Objective To summarize the experiences of single stage repair of persistent fifth aortic arch associated with stenosis and interrupted aortic arch and other cardiac anomalies,and to improve surgical effect of the diseases． Methods From Jan．2000 to May 2008，five patients with persistent fifth aortic arch were operated in this hospita1，the age at operation was 1.8-108.0 months and body weight 3.7-31.0 kg．Three patients had chronic heart failure and respiratory infection repeatedly.All patients received single stage repair. Results There were two early hospital deaths，one patient’s parents gave up all the therapy because of cardiac insufficiency, pulmonary hypertension crisis and severe pulmonary infection; another one died of severe pulmonary hypertension crisis，the low cardiac outflow and left heart failure. Three patients were followed up, followup time was 55.67±48.64 months. The results were excellent,and one patient had been followed up for 8 years,the latest magnetic resonance imaging showed that diameter of the enlarged fifth aortic arch was 9.3 mm. Conclusion Persistent fifth aortic arch operation can achieve good exposure,less incisional wound and excellent recovery through midline sternotomy.Because of systemic hypertension and the affection of associated anomalies the operation should be performed as early as possible.
Abstract:Objective To summarize the experiences of single stage repair of interrupted aortic arch （IAA） associated with cardiac anomalies. Methods From Jan. 2000 to Dec. 2005, 48 patients admited in hospital and 35 patients were operated, the mean age at operation was 1.1 years. The associated anomalies included 23 cases of ventricular septal defect, 2 cases of transposition of great arteries, 3 cases of aortopulmonary window with aortic origin of right pulmonary artery, 2 cases of truncus arteriosus, 2 cases of double outlet right ventricle, 2 cases of stenotic fifth arch and 1 case of aberrant origin of right subclavian artery with mild hypoplastic decending aorta. Among them, 34 patients underwent single stage repair and 1 kid underwent palliative correction. Results There were 4 surgical deaths. The sequelae included one diaphragm paralysis and one 3rd degree of atrioventricular block. Only 5 kids recurred mild stenosis of aortic arch anastomosis and 2 death occurred during 3 months to 4 years of follow-up. Conclusion Though early surgical mortality for primary single stage repair is now relatively low, if appropriate interventions has been accomplished during perioperative period, but outcomes of IAA remain of concern, especially in patients with associated lesions.
摘要:目的:回顾性研究大动脉转换术同时进行主动脉弓矫治的I期手术治疗完全性大动脉错位或TaussigBing合并主动脉弓畸形的早中期效果。方法:2000年1月至2008年12月,连续对26例存在主动脉弓畸形的完全性大动脉错位或TaussigBing畸形的小婴儿进行了I期手术矫治,其中完全性大动脉错位13例(TGA/VSD 11例,TGA/IVS 2例),TaussigBing 13例;主动脉弓畸形中主动脉弓中断(A型)7例,CoA19例,6例伴有冠状动脉异常类型。平均手术年龄(28±35) d,lt;2个月占62％,手术平均体重为(4.19±1.15) kg。在深低温停循环或深低温低流量下进行主动脉弓畸形矫治,采用自身组织直接吻合扩大或重建弓,伴有弓部发育不良者补片扩大成形。伴有冠状动脉畸形者在大动脉转换手术中冠状动脉移植方法予改良处理。〖HTH〗结果〖HTSS〗:手术住院死亡3例(11.5%),死因与冠脉移植无关。平均插管时间102 h,监护室时间平均8 d。术后早期生存者主动脉瓣上压力阶差gt;30 mm Hg有2例,主动脉瓣反流轻度2例。单因素分析中伴有冠状动脉异常类型者与术后早期死亡或并发症的风险相关,多因素分析示其与手术年龄、肺动脉高压、术前FS、主动脉阻断时间、术后血清乳酸水平相关。随访期3个月~7年,无死亡,术后5年实际生存率为88.5％(95％ 可信度范围CI 76％～96％),术后1年、5年无需介入干预或手术分别为91.4％、87％。结论:TGA和TaussigBing伴有主动脉弓畸形者I 期进行大动脉转换术和主动脉弓畸形矫治早中期效果良好,早期手术并发症和死亡的风险因素为年龄偏大,肺高压严重,把握手术时机是手术成功要则之一。Abstract: Objective: The study was to evaluate earlymid term results after onestage arterial switch operation (ASO) associated with aortic arch repair for D Transposition of the great arteries (DTGA) and TaussigBing Anomaly with arch abnormally in infant. 