Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect.

OBJECTIVE: Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for biventricular repair, although the optimal initial management strategy remains unknown. METHODS: From January 1991 through March 1999, 20 patients with aortic atresia or hypoplasia and ventricular septal defect underwent operation with the intent to achieve biventricular repair. Aortic atresia was present in 7 patients, and aortic valve hypoplasia was present in 13 patients. Among those patients with aortic hypoplasia, Z-scores of the aortic valve anulus ranged from -8.8 to -2.7. Associated anomalies included interrupted aortic arch (n = 12 patients), coarctation (n = 6 patients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 patient). Nine patients were staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular repair after a Norwood procedure. The conditions of 11 patients were corrected with a single procedure. RESULTS: Among the 9 patients who underwent staged repair, there were no deaths after the Norwood procedure and 1 death after biventricular repair. For the 11 patients who underwent a primary biventricular repair, there was 1 early death and 2 late deaths from noncardiac causes. Follow-up ranged from 1 to 85 months (mean, 28 months). Actuarial survival for the entire group was 78% +/- 10% at 5 years and was not significantly different between staged repair (89%) and primary biventricular repair (73%). CONCLUSIONS: Both primary and staged biventricular repair for patients with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit insertion and arch reconstruction have resulted in primary biventricular repair becoming our preferred approach.     

Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction

Background. Management of newborns with interrupted aortic arch (IAA) remains challenging. Associated severe left ventricular outflow tract obstruction (LVOTO) have often led to increased mortality with neonatal biventricular repair. We review our experience with an alternative approach for this complex surgical problem.

Methods. From May 1991 to June 1999, 28 neonates were treated for IAA. Thirteen of 28 neonates (46%) had type B IAA, ventricular septal defect (VSD) and severe LVOTO (Z value –2 to –7; mean –5 ± 1.7). Mean age was 8 days (3 to 23 days old) with average weight of 3.3 kg (2.4 to 4.2 kg). Eight of 13 (62%) had anomalous right subclavian artery. Ten of 13 (77%) had thymic aplasia and chromosome 22 region q11 deletion. All 13 patients were treated initially with a modified Norwood procedure.

Results. There were no perioperative deaths. Complications included 2 patients with recurrent arch stenosis treated with balloon dilatation. Two patients had systemic arterial shunt revision. Follow-up ranged from 2 to 99 months old (mean 39 months). There were 2 late deaths unrelated to any operation. Nine of 12 patients had a second stage palliation consisting of a bidirectional Glenn shunt. Six patients went on to have biventricular repairs (3 Ross-Konno, 2 Rastelli, 1 VSD closure with LVOT resection). One patient had a modified Fontan operation and 5 patients are awaiting potential biventricular repair.

Conclusions. Children with IAA and severe LVOTO may be managed by initial Norwood palliation with an excellent outcome likely. This initial “univentricular” approach has enabled eventual successful biventricular repair despite severe LVOTO.     

Repair of interrupted aortic arch: results after more than 20 years

Background. This study focused on the influence of concomitant anomalies, the individual surgical approach, and the probability for reinterventions.

Methods. Between 1975 and 1999, 94 patients with interrupted aortic arch were evaluated for short- and long-term results after surgical treatment.

Results. Interrupted aortic arch was associated mainly with a ventricular septal defect (85%) and left ventricular outflow tract obstruction (LVOTO, 13%). Mean follow-up was 6.7 years (median 6.9 years, 628.4 patient years). A single-stage operation was performed in 76 cases. Early mortality for two-stage procedures was 37% and late mortality was 26%, compared with single-stage procedures, with an early mortality of 12% and a late mortality of 20%, respectively. Early mortality in patients with additional LVOTO was 42% and late mortality was 50%. Freedom from reoperation at 5 years was 62%, and at 10 years was 49%. Reinterventions were performed mainly for residual arch stenosis, also with bronchus or tracheal compression, or LVOTO.

Conclusions. Arch continuity and repair of associated anomalies can be achieved with an acceptable overall risk in this often complex entity. Associated anomalies play an important role in the outcome. Single-stage repair with primary anastomosis of the arch should be the surgical goal. The long-term probability for reoperation is high.     

