Coarctation and hypoplasia of the aortic arch: will the arch grow?

Hypoplasia of the transverse aortic arch of various degrees of severity is commonly seen in infants who have coarctation of the aorta. It is more often present when the coarctation is associated with intracardiac lesions that diminish or limit forward flow in the ascending aorta and promote right to left flow through an arterial duct. The increased frequency of surgical treatment of infants with complex coarctation, which is in part related to the ability to stabilize their condition with prostaglandin E1, has posed the question of the potential for growth and development of the originally hypoplastic aortic arch after conventional repair of aortic coarctation. Review of our experience with transverse aortic arch hypoplasia, found in 33 (32%) of 102 infants undergoing coarctation repair by subclavian flap aortoplasty or classic resection and end-to-end anastomosis, revealed excellent growth of the transverse arch after repair in all patients available for linear follow-up. The currently proposed extended arch repair should be reserved for the small group of infants with transverse aortic arch to ascending aorta diameter ratios (arch indices) of less than 0.25.     

Congenital obstructive lesions of the right aortic arch.

BACKGROUND: In the setting of normal cardiac situs, a right-sided aortic arch is uncommon. When a right arch does occur, it is typically in conjunction with other congenital cardiovascular anomalies, especially defects with abnormal right ventricular outflow. Congenital obstruction of a right arch, caused by coarctation, interruption, or cervical arch, is extremely rare. METHODS: We reviewed our experience and all reported cases of right aortic arch with coarctation of the aorta, interrupted arch, or obstruction of a cervical arch in the setting of normal cardiac situs and topology. RESULTS: Since 1992, 4 such patients have undergone repair at our institution, including 1 with interrupted arch, 1 with coarctation of a mirror image arch, and 2 with obstruction of a cervical arch. In addition to these 4 patients, 38 others have been described in the published reports: 15 with interrupted arch, 19 with coarctation, and 4 with obstruction of a cervical arch. Associated cardiac defects were uncommon, except for ventricular septal defect in patients with interrupted arch, but abnormalities of the brachiocephalic vessels were frequent. Except for most of the patients with interrupted right arch, the majority of patients described have undergone successful surgical repair. CONCLUSIONS: Although obstructive arch lesions are often grouped together, the etiologies of coarctation of the aorta, interrupted arch, and cervical arch with obstruction almost certainly differ. The rarity of such lesions among patients with right aortic arch may be explained in part by the fact that the fetal hemodynamic conditions associated with persistence of a right arch do not facilitate flow-related arch obstruction. In this review, we discuss these issues in detail, along with specific surgical considerations in the management of obstruction lesions of the right aortic arch.     

Risk factors for recoarctation and results of reoperation: a 40-year review.

BACKGROUND: We analyzed a single institution 40-year experience with children that had coarctation repair to define risk factors for recoarctation and to evaluate the results of reoperation for recoarctation. MATERIAL AND METHODS: Between 1957 and 1998, 271 patients had primary surgical repair of coarctation of the aorta. Techniques for primary repair included Gore-tex patch aortoplasty (PATCH; n = 118), resection with extended end-to-end anastomosis (RXEEA; n = 69), subclavian flap aortoplasty (SFA; n = 61), resection with simple end-to-end anastomosis (ETE; n = 18), resection and interposition graft (n = 4), and extra-anatomic graft (n = 1). Techniques for recoarctation repair included PATCH, interposition graft, and extra-anatomic graft. RESULTS: Median age at initial repair was 156 days. Major associated cardiac anomalies were present in 96 patients (35%). A hypoplastic aortic arch was present in 37 patients (14%). There were three early deaths (1%) and six late deaths (2%). One patient had paraplegia (0.4%). Recoarctation occurred in 29 patients (11%) and was most frequent with ETE and SFA repairs (33% and 20%, respectively). Multiple logistic regression analysis revealed ETE repair (p = 0.0002), SFA repair (p = 0.049), and aortic arch hypoplasia (p = 0.0001) to be risk factors for recoarctation. Using PATCH as the covariate, the odds ratio to develop recoarctation was 3.5 for SFA, 17.2 for ETE, and 15.2 for hypoplastic aortic arch. There was no mortality or paraplegia after recoarctation repair (n = 23). Six patients had seven balloon angioplasties; two of these patients later required reoperation. Three patients required a second reoperation for persistent coarctation. CONCLUSIONS: In our 40-year review, simple end-to-end anastomosis and subclavian flap repair had the highest incidence of recoarctation, especially when associated with aortic arch hypoplasia. We recommend resection with extended end-to-end anastomosis for repair of neonates and infants and Gore-tex patch aortoplasty for children over the age of 1 year. Surgical repair of recurrent coarctation is safe, effective, and has a low incidence of persistent coarctation.     

