Tetralogy of Fallot with double aortic arch: successful staged repair. Case report and review of literature

The rare association of DAA with TOF is reported in a young boy who underwent successfully at the age of 3 months an emergency shunt between the anterior (left) hypoplastic aortic arch and the LPA after the vascular ring was divided. Total correction of the cardiovascular malformation was performed at the age of 4 years. The cardiac catheterization, angiographic and operative findings as well as the management of the case are described. Ten other patients with this combination of anomalies were found on review of the literature, eight of whom had only palliative systemic to pulmonary artery shunts. Knowledge of the morphology of the DAA by angiocardiography is helpful in planning the management. Surgical shunts when done early for the treatment of a patient with repeated anoxic spells may reduce the gravity of psychomotor retardation. A staged surgical repair of TOF is recommended for infants having hypoplastic pulmonary arteries.     

Surgical treatment of patients with tetralogy of Fallot in combination with a double aortic arch

A late congenital anomaly--double arch of the aorta (DAA)--was combined with Fallot's tetrad (FT) in 4 patients whose ages ranged from 21 months to 11 years. The diagnosis of DAA and FT was established before the operation in 3 patients, in one patient DAA was discovered during the operation. In three children DAA was manifested clinically by dysphagia and stridor. A palliative operation (FT) was carried out through a left posterolateral approach along the third intercostal space. One-stage separation of the vascular ring by cutting the smaller left arch of the aorta at its junction with the descending aorta and formation of anastomosis between this arch and the left pulmonary artery by the end to side method were conducted. In deficient length of the cut arch of the aorta the left subclavian artery was divided; in equal diameter of both arches the lumen of the arch was reduced to 0.5 cm with stitches before formation of the anastomosis so as to prevent hyperfunction of the shunt. In the case in which DAA was not recognized before the operation, an attempt to dilate the hypoplastic segment of the left arch led to rupture of the thinned wall and the patient died from hemorrhage. The advantages of one- and two-stage radical correction of both developmental anomalies (DAA and FT) are still debatable.     

Diagnosis and tactics in the surgical treatment of double aortic arch

Within the period of from 1978 to 1989, in 19 patients staying in the clinic, a double aortic arch (DAA) was diagnosed by means of roentgenologic investigation, esophagography, angiography. In comparison of the findings of esophagography and angiography, in 2 patients, atresia, of the left arch segment was revealed, in 9 patients, a double arch was associated with Fallot's tetrad (FT), in 4--with the interventricular septum defect (IVSD). All the patients were operated on. In 6 patients with isolated DAA, the lesser arch was transected: in 5--the left, in 1--the right one. In 13 patients with associated DAA and congenital heart defect (CHD), the simultaneous DAA transection and a palliative operation (in 9), or a radical correction of CHD (in 4) were performed. Two patients with FT 3 years later, underwent the second stage successful radical correction of a defect. A long-term result of treatment is good.     

Long-term results of aortic arch replacement using combination of deep hypothermic circulatory arrest and retrograde cerebral perfusion; comparison of result for atherosclerotic aneurysm and aortic dissection

Between 1992 and 2001, 73 patients with aortic arch lesion underwent surgical treatment. The aneurysm was characterized by atherosclerosis in 54 patients (TAA) and by aortic dissection in 19 patients (DAA). All patients received graft replacement under combination of deep hypothermic circulatory arrest and retrograde cerebral perfusion. The hospital mortality rate for all patients was 15.6%. The follow-up period of 61 survival patients ranged from 2 to 90 months (mean 33.2 months). The actuarial survival rate was 93.2%, 83.5%, and 79.7% at 1, 3, and 5 years, respectively. No significant difference was noted between TAA and DAA in the actuarial survival rate. The freedom from vascular complication was 97.9%, 95.8%, and 95.8% at 1, 3, and 5 years, respectively. The rate was significant higher in the patients of DAA than in those of TAA (TAA: 100%, 100%, and 100%, DAA: 90.9%, 81.8%, and 81.8%). The freedom from cardiac complication was 96.4%, 89.7%, and 84.1% at 1, 3, and 5 years, respectively. There was no significant difference between TAA and DAA. The freedom from cerebrovascular complication was 100%, 97.6%, and 83.4% at 1, 3, and 5 years, respectively. No significant difference was noted between TAA and DAA. The late result of surgical treatment for the aortic arch using combination of deep hypothermic circulatory arrest and retrograde cerebral perfusion was satisfied. The long-term survival rate was higher in TAA patient than in DAA one. The possibility remained that fatal vascular complication affected the long-term survival rate.     

