ACM Advances in Clinical Medicine 2161-8712 Scientific Research Publishing 10.12677/ACM.2022.124533 ACM-50951 ACM20220400000_94351877.pdf 医药卫生 法洛四联症根治术右室流出道疏通程度与近中期预后关系 The Relationship between Right Ventricular Outflow Tract Dredging Degree and Short-to-Mid-Term Results in Children with Repaired Tetralogy of Fallot 母 炜浩 2 1 安 永 2 1 重庆医科大学附属儿童医院胸心外科，重庆 null 01 04 2022 12 04 3696 3702 © Copyright 2014 by authors and Scientific Research Publishing Inc. 2014 This work is licensed under the Creative Commons Attribution International License (CC BY). http://creativecommons.org/licenses/by/4.0/

目的：分析法洛四联症根治术患儿的右室流出道疏通程度与近中期预后的关系。方法：回顾性分析2016年1月至2020年12月重庆医科大学附属儿童医院行法洛四联症根治术患儿的临床特点，统计并分析法洛四联症根治术后发生右室流出道再狭窄、低心排综合征、中重度肺动脉反流(Pulmonary Regurgitation, PR)与右室流出道(Right Ventricular Outflow Tract, RVOT)疏通程度的关系。结果：研究共纳入93例患儿，其中男61例、女32例，年龄(14.8 ± 10.0)个月，体重(9.2 ± 2.2) kg，术后发生右室流出道再狭窄3例、低心排综合征28例、中重度PR 11例，根据是否发生右室流出道再狭窄将患儿分为狭窄组和非狭窄组，再狭窄组的RVOT疏通程度小于非狭窄组(67.5% ± 16.3% vs 31.8% ± 16.4%, P < 0.001)。根据是否发生低心排综合征将患儿分为低心排综合征组和未发生低心排综合征组，两者RVOT疏通程度差异无统计学意义(67.23% ± 17.02% vs 64.26% ± 18.35%, P = 0.453)。根据是否生中重度以上PR将患儿分为中重度PR组与非中重度PR组，两者RVOT疏通程度差异无统计学意义(66.5%±17% vs 65.1% ± 21.1%, P = 0.803)。结论：TOF根治术右室流出道疏通程度越大，患者近中期越不易发生RVOT再狭窄。本中心常规手术后右室流出道疏通程度在60%左右，患者在近中期随访出现中重度肺动脉反流、低心排综合征的频率较低，但远期结局需进一步随访研究。 Objective: To analyze the relationship between right ventricular outflow tract dredging degree and short-to-mid-term results in children with TOF. Methods: A retrospective analysis was made on the clinical characteristics of children undergoing radical operation for tetralogy of Fallot in Children’s Hospital Affiliated to Chongqing Medical University from January 2016 to December 2020. The relationship between right ventricular outflow tract restenosis, low cardiac output syndrome, moderate or severe PR and right ventricular outflow tract dredging degree after radical operation for tetralogy of Fallot was statistically analyzed. Results: A total of 93 children were included in the study, including 61 males and 32 females, with an average age of 14.8 ± 10.0 years and average weight of 9.2 ± 2.2 kg. There were 3 cases of right ventricular outflow tract restenosis, 28 cases of low cardiac output syndrome and 11 cases of moderate or severe PR after operation. The children were divided into stenosis group and non-stenosis group according to the occurrence of right ventricular outflow tract stenosis. The degree of RVOT recanalization in restenosis group was lower than that in non-stenosis group (67.5% ± 16.3% vs 31.8% ± 16.4%, P < 0.001). According to the occurrence of low cardiac output syndrome, the children were divided into low cardiac output syndrome group and non-low cardiac output syndrome group. There was no significant difference in the degree of RVOT patency between the two groups (67.23% ± 17.02% vs 64.26% ± 18.35%, P = 0.453). The children were divided into moderate or severe PR group and non-moderate to severe PR group according to whether they had moderate or severe RVOT. There was no significant difference in the degree of RVOT dredging between the two groups (66.5% ± 17% vs 65.1% ± 21.1%, P = 0.803). Conclusion: The greater the recanalization degree of the right ventricular outflow tract after TOF operation is, the less likely the patients to develop RVOT restenosis in the short-to-medium term are. The degree of dredging of the right ventricular outflow tract after routine surgery in our center is about 60%. The frequency of moderate-to-severe pulmonary regurgitation and low cardiac output syndrome is low in the short-to-medium term follow-up, but the long-term outcome needs further study.

