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。     

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.     

端-侧吻合主动脉重建技术在婴儿主动脉缩窄合并心内畸形一期矫治术中的应用

目的比较婴儿主动脉缩窄(CoA)合并心内畸形一期矫治手术中采用端-侧吻合技术与扩大的端-端吻合技术的临床效果。方法 2008年1月至2011年7月广州市妇女儿童医疗中心共63例婴儿CoA合并心内畸形行一期矫治手术,按主动脉重建时的技术方法不同将63例患者分为两组,端-端吻合组:24例,男17例,女7例;年龄(4.6±2.9)个月,应用扩大的端-端吻合技术行手术治疗;端-侧吻合组:39例,男24例,女15例;年龄(3.4±2.6)个月,应用端-侧吻合技术行手术治疗。比较两组患者术后早期上、下肢动脉收缩压压差和围术期相关并发症发生情况。结果端-侧吻合组新生儿比率(23.1%vs.4.2%;χ2=3.979,P=0.045)、术前24 h内酸中毒比率(15.4%vs.0%;χ2=4.080,P=0.048)均高于端-端吻合组。术后端-端吻合组无死亡,端-侧吻合组死亡1例(2.6%)。端-侧吻合组术中停循环时间明显短于端-端吻合组[(18.6±2.7)min vs.(23.4±3.7)min,F=14.617,P=0.000]。端-端吻合组入心脏监护室(CICU)时上、下肢动脉收缩压压差<5 mm Hg、5～15 mm Hg、>15 mm Hg患者分别占20.8%、45.8%和33.3%,端-侧吻合组分别占97.4%、2.6%和0%,两组间差异有统计学意义(χ2=40.380,P=0.000)。术后24 h端-端吻合组上、下肢动脉收缩压压差<5 mm Hg、5～15 mm Hg、>15 mm Hg患者分别占45.8%、41.7%和12.5%,而端-侧吻合组占100%、0%和0%,两组间差异有统计学意义(χ2=26.620,P=0.000)。随访62例,随访时间2～36个月,所有患者均无血管瘤形成,无需再次手术处理的主动脉再狭窄。结论在婴儿CoA合并心内畸形的一期矫治手术中,与扩大的端-端吻合技术相比较,应用端-侧吻合技术可以安全、有效地显著降低术后早期出现的残余梗阻。     

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.     

主动脉缩窄35例的外科治疗

外科治疗主动脉缩窄３５例。年龄６～４２岁，平均２２．７岁。单纯主动脉缩窄３１例，合并ＰＤＡ４例。其中切除缩窄主动脉、端端吻合２０例，涤纶片血管成形术８例，应用人工血管移植术７例。手术死亡率５．７％。我们认为主动脉缩窄段切除端端吻合术的优点为消除动脉导管组织，保留锁骨下动脉和避免应用人工材料。且血管成形术简单安全。     

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.     

升主动脉-腹主动脉转流术治疗成人主动脉缩窄

目的 探讨升主动脉-腹主动脉人工血管转流术治疗成人主动脉缩窄的手术效果、随访结果并总结其临床经验.方法 2008年5月至2009年7月,应用升主动脉-腹主动脉人工血管转流术治疗成人主动脉缩窄9例,其中男4例,女5例;平均年龄42.6岁.所有病人均经桡动脉、足背动脉穿刺测压,根据术前、术后,桡动脉、足背动脉平均压差变化评价手术效果.结果 术后均治愈出院.术前桡动脉足背动脉平均压差36～63 mm Hg(1 mm Hg=0.133 kPa);术后24 h桡动脉足背动脉平均压差0～13 mm Hg,较术前明显缩小.随访1～13个月,术后上、下肢动脉平均压差均小于20 mm Hg,转流人工血管通畅,2例主动脉缩窄远端自体动脉部分闭塞.结论 升主动脉-腹主动脉人工血管转流术是治疗成人主动脉缩窄的有效手段.

