Coarctation of aorta with right aortic arch and anomalous left subclavian artery]

The occurrence of coarctation in patients with right aortic arch is extremely rare. We have encountered a 21-year-old man with anomalous left subclavian artery. He was diagnosed when he was 13. On angiography he exhibited a right aortic arch. The left common carotid artery, right common carotid artery and the right subclavian artery diverged from the proximal side of the coarctation. The left subclavian artery diverged from the distal side. The right brachial arterial pressures measured 158-72 mmHg, while the left brachial arterial pressures measured 98-80 mmHg. Clinical studies revealed no vascular ring and no other anomalies were found in this patient. An attempt at treatment was made with extra anatomical bypass grafting from the ascending aorta to the descending aorta. No pressure difference due to coarctation remained after operation.     

A case report of atypical coarctation of the aorta with coarctation and left subclavian arterial aplasia

A 18 year-old female was admitted to our hospital with hypertension of 190/100 mmHg in her right arm and 140/110 mmHg in the left arm. Femoral arterial pulsation was not palpable. Her physical status and growth of the lower limbs were normal. Aortography demonstrated slight coarctation with a pressure gradient of 10 mmHg and descending aortic stenosis with a pressure gradient of 80 mmHg. Main three branches from aortic arch were anatomically normal. However, the left subclavian artery distal to the origin of the left vertebral artery was not visualized and the axillary artery was perfused by collaterals. Atypical coarctation was replaced using a woven Dacron graft of 14 x 40 mm. Postoperatively proximal pressure decreased from 180/113 to 156/98 mmHg and there was no pressure gradient between proximal and distal of the graft. After six months blood pressure in the right arm gradually decreased to 120/78 mmHg.     

Late results of the subclavian flap repair of coarctation in infancy

From June 1979 through January 1985, 22 infants under 20 months of age (mean 8 months and 4 kg) underwent coarctation repair with a left subclavian aortoplasty. The most commonly associated lesions were ventricular septal defect (50%), hypoplastic aortic arch (45%), patent ductus arteriosus (41%), transposition of great arteries (22.7%) and other intracardiac lesions comprised 30%. Indication for surgery was congestive heart failure refractory to medical management. All patients were on inotropic support. Two patients required mechanical ventilation preoperatively. Subclavian flap angioplasty was performed with 7.0 continuous polypropylene suture. Ligation of a patent ductus arteriosus was simultaneously done in 8 patients and pulmonary artery banding in 8 others. Four patients (18%) died within 30 days after operation; 3 of them had severe tubular hypoplasia of the aortic arch. Three transient postoperative complications were encountered; chylothorax, subclavian steal syndrome and Horner's syndrome. No hospital deaths occurred in patients with isolated coarctation of the aorta. All survivors were followed for a period varying from 3 to 6.5 years (mean, 2 years). There were 3 late deaths (13.6%). Non invasive studies have shown a satisfactory result in 14 patients with a residual gradient less than 20 mmHg and in 10 of them, cardiac catheterization and cineangiography showed no evidence of significant stenosis. In one patient only, femoral pulses were absent 4 months postoperatively. These results and reports of others indicate, contrary to the age, that the presence of associated disease affects mortality. Prompt repair of coarctation of the aorta in all infants with or without symptoms is recommended and the subclavian flap procedure appears to be a simple and safe method for relief of coarctation of the aorta in critically ill infants.     

A case of coexisting descending thoracic aortic aneurysm and atypical aortic coarctation treated successfully by surgery using the thromboexclusion method

The thromboexclusion method was successfully applied to a 43-year-old male with aortitis syndrome. Preoperative aortogram showed a fusiform aneurysm of the descending aorta just below the left subclavian artery and atypical coarctation of the descending aorta distal to this aneurysm. Pressure gradient across the stenosis was about 70 mmHg. The technique of flow reversal and thromboexclusion was performed in this patient because of severe calcification in the aortic arch and the entire descending aorta. A long extra-anatomical bypass between the ascending aorta and the infrarenal abdominal aorta was made, and a permanent aortic clamp was placed across the aorta at the left subclavian artery. Hypertension in the arm disappeared immediately after the operation, and postoperative catheterization revealed no pressure gradient between the ascending and the abdominal aorta. Computed tomogram performed 18 days after the operation and aortogram done 44 days postoperatively disclosed thrombi formation in the aneurysm.     

Repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum

A case of right aortic arch with an aberrant left subclavian artery arising from a Kommerell's diverticulum of the descending thoracic aorta with a left ligamentum arteriosum completing a vascular ring about the trachea and esophagus is presented. A posterolateral left thoracotomy alone is sufficient to provide an excellent exposure for the division of the ligamentum arteriosum and the resection of the Kommerell's diverticulum. Furthermore, the development of a subclavian steal syndrome is prevented by the reimplantation of the divided left subclavian artery into the left common carotid artery through this same incision.     

Development of neo-coarctation in patients with transposed great arteries and hypoplastic aortic arch after Lecompte modification of anatomical correction

We report on three patients with kinking in the proximal aortic arch that developed after Lecompte modification of the arterial switch operation. Two patients had a previous subclavian patch repair of coarctation of the aorta and had an associated hypoplasia of the transverse aortic arch, and one patient had hemodynamically mild coarctation at the anatomical repair. A severe pressure gradient across the kinked area ("neo-coarctation") necessitating reoperation developed in one patient. The acute arch angulation appears to be due to an excessive posterior displacement of the ascending aorta by the anterior relocation of either the right or left main pulmonary artery branch from underneath the aortic arch. A foreshortened and frequently hypoplastic transverse aortic arch, a common association with coarctation of the aorta, appears to be especially vulnerable to the development of "neo-coarctation" after the Lecompte modification of the anatomical repair of transposed great arteries.     

Subclavian steal syndrome associated with the right aortic arch in a patient with Peutz-Jeghers syndrome: case report

We report an adult case who presented vertigo attacks due to subclavian steal syndrome associated with the right aortic arch in a patient with Peutz-Jeghers syndrome. A 29-year-old male diagnosed as having Peutz-Jeghers syndrome developed frequent vertigo attacks and was admitted to our hospital. Blood pressure of the left arm was 20 mmHg lower than that of the right arm. Aortagraphy showed that he had a right aortic arch and isolation of the left subclavian artery. Right vertebral angiography opacified the left vertebral artery and the subclavian artery in retrograde fashion, suggesting subclavian steal phenomenon. Blood flow studies disclosed impaired reactivity to acetazolamide in the bilateral cerebellar hemispheres. We successfully carried out left common carotid artery-transverse cervical artery bypass. Postoperative angiography confirmed the patency of the graft and the disappearance of subclavian steal phenomenon. Vasoreactivity to acetazolamide normalized in the cerebellum. Vertigo attacks were not noted during the one-year follow-up period.     

Aortic coarctation,vascular ring,and right aortic arch with aberrant subclavian artery

Right aortic arch, in all situations, is relatively rare. In association with coarctation and vascular compression, it is extremely rare. We present a patient with a right aortic arch and an aberrant left subclavian artery, in addition to coarctation. This was dealt with through a left thoracotomy by dividing the ligamentum arteriosum and placing a Dacron graft from the ascending aorta to the descending aorta.     

Extended direct anastomosis for coarctation of the aorta and interruption of the aortic arch

Between March, 1986 and May, 1988, extended direct anastomosis was performed for coarctation of the aorta (CoA) (5 infants) and interruption of the aortic arch (IAA) (3 infants). The aortic arch was hypoplastic in 3 patients. The incision was made in the inferior aspect of the aortic arch proximal to the origin of the carotid or brachiocephalic artery, which was then anastomosed to the descending aorta. Pulmonary artery banding was placed in 4 patients with associated complex cardiac anomalies. Aortic cross-clamp time was 19-54 minutes (mean 41 minutes). Pressure gradient between upper and lower extremity was 0-10 mmHg (mean 4 mmHg). There were no operative deaths and no neurologic complications. The peak flow velocity at the site of aortic reconstruction measured by Doppler echocardiographic study after surgery was 1.2-2.5 m/sec (mean 1.7 m/sec). This procedure has the advantages of leaving the subclavian artery intact and no aortic shelf tissue. And it can be applied in IAA or CoA with hypoplastic aortic arch.     

