完全性大动脉转位患儿外科术后早期并发症和近期随访

Objective To present the short-term outcomes of the patients with transposition of the great arteries after repair. Methods Between 2008 and 2009,71 patients with transposition of the great arteries (TGA) underwent corrective surgeries at this center. The 71 TGA patients were divided into TGA/IVS group whose ventricle septum was intact, and TGA/VSD group who complicated with ventricle septum defect. Their clinical data and postoperative short-term outcomes were analyzed and compared. Results The postoperative complications included 7 (9. 9% ) patients presented with low cardiac output syndrome, 12 ( 16. 9% ) with pulmonary stenosis, 21 ( 29. 6% ) with arrhythmia, 6 (8. 5%) with diaphragmatic paralysis, 6 (8. 5%) with airway abnormalities, and 13 ( 18. 3% ) with bacteria in sputum culture. Three patients (4. 2%) died after surgery. Fifty four out of the 68 patients were followed up for an average period of 12. 0 ± 7. 4 months. Four (7. 4%) patients died during follow-up. Three patients (5. 6%) underwent interventional therapies for pulmonary stenosis. The survival rate of 57 patients was 87. 7% at the end of the first postoperative year. No differences of the postoperative complications and survival rate were noted between the TGA/IVS and TGA/VSD group.Conclusions The postoperative short-term outcomes of the patients with transposition of the great arteries are good.     

功能性单心室的外科治疗及近期疗效

目的 总结功能性单心室的外科治疗经验.方法 回顾性分析我科2003年12月至2010年7月59例功能性单心室患儿,对其手术时机,手术方式及手术效果进行分析,并用Logistic回归分析Glenn手术的高危因素.结果 死亡8例(病死率13.56%),其中4例为Glenn术后死于低心排和多器官功能衰竭,2例为Banding术后死于低心排,1例为TCPC术后死于多器官功能衰竭,1例为B-T分流术后死于低心排.Glenn术后的主要并发症为上腔静脉梗阻综合症和低心排,Fontan术后的主要并发症是房性心律失常和胸腔积液.Logistic回归分析得出房室瓣反流是Glenn术后患儿死亡的高危因素.结论 功能性单心室心内畸形复杂,需根据肺血流的情况及早进行姑息手术,选择合适的术式.改良Fontan是进行生理矫正的理想术式,保留肺动脉前向血流的Glenn手术是保证患儿顺利进行Fontan术的有效方法,但需根据肺动脉狭窄的程度进行处理,避免上腔静脉综合症的发生.

Abstract：

Objective To retrospectively analyze the outcome of surgical treatment of patients with functional single ventricle. Methods Betveen December 2003 and July 2010, 59 patients with functional single ventricle underwent corrective surgeries, and were followed up at this center. Their median age was 3. 0 months old at pulmonary artery band surgery (PAB), 13. 5 months at bidirectional Glenn procedure (BDG), and 11 years old at Fontan operation. The variables of the anatomical lesions with single ventricle physiology, combined abnormalities, age at surgery, and type of the surgical procedure were retrospectively analyzed. Logistic regression analysis was also performed to determine the risk factors associated with Glenn. Results Duration of mechanical ventilation was 30. 4 ± 19. 7 hours. Length of stay in ICU was 4. 3 ± 1.6 days. Length of hospital stay was 34. 8 ± 15. 9 d.SpC2 increased from 75 ± 6. 97% to 88 ± 6. 28% after operation. Exercise tolerance was also improved remarkably. Eight deaths were noted after surgery (13. 56%). The leading complications after Glenn surgery were superior vena cava syndrome and low cardiac output syndrome. Complications after Fontan surgery were atrial tachycardia and pleural effusions. Logistic regression analysis suggested atrioventricular valve regurgitation was an important risk factor for the death of the patients had Glenn surgery. Conclusions The clinical outcome of surgical treatment of patients with functional single ventricle is satisfied. The surgical strategy should be made according to anatomic abnormality of the functional single ventricle and pulmonary circulation. Modified Fontan operations can become an alternative therapy for patients who are not suitable for double ventricle correction. Glenn with antegrade pulmonary blood flow may be a temporary palliation prior to Fontan surgery, but the pulmonary blood flow should be controlled to avoid the occurrence of superior vena cava syndrome.     

