The use of pulmonary autograft patch for type A aortic interruption and Swiss-cheese ventricular septal defect (VSD)

The surgical management of the aortic arch pathologies is controversial. Primary anastomosis and patch aortoplasties combined with end-to-end anastomosis have some complications like recurrence and aneurysm formation. Surgical repair of apical muscular (Swiss-cheese) defects is also still under debate. A 6-year-old patient with diagnosis of type A aortic arch interruption and Swiss-cheese ventricular septal defect (VSD) underwent successful intracardiac repair and aortic arch reconstruction. Aortic arch reconstruction was done by end-to-side anastomosis of distal aortic archus and thoracic aorta without cardiopulmonary bypass. The anterior side of the anastomosis was augmented by using pulmonary autograft patch and this patch was extended to the inferior surface of the archus aorta. Swiss-cheese VSD was repaired with a single patch using septal obliteration technique via transatrial approach. Pulmonary autograft patch aortoplasty and end-to-side anastomosis may be an alternative surgical management for surgical repair and it may be done without the need for cardiopulmonary bypass. In these patients associated multiple apical muscular VSDs can be repaired with a single patch, septal obliteration technique.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

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.     

Off pump repair of aortic arch anomalies with concomitant intracardiac defects via anterior approach

BACKGROUND: The authors evaluated the surgical treatment of aortic arch anomalies associated with intracardiac pathologies, through median sternotomy on beating heart without using cardiopulmonary bypass (CPB). METHODS: A consecutive series of 10 patients with aortic coarctation were operated upon. Median age at repair was 3.5 months (range, 5 days to 72 months), median weight was 4 kg (range, 2.2 to 30 kg). All aortic obstruction repairs were done via midsternotomy without using CPB and it is used only for repair of intracardiac defects. The aortic reconstruction included resection and end-to-side anastomosis in six patients and pulmonary autograft patch aortoplasty in four patients. RESULTS: There was no operative mortality. Mean follow-up value was 17.6 +/- 8.07 months. There was no restenosis. CONCLUSION: Most of the aortic coarctation and interrupted aorta type A can be well-treated surgically through median sternotomy without using CPB. Thus, the need for profound hypothermia and circulatory arrest and its potential neurological and other side effects are removed and CPB is reserved only for associated intracardiac defects, if present.     

Ross procedure in rheumatic aortic valve disease.

OBJECTIVE: To assess the results of aortic valve replacement with the pulmonary autograft in patients with rheumatic heart disease. METHODS: From October 1993 through September 2003, 81 rheumatic patients with aortic valve disease, mean age 29.5+/-11.9 years (11-56 years) underwent, the Ross procedure with root replacement technique. Forty patients were 30 years of age or below (young rheumatics). Associated procedures included mitral valve repair (n=19), open mitral commissurotomy (n=15), tricuspid valve repair (n=2), and homograft mitral valve replacement (n=2). RESULTS: Early mortality was 7.4% (six patients). Mean follow-up was 92.3+/-40.9 months (7-132 months, median 109 months). Sixty of the 73 patients whose follow-up was available (82%) had no significant aortic regurgitation. Re-operation was required in seven (8.4%) patients for autograft dysfunction with failed mitral valve repair (n=3), autograft dysfunction alone (n=2) and failed mitral valve repair alone (n=2). No re-operations were required for the pulmonary homograft. There were six (7.5%) late deaths. Actuarial survival and re-operation-free survival at 109 months were 84.5+/-4.1% and 90.5+/-3.7%, respectively. Freedom from significant aortic stenosis or regurgitation was 78.4+/-5.2% and event-free survival was 64.6+/-5.8%. When compared to rheumatics above 30 years of age, the relative risk of autograft dysfunction was high in the young rheumatics. CONCLUSION: The Ross procedure is not suitable for young patients with rheumatic heart disease. However, it provides acceptable mid-term results in carefully selected older (>30 years) patients with isolated rheumatic aortic valve disease.     

Total aortic arch graft replacement for recurrent aortic aneurysm

We successfully performed a total aortic arch replacement for a recurrent aortic aneurysm following repair of an aortic dissection. A 59-year-old man underwent a patch aortoplasty through median sternotomy for Stanford type B aortic dissection in other hospital. Three years and 6 months later an aneurysm developed. Computed tomography and magnetic resonance imaging angiography demonstrated an enlargement of the aneurysm, resulting in a diagnosis of recurrent distal aortic arch aneurysm. A graft replacement of the total aortic arch with the aid of selective cerebral perfusion was performed through a median resternotomy and left lateral thoracotomy. Additional left lateral thoracotomy offered a sufficiently optimal operating field for distal anastomosis. However, care must be taken not to overlook the bleeding from intercostal arteries. Since aortoplasty may lead to subsequent dilation and aneurysmal formation, initial replacement of the segment of the aorta is recommended, and careful long-term follow-up of the patient is important.     

