Subclavian flap aortoplasty for treatment of coarctation of the aorta in infants less than 3 months of age

Over a period of 3 years, 21 infants under 3 months of age were operated upon for coarctation of the aorta by subclavian flap aortoplasty. Associated intracardiac defects were present in 13 patients (62%), VSD being the most frequently encountered (10 patients, 48%). In 8 patients coarctation of the aorta, with or without patent ductus arteriosus, was the only cardiovascular malformation. The indication for surgical treatment was intractable congestive heart failure despite intensive medical treatment. Eight patients were on mechanical ventilatory support prior to surgery. There was no early operative mortality. One patient died in congestive heart failure due to valvular aortic stenosis 3 months after surgery for correction of the coarctation. During a follow-up of from 2 months to 3 years, 5 patients underwent a second operation for correction of intracardiac defects. Nineteen of the surviving 20 patients are in good clinical condition. One patient has clinical evidence of residual coarctation; a blood pressure difference of more than 10 mmHg between right arm and leg is presented in 4 patients. Early subclavian flap aortoplasty is recommended for patients with coarctation of the aorta, with or without associated intracardiac defects, who remain in congestive heart failure despite medical therapy.     

6kg以下小婴儿主动脉缩窄合并室间隔缺损的外科矫治

目的 :探讨新生儿、小婴儿主动脉缩窄 (CoA)合并室间隔缺损 (VSD)的外科治疗方法。方法 :1998年 12月至 2 0 0 3年 7月共收治 9例 6kg以下CoA合并VSD患儿 ,其中新生儿 3例。一期手术 3例 ,分期手术 3例 ,仅做主动脉缩窄成形术 3例 ,其中 1例加做肺动脉环缩术。 6例采用人造血管补片主动脉成形术 ,3例采用左锁骨下动脉翻转主动脉成形术。结果 :全组患儿无死亡 ,随访 8～ 36个月 ,心功能改善明显 ,超声心动图检查提示无主动脉瘤样扩张或主动脉再狭窄。结论 :大多数患儿均可采用体外循环下一期根治CoA和VSD ,但是对于VSD为限制性及不能耐受体外循环的患儿可采用二期手术。一期手术选择左外侧和正中双切口方法安全有效。     

Coarctation of the aorta in infancy

Twenty-five infants under 1 year of age (mean weight 3.4 kg) underwent repair of coarctation of the aorta between the years 1965 and 1982. Three patients had coarctation only, three had coarctation with patent ductus arteriosus (PDA), and 19 had associated intracardiac anomalies. Eleven patients underwent resection of the aorta and end-to-end anastomosis. Eight had subclavian flap arterioplasty, five had patch graft arterioplasty, and one had subclavian-to-aortic anastomosis. Additional procedures were performed on seven patients: banding of the pulmonary artery on one, repair of total anomalous pulmonary venous drainage on one, mitral valve replacement on one, aortic valvotomy on one, and aortic valvotomy plus pulmonary artery banding on one. Twenty-one survived the operation. All patients who died had associated intracardiac anomalies. The 21 survivors have been followed from 3 months to 13 years, with three late deaths that were associated with intracardiac anomalies. Five of the survivors underwent additional second operations: one had repair of the re-coarctation, one had replacement of the prosthetic mitral valve, one had aortic valvotomy, and two had pulmonary artery debanding and closure of a ventricular septal defect. Two of the 18 surviving patients have mild hypertension associated with a residual gradient. The others are in good health.     

