自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

一侧肺动脉起源于升主动脉手术治疗3例

一侧肺动脉起源于升主动脉手术治疗３例孙国成蔡振杰万明明作者单位：７１００３８西安第四军医大学唐都医院心脏外科１９９５年２月至９月间我们手术治疗右或左肺动脉起源于升主动脉３例，现报告如下：临床资料本组例１男，２．７岁；例２女，４岁；例３女，１４岁。３例...     

肺动脉窦壁"L"形切口冠状动脉移植在大动脉调转术中的应用

目的 总结肺动脉窦壁"L"形切口行冠状动脉移植在大动脉调转术中的应用体会.方法 采用在肺动脉窦壁做"L"形切口的方法行大动脉调转术(ASO)治疗心室大动脉连接异常病儿25例,其中4例为快速两期ASO治疗,男16例,女9例;年龄6d～66个月;体重2.5～15.0 kg,平均(4.7±2.9)kg.完全性大动脉转位(D-TGA)19例,其中室间隔完整型(TGA-IVS)10例,室间隔缺损(TGA-VSD)9例;右室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing,TBA)6例.术中冠状动脉移植采用在相应邻近的肺动脉窦壁做"L"形切口,形成"门板状"活瓣的方法.结果 全组手术中开放升主动脉后心脏自动复跳,未发现心肌缺血的心电图和临床表现.术后早期循环均稳定.术后早期死亡4例,均与冠脉移植无明确关系.结论 采用"L"形切口进行冠脉移植,可以相对增加冠状动脉的长度,减少游离冠状动脉范围,减小张力,同时可以减轻冠状动脉移植后的扭曲.     

ASO术中自体心包重建肺动脉根部

目的 评价自体新鲜心包片在ASO术中重建肺动脉根部的疗效.方法 2004年1月至2010年6月,共行ASO术治疗先心病儿63例,均采用裤衩状自体新鲜心包片重建肺动脉根部.术后常规随访,连续多普勒超声心动图测量收缩期肺动脉血流速,二维心脏超声测量肺动脉直径.以同龄健康儿童肺动脉流速、肺动脉直径为对照,评价疗效.结果 治愈出院57例,有效随访49例,时间3个月～6年,平均(22±4)个月.肺动脉流速与正常同龄儿对照整体流速偏快,但根据Bernoulli公式计算,压差均小于30 mm Hg.主肺动脉直径与正常同龄差异无统计学意义.肺动脉瓣关闭不全均为轻度.结论 自体新鲜心包片在ASO术中重建肺动脉根部效果良好.

Abstract：

Objective To evaluate the result of fresh autologuos pericardium for the reconstruction of new pulmonary arterial root in arterial switch operation (ASO). Methods Between January 2004 and June 2010, 63 consecutive infants with congenital heart disease were treated with ASO. A new pulmonary arterial root was reconstructed with a fresh autologuos pericardium which clipped pants-like. The followed up time was 3 months to 6 years after discharge. Patients were reexamined consecutively at 3- and 6-month; 1-, 2- and 6-year. Two-dimensional echocardiography was performed for measuring the pulmonary artery diameter. The pulmonary arterial blood speed was measured by continuous Doppler during systole. The pulmonary flow and the pulmonary artery diameter of healthy children of same age were also measure as control group. Simplified Bernoulli formula was adopted to calculate the pressure gradient through pulmonary artery anastomose for, evaluating whether it had pulmonary stenosis or not. Results Fifty seven infants were cured and discharged. Forty nine patients were finished follow up with a mean duration of( 18 ±4) months. The blood speed in the pulmonary artery anastomosis was 0.70 -2.16 m/s with a mean of (1.31 ±0.40) m/s. No pulmonary stenosis was found with the simplified Bernoulli formula. There was no significant difference regarding the pulmonary diameter and the pulmonary artery flow velocity as compared with the normal children of the same age. Conclusion The fresh autologuos pericardium is reliable for reconstruction of new pulmonary arterial root in ASO.     

