主动脉缩窄术后血管结构和功能变化

主动脉缩窄的主要病变是主动脉局限性短段管腔狭窄或闭塞引起主动脉血流障碍,血流阻力增大,因而缩窄近端血压升高,远端血供减少、血压降低.其主要治疗方法是外科手术,常用的术式有主动脉缩窄段切除及端端吻合术、缩窄段切除及人工血管移植术、主动脉缩窄段扩大和织片缝补术、应用锁骨下动脉瓣作主动脉成形术等.对于缩窄段很短的婴幼儿病例,也可采用经皮球囊导管主动脉扩张成形术.治疗目的是切除狭窄段,重建主动脉正常血流通道,使血压和循环功能恢复正常.然而,一些研究表明,病人术后仍存在血管结构和功能异常.现对主动脉缩窄术后血管结构和功能的变化及其机制综述如下.     

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。     

右肺动脉起源于升主动脉合并动脉导管未闭二例

病例1 女,5岁。发现心脏杂音1年。就诊时查体：血压126／75mmHg,无紫绀,左胸第2肋间闻及3／6级收缩期杂音,P2亢进。超声心电图提示：先天性心脏病动脉导管未闭,主动脉峡部膜样回声。以先天性心脏病动脉导管未闭合并主动脉缩窄收入院。入院后行升主动脉造影及再次心脏超声心动图检查,确诊为右肺动脉起源于升主动脉合并动脉导管未闭、肺动脉高压。     

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.     

冠状动脉硬化性心脏病合并左锁骨下动脉严重狭窄非体外循环下同期手术的临床经验

目的探讨非体外循环冠状动脉旁路移植同时主动脉-锁骨下动脉旁路治疗冠状动脉硬化性心脏病(冠心病)合并锁骨下动脉重度狭窄的手术方法及效果.方法2003年1月～2004年5月,我院治疗须行冠状动脉旁路移植术同时合并左锁骨下动脉近端重度狭窄3例,术中先行主动脉-锁骨下动脉旁路,左乳内动脉获得满意的流量后,再行非体外循环冠状动脉旁路移植.结果手术时间210～340 min,平均283 min,出血量570～1 630 ml,平均963 ml.游离左乳内动脉后量杯测流量均＜5 ml/min,主动脉-锁骨下动脉旁路后量杯测流量均＞50 ml/min,乳内动脉远端与前降支吻合后流量仪测流量12～27 ml/min,平均20 ml/min.术后临床症状缓解,未发现冠脉-锁骨下动脉窃血综合征.3例随访3～6个月,平均5个月,无心绞痛发作.结论非体外循环冠状动脉旁路移植同时主动脉-锁骨下动脉旁路手术是治疗冠心病合并锁骨下动脉重度狭窄简单而有效的方法.     

主动脉弓离断并法洛四联症、右锁骨下动脉及降主动脉侧支起源1例

病儿女,12岁。自幼发现心脏杂音,全身发绀,有蹲踞现象。查体血压右上肢90/70mmHg(1mmHg=0.133kPa),左上肢100/70mmHg,右下肢110/70mmHg,左下肢110/80mmHg。口唇及颜面发绀,心率80次/min,律齐,胸骨左缘3、4肋间可闻及4/VI级喷射性收缩期杂音,杵状指(趾)。心电图示窦性心律,电轴右偏(109°),右室肥厚。X线胸片示心胸比率0.53,双肺血稀疏,心腰凹,右室增大,双上腔静脉可疑。心脏彩色超声示右心房、室增大,左心腔内径相对较小,主肺动脉及分支内径正常。左室长轴及大动脉短轴切面显示主动脉大部分发自右室,主动脉在右前,肺动脉在左后;右室流出道8mm;室间隔与主动脉前壁回声连续中断23mm;左上腔静脉永存。动脉血气分析PCO_235mmHg,PO_252mmHg,SaO_20.87。经皮四肢末梢血氧饱和度左上肢0.87,右上肢0.86,双下肢均为0.87。左、右心血管造影见两大动脉均起自右室,右室流出道狭窄,膜部室间隔缺损,主动脉弓于左锁骨下动脉后中断,右锁骨下动脉及降主动脉由主动脉侧支形成(图1～8)。图1右心室造影示右室流出道狭窄图2、3右心室造影示升主动脉、主肺动脉同时显影,膜部室间隔缺损,右室流出道狭窄,肺动脉发育佳图4猪尾心导管经右股动脉(测压证实)到达侧支形成的降主动脉起始处造影,见造影剂向下快速充盈图5心导管经右桡动脉、右锁骨下动脉(测压证实)进入降主动脉造影,见造影剂向下快速充盈图6心导管经右桡动脉、右锁骨下动脉到达侧支形成右锁骨下动脉和降主动脉的共汇处造影,见造影剂向两处快速充盈图7心导管经左桡动脉、左锁骨下动脉进入升主动脉造影,见左、右颈总动脉和左锁骨下动脉均显影,右锁骨下动脉未显影,左锁骨下动脉以后主动脉弓中断图8图7的下一时相,见大量侧支形成右锁骨下动脉及降主动脉2004年8月在全麻低温体外循环下行法洛四联症根治术。术中见主动脉∶主肺动脉为2∶1,右室扩大,右室流出道肌性狭窄,肺动脉瓣环狭窄,直径0.5cm。肺动脉瓣二瓣化畸形,主肺动脉及左、右肺动脉发育良好。嵴下型室间隔缺损3.0cm×2.5cm大小。主动脉骑跨70%以上,二尖瓣与主动脉瓣有纤维连续。左上腔静脉永存。疏通右室流出道,血管片补片关闭室间隔缺损,垫自体心包的涤纶片跨肺动脉瓣环加宽右室流出道。考虑病儿主动脉系侧支循环丰富,对主动脉中断未处理。术终测上、下肢动脉平均压差仅10mmHg。病儿术后恢复顺利,术后10d痊愈出院。现已随访28个月,病儿恢复正常生活和学习。讨论主动脉弓离断罕见,病死率较高,在先心病婴儿尸检中,发现率为1.4%〔1〕。室间隔缺损和动脉导管未闭是最常见的合并畸形,亦可合并永存共同动脉干、主肺动脉间隔缺损、大动脉转位、右肺动脉起源异常、右肺动脉闭锁和左冠状动脉异常起源于右肺动脉等。心血管造影是术前确诊、分型及鉴别诊断的重要方法。MRI和UFCT也是诊断本症的重要手段。外科手术可一期根治,也可分二期进行,先恢复主动脉弓连续性、再矫治合并畸形。目前倾向一期手术〔1-4〕。     