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.     

Left heart growth and biventricular repair after hybrid palliation

 Open in a separate windowOBJECTIVESWe evaluated the outcomes of biventricular repair after initial hybrid palliation performed in small infants with various forms of left ventricle hypoplasia.METHODSBetween September 2010 and January 2020, a total of 27 patients had biventricular repair after hybrid palliation at a median age of 11 days. Indications for the hybrid approach included growth promotion of the left ventricle outflow tract and/or the aortic valve in 14 patients and that of the left ventricle in 13 patients. Seven reinterventions and 7 reoperations were performed during the interstage period. Significant growth of left ventricle parameters was noted during the median interstage period of 62 days. Sixteen subjects had aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients had the Ross–Konno procedure; 5 patients underwent the Yasui procedure; and 1 patient had unbalanced atrioventricular septal defect and aortic arch repair.RESULTSTwenty-three patients (85.2%) are alive at a median follow-up of 3.3 years. Two and 3 patients died early and late after achieving biventricular circulation, respectively. There were 22 reinterventions and 15 reoperations after biventricular repair.CONCLUSIONSHybrid palliation can stimulate left heart growth in some patients with left ventricle hypoplasia. More patients may eventually achieve biventricular circulation than was initially thought. Additional interventions and operations are foreseeable. Despite ventricular rehabilitation, some patients with borderline left ventricles may develop restrictive physiology.     

Left heart hypoplasia and neonatal aortic arch obstruction: is the Rhodes left ventricular adequacy score applicable?

OBJECTIVE: Although the influence of small left heart structures on outcome of a biventricular repair in neonatal critical aortic stenosis is well documented, little is known about its effect in neonates with aortic arch obstruction and coarctation. The purpose of this study was to evaluate the influence of small left heart structures on early and late results of repair and the ability to achieve a biventricular repair in neonates with coarctation and aortic arch obstruction. PATIENTS: Neonates included in this study had a left ventricular adequacy score (as proposed by Rhodes and associates for critical aortic stenosis) that would have predicted a need for a univentricular (Norwood) repair. All were ductus dependent but had antegrade ascending aortic flow and a small but nonstenotic aortic valve (<30 mm Hg gradient). Twenty neonates aged 10 +/- 9 days were identified for the study with weights averaging 3. 1 +/- 0.6 kg. Selected left heart measurements obtained by preoperative echocardiography included the following: aortic anulus 5.3 +/- 0.3 mm, mitral anulus 8.4 +/- 1.0 mm, transverse aortic arch 3.4 +/- 0.6 mm, and left ventricular volume 25 +/- 4 mL/m2. All patients underwent coarctation repair by resection and extended end-to-end anastomosis to enlarge the transverse arch as needed. Three patients underwent simultaneous pulmonary artery banding because of a hemodynamically significant ventricular septal defect. These 3 patients have subsequently had their defects successfully closed without mortality. RESULTS: There were no early or late deaths at a follow-up of 38 +/- 16 months after the operation. Three patients (3/20, 15%) have had to undergo reintervention with balloon aortoplasty because of recurrent coarctation (gradient > 20 mm Hg) in 2 and resection of subaortic stenosis in 1. Late follow-up in the remaining patients reveals 1 with moderate subaortic stenosis (gradient = 43 mm Hg), 2 with mild aortic stenosis (gradient < 30 mm Hg), and 2 with mild to moderate mitral stenosis. At late follow-up, 16 patients (16/20, 80%) are completely free of symptoms and 4 (4/20, 20%) have mild residual symptoms. CONCLUSIONS: Biventricular physiology can be successfully achieved in neonates with small left heart structures and aortic arch obstruction with minimal mortality and excellent late functional results. Standard echocardiographic measurements used to predict the need for a univentricular repair in critical aortic stenosis are not valid for the neonate with aortic arch obstruction.     