与先天性心脏病相关的NUSS术

目的 探讨同期治疗合并先天性心脏病(先心)的漏斗胸及先心术后漏斗胸应用NUSS术的方法及可行性.方法 统计我院2006年7月至2010年6月与先心相关的漏斗胸15例,A组8例合并先心的漏斗胸患儿在同期行心脏手术和NUSS术,其中男5例,女3例,年龄4岁～13岁4个月(平均6岁1个月).其中4例行室间隔缺损经胸伞封术,2例行房间隔缺损经胸伞封术,1例在体外循环下行室间隔缺损修补术,1例在体外循环下行右室双腔矫治及室间隔缺损修补术.B组7例均为先心术后的漏斗胸行NUSS术,其中男5例,女2例,先心手术年龄6个月～3岁10个月(平均1岁9个月),NUSS术年龄4岁7个月～8岁(平均6岁2个月),6例为体外循环下室间隔缺损修补术后,1例为体外循环下法乐四联症矫治术后.术后常规放置有心包纵隔和/或胸腔引流管.结果 所有患儿手术顺利,A组术后5～14 h拔除气管插管,平均(8.8±2.6)h.B组术后4～8 h拔除气管插管,平均(5.9±1.2)h,48～72 h拔除心包纵隔或胸腔引流管.无手术死亡、大出血及胸腔脏器损伤等并发症.术后检查先心矫治效果良好,肺复张良好.术后两组各出现1例切口延期愈合,经治疗后,均顺利出院.随访6个月～4年效果良好.结论 NUSS术用于治疗先心术后漏斗胸以及同期治疗合并先心的漏斗胸安全可行,可有效降低或避免二次手术的难度及风险.

Abstract：

Objective To investigate the clinical outcomes of combining corrective surgery for congenital heart disease (CHD) and NUSS surgery for pectus excavatum (PE) as one-staged operation. Methods From July 2006 to June 2010, 15 children with CHD associated with PE were recruited in this study, and divided into two groups: group A underwent one-staged and group B with twostaged operation. Group A had 8 patients including 5 males and 3 females, aging from 4 to 13.4 years (mean, 6 years and 1 month). Among the 8 patients, 6 with ventricular septal defect (VSD) and 2with atrial septal defect (ASD) underwent interventional or open repair. After the corrective surgery for their CHD, the 8 patients underwent NUSS procedure for pectus excavatum. The group B had 7patients, including 5 males and 2 females. Among them, 6 had VSD and 1 had tetralogy of fallot (TOF). At the first stage, the patients underwent corrective surgery to repair VSD and TOF under CPB. At the second stage, the patients were performed NUSS surgery to correct PE. Their ages at surgery were 4 years and 7 months to 8 years old (mean, 6 years and 2 months). Results All operations were finished successfully. The endotracheal tube was removed 5 to 14 hours after surgery on Group A patients (mean, 8.75 ± 2.59 h), and 4 to 8 hours on Group B patients (mean, 5.86 ±1. 24 h). The drainage tubes of pericardium, mediastinal or chest were removed 48 to 72 h later after surgery. The patients were followed up for 6 months to 4 years. No surgery-related death, hemorrhage, thoracic organ dysfunction and other severe complications were noted. Delayed wound healing was observed on 1 patient in each group. Others recovered from surgery and were discharged from the hospital. Conclusions It is safe to combine corrective surgery for CHD and NUSS procedure for PE as one stage operation.     

经右室穿刺肺动脉瓣球囊扩张治疗室间隔完整型肺动脉闭锁

目的 评价经右心室穿刺肺动脉瓣球囊扩张杂交手术治疗室间隔完整型肺动脉闭锁的早期治疗结果.方法 6例室间隔完整型肺动脉闭锁患儿接受右心室穿刺肺动脉瓣球囊扩张杂交手术.术前平均经皮氧饱和度(71±10)%.结果 全组无死亡及围术期并发症发生.术后经皮氧饱和度为(89±5)%;右室-肺动脉压差为(19±11)mm Hg.随访(4±3.6)个月,跨肺动脉瓣压差(45±42)mm Hg,1例术后再次行经导管肺动脉瓣球囊扩张.结论 对于右心室发育良好的室间隔完整型肺动脉闭锁,本术式早期疗效满意,安全性较高,术后肺动脉瓣反流少,可以促进右心室的发育.