经胸骨正中切口一期矫治主动脉缩窄或主动脉弓中断合并心内畸形

目的 总结经胸骨正中切口一期矫治主动脉缩窄或弓中断合并心内畸形的经验.方法 2007年1月-2008年7月手术治疗24例.包括主动脉缩窄9例,主动脉缩窄合并主动脉弓发育不良12例,主动脉弓中断3例.4例合并右室双出口(Traussig-Bing型).22例合并非限制性室间隔缺损,2例不合并室间隔缺损病儿1例合并主动脉瓣下狭窄,另l例合并肺静脉狭窄.主动脉弓降部成形均在深低温低流量持续性选择性脑灌注下进行.3例主动脉弓中断及9例主动脉缩窄病儿采用端端吻合术.12例主动脉缩窄合并主动脉弓发育不良病儿中采用扩大端端吻合术8例,端侧吻合术2例,补片成形术2例.结果 死亡2例.全组病儿围术期未出现神经系统并发症及肾功能损害.术后反复呼吸道感染2例.除l例残存压差大于20mm Hg外,最长随访18个月,尚未发现再缩窄发生.结论 主动脉缩窄或弓中断合并心内畸形一经诊断即需尽早手术.经胸骨正中切口一期矫治是安全、有效的.充分切除动脉导管组织,广泛彻底游离松解胸部各血管进行无张力吻合以及选择恰当的组织一组织吻合术式是主动脉弓降部成形手术成功及减少再缩窄发生的关键.     

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。     

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.     

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.     

婴幼儿主动脉缩窄合并心内畸形的外科治疗

目的 探讨婴幼儿主动脉缩窄合并心内畸形的外科治疗经验.方法 2000年1月至2006年12月,84例主动脉缩窄合并心内畸形患儿接受了外科手术治疗,手术年龄1个月～3岁(平均13.5个月),体重3.3～15.0 kg(平均7.3 kg).12例合并复杂心内畸形,72例合并室间隔缺损和其他简单心内畸形,23例伴有主动脉弓发育不良.一期手术62例,49例正中开胸同时矫治主动脉缩窄和心内畸形,13例左侧开胸矫治主动脉缩窄,正中开胸修补心内畸形;分期手术22例.主动脉缩窄的手术方式包括补片成形42例,切除端端吻合30例,锁骨下动脉翻转6例,血管旁路3例,球囊扩张1例.在49例正中切口一期手术中,43例应用选择性脑灌注加下半身停循环,4例应用全身低流量灌注,2例应用深低温停循环.结果 围手术期死亡8例,死亡率9.5%,其中3例为术前漏诊主动脉缩窄.结论 婴幼儿主动脉缩窄合并心内畸形的外科治疗可获得良好的近期疗效,绝大部分患儿可采取正中切口一期手术.选择性脑灌注和下半身停循环可以有效地保护脑和重要脏器.     

单纯无名动脉灌注的主动脉弓置换术21例

目的 探讨主动脉弓置换术中使用单纯无名动脉灌注进行脑保护的安全性和有效性.方法 2004年1月至2007年7月,2l例主动脉弓置换者使用单纯无名动脉灌注技术进行脑保护,男19例,女2例;年龄29-72岁,平均(46.5±11.4)岁.A型(Stanford分型)主动脉夹层19例,其中7例合并主动脉瓣关闭不全;升主动脉及主动脉弓真性动脉瘤伴降主动脉受累2例.所有病例均在深低温、使用单纯无名动脉选择性脑灌注下进行升主动脉、主动脉弓置换+降主动脉覆膜支架置入术.同时行Benlall手术6例,David手术1例.结果 升主动脉阻断(109.6.4-29.6)min;体外循环(186.7±56.2)min;最低鼻咽温(19.O±3.3)℃;选择性脑灌注时间(38.3.4-11.5)Ⅲ.m,流量每分钟(6.8±2.6)ml,l‘g.术后无中枢神经系统并发症.呼吸机辅助(38.6±29.O)h.1例因低心排输出量综合征于术后第5 d死亡.术后随访2-45个月,平均(24.0±12.5)个月,无死亡及心脑血管意外发生.结论 深低温、单纯无名动脉选择性脑灌注下行主动脉弓置换是安全、有效的.     

