唐氏綜合征患兒先天性心臟病術(shù)后并發(fā)癥和預(yù)后的病例對照研究

劉成軍 茅思遠(yuǎn) 符躍強(qiáng) 白 科 許 峰

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·論著·

唐氏綜合征患兒先天性心臟病術(shù)后并發(fā)癥和預(yù)后的病例對照研究

劉成軍 茅思遠(yuǎn) 符躍強(qiáng) 白 科 許 峰

目的 探討唐氏綜合征(DS)患兒先天性心臟病(先心病)術(shù)后的并發(fā)癥及預(yù)后。方法以 2009年1月1日至2013年6月30日DS先心病術(shù)后患兒為DS組，選擇同期非DS先心病術(shù)后患兒作為對照組。兩組根據(jù)先心病類型分別分為簡單和復(fù)雜型先心病亞組。比較兩組患兒臨床特征、術(shù)后并發(fā)癥及預(yù)后。結(jié)果DS組和對照組分別納入77例，兩組年齡、性別構(gòu)成、體重和先心病類型差異均無統(tǒng)計(jì)學(xué)意義。DS組簡單先心病亞組46例(59.7%)，復(fù)雜先心病亞組31例；對照組簡單先心病亞組47例(61.0%)，復(fù)雜先心病亞組30例。DS組機(jī)械通氣時(shí)間和ICU住院時(shí)間較對照組明顯延長(P均<0.05)，低心排綜合征、再插管、肺部感染和肺動(dòng)脈高壓的發(fā)生率較對照組亦明顯增高(P均<0.05)；病死率也高于對照組(6.5%vs1.3%)，但差異無統(tǒng)計(jì)學(xué)意義(P=0.096)。DS組和對照組簡單型先心病亞組并發(fā)癥發(fā)生率及病死率差異均無統(tǒng)計(jì)學(xué)意義(P均>0.05)。DS組復(fù)雜型先心病亞組在機(jī)械通氣時(shí)間、ICU住院時(shí)間、總住院天數(shù)較對照組相應(yīng)亞組均明顯延長，低心排綜合征、肺動(dòng)脈高壓和肺部感染的發(fā)生率亦明顯增高(P均<0.05)，病死率DS組復(fù)雜型先心病亞組有增高趨向(12.9%vs3.3%，P=0.173)。結(jié)論DS不增加簡單型先心病患兒術(shù)后并發(fā)癥和病死率；但增加復(fù)雜型先心病術(shù)后并發(fā)癥發(fā)生率的風(fēng)險(xiǎn)，有增加病死率的趨向。

唐氏綜合征； 先天性心臟?。?術(shù)后并發(fā)癥

唐氏綜合征(DS)是最常見的一種染色體疾病，先天性心臟病(先心病)是其最常見的合并畸形。有資料顯示，所有的先心病患兒中，有4%～10%伴有DS，而40%～60%的DS患兒合并有先心病[1]。在墨西哥，DS最常合并的先心病類型是動(dòng)脈導(dǎo)管未閉(PDA)、室間隔缺損(VSD)和房間隔缺損(ASD)；在歐美，房室間隔缺損(AVSD)是DS主要合并的先心病類型，可達(dá)40%～70%[1,2]。中國64.4%的DS患兒合并先心病，以間隔類缺損為主(87.0%)，其中單純ASD占15.4%，單純VSD占9.7%，AVSD占14.6%，復(fù)合類間隔缺損占47.3%[3]。既往認(rèn)為，DS合并先心病患兒預(yù)后不良，病死率較高，且術(shù)后住院時(shí)間長，易發(fā)生感染，增加病死率，常不考慮手術(shù)。但此類患兒若不手術(shù)治療，肺動(dòng)脈高壓、肺部感染等并發(fā)癥不易控制，反而影響患兒的生存質(zhì)量。隨著手術(shù)和監(jiān)護(hù)技術(shù)的提高，手術(shù)不再是DS患兒的禁忌，并能顯著提高其生存率[2,4,5]。但臨床上關(guān)于DS患兒先心病術(shù)后并發(fā)癥的發(fā)生率和預(yù)后，文獻(xiàn)報(bào)道不一[2,6～10]，有學(xué)者認(rèn)為DS患兒先心病術(shù)后有較高的并發(fā)癥風(fēng)險(xiǎn)，增加病死率[8,9]；但近年來也有文獻(xiàn)報(bào)道術(shù)后并不增加并發(fā)癥發(fā)生率，不影響患兒的預(yù)后[2,6,7,10]。本研究通過病例對照研究設(shè)計(jì)，選取DS合并先心病術(shù)后患兒為DS組，以非DS先心病術(shù)后患兒為對照組，回顧性分析兩組患兒的臨床資料、并發(fā)癥及病死率情況，探討DS合并先心病患兒術(shù)后的預(yù)后。