Abstract：

Objective To evaluate the early outcomes of balloon valvuloplasty via right ventricle pulmonary valve for the treatment of pulmonary atresia with intact ventricular septum in children.Methods Methods Between January 2010 and December 2010, 6 patients were diagnosed with pulmonary atresia with intact ventricular septum, and underwent balloon valvuloplasty via right ventricle pulmonary valve at this center. Of the 6 patients, the mean body weight was 3. 8 ± 1.6 kg, the mean transcutaneous oxygen saturation was 71 ± 10%, and the mean age was 56 ± 45 days. All of the 6 patients had tripartite right ventricles. Their tricuspid valve z-scores ranged from -2 to 2. 2 (mean zscore: 0. 4 ± 1. 5). Results During operation, the mean size of the balloons used was 7. 3 ± 1. 2 mm,the ratio of the balloon size to pulmonary valve ring was 1.0 ± 0. 1, the pressure gradient between right ventricle and main pulmonary artery was 19 ± 11 mmHg, the ratio of forward flow from right ventricle to that from ductus arteriosus was 0. 35 ± 0. 06. After surgery, the mean ventilation time was 3. 2 ± 1.0 days, and mean ICU stay was 16 ± 5 days. The post-operative transcutaneous oxygen saturation was 89 ± 5%. The patients were followed up for 4 ± 3. 6 months. No death and post-operative complications were noted. One patient underwent interventional balloon valvuloplasty 2 weeks after the first surgery. The transcutaneous oxygen saturation on last follow-up was 85 ± 6%, which was significantly improved after surgery (t = -2. 66, p = 0. 045). The postoperative pressure gradient between right ventricle and main pulmonary artery was 45 ± 42 mmHg. Four of the 6 patients had pulmonary regurgitation, 3 of whom were mild and 1 was moderate. Tricuspid valve z-scores (mean, 0. 5± 1) also improved compared with that before procedure (t = -3. 36, p = 0. 02). Conclusions Balloon valvuloplasty via right ventricle pulmonary valve is safe and effective to treat the pulmonary atresia with intact ventricle septum in children.     

应用儿童终末期肝病模型评分预测Kasai术后联合激素治疗胆道闭锁的短期预后

Objective To assess the effects of steroid therapy on the biliary atresia patients after Kasai portoenterostomy using pediatric of end-stage liver disease (PELD) scoring system. Methods Eighty patients with biliary atresia were enrolled in this study. The patients were randomly grouped into Kasai surgery group and combined therapy group. The patients of Kasai surgery group were performed Kasai portoenterostomy. The patients in combined therapy group underwent Kasai portoenterostomy and steroid therapy after surgery. Each patient's PELD score was calculated. Biochemical parameters,mortality,and PELD scores of two groups were compared. Results In the patients with a PELD less than 16, the levels of total bilirubin (TBIL), prothrombin time-international normalized ratio (INR) and PELD score of combined therapy patients were 23. 3 ± 1. 1 mg/L,2. 31 ± 0. 24,and 10. 6 ± 2.3, respectively; which were significantly lower than these parameters of the Kasai surgery group patients (TBIL,28. 9 ± 2. 1 mg/dL; INR,2. 63 ± 0. 18; PELD, 13. 2 ± 2.7). The combined therapy patients' albumin was also significantly higher than that of Kasai surgery group patients (P＜0. 05). The same results were also seen among the patients with a PELD between 16 and 28. Among the patients with a PELD higher than 28,only the difference of TBIL between the Kasai surgery group and combined therapy group was found. The mortality of the combined therapy patients was significantly lower than that of Kasai surgery group patients among the patients whose PELD ＜28. However, in the patients with a PELD≥28,no difference of the mortality was found between the two groups. Conclusions In the BA patients with PELD less than 27, Kasai portoenterostomy plus postoperative steroid therapy can improve the outcomes and prognosis.     

