复杂先天性心脏病单心室姑息术后转双心室矫治

目的 复杂先天性心脏病因心内畸形复杂和外科技术局限等原因选择单心室矫治。然而,单心室矫治的中远期预后远差于双心室矫治。本文总结既往复杂先天性心脏病行单心室姑息术转双心室矫治的临床结果。方法 回顾性分析2013年10月至2016年3月单心室姑息术后转双心室矫治的8例复杂先天性心脏病患者的临床资料,其中男5例、女3例。患儿行Glenn姑息术时中位年龄2.6(1.0~5.9)岁,转双心室矫治手术时中位年龄6.6(4.5~11.1)岁。3例完全性大动脉转位合并室间隔缺损和左室流出道狭窄,3例室间隔缺损远离型右室双出口合并肺动脉狭窄或肺动脉闭锁,1例右室双出口合并完全性房室间隔缺损和肺动脉狭窄,1例法洛四联症。结果 8例Glenn姑息术后患者完成转双心室矫治并将上腔静脉与右房重新连接,平均体外循环时间(275.6±107.1)min,平均主动脉阻断时间(165.9±63.6)min,平均住院时间(33.6±23.0)d,平均住ICU时间(20.3±21.0)d。无手术死亡。平均随访(1.4±0.7)年,无随访死亡及再手术,随访未见窦房结功能异常、肺动脉狭窄及上腔静脉-右房吻合口狭窄。所有患儿心功能明显改善,纽约心脏学会心功能分级(NYHA)均为Ⅰ~Ⅱ级。结论 尽管技术相对困难,部分单心室姑息术后的患者仍可恢复双心室矫治,近中期临床结果满意,远期结果仍需进一步随访。     

双向Glenn手术治疗成人先天性心脏病

目的分析双向Glenn手术治疗成人先天性心脏病的可行性。方法回顾性分析2004年12月至2015年12月我院行双向Glenn手术42例成人先天性心脏病患者的临床资料,其中男23例、女19例,年龄18~49(24.6±8.5)岁。术前诊断包括功能性单心室(FSV)14例,三尖瓣下移畸形(Ebstein畸形)11例,矫正型大动脉转位(C-TGA)7例,完全型大动脉转位(TGA)5例,右心室双出口(DORV)3例,三尖瓣闭锁(TA)2例。合并中度及以上房室瓣反流(AVVR)20例。右侧双向Glenn手术34例,双侧双向Glenn手术8例。21例在体外循环下手术,21例非体外循环下手术。其他合并畸形矫治包括Ebstein畸形矫治7例,房室瓣置换7例,房间隔造口3例,动脉导管结扎3例,主-肺动脉侧枝结扎术2例,完全性肺静脉异位引流(TAPVC)矫治1例,二尖瓣成形1例。结果术后早期死亡4例,死亡率9.5%。FSV和中度及以上AVVR是死亡的高危因素。血氧饱和度(Sp O2)从术前的78.8%±11.2%提高至出院时的89.3%±6.6%,P0.05。随访时间6~132(41.4±33.1)个月,无死亡病例,心功能较术前显著改善[(1.9±0.4)级vs.(2.7±0.5)级,P0.05]。结论成人先天性心脏病患者行Glenn手术是可行的,虽然早期死亡率较高,但是中远期效果令人满意,可以提高患者的Sp O2,显著改善患者的心功能,进而提高患者的生存质量。FSV和中度及以上AVVR是成人先天性心脏病行Glenn手术早期死亡的高危因素。     