 法洛四联症，右室流出道再狭窄，低心排综合征，肺动脉反流, Tetralogy of Fallot Right Ventricular Outflow Tract Restenosis Low Cardiac Output Syndrome Pulmonary Regurgitation 摘要

目的：分析法洛四联症根治术患儿的右室流出道疏通程度与近中期预后的关系。方法：回顾性分析2016年1月至2020年12月重庆医科大学附属儿童医院行法洛四联症根治术患儿的临床特点，统计并分析法洛四联症根治术后发生右室流出道再狭窄、低心排综合征、中重度肺动脉反流(Pulmonary Regurgitation, PR)与右室流出道(Right Ventricular Outflow Tract, RVOT)疏通程度的关系。结果：研究共纳入93例患儿，其中男61例、女32例，年龄(14.8 ± 10.0)个月，体重(9.2 ± 2.2) kg，术后发生右室流出道再狭窄3例、低心排综合征28例、中重度PR 11例，根据是否发生右室流出道再狭窄将患儿分为狭窄组和非狭窄组，再狭窄组的RVOT疏通程度小于非狭窄组(67.5% ± 16.3% vs 31.8% ± 16.4%, P < 0.001)。根据是否发生低心排综合征将患儿分为低心排综合征组和未发生低心排综合征组，两者RVOT疏通程度差异无统计学意义(67.23% ± 17.02% vs 64.26% ± 18.35%, P = 0.453)。根据是否生中重度以上PR将患儿分为中重度PR组与非中重度PR组，两者RVOT疏通程度差异无统计学意义(66.5% ± 17% vs 65.1% ± 21.1%, P = 0.803)。结论：TOF根治术右室流出道疏通程度越大，患者近中期越不易发生RVOT再狭窄。本中心常规手术后右室流出道疏通程度在60%左右，患者在近中期随访出现中重度肺动脉反流、低心排综合征的频率较低，但远期结局需进一步随访研究。

 关键词

法洛四联症，右室流出道再狭窄，低心排综合征，肺动脉反流

 The Relationship between Right Ventricular Outflow Tract Dredging Degree and Short-to-Mid-Term Results in Children with Repaired Tetralogy of Fallot<sup> </sup>

Weihao Mu, Yong An*

Department of Cardiothoracic Surgery, Children’s Hospital of Chongqing Medical University, Chongqing

Received: Mar. 28th, 2022; accepted: Apr. 22nd, 2022; published: Apr. 29th, 2022

 ABSTRACT

Objective: To analyze the relationship between right ventricular outflow tract dredging degree and short-to-mid-term results in children with TOF. Methods: A retrospective analysis was made on the clinical characteristics of children undergoing radical operation for tetralogy of Fallot in Children’s Hospital Affiliated to Chongqing Medical University from January 2016 to December 2020. The relationship between right ventricular outflow tract restenosis, low cardiac output syndrome, moderate or severe PR and right ventricular outflow tract dredging degree after radical operation for tetralogy of Fallot was statistically analyzed. Results: A total of 93 children were included in the study, including 61 males and 32 females, with an average age of 14.8 ± 10.0 years and average weight of 9.2 ± 2.2 kg. There were 3 cases of right ventricular outflow tract restenosis, 28 cases of low cardiac output syndrome and 11 cases of moderate or severe PR after operation. The children were divided into stenosis group and non-stenosis group according to the occurrence of right ventricular outflow tract stenosis. The degree of RVOT recanalization in restenosis group was lower than that in non-stenosis group (67.5% ± 16.3% vs 31.8% ± 16.4%, P < 0.001). According to the occurrence of low cardiac output syndrome, the children were divided into low cardiac output syndrome group and non-low cardiac output syndrome group. There was no significant difference in the degree of RVOT patency between the two groups (67.23% ± 17.02% vs 64.26% ± 18.35%, P = 0.453). The children were divided into moderate or severe PR group and non-moderate to severe PR group according to whether they had moderate or severe RVOT. There was no significant difference in the degree of RVOT dredging between the two groups (66.5% ± 17% vs 65.1% ± 21.1%, P = 0.803). Conclusion: The greater the recanalization degree of the right ventricular outflow tract after TOF operation is, the less likely the patients to develop RVOT restenosis in the short-to-medium term are. The degree of dredging of the right ventricular outflow tract after routine surgery in our center is about 60%. The frequency of moderate-to-severe pulmonary regurgitation and low cardiac output syndrome is low in the short-to-medium term follow-up, but the long-term outcome needs further study.

