro",
"score": 1,
"text": "激发试验"
},
{
"indices": [
126,
127,
128,
129,
130,
131
],
"label": "sym",
"score": 1,
"text": "激发试验阳性"
}
] |
2.连续呼吸的Astograph法原理是应用强迫振荡技术进行检测，在连续吸入不同浓度的乙酰甲胆碱时，不断监测呼吸阻力。 | [
{
"indices": [
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16
],
"label": "pro",
"score": 1,
"text": "连续呼吸的Astograph法"
},
{
"indices": [
22,
23,
24,
25,
26,
27
],
"label": "pro",
"score": 1,
"text": "强迫振荡技术"
},
{
"indices": [
43,
44,
45,
46,
47
],
"label": "dru",
"score": 1,
"text": "乙酰甲胆碱"
},
{
"indices": [
54,
55,
56,
57
],
"label": "ite",
"score": 1,
"text": "呼吸阻力"
}
] |
整个雾化系统包括12个雾化罐，第1罐为生理盐水，第12罐为支气管扩张剂，第2～11罐为浓度依次递增（49～25000μg/mL）的乙酰甲胆碱。 | [
{
"indices": [
11,
12,
13
],
"label": "equ",
"score": 1,
"text": "雾化罐"
},
{
"indices": [
19,
20,
21,
22
],
"label": "dru",
"score": 1,
"text": "生理盐水"
},
{
"indices": [
29,
30,
31,
32,
33,
34
],
"label": "dru",
"score": 1,
"text": "支气管扩张剂"
},
{
"indices": [
65,
66,
67,
68,
69
],
"label": "dru",
"score": 1,
"text": "乙酰甲胆碱"
}
] |
以吸入生理盐水时的阻力为基础阻力，当呼吸阻力增加到基础阻力的2倍时，改为吸入支气管扩张剂。 | [
{
"indices": [
3,
4,
5,
6
],
"label": "dru",
"score": 1,
"text": "生理盐水"
},
{
"indices": [
18,
19,
20,
21
],
"label": "ite",
"score": 1,
"text": "呼吸阻力"
},
{
"indices": [
38,
39,
40,
41,
42,
43
],
"label": "dru",
"score": 1,
"text": "支气管扩张剂"
}
] |
最后按照所测出的气道反应阈值得出诊断结果。 | [
{
"indices": [
8,
9
],
"label": "bod",
"score": 1,
"text": "气道"
}
] |
3.注意事项（1）检查前须停药：β受体兴奋剂（沙丁胺醇等）应停用12小时以上，缓释型停用24小时以上；甲基黄嘌呤类（茶碱）普通型停用12小时以上，缓释型停用24小时以上，抗胆碱能类药（异丙溴托胺等）停用12小时以上；抗组织胺类药停用48小时以上；糖皮质激素停用12小时以上，另外避免吸烟、咖啡、可乐饮料等6小时以上；（2）检测前常规肺功能：FEV<sub>1</sub>＞预计值70%以上。 | [
{
"indices": [
16,
17,
18,
19,
20,
21
],
"label": "dru",
"score": 1,
"text": "β受体兴奋剂"
},
{
"indices": [
23,
24,
25,
26
],
"label": "dru",
"score": 1,
"text": "沙丁胺醇"
},
{
"indices": [
51,
52,
53,
54,
55,
56
],
"label": "dru",
"score": 1,
"text": "甲基黄嘌呤类"
},
{
"indices": [
58,
59
],
"label": "dru",
"score": 1,
"text": "茶碱"
},
{
"indices": [
85,
86,
87,
88,
89,
90
],
"label": "dru",
"score": 1,
"text": "抗胆碱能类药"
},
{
"indices": [
92,
93,
94,
95,
96
],
"label": "dru",
"score": 1,
"text": "异丙溴托胺"
},
{
"indices": [
108,
109,
110,
111,
112,
113
],
"label": "dru",
"score": 1,
"text": "抗组织胺类药"
},
{
"indices": [
123,
124,
125,
126,
127
],
"label": "dru",
"score": 1,
"text": "糖皮质激素"
},
{
"indices": [
139,
140,
141,
142,
143,
144,
145,
146,
147,
148,
149,
150,
151,
152,
153,
154,
155,
156
],
"label": "pro",
"score": 1,
"text": "避免吸烟、咖啡、可乐饮料等6小时以上"
},
{
"indices": [
164,
165,
166,
167,
168
],
"label": "pro",
"score": 1,
"text": "常规肺功能"
},
{
"indices": [
170,
171,
172,
173,
174,
175,
176,
177,
178,
179,
180,
181,
182,
183,
184
],
"label": "ite",
"score": 1,
"text": "FEV<sub>1</sub>"
}
] |
（3）检测时监测：激发试验可能会诱发喘息，在检测过程中须加强肺部听诊，准备好支气管舒张剂和抢救设备。 | [
{
"indices": [
9,
10,
11,
12
],
"label": "pro",
"score": 1,
"text": "激发试验"
},
{
"indices": [
18,
19
],
"label": "sym",
"score": 1,
"text": "喘息"
},
{
"indices": [
30,
31,
32,
33
],
"label": "pro",
"score": 1,
"text": "肺部听诊"
},
{
"indices": [
38,
39,
40,
41,
42,
43
],
"label": "dru",
"score": 1,
"text": "支气管舒张剂"
},
{
"indices": [
45,
46,
47,
48
],
"label": "equ",
"score": 1,
"text": "抢救设备"
}
] |
在检测结束时须给予支气管舒张剂吸入，待患儿常规肺功能恢复FEV<sub>1</sub>＞预计值70%以上，方可以回家。 | [
{
"indices": [
9,
10,
11,
12,
13,
14
],
"label": "dru",
"score": 1,
"text": "支气管舒张剂"
},
{
"indices": [
21,
22,
23,
24,
25
],
"label": "pro",
"score": 1,
"text": "常规肺功能"
},
{
"indices": [
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42
],
"label": "ite",
"score": 1,
"text": "FEV<sub>1</sub>"
}
] |
第三节右室双出口右室双出口（doubleoutletsofrightventricle）是指当两根大血管完全或接近完全起自右心室，占出生婴儿的0.032‰。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
8,
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42
],
"label": "dis",
"score": 1,
"text": "doubleoutletsofrightventricle"
},
{
"indices": [
47,
48,
49,
50,
51,
52,
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63
],
"label": "sym",
"score": 1,
"text": "两根大血管完全或接近完全起自右心室"
},
{
"indices": [
49,
50,
51
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
61,
62,
63
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
65,
66,
67,
68,
69,
70,
71,
72,
73,
74,
75,
76
],
"label": "sym",
"score": 1,
"text": "占出生婴儿的0.032‰"
}
] |
因此，右室双出口可与双心室、单心室或任何大血管相互关系及任何房室连接类型同时存在。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
10,
11,
12
],
"label": "bod",
"score": 1,
"text": "双心室"
},
{
"indices": [
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39
],
"label": "sym",
"score": 1,
"text": "双心室、单心室或任何大血管相互关系及任何房室连接类型同时存在"
},
{
"indices": [
14,
15,
16
],
"label": "bod",
"score": 1,
"text": "单心室"
},
{
"indices": [
20,
21,
22
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
30,
31
],
"label": "bod",
"score": 1,
"text": "房室"
}
] |
【病理解剖】右室双出口不是一种单一的先天性心脏畸形，该术语仅仅用于描述各种心脏畸形时大血管的位置。 | [
{
"indices": [
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
18,
19,
20,
21,
22,
23,
24
],
"label": "dis",
"score": 1,
"text": "先天性心脏畸形"
},
{
"indices": [
37,
38,
39,
40
],
"label": "dis",
"score": 1,
"text": "心脏畸形"
},
{
"indices": [
42,
43,
44
],
"label": "bod",
"score": 1,
"text": "大血管"
}
] |
尽管这种心室大血管连接可合并任何类型的心脏节段连接类型，但绝大多数为心房正位，房室连接一致。 | [
{
"indices": [
4,
5,
6,
7,
8
],
"label": "bod",
"score": 1,
"text": "心室大血管"
},
{
"indices": [
19,
20
],
"label": "bod",
"score": 1,
"text": "心脏"
},
{
"indices": [
34,
35
],
"label": "bod",
"score": 1,
"text": "心房"
},
{
"indices": [
39,
40
],
"label": "bod",
"score": 1,
"text": "房室"
}
] |
大血管相互关系常见的类型有：①主、肺动脉瓣相对关系正常，主动脉瓣位于肺动脉的右后方，此型最多见；②大动脉总干相互平行，主动脉瓣位于右侧，即所谓“Taussing-Bing畸形”；③大动脉总干相互平行，主动脉瓣位于左侧，此型最少见。 | [
{
"indices": [
0,
1,
2
],
"label": "dis",
"score": 1,
"text": "大血管"
},
{
"indices": [
15,
16,
17,
18,
19,
20
],
"label": "bod",
"score": 1,
"text": "主、肺动脉瓣"
},
{
"indices": [
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "主、肺动脉瓣相对关系正常"
},
{
"indices": [
28,
29,
30,
31
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于肺动脉的右后方"
},
{
"indices": [
34,
35,
36
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
49,
50,
51
],
"label": "bod",
"score": 1,
"text": "大动脉"
},
{
"indices": [
49,
50,
51,
52,
53,
54,
55,
56,
