婴幼儿主动脉缩窄合并心内复杂畸形的一期手术治疗

目的:探讨婴幼儿主动脉缩窄合并心内复杂畸形一期手术的治疗方法及手术效果.方法:回顾分析2002-11至2008-09收治的婴幼儿主动脉缩窄合并心内复杂畸形一期手术治疗的临床病例.共计18例,其中男12例,女6例.年龄平均(7.7±8.8)个月,体重(6.1±2.4)kg.主动脉缩窄包括合并主动脉弓发育不良5例,单纯主动脉缩窄13例.不合并主动脉弓发育不良者,切除缩窄段,端端吻合降主动脉;合并主动脉弓发育不良者,切除缩窄段,用自体心包或自体肺动脉片加宽主动脉弓.心内复杂畸形的矫治包括完全性大动脉转位大动脉调转术8例,右心室双出口根治术6例,右心室双出口陶西平畸形大动脉调转术3例,完全性心内膜垫缺损矫治术1例.结果:所有患儿出院前超声心动图检查均显示主动脉弓降部缩窄解除,吻合口通畅,血流正常.无中枢神经系统并发症.术后早期上、下肢收缩压基本相等者4例,上肢高于下肢15～20mmHg者8例,上肢低于下肢收缩压5～10mmHg者6例.死亡3例,死亡率16.7%.1例术后当天死于低心排综合征,1例术后9天死于肺部感染,1例术后7天死于突发恶性心律失常.术后另3例出现右侧膈肌麻痹行膈肌折叠术.结论:婴幼儿主动脉缩窄合并心内复杂畸形经胸骨正中切口一期手术治疗,主动脉弓及降主动脉上段显露良好.同期矫治心内复杂畸形手术效果满意.深低温停循环区域性脑灌注可有效降低神经系统并发症的发生.     

降主动脉病变的外科治疗

常见的降主动脉病变主要包括先天性畸形、动脉瘤、降主动脉损伤及胸腹主动脉型大动脉炎等. 1 先天性降主动脉畸形 1.1 主动脉缩窄系指主动脉局限性狭窄或闭锁,多位于主动脉峡部、左锁骨下动脉以远.缩窄处的主动脉壁是连续的,并依此区别于主动脉弓离断.根据缩窄部位与动脉导管的关系,分为导管前型(婴儿型)和导管后型(成人型).导管前型多于婴儿期死于心衰,所有患者约90%在50岁前死亡.一般认为,缩窄两端血管的压差>20mmHg(1mmHg≈0.133kPa)就有手术适应证;婴儿期心衰若可控制,可将手术年龄放宽至3～4岁,否则应尽早手术,但手术死亡率高;虽无心衰,但上肢血压≥150mmHg,也应尽早手术.手术方式:①左锁骨下动脉片主动脉成形术:适于<10岁患儿紧靠左锁骨下动脉下方的局限性缩窄.采取低温(33℃)麻醉,取左后外侧胸部切口,第4肋间入胸.游离左锁骨下动脉,结扎动脉导管,阻断左锁骨下动脉近端的及缩窄段远端的主动脉,保留足够长度,切断左锁骨下动脉,沿血管轴向连续性剖开左锁骨下动脉保留段及缩窄血管段,去除狭窄的内膜支架,将锁骨下动脉片向下翻转吻合,加宽主动脉缩窄处.②缩窄段切除对端吻合术:适于缩窄段较短者,吻合时可采用间断缝合(小儿)或连续缝合(成人).③人造血管移植术:适合大段降主动脉缩窄.切除缩窄段,行人造血管替代;若缩窄段>30cm,宜保留缩窄段,以端侧吻合方法行人造血管旁路移植术.合并心内畸形者,如有可能应I期纠正.该病婴幼儿手术早期死亡率为2%～10%,主要原因为心衰处理不当;儿童及成人早期死亡率为1%.术后10年生存率为91%,晚期死亡原因主要为持续高血压、脑动脉瘤及主动脉瓣病变等.     