Keywords:Tetralogy of Fallot, Right Ventricular Outflow Tract Restenosis, Low Cardiac Output Syndrome, Pulmonary Regurgitation

Copyright © 2022 by author(s) and beplay安卓登录

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 1. 引言

法洛四联症是最常见的紫绀型先天性心脏病，发病率为0.34‰ [ 1 ] 。自1955年Lillehei首次报道了TOF (Tetralogy of Fallot)的外科治疗以来 [ 2 ] ，其手术方式不断改进，患者的长期存率不断改善，成年期的总存活率已超过85% [ 3 ] [ 4 ] 。法洛四联症根治术主要包括右室流出道疏通及室间隔修补两大部分，对于后者目前无较大争议，而前者则是法洛四联症根治术的难点与重点，如何在解除右室流出道狭窄的同时保留肺动脉功能、维护右心功能是目前研究的重点，本文对我院接受法洛四联症根治术的患儿进行回顾性研究，总结分析右室流出道疏通程度与近中期预后的关系特点。

 2. 资料与方法 2.1. 基本资料

收集2016年1月到2020年12月行一期TOF矫治手术患儿的临床资料，研究共纳入93名患儿，其中男61例、女32例，平均年龄(14.8 ± 10.0)个月，平均体重(9.2 ± 2.2) kg。

 2.2. 纳入与排除标准

纳入标准：超声心动图提示室间隔缺损、主动脉骑跨、右心室肥厚和右室流出道狭窄，经我院心胸外科明确诊断为法洛四联症的患儿。

注：TOF合并房间隔缺损、动脉导管未闭的患者也纳入此研究。

排除标准：合并右室双腔等其他复杂的心脏畸形；严重的非心脏畸形如气管狭窄等；遗传代谢疾病如21三体综合征、甲减患者；接受急诊手术或同次住院期间行其他手术等。

 2.3. 数据内容

所有患者均为一期根治手术患者。纳入变量包括基线资料，术前超声数据，术前CT数据，术中资料(手术方式、体外循环时间等)，右室流出道疏通程度计算方式为术前和术后1周内超声所测右室流出道压差的差值与术前1周内RVOT压差的比值*100%，出院后随访超声数据等变量。

 2.4. 手术方法

胸骨正中切口逐层进胸，中度低温体外循环。主动脉–双腔插管建立体外循环，根部灌注HTK心肌保护液诱导心脏停搏进行心肌保护。斜行切开右心房，或联合右室流出道、肺动脉切口途径行心内探查及手术。切除部分肥厚的隔壁束疏通RVOT，使用5/0聚丙烯连续缝合线和自体心包补片以完成VSD修补，使用6/0聚丙烯连续缝合线和Gore-Tex补片扩大RVOT，术中测量PV环的大小是否合适。当z值<2时行跨环补片。

 2.5. 统计学分析

采用spss24.0统计软件。计量资料呈正态分布，以均数标准差表示，计量资料呈非正态分布，以中位数(P25，P75)表示，两组间比较采用独立样本t检验和非参数检验；计数资料以百分比表示，组间比较采用X2检验。P < 0.05为差异有统计学意义。

 3. 结果 3.1. 基本资料

研究共纳入93例患者，其中男61例、女32例，行法洛四联症根治术时患者平均年龄14.8 ± 10月，平均体重9.2 ± 2.2 kg，术前合并房间隔缺损59例、合并动脉导管未闭23例，术前平均Mcgoon指数2.17 ± 0.50，平均体外循环时间117.8 ± 21.7分钟，平均主动脉阻断时间78 ± 15分钟，其中行跨环补片手术的患者65例，未行跨环补片手术的患者28例。见表1。

Characteristics and clinical data of 93 patient
临床资料 数值
性别
男 61
女 32
年龄(m) 14.8 ± 10
体重(Kg) 9.2 ± 2.2
术前合并症(例)
ASD 50
PDA 23
体外循环时间(min) 117.8 ± 21.7
主动脉阻断时间(min) 78 ± 15
Mcgoon指数 2.17 ± 0.50
跨瓣环补片
是 65
否 28

表1. 93名患儿的临床资料[例/范围(x ± s)]

注：ASD (Atrial septal defect)：房间隔缺损；PDA (Patent ductus arteriosus)：动脉导管未闭；Mcgoon指数：左右肺动脉的直径与膈肌平面降主动脉直径的比值。