57
],
"label": "sym",
"score": 1,
"text": "大动脉总干相互平行"
},
{
"indices": [
59,
60,
61,
62
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
59,
60,
61,
62,
63,
64,
65,
66
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于右侧"
},
{
"indices": [
72,
73,
74,
75,
76,
77,
78,
79,
80,
81,
82,
83,
84,
85,
86
],
"label": "dis",
"score": 1,
"text": "Taussing-Bing畸形"
},
{
"indices": [
90,
91,
92,
93,
94
],
"label": "bod",
"score": 1,
"text": "大动脉总干"
},
{
"indices": [
90,
91,
92,
93,
94,
95,
96,
97,
98
],
"label": "sym",
"score": 1,
"text": "大动脉总干相互平行"
},
{
"indices": [
100,
101,
102,
103
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
100,
101,
102,
103,
104,
105,
106,
107
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于左侧"
}
] |
也有两根大血管有螺旋交叉而主动脉位于肺动脉的左后方者。 | [
{
"indices": [
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24
],
"label": "sym",
"score": 1,
"text": "两根大血管有螺旋交叉而主动脉位于肺动脉的左后方"
},
{
"indices": [
4,
5,
6
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
13,
14,
15
],
"label": "bod",
"score": 1,
"text": "主动脉"
},
{
"indices": [
18,
19,
20
],
"label": "bod",
"score": 1,
"text": "肺动脉"
}
] |
但近30%的右室双出口主动脉瓣位于右侧且与肺动脉瓣在同一水平，近54%的右室双出口主动脉瓣位于肺动脉瓣的右前方。 | [
{
"indices": [
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
11,
12,
13,
14
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于右侧且与肺动脉瓣在同一水平"
},
{
"indices": [
21,
22,
23,
24
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣"
},
{
"indices": [
36,
37,
38,
39,
40
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
41,
42,
43,
44
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于肺动脉瓣的右前方"
},
{
"indices": [
47,
48,
49,
50
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣"
}
] |
右室双出口最常见且被广泛接受的分型方法是依据室间隔缺损的位置分为四种类型，即：①伴有主动脉下室间隔缺损的右室双出口；②伴有肺动脉下室间隔缺损的右室双出口；③伴有双动脉下室间隔缺损的右室双出口；④伴有远离大动脉室间隔缺损的右室双出口。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
22,
23,
24,
25,
26
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损的右室双出口"
},
{
"indices": [
61,
62,
63,
64,
65,
66,
67,
68,
69,
70,
71,
72,
73,
74,
75
],
"label": "dis",
"score": 1,
"text": "肺动脉下室间隔缺损的右室双出口"
},
{
"indices": [
80,
81,
82,
83,
84,
85,
86,
87,
88,
89,
90,
91,
92,
93,
94
],
"label": "dis",
"score": 1,
"text": "双动脉下室间隔缺损的右室双出口"
},
{
"indices": [
99,
100,
101,
102,
103,
104,
105,
106,
107,
108,
109,
110,
111,
112,
113,
114
],
"label": "dis",
"score": 1,
"text": "远离大动脉室间隔缺损的右室双出口"
}
] |
在临床上的分类为：①伴主动脉下室间隔缺损，主动脉瓣位于肺动脉右侧及右室流出道狭窄（法四型右室双出口）；②伴肺动脉下室间隔缺损，主动脉瓣位于肺动脉右侧；③伴主动脉下室间隔缺损，主动脉瓣位于肺动脉右侧，不伴有右心室流出道狭窄的右室双出口较常见。 | [
{
"indices": [
11,
12,
13,
14,
15,
16,
17,
18,
19
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损"
},
{
"indices": [
21,
22,
23,
24
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于肺动脉右侧及右室流出道狭窄"
},
{
"indices": [
27,
28,
29
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
35,
36,
37,
38,
39
],
"label": "dis",
"score": 1,
"text": "流出道狭窄"
},
{
"indices": [
41,
42,
43,
44,
45,
46,
47,
48
],
"label": "dis",
"score": 1,
"text": "法四型右室双出口"
},
{
"indices": [
53,
54,
55,
56,
57,
58,
59,
60,
61
],
"label": "dis",
"score": 1,
"text": "肺动脉下室间隔缺损"
},
{
"indices": [
63,
64,
65,
66
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
63,
64,
65,
66,
67,
68,
69,
70,
71,
72,
73
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于肺动脉右侧"
},
{
"indices": [
69,
70,
71
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
77,
78,
79,
80,
81,
82,
83,
84,
85
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损"
},
{
"indices": [
87,
88,
89,
90
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
87,
88,
89,
90,
91,
92,
93,
94,
95,
96,
97
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于肺动脉右侧"
},
{
"indices": [
93,
94,
95
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
102,
103,
104,
105,
106,
107
],
"label": "bod",
"score": 1,
"text": "右心室流出道"
},
{
"indices": [
102,
103,
104,
105,
106,
107,
108,
109
],
"label": "sym",
"score": 1,
"text": "右心室流出道狭窄"
},
{
"indices": [
111,
112,
113,
114,
115
],
"label": "dis",
"score": 1,
"text": "右室双出口"
}
] |
从病理解剖学上，无论室间隔缺损为何种类型，室间隔缺损的位置几乎是固定的，即位于隔束的两个分叉之间，而室间隔缺损位置的变化取决于大血管本身的排列、相互关系和流出道室间隔的大小。 | [
{
"indices": [
10,
11,
12,
13,
14
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
21,
22,
23,
24,
25
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34
],
"label": "sym",
"score": 1,
"text": "室间隔缺损的位置几乎是固定的"
},
{
"indices": [
36,
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47
],
"label": "sym",
"score": 1,
"text": "即位于隔束的两个分叉之间"
},
{
"indices": [
50,
51,
52,
53,
54
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
50,
51,
52,
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63,
64,
65,
66,
67,
68,
69,
70,
71,
72,
73,
74,
75,
76,
77,
78,
79,
80,
81,
82,
83,
84,
85
],
"label": "sym",
"score": 1,
"text": "室间隔缺损位置的变化取决于大血管本身的排列、相互关系和流出道室间隔的大小"
},
{
"indices": [
63,
64,
65
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
77,
78,
79
],
"label": "bod",
"score": 1,
"text": "流出道"
},
{
"indices": [
80,
81,
82
],
"label": "bod",
"score": 1,
"text": "室间隔"
}
] |
据此，在右室双出口的心脏，肌部室间隔仅有流入道及小梁部。 | [
{
"indices": [
4,
5,
6,
7,
8
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "右室双出口的心脏，肌部室间隔仅有流入道及小梁部"
},
{
"indices": [
10,
11
],
"label": "bod",
"score": 1,
"text": "心脏"
},
{
"indices": [
13,
14
],
"label": "bod",
"score": 1,
"text": "肌部"
},
{
"indices": [
15,
16,
17
],
"label": "bod",
"score": 1,
"text": "室间隔"
},
{
"indices": [
20,
21,
22
],
"label": "bod",
"score": 1,
"text": "流入道"
},
{
"indices": [
24,
25,
26
],
"label": "bod",
"score": 1,
"text": "小梁部"
}
] |
当两根大血管均发自右心室，流出道室间隔（圆锥隔或漏斗部）完全是右心室内的结构。 | [
{
"indices": [
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11
],
"label": "sym",
"score": 1,
"text": "两根大血管均发自右心室"
},
{
"indices": [
3,
4,
5
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
9,
10,
11
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
13,
14,
15
],
"label": "bod",
"score": 1,
"text": "流出道"
},
{
"indices": [
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37
],
"label": "sym",
"score": 1,
"text": "流出道室间隔（圆锥隔或漏斗部）完全是右心室内的结构"
},
{
"indices": [