双向Glenn手术治疗紫绀型复杂先天性心脏病

目的总结双向Glenn手术治疗紫绀型复杂先天性心脏病的临床经验。方法回顾性分析2007年1月至2012年12月新疆维吾尔自治区人民医院68例紫绀型复杂先天性心脏病行双向Glenn手术患者的临床资料,其中男40例、女28例,年龄3个月至22岁,平均年龄3.9岁;体重6.2~53.0（13.6±8.5）kg。术前诊断包括三尖瓣闭锁20例,单心室11例,右心室双出口10例,完全型大动脉转位7例,三尖瓣狭窄5例,肺动脉闭锁5例,矫正型大动脉转位4例,法洛四联症4例,三尖瓣下移畸形2例。其中合并右位心、右旋心14例,单心室并肺动脉高压行肺动脉环缩术后2例,完全型大动脉转位行中心分流术后1例。23例和45例紫绀型复杂先天性心脏病分别在体外循环和非体外循环下施行手术。结果术后早期死亡2例,1例死于严重的低心排血量综合征,1例死于肺部感染。脉搏血氧饱和度从术前的66.8%±11.8%提高至出院时的89.3%±7.4%（P〈0.05）。其中53例脉搏血氧饱和度术后较术前升高〉10%。红细胞压积由术前0.49±0.11降至术后的0.40±0.07（P〈0.05）。术后发生胸腔积液16例（23.5%）,乳糜胸7例（10.3%）,低心排血量综合征5例（7.4%）,心律失常4例（5.9%）,气胸1例（1.5%）。以上并发症均经相应的治疗治愈。随访55例,随访时间9个月至6年,所有吻合口血流通畅,无狭窄,无血栓形成,临床效果满意。4例患者分别于术后2~5年完成全腔静脉-肺动脉连接术,恢复顺利。结论双向Glenn手术安全、可靠,是一种治疗难以解剖根治或需一期生理矫治的紫绀型复杂先天性心脏病患者的较好手术术式。     

肺动脉环缩术治疗不同年龄伴肺动脉高压单心室

目的 总结肺动脉环缩术( PAB)在不同年龄段伴肺动脉高压先天性心脏病患儿行单心室修复术中的应用效果.方法 49例合并重度肺动脉高压仅能行单心室修复的复杂畸形患儿,男31例,女18例;年龄0.2～10岁,平均(7.8±3.8)岁;体重5～24 kg,平均(8.82 ±4.24) kg;经皮血氧饱和度( SPO2)0.85～1.00,平均(0.90±0.04);术前肺动脉平均压(mPAP) 52 ～91 mm Hg(1 mm Hg =0.133kPa),平均(54.6±16.8) mmHg.合并单心室13例,三尖瓣闭锁12例,室间隔缺损远离两大动脉伴左心室发育不良型右室双出口11例,三尖瓣重度狭窄5例,心室不均衡型完全型房室通道5例,十字交叉心3例.根据年龄分为3组:≤0.5岁17例、0.5 ～2.0岁17例、≥2.0岁15例.全组均先期行PAB,静脉吸入复合麻醉,吸入氧浓度40％时,SPO20.85,肺动脉平均压20 mm Hg.对比分析3组术后SPO2、mPAP、呼吸机使用时间、ICU停留时间以及手术死亡比率.结果 手术死亡1例系1岁6个月患儿,死因为肺部感染.全组术后肺动脉压明显下降.3组术后SPO2、mPAP、呼吸机使用时间、ICU停留时间均无明显区别.随访6 ～72个月,1例1岁2个月患儿术后2个月因误吸死亡;3例已完成双向格林或全腔肺动脉吻合术.结论PAB能有效降低不同年龄段伴肺动脉高压拟行单心室修复的先心病患儿的肺动脉压力,术后效果良好.     

一个半心室矫治术治疗三尖瓣下移畸形的效果北大核心CSCD

目的比校一个半心室矫治手术和双心室手术治疗三尖瓣下移畸形的效果,总结一个半心室矫治手术经验。方法连续收集2010年1月至2018年12月149例于首都医科大学附属北京安贞医院小儿心脏外科确诊为三尖瓣下移畸形并接受外科手术治疗的患者资料,并对生存率、再次手术率及三尖瓣反流情况等进行中远期随访。结果入组患者中男68例,女81例,中位年龄5.58岁。将患者按照最终接受的手术方式不同,分为双心室矫治组和一个半心室矫治组。一个半心室矫治组手术年龄小(中位年龄4.15岁对6.71岁,P=0.019),身高矮[(107.70±31.28)cm对(123.20±35.22)cm,P=0.014]、体质量轻[(19.69±12.22)kg对(29.65±20.41)kg,P=0.001],差异均有统计学意义。两组患者术前发绀情况、血红蛋白水平、心脏功能、心律失常、合并常见心内畸形(如房间隔缺损等)差异无统计意义。一个半心室矫治组术前合并肺动脉狭窄(10.81%对1.79%,P=0.016)和右心室发育不良(16.22%对3.57%,P=0.008)的比例显著偏高;手术体外循环时间偏长[(125.51±37.35)min对(100.44±25.24)min,P<0.001]。两组主动脉阻断时间、气管插管时间、住院时间、术后中期死亡比例、再手术率、心脏功能及瓣膜反流情况差异无统计学意义;一个半心室矫治组院内死亡比例稍高,可能与右心功能不佳、术后恢复困难有关。结论一个半心室矫治手术治疗三尖瓣下移畸形均取得较好的中期随访结果。一个半心室矫治手术的指征主要根据术前右心室功能判断,须重点关注三尖瓣下移合并右心室发育不良及肺动脉狭窄的患者,术前需评估肺动脉压力及肺血管发育,做好一个半心室矫治的准备。     

