“杂交”手术治疗20例小婴儿主动脉缩窄合并室间隔缺损

目的 评价“杂交”手术治疗6月龄内小婴儿主动脉缩窄合并室间隔缺损的早期治疗结果.方法 2010年1月至2011年7月,20例小婴儿主动脉缩窄合并室间隔缺损患儿,手术年龄(56±45)天,体重(4.5±1.6)kg.主动脉缩窄压差30 ～56 mm Hg(1 mm Hg =0.133 kPa),其中5例伴主动脉弓发育不良;室间隔缺损8 ～ 16 mm.均行“杂交”手术治疗.结果 全组无手术及围术期死亡.围术期并发症4例(20％).术中造影发现主动脉弓缩窄最严重处直径为1.5 ～ 3.4 mm,选择最大球囊直径4～12 mm,扩张后即刻主动脉缩窄处压差为0～ 27 mm Hg.全组体外循环40～ 87 min,主动脉阻断20 ～ 41min,无延迟关胸.术后患儿机械通气(2.2±1.4)天,住监护室(5±3)天.患儿均随访(10.0±3.6)个月,主动脉弓缩窄扩张处末见有动脉瘤发生,无气道受压.末次随访2例患儿主动脉弓残余梗阻,压差12 ～ 35 mm Hg,再次手术干预.结论 主动脉弓缩窄球囊扩张联合体外循环下室间隔缺损修补手术治疗小婴儿主动脉缩窄合并室间隔缺损可以避免深低温停循环,具有较高的安全性,早期疗效满意.     

主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治

目的 总结主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治经验.方法 2003年2月至2007年8月,一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压病婴6例,男、女各3例.手术年龄18 d～14个月,平均(8.3±4.5)个月;平均体重(8.5 ±2.2)kg.A型4例,B型2例.所有病例术前均有反复呼吸道感染史.手术均采用胸骨正中切口.结果 全组病例均生存.随访3～36个月,恢复良好.结论 一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压效果良好,需同时加强对围术期肺动脉高压的管理.     

婴幼儿主动脉弓中断合并心内畸形胸骨正中切口一期手术治疗

目的 探讨婴幼儿主动脉弓中断(IAA)合并心内畸形胸骨正中切口一期矫治手术效果以及影响因素.方法 2005年8月至2012年1月,采用胸骨正中切口体外循环(CPB)下一期纠治IAA合并心内畸形患婴23例,其中男12例,女11例;年龄18天～3岁,体质量(6.61 ±3.26) kg.按Rastelli 分型,A型20例,B型3例.患儿均合并粗大动脉导管未闭及大型室间隔缺损,其他合并畸形包括主肺动脉窗、右肺动脉起源于主动脉、主动脉瓣两瓣畸形和主动脉瓣下狭窄等.患儿术前均行超声心动图检查,14例行64排CT检查,4例行造影检查.22例术前明确诊断,1例术中明确诊断.行主动脉直接吻合3例,补片扩大主动脉19例,人工管道经肺动脉内连接主动脉弓1例;同期矫治合并心内畸形.结果 全组体外循环(129.76±38.98) min,主动脉阻断(74.47±24.30)min,住ICU院平均96 h.术后2例死亡,其中1例2月龄患婴死于肺动脉高压危象,1例7月龄患婴术中出血、术后死于严重低心排血量综合征.21例患儿生存,随访2个月～6年,超声检查示患儿术后心功能良好;5例术后6个月上、下肢平均压差＞30 mm Hg(1 mm Hg =0.133 kPa).无因再狭窄再次手术患儿.无神经系统并发症发生.结论 IAA采用胸骨正中一期外科治疗简化了手术过程,减少了手术死亡,提高了患儿的生活质量,早、中期预后良好,是一种值得推广的治疗方法.     