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27
],
"label": "bod",
"score": 1,
"text": "室间隔（圆锥隔或漏斗部）"
},
{
"indices": [
31,
32,
33
],
"label": "bod",
"score": 1,
"text": "右心室"
}
] |
仅有的例外是当室间隔缺损是肌部流入道或心尖小梁部，或膜周流入道向后主要向流入道延伸，即形成远离大动脉型的室间隔缺损。 | [
{
"indices": [
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23
],
"label": "sym",
"score": 1,
"text": "室间隔缺损是肌部流入道或心尖小梁部"
},
{
"indices": [
13,
14,
15,
16,
17
],
"label": "bod",
"score": 1,
"text": "肌部流入道"
},
{
"indices": [
19,
20,
21,
22,
23
],
"label": "bod",
"score": 1,
"text": "心尖小梁部"
},
{
"indices": [
26,
27,
28,
29,
30
],
"label": "bod",
"score": 1,
"text": "膜周流入道"
},
{
"indices": [
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40
],
"label": "sym",
"score": 1,
"text": "膜周流入道向后主要向流入道延伸"
},
{
"indices": [
36,
37,
38
],
"label": "bod",
"score": 1,
"text": "流入道"
},
{
"indices": [
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56
],
"label": "dis",
"score": 1,
"text": "远离大动脉型的室间隔缺损"
}
] |
漏斗部的形态也有许多类型，大动脉瓣近端与室间隔缺损顶部的形态取决于心室漏斗部褶。 | [
{
"indices": [
13,
14,
15,
16,
17,
18
],
"label": "bod",
"score": 1,
"text": "大动脉瓣近端"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26
],
"label": "bod",
"score": 1,
"text": "室间隔缺损顶部"
},
{
"indices": [
33,
34,
35,
36,
37,
38
],
"label": "bod",
"score": 1,
"text": "心室漏斗部褶"
}
] |
当两根大血管的空间关系基本正常的情况下，两根大血管短轴连线与室间隔是平行的；此时的流出道相对与室间隔是向右成角且流出道室隔几乎都是与隔束的前臂融合，此时，位于隔束的两臂之间的室间隔缺损位于主动脉下。 | [
{
"indices": [
3,
4,
5
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36
],
"label": "sym",
"score": 1,
"text": "两根大血管短轴连线与室间隔是平行的"
},
{
"indices": [
22,
23,
24
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
30,
31,
32
],
"label": "bod",
"score": 1,
"text": "室间隔"
},
{
"indices": [
41,
42,
43
],
"label": "bod",
"score": 1,
"text": "流出道"
},
{
"indices": [
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63,
64,
65,
66,
67,
68,
69,
70,
71,
72
],
"label": "sym",
"score": 1,
"text": "流出道相对与室间隔是向右成角且流出道室隔几乎都是与隔束的前臂融合"
},
{
"indices": [
47,
48,
49
],
"label": "bod",
"score": 1,
"text": "室间隔"
},
{
"indices": [
56,
57,
58,
59,
60
],
"label": "bod",
"score": 1,
"text": "流出道室隔"
},
{
"indices": [
66,
67
],
"label": "bod",
"score": 1,
"text": "隔束"
},
{
"indices": [
69,
70
],
"label": "bod",
"score": 1,
"text": "前臂"
},
{
"indices": [
77,
78,
79,
80,
81,
82,
83,
84,
85,
86,
87,
88,
89,
90,
91,
92,
93,
94,
95,
96,
97
],
"label": "sym",
"score": 1,
"text": "位于隔束的两臂之间的室间隔缺损位于主动脉下"
},
{
"indices": [
79,
80
],
"label": "bod",
"score": 1,
"text": "隔束"
},
{
"indices": [
82,
83
],
"label": "bod",
"score": 1,
"text": "两臂"
},
{
"indices": [
87,
88,
89,
90,
91
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
94,
95,
96
],
"label": "bod",
"score": 1,
"text": "主动脉"
}
] |
在极少见的情况下，流出道室间隔明显发育不良甚至缺如时，位于隔束两臂间的室间隔缺损即为双动脉下。 | [
{
"indices": [
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "流出道室间隔明显发育不良"
},
{
"indices": [
23,
24
],
"label": "dis",
"score": 1,
"text": "缺如"
},
{
"indices": [
29,
30,
31,
32,
33
],
"label": "bod",
"score": 1,
"text": "隔束两臂间"
},
{
"indices": [
35,
36,
37,
38,
39
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
42,
43,
44,
45
],
"label": "bod",
"score": 1,
"text": "双动脉下"
}
] |
因此，在两根大血管的相互关系基本正常的情况下，室间隔缺损绝大多数是主动脉下，有时是双动脉下，较少见的有远离大动脉型。 | [
{
"indices": [
6,
7,
8
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
23,
24,
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36
],
"label": "sym",
"score": 1,
"text": "室间隔缺损绝大多数是主动脉下"
},
{
"indices": [
33,
34,
35
],
"label": "bod",
"score": 1,
"text": "主动脉"
},
{
"indices": [
38,
39,
40,
41,
42,
43,
44
],
"label": "sym",
"score": 1,
"text": "有时是双动脉下"
},
{
"indices": [
41,
42,
43
],
"label": "bod",
"score": 1,
"text": "双动脉"
},
{
"indices": [
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56
],
"label": "sym",
"score": 1,
"text": "较少见的有远离大动脉型"
},
{
"indices": [
53,
54,
55
],
"label": "bod",
"score": 1,
"text": "大动脉"
}
] |
至今尚未见到大动脉位置关系正常而室间隔缺损为肺动脉下的类型。 | [
{
"indices": [
6,
7,
8
],
"label": "bod",
"score": 1,
"text": "大动脉"
},
{
"indices": [
16,
17,
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
22,
23,
24
],
"label": "bod",
"score": 1,
"text": "肺动脉"
}
] |
当两根大血管基本平行且主动脉位于右侧时（Taussing-Bing畸形），两根大血管短轴连线与三尖瓣与动脉瓣间的心室漏斗部褶平行，仅有左位的肺动脉靠近室间隔缺损。 | [
{
"indices": [
4,
5
],
"label": "bod",
"score": 1,
"text": "血管"
},
{
"indices": [
11,
12,
13
],
"label": "bod",
"score": 1,
"text": "主动脉"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34
],
"label": "dis",
"score": 1,
"text": "Taussing-Bing畸形"
},
{
"indices": [
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63
],
"label": "sym",
"score": 1,
"text": "两根大血管短轴连线与三尖瓣与动脉瓣间的心室漏斗部褶平行"
},
{
"indices": [
39,
40,
41
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
47,
48,
49
],
"label": "bod",
"score": 1,
"text": "三尖瓣"
},
{
"indices": [
51,
52,
53,
54
],
"label": "bod",
"score": 1,
"text": "动脉瓣间"
},
{
"indices": [
56,
57,
58,
59,
60,
61,
62,
63
],
"label": "bod",
"score": 1,
"text": "心室漏斗部褶平行"
},
{
"indices": [
65,
66,
67,
68,
69,
70,
71,
72,
73,
74,
75,
76,
77,
78,
79
],
"label": "sym",
"score": 1,
"text": "仅有左位的肺动脉靠近室间隔缺损"
},
{
"indices": [
70,
71,
72
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
75,
76,
77,
78,
79
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
}
] |
因此，发育良好的流出道间隔与心室漏斗部褶融合形成了肺动脉下室间隔缺损，在很少情况下，流出道室间隔明显发育不良甚至缺如时，室间隔缺损即为双动脉下。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33
],
"label": "sym",
"score": 1,
"text": "发育良好的流出道间隔与心室漏斗部褶融合形成了肺动脉下室间隔缺损"
},
{
"indices": [
8,
9,
10,
11,
12
],
"label": "bod",
"score": 1,
"text": "流出道间隔"
},
{
"indices": [
14,
15,
16,
17,
18,
19
],
"label": "bod",
"score": 1,
"text": "心室漏斗部褶"
},
{
"indices": [
25,
26,
27,
28,
29,
30,
31,
32,
33
],
"label": "dis",
"score": 1,
"text": "肺动脉下室间隔缺损"
},
{
"indices": [
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53
],
"label": "dis",
"score": 1,
"text": "流出道室间隔明显发育不良"
},
{
"indices": [
56,
57
],
"label": "dis",
"score": 1,
"text": "缺如"
},
{
"indices": [
60,
61,
62,
63,
64
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
67,
68,
69
],
"label": "bod",
"score": 1,
"text": "双动脉"
}
] |