一期升主动脉-降主动脉转流治疗主动脉缩窄合并心脏畸形

目的:主动脉缩窄合并其他心脏病手术的方法仍然存在争议。本研究的目的是评估经胸骨正中切口一期升主动脉-降主动脉转流+心脏畸形矫治术治疗主动脉缩窄合并其他心脏畸形的疗效。方法:选择我院于2009年4月至2017年6月,应用经胸部正中切口行升主动脉-降主动脉心包内转流术,同期行合并心脏畸形矫治手术的患者13例,女性3例,男性10例,平均年龄35岁(19～59岁)。其中3例bentall术,8例主动脉瓣置换,1例二尖瓣置换,1例二尖瓣成形术。结果:随访期间无死亡病例,主动脉阻断时间和体外循环时间分别是(81±33) min、(123±47) min。术后上肢血压明显改善(P0.001),有术前的(159±34) mmHg(1 mmHg=0.133 kPa)将至术后(122±17) mmHg,截止最后一次随访,患者上下肢血压无明显压差。结论:一期升主动脉-降主动脉转流治疗主动脉缩窄合并心脏畸形远期效果显著,可以作为主动脉缩窄合并其他心脏畸形的患者选择此手术方式。     

主动脉缩窄并发心内畸形的外科治疗5例

我科自 1 993年 1 2月～ 2 0 0 0年 1 2月对 5例主动脉缩窄并发心内畸形的患儿进行了外科手术。其中 2例为一期完成手术 ,3例为分期手术。1　临床资料例 1　女 ,8岁。干下型心室间隔缺损 (VSD)2 .0cm× 1 .5cm ,主动脉缩窄长度 3cm ,动脉导管位于缩窄段的远端 ,直径 2cm ,并发肺动脉高压 ,肺动脉收缩压 1 2 0mmHg( 1mmHg =0 .1 33kPa) ,平均压 70mmHg。手术采用自左第 4肋间进胸 ,显露和解剖左锁骨下动脉、动脉导管和缩窄段 ,其后进行胸骨正中切口 ,肝素化后 ,依次插升主动脉灌注管和降主动脉灌注管及上、下腔静脉引流管 ,进行上、下半身…     

外科手术Ⅰ期矫治主动脉缩窄合并心内畸形

目的:回顾性研究Ⅰ期手术矫治主动脉缩窄合并心内畸形病例,探讨手术适应证,评价矫治方法和疗效。方法:12例主动脉缩窄合并心内畸形接受Ⅰ期外科矫治的患者,统计不同手术方式患者手术体外循环时间、主动脉阻断时间、体外循环术后乳酸水平、呼吸机付诸实践、ICU住院时间及术后并发症情况。结果:12例患者中,5例采用经胸骨正中切口(经胸矫治组),7例采用胸骨正中腹部联合切口。经胸矫治组体外循环时间[(182.00±48.16)min︰(109.00±25.14)min,P=0.023 6]、主动脉阻断时间[(147.00±40.67)min︰(95.00±19.80)min,P=0.040 8]和体外循环术后乳酸水平[(4.10±1.03)mmol/L︰(2.10±0.96)mmol/L,P=0.0085]均高于胸腹联合组;而呼吸机辅助时间[(88.58±77.60)h︰(96.40±88.70)h,P=0.842 6]、ICU住院时间[(5.3±3.1)d︰(6.0±2.9)d,P=0.6982]两组差异无统计学意义。手术死亡经胸手术组1例,胸腹联合组2例;气管切开经胸矫治组2例,胸腹联合组2例。术后随访4~32个月,经胸矫治组狭窄部位无压差,胸腹联合组2例(28.6%)术后上肢压力仍高于下肢。结论:肺动脉高压是影响手术疗效的主要因素,根据不同的病理类型、年龄选用不同的手术方式,Ⅰ期手术矫治主动脉缩窄合并心内畸形安全、可靠。     