1 方法

1.1 DS組納入標(biāo)準(zhǔn) ①2009年1月1日至2013年6月30日重慶醫(yī)科大學(xué)附屬兒童醫(yī)院(我院)收治的先心病患兒；②行先心病手術(shù)治療；③有特殊面容和(或)智力障礙等表現(xiàn)者；④經(jīng)染色體檢查確診為DS。

1.2 對照組納入標(biāo)準(zhǔn) 選擇同期無特殊面容和智力障礙等表現(xiàn)的非DS先心病術(shù)后患兒，樣本量與DS組1∶1匹配，年齡、先心病類型與DS組盡量匹配。

1.3 DS組和對照組共同排除標(biāo)準(zhǔn) 兩組均排除急診手術(shù)、延遲關(guān)胸、合并氣道狹窄及其他類型遺傳代謝疾病者。

1.4 亞組分析 考慮到各種先心病的解剖畸形、病理生理不同，手術(shù)方式不同，對臨床預(yù)后的影響也不同[11]，故本研究根據(jù)先心病類型[12]將DS組和對照組分別分為簡單型先心病和復(fù)雜型先心病亞組。簡單型先心病包括VSD、ASD和PDA，復(fù)雜型先心病包括完全性房室隔缺損(CAVSD)、法洛四聯(lián)癥(TOF)、右室雙出口(DORV)、大動(dòng)脈轉(zhuǎn)位(TGA)和主肺動(dòng)脈起源異常等。

1.5 肺動(dòng)脈高壓分度[13]根據(jù)心臟超聲行肺動(dòng)脈高壓分度。輕度：肺動(dòng)脈收縮壓30～40 mmHg；中度：肺動(dòng)脈收縮壓～70 mmHg；重度：肺動(dòng)脈收縮壓>70 mmHg。先心病術(shù)后發(fā)生輕度以上肺動(dòng)脈高壓定義為術(shù)后肺動(dòng)脈高壓[2]。

1.6 資料截取 回顧性翻閱病史，截取以下臨床資料用于本文分析：①性別、年齡、體重、診斷、肺動(dòng)脈壓、體循環(huán)時(shí)間和主動(dòng)脈阻斷時(shí)間；②術(shù)后ICU住院時(shí)間、總住院時(shí)間和機(jī)械通氣時(shí)間；③術(shù)后并發(fā)癥：包括低心排綜合征(臨床征象：竇性心動(dòng)過速、少尿或無尿、肢端發(fā)涼、肝臟腫大、低血壓、心臟驟停)、心律失常、肺部并發(fā)癥(氣胸、肺不張)、肺部感染、肺動(dòng)脈高壓、再次插管和腎功能不全(氮質(zhì)血癥或需要血液凈化治療)；④預(yù)后。

2 結(jié)果

2.1 一般情況 研究期間我院共收治2 026例先心病術(shù)后患兒，其中經(jīng)染色體檢查確診為DS患兒77例，包括簡單型先心病亞組46例(ASD 7例，VSD 19例，VSD合并ASD 20例)，復(fù)雜型先心病亞組31例(CAVSD 24例，TOF 5例，DORV 1例，主肺動(dòng)脈起源異常1例)。同期選擇77例非DS先心病患兒為對照組，其中簡單型先心病亞組47例(ASD 4例，VSD 26例，VSD合并ASD 17例)，復(fù)雜型先心病亞組30例(CAVSD 26例，TOF 4例)。DS組和對照組年齡、性別、體重和先心病類型差異均無統(tǒng)計(jì)學(xué)意義(表1)。