在双动脉下室间隔缺损时，有可能两根大血管都有骑跨，Brandt等称之为“双心室双出口”伴左位主动脉，流出道室隔亦可与隔束的前支融合，因此可以有室间隔缺损与右后位的肺动脉相通。 | [
{
"indices": [
1,
2,
3,
4,
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "双动脉下室间隔缺损"
},
{
"indices": [
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23
],
"label": "sym",
"score": 1,
"text": "有可能两根大血管都有骑跨"
},
{
"indices": [
17,
18,
19
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
36,
37,
38,
39,
40,
41
],
"label": "dis",
"score": 1,
"text": "双心室双出口"
},
{
"indices": [
44,
45,
46,
47,
48
],
"label": "bod",
"score": 1,
"text": "左位主动脉"
},
{
"indices": [
50,
51,
52,
53,
54
],
"label": "bod",
"score": 1,
"text": "流出道室隔"
},
{
"indices": [
50,
51,
52,
53,
54,
55,
56,
57,
58,
59,
60,
61,
62,
63,
64
],
"label": "sym",
"score": 1,
"text": "流出道室隔亦可与隔束的前支融合"
},
{
"indices": [
58,
59
],
"label": "bod",
"score": 1,
"text": "隔束"
},
{
"indices": [
71,
72,
73,
74,
75
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
71,
72,
73,
74,
75,
76,
77,
78,
79,
80,
81,
82,
83,
84,
85
],
"label": "sym",
"score": 1,
"text": "室间隔缺损与右后位的肺动脉相通"
},
{
"indices": [
77,
78,
79,
80,
81,
82,
83
],
"label": "bod",
"score": 1,
"text": "右后位的肺动脉"
}
] |
室间隔缺损的底部边界通常由隔束的两个支组成；左右边界可以是流出道间隔、心室游离壁或心室漏斗部褶，主要取决于室间隔缺损本身为主动脉下、肺动脉下或双动脉下。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20
],
"label": "sym",
"score": 1,
"text": "室间隔缺损的底部边界通常由隔束的两个支组成"
},
{
"indices": [
13,
14
],
"label": "bod",
"score": 1,
"text": "隔束"
},
{
"indices": [
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42,
43,
44,
45,
46
],
"label": "sym",
"score": 1,
"text": "左右边界可以是流出道间隔、心室游离壁或心室漏斗部褶"
},
{
"indices": [
29,
30,
31,
32,
33
],
"label": "bod",
"score": 1,
"text": "流出道间隔"
},
{
"indices": [
35,
36,
37,
38,
39
],
"label": "bod",
"score": 1,
"text": "心室游离壁"
},
{
"indices": [
41,
42,
43,
44,
45,
46
],
"label": "bod",
"score": 1,
"text": "心室漏斗部褶"
},
{
"indices": [
53,
54,
55,
56,
57
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
61,
62,
63,
64
],
"label": "bod",
"score": 1,
"text": "主动脉下"
},
{
"indices": [
66,
67,
68,
69
],
"label": "bod",
"score": 1,
"text": "肺动脉下"
},
{
"indices": [
71,
72,
73,
74
],
"label": "bod",
"score": 1,
"text": "双动脉下"
}
] |
同时心室漏斗部褶本身亦可影响右室双出口的类型。 | [
{
"indices": [
2,
3,
4,
5,
6,
7
],
"label": "bod",
"score": 1,
"text": "心室漏斗部褶"
},
{
"indices": [
14,
15,
16,
17,
18
],
"label": "dis",
"score": 1,
"text": "右室双出口"
}
] |
当该心室漏斗部褶与隔束的后支融合时，室间隔缺损即有了后下边缘，此时对外科医生非常重要，因为该肌束内有传导束并起保护作用。 | [
{
"indices": [
2,
3,
4,
5,
6,
7
],
"label": "bod",
"score": 1,
"text": "心室漏斗部褶"
},
{
"indices": [
9,
10,
11,
12,
13
],
"label": "bod",
"score": 1,
"text": "隔束的后支"
},
{
"indices": [
18,
19,
20,
21,
22
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29
],
"label": "sym",
"score": 1,
"text": "室间隔缺损即有了后下边缘"
},
{
"indices": [
34,
35
],
"label": "dep",
"score": 1,
"text": "外科"
},
{
"indices": [
46,
47
],
"label": "bod",
"score": 1,
"text": "肌束"
},
{
"indices": [
50,
51,
52
],
"label": "bod",
"score": 1,
"text": "传导束"
}
] |
相反，当后支未向心室-漏斗部褶延伸时，形成二、三尖瓣纤维连接，此时的室间隔缺损为膜周型，传导束通常多在缺损的后下边缘走行。 | [
{
"indices": [
4,
5
],
"label": "bod",
"score": 1,
"text": "后支"
},
{
"indices": [
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16
],
"label": "sym",
"score": 1,
"text": "后支未向心室-漏斗部褶延伸"
},
{
"indices": [
8,
9,
10,
11,
12,
13,
14
],
"label": "bod",
"score": 1,
"text": "心室-漏斗部褶"
},
{
"indices": [
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29
],
"label": "sym",
"score": 1,
"text": "形成二、三尖瓣纤维连接"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27
],
"label": "bod",
"score": 1,
"text": "二、三尖瓣纤维"
},
{
"indices": [
34,
35,
36,
37,
38
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
34,
35,
36,
37,
38,
39,
40,
41,
42
],
"label": "sym",
"score": 1,
"text": "室间隔缺损为膜周型"
},
{
"indices": [
44,
45,
46
],
"label": "bod",
"score": 1,
"text": "传导束"
},
{
"indices": [
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56,
57,
58,
59
],
"label": "sym",
"score": 1,
"text": "传导束通常多在缺损的后下边缘走行"
}
] |
但有一点可以用于区分，即是否在大动脉与房室瓣间有漏斗部肌性分隔。 | [
{
"indices": [
15,
16,
17
],
"label": "bod",
"score": 1,
"text": "大动脉"
},
{
"indices": [
19,
20,
21
],
"label": "bod",
"score": 1,
"text": "房室瓣"
},
{
"indices": [
24,
25,
26,
27,
28,
29,
30
],
"label": "bod",
"score": 1,
"text": "漏斗部肌性分隔"
}
] |
同样，当右室双出口伴有肺动脉瓣下室间隔缺损造成左心室血流直接进入肺动脉时，无论从血流动力学或解剖上均与完全性大血管转位相似，鉴别点是二尖瓣肺动脉瓣的纤维连接，如果存在纤维连接即为完全性大血管转位。 | [
{
"indices": [
4,
5,
6,
7,
8
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
11,
12,
13,
14
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣"
},
{
"indices": [
16,
17,
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
23,
24,
25
],
"label": "bod",
"score": 1,
"text": "左心室"
},
{
"indices": [
26
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
32,
33,
34
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
40
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
54,
55,
56
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
66,
67,
68
],
"label": "bod",
"score": 1,
"text": "二尖瓣"
},
{
"indices": [
69,
70,
71,
72,
73,
74,
75
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣的纤维"
},
{
"indices": [
83,
84
],
"label": "bod",
"score": 1,
"text": "纤维"
},
{
"indices": [
92,
93,
94
],
"label": "bod",
"score": 1,
"text": "大血管"
}
] |
同时，右室双出口合并心脾综合征并不少见，无脾综合征（双右心房结构）绝大多数合并有完全性肺静脉异位引流，多脾综合征（双左心房结构）常合并有体静脉的回流异常、特别是下腔静脉的奇静脉延续和共同房室瓣。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14
],
"label": "dis",
"score": 1,
"text": "右室双出口合并心脾综合征"
},
{
"indices": [
20,
21,
22,
23,
24
],
"label": "dis",
"score": 1,
"text": "无脾综合征"
},
{
"indices": [
40,
41,
42,
43,
44,
45,
46,
47,
48,
49
],
"label": "dis",
"score": 1,
"text": "完全性肺静脉异位引流"
},
{
"indices": [
51,
52,
53,
54,
55
],
"label": "dis",
"score": 1,
"text": "多脾综合征"
},
{
"indices": [
57,
58,
59,
60,
61,
62
],
"label": "sym",
"score": 1,
"text": "双左心房结构"
},
{
"indices": [
57,
58,
59,
60
],
"label": "bod",
"score": 1,
"text": "双左心房"
},
{
"indices": [
65,
66,
67,
68,
69,
70,
71,
72,
73,
74,
75
],
"label": "sym",
"score": 1,
"text": "合并有体静脉的回流异常"
},
{
"indices": [
68,
69,
70
],
"label": "bod",
"score": 1,
"text": "体静脉"
},
{
"indices": [
77,
78,
79,
80,
81,
82,
83,
84,
85,
86,
87,