Therapeutic laparoscopy for superior mesenteric artery syndrome in 5 children: Treatment experience and lite-rature review北大核心CSCD

Objective To investigate the clinical characteristics and therapeutic laparoscopy treatment for superior mesenteric artery syndrome(SMAS) in children. Methods A retrospective study on 5 children with SMAS who failed to respond to the conservative treatment in the Second Hospital of Shandong University from March 2017 to March 2022 was carried out.All the patients underwent the laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure.The clinical data were analyzed and literature retrieved from Wanfang and PubMed databases were reviewed. Results There were 3 boys and 2 girls in the 5 cases with SMAS.The average age was (12.4±1.4) years, and the preoperative body mass index was (15.2±0.8) kg/m2.The patients were admitted to the hospital with vomiting, abdominal pain and emaciation symptoms, and the duration of symptoms was (8.4±7.8) months.Gastroscopy, laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure were performed successfully in all the patients, without conversions to open surgery.The average operation duration was (66.0±10.7) min.Food intake was allowed 1 day after surgery.Chylous fistula occurred 4 days after operation in one patient, whose symptom relieved after 11 days of conservative treatment.One patient still had nausea and vomiting symptoms 10 days after operation and was healed by knee-chest posture treatment.No other short-term complications were observed.The median postoperative duration of stay in the hospital was 7 (6-22) days.The patients were followed up for 9-56 (median: 21) months.All of them recovered well and gained weight.One patient had mild recurrent symptoms, and was cured after conservative treatment.A total of 15 literature on the treatment of SMAS by laparoscopic lysis of the ligament of Treitz was retrieved, and the cure rate was 40%-100%.Only one group of these cases had a cure rate below 75%. Conclusions The laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure is safe, effective, simple and minimally invasive treatment for children with SMAS.It can be used as the first choice for most pediatric patients. © 2022 Chinese Journal of Applied Clinical Pediatrics. All rights reserved.     

吸入伊洛前列素对先天性心脏病患儿术后肺动脉高压的治疗作用

Objective To study the therapeutic effects of inhaled iloprost on pediatric pulmonary hypertension after congenital heart disease operation and to investigate the mechanisms of iloprost.Methods Thirteen 10 min every time,every 4 hours for 48 h after the surgery.We used the 24 h cardiac monitoring and ultrasonic cardiography for hemodynamical monitoring.We examined the blood cAMP and cGMP by ELISA assay.Results The patients were(17.88±12.56) months old with average weight of(9.29±3.59) kg.The right atrial pressure,systolic pulmonary arterial pressure,systolic pulmonary arterial pressure/systolic blood pressure were(11.5±1.41)mm Hg,(61.64±13.6) mm Hg and 0.66±0.16 before the treatment and they were(9.88±1.88) mm Hg,(47.67±12.18) mm Hg and 0.52±0.15 at the time point of 20 min after the treatment.For all the three parameters,there were significant differences before and after treatment.The effect of iloprost continues to 120 min later.The cAMP level was(335.75±127.31) μg/L before iloprost inhalation and increased to(519.68±148.54)μg/L at the time point of 20 min after inhaling iloprost,showing a significant difference(P＜0.01).Blood pressure,cGMP level and ventilator parameters showed no notable difference before and after the treatment.Conclusion Inhaled iloprost significantly improves pulmonary hemodynamics in children with pulmonary hypertension after congenital heart disease operation,which might be achieved by increasing the blood cAMP.However,systemic blood pressure and respiratory function keep unaffected after iloprost inhalation.     