主动脉缩窄合并降主动脉瘤行升-腹主动脉搭桥术后动脉瘤自愈1例

病人　女,4 1岁。头晕、双下肢麻木15年,加重伴胸闷2月余。术前血压:右上肢16 0 5 0mmHg(1mmHg =0 133kPa) ,左上肢及双下肢均为90 70mmHg。磁共振血管造影示主动脉弓降部于左锁骨下动脉分支以近重度缩窄,狭窄以远左锁骨下动脉下方可见一动脉瘤形成,约2 0mm×30mm大小(图1)。因缩窄段累及主动脉弓远端,决定分期手术,先行升主动脉至腹主动脉搭桥术解除缩窄,二期手术切除动脉瘤。2 0 0 3年6月全麻下行升主动脉至腹主动脉搭桥术。胸腹正中联合切口,1 8cm×30cm人工血管经前纵隔及左结肠旁分别与腹主动脉及升主动脉行端侧吻合,开放后上下肢…     

同种主动脉治疗复杂先天性心脏病

目的　总结用同种带瓣主动脉和同种主动脉治疗 11例复杂先天性心脏病的经验。　方法　对 11例法洛四联症合并肺动脉闭锁和动脉导管未闭 ,矫正型大动脉转位合并室间隔缺损和肺动脉狭窄 ,完全型大动脉转位合并室间隔缺损和肺动脉狭窄 ,右心室双出口合并完全性房室通道 , 型永存动脉干 ,主动脉弓中断合并动脉导管未闭 ,主动脉缩窄合并动脉导管未闭和二尖瓣关闭不全患者施行了手术治疗 ;其中 Rastelli手术 7例 ,全腔静脉与肺动脉连接 (TCPC) 2例 ,升主动脉至降主动脉旁路移植 2例。　结果　手术死亡 1例 ;慢性心力衰竭 1例 ,内科治疗 1年后心功能 级。随访 1个月～ 7.2年 ,无远期死亡。　结论　同种主动脉是矫正复杂先天性心脏病的理想材料     

降主动脉-升主动脉吻合术矫治婴儿期主动脉缩窄合并心内畸形15例

目的探讨降主动脉-升主动脉吻合术治疗婴儿期主动脉缩窄合并主动脉弓发育不良及心内畸形的疗效。方法选取2011-05—2015-05间治疗的主动脉缩窄合并主动脉弓发育不良、心内畸形的患儿15例。患儿均采用胸骨正中切口、开胸后先游离出主动脉弓、头臂干、动脉导管、弓降部等血管。建立体外循环、选择性脑灌注下行降主动脉-升主动脉端侧吻合术,恢复全身灌注后完成心内畸形的矫治。体外循环时间86~132 min,主动脉阻断时间51~94 min。结果术后早期死亡2例,低心排出量综合征6例,室上性心动过速6例,肺炎7例。13例患儿随访2个月~3 a,无死亡及再次主动脉狭窄。结论降主动脉-升主动脉吻合术治疗婴儿期主动脉缩窄合并主动脉弓发育不良及心内畸形的临床效果满意。     

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.     