Extra-anatomic aortic bypass via sternotomy for complex aortic arch stenosis in children

OBJECTIVE: Recurrent aortic narrowing after repair of aortic coarctation or interrupted aortic arch, as well as diffuse, long-segment aortic hypoplasia, can be difficult to manage. Extra-anatomic ascending aorta-descending aorta bypass grafting through a sternotomy is an alternative approach for this problem. METHODS: Since 1985, 19 patients aged 2 months to 18 years (mean 10.7 years) underwent extra-anatomic bypass with 10- to 30-mm Dacron grafts. The initial diagnosis was coarctation with hypoplastic arch in 15, interrupted aortic arch in 3, and diffuse long-segment aortic hypoplasia in 1. Seventeen of the children had a total of 22 previous operations: transthoracic interposition or bypass graft (n = 7), end-to-end anastomosis (n = 7), subclavian arterioplasty (n = 6), and synthetic patch (n = 2). The mean time from initial repair was 8.0 years (range 0.6-18 years). Three children had previous sternotomies. Cardiopulmonary bypass was avoided in all but 6 patients (5 with simultaneous intracardiac repairs). RESULTS: No hospital or late deaths occurred. On follow-up from 4 months to 14.7 years (mean 7.9 years), no reoperations for recurrent stenosis were performed. Two patients have arm-to-leg pressure gradients: 20 mm Hg at rest in 1 patient and a 60-mm Hg systolic exercise gradient with no resting gradient in the other. One patient required exclusion of an aortic aneurysm at the old coarctation repair site 13 years after extra-anatomic bypass. Three children had subsequent successful cardiac operations. CONCLUSIONS: Extra-anatomic bypass is an effective and relatively easy approach for selected cases of complex or reoperative aortic arch obstruction. It should be considered as an alternative operative technique for complex aortic arch reconstruction.     

Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome

OBJECTIVE: Aortic arch obstruction is a commonly associated problem in the Taussig-Bing anomaly. Between 1983 and 1995, 28 consecutive patients with Taussig-Bing anomaly underwent arterial switch operation with baffling of the left ventricle to neoaorta. Group A: 11/28 had associated aortic arch obstruction. Group B: 17/28 had isolated Taussig-Bing anomaly. We assessed whether the coexistence of subpulmonary ventricular septal defect and aortic arch obstruction pose an incremental risk factor. PATIENTS AND RESULTS: Group A: Mean age and weight were 1.4+/-1.3 months and 3.5+/-0.4 kg. The aortic arch obstruction included: hypoplasia (5/11), interruption (4/11) and discrete coarctation (2/11). Seven patients had a one-stage correction, and four had initial arch repair followed by arterial switch operation. There were no hospital deaths (CL 0-28%). Over a follow-up of 638 patient-months (mean 64+/-39), there have been no late deaths, and all patients are in New York Heart Association class 1. There have been three cases of recurrent aortic arch obstruction (two requiring reoperation, and one requiring balloon dilation). One patient has been reoperated upon for right ventricular outflow tract obstruction. The actuarial survival and freedom from reoperation rates at 6 years were 100% (CL = 66-100%) and 72.9% (CL=38-92%) respectively. Group B: Mean age and weight were 5.9+/-8.4 months and 5+/-2.1 kg. All patients had a one-stage operation. There were two early deaths (11.8%, CL = 1-36%) and one late death over a follow-up of 678 patient-months (mean 52+/-31). All survivors are in New York Heart Association class 1 and there have been no reoperations. The actuarial survival and freedom from reoperation rates at 6 years were 81% (CL = 56-93%) and 100% (CL = 76-100%) respectively. CONCLUSIONS: 1. Aortic arch obstruction has not adversely affected early or late survival (P>.05) or late functional class. 2. Patients with Taussig-Bing anomaly and aortic arch obstruction may have a higher reoperation rate than those with normal arch anatomy. 3. Taussing-Bing anomaly, with or without aortic arch obstruction, can be repaired with arterial switch operation during the neonatal period with good outcome.     