吸入伊洛前列素对先天性心脏病患儿术后肺动脉高压的治疗作用

Objective To study the therapeutic effects of inhaled iloprost on pediatric pulmonary hypertension after congenital heart disease operation and to investigate the mechanisms of iloprost.Methods Thirteen 10 min every time,every 4 hours for 48 h after the surgery.We used the 24 h cardiac monitoring and ultrasonic cardiography for hemodynamical monitoring.We examined the blood cAMP and cGMP by ELISA assay.Results The patients were(17.88±12.56) months old with average weight of(9.29±3.59) kg.The right atrial pressure,systolic pulmonary arterial pressure,systolic pulmonary arterial pressure/systolic blood pressure were(11.5±1.41)mm Hg,(61.64±13.6) mm Hg and 0.66±0.16 before the treatment and they were(9.88±1.88) mm Hg,(47.67±12.18) mm Hg and 0.52±0.15 at the time point of 20 min after the treatment.For all the three parameters,there were significant differences before and after treatment.The effect of iloprost continues to 120 min later.The cAMP level was(335.75±127.31) μg/L before iloprost inhalation and increased to(519.68±148.54)μg/L at the time point of 20 min after inhaling iloprost,showing a significant difference(P＜0.01).Blood pressure,cGMP level and ventilator parameters showed no notable difference before and after the treatment.Conclusion Inhaled iloprost significantly improves pulmonary hemodynamics in children with pulmonary hypertension after congenital heart disease operation,which might be achieved by increasing the blood cAMP.However,systemic blood pressure and respiratory function keep unaffected after iloprost inhalation.     

吸入伊洛前列素对先天性心脏病患儿术后肺动脉高压的治疗作用

Objective To study the therapeutic effects of inhaled iloprost on pediatric pulmonary hypertension after congenital heart disease operation and to investigate the mechanisms of iloprost.Methods Thirteen 10 min every time,every 4 hours for 48 h after the surgery.We used the 24 h cardiac monitoring and ultrasonic cardiography for hemodynamical monitoring.We examined the blood cAMP and cGMP by ELISA assay.Results The patients were(17.88±12.56) months old with average weight of(9.29±3.59) kg.The right atrial pressure,systolic pulmonary arterial pressure,systolic pulmonary arterial pressure/systolic blood pressure were(11.5±1.41)mm Hg,(61.64±13.6) mm Hg and 0.66±0.16 before the treatment and they were(9.88±1.88) mm Hg,(47.67±12.18) mm Hg and 0.52±0.15 at the time point of 20 min after the treatment.For all the three parameters,there were significant differences before and after treatment.The effect of iloprost continues to 120 min later.The cAMP level was(335.75±127.31) μg/L before iloprost inhalation and increased to(519.68±148.54)μg/L at the time point of 20 min after inhaling iloprost,showing a significant difference(P＜0.01).Blood pressure,cGMP level and ventilator parameters showed no notable difference before and after the treatment.Conclusion Inhaled iloprost significantly improves pulmonary hemodynamics in children with pulmonary hypertension after congenital heart disease operation,which might be achieved by increasing the blood cAMP.However,systemic blood pressure and respiratory function keep unaffected after iloprost inhalation.     

吸入伊洛前列素对先天性心脏病患儿术后肺动脉高压的治疗作用

Objective To study the therapeutic effects of inhaled iloprost on pediatric pulmonary hypertension after congenital heart disease operation and to investigate the mechanisms of iloprost.Methods Thirteen 10 min every time,every 4 hours for 48 h after the surgery.We used the 24 h cardiac monitoring and ultrasonic cardiography for hemodynamical monitoring.We examined the blood cAMP and cGMP by ELISA assay.Results The patients were(17.88±12.56) months old with average weight of(9.29±3.59) kg.The right atrial pressure,systolic pulmonary arterial pressure,systolic pulmonary arterial pressure/systolic blood pressure were(11.5±1.41)mm Hg,(61.64±13.6) mm Hg and 0.66±0.16 before the treatment and they were(9.88±1.88) mm Hg,(47.67±12.18) mm Hg and 0.52±0.15 at the time point of 20 min after the treatment.For all the three parameters,there were significant differences before and after treatment.The effect of iloprost continues to 120 min later.The cAMP level was(335.75±127.31) μg/L before iloprost inhalation and increased to(519.68±148.54)μg/L at the time point of 20 min after inhaling iloprost,showing a significant difference(P＜0.01).Blood pressure,cGMP level and ventilator parameters showed no notable difference before and after the treatment.Conclusion Inhaled iloprost significantly improves pulmonary hemodynamics in children with pulmonary hypertension after congenital heart disease operation,which might be achieved by increasing the blood cAMP.However,systemic blood pressure and respiratory function keep unaffected after iloprost inhalation.     