Stenting for infantile adult aortic coarctation with successful conception of zygomatic twins at 4 years’ post-intervention

INTRODUCTIONAortic coarctation is a congenital defect which rarely presents in adulthood but results in significant morbidity and mortality. Endovascular techniques present novel therapeutic options for managing this anomaly with comparable results to traditional open surgical repair.PRESENTATION OF CASEWe convey a case of postductal aortic coarctation in a symptomatic 31-year-old woman, undetected, despite 27 years history of congenital bicuspid valve with aortic incompetence. We staged a successful endovascular repair following presentation with hypertension, abdominal pain, and worsening lower limb claudication. This lady recovered well, eventually giving birth to twins after a successful caesarean section delivery. The anatomical, clinical and technical features of the case are appraised.DISCUSSIONAortic coarctation is classified anatomically as preductal (infantile) or postductal (adult) and the morphological spectrum of abnormality ranges from a discrete stenosis distal to the left subclavian to a hypoplastic transverse arch and aortic isthmus presenting in infancy. Its incidence is 0.2–0.6 per 100 live births. Traditional open surgical repair of aortic coarctation is associated with high morbidity and mortality. Advances in endovascular techniques with their associated shorter hospital time and lower costs, reduced short term morbidity and mortality mean that they are likely to become mainstays of treatment.CONCLUSIONThe presentation of aortic coarctation for the first time in an adult is extremely unusual. Error and delayed diagnosis can never be fully exempted in the practice of medicine even with the most advanced diagnostic tools. Follow-up is mandatory by trans-oesophageal echo in all endovascular patients.     

Left atrial epicardial ablation associated to a Bentall procedure.

Aortic coarctation is quite a common congenital disease and very often associated with other cardiac malformations. A 21-year-old patient presented to our observation with aortic coarctation, aortic valve regurgitation on a dilated aortic root and chronic atrial fibrillation. We performed a two-step operation: the aortic coarctation was treated first and 1 month later a Bentall procedure associated to an epicardial ablation was performed. Since most of the ablation was performed before aortic cross clamping, the ischemic time was only slightly increased. At 3 months follow-up the patient is still on normal sinus rhythm.     

Dilatation of ascending aorta in patients with repaired coarctation

The long-term outcome following repair of typical aortic coarctation in adulthood may be complicated by disorders of the ascending aorta. Follow-up averaging 15 years revealed a 3.8% incidence of dilatation of the ascending aorta after such late repair. Hypertension and concomitant aortic valvular disease were common in these patients. Aortic dilatation can appear years after coarctation repair, irrespective of the operative technique and its success, and can lead to death from aortic dissection or rupture of an aortic aneurysm. Careful follow-up after coarctation repair in adulthood is advisable to detect late aortic complications.     

Abdominal aortic coarctation inducing aortic occlusion and renovascular hypertension.

Abdominal aortic coarctation is a rare, non-atherosclerotic disease. It is a functionally significant at an early age when associated with aortic branch stenosis and renovascular hypertension. The pathogenesis of aortic constrictive lesions remains unknown, but may be related to developmental error or aortic growth arrest and various hypotheses have been reported. When the renal arteries are involved by the coarctation, severe hypertension is common at an early age and in untreated patients, life-threatening complications commonly occur. Patients who reach the age of 40 years generally have the coarctation below the renal arteries but even when the renal arteries are not involved by the coarctation, renovascular disease may still occur due to secondary atherosclerosis. Aortic thrombosis secondary to abdominal aortic coarctation with renovascular disease and lower limb ischemia, occurring in a 63-year old woman, is reported.     

Adult aortic coarctation discovered incidentally after the rupture of sinus of Valsalva aneurysm: combined surgical and interventional approach

Combination of ruptured sinus of Valsalva aneurysm (SVA), and a coexisting asymptomatic adult aortic isthmic coarctation is extremely rare. The timing and sequence of surgical and/or interventional repair of these two pathologies are controversial. We present a case of a 37-year-old male who was admitted to our department because of severe acute congestive heart failure and signs of ruptured aneurysm of the SV into the right ventricle. Transthoracic and transoesophageal echocardiography confirmed the communication between an important right coronary SVA and right ventricle, bicuspid aortic valve, mild aortic regurgitation, and revealed severe aortic coarctation. Because of the severe dilation of right sinus of Valsalva a surgical repair of the ruptured aneurysm was performed. Aortic coarctation was treated four weeks later by a percutaneous stent-graft implantation. This case report supports the concept that hybrid approach is feasible in patients with ruptured SVA and aortic coarctation in adulthood.     