全弓置换和支架象鼻手术治疗A型主动脉夹层时左锁骨下动脉的处理分析

目的 总结选择性结扎左锁骨下动脉、仅重建无名动脉和左颈总动脉方法在A型主动脉夹层全弓置换和支架象鼻手术中应用的临床经验.方法 2008年1月至2010年6月,29例A型主动脉夹层患者在接受全弓置换和支架象鼻手术时,因左锁骨下动脉显露困难,术中将其直接结扎.本组男性21例,女性8例,年龄19～55岁,平均年龄(44±12)岁.其中急性夹层12例,亚急性夹层4例,慢性夹层13例.所有患者依据术前影像学和术中循环、压力指标判断患者大脑Willis环和双侧椎动脉的侧支循环情况,如侧支良好,则直接结扎左锁骨下动脉、仅重建无名动脉和左颈总动脉;如果侧支不足,则结扎后加行升主动脉-左腋动脉旁路术.结果 29例手术均顺利完成,1例术后死于肺部感染,其余恢复顺利.术后左上肢血压(78±17)mmHg(1 mmHg=0.133 kPa),明显低于右上肢的(126±24)mmHg(P＜0.01),但左侧指氧饱和度、皮温、肌力及感觉运动功能与右侧相比无明显差异.随访1～27个月,无左锁骨下动脉盗血综合征与左上肢肌萎缩发生.结论 在对A型主动脉夹层行全弓置换和支架象鼻手术时,如果动脉瘤体较大、左锁骨下动脉位置较深、显露困难时,可以在充分评估侧支循环的前提下直接予以结扎,可简化手术操作和手术难度,术后无明显不良后果.

Abstract：

Objective To summarize the experiences of ligating left subclavian artery(LSA)in total arch replacement and stented elephant trunk implantation for Stanford type A aortic dissection patients with difficulty in exposing the LSA. MethodsTotal arch replacement and stented elephant trunk implantation were performed on 79 consecutive patients from January 2008 to June 2010. Twenty-nine cases of the cohort undertook LSA ligation due to bad exposure. There were 21 males and 8 females patients, aged from 19 to 55 years with a mean of(44 ± 12)years. There were 12 acute dissections, 4 sub-acute dissections and 13 chronic dissections. Based on thoroughly evaluation of the Willis' circle and bilateral vertebral arteries through pre-operative imaging and inrto-operative circulative parameters, if the collateral circulation was considered sufficient, LSA was ligated directly and only the innominate artery and carotid artery were reconstructed; if considered insufficient, an additional bypass from ascending aorta to left axillary artery was performed. Results All the 29 operations were completed successfully. There was one patient died from pulmonary infection and the others recovered well. Blood pressure of left arms were lower than right postoperatively[(78 ± 17)mmHg vs.(126 ± 24)mmHg, 1 mmHg = 0. 133 kPa, P ＜ 0. 01], but oxygen saturation, skin temperature and strength of the left hand were normal compared to the right. All the survived patients have been followed 1-27 months and none of them presented with any symptoms of left subclavian artery steal syndrome and ischemia of left arms. Conclusions Ligation of LSA under strict evaluation of collateral circulation could be safe in Type A dissection patients with bad exposure due to big ascending aortic aneurysm and will simplify the procedure significantly.     

