分期介入治疗主动脉缩窄合并右锁骨下动脉瘤1例

主动脉缩窄(CoA)是指主动脉局限狭窄,管腔缩小,造成血流量减少.主动脉缩窄可以单独出现,也可以合并二叶式主动脉瓣、室间隔缺损、动脉导管未闭、大动脉转位等病变.主动脉狭窄合并右锁骨下动脉瘤非常罕见,本文报导一例主动脉缩窄合并右锁骨下动脉及降主动脉瘤,一期应用定制的Armada 35球囊扩张导管及覆膜支架治疗主动脉狭窄,...     

Reverse subclavian flap repair of hypoplastic transverse aorta in infancy

BACKGROUND: Management of hypoplastic aortic arch associated with coarctation in infancy can be challenging. Reverse subclavian flap aortoplasty plus coarctation resection offers simplicity without needing foreign material or cardiopulmonary bypass. METHODS: Since 1988, 46 of 162 infants less than 3 months undergoing coarctation repair had hypoplastic arch enlargement with reverse subclavian flap aortoplasty. Median age was 11 days; mean weight was 3.2 kg. Thirty-seven patients (80%) had associated cardiac defects including single or multiple ventricular septal defects (14 infants), transposition of the great arteries (7), aortic or mitral stenosis (5), and complete atrioventricular septal defect (5 infants). Twenty-eight patients had pulmonary artery banding; 2 had an arterial switch operation through a separate median sternotomy. RESULTS: There were two hospital deaths: one 4 months postoperatively in a patient requiring a Norwood procedure the next day for underestimated left ventricular hypoplasia; the other of sepsis more than 1 month postoperatively. On follow-up from 1 to 129 months (mean, 38 months), there were five recurrent obstructions: three at the coarctation site treated with balloon dilatation and two at the arch site. Twenty-six children had their heart defects corrected with 29 subsequent operations including an arterial switch operation for transposition of the great arteries/ ventricular septal defect (3 infants), relief of aortic or mitral stenosis +/- ventricular septal defect closure (5), multiple ventricular septal defect closure (3), a bidirectional Glenn (2), complete atrioventricular septal defect (2), and anomalous left coronary with ventricular septal defect repair (1 infant). Four children await debanding and ventricular septal defect closure or Glenn anastomosis. There have been two late deaths (overall survival, 91%). CONCLUSIONS: Reverse subclavian flap aortoplasty is excellent for relief of arch hypoplasia and coarctation in infants with low recurrence rates and acceptable operative and intermediate survival.     

Aberrant right subclavian artery with preductal coarctation of the aorta

A 9-day-old boy diagnosed with an aberrant right subclavian artery, a preductal coarctation, and a ventricular septal defect underwent a subclavian flap coarctectomy. Subsequently, he underwent surgical closure of the ventricular septal defect at the age of 11 months. This case is extremely rare with regard to having an aberrant right subclavian artery originating from the proximal site of a preductal coarctation of the aorta.     

Transpericardial extra-anatomic intrathoracic aortic bypass conduits in the management of coarctation of the aorta

Two varied cases of coarctation of the aorta are described, in which transpericardial ascending to descending thoracic aortic conduits were routed around the right atrium, utilizing deep hypothermic circulatory arrest. The approach was through a median sternotomy in two patients. The first case is a 52-year-old female who underwent combined aortic valve replacement for aortic stenosis and repair of aortic coarctation distal to the left subclavian artery. The second case was a fifth time reoperation in a 12-year-old girl with recurrent coarctating. Both patients remain well and asymptomatic.     

Successful repair of a coarctation complex with an anomalous right subclavian artery in an infant

A 46-day-old infant weighing 2250 g with the coarctation of the aorta, ventricular septal defect, PDA, the anomalous right subclavian artery, and the persistent left superior vena cava initially underwent by the reversed subclavian flap aortoplasty with the anomalous right subclavian artery and pulmonary arterial banding. The reversed subclavian flap aortoplasty was useful for the coarctation complex with the anomalous right subclavian artery. Two months later he gained weight to 2500 g, and then VSD closure and debanding of PAB was performed successfully. This two-staged operation was recommended for the poor risk coarctation complex with the anomalous subclavian artery.     