Abstract：

Objective To explore the surgical effects and follow-up results in treating adult aortic coarctation patients using ascending aorta-abdominal aorta vascular prosthesis bypass and summarize the clinical experiences. Methods From May 2008 to July 2009, ascending aorta-abdominal aorta vascular prosthesis bypass surgery was performed in nine patients with adult aortic coarctation, among which, four were male, and five were female, with the average age of 42.6 years old. All patients had upper extremity hypertension, the systolic blood pressure difference between their upper extremities and lower extremities was 55 - 100 mm Hg, mean (70.2 ± 15. 6) mm Hg. Among which, seven cases showed descending aorta aneurysmal dilatation at coarctation segment distal end, with the wall thinning; two cases showed long segment stenosis; three cases showed aortic wall near coarctation segment was calcified. All cases belonged to complex aortic coarctation. All patients underwent radial artery and dorsalis pedis artery puncture manometry, the surgical effects were evaluated according to mean pressure difference changes between radial artery and dorsalis pedis artery before and after operations. Results All patients were cured and dispertension has been significantly improved, before operation, the mean pressure difference between radial artery and dorsalis pedis artery was 36 - 63 mm Hg, mean [(48.2 ± 5.6 ) mm Hg]; 24 hours after operation, the mean pressure difference between radial artery and dorsalis pedis artery was 0 - 13 mm Hg, mean [(6.2 ± 1.6) mm Hg], significantly reduced ( P ＜per extremity hypertension disappeared, no need for oral antihypertensive drugs, the mean pressure differences between upper extremities and lower extremities after operations were all less than 20 mm Hg, thoracoabodominal aorta main vessels multi-slice CT examination three months after operation showed that bypass vascular prosthesis was unobetructed, two cases showed that autologous artery at aortic coarctation distal end were partly occluded. Conclusion Ascending aorta-abdominal aorta vascular prosthesis bypass would be an effective means for the treatment of adult aortic coarctation patients.     

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.     

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.     

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.     

升主动脉至胸降主动脉人工血管转流在主动脉缩窄外科治疗中的应用

目的：分析总结使用升主动脉至胸降主动脉人工血管转流术治疗成人型主动脉缩窄10例临床病例资料,探讨升主动脉至胸降主动脉人工血管转流术在临床中治疗主动脉缩窄的意义。方法中国医学科学院、北京协和医院心脏外科2006年1月-2015年12月共收治成人主动脉缩窄患者10例,其中男性患者7例,女性患者3例,年龄14~35岁,中位年龄27岁,其中1例合并主动脉瓣二瓣畸形及主动脉根部瘤样扩张,余均为单纯主动脉缩窄。10例患者静息情况下测量上下肢压差均大于20 mmHg (1 mmHg=0.133 kPa),术前评估无明确手术禁忌。10例均在体外循环下行升主动脉至胸降主动脉人工血管转流术,合并主动脉瓣二瓣畸形及主动脉根部扩张的患者,同时行Bentall术。结果无围术期死亡及大出血、脊髓缺血性损害等严重并发症,嘱托患者出院后3~6个月门诊复查,复测上下肢压差均小于20 mmHg,CTA结果提示未见动脉瘤、人工血管血栓形成等表现。结论利用人工血管从升主动脉转流至降主动脉是治疗胸主动脉缩窄的有效方法。     

Dacron patch aortoplasty for coarctation of aorta

Our experience with dacron patch aortoplasty in 26 unselected patients of coarctation of thoracic aorta has been presented. The technique is very simple, safe and useful for routine repair of coarctation of aorta.     

Surgery for aortic coarctation: a 30 years experience

Objective: A retrospective study to review the experience of a single center with surgery for aortic coarctation over a period of 30 years (1970–1999). Methods: Criteria for inclusion: (a) aortic coarctation, isolated or associated with congenital heart defect; (b) surgery between 1970 and 1999. Data recorded: (1) date of surgery; (2) age at surgery; (3) associated lesions; (4) surgical technique; (5) simultaneous surgical procedures; (6) early and late surgical results in term of: (a) deaths; (b) need for reoperation because of re-coarctation or other cardiac lesion; (c) residual/recurrent pressure gradient, evaluated at cuff/Doppler at rest; (d) systemic hypertension, requiring medical treatment. Results: One hundred and forty-one patients underwent surgery for aortic coarctation: 30 neonates, 29 infants, 45 children and 37 adults. Associated lesions were found in 8/37 (=21.6%) adults and in 73/104 (=70.1%) pediatric patients. There were no hospital deaths. During the follow-up there were one late death in the adults group (1/37=2.7%) and three late deaths in the pediatric group (3/104=2.9%), all unrelated with aortic coarctation. Re-operation because of re-coarctation occurred only in ten late survivors of the pediatric group (10/101=9.9%), 9/10 operated on before 1980 (P<0.00001). End-to-end anastomosis, enlarged to the aortic arch in neonates, was associated with the lowest incidence of re-coarctation (P<0.005). A significant (>20 mmHg at rest) pressure gradient was found in none of the adults, and in seven of the 91 pediatric patients (7/91=7.7%) late survivors. Three adults (3/36=8.3%) late survivors are on medical treatment to control systemic hypertension. Conclusions: The long-term results of our retrospective study confirm that surgery has to be considered the gold standard for the treatment of aortic coarctation. The interventional angioplasty techniques have to provide long-term outcome at least similar to the results obtained with surgery.     

“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.