Surgical treatment of interrupted aortic arch with extraanatomical bypass simultaneous to coronary artery bypass grafting and aortic valve replacement

An interrupted aortic arch accompanied by further surgically reparable cardiac lesions is a rare combination in adult patients. We describe treatment of an interrupted aortic arch, coronary artery bypass grafting (CABG), and aortic valve replacement (AVR) performed simultaneously through median sternotomy in a 64-year-old man. The patient underwent surgery performed using standard cardiopulmonary bypass with cannulation of the ascending aorta and the right atrium, hypothermia (24.6degreesC), and blood cardioplegic arrest. Four aortocoronary vein grafts and pericardial aortic valve replacement were carried out. Finally, the posterior pericardium was opened, and a 16-mm prosthesis was anastomosed to the descending aorta during side clamping using a 4-0 monofilament continuous suture. Optimal placement of the prosthesis was obtained by guiding it to the ascending aorta laterally to the right atrium and passing it between the inferior vena cava and right inferior lung vein. The operation was carried out without complications, and the postoperative course was uneventful. Magnetic resonance imaging showed competent aortic valve prosthesis and highly decreased collateral flow via the internal mammary arteries. Postoperatively both inguinal pulses were present, and the patient was free of angina. In the presence of an interrupted aortic arch, extraanatomical bypass via the posterior pericardium between the ascending and descending aorta can safely be performed at the same time as CABG and AVR through a median sternotomy.     

A case of tracheo-bronchial stenosis after extended end-to-end aortic arch anastomosis for interrupted aortic arch treated with suspension of the ascending artery and pulmonary artery

A 9-day-old boy had pulmonary artery banding and extended end-to-end aortic arch anastomosis for ventricular septal defect (VSD) and type A interrupted aortic arch. Severe dyspnea gradually developed. At 3 months of age, intracardiac repair of VSD was performed. Weaning from the ventilator was difficult. Endoscopic examination and chest CT revealed stenosis of the right and left main bronchi and compression of tracheal bifurcation and the right and left main bronchi by the ascending aorta and pulmonary artery. Suspension of the ascending aorta and pulmonary artery was performed 15 days after VSD closure. Nine days after this procedure, the patient was weaned from respirator. Postoperative course was uneventful. Bronchial stenosis may be caused from extended end-to-end aortic arch anastomosis.     

Right aortic arch with mirror-image branching and coarctation of the aorta--a case report]

The right aortic arch with coarctation of the aorta was reported. A 56-year-old woman admitted to the hospital because of headache and hypertension. Cardiac catheterization revealed the right aortic arch with coarctation of the aorta and 80 mmHg pressure gradient across the coarctation. The bypass operation with a 14 mm Dacron graft between the ascending to descending aorta was performed. There was no peak systolic pressure gradient between the ascending and descending aorta after bypass operation. This patient is the fourth case report with both mirror-image type right aortic arch and coarctation of the aorta.     

Off-pump aortic arch repair through a median sternotomy for type B interrupted aortic arch with single ventricle physiology

BACKGROUND: The aortic arch repair for interrupted aortic arch (IAA) with the hypoplastic ascending aorta through a median sternotomy requires cardiopulmonary bypass (CPB), which is very invasive in neonates and complicates pulmonary artery banding (PAB) is staged repair. METHODS: A 22-day-old neonate with a type B IAA having a functional single ventricle underwent arch repair and PAB through a median sternotomy without CPB. A partial occlusion clamp could be placed on the ascending aorta without cerebral malperfusion and the descending aorta could be directly anastomosed to the ascending aorta in an end-to-side fashion under stable circulatory condition. Thereafter, the tight PAB was performed with a circumference of 23mm without any difficulty. RESULTS: The postoperative echocardiogram revealed no stenosis on the anastomotic site and the patient was discharged uneventfully. CONCLUSION: This approach is effective in neonates with IAA who require staged repair, and least invasive for them.     

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.     