自体肺动脉在先天性心脏病主动脉弓降部重建手术中的应用

目的 总结应用自体肺动脉在先天性心脏病主动脉弓降部重建手术中应用的初步经验.方法 2010年7月至2012年12月,13例患儿在主动脉弓降部重建手术中应用自体肺动脉血管片修补.男7例,女6例;年龄1个月～16岁,中位值8个月;体质量3.5 ～52.0kg,中位值12.6 kg.主动脉弓离断合并室间隔缺损,动脉导管未闭4例(年龄分别为5个月、2岁、5岁、16岁),室间隔缺损合并主动脉缩窄,主动脉弓发育不良9例.全组手术为正中切口Ⅰ期矫治手术,主动脉弓离断患儿采用双动脉插管灌注,其余患儿采用升主动脉插管,上、下腔静脉插管建立体外循环,9例患儿采用深低温停循环,4例采用深低温低流量选择性脑灌注行弓降部重建;降温至深低温(18℃)过程中完成心内畸形的修补.4例主动脉弓离断的患儿利用预先剪下的自体肺动脉血管缝制血管管道,直径10～20 mm,根据患儿体表面积与体质量制定,两端分别连接降主动脉和弓部.9例室间隔缺损合并主动脉缩窄和主动脉弓发育不良者,先切除狭窄段,然后建立主动脉弓降部的部分连接,再利用修剪成椭圆形的自体肺动脉血管片扩大主动脉弓底部,完成重建弓降部.自体心包片修补主肺动脉缺损处.结果 术后1例死于多器官功能衰竭.余12例术后均完全清醒,无神经系统并发症,上、下肢血压无明显压差,无左主支气管压迫.随访12 ～24个月,中位时间13个月,上、下肢血压无明显压差;全部患儿术后3、6、12个月复查超声心动图,均提示弓降部血流通畅,无明显压差.结论 自体肺动脉血管可以应用于先心病主动脉弓离断或长段主动脉弓缩窄合并室间隔缺损的主动脉弓降部重建.术后早期及中期随访结果良好,自体肺动脉管道有潜在的生长能力,远期结果仍需更长时间的随访.     

一期手术矫治先天性主动脉弓中断

目的　探讨先天性主动脉弓中断 (IAA)一期手术矫治的手术方法、疗效 ,总结其临床经验。　方法　对10例少见的先天性 IAA进行一期手术矫治 ,平均手术年龄 2 .7± 2 .4岁 ,其中 5例为 A型 IAA,3例为 B型 ,另 2例IAA合并残存第 5弓狭窄 ;8例患者均合并其它心血管畸形和重度肺动脉高压。一期矫治术中有 7例进行了主动脉弓直接端侧或端端吻合连接术 ,2例行 Gore- Tex管道连接重建主动脉弓 ,1例 IAA合并残存第 5弓狭窄用自身心包补片作狭窄处扩大成形术 ;8例患者于矫治 IAA的同时矫治心血管其他畸形。　结果　术后早期发生心功能不全、心律失常、肺动脉高压危象等并发症 5例 ,其中近 10年仅发生 1例。住院死亡 3例 ,近 10年连续 6例无住院死亡。术后早期 5例肺动脉收缩压 /体循环动脉收缩压 (Pp/ Ps)由术前的 0 .84± 0 .0 4显著下降至正常范围 (0 .2 8± 0 .0 3) ,1例主动脉弓部压力阶差为 30 mm Hg(1k Pa=7.5 mm Hg)。随访 7例 ,平均随访 2 .6± 4 .0年 ,均存活 ,其中有 3例主动脉弓部压力阶差≥ 30 mm Hg。心功能均正常。　结论　先天性 IAA一旦诊断明确 ,应尽早进行一期矫治术 ;主动脉弓直接吻合连接术效果较佳。     