婴幼儿主动脉缩窄合并心内畸形的Ⅰ期手术纠治

目的:总结主动脉缩窄合并心内畸形的外科治疗经验。方法:回顾分析2007年1月至2011年6月我院收治的婴幼儿主动脉缩窄合并心内畸形Ⅰ期手术治疗的临床病例。共计42例,其中男性26例,女性16例。平均年龄(1.6±0.9)岁(6 d～3岁),平均体质量(7.2±2.5)kg(2.8～12kg)。主动脉缩窄合并心内畸形:37例合并室间隔缺损,5例合并房间隔缺损,7例同时合并主动脉瓣二瓣畸形,手术方法采用双切口I期纠治5例,正中切口I期纠治37例,其中锁骨下动脉翻转法(8例)、人工补片法(6例)、端端吻合法(15例)、端端吻合+补片法(13例)。结果:本组42例中,死亡1例,术后9 d死于肺部感染,病死率2.4%。术后均复查心脏彩超显示降主动脉血流通畅,无明显狭窄,3例提示降主动脉内仍存在20～40 mmHg(1 mmHg=0.133 kPa)压差,心脏彩超示吻合口处狭窄,血流速度增快。结论:婴幼儿主动脉缩窄合并心内畸形采用深低温选择性脑灌注方法经胸骨正中切口I期手术治疗可以达到满意疗效。彻底切除缩窄段及导管组织是手术成功的关键。     

Histo-anatomical backgrounds of subclavian flap aortoplasty in coarctation of the aorta

After repair of coarctation of the aorta using the technique of resection and end-to-end anastomosis, the internal diameters of the aortic isthmus and descending aorta often fail to increase. Better results seem possible with aortoplasty using the left subclavian flap technique. In order to clarify this matter, we investigated the structure of the left subclavian artery comparing it with that of the descending aorta and aortic isthmus: we studied the internal diameter, the thickness of the tunica media and the packing density of its elastic fibers in these vascular elements using a postmortem material of children with a coarctation of the aorta. The ages ranged from 4 days to 13 months with one child of 8 years. All 16 cases had one or more additional cardiac lesions. Operation had been performed in 3 children: 2 end-to-end anastomoses and one subclavian bypass of the aortic arch. Data were compared with observations on autopsy cases of children without cardiovascular abnormalities. The mean findings were that the calibers of the left subclavian artery and the descending aorta were within normal limits but that the caliber of the aortic isthmus was smaller than in normal children. The measurements on the tunica media showed that although, generally, the thickness of the media of the left subclavian artery was smaller than that of the aortic isthmus and descending aorta of the same individual, it contained relatively more elastic fibers than the matching vessels. This may indicate that the structure of the left subclavian artery is well suited to grow out as a part of the aortic arch.(ABSTRACT TRUNCATED AT 250 WORDS)     

小儿主动脉缩窄的诊断和治疗（附七例报告）

报告小儿主动脉缩窄７例，占同期心血管病手术０．５％。导管前型４例，均伴有动脉导管未闭（ＰＤＡ），其中１例伴有室间隔缺损（ＶＳＤ）；导管后型３例，伴有ＰＤＡ及ＶＳＤ各１例。５例采用缩窄段切开和人造血管补片主动脉成形术，采用缩窄段切除主动脉端端吻合术及左锁骨下动脉片翻转吻合主动脉成形术各１例，均取得良好疗效。对该病的诊断、手术方法选择和手术并发症及处理进行讨论。     

Intravascular ultrasonic assessment of post-operative remodelling of aortic coarctation repaired by use of a subclavian flap

BACKGROUND: We have used intravascular ultrasound in an attempt to clarify the extent of vascular remodelling of the aortic arch after the repair of aortic coarctation by use of a subclavian flap. METHODS: We investigated 13 patients with coarctation of the aorta, ranging in age from 1.4 to 43.0 months, with a mean of 20.8 months, who underwent aortoplasty by incorporation of a subclavian flap. The mean postoperative period was 19.6 months, with a range from 0.03 to 41.2 months. The luminal morphology of the aortic arch was evaluated by intravascular ultrasound at the time of post-operative catheterization. RESULTS: We observed 3 cases longitudinally. Over the period of observation, we found three types o f morphology of the aorta at the site of incorporation of the subclavian flap, namely a snowman shape with two inflection points, a pisiform shape with one inflection point, and a round shape without any points of inflection. There was a correlation between the cross-sectional shapes at the site of the subclavian flap in the postoperative period (p < 0.01). In each case, we measured the cross-sectional area at the site of subclavian flap, at the descending aorta, and at the distal aortic arch. The cross-sectional area, and the increment of the cross-sectional area at the site of subclavian flap, was larger. CONCLUSION: The shape of the lumen subsequent to repair of aortic coarctation changes progressively from a snowman, to a pisiform, and finally to a round shape. Greater growth of the subclavian flap compared to the native wall of the aorta was observed for at least the first 4 years after repair. This finding may improve our understanding of the remodelling process of the arterial trunks after surgical repair.     