Taussig-Bing畸形合并主动脉弓病变一期外科治疗

目的总结一期手术治疗Taussig-Bing畸形合并主动脉弓病变的经验。方法2000年5月至2006年12月手术治疗12例Taussig-Bing畸形合并主动脉弓病变病儿，其中主动脉弓中断5例。采用大动脉调转（arterial switch operation，ASO）的手术方法矫治Taussig-Bing畸形，将胸主动脉与主动脉弓下缘端端吻合或与升主动脉端侧吻合矫治主动脉弓病变。结果住院死亡3例（25％），使用呼吸机平均（7．4±2．1）d，ICU滞留平均（11．7±4．6）d。生存病儿无因残余解剖问题再次手术者。结论Taussig-Bing畸形合并主动脉弓病变病理解剖比较复杂，手术方式的选择、手术技巧和合并主动脉弓病变对Taussig-Bing畸形的治疗效果有影响。     

经胸骨正中切口一期矫治小儿主动脉缩窄及合并畸形

目的探讨经胸骨正中切口一期矫治小儿主动脉缩窄(CoA)及合并畸形的手术方法和治疗效果。方法2002年6月至2005年8月手术治疗CoA及合并畸形36例,其中男27例,女9例;年龄18d~8岁,平均(14·3±9·5)个月,其中小于1岁27例,小于3月龄18例;体重3~23kg,平均(6·6±3·0)kg。有症状的婴儿型27例,无症状的大儿童型8例,经左胸切口矫治CoA后再狭窄1例;合并室间隔缺损、动脉导管未闭、房间隔缺损、主动脉弓发育不良、完全性大动脉转位及主动脉瓣下狭窄等心内畸形。36例均经胸骨正中切口以扩展端端吻合术一期矫治CoA及合并畸形。结果手术死亡1例,系术前反复心力衰竭及充血性肺炎,术后15d死于心肺功能衰竭。35例随访4~36个月,晚期死亡1例。生存34例恢复良好,无再缩窄发生。结论经胸骨正中切口一期矫治CoA及合并畸形效果良好;扩展端端吻合法是一种值得推荐的技术。     

婴幼儿主动脉缩窄的外科治疗

目的评价近年来婴幼儿主动脉缩窄（CoA）手术疗效。方法外科治疗118例婴幼儿主动脉缩窄病例。年龄21d～3岁,平均（1．8±1．1）岁;体重2．9～13．5 kg,平均（7．5±2．2）kg。单纯CoA或伴动脉导管未闭（PDA）32例,合并其他心内畸形86例,后者大部分在正中切口深低温停循环（DHCA）或深低温低流量（DHLF）下一期纠治CoA和合并畸形。术中停循环21～48min,平均（32．13±11．72）min。结果因肺高压危象和心律失常死亡2例,病死率1．7％。呼吸机应用11～256 h,平均（98．51±6．68）h。术后随访6～24个月,无神经系统并发症,6例有声音嘶哑,其中5例经正中胸骨切口、1例外侧切口进胸施术。超声检查示2例有残余主动脉缩窄,压力阶差分别为29和36min Hg（1mmHg=0．133 kPa）。结论CoA无论是否合并心内畸形,均主张早期手术,正中切口一期根治术手越来越成为首选方案;主动脉远端与主动脉弓下缘广泛端端吻合术（EEEA）等手术方法的应用扩大了手术根治的指征,也提高了术后疗效。     

肺动脉右心室直接吻合纠治复杂先天性心脏病

目的 探讨肺动脉右心室直接吻合纠治复杂先天性心脏病的效果。方法 2001年3月至2004年6月，对17例复杂先天性心脏病病儿采用肺动脉右心室直接吻合方法予以纠治，其中3周岁以下者11例。肺动脉闭锁合并室间隔缺损6例，完全性大动脉错位合并室间隔缺损和肺动脉狭窄4例，永存动脉干4例，法洛四联症合并冠状动脉横跨右心室流出道3例。12例一期根治，5例分期纠治。结果 手术死亡1例。5例分期手术中的3例已成功二期根治。随访1～40个月，所有病儿生长发育良好，心功能0～Ⅰ级。结论 肺动脉右心室直接吻合应用于纠治某些复杂先天性心脏病可取得满意效果。对无同种带瓣管道可选用的婴幼儿病例，此术式尤为适用。     

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.