主动脉弓阻塞合并心脏畸形18例的一期矫治

目的总结一期矫治主动脉弓阻塞合并心脏畸形的诊治经验。方法2005年1月至2006年6月一期矫治18例主动脉弓阻塞合并心脏畸形。其中主动脉弓中断(IAA)6例,主动脉缩窄(COA)12例。手术均为正中开胸低温体外循环下一期畸形矫治术。结果手术采用降主动脉与主动脉弓端端吻合11例,端侧吻合6例,Gore-Tex片加宽弓成形1例。围术期死亡1例,总死亡率5.56%。喉返神经损伤2例,术后左主支气管受压1例。随访1～11个月,1例上下肢压差25mmHg,6例压差小于20mmHg,7例端侧吻合无压差。存活者无症状,生长发育良好。结论正中开胸一期矫治COA及IAA合并心脏畸形可获得满意的近期疗效。手术成功的关键是充分游离降主动脉,尽可能行端侧吻合术。     

先天性主动脉缩窄的介入治疗

先天性主动脉缩窄指自无名动脉至第一对肋间动脉之间的主动脉管腔狭窄,多为局限性,也可为长管状[1] 。临床上,如收缩期上下肢之间动脉压力阶差>2 0mmHg(1mmHg =0 133kPa) ,结合相应的影像学改变即可诊断为先天性主动脉缩窄[2 ] 。主动脉缩窄占先天性心血管疾病发病率的1 0 %～3 0 % ,本病可单独存在,但常合并其他心血管畸形,如室间隔缺损、动脉导管未闭等[3 ] 。长期以来,治疗主动脉缩窄以外科手术为主。术式包括:(1)缩窄部切除,主动脉对端吻合;(2 )左锁骨下动脉与缩窄后主动脉吻合;(3)缩窄部切开补片加宽手术;(4)人工血管转流术或人…     

主动脉弓缩窄或中断合并其他心内畸形的手术配合

目的探讨主动脉缩窄(CoA)或主动脉弓中断(IAA)合并其他心内畸形的一期矫治手术的手术配合。方法2013年1月至2015年8月广东省妇幼保健院手术治疗主动脉弓缩窄或中断合并其他心内畸形患儿57例,其中主动脉缩窄42例,主动脉弓中断15例。年龄57.6 d(3 d~2岁),体质量3.8(2.1~13.0)kg。患儿均经胸骨正中切口在深低温体外循环下行一期矫治手术。术前手术室做好充分评估与充足的物品、药品、设施等准备,患儿人室后配合麻醉做好各项术前准备;手术中做好台上配合、温度管理、体位摆放、皮肤管理等;手术后再次核对检查,做好交接。结果 56例患儿顺利完成手术,脱离体外循环,治愈出院,1例死亡。结论主动脉缩窄或中断合并其他心内畸形一期矫治手术,手术室护士完善的手术配合对手术成功提供了重要保障。     

扩大端侧吻合一期矫治208例小儿主动脉缩窄合并室间隔缺损的疗效

目的总结本中心近年来以扩大端侧吻合法一期矫治小儿主动脉缩窄合并室间隔缺损的手术疗效及经验。方法选取2010年1月—2017年10月在本院行一期手术治疗的主动脉缩窄合并室间隔缺损的患儿,共208例,其中男性137例,女性71例,中位年龄54(5～730)天,中位体质量4.0(1.8～9.9)kg。胸骨正中单一切口下,以扩大端侧吻合矫治主动脉缩窄,同期修补室间隔缺损。所有手术由同一组外科医师完成。应用SPSS 20.0统计软件进行数据录入和分析。结果术后早期死亡6例(2.9%),晚期死亡3例(1.4%);再缩窄16例(7.7%),再手术19例(9.1%)。脑钠肽在术前即存在明显升高,但术后未发生明显变化;主动脉缩窄段压差在术后明显下降,经皮血氧饱和度在术后明显上升。C反应蛋白、乳酸和肌酐水平在术后明显上升。术前合并高血压104例(53.0%),术后血压降至正常30例(28.8%),术后血压继续升高35例(33.7%)。术后吻合口压差较高是术后再缩窄的高危因素。结论胸骨正中单一切口下,扩大端侧吻合法一期矫治主动脉缩窄合并室间隔缺损,死亡率和再缩窄率低,取得良好的近中期疗效。术后吻合口压差较高是主动脉缩窄复发的高危因素。     