Growth of the hypoplastic aortic arch after simple coarctation resection and end-to-end anastomosis.

Surgical treatment of a hypoplastic aortic arch associated with an aortic coarctation is controversial. The controversy concerns the claimed need to surgically enlarge the diameter of the hypoplastic arch, in addition to resection and end-to-end anastomosis. The purpose of this prospective study is to determine the fate of the hypoplastic aortic arch after resection of the aortic coarctation and end-to-end anastomosis. Between July 1, 1988, and January 1, 1990, 15 consecutive infants less than 3 months of age with an aortic coarctation were evaluated echocardiographically. A Z-value was calculated, being the number of standard deviations the aortic arch differs from the expected value, derived from a control group. Eight of these 15 infants had a hypoplastic aortic arch with a mean Z-value of -7.14 +/- 1.39. The other seven infants had a "normal" aortic arch with a mean Z-value of -1.85 +/- 1.08. All 15 infants underwent simple coarctation resection and end-to-end anastomosis. Six months after operation the mean Z-value increased significantly in those with a hypoplastic arch to -1.08 +/- 0.69 (p less than 0.0001) and in those with a "normal" aortic arch to 0.106 +/- 0.99 (p = 0.004). No infant died in our series (0%; CL 0% to 12%) and a recoarctation developed once (12.5%; CL 2% to 36%). Therefore we believe that simple resection and end-to-end anastomosis is the operation of choice for aortic coarctation associated with a hypoplastic aortic arch despite the presence of a ventricular septal defect and that enlargement of the hypoplastic aortic arch is not necessary.     

Combined surgical and endovascular approach to treat a complex aortic coarctation without extracorporeal circulation

Various therapeutic approaches have been proposed to treat complex coarctation of the aorta (eg, recoarctation, which requires repetitive interventions, or coarctation with a hypoplastic aortic arch). Resection followed by end-to-end anastomosis or by graft interposition is technically demanding and exposes the patient to considerable perioperative risks. Cardiopulmonary bypass and deep hypothermic circulatory arrest may be necessary to control the distal aortic arch. The role of stent technology in treating this type of lesion has not yet been defined. We present a 21-year-old woman with a recurrent coarctation of the aorta associated with a hypoplastic aortic arch and a pseudoaneurysm of the proximal descending aorta. She had undergone 4 previous interventions. Treatment consisted of a combined surgical and endovascular approach without cardiopulmonary bypass and included extraanatomic aortic bypass, partial debranching of the supraaortic vessels, and stent-graft insertion to exclude the aneurysm.     

Chirurgische Therapie der Aortenisthmusstenose

The aortic isthmus represents the region of the smallest aortic diameter and is located at the beginning of the descending aorta between the left subclavian artery and the origin of the first intercostal artery. Aortic coarctation (CoA) is defined as a pathologic narrowing of the aortic isthmus and accounts for 5–8?% of all congenital heart defects. A CoA can exist as an isolated entity or in combination with other cardiac anomalies. Due to the close relationship and interaction between the ductal orifice and development of coarctation, the term juxtaductal CoA prevails. Neonates with CoA and ductal-dependent lower body perfusion need urgent surgical repair. Spontaneous ductal closure can be prevented by infusion of prostaglandin E1 and if ineffective, emergency surgery is necessary. In contrast, in older children who suffer from arterial hypertension of the upper body, CoA can be corrected by intervention or surgery, as necessary. In most centers coarctation resection with direct end-to-end anastomosis has gained acceptance as the surgical repair of choice. In cases of associated aortic arch hypoplasia, which is common in 50?% of neonatal CoA cases, an extended resection and repair with simultaneous aortic arch enlargement by direct descending aorta to aortic arch anastomosis is recommended. Pronounced proximal arch hypoplasia should be corrected by an anterior approach facilitated by a cardiopulmonary bypass (CPB). Alternative operative techniques need individual implementation after careful decision making.     