肺动脉融合术治疗肺动脉闭锁合并大主肺侧支动脉

目的 总结肺动脉融合术治疗肺动脉闭锁、室间隔缺损合并大主肺侧支动脉的初步经验.方法 1999年12月至2007年6月完成肺动脉闭锁,室间隔缺损合并大主肺侧支动脉(PA/VSD/NAPCAs)的肺动脉融合术17例,其中男7例,女10例;年龄0.8～18岁,平均(6.0±5.6)岁;体重6.5～55.0 kg,平均(20.0±14.9)kg.术前固有肺动脉指数(PAJ)为42.0～366.1mm2/m2,平均(133.7±87.8)mm2/m2.手术入路包括正中剖胸10例,正中+左后外侧剖胸5例,正中+右后外侧剖胸2例.一期肺动脉融合及心内畸形矫治术12例,肺动脉融合及姑息性体肺分流术4例,肺动脉融合及姑息性右室流出道扩大1例.全组病儿术前造影发现大主肺侧支动脉共44支;术中融合29支,结扎5支.肺动脉闭锁矫治术及肺动脉融合术前介入栓堵3个侧支1例;肺动脉融合及体肺分流术后介入栓堵2个残留侧支1例.结果 全组术中转流174.3min,主动脉阻断88.7min.术后呼吸机辅助7.4d,术后ICU 11.8d.生存11例,平均新建肺动脉指数(TNPAI)249mm2/m2.6例死亡,其中2例TNPAI<200mm2/m2,死于肺血管发育不良,术后发生低心排出量综合征(低心排)和突发室性心律失常;1例因低心排无法脱离体外循环,尸检发现肺小动脉明显肌型化,管腔高度狭窄,证实术前局部肺段已有严重的肺高压;3例>450 mm2/m2分别死于严重肺部感染、渗出、急性肾功能衰竭,反复气道大量出血和顽固性室性心律失常.其他主要并发症为大脑皮层盲、膈肌麻痹和阴沟杆菌性肺炎肺出血各1例.结论 完全矫治+肺动脉融合是治疗肺动脉闭锁/室间隔缺损合并大主肺侧支动脉最根本的治疗手段,但手术操作复杂.由于同一病儿肺动脉病变的多样性,常见MAPCAs高压及狭窄并存.适应证选择和手术技巧的提高仍需不断的探索.     

肺移植治疗儿童肺动脉高压的临床疗效

目的 探讨同种异体肺移植治疗儿童肺动脉高压(PAH)的临床疗效.方法 受者例1、2为特发性肺动脉高压( IPAH)患儿,均经右心导管术确诊为IPAH,术前心功能不全Ⅳ级,肺动脉收缩压、平均压分别为110、70 mm Hg(1 mm Hg =0.133 kPa)和148、72 mm Hg,在全麻体外膜肺氧合(ECMO)支持下行序贯式双侧单肺移植术,术中ECMO支持时间分别为550 min和450 min,出血量分别为3000 ml和1200 ml.受者例3为先天性心脏病室间隔缺损合并艾森曼格综合征心内直视探查术后,超声心动图(UCG)提示先天性心脏病室间隔缺损,双向分流,肺动脉收缩压、平均压为110、60 mm Hg,在全麻低温体外循环(CPB)下行室间隔缺损修补术同期右侧单肺移植术,术中CPB时间244 min.3例受者与供者体型较匹配,ABO血型相同.结果 受者例1、2术后ECMO分别维持16h、13 h后撤离,术后第3、4天均出现不同程度的血流动力学不稳定,诊断为急性左心衰,均于术后第3、6天行气管切开呼吸机辅助正压通气,经强心、利尿、扩血管等治疗,分别于术后第33天、12天脱离呼吸机.受者例3术后3天内移植肺出现中等程度再植反应性肺水肿,术后第7天气管切开,第12天撤离呼吸机;术后第14天出现急性排斥1次,治疗后缓解.3例受者术后UCG提示心脏形态和心功能明显改善,受者例3室间隔缺损修补完整,无残余分流.分别于术后第93天、32天、62天康复出院,心功能均达Ⅰ级,肺动脉收缩压、平均压分别降为54、32 mm Hg,60、36 mm Hg和53、39 mm Hg.术后已随访41、21、82个月,患儿正常工作学习,至今生活质量良好.结论 对终末期经内科保守治疗效果欠佳的PAH患儿行肺移植能很好改善生活质量.     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