Abnormalities of the aortic arch in adults. A rare cause of tracheo-oesophageal compression. Apropos of 2 cases

Two cases of symptomatic aortic arch anomalies in adults are reported: a case of Neuhauser's ligamentum arteriosum with compressive retro-oesophageal diverticulum, and a case of double aortic arch revealed by postoperative tracheal compression. In both cases, surgical correction was made very difficult by the aneurysmal progression of the compressive vascular anomalies and resection of the thoracic aorta under cardiopulmonary by-pass was necessary. These therapeutic difficulties therefore highlight the differences between aortic arch anomalies in adults and those in infants which can be simply and effectively treated with no mortality in our experience. These observations argue in favour of systematic surgical correction of any aortic arch anomalies in infants, even when they are responsible for few symptoms.     

先天性心脏病合并右位主动脉弓的诊断与治疗

目的 探讨先天性心脏病合并右位主动脉弓的诊断与治疗。 方法 回顾性分析2012年1月1日至2013年4月1日北京儿童医院小儿心脏中心27例先天性心脏病合并右位主动脉弓行外科手术治疗患儿的临床资料,其中男20例,女7例;年龄（10.96±12.08） 个月,体重（7.70±3.13） kg。法洛四联症14例,室间隔缺损9例,动脉导管未闭1例;肺动脉吊带1例;单纯双主动脉弓2例;均合并右位主动脉弓。其中13例合并血管环畸形,包括迷走左锁骨下动脉畸形和双主动脉弓畸形。所有患儿均接受手术治疗,在矫治心内畸形的同时,进行左锁骨下动脉移植,矫治迷走左锁骨下动脉畸形,双主动脉弓主要是切断一侧非优势弓。 结果 围术期死亡3例,其中1例术后无法停体外循环死亡,1例未合并血管环的患儿术后7 d死亡,1例合并双主动脉弓患儿术后并发急性呼吸窘迫综合征（ARDS） 死亡;1例放弃治疗。随访23例,随访时间3～17个月,心脏超声心动图提示心内畸形矫治满意,恢复良好,双侧上肢动脉波动良好。 结论 对先天性心脏病合并右位主动脉弓患者行外科矫治时应慎重,术前尽量完善相关检查,选择增强CT、磁共振成像检查,明确诊断,制定手术计划。一期同期矫治血管环畸形及其它先天性心脏病,手术效果满意,近期效果良好。     

Surgical treatment of the ascending aorta aneurysm in Marfan's syndrome

Retrospective analysis of the treatment results was done in 115 patients with Marphan's syndrome, operated on for the ascending aorta aneurysm. In 100 patients the complete replacement of ascending aorta and aortal valve was performed, using valvecontaining conduit with reimplantation of coronary arteries ostium into vascular prostheses (operation according to Bentalla--De Bono), in 62 of them--in our modification; in 6--the aortal valve prosthesis and aneurysmorrhaphy, in 7--the aortal valve prosthesis and aneurysmal resection with external enveloping of ascending aorta using vascular prosthesis (Robicsek operation); in 2--the valvepreserving operation on aortal valve with replacement of ascending aorta using vascular prosthesis. General mortality in hospital was 13%, including that after aneurysmorrhaphy--50%, the Robicsek operation--14.3%, Bentala--De Bono--11%. Late results (in terms from 1 year to 17.5 years) were studied up in 84 patients. The author considers Bentalla--De Bono operation the method of choice for the ascending aorta aneurysm in patients with Marphan's syndrome. The introduction of modification, proposed by him, promotes prophylaxis of the bleeding occurrence in immediate postoperative period.     

Open aortic valvuloplasty in surgical treatment of associated heart diseases

The article presents indications to surgical correction of aortal stenosis and its variants in patients with rheumatic diseases combined with a lesion of other valves of the heart. The possibility to fulfill valve-saving plastic operations on the stenosed aortal valve was shown in 110 operations. The results obtained proved advantages of plastic operations over prostheses of the stenosed aortas without calcinosis universalis. Such operations allowed the intrahospital lethality to be considerably decreased. In later terms only two patients needed prostheses of the valve. The functional class was improved in the overwhelming majority of the patients.     

Short and long-term results of surgical correction of the aortal valve dysplasia

Nearest and long-term results of surgical treatment of 108 patients with dysplasia of aortal valve were analyzed. Necessity of individual approach to choice of method of surgical correction (prosthesis or valvuloplasty of aortal valve) depending on degree of dysplasia and/or secondary valvular pathology, it anatomic variants, presence of concomitant infectious endocarditis and age of patients is noted. This permitted to reduce hospital lethality from 8.8 to 3.9%. Survival in 1, 5 and 10 years after prosthesis and valvuloplasty of aortal valve was 92.3 +/- 3.3, 86.8 +/- 4.8 and 85.7 +/- 5.7% versus 100, 94.1 +/- 4.1, 94.1 +/- 4.1% respectively. Quality life in remote period was evaluated as good in 80.3% operated patients, as satisfactory--in 11.1% and unsatisfactory--in 8.6%.     