Total aortic arch aneurysm repair using a stent graft without cardiac or cerebral ischemia

A 74-year-old man with an aortic arch aneurysm and a chronic type IIIb aortic dissection underwent total aortic arch repair without cerebral or cardiac ischemia. After confirming no atheromatous change in the ascending aortic wall, a custom-designed 4-limbed graft, prepared for both arterial return of cardiopulmonary bypass and reconstruction of the arch vessels, was anastomosed onto the right side of the ascending aorta. The 3 arch vessels were then bypassed sequentially during systemic cooling and monitoring cerebral perfusion with near-infrared oxymetry. After aortic cross-clamping, a stent graft was inserted into the distal arch from the distal ascending aorta, maintaining cerebral and cardiac perfusion. This procedure is indicated especially in a high-risk patient who has an aortic arch aneurysm without severe atheromatous change in the ascending aorta and the arch vessels.     

Acute Type A Dissection Treated with Combined Endovascular Repair of Arch and Surgical Bypass of Arch Vessels from Descending Aorta

We report a case of an endovascular repair of a recurrent dissecting aneurysm of the aortic arch and dissection of carotid vessels, 3 years after surgical repair of aortic valve and ascending aorta for a type A dissection. We performed a bypass from the descending aorta to right, left common carotid artery (CCA), to left subclavian artery with no cardiopulmonary bypass and thereafter, total ascending and aortic arch stent grafting. We suggest considering total aortic arch stent grafting with bypass of arch vessels in cases of complicated acute type A dissection. In cases where the ascending aorta cannot be used as donor site for bypass, we suggest the use of the descending aorta.     

Off-Pump Extraanatomic Bypass from the Ascending to the Descending Aorta for Re-Operation of Interrupted Aortic Arch in an Adolescent

Abstract   The appearance of re-stenosis after repair of an interrupted aortic arch may be a surgical challenge due to adhesions. Here, we describe an approach using off-pump coronary artery bypass grafting techniques to reach the descending aorta through a median sternotomy in a patient with aortic arch stenosis after conduit repair. The 17-year-old patient with diagnoses of interrupted aortic arch and ventricular septal defect presented after two previous operations (one left lateral thoracotomy and one median sternotomy) with a stenosed vascular graft between ascending and descending aorta. Surgery was done via re-sternotomy without cardio-pulmonary bypass. An extraanatomic graft was used to connect ascending and descending aorta. When performing the distal anastomosis, the heart was exposed using a standard suction device. This case demonstrates that the use of modern techniques may facilitate surgical approaches dramatically. In our opinion the above-described technique is the first choice for all patients requiring arch repair following multiple previous operations, performed via sternotomy and thoracotomy.     

Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.     

Surgical management of patients with interrupted aortic arch and severe subaortic stenosis

Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 +/- 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 +/- 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 +/- 0.03 and 0.56 +/- 0.06, respectively, compared with 0.81 +/- 0.12 and 0.95 +/- 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction (p less than 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.     

Successful complete repair of interrupted aortic arch and associated with DiGeorge syndrome in neonate]

We report a rare case of interrupted aortic arch and a right aortic arch associated with DiGeorge syndrome, in neonate. Through a median sternotomy bypass was established placing an arterial perfusion cannula both in the ascending aorta, and in the main pulmonary artery. The right and left pulmonary arteries were temporarily occluded, while this pulmonary cannula perfused the lower part of the body. The arch reconstruction was performed during profound hypothermic total circulatory arrest. The right descending aorta had an adequate length and direct anastomosis was carried out without any tension. The VSD was repaired through a right atrial approach. The patient had hypocalcemia and thymic abnormalities which was consistent with the DiGeorge syndrome. He was treated with calcium gluconate and alfacalcidol, but no serious infection due to immunodeficiency was seen after operation. Post operative catheterization revealed no pressure gradient at the site anastomosis of the aortic arch and satisfactory results.     

Reverse subclavian flap and aorto-pulmonary window technique for repair of interrupted aortic arch and truncus arteriosus

We report a surgical strategy for repairing an interrupted aortic arch (IAA) with truncus arteriosus (TA) by using a reverse subclavian flap and an aorto-pulmonary (A-P) window technique for preserving the pulmonary artery architecture. A 10-day-old neonate with type B IAA and type I TA with echocardiographic evidence of a significant distance between the ascending and descending aorta underwent surgical repair at the Bristol Royal Hospital for Children. The superior part of the arch was reconstructed using a reverse subclavian flap and the undersurface with a pulmonary homograft patch. The ascending aorta was separated from the pulmonary arteries using a Gore-Tex patch (A-P window type of repair) without disconnecting the branch pulmonary arteries, in order to preserve their architecture. The continuity between the right ventricle and the pulmonary artery bifurcation was established using a 12 mm Contegra conduit. The postoperative course was uneventful, and the neonate was discharged after 12 days. At follow-up, the patient remains well, gaining weight, with no echocardiographic evidences of obstruction. Reverse subclavian flap with homograft patch combined with and 'A-P window' technique for preservation of the pulmonary artery architecture is a useful and effective surgical strategy for neonates presenting with IAA associated with TA.     