Subclavian Steal Syndrome in the Pediatric Age Group

Four pediatric patients with subclavian steal syndrome (SSS) are described. One patient with congenital SSS had persistence of the lesion following surgical repair of coarctation of the aorta due to a gradient across a hypoplastic aortic arch. In 3 patients SSS developed on an acquired basis as a sequel to corrective operation, for coarctation in 1 patient, and interrupted aortic arch in 2 patients. Contributory factors included hypoplasia of the aortic arch, anatomical variation of the origin of the vertebral artery, and discrete recoarctation involving the ostium of the subclavian artery. Treatment was by vertebral artery ligation in 3 patients and by prosthetic patch arterioplasty in the patient with recoarctation. The 4 patients remain asymptomatic on follow-up of 16 to 25 months.     

A case of ruptured cerebral aneurysm associated with coarctation of the aorta

A 15-year-old boy with subarachnoid hemorrhage was planned for emergency cerebral aneurysm clipping under general anesthesia. He had different blood pressure between the upper limbs and we found coarctation of the aorta at left subclavian artery bifurcation in the preoperative angiography. To prevent re-rupture of cerebral aneurysm and ischemia of abdominal organs, we monitored arterial blood pressure in bilateral radial arteries and non-invasive blood pressure in the left thigh, and his blood pressure was maintained within 120-150 mmHg of systolic pressure in the right radial artery and 50-70 mmHg of mean arterial pressure in the left radial artery and the left thigh during general anesthesia. The preoperative period elapsed uneventfully and the patient was planned for repair of coarctation of the aorta after discharge.     

A rare case of a blunt thoracic aortic injury in a patient with an aberrant right subclavian artery: A case report and literature review

IntroductionBlunt thoracic aortic injuries (BTAIs) are an uncommon traumatic injury that if not treated promptly, can result in death. We present the case of a BTAI with aberrant aortic anatomy.Presentation of caseA 60-year-old female was involved in a motor vehicle crash where she suffered significant polytrauma including a BTAI. She was also found to have an aberrant right subclavian artery originating from the aortic arch. Thoracic Endovascular Aortic Repair (TEVAR) with a right common carotid artery to right subclavian artery bypass was accomplished. She required three more vascular surgical interventions, two for persistent type II endoleak and the third for left upper extremity acute limb ischemia. She had a 2-month hospital course for her devastating injuries and was eventually discharged home. A follow-up CT angiogram showed a stable thoracic aortic arch stent.DiscussionBTAIs are uncommon in the trauma population. In our patient who had an aberrant right subclavian artery, further procedures were required in the form of a right common carotid artery to right subclavian artery bypass and embolizations to resolve endoleaks.ConclusionBlunt thoracic aortic injuries are life threatening and require urgent intervention. TEVAR is associated with better outcomes. An aberrant right subclavian artery originating from the aortic arch, distal to the left subclavian artery is an anatomic variant that adds significant complexity to TEVAR. TEVAR is still an option for repair of blunt thoracic aortic injuries despite anatomic variations as open repair still carries an increased risk of morbidity and mortality.     

Surgical treatment for graft stenosis after repair of an interrupted aortic arch: Report of two cases

We report herein two cases of patients who underwent successful reoperation for graft stenosis after repair of an interrupted aortic arch (IAA). The first patient was a 10-year-old girl who suffered from upper limb hypertension 9 years after her initial operation. Cardiac catheterization revealed a pressure gradient of 55 mmHg across the repaired arch. At reoperation, a left subclavian turndown anastomosis was performed, following which the hypertension resolved and a car-diac catheterization done 5 years later demonstrated sufficient growth of the restored arch with no significant gradient. The second patient was a 17-year-old boy who suffered from general fatigue and intermittent hypertension 12 years after his initial operation. Cardiac catheterization revealed a gradient of 60 mmHg across the repaired arch. He underwent an extraanatomic ascending to descending aortic bypass employing an additional 18-mm graft, and a postoperative cardiac catheterization showed no gradient between the ascending and descending aorta. Our experience has shown that IAA should be repaired without prosthetic grafts if possible. Although extraanatomic bypass is useful for reducing the operative risks at reoperation, a large graft should be used to avoid the need for a third operation. For young children expected to outgrow a second graft, performing an endogenous anastomosis, such as a left subclavian turndown anastomosis, should be considered as an alternative. Received: June 14, 1999 / Accepted: March 24, 2000     

One-stage repair with separated cardiopulmonary bypass for coarctation of the aorta with left aortic arch and right thoracic descending aorta

We treated a 7-day-old neonate with aortic coarctation, left aortic arch, right-side descending aorta, aberrant right subclavian artery, and outlet ventricular septal defect. Surgical one-stage repair consisting of coarctation repair by extended direct anastomosis, patch closure of the ventricular septal defect, and translocation of the right subclavian artery was performed under a separated (upper and lower body) perfusion with ascending and thoracic descending aortic cannulation. The postoperative course was complicated with massive chylous effusion, which was successfully treated by thoracic duct ligation.     