54例主动脉缩窄的外科治疗

我院 1984年 6月至 1998年 6月 ,共收治 54例主动脉缩窄病人。男性 2 7例 ,女性 2 7例 ,平均年龄 15.8岁 ( 5个月～ 39岁 )。其中婴儿型 18例 ,成人型 36例 ,合并动脉导管未闭 33例 ,室间隔缺损 2 0例 ,房间隔缺损 4例 ,主动脉瓣二瓣化畸形 4例 ,主动脉瓣下狭窄 3例 ,二尖瓣狭窄 2例 ,二尖瓣脱垂 1例。其中 39例进行狭窄处补片扩大成形术 ,5例进行狭窄处切除主动脉端端吻合术 ,3例进行了狭窄处切除人工血管移植术 ,4例进行了人工血管转流手术 ,3例用主肺动脉内隧道的方法 ,实现了升主动脉 降主动脉的连接 ,同时行室缺修补。手术死亡 1例 ( 1.85% )。远期随访死亡 3例 ( 5.6% ) ,其余效果良好     

Critical aortic coarctation: patch aortoplasty in infants less than age 3 months.

Twenty-three infants less than age 3 months (mean age 31 days) underwent patch aortoplasty for relief of coarctation of the aorta. All had intractable congestive heart failure, despite aggressive medical therapy. Each infant had other cardiac anomalies, including patent ductus arteriosus (83 percent) and ventricular septal defect (74 percent). All patients underwent closure of the ductus arteriosus and patch angioplasty of the aorta to produce a luminal diameter of at least 16 mm. In addition, 9 of the 17 patients (53 percent) with a large shunt ventricular septal defect underwent pulmonary arterial banding. There was one hospital death 42 days after operation secondary to bowel perforation and sepsis. Hospitalization beyond 21 days postoperatively was always due to other unrepaired cardiac lesions. The three late deaths at 3, 9 and 18 months after operation were associated with additional major anomalies. Fourteen patients have had postoperative catheterization. No gradient was found across the site of coarctation repair, but one patient had a gradient between the left carotid and left subclavian arteries. Surgical repair of critical coarctation of the aorta in infants can safely be offered despite the presence of other cardiac anomalies.     

Aortic coarctation surgically treated in the 1st year of life. Results in 36 cases

Thirty-six infants under one year of age underwent surgical repair of coarctation of the aorta between 1968 and 1983 in our institution. Coarctation was isolated or associated to a patent ductus arteriosus in sixteen cases, while in twenty it was associated with significant intracardiac disease. Twenty-nine patients were operated on in the first three months of life and in twenty a severe heart failure was present before the operation. Nine patients (25%) died while in the hospital: all of them were less than three months of age and all but one were affected by major intracardiac anomalies and severe heart failure. Surgical repair was by subclavian flap aortoplasty in twenty-nine cases, resection with end-to-end anastomosis in three, patch aortoplasty in three and Blalock-Park anastomosis in one. Operative mortality was unaffected by the surgical technique. The surviving children were followed-up for 30 +/- 7 months; one late death occurred suddenly, two months after the repair of a ventricular septal defect. An arm/leg pressure gradient, indicative of recoarctation, was detected in five cases: only one had been repaired by the subclavian flap technique, while the others were the only survivors of the end-to-end anastomosis and patch aortoplasty group. Subclavian flap aortoplasty is suggested as the operation of choice for coarctation of the aorta in the first year of life.     