Extensive aortic reconstruction for aortic aneurysms in Marfan syndrome

Background. Marfan syndrome patients frequently develop aneurysms or dissections involving multiple segments of the aorta, and occasionally require staged replacement of the entire aorta. This study reviews the surgical outcome of patients with Marfan syndrome who underwent extensive aortic reconstruction. Extensive reconstruction is defined as reconstruction of more than two segments of the ascending, arch, descending thoracic, or abdominal aorta.

Methods. From March 1973 to December 1997, 101 patients with Marfan syndrome underwent aortic operation. Twenty-six patients (25.7%) had extensive aortic reconstruction. All 26 patients suffered from aortic dissection: 13 patients had Stanford type A and 13 had type B dissection. Twenty-three patients (88.4%) had annuloaortic ectasia and aortic regurgitation. Surgical procedures included composite valve graft replacement (n = 23, 88.4%), aortic arch reconstruction (n = 15, 57.7%), graft replacement of the descending thoracic aorta (n = 6, 23.1%), and graft replacement of the thoracoabdominal aorta (n = 16, 61.5%). Five patients (19.2%) had total thoracoabdominal aortic replacement, and three patients (11.5%) had replacement of the entire aorta. Twenty-one patients (80.8%) required multiple operations.

Results. Follow-up was complete in all patients. The 30-day survival rate was 88.5%. None of the survivors had paraplegia or paraparesis. The overall long-term survival rate was 88.5 ± 6% at 1 year, and 81.7 ± 9% at 9 years.

Conclusions. Aortic surgery prolongs survival in patients with Marfan syndrome, and currently there is a relatively low associated morbidity and mortality even for aggressive surgical treatment.     

自体肺动脉组织一期矫治主动脉弓病变合并心内畸形

目的 探讨自体肺动脉组织一期矫治主动脉弓病变合并心内畸形的手术方法和治疗效果.方法 1993年4月至2009年11月,共25例主动脉弓病变合并心内畸形的患儿接受了一期矫治手术.男性17例,女性8例;年龄26 d～6.5岁,平均11个月;体质量3.0～14.5 kg,平均12.4 kg.其中6例患儿为主动脉弓缩窄,6例为主动脉弓中断,13例为主动脉弓发育不良.手术技术包括切开肺动脉前壁,切除动脉导管,应用部分自体肺动脉壁重建主动脉弓,重建肺动脉,并同时矫治合并心内畸形.结果 24例患儿术后恢复顺利,1例患儿死于围手术期肺动脉高压危象.随访心脏超声及心脏CT检查证实主动脉弓重建及心内畸形矫治满意.术后未发生神经系统及其他并发症.随访过程中,患儿生长发育正常,主动脉弓无再狭窄.结论 自体肺动脉具有生长潜力且柔韧性好,是一种理想的重建主动脉弓的材料.一期矫治手术治疗先天性主动脉弓病变合并心内畸形可以得到满意的结果.     

Transverse arch hypoplasia may predispose patients to aneurysm formation after patch repair of aortic coarctation

BACKGROUND: Thoracic aortic aneurysm after patch repair of aortic coarctation is a potentially lethal complication. We hypothesized that transverse arch hypoplasia in association with patch repair of aortic coarctation predisposes aneurysm formation. METHODS: A retrospective analysis was performed on all patients undergoing isolated aortic coarctation repair at the University of Virginia Health Systems between 1970 and 1995. Of 244 repairs, 38 patients underwent patch repair. These 38 patients were divided into two groups. The aneurysm group (n = 15) had aortic aneurysms develop at the repair site, which required aneurysmectomy. The nonaneurysm group (n = 23) did not have any aneurysms develop. Univariate analysis and Fisher's exact text were used to identify factors that independently predict aneurysm formation. RESULTS: Univariate analysis demonstrated aortic arch hypoplasia associated with patch repair independently predicts future aneurysm formation (p < 0.01). Patients who had an aneurysm develop also had a similar incidence of bicuspid aortic valves, mild restenosis, and late hypertension compared with patients in the nonaneurysm group. CONCLUSIONS: Aneurysm formation after patch repair of aortic coarctation develops into a subgroup of patients. Aortic arch hypoplasia associated with coarctation independently predicts future aneurysm formation.     