Maximal voluntary exercise variables in children with postoperative coarctation of the aorta.

Thirty-one children with postoperative coarctation of the aorta underwent maximal graded bicycle ergometry using an electronically braked ergometer and the James protocol; 18 also underwent expiratory gas measurement using a mass spectrometer. Twenty-two age- and gender-matched normal subjects were used as a control group. The mean age at operation was 41 months and the mean age at evaluation was 134 months (mean follow-up interval 93 months). The original surgical repair was subclavian flap repair in 8 patients, end to end anastomosis in 21, patch aortoplasty in 1 patient and tubular graft in 1. Patients exercised until exhaustion and maximal exercise variables were obtained. The maximal voluntary peak heart rate was 183 beats/min (94.6% of predicted value), indicating excellent effort. Mean power was 111% of predicted value and, when measured, maximal oxygen consumption was 89% of predicted value with an anaerobic threshold at 63 +/- 3.5% of exercise time. The observed work variables were not different from values in the control group and were not affected by the type of repair. The mean peak systolic blood pressure was 152 +/- 7.6 mm Hg versus 147 +/- 5.7 mm Hg in the control group (p = NS). Patients who had associated intracardiac lesions had significantly lower maximal oxygen consumption (85 +/- 3% vs. 98 +/- 4% of predicted value). The results suggest that adequate cardiopulmonary function, normal or above average work capacity and normal exercise systolic blood pressure can be obtained in children with satisfactory repair of coarctation of the aorta performed before school age.     

Surgical treatment of coarctation of the aorta in the first year of life. Immediate and late results in 35 patients

Between February 1974 and September 1981, 35 consecutive infants younger than 12 months underwent repair of coarctation of the aorta. The mean age was 57 days and 24 were younger than 60 days. The indications for operation were congestive heart failure, metabolic acidosis and severe systemic hypertension. Only 3 cases had isolated coarctation, whereas the remaining 32 had associated anomalies. The first 4 patients were submitted to primary repair; after this, patch aortoplasty was utilized in 14 infants and in the remaining 17 patients the aortoplasty using the left subclavian artery was performed. The hospital mortality rate was 11.4% (4 cases) and there were 2 late deaths (5.7%). Follow-up over a 7-year period shows no clinical or hemodynamic evidence of recoarctation in any of the survivors.     

Surgical management of coarctation of aorta

Between 1961 and 1988, 68 patients underwent operation for coarctation of the aorta. The average age of presentation of these subjects was higher than in other series. 7.1 percent were asymptomatic, a finding which is not seen in reports from the west. Operative procedures included resection and end to end anastomosis, resection and graft interposition, bypass grafting, patch aortoplasty and subclavian flap aortoplasty. However, the technique of patch aortoplasty, routinely performed in the last 24 years of this series seemed by far the most satisfactory procedure. Subclavian flap aortoplasty was carried out in a selected group of younger children. Associated cardiac anomalies influenced the results adversely. The overall operative mortality was 5.8 percent. Hypertension did not regress in 11.7 per cent of patients inspite of a successful operation as judged by the return of peripheral pulses in the lower limbs. Re-coarctation was not seen in this series. The overall results of operation for coarctation of the aorta have been very satisfactory and comparable with those in other published series.     

Modified End-to-End Anastomosis Combined with Subclavian Flap Aortoplasty for Repair of Coarctation of the Aorta with Extended Hypoplasia of the Aortic Isthmus

Abstract Recoarctation is a serious complication of the surgical repair of coarctation of the aorta. A combined technique using end-to-end anastomosis and subclavian flap angioplasty has shown good results. However, this technique is not entirely free from longitudinal traction in cases of extended hypoplasia of the aortic isthmus. To obviate these problematic sequelae, we have modified the combined technique for repair of coarctation of the aorta having extended hypoplasia of the aortic isthmus. Almost the entire aortic isthmus is left unresected and a lesser period of interruption of blood flow through the descending aorta is required. During the period from 1991 to 1998, five infants with this abnormality underwent surgical repair with the modified method. The results were excellent with no postoperative death and no recoarctation during the follow-up period.     