姑息性右室-肺动脉连接术在重症紫绀型先天性心脏病中的应用

目的探讨姑息性右室-肺动脉连接术在重症紫绀型先天性心脏病治疗中的临床应用。方法回顾性分析郑州市第七人民医院心脏外科2011年1月至2015年1月期间所有行姑息性右室-肺动脉连接术治疗的重症紫绀型先天性心脏病患者25例的临床资料,其中男17例、女8例,年龄31(5~108)个月,体重3.5~37.2(12.82±6.73)kg。结果 25例姑息性右室-肺动脉连接术后早期死亡2例(术后30 d内),早期死亡率8.0%(2/25)。患者术后动脉血氧饱和度与术前差异有统计学意义(62.43%±7.83%vs.81.62%±6.25%,P0.05)。术后随访6个月至3年(每3个月复查一次超声心动图),23例患者Mc Goon比值(1.05±0.14 vs.1.61±0.18,P0.05)和Nakata指数[(112.37±14.38)mm2/m2 vs.(165.74±22.62)mm2/m2,P0.05]均明显上升,且差异有统计学意义。17例患者行二期根治手术治疗。结论姑息性右室-肺动脉连接术能够有效促进重症紫绀型先天性心脏病患者的自身肺血管床发育,为行二期根治术创造条件。     

肺动脉环缩术在矫正型大动脉转位形态学左心室功能锻炼中的应用

目的探讨肺动脉环缩术在矫正型大动脉转位形态学左心室功能锻炼的临床应用效果。方法回顾性分析2007年1月至2011年12月上海交通大学医学院附属上海儿童医学中心手术治疗矫正型大动脉转位患者89例中行肺动脉环缩术11例的临床资料,其中男9例,女2例;年龄除1例12岁外,其余为3～42(16.40±11.67)个月;体重6～32(11.70±7.20)kg。所有患者均经超声心动图和心血管造影检查确诊。结果 11例行肺动脉环缩术患者无死亡,术前肺循环与体循环压力比(Pp/Ps)值0.3～0.6(0.44±0.09),术后为0.6～0.8(0.70±0.04),差异有统计学意义(P<0.01)。术前三尖瓣反流轻度2例(18.2%),中度5例(45.4%),重度4例(36.4%);术后无反流2例(18.2%),轻度反流7例(63.6%),轻-中度反流2例(18.2%)。11例中5例术后(15.20±8.31)个月行二期双心室解剖纠治术,其中1例死亡;余6例行单纯肺动脉环缩术后随访(18.83±3.43)个月,超声心动图检查提示:三尖瓣反流轻微2例(33.3%),轻度3例(50.0%),中度1例(16.7%)。结论矫正型大动脉转位通过肺动脉环缩术可减轻三尖瓣反流,锻炼形态学左心室功能,为二期解剖纠治做好准备,手术效果较好。但术后必须定期随访,观察术后形态学左心室功能和三尖瓣反流情况。     

Double Switch术治疗先天性矫正性大动脉转位二例

目的 介绍解剖矫治先天性矫正性大动脉转位的手术特点。方法 2例矫正性大动脉转位患者,1例9个月,合并室间隔缺损、肺动脉高压,行Senning加动脉调转术;1例23岁,合并室间隔缺损、肺动脉狭窄、三尖瓣关闭不全,行Rastelli加Senning术。结果 2例患者全部成活,其中1例术前出现Ⅲ度房室传导阻滞,安装永久起搏器,术后超声检查心室功能正常,心内隧道和心外管道通畅。无三尖瓣反流。结论 Double Switch手术是一种理想的治疗先天性矫正性大动脉转位的方法。     