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.     

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.     

Bilateral subclavian steal associated with severe coarctation of the thoracic aorta and an aberrant right subclavian artery

A 10-year-old girl having bilateral subclavian steal associated with severe coarctation of the thoracic aorta and an aberrant right subclavian artery was found, on admission, to have no difference between upper and lower extremity blood pressure, but echocardiography revealed severe thoracic aorta coarctation and systolic blood pressure in the carotid arteries exceeding 200 mmHg estimated by Doppler ultrasonography. Magnetic resonance imaging and angiography demonstrated bilateral subclavian steal without esophageal compression. We reconstructed the aortic arch using the left subclavian artery and a reversed Blalock-Park procedure, then repaired the coarctation with a 14 mm woven double velor vascular graft. The girl was symptom-free following uncomplicated recovery from surgery. Doppler ultrasonography 2 weeks after surgery showed the pressure gradient across the aortic arch had decreased from 180 mmHg to 60 mmHg. This residual gradient at the anastomosis between the ascending aorta and left subclavian artery may improve as native vessels grow.     

Cervical aortic arch with retroesophageal aortic obstruction: report of a case with successful surgical intervention

The clinical, roentgenographic, hemodynamic, and angiographic features of a patient with a right cervical aortic arch and retroesophageal aortic obstruction associated with a ventricular septal defect are presented. Surgical relief of the aortic obstruction was successfully achieved by placement of a bypass graft between the left common carotid artery and the descending thoracic aorta.     

Conversion of lusoric artery into right subclavian artery in one-stage neonatal repair of aortic arch anomalies and intracardiac defects.

In 2 patients, a lusoric artery was compressing the mediastinal structures during a one-stage repair of type B aortic arch interruption and ventricular septal defect. In order to prevent the long-term complications of sacrifice of the subclavian artery, the lusoric artery was not simply divided but converted into a regular right subclavian artery by re-implantation of the abberant vessel at the origin of the right carotid artery during the one-stage repair. At follow up, both patients have normal right radial arterial pulsations. In one patient, angiography was repeated and confirmed a well functioning right subclavian artery. Conversion of a lusoric artery into a right subclavian artery during one-stage repair of aortic arch anomalies and intracardiac defects is not only feasible but also indicated to preserve subclavian artery function and to prevent compression of the mediastinal structures by a vascular ring.     

Interrupted left aortic arch and isolated right subclavian artery from the right pulmonary artery

We report a 10-day-old newborn, weighing 2.9 kg with an interrupted left aortic arch type B, a large subarterial ventricular septal defect and a right ductus connecting the right pulmonary artery to an isolated right subclavian artery. The patient underwent successful total surgical repair and the isolated right subclavian artery was ligated. He was discharged from hospital without complication and maintains excellent perfusion to the right arm via collaterals.     

主动脉缩窄或主动脉弓中断合并心内畸形一期修复

目的 探讨采用经胸骨正中切口一期修复主动脉缩窄(CoA)或主动脉弓中断(IAA)合并心内畸形的治疗效果.方法 2002年7月至2009年6月,经胸骨正中切口行降主动脉远端和主动脉弓下缘端侧吻合术一期修复CoA或IAA合并心内畸形病儿43例,其中CoA 34例,IAA 9例(A型6例、B型3例),合并心内畸形包括室间隔缺损42例、动脉导管未闭34例、房间隔缺损12例、主动脉瓣下隔膜狭窄5例、二尖瓣关闭不全2例,右心室双出口1例.结果 手术死亡1例,为术后肺动脉高压和严重低心排血量综合征者.术后并发症包括严重低心排血量综合征3例,低氧血症6例,肺部炎症11例,肺不张14例,声音嘶哑19例,室上性心动过速23例.8例失访.34例随访3个月～5年,生活质量明显改善,心脏超声心动图和CT检查显示吻合口无明显再缩窄发生.结论 经胸骨正中切口,采用主动脉远端和主动脉弓下缘端侧吻合技术一期修复CoA或IAA合并心内畸形的手术早、中期效果良好,能明显减少术后再狭窄.     