目的 分析和评估纠正型大血管错位伴肺动脉狭窄病儿采用双调转(Double-Switch)手术的治疗效果.方法 2001年8月至2008年12月采用Double-Switch手术行纠正型大血管错位伴肺动脉狭窄21例.其中男11例,女10例;年龄3.5个月至6.3岁,平均(31±18)个月;体重6～28 kg,平均(13.1±6.5)kg.室间隔缺损(VSD)为膜周型15例,远离大动脉1例,肺动脉下5例.轻度二尖瓣反流2例,中度反流2例.采用Senning+Rastelli手术方法 .13例右心室与肺总动脉的连接采用牛颈静脉管道.结果 全组均生存.体外循环转流156～287 min,平均(202.0±35.8)min;主动脉阻断93～161min,平均(138.0±19.8)min.均无完全性房室传导阻滞.随防2～5年,上腔静脉流速增快3例(1.8～2.2)m/s,其中1例术后2.6年再次手术解除上腔静脉梗阻,余2例在随防中;肺静脉回流均无明显梗阻;牛颈静脉管道通畅,解剖右室流出道连接处梗阻2例,压力阶差在30～45 mm Hg(1 mm Hg=0.133kPa),尚在随防中.结论 Double-Switch手术纠治纠正型大血管错位伴肺动脉狭窄的复杂型先心病,取得了较好效果,但仍需进一步随访,以观察这类复杂手术方法 的长期疗效.     

先天性双主动脉弓畸形的外科治疗

目的 总结双主动脉弓畸形外科治疗经验.方法 外科治疗8例双主动脉弓病儿中男5例,女3例;年龄1～48个月,平均(14.93±15.52)个月;体重5.5～15 kg,平均(9.56±3.51) kg.左右弓均衡型1例,右弓优势型7例.6例为单纯双主动脉弓畸形,均在胸部左后外侧切口进行矫治.另外1例合并室间隔缺损,肺动脉高压,1例合并肺动脉吊带,均在正中切口体外循环下进行矫治.结果 7例术后积极治疗,均治愈出院.1例合并肺动脉吊带病儿因气管狭窄段长,呼吸困难严重,尽管血管畸形矫治满意,但仍不能脱离呼吸机,家属放弃治疗,自动出院.结论 双主动脉弓是一种引起小儿严重呼吸系统感染的严重疾病,早发现,早治疗,手术治疗效果良好.     

肺动脉内膜剥脱术治疗慢性栓塞性肺动脉高压——UCSD32例手术经验

目的 分析肺动脉内膜剥脱术(PEA)治疗慢性栓塞性肺动脉高压的围手术期资料,探讨美国加州大学圣迭戈分校(UCSD)手术经验.方法 回顾性研究UCSD 32例肺动脉血栓内膜剥脱手术资料,其中男17例,女15例;平均年龄(47.56±16.04)岁,平均病程(3.90±4.61)年;15例有深静脉血栓病史.采用全麻、胸骨正中切口、深低温、间断停循环双侧肺动脉内膜剥脱的手术方法.结果 根据术中病理标本Jamieson分型,Ⅰ型占21.8%,Ⅱ型占28.1%,Ⅲ型占37.5%.平均转机(236.32±37.27)min,主动脉阻断(111.69±28.14)min,停循环(38.00±13.58)min.术后机械通气(66.23±99.24)h,住ICU(4.62±4.50)天,无死亡.病人肺动脉收缩压由术前(81.03±16.92)mm Hg(1 mm Hg=0.133 kPa)降至术后(51.20±12.16)mm Hg,肺血管阻力由术前(88.91±42.32)kPa·s·L-1降至术后的(34.38±15.68)kPa·s·L-1,心排量由术前(3.65±1.08)L/min增加到术后(5.85±1.21)L/min,中心静脉压由(13.07±2.11)cmH2O(1 cmH2O=0.098 kPa)降至(9.86±3.02)cmH2O.术后短期随访显示,病人心功能(NYHA)恢复到Ⅰ级19例、Ⅱ级13例,生活质量明显改善.结论 PEA是治疗慢性栓塞性肺动脉高压的重要手段,手术成功率逐年提高;深低温、间断停循环、双侧肺动脉内膜剥脱及内膜外翻技术为PEA标准术式.多中心资料证实该术式可以有效降低肺动脉压和肺血管阻力,明显改善血流动力学指标和心肺功能.多数国内医疗中心没有足够手术经验,应尽量避免选择肺动脉压收缩≥100mmHg,肺血管阻力≥100kPa·s·L-1及Ⅲ型病变者行PEA手术.     

AP window and anomalous origin of right coronary artery from the window.