88,
89,
90,
91,
92,
93,
94,
95
],
"label": "sym",
"score": 1,
"text": "特别是下腔静脉的奇静脉延续和共同房室瓣"
},
{
"indices": [
80,
81,
82,
83,
84,
85,
86,
87
],
"label": "bod",
"score": 1,
"text": "下腔静脉的奇静脉"
},
{
"indices": [
91,
92,
93,
94,
95
],
"label": "bod",
"score": 1,
"text": "共同房室瓣"
}
] |
而且还要注意窦房结异位。 | [
{
"indices": [
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "窦房结异位"
}
] |
【病理生理】室间隔缺损与大血管的相对关系、有无合并流出道狭窄及体肺循环的相对阻力决定了患儿的血流动力学状态。 | [
{
"indices": [
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
12,
13,
14
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
25,
26,
27,
28,
29
],
"label": "dis",
"score": 1,
"text": "流出道狭窄"
},
{
"indices": [
32,
33,
34
],
"label": "bod",
"score": 1,
"text": "肺循环"
}
] |
其血流动力学可类似于室间隔缺损、右心室型单心室、法洛四联症或完全性大血管转位。 | [
{
"indices": [
10,
11,
12,
13,
14
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
16,
17,
18,
19,
20,
21,
22
],
"label": "dis",
"score": 1,
"text": "右心室型单心室"
},
{
"indices": [
24,
25,
26,
27,
28
],
"label": "dis",
"score": 1,
"text": "法洛四联症"
},
{
"indices": [
30,
31,
32,
33,
34,
35,
36,
37
],
"label": "dis",
"score": 1,
"text": "完全性大血管转位"
}
] |
可有青紫、充血性心力衰竭或两者同时存在。 | [
{
"indices": [
2,
3
],
"label": "sym",
"score": 1,
"text": "青紫"
},
{
"indices": [
5,
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "充血性心力衰竭"
}
] |
几乎所有的患儿均有不同程度的低氧血症。 | [
{
"indices": [
14,
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "低氧血症"
}
] |
（一）室间隔缺损位置与氧饱和度关系：所有伴有肺动脉下室间隔缺损的患儿，无论伴或不伴有肺动脉狭窄或肺血管阻塞性疾病，肺循环氧饱和度均高于体循环氧饱和度。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
11,
12,
13,
14
],
"label": "ite",
"score": 1,
"text": "氧饱和度"
},
{
"indices": [
22,
23,
24,
25,
26,
27,
28,
29,
30
],
"label": "dis",
"score": 1,
"text": "肺动脉下室间隔缺损"
},
{
"indices": [
42,
43,
44,
45,
46
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
48,
49,
50,
51,
52,
53,
54,
55
],
"label": "dis",
"score": 1,
"text": "肺血管阻塞性疾病"
},
{
"indices": [
57,
58,
59,
60,
61,
62,
63
],
"label": "ite",
"score": 1,
"text": "肺循环氧饱和度"
},
{
"indices": [
57,
58,
59,
60,
61,
62,
63,
64,
65,
66,
67,
68,
69,
70,
71,
72,
73
],
"label": "sym",
"score": 1,
"text": "肺循环氧饱和度均高于体循环氧饱和度"
},
{
"indices": [
67,
68,
69,
70,
71,
72,
73
],
"label": "ite",
"score": 1,
"text": "体循环氧饱和度"
}
] |
伴主动脉下室间隔缺损时，约60%的患儿体循环氧饱和度高于肺循环，伴或不伴有肺动脉狭窄或肺血管阻塞性疾病并不一定影响其氧饱和度变化。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "伴主动脉下室间隔缺损"
},
{
"indices": [
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30
],
"label": "sym",
"score": 1,
"text": "约60%的患儿体循环氧饱和度高于肺循环"
},
{
"indices": [
19,
20,
21,
22,
23,
24,
25
],
"label": "ite",
"score": 1,
"text": "体循环氧饱和度"
},
{
"indices": [
28,
29,
30
],
"label": "bod",
"score": 1,
"text": "肺循环"
},
{
"indices": [
37,
38,
39,
40,
41
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
43,
44,
45,
46,
47,
48,
49,
50
],
"label": "dis",
"score": 1,
"text": "肺血管阻塞性疾病"
},
{
"indices": [
58,
59,
60,
61
],
"label": "ite",
"score": 1,
"text": "氧饱和度"
}
] |
伴双动脉下室间隔缺损时，肺循环氧饱和度多高于体循环氧饱和度。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "伴双动脉下室间隔缺损"
},
{
"indices": [
12,
13,
14,
15,
16,
17,
18
],
"label": "ite",
"score": 1,
"text": "肺循环氧饱和度"
},
{
"indices": [
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28
],
"label": "sym",
"score": 1,
"text": "肺循环氧饱和度多高于体循环氧饱和度"
},
{
"indices": [
22,
23,
24,
25,
26,
27,
28
],
"label": "ite",
"score": 1,
"text": "体循环氧饱和度"
}
] |
而伴远离大动脉的室间隔缺损时，体、肺循环氧饱和度差异较大。 | [
{
"indices": [
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "远离大动脉的室间隔缺损"
},
{
"indices": [
15,
16,
17,
18,
19,
20,
21,
22,
23
],
"label": "ite",
"score": 1,
"text": "体、肺循环氧饱和度"
},
{
"indices": [
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27
],
"label": "sym",
"score": 1,
"text": "体、肺循环氧饱和度差异较大"
}
] |
（二）心室、动脉压力由于主动脉发自右心室，右心室压力接近体循环。 | [
{
"indices": [
3,
4
],
"label": "bod",
"score": 1,
"text": "心室"
},
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19
],
"label": "sym",
"score": 1,
"text": "心室、动脉压力由于主动脉发自右心室"
},
{
"indices": [
6,
7
],
"label": "bod",
"score": 1,
"text": "动脉"
},
{
"indices": [
12,
13,
14
],
"label": "bod",
"score": 1,
"text": "主动脉"
},
{
"indices": [
17,
18,
19
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
21,
22,
23
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27,
28,
29,
30
],
"label": "sym",
"score": 1,
"text": "右心室压力接近体循环"
},
{
"indices": [
28,
29,
30
],
"label": "bod",
"score": 1,
"text": "体循环"
}
] |
当存在肺动脉瓣上或瓣下狭窄时，肺动脉压力将明显降低。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11,
12
],
"label": "dis",
"score": 1,
"text": "肺动脉瓣上或瓣下狭窄"
},
{
"indices": [
15,
16,
17,
18,
19,
20,
21,
22,
23,
24
],
"label": "sym",
"score": 1,
"text": "肺动脉压力将明显降低"
},
{
"indices": [
15,
16,
17,
18,
19
],
"label": "ite",
"score": 1,
"text": "肺动脉压力"
}
] |
【临床表现】患儿可有青紫、充血性心力衰竭的症状，亦可毫无症状。 | [
{
"indices": [
10,
11
],
"label": "sym",
"score": 1,
"text": "青紫"
},
{
"indices": [
13,
14,
15,
16,
17,
18,
19,
20,
21,
22
],
"label": "sym",
"score": 1,
"text": "充血性心力衰竭的症状"
},
{
"indices": [
13,
14,
15,
16,
17,
18,
19
],
"label": "dis",
"score": 1,
"text": "充血性心力衰竭"
}
] |
其他类型的右室双出口体肺循环平衡良好，往往在新生儿期后才逐渐出现青紫或缺氧发作。 | [
{
"indices": [
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33
],
"label": "sym",
"score": 1,
"text": "往往在新生儿期后才逐渐出现青紫"
},
{
"indices": [
35,
36
],
"label": "sym",
"score": 1,
"text": "缺氧"
}
] |
伴肺动脉下室间隔缺损的右室双出口常表现为安静时轻度青紫，哭吵后青紫加剧。 | [
{
"indices": [
1,
2,
3,
4,
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "肺动脉下室间隔缺损"
},
{
"indices": [
11,
12,
13,
14,
15
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
20,
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "安静时轻度青紫"
},
{
"indices": [
28,
29,
30,
31,
32,
33,
34
],
"label": "sym",
"score": 1,
"text": "哭吵后青紫加剧"
}
] |
右室双出口无特异性的体征。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "bod",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11
],
"label": "sym",
"score": 1,
"text": "右室双出口无特异性的体征"
}
] |
（一）主动脉下室间隔缺损伴肺动脉狭窄临床表现与法洛四联症相似。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损"
},
{
"indices": [
13,
14,
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
23,
24,
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "法洛四联症"