6kg以下小婴儿主动脉缩窄合并室间隔缺损的外科矫治

目的 :探讨新生儿、小婴儿主动脉缩窄 (CoA)合并室间隔缺损 (VSD)的外科治疗方法。方法 :1998年 12月至 2 0 0 3年 7月共收治 9例 6kg以下CoA合并VSD患儿 ,其中新生儿 3例。一期手术 3例 ,分期手术 3例 ,仅做主动脉缩窄成形术 3例 ,其中 1例加做肺动脉环缩术。 6例采用人造血管补片主动脉成形术 ,3例采用左锁骨下动脉翻转主动脉成形术。结果 :全组患儿无死亡 ,随访 8～ 36个月 ,心功能改善明显 ,超声心动图检查提示无主动脉瘤样扩张或主动脉再狭窄。结论 :大多数患儿均可采用体外循环下一期根治CoA和VSD ,但是对于VSD为限制性及不能耐受体外循环的患儿可采用二期手术。一期手术选择左外侧和正中双切口方法安全有效。     

Surgical treatment of coarctation of the aorta in the first year of life. Immediate and late results in 35 patients

Between February 1974 and September 1981, 35 consecutive infants younger than 12 months underwent repair of coarctation of the aorta. The mean age was 57 days and 24 were younger than 60 days. The indications for operation were congestive heart failure, metabolic acidosis and severe systemic hypertension. Only 3 cases had isolated coarctation, whereas the remaining 32 had associated anomalies. The first 4 patients were submitted to primary repair; after this, patch aortoplasty was utilized in 14 infants and in the remaining 17 patients the aortoplasty using the left subclavian artery was performed. The hospital mortality rate was 11.4% (4 cases) and there were 2 late deaths (5.7%). Follow-up over a 7-year period shows no clinical or hemodynamic evidence of recoarctation in any of the survivors.     

Surgical management of coarctation of aorta

Between 1961 and 1988, 68 patients underwent operation for coarctation of the aorta. The average age of presentation of these subjects was higher than in other series. 7.1 percent were asymptomatic, a finding which is not seen in reports from the west. Operative procedures included resection and end to end anastomosis, resection and graft interposition, bypass grafting, patch aortoplasty and subclavian flap aortoplasty. However, the technique of patch aortoplasty, routinely performed in the last 24 years of this series seemed by far the most satisfactory procedure. Subclavian flap aortoplasty was carried out in a selected group of younger children. Associated cardiac anomalies influenced the results adversely. The overall operative mortality was 5.8 percent. Hypertension did not regress in 11.7 per cent of patients inspite of a successful operation as judged by the return of peripheral pulses in the lower limbs. Re-coarctation was not seen in this series. The overall results of operation for coarctation of the aorta have been very satisfactory and comparable with those in other published series.     

Value of dacron aortoplasty in the treatment of coarctation of the aorta during the 1st six months of life]

Between September 1st 1974 and June 1st 1976, 12 infants under the age of 6 months have been operated on by a dacron patch aortoplasty for coarctation of the aorta. The associated lesions were a patent ductus arteriosus in each case, a VSD in 8 cases, and a trans-position of the great arteries in 5 cases. A pulmonary artery banding was performed with the aortoplasty in 8 cases, and a Blalock-Hanlon operation in one instance. There were two operative deaths (17 p. 100), amongst them one in a 1400 g premature infant, and a late death (3rd month). Two infants have a clinical aspect of recoarctation. In four infants, the post-operative hemodynamic and angiographic study carried out before the treatment of associated intra-cardiac lesions, shows a good result of the coarctation repair. This particular technique, although not performed as a routine in the infant with coarctation of the aorta, seems to be of interest in the most severe forms of the disease, with diffuse isthmus narrowing and intra-cardiac defects.     

Reoperation for coarctation of the aorta

Between 1957 and 1980 reoperation for coarctation of the aorta was performed in 21 patients at one institution for an overall incidence rate of 7.9 percent. The incidence rate of reoperation was 38 percent for patients younger than age 3 years and 1.5 percent for patients 3 years or older at initial repair. Before reoperation 14 of the 21 patients were symptomatic, 19 had systolic hypertension of the upper limbs and 20 had a documented coarctation pressure gradient at rest (mean 42.4 mm Hg). Surgical techniques used at reoperation were patch aortoplasty in 12 patients, graft interposition in 4, end to end anastomosis in 3 and end to side left subclavian to descending aorta bypass graft in 2. There was one surgical death. The 20 survivors have been followed up a mean of 4.3 years. There has been significant symptomatic improvement (p < 0.001). Upper limb hypertension has also lessened significantly (p < 0.001) after reoperation;15 patients are no longer hypertensive and 3 have a lesser degree of hypertension. The coarctation pressure gradient at rest has significantly decreased (p < 0.001); 13 patients have no residual gradient and 7 have a mild gradient of 20 mm Hg or less. Graded treadmill exercise testing performed in five patients after reoperation documented upper limb hypertension in four and a marked increase in coarctation gradient with exercise in three.