Aortopulmonary window (APW) is a rare malformation. We recently operated on a child with APW, ventricular septal defect, right aortic arch, and anomalous right coronary artery from the APW. This patient also had a chromosomal abnormality. He underwent the repair of this complex lesion in a staged operation.     

Aortopulmonary window

Aortopulmonary window is a rare defect caused by failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. Aortopulmonary window may occur as an isolated lesion or it can be associated with other cardiac abnormalities in one third to one half of cases. The most common associated lesions are arch abnormalities, specifically interrupted aortic arch and coarctation of the aorta. Antenatal diagnosis is rare. In the current era, early mortality following repair of simple aortopulmonary window approaches zero and depends on the presence of associated lesions, especially interrupted aortic arch. Long-term outcome should be excellent. Early morbidity includes pulmonary artery stenosis and residual aortopulmonary septal defects. Long-term follow-up is indicated to look for recurrent lesions such as the development of branch pulmonary artery stenosis and arch obstruction.     

Surgical management of aortopulmonary window.

OBJECTIVE: Aortopulmonary window is a rare congenital malformation involving a window-like communication between the ascending aorta and the pulmonary artery. Here, we present our experience regarding the surgical repair of an aortopulmonary window, and also assess the long-term outcome. METHODS: Thirteen children with an aortopulmonary window associated with various congenital lesions underwent a repair of the defect. The age at operation ranged from 3 days to 1 year (median age, 19 days). The patient's weight ranged from 2.1 to 7.0 kg (mean weight, 3.6 kg). The associated lesions included an interrupted aortic arch (5 patients), a ventricular septal defect (2), an atrial septal defect (1), mitral valve regurgitation (1), and tricuspid atresia [Ic] with mitral valve regurgitation (1). The aortopulmonary window was repaired with a cardiopulmonary bypass in 11 patients, and 2 patients were ligated without a cardiopulmonary bypass. RESULTS: One patient associated with tricuspid atresia died (mortality rate of 7.7%). There has been no late death during a mean follow-up of 7 years and 3 months. CONCLUSIONS: The surgical results for an aortopulmonary window are encouraging, even if such patients are associated with major cardiac anomalies and an interrupted aortic arch. Most have shown a good long-term outcome.     

Staged repair of interrupted aortic arch with ventricular septal defect compared with primary repair in infancy

Interrupted aortic arch is a poor prognosis cardiac anomaly with nearly 100 percent mortality if not recognized and treated early. The associated intracardiac lesions often lead to death if only the arch defect is repaired. Several recent reports have described patients with interrupted aortic arch who were treated as infants by primary repair of the arch defect with simultaneous repair of the intracardiac lesions. The improved survival data from these series have been attributed to the simultaneous repair of both lesions. We report herein on nine patients with both interrupted aortic arch and ventricular septal defect seen at Children's Hospital and Medical Center in Seattle from 1979 to 1987. Three patients had partial expression of DiGeorge's syndrome. All patients underwent primary repair of the interrupted aortic arch with concomitant pulmonary artery banding during infancy (mean age 18 days, range 2 days to 4 months). Operative mortality was 11 percent (1 of 9 patients). Eight patients had eventual repair of the ventricular septal defect (mean age 18 months, range 6 to 29 months) with one death occurring at 5 months postoperatively (12 percent mortality). The overall mortality of these nine patients was 22 percent. Staged repair of interrupted aortic arch with associated ventricular septal defect can be performed with results comparable to simultaneous primary repair in infancy. The improved survival from either approach is more likely to be attributable to improved perioperative stabilization, particularly the use of prostaglandin E.     

Two cases of Konno's operation for aortic stenosis developing after repair of interruption of the aortic arch with ventricular septal defect

Two patients, 8-year-old and 7-year-old boys, underwent Konno's operation for left ventricular outflow tract obstruction, which had developed after the repair of interrupted aortic arch and ventricular septal defect. Both cases had bicuspid aortic valve and severe subaortic stenosis. The annular diameters were 15 mm and 16 mm, and pressure gradients between the left ventricle and the ascending aorta were 110 mmHg and 105 mmHg, respectively. A SJM prosthetic valve (23 mm, 25 mm) was used. Postoperative courses were good. Left ventricular outflow anomalies are frequently found in cases of interrupted aortic arch complicated with ventricular septal defect. The stenosis not infrequently develops and becomes apparent several years after primary or staged repair for interrupted arch with VSD as in the present cases. Therefore, careful observation should be carried out following the repair. Konno's operation can be safely performed for this lesion with low mortality and morbidity for valvular and subvalvular aortic stenosis even with small aortic ring.     