 3.2. TOF根治术后并发低心排综合征与RVOT疏通程度的关系

TOF根治术后发生低心排综合征共28例，根据术后有无低心排综合征分为两组进行比较，低心排综合征组M值(Mcgoon值)明显低于未发生低心排综合征组(2.3 ± 0.48 vs 1.89 ± 0.44, P < 0.001)，发生低心排综合征组的患儿年龄较小(16.26 ± 11.22月vs 11.5 ± 5.27月，P < 0.01)、体重较轻(9.52 ± 2.44 Kg vs 8.48 ± 1.51 Kg, P < 0.05)。但两组患儿的RVOT疏通程度差异无统计学意义(67.23% ± 17.02% vs 64.26% ± 18.35%, P = 0.453)，见表2。

Comparison of clinical data between low cardiac output syndrome group and non-syndrome grou
无 有 P
例数 65 28
Mcgoon值 2.3 ± 0.48 1.89 ± 0.44 0.000
体循时间(min) 117.6 ± 20.27 120 ± 23.55 0.619
主动脉阻断时间(min) 78.55 ± 14.88 76.82 ± 13.83 0.600
疏通程度(%) 67.23 ± 17.02 64.26 ± 18.35 0.453
年龄(m) 16.26 ± 11.22 11.5 ± 5.27 0.007
体重(Kg) 9.52 ± 2.44 8.48 ± 1.51 0.039
术前RVOT压差(mmHg) 55.89 ± 18.15 58.98 ± 11.85 0.411
术前跨肺动脉瓣压差(mmHg) 73.04 ± 20.01 71.71 ± 18.47 0.765

表2. 术后无低心排综合征组和有低心排综合征组临床资料对比[例/范围(x ± s)]

 3.3. TOF根治术后并发中重度以上PR与RVOT疏通程度与关系

TOF根治术后发生中重度以上PR共11例，根据TOF根治术后有无并发中重度以上PR分为两组进行比较，可发现两组的RVOT疏通程度差异无统计学意义(66.5% ± 17% vs 65.1% ± 21.1%, P = 0.803)。见表3。

Comparison of clinical data between moderate-or-severe PR group and non-PR grou
无 有 P
例数 82 11
年龄(m) 14.6 ± 9.9 16.5 ± 11.6 0.548
体重(Kg) 9.2 ± 2.3 9.4 ± 2.1 0.802
Mcgoon 2.2 ± 0.5 2.1 ± 0.3 0.641
体外循环时间(min) 116.9 ± 21.2 124 ± 24.8 0.347
主动脉阻断时间(min) 78 ± 14.7 78.5 ± 13.9 0.919
术前跨肺动脉瓣压差(mmHg) 72.3 ± 20 75.4 ± 15.5 0.618
疏通程度(%) 66.5 ± 17 65.1 ± 21.1 0.803

表3. 末次随访无中重度及以上PR组与有中重度及以上PR组临床资料对比

 3.4. TOF根治术后并发右室流出道再狭窄与RVOT疏通程度与关系

TOF根治术后发生右室流出道再狭窄共3例，按照术后随访跨肺压差是否大于50 mmHg分组，可发现两组的RVOT疏通程度有明显差异(67.5% ± 16.3% vs 31.8% ± 16.4%, P < 0.01)，术后随访跨肺压差<50 mmHg组的RVOT疏通程度较大。见表4。

Comparison of clinical data between peak gradient across PV < 50 mmHg group and the group ≥ 50 mmH
小于50 大于或等于50 P
例数 90 3
年龄(m) 15 ± 10 23 ± 22 0.152
体重(Kg) 9.1 ± 2 12.7 ± 5.5 0.377
Mcgoon值 2.17 ± 0.5 2.34 ± 0.73 0.570
体循时间(min) 118 ± 22 121 ± 7 0.826
主动脉阻断时间(min) 78 ± 15 82 ± 10 0.662
术前跨肺动脉瓣压差(mmHg) 72.4 ± 19.8 78.67 ± 3.2 0.588
疏通程度(%) 67.5 ± 16.3 31.8 ± 16.4 0.000

表4. 末次随访跨肺瓣压差小于50 mmHg组与大于或等于50 mmHg组临床资料对比

 4. 讨论

从1888年Fallot第一次完整描述TOF以来，随着科学进步的发展和对TOF研究结果的丰富，临床医师对TOF的认知都有了长足的进步 [ 3 ] 。1945年，由约翰霍普金斯大学的阿尔弗雷德·布莱克(Alfred Blalock)第一次通过手术修复了TOF [ 3 ] [ 5 ] 。近几十年来TOF的治疗有了显著的改善，大多数TOF患者术后将享受良好的生存周期。目前研究的方向是如何提高TOF患者术后的生存质量，减少术后中晚期并发症的发生、延缓并发症的进展已经成为TOF相关的研究重点 [ 6 ] ，因此早期识别并尽可能地避免或减少并发症的发生应得到临床医师的重视。

