永存第五弓残存伴狭窄合并主动脉弓中断的外科治疗

目的总结永存第五弓残存伴狭窄合并主动脉弓中断的外科治疗经验,以提高手术疗效。方法自2000年1月至2008年5月,共手术治疗永存第五弓残存伴狭窄合并主动脉弓中断5例,手术年龄1．8～108．0个月,体重3．7～31．0kg。3例患者术前存在慢性心功能不全及反复呼吸道感染,5例患者均一期纠治第五弓狭窄／及合并的心血管畸形。结果手术死亡2例,1例术后并发心功能不全,肺动脉高压危象,严重肺部感染,依赖呼吸机,撤机困难,家属放弃治疗;另1例合并室间隔缺损,肺动脉高压,死于反应性肺动脉高压危象、低心排血量综合征和左心功能衰竭。术后随访3例,随访时间55．67±48．64个月,随访期间无死亡,无并发症发生。其中1例患者已随访8年,恢复正常学习,心脏磁共振成像（MRI）提示：第五弓与降主动脉连接处（Gore—Tex补片扩大修补处）轻度狭窄,直径9．3mm。结论永存第五弓残存伴狭窄合并主动脉弓中断患者手术采用胸骨正中切口径路,操作简便,暴露清楚,剖面小,有利于术后恢复。由于该病晚期亦出现体动脉高压,应尽早明确诊断及时手术治疗。     

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。     

Repair of Truncus Arteriosus and Interrupted Aortic Arch

A total of seven patients with truncus arteriosus and interrupted aortic arch (IAA) comprises our surgical experience in this condition. All underwent primary complete repair via median sternotomy between June 1985 and December 1989. Median age at repair was 8 days and median weight, 3.2 kg. Anatomy of these seven patients was truncus arteriosus type "1 1/2" in five patients and type II in two patients, IAA type B in six patients and type A in one patient. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta. Right ventricle to pulmonary artery continuity was established with a porcine valved conduit in four patients, aortic homograft in two, and aortic homograft monocusp patch in one. Three patients have required five reoperations (three in one patient). One reoperation was due to compression of the left main bronchus from the reconstructed aorta, one was due to obstruction of the aorta at the site of IAA repair, and one was due to compression of the left main bronchus, right pulmonary artery, and residual stenosis across the hypoplastic ascending aorta. There were no early or late deaths and all seven survivors are currently well with a mean follow-up of 29 months from initial repair.     

A Modification of Extended Aortic Arch Anastomosis Augmented with Subclavian Flap Aortoplasty for Interrupted or Hypoplastic Aortic Arch

Abstract   Background: Surgical repair for hypoplastic aortic arch in neonates carries a substantial risk of recurrent obstruction. Simple arch anastomosis is not always a solution in cases of extended arch hypoplasia. We present our modified technique of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. Method: We describe two neonates: interrupted aortic arch and transverse arch hypoplasia associated with aortic coarctation, who underwent a modification of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. Results: The patients recovered without any pressure gradient at the anastomotic site. Postoperative aortography showed no arch obstruction and they successfully underwent second stage repair. Conclusion: Our technique provides extensive augmentation of the aortic arch with a tension-free, wide and non-circumferential suture line which preserves potential for growth. The technique described may avoid persistent or repeat arch obstruction.     

一期手术矫治主动脉弓中断及伴发畸形

目的总结主动脉弓中断(IAA)及伴发畸形的手术治疗经验。方法自2000年1月至2005年12月我科共收治48例IAA及伴发畸形患者(平均年龄1.1岁、平均体重7.0kg),合并畸形有室间隔缺损23例,完全性大动脉错位2例,主-肺动脉窗伴右肺动脉起源于主动脉3例,第5主动脉弓残存狭窄2例,右心室双出口2例,永存动脉干2例,迷走右锁骨下动脉伴降主动脉发育不良1例。48例中35例接受手术纠治,其中34例IAA及伴发畸形一期解剖纠治,1例伴右心室双出口患者行主动脉弓吻合及肺动脉环缩术纠治。结果手术死亡4例。术后26例随访3个月~4年,死亡2例。术后发生并发症7例,Ⅲ°房室传导阻滞和膈神经麻痹各1例,轻度吻合口梗阻5例,其余患者无残余解剖问题,心功能良好。结论在新生儿期施行IAA一期矫治手术成功率高,围术期合适的干预是治疗成功的关键;由于伴发畸形复杂,远期生存率不容乐观。     