婴幼儿危重先天性心脏病的急诊外科治疗

目的探讨婴幼儿危重先天性心脏病急诊外科治疗的可行性以及手术适应证选择、手术技术和围术期处理.方法 1998年5月～2003年5月为67例危重先天性心脏病婴幼儿(年龄14天～32个月,平均年龄11.8±8.9个月;体重2.6～14.8 kg,平均体重8.4±3.0 kg)施行急诊或亚急诊手术.室间隔缺损39例,法洛四联症13例,完全房室间隔缺损5例,完全性肺静脉异位引流4例,房间隔缺损合并肺动脉瓣狭窄3例,完全型大动脉转位2例,先心病术后肺动脉瓣赘生物1例.64例行根治手术,3例行姑息手术.结果术前准备时间0～9天,平均3.7±2.6天;术后呼吸机辅助呼吸时间1～14天,平均3.8±3.2天;ICU时间2～18 天,平均5.7±2.8天.全组住院死亡5例,手术死亡率7.5%(5/67).其余患者术后并发症为:低心排血量综合征14例,肺不张14例,肺部感染8例,肺动脉高压危象2例,气胸和膈肌麻痹各1例,均治愈.随访2～60个月,晚期死亡1例,其余患者心功能Ⅰ～Ⅱ级,效果满意.结论婴幼儿危重先天性心脏病急诊、亚急诊外科治疗是安全的,可以挽救大部分患者的生命,并取得较好效果,关键在于准确选择适应证、围术期加强营养和心肺功能支持.     

改良Fontan手术治疗复杂先天性心脏病

目的 总结改良Fontan手术治疗复杂先天性心脏病的临床经验.方法 1996年11月～2005年5月,采用改良Fontan手术纠治124例复杂先天性心脏病(病种包括三尖瓣闭锁、单心室、右心室双出口、大动脉错位、肺动脉闭锁、矫正型大动脉转位、右心室发育不良等)患者,手术年龄7.6±5.5岁.常温非体外循环下手术19例,体外循环下手术105例.右心房-肺动脉连接17例,右心房-右心室连接19例,全腔静脉-肺动脉连接术(TCPC)88例.23例行分期手术.结果 术后早期(术后30d)死亡17例(13.7%),其中行右心房-肺动脉连接者死亡率为23.5%(4/17),行右心房-右心室连接者死亡率为15.8%(3/19),行TCPC者死亡率为11.4%(10/88),同期预留或术后开窗手术死亡率为14.6%(6/41),分期手术患者死亡率为8.7%(2/23).死亡原因低心排血量、多器官功能衰竭和心室颤动等.术后早期并发症发生率为16.9%(21/124),主要为胸腔积液、心律失常、心包积液和低心排血量综合征等.术后随访89例,随访时间6～65个月.远期再住院率6.5%,再手术率0.9%.3例出现再发性胸腔积液,3例出现心包积液,1例出现下腔静脉梗阻,均经相应的治疗后治愈.其余患者心功能恢复好.结论 改良Fontan手术是治疗复杂先天性心脏病中功能性单心室的最佳手术方案;房间隔开窗可明显提高术后早期疗效,减少渗出.     

Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow

Objective: The infant with a functional single ventricle (SV) and unobstructed pulmonary blood flow (UPBF) requires early protection of the pulmonary vascular bed to ensure suitability for a subsequent Fontan procedure. Systemic obstruction by aortic arch obstruction, subaortic stenosis, or combination of both, has been widely recognized as an important risk factor for poor outcome in children with SV–UPBF who are palliated with pulmonary artery banding (PAB). We reviewed our experience with primary PAB in the subset of patients with SV–UPBF to identify risk factors for subsequent palliative procedures and Fontan completion. Methods: Between January 1990 and May 2004, 80 patients (median age, 14 days) with functional SV and UPBF underwent PAB as their primary palliative procedure. Thirty-five neonates had concomitant aortic coarctation or interrupted aortic arch repair (44%). A Damus–Kaye–Stansel procedure was subsequently performed in 19 patients, and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in five. Results: There were 4 operative deaths, and 15 late deaths. The actuarial overall survival is 84% at 1 year, 76% at 5 and 15 years. Follow-up is complete in all but six children at a mean interval of 4.9±3.7 years (range, 2 months–15 years). Thirty-seven patients (49%; 37 of 76) have undergone the hemi-Fontan procedure (with three hospital deaths) and 40 patients (53%; 40 of 76; 12 children without previous hemi-Fontan) have undergone the completion Fontan procedure without mortality or Fontan takedown. Conclusion: In infants with single ventricle physiology with or without systemic outflow obstruction and unobstructed pulmonary blood flow, a strategy of pulmonary artery banding carries acceptable operative and mid-term mortality in a high-risk group of patients. Pulmonary artery banding does not compromise performance of subsequent Damus–Kaye–Stansel procedure or completion Fontan palliation.     