经颈动脉腔内技术重建主动脉弓治疗Stanford A型夹层动脉瘤(附1例报告)

目的探讨血管腔内技术重建主动脉弓治疗升主动脉、主动脉弓病变的可行性。方法2005年,对1例StanfordA型夹层动脉瘤,腔内修复主动脉病变之前做右颈总动脉-左颈总动脉-左锁骨下动脉的旁路术;经右颈总动脉将修改的分叉支架型血管主体放入升主动脉,长臂位于无名动脉。短臂应用延长支架型血管延伸至降主动脉。通过腔内技术重建主动脉弓实现累及升主动脉和主动脉弓主动脉病变的微创治疗。结果腔内修复术后移植物形态良好,血流通畅,病变被隔绝,脑、躯干、四肢循环稳定。无严重并发症。结论该手术方案设计合理、技术可行。可能成为复杂胸主动脉病变新的腔内治疗模式。     

Double aortic arch with aneurysm-a surgical case report.

An unusual case of double aortic arch with aneurysm is described. A 61-year-old, hypertensive and syphilitic male patient complained of increasing difficulty in swallowing of approximately five months duration. Barium swallow and subsequent aortography showed the presence of an aneurysm of the ascending aorta involving the left (anterior) arch of the double aortic arch. The tightening of the vascular ring by aneurysmal dilatation of the left aortic arch appeared to be responsible for the delayed onset of the symptom. Surgical correction was made by removing the aneurysmal ascending aorta and left aortic arch and a Dacron graft was inserted between the ascending aorta and the right (posterior) aortic arch. The patient died of cerebral complication about forty hours postoperatively. Surgical problems associated with this unusual condition were discussed retrospectively from the operative and autopsy findings. In reviewing the pertinent literatures this case appeared to be the first operated case of double aortic arch associated with aneurysm.     

Transcarotid artery endovascular reconstruction of the aortic arch by modified bifurcated stent graft for Stanford type A dissection

A 40-year-old man with Stanford type B dissection underwent his first endovascular repair (EVAR) in April 2004 by Talent thoracic stent graft. He had an uncomplicated recovery and maintained good blood pressure control. However, a new retrograde dissection appeared in September 2004. The new dissection involved his aortic arch and ascending thoracic aorta to the opening of the coronary arteries. To reconstruct the aortic arch, bypasses between the right common carotid artery (RCCA), left common carotid artery and left subclavian artery were performed before endovascular repair. A modified bifurcated Talent stent graft was deployed from the RCCA to the ascending thoracic aorta with a long limb in the innominate artery and a short limb in the aortic arch. A further two pieces of graft were deployed via the common femoral artery. The ascending thoracic aorta and aortic arch were reconstructed completely by the bifurcated stent graft. The final angiography confirmed that there was good stent graft configuration, normal blood flow, and stable haemodynamics. No endoleak or other major complications were encountered. This result indicated that it is possible to reconstruct the aortic arch with a bifurcated stent graft and could be a new endovascular repair model for complex thoracic aortic aneurysm and dissection.     

Neonatal repair of right interrupted aortic arch with cerebro-myocardial perfusion technique

Right interrupted aortic arch and descending aorta is exceedingly rare and most likely cause respiratory presentation, since patent ductus arteriosus (PDA) courses over the right mainstem bronchus. We report a case of successful neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant right subclavian artery ventricular septal defect (VSD) in a 2.7?kg term neonate with DiGeorge syndrome. Patient presented in severe respiratory distress and acidosis at one day old. Two-dimensional (2D) echocardiography revealed aortic arch interruption beyond the common carotid arteries with large perimembranous outlet VSD. Aortic annulus diameter was 4.8?mm and there was no left ventricle (LV) outflow tract obstruction. Three-dimensional (3D) CT-scan confirmed these findings and identified a right-sided ductal arch that continued over the right mainstem bronchus into a right-sided descending aorta and aberrant right subclavian artery. Brachiocephalic perfusion and ductal perfusion was employed for cooling during cardiopulmonary bypass. Under deep hypothermia (27 °C rectal temperature), selective cerebro-myocardial perfusion was used for successful aortic arch repair without sacrificing the aberrant right subclavian artery. A direct tension-free anastomosis was attained. Her postoperative course was uneventful and her respiratory symptoms disappeared postoperatively. Early surgical correction is mandatory for these patients with unique anatomy and presentation.     