Mid- to Long-Term Results of the Two-Stage Approach for Type B Interrupted Aortic Arch and Ventricular Septal Defect

Background. Type B interrupted aortic arch with ventricular septal defect is a complex congenital heart defect that may have associated left ventricular outflow tract obstruction. Surgical management has evolved from a two-stage approach to the currently favored single-stage approach. The following data summarize our experience with the two-stage approach over a 15-year period.

Methods. Between 1980 and 1995, 27 consecutive patients with type B interrupted aortic arch and ventricular septal defect underwent surgical management using the two-stage approach. There were 15 girls and 12 boys; 21 patients had the DiGeorge syndrome.

Results. Stage I was performed at a median age of 4 days. Twenty-six (96%) of 27 patients survived first-stage palliation. One patient survived stage I palliation but died before undergoing stage II. Twenty-five patients underwent second-stage repair at a median age of 6 weeks (range, 1 to 46 weeks). There were 2 early deaths and 1 late death. Actuarial analysis demonstrates 1- and 5-year survival rates of 85% and 81%, respectively. Twenty-two survivors have been followed up for an average of 8 ± 2 years. Freedom from reoperation for arch graft enlargement has been 86% at 3 years and 55% at 5 years. Freedom from reoperation for left ventricular outflow tract obstruction has been 82% at both 3 and 5 years.

Conclusions. The two-stage approach can achieve good mid- to long-term palliation of patients with type B interrupted aortic arch and ventricular septal defect. These results should provide a reference from which to gauge the long-term success of the single-stage approach.     

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.     

Surgical treatment of acute type A dissection: is rupture a risk factor?

Background. The purpose of this study was to evaluate the significance of aortic rupture on clinical outcome in patients after aortic repair for acute type A dissection.

Methods. One hundred and twenty patients underwent aortic operations with resection of the intimal tear and open distal anastomosis. Median age was 60 years (range 16 to 87); 78 were male. Thirty-six patients had only ascending aortic replacement, 82 had hemiarch repair, and 2 had the entire arch replaced. Retrograde cerebral perfusion was utilized in 66 patients (53%). Rupture defined as free blood in the pericardial space was present in 60 patients (50%). Univariate and multivariate analyses were performed to assess the risk factors for mortality and neurologic dysfunction.

Results. Overall hospital mortality rate was 24.2% ± 4.0% (± 70% confidence level) but did not differ between patients with aortic rupture or without (p = 0.83). The incidence of permanent neurologic dysfunction was 9.4% overall, 10.5% with rupture and 8.3% without rupture (p = 0.75). Multivariate analysis revealed absence of retrograde cerebral perfusion and any postoperative complication as statistically significant indicators for in-hospital mortality (p < 0.05). Overall 1- and 5-year survival was 85.3% and 33.7%; among discharged patients, survival in the nonruptured group was 89% and 37%, versus 81% and 31% in the ruptured group (p = 0.01).

Conclusions. Aortic rupture at the time of surgery does not increase the risk of hospital mortality or permanent neurologic complications in patients with acute type A dissections. However, aortic rupture at the time of surgery does influence long-term survival.     

Early palliation of univentricular hearts with subaortic stenosis and ventriculoarterial discordance. The arterial switch option.