姑息性手术在复杂先天性心脏病患者中的应用

目的探讨姑息性手术在复杂先天性心脏病中的应用策略,提高其治疗效果及生存率。方法回顾性研究华中科技大学同济医学院附属协和医院2004年1月至2011年5月95例复杂先天性心脏病患者接受姑息性手术的临床资料,男62例,女33例;年龄1个月～37岁。施行改良Blalock-Taussig shunt(B-T分流术)12例,改良Brock’s手术23例,双向Glenn手术55例和肺动脉环缩术(Banding手术)5例;对术式的选择、手术效果影响因素等方面进行分析。结果本组共死亡10例,总病死率为10.5%(10/95),其余患者顺利出院。主要并发症为低心排血量综合征、低氧血症和肺部感染等。随访5个月～6年,心功能(NYHA)Ⅰ～Ⅱ级。9例行改良Brock’s手术后患者已完成根治术,6例双向Glenn手术后患者完成全腔静脉-肺动脉吻合术。讨论由于相当部分患者错过了最佳手术干预时机,而影响手术效果;在姑息手术方式的选择策略上,需根据患者肺动脉发育状况、心内畸形特点、最终治疗目标等共同决定。     

小儿重症法洛四联症二期根治术

目的　临床评价小儿重症法洛四联症二期根治术手术疗效。方法　1987年3月至1998年2月51例重症法洛四联症病儿,年龄2.1～13.9岁,平均(5.1±1.9)岁;体重11.1～28.0kg,平均(14.2±3.8)kg。姑息术与二期根治术时间间隔3～22个月。结果　1例远端肺血管发育差者,术后心衰死亡。手术成功率98%,术后随访满意。结论　二期根治术能提高重症法洛四联症病儿术后生存率。     

Mechanical limitation of pulmonary blood flow facilitates heart transplantation in older infants with hypoplastic left heart syndrome.

OBJECTIVES: Progression of pulmonary vascular disease limits heart transplantation for hypoplastic left heart syndrome (HLHS) to early infancy. Our objective was to assess the impact of bilateral pulmonary artery banding (PAB) on the operative courses of HLHS infants transplanted at ages older than 4 months. METHODS: Courses of all HLHS patients in our center who remained listed to age >or=120 days before heart transplantation were assessed. Patients undergoing transplantation after standard management (control group) were compared to patients having a prior pulmonary blood flow limiting procedure (PAB group). RESULTS: Of 16 identified patients, one crossed over to stage I Norwood on day 185 and died post-operatively. Fifteen patients were transplanted at age >or=120 days (control group n=9, PAB group n=6). Four PAB patients had open PA band placement. Two PAB patients underwent experimental percutaneous bilateral internal pulmonary artery flow limiting device insertion. The PAB group mean age at banding was 141+/-54 days, and mean interval from PAB to transplant was 35+/-31 days (range 1.5-68 days). No differences in age at transplant, weight at transplant, warm graft ischemia time or total graft ischemia time were detected between groups. Mean times of mechanical ventilation (control 143+/-69h vs. PAB 44+/-13h), inhaled nitric oxide (control 126+/-70h vs. PAB 37+/-9h), inotropic support (control 171+/-64h vs. PAB 87+/-17h), intensive care unit (ICU) stay (control 8.3+/-2.7 days vs. PAB 4.5+/-1.4 days), and hospital stay (control 10.4+/-3.9 days vs. PAB 7.0+/-1.1 days) were all lower in the PAB group (P<0.05 all comparisons). Two control patients died, three required extracorporeal membrane oxygenation (ECMO), and six did not tolerate primary chest closure. No PAB patient died or required ECMO. All PAB patients tolerated primary chest closure. All PAB patients had widely patent branch pulmonary arteries with no re-interventions to date. All hospital survivors remain alive (mean follow-up, control 50.2 months, PAB 11.5 months). CONCLUSIONS: Pre-transplant mechanical limitation of pulmonary blood flow simplified management and reduced morbidity for HLHS patients undergoing heart transplantation at ages >or=4 months. This strategy extends the permissible transplant waiting time in older infants with HLHS.     