}
] |
第一心音正常，第二心音单一，有时在心尖区可及第三心音。 | [
{
"indices": [
0,
1,
2,
3,
4,
5
],
"label": "sym",
"score": 1,
"text": "第一心音正常"
},
{
"indices": [
2,
3
],
"label": "bod",
"score": 1,
"text": "心音"
},
{
"indices": [
7,
8,
9,
10,
11,
12
],
"label": "sym",
"score": 1,
"text": "第二心音单一"
},
{
"indices": [
9,
10
],
"label": "bod",
"score": 1,
"text": "心音"
},
{
"indices": [
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25
],
"label": "sym",
"score": 1,
"text": "有时在心尖区可及第三心音"
},
{
"indices": [
17,
18,
19
],
"label": "bod",
"score": 1,
"text": "心尖区"
},
{
"indices": [
24,
25
],
"label": "bod",
"score": 1,
"text": "心音"
}
] |
（二）肺动脉下室间隔缺损伴或不伴有肺动脉狭窄临床表现与完全性大血管转位伴室间隔缺损相似。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "肺动脉下室间隔缺损"
},
{
"indices": [
17,
18,
19,
20,
21
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
27,
28,
29,
30,
31,
32,
33,
34
],
"label": "dis",
"score": 1,
"text": "完全性大血管转位"
},
{
"indices": [
36,
37,
38,
39,
40
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
}
] |
通常在婴儿期即表现为青紫和充血性心力衰竭。 | [
{
"indices": [
10,
11
],
"label": "sym",
"score": 1,
"text": "青紫"
},
{
"indices": [
13,
14,
15,
16,
17,
18,
19
],
"label": "dis",
"score": 1,
"text": "充血性心力衰竭"
}
] |
如伴有肺动脉狭窄时，青紫出现时间更早但常无心力衰竭表现。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
10,
11,
12,
13,
14,
15,
16,
17
],
"label": "sym",
"score": 1,
"text": "青紫出现时间更早"
},
{
"indices": [
18,
19,
20,
21,
22,
23,
24,
25,
26
],
"label": "sym",
"score": 1,
"text": "但常无心力衰竭表现"
},
{
"indices": [
21,
22,
23,
24
],
"label": "dis",
"score": 1,
"text": "心力衰竭"
}
] |
肺血流量增加者可在心尖部闻及滚动样舒张期杂音。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21
],
"label": "sym",
"score": 1,
"text": "肺血流量增加者可在心尖部闻及滚动样舒张期杂音"
},
{
"indices": [
0,
1,
2,
3
],
"label": "bod",
"score": 1,
"text": "肺血流量"
},
{
"indices": [
9,
10,
11
],
"label": "bod",
"score": 1,
"text": "心尖部"
}
] |
（三）主动脉下室间隔缺损不伴肺动脉狭窄临床表现与大型室间隔缺损伴肺动脉高压类似。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损"
},
{
"indices": [
14,
15,
16,
17,
18
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
},
{
"indices": [
24,
25,
26,
27,
28,
29,
30
],
"label": "dis",
"score": 1,
"text": "大型室间隔缺损"
},
{
"indices": [
32,
33,
34,
35,
36
],
"label": "dis",
"score": 1,
"text": "肺动脉高压"
}
] |
通常青紫轻微但气急和充血性心力衰竭表现明显，有反复的上呼吸道感染。 | [
{
"indices": [
2,
3,
4,
5
],
"label": "sym",
"score": 1,
"text": "青紫轻微"
},
{
"indices": [
7,
8
],
"label": "sym",
"score": 1,
"text": "气急"
},
{
"indices": [
10,
11,
12,
13,
14,
15,
16
],
"label": "dis",
"score": 1,
"text": "充血性心力衰竭"
},
{
"indices": [
26,
27,
28,
29,
30,
31
],
"label": "dis",
"score": 1,
"text": "上呼吸道感染"
}
] |
（四）主动脉下室间隔缺损伴肺血管阻塞性疾病如上组类型伴有器质性肺高压时，此时肺动脉血流减少，心功能衰竭及反复呼吸道感染少见。 | [
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11
],
"label": "dis",
"score": 1,
"text": "主动脉下室间隔缺损"
},
{
"indices": [
13,
14,
15,
16,
17,
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "肺血管阻塞性疾病"
},
{
"indices": [
28,
29,
30,
31,
32,
33
],
"label": "dis",
"score": 1,
"text": "器质性肺高压"
},
{
"indices": [
38,
39,
40
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
38,
39,
40,
41,
42,
43,
44
],
"label": "sym",
"score": 1,
"text": "肺动脉血流减少"
},
{
"indices": [
46,
47,
48,
49,
50
],
"label": "dis",
"score": 1,
"text": "心功能衰竭"
},
{
"indices": [
52,
53,
54,
55,
56,
57,
58
],
"label": "sym",
"score": 1,
"text": "反复呼吸道感染"
},
{
"indices": [
54,
55,
56,
57,
58
],
"label": "dis",
"score": 1,
"text": "呼吸道感染"
}
] |
可出现青紫和杵状指（趾）。 | [
{
"indices": [
3,
4
],
"label": "sym",
"score": 1,
"text": "青紫"
},
{
"indices": [
6,
7,
8,
9,
10,
11
],
"label": "sym",
"score": 1,
"text": "杵状指（趾）"
},
{
"indices": [
8
],
"label": "bod",
"score": 1,
"text": "指"
},
{
"indices": [
10
],
"label": "bod",
"score": 1,
"text": "趾"
}
] |
体格检查常无杂音闻及，第二心音响亮而单一，同时可及肺动脉反流所致的舒张期杂音。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7,
8,
9
],
"label": "sym",
"score": 1,
"text": "体格检查常无杂音闻及"
},
{
"indices": [
0,
1,
2,
3
],
"label": "pro",
"score": 1,
"text": "体格检查"
},
{
"indices": [
11,
12,
13,
14,
15,
16,
17,
18,
19
],
"label": "sym",
"score": 1,
"text": "第二心音响亮而单一"
},
{
"indices": [
13,
14
],
"label": "bod",
"score": 1,
"text": "心音"
},
{
"indices": [
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37
],
"label": "sym",
"score": 1,
"text": "可及肺动脉反流所致的舒张期杂音"
},
{
"indices": [
25,
26,
27,
28,
29
],
"label": "dis",
"score": 1,
"text": "肺动脉反流"
}
] |
【辅助检查】（一）心电图常表现为窦性心律、电轴右偏及不同程度的右心室肥大，右胸前导联QRS波常表现为qR型。 | [
{
"indices": [
9,
10,
11
],
"label": "pro",
"score": 1,
"text": "心电图"
},
{
"indices": [
16,
17,
18,
19
],
"label": "sym",
"score": 1,
"text": "窦性心律"
},
{
"indices": [
18,
19
],
"label": "bod",
"score": 1,
"text": "心律"
},
{
"indices": [
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35
],
"label": "sym",
"score": 1,
"text": "电轴右偏及不同程度的右心室肥大"
},
{
"indices": [
31,
32,
33
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52
],
"label": "sym",
"score": 1,
"text": "右胸前导联QRS波常表现为qR型"
},
{
"indices": [
37,
38
],
"label": "bod",
"score": 1,
"text": "右胸"
}
] |
左心室常不扩大，如果有明显的左心室大则提示有可能合并限制性的室间隔缺损。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6
],
"label": "sym",
"score": 1,
"text": "左心室常不扩大"
},
{
"indices": [
0,
1,
2
],
"label": "bod",
"score": 1,
"text": "左心室"
},
{
"indices": [
14,
15,
16,
17
],
"label": "dis",
"score": 1,
"text": "左心室大"
},
{
"indices": [
26,
27,
28,
29,
30,
31,
32,
33,
34
],
"label": "dis",
"score": 1,
"text": "限制性的室间隔缺损"
}
] |
（二）胸部X线无特征性的改变，心脏大小及肺血多少取决于当时的血流动力学状态，可为小心脏，肺血减少，也可表现为大心脏，肺血明显增多。 | [
{
"indices": [
3,
4,
5,
6
],
"label": "pro",
"score": 1,
"text": "胸部X线"
},
{
"indices": [
3,
4,
5,
6,
7,
8,
9,
10,
11,
12,
13
],
"label": "sym",
"score": 1,
"text": "胸部X线无特征性的改变"
},
{
"indices": [
15,
16
],
"label": "bod",
"score": 1,
"text": "心脏"
},
{
"indices": [
20,
21
],
"label": "bod",
"score": 1,
"text": "肺血"
},
{
"indices": [
38,
39,
40,
41,
42
],
"label": "sym",
"score": 1,
"text": "可为小心脏"
},
{
"indices": [
41,
42
],
"label": "bod",
"score": 1,
"text": "心脏"
},
{
"indices": [
44,
45,
46,
47
],
"label": "sym",
"score": 1,
"text": "肺血减少"
},
{
"indices": [
44,
45
],
"label": "bod",
"score": 1,
"text": "肺血"
},
{