〖WTHZ〗Methods〖WTBZ〗: Between January 2000 and December 2008, a primary operation including aortic arch repair through a midline sternotomy was performed in 26 patients, 13 patients with DTGA and 13 TaussigBing. Most patients (62%) underwent operation during the first two months. The repair of arch was accomplished under deep hypothermic circulatory arrest or low flow, employing a wide pericardial patch to reconstruction of arch in some patients or direct ananstomosis. Results: There were 3 (11.5%) hospital deaths. The high risk factors for early mortality and morbidity were unsuitable reconstructed arch, higher age, severe pulmonary hypertension and longer aortic crossclamp time. There were no late deaths. Actuarial 5year survival was 88.5% (95% CI 70% to 96%). Actuarial freedom from overall reintervention, reoperation among operative survivors was 91.4% at 1 year and 87% at 5 years, respectively. Conclusion: the singlestage repair for DTGA and TaussigBing with aortic arch abnormally is suitable choice for infant, and followup of operative survivors is favorable. Optimal operative time was as sooner as possible.
Objective To explore of a surgical approach of posterior pericardial ascending-to-descending aortic bypass through a median sternotomy for complex coarctation and interrupted aortic arch adult patients with coexistent cardiac disorder. Methods We retrospectively reviewed the clinical data of 2 adult patients with complex coarctation and 1 adult patient with interrupted aortic arch and all with coexistent cardiac disorder who underwent ascending-to-descending aortic bypass in our hospital between April 2010 and January 2015. There were 2 males and 1 female with age of 35.6 (27-46) years. One patient was with complex coarctation, and prolapse of anterior mitral leaflet with moderate regurgitation. One patient was with complex coarctation, and bicuspid aortic valve with severe aortic regurgitation, and ascending aortic aneurysm. One patient was with interrupted aortic arch (type A), and bicuspid aortic valve with mild stenosis, and secundum atrial septal defect. The surgical approach used in all patients was the median sternotomy. After aorta, femoral artery and bicaval cannulation, hypothermic cardiopulmonary bypass was established. With posterior pericardial ascending-to-descending aortic bypass procedure for repair of complex coarctation and interrupted aortic arch with coexistent cardiac disorder. Results There was no death. The symptoms of the patients obviously improved. All the patients were alive with ascending-to-descending aortic bypass procedure at a mean follow-up ranged from 2 to 59 months. Except that one patient had residual upper-extremity hypertension, and needed antihypertensive medications taken postoperatively, other patients’ systolic blood pressure returned to normal level. All patients’ lower-extremity fatigability resolved. Postoperative computed tomography angiography (CTA) of the patients showed that dacron graft was unobstructed with no graft-related complications of kinking and narrowing, development of false aneurysms or other complications. Conclusion The surgical management of adult patients’ complex coarctation and interrupted aortic arch with coexistent cardiac disorder, a one-stage approach using pericardial ascending-to-descending aortic bypass through a median sternotomy is an alternative surgery.