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

BACKGROUND: Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. METHODS: A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. RESULTS: Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end-diastolic volume significantly increased but tricuspid valve diameter did not change. CONCLUSIONS: The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.     

分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的 总结分期全腔静脉.肺动脉连接术(TCPC)治疗复杂先天性心脏病的经验.方法 1998年6月至2008年3月,22例先天性心脏病复杂畸形患者接受分期TCPC.本组中单心室合并肺动脉狄窄9例,合并肺动脉闭锁3例;完全性大动脉转位,十字交叉心,肺动脉瓣狭窄1例;完全性房室通道,左心窒发育不良,肺动脉闭锁,房室瓣少-中量反流1例;完全性房室通道,动脉导管未闭,肺动脉狭窄,双向Glenn术后伴吻合口狭窄房室瓣大量反流1例;镜面右位心,功能性单心室,肺动脉闭锁,动脉导管未闭,体肺侧支,双侧双向Glenn术后2年,右下肺动-静脉瘘1例;三尖瓣闭锁并肺动脉狭窄4例;三尖瓣闭锁伴肺动脉闭锁,动脉导管未闭1例;镜面右位心,右心室双出口,窒间隔缺损,左心室发育小良,功能性单心房,肺动脉瓣狭窄,三尖瓣少量反流,粗大体肺侧支1例.第一期体肺分流术后,二期双向Glenn术5例,第一期单侧或双侧双向Glenn手术17例.行双向Glenn手术的年龄为(5.9±4.4)岁,Glenn术前肺动脉压为17～20 mm Hg(1 mm Hg=0.133 kPa);房室瓣膜关闭不全3例;一期手术前Nakata指数小于200 mm2/m2者4例.接受TCPC术平均年龄为(9.6±4.9)岁,与双向Glenn术间隔时间(3.7 ±1.2)年,术前经上腔静脉置管测得肺动脉压力均小于15 mm Hg.全组22例均采用心外管道TCPC,房窜瓣成形1例.结果 全组住院死亡1例,病死率为4.5%.死亡病例为单心室,肺动脉闭锁,第一期体肺分流术后,二期左肺动脉成形双向Glenn术后5年第三期行TCPC,术后左肺反复多次大出血死亡.余患者均顺利出院,术后中心静脉压12～18 mm Hg.出院前经皮测血氧饱和度为90%～96%,活动量明显增加,发绀及气促症状消失,心功能均为NYHA分级Ⅰ～Ⅱ级.结论 对于高危患者根据病情选择分期TCPC,可以使更多有高危因素不能行Fontan手术的患者得到救治的机会,扩大手术适应证,并取得满意疗效.     