A Modification of Extended Aortic Arch Anastomosis Augmented with Subclavian Flap Aortoplasty for Interrupted or Hypoplastic Aortic Arch

Abstract   Background: Surgical repair for hypoplastic aortic arch in neonates carries a substantial risk of recurrent obstruction. Simple arch anastomosis is not always a solution in cases of extended arch hypoplasia. We present our modified technique of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. Method: We describe two neonates: interrupted aortic arch and transverse arch hypoplasia associated with aortic coarctation, who underwent a modification of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. Results: The patients recovered without any pressure gradient at the anastomotic site. Postoperative aortography showed no arch obstruction and they successfully underwent second stage repair. Conclusion: Our technique provides extensive augmentation of the aortic arch with a tension-free, wide and non-circumferential suture line which preserves potential for growth. The technique described may avoid persistent or repeat arch obstruction.     

Repair of Truncus Arteriosus and Interrupted Aortic Arch

A total of seven patients with truncus arteriosus and interrupted aortic arch (IAA) comprises our surgical experience in this condition. All underwent primary complete repair via median sternotomy between June 1985 and December 1989. Median age at repair was 8 days and median weight, 3.2 kg. Anatomy of these seven patients was truncus arteriosus type "1 1/2" in five patients and type II in two patients, IAA type B in six patients and type A in one patient. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta. Right ventricle to pulmonary artery continuity was established with a porcine valved conduit in four patients, aortic homograft in two, and aortic homograft monocusp patch in one. Three patients have required five reoperations (three in one patient). One reoperation was due to compression of the left main bronchus from the reconstructed aorta, one was due to obstruction of the aorta at the site of IAA repair, and one was due to compression of the left main bronchus, right pulmonary artery, and residual stenosis across the hypoplastic ascending aorta. There were no early or late deaths and all seven survivors are currently well with a mean follow-up of 29 months from initial repair.     

Palliative reconstructive surgery for hypoplastic left heart syndrome

From August, 1985, through August, 1987, 104 consecutive, nonselected neonates underwent palliation of hypoplastic left heart syndrome. The technique included pulmonary artery homograft augmentation of the diminutive ascending aorta and aortic arch, atrial septectomy, transection of the main pulmonary artery with patch closure of the distal main pulmonary artery, anastomosis of the proximal main pulmonary artery to the augmented ascending aorta, and a 4-mm, modified, right Blalock-Taussig (N = 21) or central (N = 83) shunt. There were 30 early and 11 late deaths. Early mortality was most commonly associated with hypoventilation. Complications included development of aortic arch obstruction (N = 11) and progressive hypoxemia (N = 11). Alterations in surgical techniques and perioperative management should permit continued improvement in early and long-term survival.     

Aortic arch reconstruction with pulmonary autograft patch in coarctation and interruption of the aorta

The surgical management of the aortic arch pathologies is still subject to discussion. Primary end-to-end anastomosis has some complications such as bronchial compression, tension in the suture lines, and probability of recurrence. On the other hand, patch aortoplasties combined with end-to-end anastomosis carry the risk of aneurysm formation and recurrence. Considering the growth potential, pulmonary autograft patch use in aortic arch reconstructions has recently been introduced into clinical practice. In this study, we present the early findings of combined end-to-end anastomosis and pulmonary autograft patchplasty procedure in six patients. According to our experience the technique applied in this report seems to be more advantageous than other conventional approaches.     