Repair of aortic coarctation and aneurysm of ductus arteriosus in the presence of right-sided arch and descending aorta

Association of right aortic arch and coarctation of the aorta is rare. A patient with aortic coarctation was presented to our clinic. Detailed radiological work-up revealed aortic coarctation, aneurysm of ductus arteriosus, mirror image brachiocephalic vessels, right-sided arch, and right-sided descending aorta. The patient underwent successful operative repair through a right thoracotomy. She is normotensive and doing well two years postoperatively.     

A case of atherosclerotic aneurysm of the right subclavian artery with the right axillary arterial stenosis and enlargement of the ascending aorta

A 54-year-old man presented with neck pain and hoarseness. Angiography showed a rare right subclavian artery aneurysm, enlargement of the ascending aortic aneurysm, and axillary artery stenosis. These aneurysms would normally be treated with end-to-end anastomosis, but due to the complexity of the lesion in this case, we performed more extensive surgery. This consisted of successful reconstruction of the subclavian artery and replacement of the ascending aorta and aortic arch.     

Aortic coarctation with hypoplastic aortic arch. Results of extended end-to-end aortic arch anastomosis

Between 1980 and 1986, 80 infants (less than or equal to 3 months old) with symptomatic aortic coarctation and associated severe tubular hypoplasia of the transverse aortic arch underwent surgical treatment. Extended end-to-end aortic arch anastomosis was used in an attempt to correct both the isthmic stenosis and the hypoplasia of the transverse arch. After complete excision of the coarctation tissue, a long incision was made in the inferior aspect of the aortic arch, which was then anastomosed to the obliquely trimmed distal aorta. Pure coarctation was present in 17 patients (group I); 24 infants had an additional ventricular septal defect (group II), and 39 patients had associated complex heart disease (group III). The overall early mortality rate was 26% (confidence limits 21% to 32%) (18% in group I, 17% in group II, and 36% in group III). The early risk declined with time and was 18% (confidence limits 12% to 26%) for the last 2 years (seven deaths in 39 patients). Follow-up was 100% for a mean of 19 months. Actuarial survival rate at 3 years was 82% for group I, 78% for group II, and 32% for group III. Recurrent coarctation (gradient greater than or equal to 20 mm Hg) occurred in six operative survivors (10%, confidence limits 6% to 16%) and necessitated reoperation in three. Freedom from recoarctation at 4 years was 88%. Because extended end-to-end aortic arch anastomosis provides adequate correction of the aortic obstruction and entails a low risk of restenosis, it is our procedure of choice in infants with coarctation and severe hypoplasia of the aortic arch.     

New aspects of the surgical treatment of aortic coarctation

The article substantiates the urgency of the problem and shows the results of surgical treatment of coarctation of the aorta in 103 patients who were operated on in the recent 5 years. The operation of choice was resection of the coarctation with the formation of an end-to-end anastomosis (90.3% of operations). Establishment of the anastomosis by methods developed at the department makes it possible to achieve good results and avoid complications in the immediate and late-term postoperative periods. An anomalous arterial branch, a reduced branch of the 5th aortic arch called Abbott's artery, was ligated in 40% of cases. No fatal outcomes occurred in the last 5 years in surgical treatment of coarctation of the aorta.     

Successful repair of transposition of the great arteries with right aortic arch and coarctation of the aorta

A one-month-old girl with a diagnosis of situs solitus, d-transposition of the great arteries, coarctation of the aorta, and right aortic arch underwent a successful rapid two-stage repair. The combined anatomy of coarctation and right aortic arch is relatively rare, and even rarer in combination with transposition of the great arteries. We report the first successful surgical intervention to correct these anomalies.     