A case of simple coarctation]

We report a successful surgical repair of the simple coarctation of a 80-day-old girl by extended end-to-end aortic arch reconstruction. She was admitted to our hospital at the age of 4 days because of poor pulsation of femoral arteries. The systolic blood pressure gradient between the arm and the leg was 30 mmHg. Echocardiography on admission revealed a simple coarctation and patent foramen ovale, with the mildly impaired left ventricular contraction (left ventricular fractional shortening was 23%). Although aortography demonstrated an isolated interrupted segment at the aortic isthmus with collaterals (type A classification of Celoria-Patton), the tubular connection between the distal arch and the descending aorta, of which intralumen was obstructed with abundant ductal tissues, was found at operation. The obstruction of the lumen of aortic isthmus in our case, which was originally patent, might be caused by ductal closure and present as a simple coarctation.     

Percutaneous transluminal angioplasty and stent placement for subclavian steal syndrome with concomitant anterograde flow in the left internal mammary artery graft for coronary artery bypass--case report

A 63-year-old man presented with subclavian steal syndrome associated with left internal mammary artery (IMA) bypass graft to a coronary artery. He was admitted with a history of oppressive sensation in the chest, dizziness, and light headedness on exertion for 2 weeks in March 2002. He had undergone myocardial revascularization consisting of a left IMA-to-left anterior descending coronary artery graft in April 1988. His blood pressure was 140/70 mmHg in the right arm and 80/64 mmHg in the left arm. Aortic arch arteriography revealed complete occlusion of the left subclavian artery proximal to the left IMA takeoff and subclavian steal with anterograde flow of the left IMA. Percutaneous angioplasty and stent placement with protection of the left IMA bypass graft using a balloon catheter was successfully performed without complication by cerebral or myocardial ischemia. Complete recanalization of the occluded left subclavian artery and anterograde flow of the left vertebral artery were achieved. His symptoms disappeared and blood pressure in the left arm recovered. This variant of coronary subclavian steal might require protection of the left IMA during angioplasty and stent placement.     

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.     

Surgical repair of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum

Five cases of right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum, with or without Kommerell's diverticulum, are presented. Either right or left thoracotomy with divesion of the aberrant retroesophageal left subclavian artery can be accomplished with good results. However, a right thoracotomy is recommended in the presence of a large Kommerell diverticulum because such a diverticulum should be excised. The development of a subclavian steal syndrome later in life may be prevented by reimplantation of the divided left subclavian artery into the aorta or the left common carotid artery.     

Implications of anomalous right subclavian artery in the repair of neonatal aortic coarctation

Spinal cord perfusion is predominantly from the anterior spinal artery, which arises from the vertebral arteries by way of the subclavian arteries. Anomalous origin of the right subclavian artery and coarctation of the aorta is considered to be an increased risk factor for spinal cord damage, possibly because of the minimal collateral circulation during aortic clamping. The aim of this study is to review 5 consecutive cases of neonatal aortic coarctation with ARSA. METHODS: Five neonates (0.8 to 4.6 kg) underwent operation between July 1999 and December 2000 with resection of the coarctation and end-to-end anastomosis. Both subclavian arteries (n = 5) and left carotid artery (n = 4) were clamped, leaving the right carotid artery as the sole provider of perfusion for the spinal cord. RESULTS: Despite clamping of both subclavian arteries, right radial artery pressure was measurable in 4 of the 5 cases. Aortic cross-clamp times varied from 12 to 26 minutes at a core temperature of 34 degrees to 35 degrees C. There was no operative mortality. None of the neonates developed any major neurologic sequelae. CONCLUSIONS: When clamping the two subclavian arteries during coarctation repair, the spinal artery is left with collateral blood flow that can theoretically originate from the carotid arteries through the circle of Willis and retrogradely down the vertebral arteries. The presence of such collateral circulation was documented as recordable blood pressure in the right radial artery during surgical repair.