668例婴幼儿室间隔缺损的手术治疗

目的：探讨近5年间外科手术治疗婴幼儿期室间隔缺损(VSD)的结果和经验。方法：对1998--2002年的668例婴幼儿VSD修补术进行总结：年龄1～36个月(平均15-8个月)，体重3．0～15．5k(平均8．1kg)。膜周部VSD 562例，干下型VSD 102例．膜周部并肌部VSD4例。术前中度以上肺动脉高压386例(57．8％)。因难治性肺炎或心衰而急诊手术89例(13．3％)。采用中度低温体外循环下手术。结果:全组手术死亡6例，手术病死率为0．9％(6／668)：术后主要并发症为肺部感染、心律失常和肺动脉高压症。结论：随着麻醉、体外循环、手术技术和围术期监护处理措施的进步，在婴幼儿期施行室间隔缺损的外科手术已成为安全的治疗方式。     

婴儿法洛四联症术后并发症处理及一年随访

目的 总结婴儿法洛四联症(TOF)术后早期并发症的处理及术后1年的随访结果 .方法 收集2004年1月至2007年3月安徽省立儿童医院婴儿TOF术后35例,年龄4～12个月.全组均在全身麻醉、低温体外循环下进行手术,其中一期根治30例,姑息术5例.结果 10例出现术后早期并发症,合并低心排血量综合征6例,严重心律失常2例,灌注肺2例.随访1年,发现右室流出道严重残余梗阻2例,残余漏1例.围手术期死亡2例,死因均为低心排血量综合征,术后4个月死亡1例,死因为单肺灌注,病死率8.6%.31例患儿心功能评级为纽约心脏协会(NYHA)分级Ⅰ或Ⅱ级.结论 充分的术前准备、合理的手术方法 和心肌保护、及早发现和正确处理并发症、对少尿患儿尽早行腹膜透析,可有效地降低婴儿TOF围手术期的病死率.对于有症状的TOF病例,应在婴儿期行根治手术,能收到满意的效果.     

两种手术方法对肺动脉闭锁伴室间隔缺损预后影响的探讨

目的对近年来本院实施的肺动脉闭锁伴室间隔缺损(PA/VSD)一期根治术患儿手术方式及右室流出道重建方法进行总结,结合术后院内各项指标进行比较分析;对比同期一期姑息PA/VSD手术的术后生存率、并发症等情况,藉此对一期手术方案的选取进行评价。方法本院2008年1月至2014年3月施行单纯PA/VSD一期根治手术129例为根治组,收集患儿性别、手术时年龄、身高、体重、术前经皮氧饱和度、McGoon、Nakata指数、手术方式、右室流出道重建材料、术中体外循环、主动脉阻断时间、术后置CICU时间、住院时间、术后最大正性肌力药物评分、院内并发症及死亡率情况;另外收集本院120例P/VSD一期姑息手术患儿相应资料作为对比,为姑息组。结果一期根治组早期院内死亡10例,病死率7.8%;术前McGoon指数1.6±0.5,体外循环时间(130.0±45.6)min;主动脉阻断时间(77.2±23.5)min;呼吸机支持时间(87.0±92.8)h;住院时间(22.6±11.4)d。一期姑息组院内死亡11例,病死率9.2%;McGoon指数1.2±0.3,体外循环时间(89.7±46.4)min;呼吸机支持时间(47.4±50.5)h;住院时间(18.6±7.0)d;结论术前McGoon≥1.2,SpO_2≥75%可视为一期根治术的适应证;术中监测RVP/LVP比值是预防术后早期院内死亡的重要方法;对于伴有粗大主肺动脉侧支(MAPCAs)的患儿,提倡采取早期根治手术。     

个体化手术方案矫治儿童右室双出口的体会

目的 探讨儿童右室双出口(DORV)的外科治疗效果.方法 自1984年8月至2010年6月共手术治疗118例DORV,其中男62例,女56例,年龄10 d～12岁,平均(3.2±2.6)岁,体重3.0～22.5 kg,平均(9.6±3.7)kg.根据SIS-EACTS数据库分类:VSD型86例,TOF型22例,TGA型3例,VSD远离大动脉型7例.手术方法包括采用单纯心室内隧道修补21例,心室内隧道修补+右室流出道扩大补片84例,其中82例自体心包扩大补片,2例行同种带瓣外管道;Switch术4例,双向格林术6例,Fontan术1例,肺动脉环缩术1例,B-T分流术1例.回顾性分析DORV的手术方法和效果.结果 住院死亡9例,病死率7.6%,主要死亡原因为低心排综合症.2004年前手术51例,死亡7例(病死率13.7%),2005年后手术67例,死亡2例(病死率2.9%).存活病例随访2个月～10年,超声心动图检查未见左室流出道梗阻,1例患儿术后2年发现右室流出道残余梗阻,再次手术疏通右室流出道,余患儿效果良好,无晚期死亡病例.结论 根据DORV患儿的解剖特点和年龄,制订个体化的手术方案,有助于提高手术成功率.