TOF根治术术后的并发症在不同阶段有着不同的表现，通常术后早期并发症包括：心律失常、低心排综合征、残余缺损、右心室舒张功能障碍等 [ 7 ] [ 8 ] [ 9 ] 。本研究表明，患者术后早期发生低心排综合征与M值、年龄、体重相关，M值、年龄、体重越小，发生低心排综合征的概率越大。但是否低心排综合征两组右室流出道疏通程度的差异无明显统计学意义，而通过随访发现，两组患儿心功能均处于正常水平，尽管低心排综合征组患儿术后住院时间相对较长，但两组患儿在近中期的随访中均未表现出明显的心功能不全，故考虑TOF患者右室流出道疏通在60&左右时近中期随访心功能良好。

TOF根治术中远期并发症包括肺动脉瓣反流(pulmonary regurgitation, PR)、右室流出道再狭窄，最终的结局是心律失常、心力衰竭，甚至死亡。为了延长患者生存周期、提高患者生存质量，维护患者的右心功能相当重要。在术后早期，右心功能通常较术前有恢复，并不会出现明显下降，但随着时间的增加，患者都有不同程度的右心功能下降。而右心功能的下降可能与心肌完整性被破坏、术后右室流出道残余梗阻导致压力负荷增加、肺动脉瓣返流导致容量负荷过大、电生理异常等因素有关 [ 10 ] [ 11 ] 。所以，避免以上并发症的发生、早期发现并尽早控制并发症的进展，能有效改善右心功能、延缓右心功能下降的出现。

在疾病认识的早期，因为患者在几年内甚至十几年内没有明显的临床症状，所以认为PR并不是一种恶性病理改变，但随着进一步的研究发现它可以预测运动能力下降和进行性右心室扩张。其原因考虑可能是由于小儿的心室舒张期较短、心室顺应性较好，心脏处于自我代偿阶段。但当患者成年后，随着心率减低、心脏顺应性下降、心室舒张期增加，长期存在的肺动脉瓣反流会使右心室容量持续超负荷，这会导致心室重构、扩张，加重心功能的损害，最终结局是心脏功能失代偿、心律失常和猝死 [ 12 ] [ 13 ] [ 14 ] 。因此有学者建议，对于中重度PR合并心功能不全或心律失常的患者，或者对于无症状中重度PR患者，若存在进行性右心室扩大和心功能不全、合并重度三尖瓣反流、RVOT再狭窄的情况，建议行肺动脉瓣置换术。本研究中是否发生中重度PR两组患儿的右室流出道疏通程度差异无统计学意义，但大部分患儿的心功能无降低、无重度三尖瓣返流，且换瓣年龄小，后续可能面临多次换瓣手术的可能，故未积极予以肺动脉瓣置换术。

同时相关研究提示中重度PR还与是否行跨环补片有关 [ 15 ] 。漏斗部肌束的切除和跨环补片破坏了RVOT和肺动脉瓣的完整性，导致PR发生。

右心室流出道再狭窄是TOF根治术术后常见的并发症，长期存在的重度狭窄会导致右心室压力负荷过大、右心功能下降，是再手术的常见原因之一。有研究表明，术后保留轻度的肺动脉瓣狭窄(右心室收缩压60~70 mm Hg，跨瓣压差15~30 mm Hg)，可显著降低远期肺动脉瓣反流，降低后期肺动脉瓣置换几率 [ 16 ] 。本研究表明随访跨瓣压差大于50 mmHg组的患儿右室流出道疏通程度明显更小，因此临床医师可将右室流出道疏通50%~80%，可一定程度上避免术后早期发生再狭窄而再手术的可能，但建议保留一定的压差，有利于避免PR发生。

 5. 结论

TOF根治术右室流出道疏通程度越大，患者近中期越不易发生RVOT再狭窄。本中心常规手术后右室流出道疏通程度在60%左右，患者在近中期随访出现中重度肺动脉反流、低心排综合征的频率较低，但远期结局需进一步随访研究。

 文章引用

母炜浩,安 永. 法洛四联症根治术右室流出道疏通程度与近中期预后关系The Relationship between Right Ventricular Outflow Tract Dredging Degree and Short-to-Mid-Term Results in Children with Repaired Tetralogy of Fallot[J]. 临床医学进展, 2022, 12(04): 3696-3702. https://doi.org/10.12677/ACM.2022.124533

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