In conclusion, the incidence of reoperation is significantly increased in patients who are younger than age 3 years at initial coarctation repair. Reoperation is a safe and effective procedure. It has a low mortality rate (4.8 percent), relieves symptoms and decreases hypertension and the coarctation pressure gradient. Patch aortoplasty appears to be the operative procedure of choice. Moderate to severe hemodynamic abnormalities may persist during exercise after reoperation for coarctation of the aorta.     

Congenital heart disease: diagnostic approaches, surgical indications and operative results

Our management policies of the main congenital cardiac defects which require emergency surgical intervention in neonate and infancy were reported. Total anomalous pulmonary venous drainage (TAPVD): Recent refinements in two-dimensional (2-D) echocardiography have made it possible to operate for this anomaly using only this modality. When a combination of coarctation is suspected and/or the location of the drainage of the pulmonary vein is obscure, catheterization and angiocardiography are added for detailed diagnosis. It is, however, our policy not to perform right-sided angiocardiography for patients younger than three months of age. Symptomatic cases are operated urgently, although not always on an emergency basis. In our experience, a 12 approximately 24 hour delay with intensive cardiorespiratory and metabolic care may improve the preoperative conditions considerably and increase the chances of a successful surgical repair. Coarctation of the aorta (CoA) and interruption of the aortic arch (IAA): Diagnosis of CoA by echocardiography and aortography by radial artery injection is well established. In neonates and infants with CoA or CoA + patent ductus arteriosus (PDA) and/or ventricular septal defect (VSD), emergency repair of coarctation (usually with subclavian flap aortoplasty) without pulmonary artery banding (PAB) is undertaken on the day of established diagnosis. If a combination of complex cardiac anomalies such as transposition of the great arteries (TGA) and Taussig-Bing anomaly is suspected, catheterization and angiocardiography are added for the detailed diagnosis. Repair of coarctation combined with PAB has been our choice of procedure in these infants. If IAA is suspected, catheterizaton, angiographic and detailed echocardigraphic studies are performed to define the precise anatomy of the aortic arch and associated intracardiac lesions, paying particular attention to the left ventricular outflow tract, as soon as such patients become clinically stable by intensive medical treatment using prostaglandin E1 (PGE1), digitalis and diuretics. Once the diagnosis is established surgical treatment should be carried out without delay. Initial palliation by aortic arch reconstruction with PAB followed by two-stage definitive intracardiac repair have been our choice of procedure in neonates and infants with IAA without severe left ventricular outflow tract obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Surgical experience of coarctation of the aorta in infants and young children

We evaluated the effectiveness of surgical treatment for coarctation of the aorta in infants and young children, based on our experience over 7 years. From March 1999 to December 2005, 168 cases of coarctation of the aorta in patients aged 21 days to 3 years were treated by end-to-end, extended end-to-end, or extended end-to-side ascending aorta and aortic arch anastomosis. The mortality rate was 0.6%. In 138 (82%) patients, the pressure in the lower extremity was higher than in the upper extremity. During 6-24 months of follow-up, hoarseness appeared in 6 patients. Echocardiography revealed stenosis of the end-to-end anastomosis in only 2 patients. We suggest that the best option for surgical treatment of coarctation of the aorta with associated cardiac malformations is a one-stage procedure using a median sternotomy approach. It is better to perform extended end-to-end anastomosis or anastomosis between the distal descending aorta and the left wall of the ascending aorta, or to extend this anastomosis to the transverse arch.