Surgical management of aortopulmonary window: a 40-year experience.

OBJECTIVES: An aortopulmonary window (APW) is a communication between the pulmonary artery (PA) and the ascending aorta in the presence of two separate semilunar valves. The purpose of this review is to describe the evolution of surgical techniques and results of surgical correction of APW at a single center over a 40-year time period. METHODS: Between 1961 and 2001, 22 patients underwent repair of APW. Age ranged from 11 days to 13 years (median 0.3 years). Associated cardiac lesions included interrupted aortic arch (IAA) (four), right PA origin from the aorta (four), ventricular septal defect (three), atrial septal defect (one), tetralogy of Fallot (one), and transposition of the great arteries (one). Mean preoperative pulmonary vascular resistance was 5.4 U/m2 (n=17). Two patients had attempted ligation without cardiopulmonary bypass (CPB), one patient had division and oversewing of the APW between clamps on CPB. Ten patients had the APW divided on CPB with primary aortic closure. Three patients had circulatory arrest for APW division, IAA repair, and anastomosis right PA to main PA. Most recently, six patients have had open transaortic patch closure (one of these had simultaneous arterial switch, one had simultaneous IAA repair). Follow-up in operative survivors ranges from 1 month to 26 years (median 8 years). RESULTS: There were five early deaths and one late death (pulmonary hypertension) in the first 16 patients where the primary strategy was APW division (37% mortality). There have been no deaths in the most recent six patients having transaortic patch closure. The patients with transaortic patch closure at a maximum of 8 years follow-up are demonstrating normal PA and aortic growth. CONCLUSIONS: Early correction of APW with a transaortic patch and repair of all other associated cardiac anomalies at the time of diagnosis is advised.     

Surgical management of tunnel-like subaortic stenosis via ventricular septal defect in a patient with the interrupted aortic arch

A 24-day-old male with interrupted aortic arch (type B), ventricular septal defect, and tunnel-like subaortic stenosis underwent a one-stage surgical treatment The operative procedure comprised reconstruction of the aortic arch, transatrial excision of the subaortic fibromuscular tissue via the ventricular sepatal defect, and patch closure of the defect. The patient tolerated the procedure well and the postoperative echocardiography demonstrated a residual pressure gradient across the left ventricular outflow tract of 20 mmHg. Our result suggests that the transatrial surgical management of subaortic stenosis via the ventricular sepatal defect produces a safe and promising surgical option.     

主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.     

主动脉缩窄或主动脉弓中断合并心内畸形一期修复

目的 探讨采用经胸骨正中切口一期修复主动脉缩窄(CoA)或主动脉弓中断(IAA)合并心内畸形的治疗效果.方法 2002年7月至2009年6月,经胸骨正中切口行降主动脉远端和主动脉弓下缘端侧吻合术一期修复CoA或IAA合并心内畸形病儿43例,其中CoA 34例,IAA 9例(A型6例、B型3例),合并心内畸形包括室间隔缺损42例、动脉导管未闭34例、房间隔缺损12例、主动脉瓣下隔膜狭窄5例、二尖瓣关闭不全2例,右心室双出口1例.结果 手术死亡1例,为术后肺动脉高压和严重低心排血量综合征者.术后并发症包括严重低心排血量综合征3例,低氧血症6例,肺部炎症11例,肺不张14例,声音嘶哑19例,室上性心动过速23例.8例失访.34例随访3个月～5年,生活质量明显改善,心脏超声心动图和CT检查显示吻合口无明显再缩窄发生.结论 经胸骨正中切口,采用主动脉远端和主动脉弓下缘端侧吻合技术一期修复CoA或IAA合并心内畸形的手术早、中期效果良好,能明显减少术后再狭窄.