"indices": [
49,
50,
51,
52,
53,
54,
55,
56
],
"label": "sym",
"score": 1,
"text": "也可表现为大心脏"
},
{
"indices": [
55,
56
],
"label": "bod",
"score": 1,
"text": "心脏"
},
{
"indices": [
58,
59,
60,
61,
62,
63
],
"label": "sym",
"score": 1,
"text": "肺血明显增多"
},
{
"indices": [
58,
59
],
"label": "bod",
"score": 1,
"text": "肺血"
}
] |
（三）超声心动图剑突下扫查对诊断右室双出口非常有用（图9-9），诊断标准为两根大血管全部或大部分发自右心室，双圆锥常见但并不是诊断的必须条件。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "pro",
"score": 1,
"text": "超声心动图"
},
{
"indices": [
8,
9
],
"label": "bod",
"score": 1,
"text": "剑突"
},
{
"indices": [
16,
17,
18,
19,
20
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
39,
40,
41
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
50,
51,
52
],
"label": "bod",
"score": 1,
"text": "右心室"
}
] |
胸骨旁长轴及短轴可检测大血管的相互关系及动脉下圆锥。 | [
{
"indices": [
0,
1,
2,
3,
4,
5,
6,
7
],
"label": "bod",
"score": 1,
"text": "胸骨旁长轴及短轴"
},
{
"indices": [
11,
12,
13
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
20,
21,
22,
23,
24
],
"label": "bod",
"score": 1,
"text": "动脉下圆锥"
}
] |
由于右心室双出口的解剖类型变化较多且伴发畸形多样，必须进行全面的循序分段诊断。 | [
{
"indices": [
2,
3,
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "右心室双出口"
},
{
"indices": [
20,
21
],
"label": "dis",
"score": 1,
"text": "畸形"
}
] |
（四）心导管造影在心外手术以前，通常需要通过心导管及造影证实超声心动图诊断并获得详细的血流动力学信息。 | [
{
"indices": [
3,
4,
5,
6,
7
],
"label": "pro",
"score": 1,
"text": "心导管造影"
},
{
"indices": [
9,
10,
11,
12
],
"label": "pro",
"score": 1,
"text": "心外手术"
},
{
"indices": [
22,
23,
24
],
"label": "equ",
"score": 1,
"text": "心导管"
},
{
"indices": [
26,
27
],
"label": "pro",
"score": 1,
"text": "造影"
},
{
"indices": [
30,
31,
32,
33,
34
],
"label": "pro",
"score": 1,
"text": "超声心动图"
}
] |
其血流动力学表现可类似于大分流量的左向右分流、右心室型单心室、法洛四联症、完全性大血管转位及肺动脉狭窄。 | [
{
"indices": [
23,
24,
25,
26,
27,
28,
29
],
"label": "dis",
"score": 1,
"text": "右心室型单心室"
},
{
"indices": [
31,
32,
33,
34,
35
],
"label": "dis",
"score": 1,
"text": "法洛四联症"
},
{
"indices": [
37,
38,
39,
40,
41,
42,
43,
44
],
"label": "dis",
"score": 1,
"text": "完全性大血管转位"
},
{
"indices": [
46,
47,
48,
49,
50
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
}
] |
超声心动图所获得的信息可用于选择合适的透照位置以明确大血管的起源及室间隔缺损的位置。 | [
{
"indices": [
0,
1,
2,
3,
4
],
"label": "pro",
"score": 1,
"text": "超声心动图"
},
{
"indices": [
26,
27,
28
],
"label": "bod",
"score": 1,
"text": "大血管"
},
{
"indices": [
33,
34,
35,
36,
37
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
}
] |
由于大动脉多为侧侧关系且半月瓣在同一水平，采用双向DSA行前后位及侧位投照较为理想（图9-9）。 | [
{
"indices": [
2,
3,
4
],
"label": "bod",
"score": 1,
"text": "大动脉"
},
{
"indices": [
12,
13,
14
],
"label": "bod",
"score": 1,
"text": "半月瓣"
},
{
"indices": [
23,
24,
25,
26,
27
],
"label": "pro",
"score": 1,
"text": "双向DSA"
}
] |
图9-30右室双出口经剑突下超声心动图主动脉、肺动脉分别位于右、左侧，半月瓣位于同一水平，均发自右心室，可见双圆锥，右心室肥厚明显图9-31右室双出口右心室造影主肺动脉均发自右心室，主动脉瓣位于肺动脉瓣的右前方且与肺动脉瓣在同一水平（A.右心室前后位造影；B.右心室侧位造影）【治疗】在治疗以前必须考虑以下几点：双心室修补是否可能？ | [
{
"indices": [
5,
6,
7,
8,
9
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
5,
6,
7,
8,
9,
10,
11,
12,
13,
14,
15,
16,
17,
18,
19,
20,
21,
22,
23,
24,
25,
26,
27,
28,
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42,
43
],
"label": "sym",
"score": 1,
"text": "右室双出口经剑突下超声心动图主动脉、肺动脉分别位于右、左侧，半月瓣位于同一水平"
},
{
"indices": [
11,
12
],
"label": "bod",
"score": 1,
"text": "剑突"
},
{
"indices": [
14,
15,
16,
17,
18
],
"label": "pro",
"score": 1,
"text": "超声心动图"
},
{
"indices": [
19,
20,
21
],
"label": "bod",
"score": 1,
"text": "主动脉"
},
{
"indices": [
23,
24,
25
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
35,
36,
37
],
"label": "bod",
"score": 1,
"text": "半月瓣"
},
{
"indices": [
45,
46,
47,
48,
49,
50
],
"label": "sym",
"score": 1,
"text": "均发自右心室"
},
{
"indices": [
48,
49,
50
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
54,
55,
56
],
"label": "sym",
"score": 1,
"text": "双圆锥"
},
{
"indices": [
58,
59,
60,
61,
62,
63,
64
],
"label": "sym",
"score": 1,
"text": "右心室肥厚明显"
},
{
"indices": [
58,
59,
60
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
70,
71,
72,
73,
74
],
"label": "dis",
"score": 1,
"text": "右室双出口"
},
{
"indices": [
70,
71,
72,
73,
74,
75,
76,
77,
78,
79,
80,
81,
82,
83,
84,
85,
86,
87,
88,
89
],
"label": "sym",
"score": 1,
"text": "右室双出口右心室造影主肺动脉均发自右心室"
},
{
"indices": [
75,
76,
77,
78,
79
],
"label": "pro",
"score": 1,
"text": "右心室造影"
},
{
"indices": [
80,
81,
82,
83
],
"label": "bod",
"score": 1,
"text": "主肺动脉"
},
{
"indices": [
87,
88,
89
],
"label": "pro",
"score": 1,
"text": "右心室"
},
{
"indices": [
91,
92,
93,
94
],
"label": "bod",
"score": 1,
"text": "主动脉瓣"
},
{
"indices": [
91,
92,
93,
94,
95,
96,
97,
98,
99,
100,
101,
102,
103,
104,
105,
106,
107,
108,
109,
110,
111,
112,
113,
114,
115
],
"label": "sym",
"score": 1,
"text": "主动脉瓣位于肺动脉瓣的右前方且与肺动脉瓣在同一水平"
},
{
"indices": [
97,
98,
99,
100
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣"
},
{
"indices": [
107,
108,
109,
110
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣"
},
{
"indices": [
119,
120,
121,
122,
123,
124,
125,
126
],
"label": "pro",
"score": 1,
"text": "右心室前后位造影"
},
{
"indices": [
130,
131,
132,
133,
134,
135,
136
],
"label": "pro",
"score": 1,
"text": "右心室侧位造影"
},
{
"indices": [
156,
157,
158,
159,
160
],
"label": "pro",
"score": 1,
"text": "双心室修补"
}
] |
心室发育是否正常？ | [
{
"indices": [
0,
1
],
"label": "bod",
"score": 1,
"text": "心室"
}
] |
是否仅可进行Fantan一类的手术？ | [
{
"indices": [
6,
7,
8,
9,
10,
11
],
"label": "pro",
"score": 1,
"text": "Fantan"
}
] |
如果有可能进行双心室修补，是否存在肺动脉狭窄？ | [
{
"indices": [
7,
8,
9,
10,
11
],
"label": "pro",
"score": 1,
"text": "双心室修补"
},
{
"indices": [
17,
18,
19,
20,
21
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
}
] |
是否需要人工带瓣管道？ | [
{
"indices": [
4,
5,
6,
7,
8,
9
],
"label": "equ",
"score": 1,
"text": "人工带瓣管道"
}
] |
在小婴儿，由于生长发育较快，一般须避免使用人工带瓣管道。 | [
{
"indices": [
21,
22,
23,
24,
25,
26
],
"label": "equ",
"score": 1,
"text": "人工带瓣管道"
}
] |
手术而不采用以后必须更换的人工带瓣管道。 | [
{
"indices": [
0,
1
],
"label": "pro",
"score": 1,
"text": "手术"
},
{
"indices": [
13,
14,
15,
16,
17,
18
],
"label": "equ",
"score": 1,
"text": "人工带瓣管道"
}
] |
在室间隔缺损关闭后，是否有可能进行循环的转换手术，如有可能，是否可进行大动脉转换术（如冠状动脉是否适合做大动脉转换术）？ | [