The optimal Fontan-type operation greatly depends on appropriate initial palliation. Several surgical techniques have been used in infancy to palliate patients with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis. The two most common are pulmonary artery banding and Damus-Norwood procedures. Palliative arterial switch operation is another surgical option that was used in this early series of seven infants. The principle of this operation is to "switch" the subaortic obstruction into a subpulmonary obstruction; the coronary artery relocation on the large pulmonary trunk creates a harmonious aortic root and the connection of the rudimentary ventricular chamber to the pulmonary artery trunk creates a natural protection of the pulmonary vascular bed through the restrictive bulboventricular foramen. Seven infants with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis underwent a palliative arterial switch operation. All infants had an associated aortic arch obstruction of various degrees, including one with interrupted aortic arch, five with coarctation with severe arch hypoplasia, and one with isolated arch hypoplasia. There were three with double-inlet left ventricle, three with tricuspid atresia, and one with transposition of the great arteries with ventricular septal defect and severe right ventricular hypoplasia. The subaortic obstruction was patent at birth in five patients who underwent a palliative switch operation in the first 2 months of life, and rapidly occurred following a previous neonatal pulmonary artery banding associated with arch repair in two patients who underwent a switch operation at 5 and 8 months of age, respectively. The operation includes aortic arch repair without prosthetic material, an atrial septectomy, and the arterial switch. An associated pulmonary shunt was required in five patients and a pulmonary artery banding in one. There was one early death in a patient with [S,L,L] anatomy and congenital atrioventricular block, leading to an early mortality of 14% (95% confidence limits: 1% to 28%). There was one late death. Four survivors are waiting for a Fontan-type procedure, and one survivor had satisfactory right ventricular growth. Early palliative arterial switch operation offers several advantages: reconstruction of a harmonious aortic root, natural protection of the pulmonary bed through the restrictive bulboventricular foramen, prevention of deleterious myocardial hypertrophy, and arch reconstruction without the introduction of a foreign material. This aggressive technique may provide a satisfactory palliation in infants with univentricular hearts and ventriculoarterial discordance, when the bulboventricular foramen/aortic anulus ratio is less than 0.8 or when the subaortic stenosis is severe enough to be associated with an arch obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Aortic arch repair with right brachial artery perfusion

Background. To determine the effectiveness of unilateral selective cerebral perfusion for aortic arch repair and to discuss possible modifications to enhance technical simplicity.

Methods. In the period between January 1996 and April 2001, 104 patients underwent aortic arch repair with the use of right brachial artery low flow (8 to 10 mL/kg per minute) antegrade selective cerebral perfusion under moderate hypothermia (26°C). Mean patient age was 52 ± 12 years. Sixty-four patients presented with Stanford type A aortic dissection, including 12 with acute dissection; 38 patients had aneurysmal dilatation of the ascending aorta and aortic arch; and 2 patients had isolated arch aneurysm. Ascending and partial arch replacement was performed in 50 patients; ascending and total arch replacement in 33 patients; ascending and descending arch replacement in 19 patients; and isolated arch replacement in 2 patients.

Results. Mean antegrade cerebral perfusion time was 39 ± 22 minutes. One patient with acute proximal dissection died because of cerebral complications. One other patient developed right hemiparesis, which resolved during the second postoperative month without sequela. Other than these 2 cases (1.9%), no other neurologic event was observed.

Conclusions. The technique of low flow antegrade selective cerebral perfusion through the right brachial artery may be used for a vast majority of aortic aneurysms and dissections requiring arch repair. This technique does not necessitate deep hypothermia, requires shorter cardiopulmonary bypass and operation times, has the advantage of simplicity, provides optimal vascular repair without time restraints and, in terms of clinical results, is as safe as other techniques for cerebral protection.     

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.     

Neonatal aortic arch reconstruction avoiding circulatory arrest and direct arch vessel cannulation.

BACKGROUND: Aortic arch reconstruction in neonates routinely requires deep hypothermic circulatory arrest. We reviewed our experience with techniques of continuous low-flow cerebral perfusion (LFCP) avoiding direct arch vessel cannulation. METHODS: Eighteen patients, with a median age of 11 days (range 1 to 85 days) and a mean weight of 3.2 +/- 0.8 kg, underwent aortic arch reconstruction with LFCP. Seven had biventricular repairs with arch reconstruction, 9 underwent the Norwood operation and 2 had isolated arch repairs. In 1 Norwood and 7 biventricular repair patients, LFCP was maintained by advancing the cannula from the distal ascending aorta into the innominate artery. In 8 of 9 Norwood patients, LFCP was maintained by directing the arterial cannula into the pulmonary artery confluence and perfusing the innominate artery through the right modified Blalock-Taussig shunt fully constructed before cannulation for cardiopulmonary bypass. In 2 patients requiring isolated arch reconstruction, the ascending aorta was cannulated and the cross-clamp was applied just distal to the innominate artery. RESULTS: LFCP was maintained at 0.6 +/- 0.2 L x min(-1) x m(-2) for 41.0 +/- 13.9 minutes at 18.5 degrees C +/- 1.1 degrees C. In 10 of the 18 patients, blood pressure during LFCP was 15 +/- 8 mm Hg remote from the innominate artery (left radial, umbilical or femoral arteries). In 8 of the 18 patients, right radial pressure during LFCP was 24 +/- 10 mm Hg. The mean mixed-venous saturation was 79.8% +/- 10% during LFCP. Two patients had preoperative seizures, whereas none had seizures postoperatively. One patient died. CONCLUSIONS: Neonatal aortic arch reconstruction is possible without circulatory arrest or direct arch vessel cannulation. These techniques maintained adequate mixed-venous oxygen saturations with no associated adverse neurologic outcomes.     