Classic Blalock-Taussig shunt in neonates

BACKGROUND: The choice of palliative procedure for neonates with congenital cyanotic heart defects remains controversial. Several reports have questioned whether the classic Blalock-Taussig shunt is adequate in neonates, because it shows a high rate of mortality and early shunt failure. The purpose of this study is to evaluate the early and late results of classic Blalock-Taussig shunt in neonates. METHODS: From December 1981 to December 1996, 31 neonates underwent a classic Blalock-Taussig shunt. Mean age at operation was 15.9 days (range, 2 to 28 days), and mean body weight was 3214 g (range, 2229 to 4468 g). The major diagnoses were pulmonary atresia with intact ventricular septum (6 patients) and with ventricular septal defect (4), univentricular heart (6), tricuspid atresia (5), tetralogy of Fallot (4), transposition of the great arteries (3), and double outlet right ventricle (3). RESULTS: There were no early deaths. There was one early shunt failure. The patient underwent a replacement of Blalock-Taussig shunt by vascular graft on the 1st postoperative day. Two patients with non-confluent central pulmonary artery underwent a contralateral modified Blalock-Taussig shunt on the 22nd and 42nd postoperative day, respectively. There were two late deaths before the definitive repair was performed. Twelve patients required an additional shunt operation. The mean interval between the initial and the second shunt procedure was 27.7 months (range, 6 to 67 months). Ten patients underwent definitive operation and the mean interval to that procedure was 55.5 months (range, 14 to 121 months). Shunt patency was 94.7+/-5.1% at 3 years. The freedom from cardiac event (cardiac deaths or repeat shunting procedure) was 80.5+/-7.1% at 1 year and 54.9+/-9.4% at 3 years. CONCLUSIONS: The classic Blalock-Taussig shunt has a low operative mortality and has provided excellent long-term palliation in the neonate. We conclude that a classic Blalock-Taussig shunt should be considered a reasonable choice in neonates requiring a palliative procedure for congenital cyanotic heart defects.     

Arterial switch after failed atrial baffle procedures for transposition of the great arteries

BACKGROUND: Late failure of the systemic right ventricle after atrial baffle procedures in patients with transposition of the great arteries poses significant management problems. We reviewed our experience with staged conversion to arterial switch operation (ASO) in these patients. METHODS: Between 1984 and 1999, 11 patients underwent pulmonary artery band (PAB) to prepare the left ventricle for ASO conversion. One additional patient had subpulmonic stenosis and was naturally prepared. Mean age at the initial PAB was 12.2+/-7 years (range, 1.9 to 23 years). Four patients underwent reoperation to tighten the PAB before ASO. Mean interval from PAB to ASO was 1.3+/-0.9 years. RESULTS: There was no mortality from PAB. Six patients had ASO conversion and 2 died. Recent surgical modifications at the time of ASO were used to prevent neoaortic valve insufficiency and to cryoablate atrial reentry tachycardia. Four patients developed biventricular failure after PAB and had orthotopic cardiac transplantation (OCT) 14+/-10 months after PAB. The other 2 patients are still with PAB: 1 is awaiting ASO conversion and the other has insufficient left ventricular hypertrophy necessary for ASO conversion despite two preparatory PABs. CONCLUSIONS: A select group of patients with right ventricular failure after atrial baffle operations can undergo staged conversion to ASO with the opportunity for excellent long-term outcome. Surgical modifications at the time of ASO can address the problems of neoaortic insufficiency and persistent atrial arrhythmias. PAB may be a therapeutic endpoint in some patients not responding with adequate left ventricular hypertrophy. Those patients who develop biventricular failure after PAB will require cardiac transplantation.     

低出生体重儿及早产儿心脏手术的早期疗效分析

目的分析60例先天性心脏病(先心病)低出生体重儿(low birth weight infant)和早产儿行外科手术治疗的早期疗效。方法回顾性分析自2003年5月至2011年10月广东省心血管病研究所60例患先天性心脏病的低出生体重儿(体重2 500 g)和早产儿施行心脏手术治疗的临床资料,其中男43例,女17例;年龄4～55(24.9±12.5)d;孕周26～42(33.5±4.1)周,其中47例为早产儿;出生时体重640～2 500(1 729.3±522.5)g,手术时体重650～2 712(1 953.2±463.6)g。术前所有患者均在新生儿重症监护病房(NICU)接受治疗,在全身麻醉下行先心病矫治手术,其中行非体外循环手术29例,体外循环手术31例。术后住NICU进行监护治疗,并观察并发症发生情况和死亡情况。结果住院期间共死亡13例,总病死率为21.7%(13/60)。其中术中死亡4例,术后早期(72 h内)死亡6例,放弃治疗3例。体外循环时间(121.0±74.7)min,升主动脉阻断时间(74.8±44.7)min,术后呼吸机辅助呼吸时间(136.9±138.1)h。术后延期关胸13例,床旁开胸探查止血8例,发生重症肺炎10例,肺动脉高压危象2例,低心排血量综合征8例,均经相应的治疗治愈或好转。随访47例,随访时间2～12个月,患者均生存。结论对危重先心病低出生体重儿或早产儿早期施行外科手术治疗是安全、有效的。