Extended end-to-end repair and enlargement of the entire arch in complex coarctation

BACKGROUND: Treatment of hypoplasia of the entire arch in coarctation is a surgical challenge. The current approaches have technical difficulties, high recurrence rates, and increased morbidity and mortality. METHODS: Over a 14-month period, a combined extended end-to-end repair with patch enlargement of the concavity of the entire arch was performed in 6 neonates and 1 infant. Through a midsternotomy and using cardiopulmonary bypass and hypothermia, extended end-to-end repair was performed initially leaving the proximal anastomosis open. The enlarging polytetrafluoroethylene patch was then sutured starting at the incised descending aorta distal to the extended end-to-end repair and continued retrogradely through the transverse arch to the ascending aorta proximal to the aortic cannulation site. One neonate had a patent ductus arteriosus and another had ventricular septal defect closure. One neonate had arterial switch and 3 had Norwood-type procedures performed with the enlarging patch extended to the pulmonary artery anastomosis. The remaining infant had arch enlargement performed after an arterial switch procedure and extended end-to-end repair. RESULTS: All patients did well and showed no residual gradient up to 1 year follow-up. Two patients successfully had bidirectional Glenn shunt at 9 months of age, and one had closure of residual arterial septal defect at 8 months of age. CONCLUSION: The combined extended end-to-end repair and arch enlargement procedure should minimize recurrence rates because of a tension-free enlargement of the entire aortic arch and elimination of the coarctation ridge and ductile tissues. Combined with the arterial switch and Norwood-type procedures, the approach results in a large neoaorta.     

Interdigitating arch reconstruction eliminates recurrent coarctation after the Norwood procedure

BACKGROUND: We sought to determine whether evolving techniques of aortic arch reconstruction used during the Norwood procedure decreased the incidence of postoperative aortic arch obstruction. METHODS: Our technique for aortic arch reconstruction in patients undergoing the Norwood procedure has evolved from using an allograft patch (classic group, n = 26) to primary connection of the pulmonary artery and arch (autologous group, n = 20). More recently, we have used a novel technique involving coarctation excision, an extended end-to-end anastomosis on the back of the arch, and a counterincision on the anterior descending aorta to sew in an allograft patch for total arch reconstruction (interdigitating group, n = 33). Cardiac catheterizations performed before stage II palliation were reviewed for aortic diameters at multiple levels in 79 infants (median age, 4.2 months). Aortic arch obstruction was defined as a ratio between the diameters of the arch anastomosis and the descending aorta (coarctation index) of less than 0.7. RESULTS: Overall, 15 (19%) children had aortic arch obstruction. All 15 required aortic intervention (balloon angioplasty, n = 12; surgical patch angioplasty, n = 2; both, n = 1). Aortic arch obstruction rates for the classic, autologous, and interdigitating groups were 46% (n = 12), 15% (n = 3), and 0%, respectively ( P > .001). CONCLUSION: Reconstruction of the aortic arch with excision of ductal and coarctation tissue is associated with lower aortic arch obstruction rates in patients undergoing the Norwood procedure. Arch reconstruction with a novel interdigitating technique decreases the incidence of aortic arch obstruction.     

A simple, safe, and rapid technique for the management of recurrent coarctation of the aorta

Six patients treated in infancy for coarctation of the aorta developed severe stenosis of the end-to-end aortic anastomosis that produced marked collateral circulation and upper extremity hypertension. Operation relieved the anastomotic obstruction in all 6 patients. A resection of the stenotic anastomosis with end-to-end aortic anastomosis was performed in 2 patients, bypass grafting between the transverse aortic arch and the distal aorta was done in 3 patients, and bypass grafting between the enlarged left subclavian artery and the distal aorta was performed in 1 patient. Interposed graft segments were short and had a diameter equal to or greater than the diameter of the aorta above or below the stenosis. Insertion of such short graft segments over an anastomotic obstruction provides an easy, rapid, and safe method for relieving hypertension and restoring distal pulsatile blood flow. Bypass grafting should be reserved for patients in whom anatomical considerations present an unusual hazard for a second aortic resection.     

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

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

Anatomically positioned aorta ascending-descending bypass grafting via left posterolateral thoracotomy for reoperation of aortic coarctation.