Transverse arch hypoplasia may predispose patients to aneurysm formation after patch repair of aortic coarctation

BACKGROUND: Thoracic aortic aneurysm after patch repair of aortic coarctation is a potentially lethal complication. We hypothesized that transverse arch hypoplasia in association with patch repair of aortic coarctation predisposes aneurysm formation. METHODS: A retrospective analysis was performed on all patients undergoing isolated aortic coarctation repair at the University of Virginia Health Systems between 1970 and 1995. Of 244 repairs, 38 patients underwent patch repair. These 38 patients were divided into two groups. The aneurysm group (n = 15) had aortic aneurysms develop at the repair site, which required aneurysmectomy. The nonaneurysm group (n = 23) did not have any aneurysms develop. Univariate analysis and Fisher's exact text were used to identify factors that independently predict aneurysm formation. RESULTS: Univariate analysis demonstrated aortic arch hypoplasia associated with patch repair independently predicts future aneurysm formation (p < 0.01). Patients who had an aneurysm develop also had a similar incidence of bicuspid aortic valves, mild restenosis, and late hypertension compared with patients in the nonaneurysm group. CONCLUSIONS: Aneurysm formation after patch repair of aortic coarctation develops into a subgroup of patients. Aortic arch hypoplasia associated with coarctation independently predicts future aneurysm formation.     

Interdigitating arch reconstruction eliminates recurrent coarctation after the Norwood procedure

BACKGROUND: We sought to determine whether evolving techniques of aortic arch reconstruction used during the Norwood procedure decreased the incidence of postoperative aortic arch obstruction. METHODS: Our technique for aortic arch reconstruction in patients undergoing the Norwood procedure has evolved from using an allograft patch (classic group, n = 26) to primary connection of the pulmonary artery and arch (autologous group, n = 20). More recently, we have used a novel technique involving coarctation excision, an extended end-to-end anastomosis on the back of the arch, and a counterincision on the anterior descending aorta to sew in an allograft patch for total arch reconstruction (interdigitating group, n = 33). Cardiac catheterizations performed before stage II palliation were reviewed for aortic diameters at multiple levels in 79 infants (median age, 4.2 months). Aortic arch obstruction was defined as a ratio between the diameters of the arch anastomosis and the descending aorta (coarctation index) of less than 0.7. RESULTS: Overall, 15 (19%) children had aortic arch obstruction. All 15 required aortic intervention (balloon angioplasty, n = 12; surgical patch angioplasty, n = 2; both, n = 1). Aortic arch obstruction rates for the classic, autologous, and interdigitating groups were 46% (n = 12), 15% (n = 3), and 0%, respectively ( P > .001). CONCLUSION: Reconstruction of the aortic arch with excision of ductal and coarctation tissue is associated with lower aortic arch obstruction rates in patients undergoing the Norwood procedure. Arch reconstruction with a novel interdigitating technique decreases the incidence of aortic arch obstruction.     

Repair of a complex aortic arch anomaly associated with cutaneous hemangioma

Aortic coarctation and cutaneous hemangioma is a rare association. We describe the case of a neonate with abnormal looping of the aortic arch associated with hemangioma of the head and neck who underwent complex surgical repair without cardiopulmonary bypass.     

Aortic valvular insufficiency and postductal aortic coarctation with small aorta syndrome: one-stage surgical management using extra anatomic bypass through median sternotomy

A 30-year-old man who had undergone repair for coarctation of the thoracic aorta at age 7 and mitral valve annuloplasty at age 9 was admitted for shortness of breath and claudication of both lower legs. The preoperative angiogram showed severe aortic regurgitation, moderate coarctation of the thoracic aorta beyond the left subclavian artery, a degree of hypoplasia of the infrarenal abdominal aorta, and total occlusion of both external iliac arteries. Aortic valve replacement, ascending-to-bilateral femoral arterial bypass, and end expanded polytetra fluoro ethylene (ePTFE) graft-to-descending aorta bypass was performed via a median sternotomy. Ascending-to-descending aortic bypass via the posterior pericardium allows simultaneous intracardiac repair or an alternative approach for the patient with complex coarctation.     

Management of recurrent coarctation of the aorta: a new experimental technique

A new technique is described for the management of recurrent coarctation of the aorta. It involves enlarging the narrowed segment by an onlay patch sutured to the adventitia and outer media of the aortic wall. The procedure was used in 6 mongrel dogs with preexisting surgically created coarctation. Aortic cross-clamping time ranged between 7.5 and 11 minutes (mean, 8.8 +/- 1.3 minutes). There were no operative deaths or complications. Gross and microscopic examination of the aorta 6 to 12 months (mean, 9 +/- 2.2 months) postoperatively revealed a 290 to 380% (mean, 350 +/- 30%) increase in the diameter of the repaired area and no evidence of thrombosis or pseudoaneurysm formation. The need for minimal dissection and the brief period of aortic cross-clamping make this approach an attractive alternative in the surgical treatment of patients with difficult cases of recoarctation.