2.2 DS組與對照組臨床特征和術(shù)后并發(fā)癥比較 如表1所示，DS組術(shù)前肺動(dòng)脈高壓、體循環(huán)時(shí)間、主動(dòng)脈阻斷時(shí)間和總住院天數(shù)與對照組比較差異均無統(tǒng)計(jì)學(xué)意義(P均>0.05)，術(shù)后機(jī)械通氣時(shí)間和ICU住院時(shí)間較對照組明顯延長(P均<0.05)。DS組術(shù)后低心排綜合征、再插管、肺部感染和肺動(dòng)脈高壓的發(fā)生率較對照組明顯增高(P均<0.05)。DS組死亡5例，其中2例因嚴(yán)重低心排綜合征術(shù)后3 d內(nèi)死亡，2例因多臟器功能衰竭死亡，1例因反復(fù)肺部感染并發(fā)急性呼吸窘迫綜合征死亡。對照組1例因嚴(yán)重低心排綜合征死亡。

2.3 簡單型先心病和復(fù)雜型先心病亞組臨床特征和術(shù)后并發(fā)癥比較 如表1所示，DS組和對照組簡單型先心病亞組在術(shù)后機(jī)械通氣時(shí)間、ICU住院時(shí)間、總住院時(shí)間、并發(fā)癥發(fā)生率及病死率上差異均無統(tǒng)計(jì)學(xué)意義(P均>0.05)。與對照組復(fù)雜型先心病亞組比較，DS組復(fù)雜型先心病亞組術(shù)后機(jī)械通氣時(shí)間、ICU住院時(shí)間、總住院天數(shù)顯著延長；低心排綜合征、肺動(dòng)脈高壓和肺部感染的發(fā)生率明顯增高(P均<0.05)；病死率有增高的趨向(12.9%vs3.3%,P=0.173)。

NotesP1: Comparisons of simple CHD betweetn DS and control groups;P2: Comparisons of complex CHD between DS and control groups;P3: Comparisons of total CHD between DS and control groups; PPH: Preoperative pulmonary hypertension; LOCS: Low cardiac output syndrome

3 討論

雖然近年來DS患兒存活率明顯增高，但總病死率是非DS患兒的5倍多，引起DS患兒死亡的主要原因是先心病和呼吸道感染[14,15]。DS合并先心病早期行矯治手術(shù)效果良好，手術(shù)風(fēng)險(xiǎn)無明顯增加，且隨著手術(shù)技術(shù)及治療方法的改進(jìn)，術(shù)后長期存活率明顯提高，預(yù)期壽命延長[16,17]，Al-Hay等[18]報(bào)道147 例CAVSD患兒中72%合并有DS，但與非DS患兒相比，DS并不增加手術(shù)的病死率，而且遠(yuǎn)期再手術(shù)率甚至還低于染色體檢查正常的患兒。DS也不是手術(shù)的危險(xiǎn)因素，與非DS患兒相比，DS患兒的心臟手術(shù)存活率和遠(yuǎn)期效果差異均無統(tǒng)計(jì)學(xué)意義[4]。國內(nèi)資料顯示，DS合并先心病術(shù)后病死率為2.9%，與同期先心病手術(shù)總體病死率(2.5%)相比仍然是可以接受的，而且中期隨訪結(jié)果也令人滿意，表明手術(shù)治療對于DS合并先心病患兒是安全有效的[19]。本文資料顯示，DS組病死率略高于對照組，但兩組差異無統(tǒng)計(jì)學(xué)意義，經(jīng)亞組分析，兩組簡單型先心病亞組間病死率差異無統(tǒng)計(jì)學(xué)意義，但DS復(fù)雜型先心病亞組較對照組有增高趨向(12.9%vs3.3%)。

近年來有文獻(xiàn)報(bào)道DS患兒先心病術(shù)后并不增加并發(fā)癥發(fā)生率[10]，但DS患兒存在免疫缺陷(主要表現(xiàn)為T淋巴細(xì)胞功能障礙)、生長發(fā)育落后、免疫力低下，可能合并肺血管梗阻性病變，多數(shù)文獻(xiàn)報(bào)道術(shù)后早期仍有較高的并發(fā)癥，其中最多見的是肺部感染[2,4]。Fudge等[2]研究發(fā)現(xiàn)，合并ASD、VSD和TOF的DS患兒住院時(shí)間較非DS患兒延長，肺部感染發(fā)生率增高。因肺部感染的發(fā)生率增加，DS患兒先心病術(shù)后機(jī)械通氣和ICU住院時(shí)間明顯延長[2,20]，繼而導(dǎo)致呼吸機(jī)相關(guān)性肺炎(VAP)的發(fā)生[21]。Van Trotsenburg等[7]報(bào)道DS患兒心臟手術(shù)后呼吸道感染的發(fā)生率為34%，且常發(fā)展為敗血癥，成為術(shù)后早期死亡的重要原因?？人苑瓷錅p弱、分泌物增多和胃食管反流等是導(dǎo)致DS先心病術(shù)后肺部感染的重要因素[14]，同時(shí)也導(dǎo)致氣管插管拔管失敗的概率增大，再插管率增加。本文資料顯示，DS組術(shù)后機(jī)械通氣時(shí)間和ICU住院時(shí)間較對照組明顯延長，肺部感染發(fā)生率明顯高于對照組(22.1%vs7.8%),經(jīng)亞組分析，DS組復(fù)雜型先心病亞組術(shù)后機(jī)械通氣時(shí)間、ICU住院時(shí)間、肺部感染發(fā)生率明顯高于對照組相應(yīng)亞組，而在簡單型先心病兩亞組差異無統(tǒng)計(jì)學(xué)意義。因此術(shù)后要加強(qiáng)呼吸道管理，無菌操作，防止VAP的發(fā)生，并根據(jù)病原學(xué)結(jié)果選擇敏感抗生素控制肺部感染，盡量縮短病程。