Contemporary Multicenter Outcomes for Truncus Arteriosus With Interrupted Aortic Arch

BackgroundTruncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited.MethodsA retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed. Major adverse cardiac events (MACE) were defined as postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. TA-IAA patients were compared with TA patients who underwent repair during the study period from the same institutions.ResultsWe reviewed 35 patients with TA-IAA. MACE occurred in 12 patients (34%). Improvement over time was observed during the study period with 11 events (92%) occurring in the first half of the study period (P = .03). Factors associated with MACE included moderate or severe truncal valve insufficiency (P < .01), concomitant truncal valve repair (P = .04), and longer cardiopulmonary bypass duration (P = .02). In comparison with 216 patients who underwent TA repair, patients with TA-IAA had a higher rate of MACE, but this finding was not statistically significant (34% vs 20%, respectively; P = .07). Additionally no differences between TA-IAA and TA groups were observed for unplanned reoperations (14% vs 22%, respectively; P = .3), hospital length of stay (24 vs 23 days, P = .65), or late deaths (7% vs 7%, P = 1.00).ConclusionsIn this contemporary, multicenter cohort the rate of MACE after repair of TA-IAA was high but improved during the study period. Early childhood outcomes of patients with TA-IAA were similar to those with TA.     

Primary Reconstruction of Interrupted Aortic Arch - Surgical Management and Results

Type B interrupted aortic arch associated with other extracardiac and intracardiac congenital lesions is a lethal defect. Surgical management with a one- or two-stage approach is complex, difficult and associated with high morbidity and mortality. Between January 1996 and December 1999, 5 consecutive patients with type B interrupted aortic arch were operated on. The group included 3 girls and 2 boys weighing from 2.4 kg to 3.5 kg (mean 2.84 kg) and aged from 4 to 35 days (mean 15 days). All patients underwent a primary reconstruction of the aortic arch and one-stage repair of the associated heart defects. Two patients developed symptoms of mild aortic arch narrowing 7-12 months postoperatively. Balloon angioplasty of the obstructed site was successfully carried out in one of the patients. In the second child, angioplasty was not successful and surgery was used. All the patients survived and are being followed-up. This experience supports a strategy of primary one-stage repair of IAA type B, including direct anastomosis for aortic arch reconstruction, and all associated heart anomalies in the neonatal period.     

Severe Subaortic Stenosis in Interrupted Aortic Arch in Infancy and Childhood

Thirteen out of a total 50 infants with interrupted aortic arch (IAA) seen between 1979-1988 had or developed severe subaortic stenosis (SAS). One had type A interruption and 12 type B. All had a large ventricular septal defect (VSD). The infundibular septum was displaced posteriorly in eight infants, severely narrowing the left ventricular outflow tract (LVOT). Three had fibromuscular narrowing of the LVOT, one each a subaortic muscle bar and membrane. The aortic root and subaortic area were small and measured between 3-8 mm. An anomalous right subclavian artery was noted in eight of the 12 type B IAA, the anomalous vessel arising from the descending aorta in seven. Thirteen infants with IAA and SAS were submitted to surgery, 12 having their subaortic area resected with three perioperative deaths early in the series and three late deaths where active treatment was ceased, including one infant with renal dysplasia who had had a successful establishment of arch continuity and pulmonary artery banding. Of the seven survivors, six have residual Doppler gradients of between 20-50 mmHg, two requiring a second resection and one a third resection. Alternate management programs are suggested based on anatomical evaluation and echocardiography. Any newborn presenting with IAA requires careful evaluation of the subaortic area, best seen on cross-sectional echocardiography. The improved surgical survival in infants following complete repair of IAA has led this important associated anomaly of subaortic stenosis to assume greater importance as a cause of mortality and long-term morbidity.