Critical aortic coarctation: patch aortoplasty in infants less than age 3 months.

Twenty-three infants less than age 3 months (mean age 31 days) underwent patch aortoplasty for relief of coarctation of the aorta. All had intractable congestive heart failure, despite aggressive medical therapy. Each infant had other cardiac anomalies, including patent ductus arteriosus (83 percent) and ventricular septal defect (74 percent). All patients underwent closure of the ductus arteriosus and patch angioplasty of the aorta to produce a luminal diameter of at least 16 mm. In addition, 9 of the 17 patients (53 percent) with a large shunt ventricular septal defect underwent pulmonary arterial banding. There was one hospital death 42 days after operation secondary to bowel perforation and sepsis. Hospitalization beyond 21 days postoperatively was always due to other unrepaired cardiac lesions. The three late deaths at 3, 9 and 18 months after operation were associated with additional major anomalies. Fourteen patients have had postoperative catheterization. No gradient was found across the site of coarctation repair, but one patient had a gradient between the left carotid and left subclavian arteries. Surgical repair of critical coarctation of the aorta in infants can safely be offered despite the presence of other cardiac anomalies.     

Coarctation repair in children with Turner's syndrome

Ravelo recently reported the results of surgical repair of coarctation of the aorta in eight patients with Turner's syndrome. Three of these patients had serious hemorrhagic complications. At Texas Children's Hospital, we have operated on four patients with Turner's syndrome and coarctation of the aorta without complications. Of the 12 patients in both series, nine patients had uncomplicated courses when they had Dacron aortoplasty (4/12) or end-to-end anastomosis (5/12). The three other patients with end-to-end anastomoses had significant perioperative hemorrhage. Whereas Ravelo reported serious complications in 38% (3/8), the combined series suggest that only about 25% (3/12) of patients with Turner's syndrome undergoing repair of coarctation of the aorta may experience significant perioperative hemorrhage. We conclude, as did Ravelo, that attention to technical details should keep surgical morbidity in these patients to a minimum.     

Reoperation for coarctation of the aorta

Between 1957 and 1980 reoperation for coarctation of the aorta was performed in 21 patients at one institution for an overall incidence rate of 7.9 percent. The incidence rate of reoperation was 38 percent for patients younger than age 3 years and 1.5 percent for patients 3 years or older at initial repair. Before reoperation 14 of the 21 patients were symptomatic, 19 had systolic hypertension of the upper limbs and 20 had a documented coarctation pressure gradient at rest (mean 42.4 mm Hg). Surgical techniques used at reoperation were patch aortoplasty in 12 patients, graft interposition in 4, end to end anastomosis in 3 and end to side left subclavian to descending aorta bypass graft in 2. There was one surgical death. The 20 survivors have been followed up a mean of 4.3 years. There has been significant symptomatic improvement (p < 0.001). Upper limb hypertension has also lessened significantly (p < 0.001) after reoperation;15 patients are no longer hypertensive and 3 have a lesser degree of hypertension. The coarctation pressure gradient at rest has significantly decreased (p < 0.001); 13 patients have no residual gradient and 7 have a mild gradient of 20 mm Hg or less. Graded treadmill exercise testing performed in five patients after reoperation documented upper limb hypertension in four and a marked increase in coarctation gradient with exercise in three.

In conclusion, the incidence of reoperation is significantly increased in patients who are younger than age 3 years at initial coarctation repair. Reoperation is a safe and effective procedure. It has a low mortality rate (4.8 percent), relieves symptoms and decreases hypertension and the coarctation pressure gradient. Patch aortoplasty appears to be the operative procedure of choice. Moderate to severe hemodynamic abnormalities may persist during exercise after reoperation for coarctation of the aorta.     