Echocardiography in the preoperative evaluation of vascular rings.

Vascular rings may produce tracheal and/or esophageal compression in infants and children. Traditionally recognized fluoroscopically, the exact anatomy of the ring and appropriate surgical correction are determined intraoperatively. The role of preoperative echocardiography was examined. Twenty patients with symptomatic vascular rings were evaluated preoperatively with echocardiography at this institution. Their ages ranged from 10 days to 11 years (mean, 17 months). There were 9 boys and 11 girls. Most (17/20) presented with respiratory symptoms in the first year of life, although in 3 patients dysphagia was the primary complaint (at birth, 4 months, 9 years). All underwent initial evaluation with a barium esophagogram prior to the echocardiogram. Surgical correction was subsequently performed and the exact anatomy identified. All barium esophagograms were interpreted prospectively as demonstrating a "vascular ring." Although often suspected fluoroscopically, the actual type of ring was correctly identified by echocardiogram in all cases including determination of the dominant arch and associated anomalies. The types of vascular rings included double aortic arch (10), right aortic arch with left ligamentum arteriosum and/or aberrant left subclavian artery (6); aberrant right subclavian artery (2), and pulmonary artery sling (2). Barium esophagogram remains the best screening test for children in whom a vascular ring is suspected. However, echocardiography is a useful noninvasive complementary examination to confirm the diagnosis, clarify anatomy, and exclude other major intracardiac pathology prior to surgical correction.     

Post-operative Outcomes for Vascular Rings: A Systematic Review and Meta-analysis

ObjectivesVascular rings may cause tracheal and/or oesophageal compression. For many patients, symptoms/signs have been present for a long period before diagnosis. However, in the era of prenatal diagnosis, some units advocate universal early surgery. The risks and efficacy of surgery must be known to adequately counsel for the operation. This meta-analysis sought to define the morbidity and mortality associated with surgical correction, and persistent post-operative symptoms.MethodsPubMed, Cochrane Library and CINAHL databases were searched for studies that described the outcome of patients undergoing surgery for a double or right aortic arch (DAA or RAA). Non-comparative and random effects model-based meta-analyses were conducted to calculate the pooled rates of mortality, surgical complications, reintervention, and persistent follow-up symptoms.ResultsNineteen eligible studies were included comprising 18 studies describing outcomes for DAA surgery and 15 for RAA surgery. For DAA surgery, overall mortality rate was 0% [95% confidence interval (CI) 0.0–1.0], post-surgical complication rate 18% [95% CI: 12.0–23.0], prevalence of reintervention 3% [95% CI: 1.0–5.0] and prevalence of symptoms at last follow-up was 33% [95% CI: 17.0–52.0]. For RAA surgery, overall pooled mortality was 0% [95% CI: 0.0–0.0], prevalence of post-surgical complications was 15% [95% CI: 8.0–23.0], reintervention rate was 2% [95% CI: 0.0–4.0], prevalence of symptoms at last follow-up was 40% [95% CI: 26.0–55.0].ConclusionsWhile surgery to correct a vascular ring is safe, the rate of persistent symptoms is high and further strategies must be sought to reduce this burden.     

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.     

Antegrade endovascular repair of a coarctation-associated aneurysm through an upper hemi-sternotomy

Late aneurysm formation is a well-described complication after surgical correction of aortic coarctation. Endovascular repair of such aneurysms avoids the morbidity of conventional reoperative thoracic surgery. We describe a unique case of antegrade endovascular repair of a distal coarctation-associated aneurysm with vascular access acquired through the aortic arch by an upper hemi-sternotomy.     

Fully supported open stent grafting applied with a Matsui-Kitamura (MK) stent in treatment of distal arch aneurysm

Objective Our purpose was to examine the use of fully supported open stent grafting (OSG) with a Matsui-Kitamura (MK) stent for treatment of distal arch aneurysm (DAA). Methods Surgery was performed using a newly developed device in seven DAA patients (six men and one woman) from 58 to 86 years of age (mean, 73 years old) from August 2005 to June 2007. The aorta was transected at the arch between bracheocephalic artery and left subclavian artery under circulatory arrest with total cardiopulmonary bypass and selective cerebral perfusion; then the stent grafting (SG) system was inserted and positioned with a 14 Fr. pusher. The SG and a transected edge were then sutured and anastomosed with the arch graft. The surgery was completed by constructing three cerebral branches. Results The mean SG diameter and length were 34.6 mm (range, 32–38 mm) and 150 mm (120–200 mm), respectively. The mean time of circulatory arrest time and surgery were 41 min (35–55 min) and 358 min (269–450 min), respectively. Simultaneous mitral valve replacement was performed in one patient. All surgeries were completed successfully, and complete thrombosis of the aneurysm was obtained. Paraparesis and respiratory failure occurred in one patient each, and one patient died of brainstem infarction 1 month after surgery. Conclusion These initial results suggest that the OSG method is a useful surgical procedure for the treatment of DAA.     