Anatomic correction of transposition of the great arteries with ventricular septal defect. Experience with 118 cases

One hundred eighteen patients, 100 with transposition of the great arteries plus ventricular septal defect and 18 with double-outlet right ventricle and subpulmonary ventricular septal defect have undergone arterial switch and patch closure of the ventricular septal defect since February 1983. In transposition of the great arteries the ventricular septal defect was perimembranous in 70 cases, trabecular in 28, and infundibular in 10. Eleven patients had multiple ventricular septal defects. In addition to 18 patients with double-outlet right ventricle, malalignment of the conal septum was present in 19 cases. Coronary type A distribution was recognized in 79 cases, type C in one, type D in 24, and type E in 14. Great arteries were side by side in 19% of cases. Aortic coarctation was present in 31 cases, and subaortic obstruction in 9. Age at operation ranged from 4 days to 4 years (mean, 3.5 +/- 8.3 months), and mean weight was 4.0 +/- 1.6 kg. Thirty-seven infants were younger than age 1 month. Thirty-six patients underwent previous operations: pulmonary artery banding alone (n = 13), pulmonary artery banding and coarctation repair (n = 13), and coarctation repair alone (n = 10). Mean time between the first procedure and the switch was 2.2 months. Six patients with aortic coarctation underwent one-stage repair, through median sternotomy, aortic reconstruction, closure of the ventricular septal defect, and arterial switch. Perioperative mortality was 13.5% (70% confidence limit 10% to 17.6%, n = 16). It was directly related to coronary artery kinking in 50% of deaths and to anatomy and size discrepancy of the great arteries in the remaining deaths. Univariate analysis could not find any significant risk factor of in-hospital mortality. Mean follow-up of 30.3 +/- 23.5 months was achieved in all but 2 survivors. There was one late death. Ten patients underwent 11 reoperations for recoarctation (n = 1), pulmonary stenosis (n = 7), residual ventricular septal defect (n = 2), and stenosis of superior vena cava (n = 1). Two patients needed a permanent pacemaker. Actuarial survival and freedom from reoperation at 5 years were 84.5% +/- 3.6% and 85.7% +/- 4.6%, respectively. We conclude that anatomic correction of complex transposition is a safe method that offers good early and midterm results.     

升主动脉-降主动脉人工血管旁路移植术在复杂主动脉缩窄中的应用5例

目的探讨正中开胸行升主动脉-降动脉人工血管旁路移植术治疗复杂主动脉缩窄的手术细节。方法 5例复杂型主动脉缩窄患者正中开胸行升主动脉-降主动脉旁路手术,其中4例行膈肌上吻合(其中1例经左心缘路径,3例经右心缘路径),1例行膈肌下吻合。分别观察术后上下肢压差及并发症情况。结果 5例患者均无死亡,术后上下肢压差均降低至20 mmHg以下,恢复可,术后并发症包括咳嗽1例及黑朦2例。结论正中开胸行升主动脉-降动脉人工血管旁路移植术治疗主动脉缩窄效果可,但手术细节对术后并发症的影响仍需进一步研究。     

Left-sided lesions after anatomic repair of transposition of the great arteries, ventricular septal defect, and coarctation: surgical factors