Long-term outcome after repair of coarctation in infancy: subclavian angioplasty does not reduce the need for reoperation

To assess the influence of surgical technique on the need for reoperation after coarctation repair in infancy, follow-up data were analyzed for 125 consecutive infants (less than 12 months) who underwent repair of coarctation of the aorta by subclavian angioplasty or resection and end to end anastomosis. Sixty-three infants underwent coarctation repair by resection between 1960 and 1980, and 62 underwent subclavian angioplasty between 1977 and 1985. The mean age (+/- SEM) at operation for infants with subclavian flap angioplasty was 1.54 +/- 0.93 months and for infants with resection was 2.70 +/- 0.93 months (p = 0.02). There was no difference between the groups in patient weight at initial repair or the proportion of patients with complex anatomy or aortic arch hypoplasia. Follow-up duration for the subclavian flap group was 2.55 +/- 0.51 years (range 0.3 to 8.2), and for the resection group was 7.97 +/- 3.61 years (range 0.6 to 21). Indication for reoperation was the presence of a coarctation gradient at rest of 40 mm Hg or greater and arm hypertension. Reoperation was required in 5 patients in the subclavian flap group and 12 patients in the resection group. The mean reoperation rate after subclavian flap repair was 0.0356 reoperations per patient-year, and after resection was 0.0342 reoperations per patient-year (p = 0.94). To determine an individual's risk of requiring reoperation from these group measures, a reoperation risk model was developed. The risk of reoperation by the fifth postoperative year was found to be 16.3% after subclavian flap repair and 15.7% after resection.(ABSTRACT TRUNCATED AT 250 WORDS)     

Balloon angioplasty for recurrent coarctation of aorta. Immediate and long-term results.

BACKGROUND. As angioplasty techniques have been refined and larger low-profile balloons developed, a nonsurgical approach to recoarctation has become available. Several reports have documented both the efficacy and safety of this procedure. However, there are little data available on the long-term follow-up of these patients. This report details the initial results and long-term evaluation of both the relief of obstruction and the presence of hypertension after balloon angioplasty for recurrent coarctation. METHODS AND RESULTS. Balloon angioplasty for recurrent coarctation of the aorta was performed 29 times in 26 patients at a median age of 4 years and 9 months (range, 4 months to 29 years), with eight patients less than 1 year old. Initial surgical techniques were end-to-end anastomosis in 11 patients, subclavian flap aortoplasty in 11 patients, and patch aortoplasty in four patients. Angioplasty was performed at a median interval of 2 years and 7 months (range, 4 months to 23 years) after surgery. Mean peak systolic pressure difference across the coarctation decreased from 40.0 +/- 16.8 to 10.3 +/- 9.5 mm Hg (p less than 0.05) after the initial angioplasty, and mean diameter of the aortic lumen at the coarctation site increased from 5.8 +/- 3.5 to 9.0 +/- 4.3 mm (p less than 0.05). There was no mortality, and only one patient developed an aneurysm (4%). Three patients underwent repeat angioplasty for a pressure difference of more than 20 mm Hg. Long-term follow-up is available on 24 of 26 patients with a mean follow-up of 42 +/- 24 months (range, 12-88 months). Mean peak systolic pressure difference across the area of coarctation decreased from 40.3 +/- 17.4 before angioplasty to 8.5 +/- 8.3 mm Hg after final angioplasty (p less than 0.05) and 7.5 +/- 7.5 mm Hg at follow-up. Mean peak systolic blood pressure in the upper extremities decreased from 133.1 +/- 14.9 before angioplasty to 111.1 +/- 14.1 mm Hg at long-term follow-up (p less than 0.05). CONCLUSIONS. Balloon angioplasty should be considered the treatment of choice for relief of recurrent aortic coarctation.     

单纯主动脉缩窄的外科治疗

目的总结单纯主动脉缩窄外科治疗的经验,评价手术时机和方法。方法1990年6月至2005年8月,手术治疗单纯主动脉缩窄17例,其中行主动脉缩窄切除对端吻合术5例,人工血管移植术3例,补片成形术7例,Vosschulte成形术1例,左锁骨下动脉-降主动脉人工血管转流术1例。结果术毕上下肢平均动脉压的压差<10mmHg者13例,上肢平均动脉压仍高于下肢10～30mmHg者4例。术后早期高血压者14例。随访5个月至10年,下肢血压高于上肢者13例,无明显差异者3例,下肢血压仍明显低于上肢者1例,仍有高血压者5例;成形部位动脉瘤样扩张1例,再缩窄1例。结论早期诊断、早期治疗是提高生存率和减少术后高血压的关键。手术方法应根据患者年龄、病理特征和术者经验选择。     