Arch reconstruction without circulatory arrest: Scientific basis for continued use and application to patients with arch anomalies

Aortic arch hypoplasia is a common constituent of congenital heart disease. While repair of these lesions has been performed routinely during deep hypothermia and circulatory arrest, new approaches are emerging. One such approach, regional low-flow perfusion, will be described here. This technique exploits the anticipated modified Blalock-Taussig shunt as a perfusion conduit. With control of the brachiocephalic vessels and the descending thoracic aorta, circulatory support can be provided to the neonate with exposure identical to that obtained by circulatory arrest. While first applied to children undergoing the Norwood operation for hypoplastic left heart syndrome, this technique has recently been applied to children requiring complex arch surgery in the setting of biventricular repair. To date, 36 neonates requiring arch reconstruction (27 Norwood operations, 9 biventricular repairs) have been supported with regional low-flow perfusion. Thirty-day and hospital discharge survival has been 74% (20/27) for neonates undergoing Norwood operation, and 88% (8/9) for those undergoing biventricular repair. We will review the operative technique, methodologies, and clinical studies that led us to conclude that regional low-flow perfusion provides cerebral, as well as somatic, circulatory support to the neonate undergoing arch reconstruction. Copyright © 2002 by W.B. Saunders Company     

Aortic valve sparing operations: an update

Background. Aortic valve sparing operations in patients with ascending aorta and/or aortic root aneurysms have been performed for a decade in our institution. Initially only patients with normal aortic valve leaflets had these operations, but more recently we utilized them in patients with prolapse of a single leaflet and in those with a bicuspid aortic valve. This article is an update on the clinical results of these operations.

Methods. From May 1988 to December 1997, 126 patients with ascending aorta and/or aortic root aneurysms and aortic insufficiency underwent replacement of the ascending aorta with reconstruction of the aortic root and preservation of the native aortic valve. There were 85 men and 41 women, with a mean age of 54 years (range, 14 to 84). Thirty-two patients had the Marfan syndrome; 17 patients had acute and 10 had chronic type A aortic dissection; 23 had a transverse arch aneurysm; 26 had coronary artery disease, and 8 had mitral regurgitation. The aortic valve sparing operation consisted of simple adjustment of the sinotubular junction in 33 patients, adjustment of the sinotubular junction and replacement of one or more aortic sinuses in 60, and reimplantation of the aortic valve in a tubular Dacron (C.R. Bard, Haverhill, PA) graft in 33. Fifteen patients also had repair of aortic leaflet prolapse. Only 4 patients had a bicuspid aortic valve.

Results. There were 3 operative deaths due to cardiac failure. Patients were followed from 2 to 117 months, with a mean of 31. There were 11 late deaths: 7 cardiovascular and 4 from unrelated causes. The actuarial survival was 72 ± 8% at 7 years. Two patients required aortic valve replacement; the freedom from aortic valve replacement was 97 ± 2% at 7 years. Doppler echocardiography revealed absent, trivial or mild aortic insufficiency in most patients; only 9 patients had moderate aortic insufficiency.

Conclusions. Aortic valve sparing operations are feasible in most patients with ascending aorta and/or aortic root aneurysms who have normal or near normal aortic leaflets. The functional results of the repaired aortic valve are excellent, and the repair appears to be durable.