DS合并先心病的心臟畸形通常較復(fù)雜，多為復(fù)合畸形，容易早期形成肺動(dòng)脈高壓，甚至發(fā)生艾森曼格綜合征，其原因大致分為2個(gè)方面：一方面是由于心臟結(jié)構(gòu)畸形引起的心內(nèi)分流，肺小動(dòng)脈血流量增加，早期肺動(dòng)脈呈反射性痙攣，肺循環(huán)阻力增加，肺動(dòng)脈壓升高[22]；另一方面是DS合并先心病患者存在肺組織發(fā)育不全，主要表現(xiàn)為肺腺泡發(fā)育不全和殘留雙重肺毛細(xì)血管網(wǎng)，導(dǎo)致早期出現(xiàn)不可逆的肺血管病變，并呈進(jìn)行性發(fā)展，加重肺動(dòng)脈高壓[23,24]。早期介入治療或外科手術(shù)對肺動(dòng)脈高壓均有明顯改善作用[3]，避免發(fā)生艾森曼格綜合征而失去手術(shù)機(jī)會。本文DS組與對照組術(shù)前肺動(dòng)脈高壓的發(fā)生率差異無統(tǒng)計(jì)學(xué)意義，也未發(fā)現(xiàn)艾森曼格綜合征的患兒，這與患兒手術(shù)年齡較小，早期手術(shù)干預(yù)有關(guān)。本文2組患兒術(shù)后肺動(dòng)脈高壓發(fā)生率均明顯下降，但術(shù)后DS組肺動(dòng)脈高壓的發(fā)生率明顯高于對照組(44.2%vs27.3%，P<0.05)，國外文獻(xiàn)也有類似報(bào)道[2,25]。經(jīng)亞組分析，DS組復(fù)雜型先心病亞組術(shù)后肺動(dòng)脈高壓的發(fā)生率明顯高于對照組相應(yīng)亞組，而在簡單型先心病兩亞組術(shù)后肺動(dòng)脈高壓的發(fā)生率差異無統(tǒng)計(jì)學(xué)意義。術(shù)后肺動(dòng)脈高壓也可能與手術(shù)持續(xù)時(shí)間、體外循環(huán)時(shí)間和先心病復(fù)雜程度有關(guān)。部分患兒體外循環(huán)心內(nèi)直視手術(shù)后易發(fā)生反應(yīng)性肺動(dòng)脈高壓，甚至肺高壓危象，導(dǎo)致嚴(yán)重的低心排綜合征，因此對于術(shù)前有重度肺動(dòng)脈高壓、或術(shù)后有肺動(dòng)脈高壓表現(xiàn)的患兒，術(shù)后要合理鎮(zhèn)靜、鎮(zhèn)痛，中度過度通氣，適宜氧合，維持PaCO2在30 mmHg左右(手術(shù)日)，PaO290～100 mmHg，避免肺高壓危象的發(fā)生，有條件可行肺動(dòng)脈壓監(jiān)測[26],本文資料未發(fā)現(xiàn)體外循環(huán)術(shù)后肺動(dòng)脈高壓危象患兒。