Tracheal surgery in children: an 18-year review of four techniques

Objective: Review the short- and long-term outcomes of a single institution experience in infants with congenital tracheal stenosis, comparing four different operative techniques used from 1982 through 2000. Methods: Hospital and clinic records of 50 infants and children who had surgical repair of congenital tracheal stenosis secondary to complete tracheal rings were reviewed. Age at surgery ranged from 7 days to 72 months (median, 5 months, mean 7.8±12 months). Techniques included pericardial patch tracheoplasty (n=28), tracheal autograft (n=12), tracheal resection (n=8), and slide tracheoplasty (n=2). All procedures were done through a median sternotomy with cardiopulmonary bypass. Seventeen patients had a pulmonary artery sling (35%), and 11 had an intracardiac anomaly (22%). Results: There were three early deaths (6% early mortality), two after pericardial tracheoplasty and one after autograft. There were six late deaths (12% late mortality), five after pericardial tracheoplasty and one after slide tracheoplasty. Length of stay (median) was 60 days (pericardial tracheoplasty), 28 days (autograft), 14 days (resection), and 18 days (slide). Reoperation and/or stent placement was required in seven patients (25%) after pericardial tracheoplasty, in two patients (17%) after autograft, in no patients after resection, and in one patient (50%) after slide tracheoplasty. Conclusions: Our current procedures of choice for infants with congenital tracheal stenosis are resection with end-to-end anastomosis for short-segment stenoses (up to eight rings) and the autograft technique for long-segment stenoses. Associated pulmonary artery sling and intracardiac anomalies should be repaired simultaneously.     

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.     

Brom's three-patch technique for repair of supravalvular aortic stenosis.

OBJECTIVE: Case histories of all patients (n = 29) operated on for supravalvular aortic stenosis from 1962 to the present were reviewed to study different techniques and outcomes. The technique of symmetric aortoplasty with 3 patches (1 in each sinus) is described and compared with other methods. METHODS: Case reports were reviewed and follow-up was completed by contacting the patient's (pediatric) cardiologist. We aimed for a last follow-up visit, including Doppler echocardiographic studies, in a period no more than 12 months earlier than December 1997. Supravalvular aortic stenosis was discrete in 25 and diffuse with involvement of the aortic arch and arch vessels in 4 patients. Additional anomalies were bicuspid aortic valve (n = 5), coarctation (n = 3), ascending aortic aneurysm (n = 1), mitral valve insufficiency (n = 2), pulmonary valvular stenosis (n = 1), and peripheral pulmonary artery stenosis (n = 2). Eleven patients had Williams syndrome and 1 patient had Noonan syndrome. Symmetric aortoplasty with 3 patches (1 in each sinus) was used in 13 patients, whereas other nonsymmetric methods (1, 2, or Y-shaped patches) were used in 16 patients. Mean follow-up was 10.5 years (range: 4 months-36 years). RESULTS: All techniques adequately decreased the pressure gradient. Progression of preoperative aortic valve insufficiency or de novo regurgitation was not observed except in 1 patient in whom the patches inserted were too large. CONCLUSIONS: No difference could be demonstrated in outcome for any surgical technique; however, reconstruction of the aortic root with autologous pericardial patches in each sinus after transection of the aorta has the advantage of symmetry while restoring the normal aortic root anatomy.     

Pulmonary homograft: should it be used in the aortic position?

OBJECTIVE: Retrospective analysis was performed to determine the suitability of pulmonary homograft as an aortic valve substitute. METHODS: From January 1994 through June 1999, 147 patients (mean age, 32.2 +/- 17.3 years) underwent aortic valve replacement with either an aortic homograft (group 1: n = 103, 25 fresh antibiotic preserved and 78 cryopreserved) or a pulmonary homograft (group 2: n = 44, 11 antibiotic preserved and 33 cryopreserved). In group 1 a scalloped subcoronary technique was used in 64 patients, and a root replacement technique was used in 39 patients. In group 2 the scalloped subcoronary technique was used in 34 patients, and the root replacement technique was used in 10 patients. RESULTS: There were 131 operative survivors (group 1 = 91; group 2 = 40). Follow-up ranged from 2 to 62 months. In group 1 none of the patients had significant aortic regurgitation during the hospital stay. Three patients (all having undergone the scalloped subcoronary technique) had moderate aortic regurgitation after 6 to 32 months. In group 2, 10 patients (9 having undergone the scalloped subcoronary technique and 1 having undergone the root replacement technique) developed significant regurgitation: 2 intraoperatively, 5 in the early postoperative period before discharge from the hospital, and 3 during late follow-up 6 to 12 months postoperatively. Among the various risk factors analyzed for overall homograft failure, use of a pulmonary homograft was the single independent predictor of valve failure (odds ratio, 8.6; 95% confidence interval, 1.9-39; P =.006). CONCLUSION: Pulmonary homograft, when inserted by means of a scalloped subcoronary technique, is not a suitable aortic valve substitute.     