Truncus arteriosus with interrupted aortic arch: successful correction in a neonate

Truncus arteriosus (type II) with interrupted aortic arch (type B) was successfully repaired at 11 days of age using anterior translocation of the pulmonary arteries, resection of the ductus arteriosus, and direct anastomosis between the descending aorta and truncus. This technique permitted wide reconstruction of the aortic arch and minimized the number of suture lines. It also positioned the right ventricle-pulmonary artery conduit anteriorly, which may simplify its subsequent replacement.     

Isolated myocardial perfusion during arch repair

Repair of coarctation of the aorta with severe hypoplasia of the aortic arch or interrupted aortic arch was performed in 5 patients using a modification of the usual technique that consisted of isolated myocardial perfusion during arch repair. The aortic cross-clamp was placed on the ascending aorta distal to the aortic cannula. Cardiopulmonary bypass flow was reduced to about 10% of full flow, achieving a line pressure of 35 to 45 mm Hg to keep the heart perfused and beating during arch repair. Once the aortic arch was repaired, total body perfusion was continued as usual and intracardiac repair was performed. Isolated myocardial perfusion for aortic arch reconstruction reduces myocardial ischemic time.     

A combination of reoperation for pseudoaneurysm following the Cabrol procedure and total aortic arch replacement in a patient with Marfan syndrome —A case with an aberrant right subclavian artery—

A 44-year-old male with Marfan’s syndrome had undergone an initial operation for DeBakey type I acute aortic dissection with annulo-aortic ectasia. He had undergone replacement of the ascending aorta and aortic valve with a composite graft and reconstruction of the coronary artery by the Cabrol procedure. At 5 years after the initial surgery he experienced chest pain and was subsequently examined. Computed tomography revealed a pseudoaneurysm in the ascending aorta and the residual aortic dissection. The maximum diameter of the pseudoaneurysm was 85 mm and the maximum diameter of the aortic arch was 55 mm. The aortic arch was associated with an aberrant right subclavian artery. Angiography revealed that the pseudoaneurysm was caused by leakage at the coronary ostium-graft anastomoses. We repaired the anastomoses and performed total aortic arch replacement with reconstruction of four arch branches. The postoperative course was uneventful without any complications. We report this case because there have been few reports regarding arch replacement in cases with an aberrant right subclavian artery.     

A surgical case of supravalvular aortic stenosis with severe hypoplastic ascending aorta (diffuse type) in Williams-Beuren syndrome

We report a six-year-old boy who underwent ascending aortic reconstruction for supravalvular aortic stenosis of diffuse type associated with Williams-Beuren syndrome. The diagnosis was first made at the age of six months. Because of progressive left ventricular hypertrophy, cardiac catheterization was performed at the age of five years and showed left ventricular pressure of 200 mmHg, the ascending aortic pressure of 202 mmHg, the descending aortic pressure of 115 mmHg, and left ventricular end-diastolic volume of 33.5 ml (90% of normal). Whole ascending aorta except sinus Valsalva was severely hypoplastic, so called diffuse type of supravalvular aortic stenosis. The ascenidng aorta was only 6.3 mm in diameter, whereas the diameter of the aortic annulus was 20.6 mm. The ascending aortic reconstruction of Doty’s type was performed from the aortic root to the distal aortic arch with a equine pericardium reinforced by Dacron velour using selective perfusion to the right bracheocepharic artery, the left common carotid artery and the right femoral artery to avoid circulatory arrest. Postoperative course was uneventful and post operative catheterization revealed left ventricular pressure of 128 mmHg, the ascending aortic pressure of 126 mmHg and the descending aortic pressure of 90 mmHg. Mild residual pressure gradient was probably due to hypoplastic descending aorta. In conclusion, patch aortic reconstruction for ascending aorta under selective cerebral perfusion for a six-year old boy can be performed without postoperative neurological complication.