Abstract：

Objective To summarize our experience of surgical treatment for double-outlet right ventricle (DORV) in children. Methods From August 1984 to June 2010, 118 patients with DORV underwent surgical repair at this center. Among the 118 patients, 62 were males and 56 were females.Their ages ranged from 10 days to 12 years old (mean age, 3. 2 ± 2. 6). Their weight ranged from 3. 0to 22. 5 kg (mean weight, 9. 6 ± 3. 7 kg). According to the STS-EACTS international nomenclature,86 patients were DORV associated with ventricular septal defect (VSD), 22 were DORV with tetralogy of Fallot (TOF), 3 were DORV with transposition of the greater arteries (TGA), and 7 were DORV with remote VSD. Corrective surgeries included 22 interventricular repairs, 84 interventricular repairs plus right ventricular outflow tract reconstruction (pericardium was used for the reconstruction in 82 cases, and valved extracardiac conduits was used in 2 cases), 4 switch procedures, 6 bidirectional Glenn procedures, 1 Fontan procedure, 1 Pulmonary artery banding procedure, and 1 Blalock-Taussig Shunt procedure. The surgical safety and efficacy were studied by retrospectively analyzing the clinical data of the 118 cases. Results Nine patients died after surgery (7. 6%). The leading cause of death was low cardiac output syndrome. Of the 51 patients underwent corrective surgery before 2004,7 died after surgery (13. 7%). However, only 2 died in the 67 patients who had surgery after 2005(2. 9%). The patients were followed up for 2 months to 10 years. Cardiac ultrasonography didn't show any obstruction of left ventricular outflow tract, but 1 patient developed left ventricular outflow tract obstruction 2 years after surgery and received corrective surgery. No long-term death and other complications were noted. Conclusions Surgical strategy for the children with double-outlet right ventricle should be made based on patients individual anatomic anomalies.     

先天性心脏病肺动脉高压手术前后右心功能变化和治疗

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿，用心导管的方法进行术前、术后5～7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排指数显著高于术后；术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高，缺损修补后右室舒张压恢复正常，收缩压的降低与肺动脉压力下降有关；大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出量心力衰竭，且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物；降低肺动脉压力、减少左向右分流、根治心内畸形是合理的选择；术后右室收缩压持续不能恢复至正常水平，提示继发性肺血管梗阻性病变存在。     

小儿体外循环心脏术后呼吸机相关性肺炎的危险因素分析

目的 探讨小儿体外循环心脏术后呼吸机相关性肺炎(ventilator-associated pneu monia,VAP)的危险因素.方法对2003年1月至2010年6月连续体外循环(cardiopulmonany bypass,CPB)心脏术后机械通气时间超过48 h的116例患儿的临床资料进行回顾性分析,根据发生VAP与否进行分组,并进行术后VAP的单因素和多因素Logistic回归分析.结果CPB心脏术后VAP总发病率为4.18％(57/1 365),发生VAP患儿病死率为26.32％(15/57).单因素分析显示:重度肺动脉高压、CPB时间、主动脉阻断时间、二次体外循环支持、白蛋白、术后PaO2/FiO2、机械通气时间、每千克体质量引流液总量、二次气管插管、气管切开、留置胃管、应激性溃疡预防用药与术后发生VAP有关.经多因素Logistic回归分析显示:CPB时间≥150m in、机械通气时间≥4 d、每千克体质量引流液总量≥18ml和气管切开为小儿CPB术后发生VAP的危险因素.57例VAP患儿中共培养病原菌90株,其中革兰阴性菌54株(60.0％),革兰阳性菌32株(35.6％),真菌4株(4.4％).结论临床上可根据VAP的危险因素预先采取措施,以减少VAP的发生.     