Anatomic reconstruction for recurrent aortic obstruction in infants and children

Background

Patients undergoing operative repair of aortic obstruction are at a lifelong risk of recurrent obstruction, and there is controversy regarding the optimal surgical technique. We have used an alternative strategy for recurrent aortic obstruction, typically involving anatomic reconstruction by means of a median sternotomy, and describe our techniques and results.

Methods

Twenty-one patients presented with recurrent aortic arch obstruction. Mean age and weight were 7.8 ± 5.4 years (range, 0.21 to 15.2 years) and 30.6 ± 21.8 kg (range, 3.6 to 90 kg), respectively. Recurrence involved the aortic arch to some degree in each case, as the mean preoperative transverse aortic arch z score was −2.9 ± 1.6 (range, −7.0 to 0.1). Thoracotomy was possible in 2 patients, using re-resection with end-to-end anastomosis (n = 1) and patch aortoplasty (n = 1). The remaining 19 patients required median sternotomy, cardiopulmonary bypass, and deep hypothermic circulatory arrest for complete relief of obstruction by aortic arch advancement (n = 10), patch aortoplasty (n = 8), or interposition grafting (n = 1).

Results

There was 1 hospital death. Invasive blood pressure monitoring revealed no residual arm-to-leg gradient in 19 patients and a 20-mm Hg gradient in 2 patients. There have been no late deaths. No patients have undergone subsequent aortic intervention, and all are asymptomatic up to 85 months postoperatively. Two patients are currently followed with a 10-mm Hg arm-to-leg blood pressure gradient.

Conclusions

Anatomic reconstruction for recurrent aortic obstruction can be safely accomplished in the majority of patients. We favor median sternotomy because of the ability of establishing cardiopulmonary bypass, the facility of anatomic reconstruction techniques, and the ability to repair concomitant cardiovascular lesions.     

Infant coarctation: a spectrum in clinical presentation and treatment

Between 1975 and 1985, 125 infants 2 to 365 days old (majority, 30 days old or less) with coarctation of the aorta underwent surgical repair. Forty-seven patients (38%) had severe congestive heart failure (CHF), metabolic acidosis, and poor systemic perfusion. The predominant operative technique was synthetic patch aortoplasty (100 patients); the remaining 25 had an end-to-end anastomosis. There were no operative deaths. Perioperative complications were minimized with the synthetic patch technique (less than 15%). For patients surviving at least 3 months after repair, the arm-leg systolic blood pressure gradient was relieved in 82% (71/87) of the patients having patch aortoplasty versus 65% (15/23) of the patients with end-to-end anastomosis. Although the rate of reoperation between the two groups was similar (patch, 5 [6%]; end-to-end, 3 [13%], two of the reoperations in the patch group were for preexisting hypoplastic transverse aortic arch. Late deaths (20 patients, 16%) were due to other major associated cardiac anomalies. Patch aneurysms have not occurred. Expedient use of synthetic patch aortoplasty has decreased perioperative complications, relieved coarctation gradients for CHF, increased early survival even in the presence of complex or associated cardiac anomalies, and has an acceptable rate of recurrent coarctation (6 to 13%).     

主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.     

Single-Stage Arterial Switch With Aortic Arch Enlargement for Transposition Complexes With Aortic Arch Obstruction

Background. Patients with transposition complexes and aortic arch obstruction are a surgical challenge with significant mortality. We have adopted an aggressive approach of concurrent aortic arch repair and arterial switch operation with excellent results.

Methods. Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair. One patient underwent a two-stage repair because of hemodynamic instability. The median age of repair was 27 days and the median weight was 3.5 kg. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 12 patients. One patient with severe subaortic stenosis underwent a modified Damus-Kaye-Stansel operation with concomitant aortic arch enlargement. The aortic arch was enlarged in 12 of 13 patients with a pulmonary homograft patch.

Results. There have been no early deaths and only one late death at 39 months postoperatively from hepatoblastoma. The mean follow-up is 42 months. There have been no reoperations for recurrent aortic arch obstruction. All survivors are currently well from a cardiac point of view.

Conclusions. Concomitant single-stage repair for transposition complexes with aortic arch obstruction achieves excellent survival and should be the surgical procedure of choice.     

Early repair of coarctation of the aorta.