{
"indices": [
1,
2,
3,
4,
5
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
20,
21,
22,
23
],
"label": "pro",
"score": 1,
"text": "转换手术"
},
{
"indices": [
35,
36,
37,
38,
39,
40
],
"label": "pro",
"score": 1,
"text": "大动脉转换术"
},
{
"indices": [
43,
44,
45,
46
],
"label": "bod",
"score": 1,
"text": "冠状动脉"
},
{
"indices": [
52,
53,
54,
55,
56,
57
],
"label": "pro",
"score": 1,
"text": "大动脉转换术"
}
] |
有时关闭室间隔缺损可使患儿得到生存，而有时关闭室间隔缺损并解除肺动脉狭窄可得到良好的效果。 | [
{
"indices": [
23,
24,
25,
26,
27
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
31,
32,
33,
34,
35
],
"label": "dis",
"score": 1,
"text": "肺动脉狭窄"
}
] |
一般主张在2岁以后才作根治术，手术的目的是：①建立左心室与主动脉间的通道：用Dacron补片在室间隔缺损与主动脉瓣下流出道间建立人工隧道，注意避免此连接的梗阻，有时需扩大室间隔缺损；②建立右心室与肺动脉间的通路：可通过右心室流出道扩大补片、肺动脉瓣切开或在右心室与肺动脉间用人工带瓣管道连接；③修复伴发畸形。 | [
{
"indices": [
11,
12,
13
],
"label": "pro",
"score": 1,
"text": "根治术"
},
{
"indices": [
25,
26,
27
],
"label": "bod",
"score": 1,
"text": "左心室"
},
{
"indices": [
29,
30,
31
],
"label": "bod",
"score": 1,
"text": "主动脉"
},
{
"indices": [
38,
39,
40,
41,
42,
43,
44,
45
],
"label": "equ",
"score": 1,
"text": "Dacron补片"
},
{
"indices": [
47,
48,
49,
50,
51
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
53,
54,
55,
56,
57,
58,
59,
60
],
"label": "bod",
"score": 1,
"text": "主动脉瓣下流出道"
},
{
"indices": [
62,
63,
64,
65,
66,
67
],
"label": "pro",
"score": 1,
"text": "建立人工隧道"
},
{
"indices": [
77,
78
],
"label": "dis",
"score": 1,
"text": "梗阻"
},
{
"indices": [
85,
86,
87,
88,
89
],
"label": "dis",
"score": 1,
"text": "室间隔缺损"
},
{
"indices": [
94,
95,
96
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
98,
99,
100
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
109,
110,
111
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
112,
113,
114
],
"label": "bod",
"score": 1,
"text": "流出道"
},
{
"indices": [
117,
118
],
"label": "equ",
"score": 1,
"text": "补片"
},
{
"indices": [
120,
121,
122,
123
],
"label": "bod",
"score": 1,
"text": "肺动脉瓣"
},
{
"indices": [
128,
129,
130
],
"label": "bod",
"score": 1,
"text": "右心室"
},
{
"indices": [
132,
133,
134
],
"label": "bod",
"score": 1,
"text": "肺动脉"
},
{
"indices": [
137,
138,
139,
140,
141,
142
],
"label": "equ",
"score": 1,
"text": "人工带瓣管道"
},
{
"indices": [
151,
152
],
"label": "dis",
"score": 1,
"text": "畸形"
}
] |
最近报道，右心室双出口在新生儿期行根治术的死亡率可低至4%～8%。 | [
{
"indices": [
5,
6,
7,
8,
9,
10
],
"label": "dis",
"score": 1,
"text": "右心室双出口"
},
{
"indices": [
17,
18,
19
],
"label": "pro",
"score": 1,
"text": "根治术"
}
] |
四、新生儿常用基本通气模式（一）持续气道正压持续气道正压（continuouspositiveairwaypressure，CPAP）也称自主呼吸（spontaneousbreathing，Spont），是指有自主呼吸的婴儿在整个呼吸周期中（吸气和呼气）接受呼吸机供给的高于大气压的气体压力，其作用为吸气时气体易于进入肺内，减少呼吸功；呼气时可防止病变肺泡萎陷，增加FRC，改善肺泡通气、血流比，从而升高PaO<sub>2</sub>。 | [
{
"indices": [
16,
17,
18,
19,
20,
21
],
"label": "pro",
"score": 1,
"text": "持续气道正压"
},
{
"indices": [
22,
23,
24,
25,
26,
27
],
"label": "pro",
"score": 1,
"text": "持续气道正压"
},
{
"indices": [
29,
30,
31,
32,
33,
34,
35,
36,
37,
38,
39,
40,
41,
42,
43,
44,
45,
46,
47,
48,
49,
50,
51,
52,
53,
54,
55,
56,
57,
58,
59,
60
],
"label": "pro",
"score": 1,
"text": "continuouspositiveairwaypressure"
},
{
"indices": [
62,
63,
64,
65
],
"label": "pro",
"score": 1,
"text": "CPAP"
},
{
"indices": [
69,
70,
71,
72
],
"label": "pro",
"score": 1,
"text": "自主呼吸"
},
{
"indices": [
74,
75,
76,
77,
78,
79,
80,
81,
82,
83,
84,
85,
86,
87,
88,
89,
90,
91,
92,
93
],
"label": "pro",
"score": 1,
"text": "spontaneousbreathing"
},
{
"indices": [
95,
96,
97,
98,
99
],
"label": "pro",
"score": 1,
"text": "Spont"
},
{
"indices": [
129,
130,
131
],
"label": "equ",
"score": 1,
"text": "呼吸机"
},
{
"indices": [
159,
160
],
"label": "bod",
"score": 1,
"text": "肺内"
},
{
"indices": [
174,
175,
176,
177
],
"label": "bod",
"score": 1,
"text": "病变肺泡"
},
{
"indices": [
174,
175,
176,
177,
178,
179
],
"label": "sym",
"score": 1,
"text": "病变肺泡萎陷"
},
{
"indices": [
183,
184,
185
],
"label": "ite",
"score": 1,
"text": "FRC"
},
{
"indices": [
189,
190
],
"label": "bod",
"score": 1,
"text": "肺泡"
},
{
"indices": [
194
],
"label": "bod",
"score": 1,
"text": "血"
}
] |
主要用于低氧血症、轻型RDS和频发的呼吸暂停。 | [
{
"indices": [
4,
5,
6,
7
],
"label": "dis",
"score": 1,
"text": "低氧血症"
},
{
"indices": [
9,
10,
11,
12,
13
],
"label": "dis",
"score": 1,
"text": "轻型RDS"
},
{
"indices": [
18,
19,
20,
21
],
"label": "dis",
"score": 1,
"text": "呼吸暂停"
}
] |
多主张应用鼻塞CPAP，但因易吞入空气导致腹胀，使用时应放置胃管以排气；经气管插管作CPAP，可增加气道阻力和呼吸功，只是在应用或撤离呼吸机前的短时间内应用。 | [
{
"indices": [
5,
6,
7,
8,
9,
10
],
"label": "pro",
"score": 1,
"text": "鼻塞CPAP"
},
{
"indices": [
21,
22
],
"label": "sym",
"score": 1,
"text": "腹胀"
},
{
"indices": [
21
],
"label": "bod",
"score": 1,
"text": "腹"
},
{
"indices": [
30,
31
],
"label": "equ",
"score": 1,
"text": "胃管"
},
{
"indices": [
37,
38,
39,
40
],
"label": "pro",
"score": 1,
"text": "气管插管"
},
{
"indices": [
42,
43,
44,
45
],
"label": "pro",
"score": 1,
"text": "CPAP"
},
{
"indices": [
50,
51
],
"label": "bod",
"score": 1,
"text": "气道"
},
{
"indices": [
67,
68,
69
],
"label": "equ",
"score": 1,
"text": "呼吸机"
}
] |
压力一般为3～8cmH<sub>2</sub>O，压力＞8cmH<sub>2</sub>O（尤其当肺顺应性改善时）可影响静脉回流及降低心排血量，还会造成潮气量减低和PaCO<sub>2</sub>升高。 | [
{
"indices": [
49
],
"label": "bod",
"score": 1,
"text": "肺"
},
{
"indices": [
58,
59,
60,
61,
62,
63
],
"label": "sym",
"score": 1,
"text": "影响静脉回流"
},
{
"indices": [
60,
61
],
"label": "bod",
"score": 1,
"text": "静脉"
},
{
"indices": [
65,
66,
67,
68,
69,
70
],
"label": "sym",
"score": 1,
"text": "降低心排血量"
},
{
"indices": [
67
],
"label": "bod",
"score": 1,
"text": "心"
},
{
"indices": [
69
],
"label": "bod",
"score": 1,
"text": "血"
},
{
"indices": [
76,
77,
78
],
"label": "ite",
"score": 1,
"text": "潮气量"
},
{
"indices": [
76,
77,
78,
79,
80
],
"label": "sym",
"score": 1,
"text": "潮气量减低"
},
{
"indices": [
82,
83,
84,
85,
86,
87,
88,
89,
90,
91,
92,
93,
94,
95,
96,
97,
98,
99
],
"label": "sym",
"score": 1,
"text": "PaCO<sub>2</sub>升高"
},
{
"indices": [
82,
83,
84,
85
],
"label": "ite",
"score": 1,
"text": "PaCO"
}
] |
CPAP不宜使用纯氧作气源。 | [
{
"indices": [
0,
1,
2,
3
],
"label": "pro",
"score": 1,
"text": "CPAP"
}
] |