OBJECTIVE: Operation for aortic recoarctation and/or residual hypoplastic arch represents a surgical challenge because of surrounding scar tissue in the coarctation area, hazard of spinal cord ischemia due to aortic cross-clamping, laceration of the recurrent nerve, and the choice of the best approach. We demonstrate the first results of an anatomically guided technique via the prior left thoracotomy approach without establishment of cardiopulmonary bypass. METHODS: Since 1989, five patients underwent anatomically positioned ascending-descending bypass grafting for treatment of recoarctation. Indication was a non-dilatable hypoplastic aortic arch segment; in two cases an additional isthmic restenosis was present. Inclusion criteria for our technique was an aorta ascending diameter large enough to allow partial clamping. Primary repair of aortic coarctation was end-to-end anastomosis in four patients and patch angioplasty in one. Mean age at primary repair was 5.5 years and at reoperation 16.1 years. Systolic pressure gradients at rest ranged from 35 to 70 mmHg; upper extremity hypertension was present in all patients. Operative technique consisted of performing a dacron or PTFE aorta ascending-descending bypass graft parallel to the aortic arch, size 18 or 20 mm in diameter, via the prior left thoracotomy. RESULTS: There were no intraoperative complications and all patients survived. Postoperative complications were left lung atelectasis with necessity of reintubation, pericardial effusion, and transient left diaphragm elevation, each in one patient. After 7-90 months all patients are free of symptoms, have normal blood pressure (with two patients being under anti-hypertensive medication), and have no echocardiographically measurable pressure gradients. CONCLUSIONS: Anatomically positioned aorta ascending-descending bypass grafting via the prior left posterolateral thoracotomy without cardiopulmonary bypass is a safe and efficient method for operation of complex recoarctation in patients with an acceptable size of the aorta ascendens.     

经胸骨正中切口一期矫治主动脉缩窄或主动脉弓中断合并心内畸形

目的 总结经胸骨正中切口一期矫治主动脉缩窄或弓中断合并心内畸形的经验.方法 2007年1月-2008年7月手术治疗24例.包括主动脉缩窄9例,主动脉缩窄合并主动脉弓发育不良12例,主动脉弓中断3例.4例合并右室双出口(Traussig-Bing型).22例合并非限制性室间隔缺损,2例不合并室间隔缺损病儿1例合并主动脉瓣下狭窄,另l例合并肺静脉狭窄.主动脉弓降部成形均在深低温低流量持续性选择性脑灌注下进行.3例主动脉弓中断及9例主动脉缩窄病儿采用端端吻合术.12例主动脉缩窄合并主动脉弓发育不良病儿中采用扩大端端吻合术8例,端侧吻合术2例,补片成形术2例.结果 死亡2例.全组病儿围术期未出现神经系统并发症及肾功能损害.术后反复呼吸道感染2例.除l例残存压差大于20mm Hg外,最长随访18个月,尚未发现再缩窄发生.结论 主动脉缩窄或弓中断合并心内畸形一经诊断即需尽早手术.经胸骨正中切口一期矫治是安全、有效的.充分切除动脉导管组织,广泛彻底游离松解胸部各血管进行无张力吻合以及选择恰当的组织一组织吻合术式是主动脉弓降部成形手术成功及减少再缩窄发生的关键.     

Aortic coarctation in the first three months of life. An anatomopathological study with respect to treatment

Operation for coarctation in infancy is a lifesaving procedure, but it still carries a high mortality and is followed frequently by recurrence of stenosis. This is especially true when the procedure is performed in the first 3 months of life. To determine a correlation, if any, between anatomy of coarctation and surgical procedures, we have studied a series of 42 autopsy specimens from patients younger than 3 months who had aortic coarctation. We found that concepts of coarctation based solely on a discrete lesion or isthmic hypoplasia were simplistic. The anatomy was much more complex, the findings being relevant to surgical techniques currently in use (resection and end-to-end anastomosis, subclavian flap aortoplasty). Of equal significance in the cases we studied to the presence of a shelf of ductal tissue within the aortic lumen or hypoplasia of a segment of the arch was the finding of "waist" lesions. In these cases the wall of the aorta was constricted to form the obstruction. Such "waists" coexisted with discrete shelves or with shelves and isthmic narrowing. While we recognize the danger of extrapolating from autopsy findings to predict clinical results, our observations suggest that surgical procedures might best be tailored to the precise anatomy present. It seemed that in may cases a subclavian flap procedure offered the best chance of success. Cases with anatomy suitable for resection and end-to-end anastomosis were less frequent. Percutaneous angioplasty seemed at best to offer only palliative options in limited cases.     

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.     

A case of simple coarctation]

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