BACKGROUND: Repair of coarctation of the aorta with hypoplasia and elongation of the proximal aortic arch is a technically demanding procedure with a substantial rate of recurrent stenosis at the coarctation repair site. In addition, a high incidence of hypertension has been reported in patients who underwent repair beyond infancy. PATIENTS AND METHODS: Between January 1991 and June 1997, 52 patients (34 neonates and 18 infants with a median age of 37 days; range 2 days to 8 months) with a mean peak systolic upper to lower extremity resting gradient of 33.5 +/- 18.9 mmHg underwent repair of aortic coarctation. The echocardiographically measured median diameter of the aortic arch immediately distal to the innominate artery was 5.4 mm (range 4.0 to 8.1 mm). Eight patients (15%) were considered hypertensive. In 41 patients, through a left thoracotomy, an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch. In 12 of these patients (who all had a hypoplastic and elongated aortic arch) this procedure was preceded by the construction of an extended side-to-side left carotid-subclavian arterioplasty. The remaining 11 patients, all with hypoplasia of the aortic arch, had concomitant complete repair of intracardiac anomalies through a median sternotomy. In 8 of these patients, in addition to anastomosis of the descending aorta to the undersurface of the proximal aortic arch, the ascending aorta and aortic arch were augmented with a pulmonary homograft patch. RESULTS: One neonate with associated Shone's syndrome died (2%) on the first postoperative day. There was no late mortality. Early postoperative complications included recurrent laryngeal nerve injury in 1 patient and prolonged chest tube drainage in 4 patients. At a median follow-up of 55 months (range 15 to 92 months), only 3 patients (5. 7%) developed a recurrent stenosis at the coarctation repair site. The remaining 48 patients are free of recurrent stenosis by echocardiography and clinical examination. None of the patients had systemic hypertension. CONCLUSIONS: 1. Coarctation repair consisting of resection of all ductal tissue with end-to-side anastomosis of the descending aorta to the undersurface of the (proximal) aortic arch, if necessary combined with a side-to side left carotid-subclavian arterioplasty, may lead to excellent results. 2. This technique can be applied with low mortality and morbidity via a left thoracotomy if the proximal aortic arch is at least 5 mm in diameter. 3. In view of the low mortality, the low incidence of restenosis at the coarctation repair site, and the absence of substantial morbidity including the development of hypertension, we advocate repair of aortic coarctation at neonatal age or in early infancy to avoid the detrimental sequelae of delayed repair of coarctation, in particular hypertension.     

The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair

BACKGROUND: Classic first-stage Norwood repair of hypoplastic left heart syndrome uses a homograft patch enlargement to obtain an unobstructed aorta and coronary arteries. Because of possible disadvantages of the homograft, such as lack of growth, degeneration and calcification, and availability, we have tried to repair the aorta without patch supplementation. METHODS: Between February 1993 and September 1997, 120 patients, aged birth to 47 days (median 4 days) and weighing 1.7 to 4.4 kg (median 3.1 kg), underwent first-stage palliation for hypoplastic left heart syndrome. The diameter of the ascending aorta ranged from 1.5 to 8.0 mm (median 3.0 mm). Eight patients had an aberrant right subclavian artery arising from the descending thoracic aorta. In 95 patients (group I), all duct tissue was excised and the descending aorta was anastomosed to the aortic arch, which had been opened back into the ascending aorta. Then to this confluence was anastomosed the proximal main pulmonary artery. In the remaining 25 patients (group II), continuity of the aortic arch was maintained and the repair was performed with a Damus-Kaye-Stansel anastomosis. The size of the systemic-to-pulmonary shunt was 3 mm in 48 patients, 3.5 mm in 70, and 4.0 mm in 2. RESULTS: Circulatory arrest time ranged from 19 to 105 minutes (median 54 minutes). A homograft patch was necessary for the arch reconstruction in 18 patients (15%); 9 group I patients (10%) and 9 group II (36%) (P =.001). There were 82 hospital survivors (68%); 69 group I patients (73%) and 13 group II (52%) (P =.04), 71 patients without a patch (70%) and 11 with a patch (61%) (P >.2). By multiple logistic regression, the aberrant right subclavian artery was a significant risk factor for hospital death (P =.008). There were 6 late deaths. Sixteen of 71 patients (23%) who underwent second-stage palliation had a neoaortic arch obstruction develop, with a peak gradient greater than 10 mm Hg; 14 group I patients (23%) and 2 group II (22%) ( P >.2), 15 without a patch (23%) and 1 with a patch (17%) (P >.2). Overall survivals were 57% at 1 year and 55% at 2 years. CONCLUSION: The modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome is possible in the majority of patients without the use of exogenous materials and does not result in an increased incidence of neoaortic arch obstruction. Repair of the aorta without patch supplementation may improve the potential for long-term growth of the new aorta.