Surgical treatment of small aneurysms of the ascending aorta by the method of plasty and bandage

On the basis of cardio-vascular department of the Saint Petersburg Medical academy of postdiploma education the authors made an analysis of surgical treatment of 23 patients with small (45-55 mm) aneurysms of the ascending portion of the aorta. Mean age of the patients by the time of operation was 55 years (from 35 to 75 years). The causes of the disease were as follows: poststenotic dilatation of the aorta--in 15 patients, atherosclerosis--in 5 patients, degenerative media pathology--in 3 patients. Critical aortal stenosis was revealed in 15 patients, 12 of them had insufficiency of the aortal valve. All the patients were subjected to longitudinal plasty of the aneurysm and banding with a vascular prosthesis, under conditions of mild hypothermic chemical cardioplegia. Good results were followed up for 5-10 years.     

Aneurysm of right-sided cervical arch: surgical removal and graft replacement.

Right-sided cervial aortic arch is a rare congenital anomaly which may cause respiratory symptoms or dysphagia. In the past, surgical correction of the cervical arch has not been attempted except in one patient in whom an erroeous diagnosis of aneurysm of the innominate artery led to an unsuccessful operation. A case is reported of a 39-year-old woman with an extensive fusiform aneurysm of a right-sided cervical arch. The arch aneurysm was successfully removed surgically and normal circulation was restored to the arch vessels. At the same operation, a ventricular septal defect was repaired with the aid of temporary cardiopulmonary bypass.     

Atherosclerotic aneurysm in the circumflex retroesophageal right aortic arch

A 63-year-old man had a distal arch aneurysm combined with a circumflex retroesophageal right aortic arch, which is a rare form of vascular ring. By using combined median sternotomy and left thoracotomy under deep hypothermia and retrograde cerebral perfusion, total arch replacement with a vascular graft was successful. We describe our surgical strategy for arch aneurysm associated with an uncommon congenital vascular malformation.     

Congenital vascular rings: surgical management of 111 cases

Between October 1974 and May 1987, 111 congenital vascular rings were submitted to surgical correction. There were 83 infants (age: 5 days-12 months, median: 7 months; weight: 1.9-8.5 kg, median: 7.1 kg), and 28 children (age: 1-13 years, median: 3.5 years; weight: 7.5-48 kg, median: 26.5 kg). Patients were divided into five groups: (1) double patent aortic arch (44 cases), (2) double aortic arch with atresia in different parts of the left arch (36 cases), (3) right aortic arch with left ligamentum arteriosum (21 cases), (4) left aortic arch with aberrant right subclavian artery and truncus caroticus (8 cases), and (5) pulmonary artery sling (2 cases). We had no intraoperative mortality but in the postoperative period, 2 neonates died of severe bacterial infections of the respiratory tract.     

Surgical treatment of giant aneurysms of ascending parts and arch of the aorta in condition of deep hypothermia and circulatory arrest with artificial assisted circulation

Sternotomy in giant (10 cm and more in diameter) aneurysms is highly dangerous due to possibility of injury of aneurysmatic wall with fatal bleeding. That is why sternotomy in condition of artificial circulation (AC) and cooling of patients to 29-30 degrees C are preferred. Two cases of successful surgical treatment of critical patients with giant aneurysms of ascending parts and arch of the aorta in condition of femoro-femoral AC, hypothermia (20 degrees C) and circulatory arrest are presented. One of the patients had a giant false posttraumatic aneurysm of an ascending part of the thoracic aorta with fistula between aneurysm and pulmonary artery. Suturing of defects of ascending aorta and pulmonary artery wall, aneurysmorrhaphia of pulmonary artery were performed. The other patient with acute disruption of a giant dissected aortal aneurysm and hemomediastinum underwent prosthesis of ascending part and arch of the aorta.     

Adult aortic arch atresia.

Atresia in the aortic arch is a rare and severe congenital cardiovascular anomaly. Without surgical therapy, only a few patients can survive to adulthood. A 29-year-old woman with atresia of the aortic arch (Celoria-Patton Type A) without any intracardiac shunt underwent primary surgical correction involving reconstruction of the aortic arch with prosthetic interposition between the transverse aortic arch and the descending aorta and division of the persistent ductus arteriosus. Her postoperative course was uneventful, and she is now doing well at three years to date after surgical treatment.