低心排綜合征是先心病特別是復(fù)雜型先心病術(shù)后較常見的并發(fā)癥，也是導(dǎo)致患兒死亡的重要原因。目前認(rèn)為可由多種原因引起，可能與患兒年齡、先心病類型、術(shù)前肺部感染、肺動(dòng)脈高壓程度、主動(dòng)脈阻斷時(shí)間和體外循環(huán)時(shí)間等因素有關(guān)[27,28]。有研究顯示，DS患兒先心病術(shù)后左室心搏做功與舒張末期容積的比值稍低，左室射血分?jǐn)?shù)較低，左室功能改善不顯著[29]，可能也是引起低心排綜合征的原因之一。本研究發(fā)現(xiàn)，DS患兒較對照組術(shù)后更易發(fā)生低心排綜合征(P=0.020)，國外文獻(xiàn)也有類似報(bào)道[2,25]。低心排綜合征發(fā)生率在簡單型先心病兩亞組差異無統(tǒng)計(jì)學(xué)意義，但在復(fù)雜型先心病患兒中差異有統(tǒng)計(jì)學(xué)意義(P=0.036)。還有文獻(xiàn)報(bào)道，DS患兒先心病術(shù)后易發(fā)生氣胸、肺不張、心律失常和腎功能不全等[2]，但本研究資料顯示，兩組上述并發(fā)癥的發(fā)生率差異均無統(tǒng)計(jì)學(xué)意義。

綜上所述，DS能增加復(fù)雜型先心病術(shù)后早期并發(fā)癥發(fā)生率，并導(dǎo)致住院時(shí)間延長，有增高病死率的趨向；不增加簡單型先心病術(shù)后的并發(fā)癥發(fā)生率和病死率。

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(本文編輯：張萍)

The postoperative complications and prognosis of the children with Down′s syndrome undergoing congenital heart disease surgery: a case-control study

LIUCheng-jun,MAOSi-yuan,FUYue-qiang,BAIKe,XUFeng

(PICU,Children′sHospital,ChongqingMedicalUniversity,Chongqing400014,China)

FU Yue-qiang，E-mail:fuyueqiang@sina.com

ObjectiveTo explore the postoperative clinical characteristics, complications and prognosis of patients with Down′s syndrome(DS) undergoing congenital heart disease(CHD) surgery.MethodsChildren with DS undergoing CHD surgery between January 1, 2009 and June 30, 2013 were recruited as DS group, and children with non-DS undergoing CHD surgery in the same period were selected as the control group. The age and types of CHD in DS group and control group were matched. Two groups were all divided into simple CHD subgroup and complex CHD subgroup according to the type of CHD.The clinical characteristics, complications and prognosis of two groups were retrospectively analyzed.ResultsA total of 77 cases were enrolled in DS group and 77 cases in control group. The age, gender, bodyweight and types of CHD did not differ between DS group and control group.Ventilation duration, PICU stay, and the incidence of low cardiac output syndrome(LCOS)，pulmonary infection, reintubation, pulmonary hypertension were much higher in DS group than those in control group [52.2(35.5-83.2) hvs29.4(21.5-55.8) h；(5.1±2.4) dvs(3.8±3.6) d，respectively, allP<0.05]. No statistically significant differences in in-hospital mortality rates were seen for patients in two groups(6.5% vs 1.3%,P>0.05). By hierarchical analysis, there was no significant difference in clinical characteristics, complications and mortality rates in DS and control group in simple CHD subgroup, but in complex CHD subgroup, ventilation duration, PICU stay, hospital stay [87.4(65.3-122.6) hvs48.3(25.3-73.5) h, (7.2±2.6) dvs(4.4±2.8) d, (24.4±8.3) dvs(19.5±5.5) d, respectively, allP<0.05)and the incidence of LCOS，pulmonary infection and pulmonary hypertension were much higher in DS group than those in control group (45.2%vs20.0%, 32.3%vs10.0%, 67.7%vs30.0%, respectively, allP<0.05). The mortality rate was higher in complex CHD of DS group than that in control group(12.9%vs3.3%,P>0.05).ConclusionDS did not increase mortality rate of children undergoing CHD surgery and had no effect on postoperative complications of simple CHD, but increased the risk of postoperative complications and mortality of complex CHD.

Down′s syndrome; Congenital heart disease; Postoperative complications

重慶醫(yī)科大學(xué)附屬兒童醫(yī)院重癥醫(yī)學(xué)科 重慶，400014

符躍強(qiáng)，E-mail:fuyueqiang@sina.com

10.3969/j.issn.1673-5501.2015.03.005

2015-01-22

2015-05-20)