完全性大动脉转位患儿外科术后早期并发症和近期随访

目的 探讨完全性大动脉转位(TGA)患儿术后早期并发症及近期预后情况.方法 对2008年至2009年行手术治疗的71例TGA患儿基本资料进行回顾性分析,并根据室间隔发育情况分为室间隔完整组(TGA/IVS)和室间隔缺损组(TGA/VSD),探讨术后早期并发症及近期预后情况.结果 术后早期出现低心输出量综合征7例(9.9%)、肺动脉狭窄12例(16.9%)、心律失常21例(29.6%)、膈肌麻痹6例(8.5%)、气道异常6例(8.5%)、下呼吸道分泌物培养细菌阳性13例(18.3%);术后院内死亡3例(4.2%).随访68例出院患儿,失访14例(20.6%),余54例(79.4%)随访时间(12.0±7.4)个月,其中4例(7.4%)死亡,3例(5.6%)因肺动脉狭窄需要介入治疗.可随访的57例患儿术后1年存活率87.7%.TGA/IVS组和TGA/VSD组在术后早期并发症和近期存活率方面差异均无统计学意义.结论 TGA患儿术后尽管可能出现一些并发症,但其预后良好,近期存活率较高.     

小儿法乐四联症围术期的治疗策略

目的总结法乐四联症（TOF）患儿手术治疗经验,探讨手术时机、手术适应证和围术期处理。方法回顾性分析我科2004年1月至2010年6月收治的132例小儿TOF的临床资料,其中男98例,女34例;年龄4个月至12岁,平均12．85岁。术前均行超声心动图或心导管造影检查确诊,均在体外循环下行根治性手术,部分病例以自体心包或带瓣自体心包扩大右室流出道和（或）肺动脉,对合并畸形作相应处理。结果本组围术期死亡4例（3．03％）,其中2例死于术后多器官功能衰竭,2例死于低心排出量综合征。随访129例,随访时间12～90个月;失访3例。6例残余微量分流,生长发育正常,无不适。结论对症状显著的TOF患儿应尽早手术矫治,提高手术水平,加强术后监护是确保手术成功的关键。     

High-Frequency Oscillatory Ventilation for Cardiac Surgery Children With Severe Acute Respiratory Distress Syndrome

Acute respiratory distress syndrome (ARDS) in children after open heart surgery, although uncommon, can be a significant source of morbidity. Because high-frequency oscillatory ventilation (HFOV) had been used successfully with pediatric patients who had no congenital heart defects, this therapy was used in our unit. This report aims to describe a single-center experience with HFOV in the management of ARDS after open heart surgery with respect to mortality. This retrospective clinical study was conducted in a pediatric intensive care unit. From October 2008 to August 2012, 64 of 10,843 patients with refractory ARDS who underwent corrective surgery at our institution were ventilated with HFOV. Patients with significant uncorrected residual lesions were not included. No interventions were performed. The patients were followed up until hospital discharge. The main outcome measure was survival to hospital discharge. Severe ARDS was defined as acute-onset pulmonary failure with bilateral pulmonary infiltrates and an oxygenation index (OI) higher than 13 despite maximal ventilator settings. The indication for HFOV was acute severe ARDS unresponsive to optimal conventional treatment. The variables recorded and subjected to multivariate analysis were patient demographics, underlying disease, clinical data, and ventilator parameters and their association with hospital mortality. Nearly 10,843 patients underwent surgery during the study period, and the ARDS incidence rate was 0.76 % (83/10,843), with 64 patients (77 %, 64/83) receiving HFOV. No significant changes in systemic or central venous pressure were associated with initiation and maintenance of HFOV. The complications during HFOV included pneumothorax for 22 patients. The overall in-hospital mortality rate was 39 % (25/64). Multiple regression analyses indicated that pulmonary hypertension and recurrent respiratory tract infections (RRTIs) before surgery were independent predictors of in-hospital mortality. The findings show that HFOV is an effective and safe method for ventilating severe ARDS patients after corrective cardiac surgery. Pulmonary hypertension and RRTIs before surgery were risk factors for in-hospital mortality.