Objective To review the clinical experience of one-stage repair of interrupted aortic arch(IAA) in neonate with cardiac anomalies. Methods We retrospectively analyzed the clinical data of 21 patients (18 males, 3 females) with IAA total repair in our hospital between May 2003 and September 2014. The average age of patients was 6–26 (15.9±5.8) days and the mean body weight was 3.3±0.4 kg. Fourteen patients belonged to IAA type A, and 7 patients to type B. All patients were complicated with ventricular septal defect, atrial septal defect, and patent ductus artefious. All patients with cardiac anomalies underwent one-stage repair through median sternotomy. The aortic continuity was reestablished by anastomosis between the descending aortic segment and aortic arch. Results CPB time was 92–174 (132.6±27.1) min, and aortic cross clamping time was 48-118 (70.9±18.8) min. Hospital day was 4-52 (28.0±12.1) d. There were 3 postoperative deaths. Causes of death included a cardiac arrest in one patient, hematosepsis in one patient, and a pulmonary hypertension crisis in one patient. Eighteen patients were followed up for 3 months to 11 years and the results were excellent. Conclusion One-stage repair of IAA in neonate with cardiac anomalies can improve life quality of patients and achieve good results.
Objective To evaluate the outcome of surgical repair of interrupted aortic arch (IAA) combined with anomalies. Methods We retrospectively analyzed the clinical data of 48 patients with IAA combined with anomalies undergoing one-stage biventricular repair in Shanghai Children's Medical Center from November 2006 to April 2016. There were 25 males and 23 females with a median age of 29 d (range, 8 to 91 d) and a mean weight of 3.80±0.67 kg. All patients underwent end-to-end anastomosis with patch augmentation, and relief of left ventricular outflow tract obstruction (LVOTO) was performed in 11 patients. Results In IAA children with anomalies, 39 (81.3%) suffered noncomplex lesions and 9 (18.8%) complex lesions. Mean follow-up was 72.1±19.7 months for 38 patients. There were 6 in-hospital deaths and 3 patients died during follow-up. The early survival rate was 87.5%, 5-year rate 83.3% and 10-year rate 81.3%. Reintervention was required in 10 patients, including 8 with subsequent LVOTO and 2 with anastomotic stenosis. Conclusion End-to-end anastomosis with patch augmentation is effective for IAA.
Coarctation of the aorta and interrupted aortic arch are congenital anomalies affecting the aortic arch. Because of the poor natural prognosis, many patients will need early surgical repair or even emergency surgery. With the improvement of the surgical techniques, cardiopulmonary bypass techniques and perioperative intensive care, surgical mortality has now dramatically decreased. However, aortic arch restenosis, left ventricular outflow tract obstruction and long-term hypertension are problems that may be still encountered during the follow-up period. By reviewing large amounts of literature and discussing among experts, we achieved a consensus on many aspects of the management strategy. We hope this consensus will help Chinese colleagues further improve the overall surgical outcomes of coarctation of aorta and interrupted aortic arch.
Objective To analyze the risk factors for death in children with interruption of aortic arch (IAA) and ventricular septal defect (VSD) after one-stage radical surgery. Methods A retrospective analysis was performed on patients with IAA and VSD who underwent one-stage radical treatment in the First Hospital of Hebei Medical University from January 2006 to January 2017. Cox proportional hazards regression model was used to analyze the risk factors for death after the surgery. Results A total of 152 children were enrolled, including 70 males and 82 females. Twenty-two patients died with a mean age of 30.73±9.21 d, and the other 130 patients survived with a mean age of 37.62±11.06 d. The Cox analysis showed that younger age (OR=0.551, 95%CI 0.320-0.984, P=0.004), low body weight (OR=0.632, 95%CI 0.313-0.966, P=0.003), large ratio of VSD diameter/aortic root diameter (VSD/AO, OR=2.547, 95%CI 1.095-7.517, P=0.044), long cardiopulmonary bypass time (OR=1.374, 95%CI 1.000-3.227, P=0.038), left ventricular outflow tract obstruction (LVOTO, OR=3.959, 95%CI 1.123-9.268, P=0.015) were independent risk factors for postoperative death. Conclusion For children with IAA and VSD, younger age, low body weight, large ratio of VSD/AO, long cardiopulmonary bypass time and LVOTO are risk factors for death after one-stage radical surgery.