Preoperative and postoperative "aneurysm" associated with coarctation of the aorta

The reported incidence of aortic aneurysm after surgical repair or balloon angioplasty for aortic coarctation varies widely. To determine the incidence of aneurysm formation after surgery, preoperative and postoperative cineangiograms from 65 patients who underwent operation at age 1.5 +/- 3.4 years were examined. Repair included a prosthetic patch in 14 patients, end to end anastomosis in 28 and subclavian flap in 23. Aneurysm was documented by change in contour or irregularities in contour at the repair site or by abnormal dimensions at the repair site, defined by the ratio of the widest measurement at the repair site to the measurement of the aorta at the diaphragm. An aneurysmal bulge above the ductus diverticulum was identified in 14 (23%) of 60 patients preoperatively; the area showed no change 4.72 +/- 4.07 years after surgery. Significant changes at the repair site were seen in only three patients, all of whom had Dacron patch repair. One patient had a change in contour at the repair site, one had an abnormally high repair site to diaphragmatic aorta ratio and one had a progressive increase in this ratio. Thus, during childhood years, 3 (5%) of 65 patients were diagnosed as having aneurysm at the surgical repair site. In conclusion, 1) comparison with preoperative cineangiograms, especially for aneurysmal bulges above the ductus arteriosus, is essential before an aneurysm can be attributed to coarctation repair by any technique, and 2) aneurysm developed only in patients subjected to Dacron patch repair.     

The influence of different surgical procedures on hypertension after repair of coarctation

We measured resting and exercise haemodynamics, as well as 24-hour ambulatory blood pressure, so as to study the influence on development of hypertension in children after repair of coarctation by either construction of a subclavian flap or end-to-end anastamosis. The patients in both groups were studied a mean time of 13 years after surgery. Thus, we divided 43 children who had undergone surgical repair of coarctation, and who were not on antihypertensive therapy, into a group of 22 patients who had undergone subclavian flap repair, with a mean age of 14 plus or minus 2.6 years, and another group of 21 patients undergoing end-to-end anastomosis, with a mean age of 13.5 plus or minus 3.9 years. We examined blood pressure at rest and during exercise, along with the measurement of cardiac output using impedance cardiography, and during 24-hour ambulatory monitoring. We recorded systolic and diastolic blood pressures, pulse pressure, cardiac output and total peripheral vascular resistance at rest and at peak exercise. During ambulatory monitoring, we measured mean pressures over 24 hours, in daytime and nighttime, 24-hour pulse pressure, and 24-hour mean arterial pressure. Student's t test was used to judge significance, accepting this when p was less than 0.05. The group repaired using the subclavian flap showed significantly disadvantageous differences for diastolic blood pressure at rest, systolic blood pressure at peak exercise and for 24-hour systolic and diastolic blood pressure, 24-hour mean arterial pressure, and daytime and nighttime systolic blood pressure during ambulatory monitoring. Our findings suggest that, after repair using the subclavian flap in comparison to end-to-end anastomosis, patients show a higher incidence of late hypertension, both during exercise and ambulatory monitoring. The data indicate different residual aortic stiffnesses, these being lower after end-to-end anastomosis, which may be due to the greater resection of the abnormal aortic tissue when coarctation is repaired using the latter technique.     

Surgical experience of coarctation of the aorta in infants and young children

We evaluated the effectiveness of surgical treatment for coarctation of the aorta in infants and young children, based on our experience over 7 years. From March 1999 to December 2005, 168 cases of coarctation of the aorta in patients aged 21 days to 3 years were treated by end-to-end, extended end-to-end, or extended end-to-side ascending aorta and aortic arch anastomosis. The mortality rate was 0.6%. In 138 (82%) patients, the pressure in the lower extremity was higher than in the upper extremity. During 6-24 months of follow-up, hoarseness appeared in 6 patients. Echocardiography revealed stenosis of the end-to-end anastomosis in only 2 patients. We suggest that the best option for surgical treatment of coarctation of the aorta with associated cardiac malformations is a one-stage procedure using a median sternotomy approach. It is better to perform extended end-to-end anastomosis or anastomosis between the distal descending aorta and the left wall of the ascending aorta, or to extend this anastomosis to the transverse arch.