儿科
词汇介绍
拓展阅读
解析
tetralogy 英 /tɪ'trælədʒɪ/ 美 /te'trælədʒi, tə-/
释 义 n. 四部曲;四联剧
例 句 The percutaneous balloon dilatation of the stenotic right outflow tract is one of the effective palliative therapies for infants with tetralogy of Fallot. 经皮球囊导管扩张狭窄的右心室流出口,是对法洛四联症患儿合并缺氧发作的有效姑息疗法之一。
Fallot
释 义 n. (Fallot)人名;(法)法洛
例 句 Cardiac defects included 63 Fallot's Tetralogy, 5 double-outlet of right ventricle, 4 anomalous pulmonary venous and 10 other defects. 病种包括法洛四联症63例,右心室双出口5例,肺静脉畸形引流4例,其他病变10例。
概述
概述
法洛四联症 (TOF)是婴儿期后最常见的青紫型先天性心脏病,约占所有先天性心脏病的12%。法洛四联症由4种畸形组成,右心室流出道梗阻、室间隔缺损、主动脉骑跨、右心室肥厚,此外本病可合并其他心血管畸形,如25%的法洛四联症患儿为右位型主动脉弓;其他如左上腔静脉残留、冠状动脉异常、房间隔缺损、动脉导管未闭、肺动脉瓣缺如等。
病理解剖
右心室流出道梗:狭窄范围可自右心室漏斗部入口至左、右肺动脉分支。可为漏斗部狭窄、动脉瓣狭窄或两者同时存在。常有肺动脉瓣环、肺动脉总干的发育不良和肺动脉分支的非对称性狭窄。狭窄的严重程度差异较大。
室间隔缺损:缺损为膜部周围型缺损并向流出道延伸,多位于主动脉下,有时可向肺动下方延伸,称对位不良型室间隔缺损。
主动脉骑跨:主动脉根部粗大且顺钟向旋转右移并骑跨在室间隔缺损上,骑跨范围在15%-95%。
右心室肥厚:属继发性病变。
临床表现
青紫:为其主要表现,其程度和出现的早晚与肺动脉狭窄程度有关。多见于毛细血管丰富的浅表部位,如唇、指(趾)甲床、球结合膜等。因血氧含量下降,活动耐力差,稍一活动,如啼哭、情绪激动、体力劳动、寒冷等,即可出现气急及青紫加重。
蹲踞症状:患儿多有蹲踞症状,每于行走、游戏时常主动下蹲片刻。蹲踞时下肢屈曲,使静脉回心血量减少,减轻了心脏负荷,同时下肢动脉受压,体循环阻力增加,使右向左分流量减少,缺氧症状暂时得以缓解。不会行走的小婴儿常喜欢大人抱起,双下肢屈曲状。
杵状指(趾): 患儿长期处于缺氧环境中,可使指(趾)端毛细血管扩张增生,局部软组织和骨组织也增生肥大,表现为指(趾)端膨大如鼓槌状。
阵发性缺氧发作:多见于婴儿,发生的诱因为吃奶、哭闹、情绪激动、贫血、感染等。表现为阵发性呼吸困难,严重者可引起突然昏厥、抽搐、甚至死亡。其原因是由于在肺动脉漏斗部狭窄的基础上突然发生该处肌部痉挛引起一时性肺动脉梗阻,使脑缺氧加重。年长儿常诉头痛、头晕。
辅助检查
①血液检查;②X线检查;③心电图;④超声心动图;⑤心导管和心血管造影 。
Echo-Doppler Assessment of Left Filling Pressures in Adults With Repaired Tetralogy of Fallot复制标题
多普勒超声评价成人法洛四联症修复后左侧充盈压
发表时间:2019-08-01
影响指数:5.8
作者: William R. Miranda
期刊:Circ Cardiovasc Imaging
The prevalence of left ventricular (LV) diastolic dysfunction is expected to rise as patients with tetralogy of Fallot (ToF) age. Elevated LV filling pressures by cardiac catheterization carry worse prognosis in this population,1 but data on echo-Doppler assessment of LV diastolic function/filling pressures in adults with ToF are limited. We identified 48 adults (age ≥18 years) with repaired ToF undergoing right heart catheterization and transthoracic echocardiography within 7 days (median, −1 day [−1 to −2]) between June 2004 and December 2018. None had prior mitral valve replacement/repair. Echocardiographic and invasive hemodynamic data were abstracted from the reports. Cardiac catheterization was performed in a fasting state and elevated LV filling pressures defined as pulmonary artery wedge pressure (PAWP) >12 mmHg.2 Data are presented as counts, mean±SD, or medians (25th to 75th percentile). Groups were compared using t test and Wilcoxon test for continuous data and χ2 and Fisher exact tests for nominal variables, whichever appropriate.
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