OBJECTIVE: This study was undertaken to identify potential anatomic and surgical factors creating left-sided lesions, namely recoarctation of the aorta and neoaortic regurgitation, after anatomic repair of transposition of the great arteries with ventricular septal defect and aortic coarctation. METHODS: From 1983 to September 2002, 109 survivors out of 120 patients were studied. Two-stage repair was performed in 42 patients (group A), and single-stage repair was performed in 67 (groups B and C). Before repair, the diameters of the ascending aorta and main pulmonary artery were measured. In the patients with single-stage repair, coarctation was repaired by extended end-to-end anastomosis in 35 patients (group B) and by pulmonary homograft patch augmentation in 32 patients (group C). The ventricular septal defect was closed through the pulmonary artery in 70 patients and through the right ventricle or atrium in 39 patients. The neoaorto-aortic discrepancy was treated by V-shaped resection of the posterior sinus of Valsalva in 7 cases, pulmonary homograft patch in 32 cases, and anterior splitting of the ascending aorta in all cases. Before discharge from the hospital, neoaortic root and ascending aorta diameters and aortic regurgitation grade were recorded. Neoaortic regurgitation progression and reintervention were the end points of follow-up (97.2 +/- 61.2 months). RESULTS: Early and late survivals were significantly better in group C (P <.001). Risk factors for neoaortic regurgitation at discharge by univariate analysis were single-stage repair (P <.05) and ventricular septal defect closure through the pulmonary artery (P =.0076). On multivariate analysis, the latter was the only risk factor for neoaortic regurgitation at discharge and at last follow-up. Multivariate analysis showed that higher neoaortic root/ascending aorta ratio and ventricular septal defect closure through the pulmonary artery were risk factors for neoaortic regurgitation evolution at last follow-up. There were 29 reinterventions, 19 for recoarctation of the aorta and 10 for neoaortic regurgitation with or without aortic root dilatation. Group B (P <.05), high neoaortic root/ascending aorta ratio (P <.01), and progressive neoaortic regurgitation (P <.05) were risk factors for recoarctation of the aorta. Group A was a risk factor for aortic valve replacement at 10 years (P <.05). CONCLUSION: Neonatal single-stage repair with pulmonary homograft aortic augmentation remains the optimal approach to transposition of the great arteries with ventricular septal defect and aortic coarctation. It provides better early and late survivals and freedoms from left-sided lesions. Avoidance of late recoarctation of the aorta and progressive neoaortic regurgitation requires meticulous closure of the ventricular septal defect and evenly sized reconstruction of the aorta from root to distal arch.     

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels. METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications. CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.     

A vascular ring: right aortic arch and descending aorta with left ductus arteriosus

A case is presented of vascular ring caused by right aortic arch with mirror-image branching and left ductus arteriosus. In this case, the descending aorta was located right of the tracheoesophagus and the left ductus arteriosus connected to the descending aorta far below the arch, producing compression of the esophagus only. Through median sternotomy, the ligation and division of the ductus was performed with concomitant repair of ventricular septal defect.     

Spontaneous closure of ductus arteriosus in interrupted aortic arch with ventricular septal defect

A 2-month-old boy diagnosed with interrupted aortic arch type B was treated with a two-stage procedure. His ductus arteriosus had closed spontaneously. Collaterals via both vertebral arteries developed. A 15-mm stenotic segment existed between the left subclavian artery and the descending aorta. The direct anastomosis between the common carotid artery and the descending aorta was performed as a first palliation at the age of 3 months. The left subclavian artery was reconstructed by end-to-side anastomosis to the descending aorta. The postoperative course was uneventful. The closure of ventricular septal defect and pulmonary artery debanding were performed as a second operation 4 months after the first palliation. The patient is alive and well 7 months after the second operation.     

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.     

Single-stage repair of acute type A aortic dissection associated with aortic coarctation, perimembranous ventricular septal defect, and bicuspid aortic valve

A 17-year-old man with coarctation of the thoracic aorta, bicuspid aortic valve, perimembranous ventricular septal defect, and acute type A dissection of the ascending aorta underwent emergency repair for dissection by means of single-stage replacement of not only the aortic valve and ascending aorta, but also simultaneous repair of the coarctation by graft interposition. These combined conditions comprised some difficulties that included decisions on the optimal timing and sequence of surgical repair, technique, and circulatory support.