Transposition of the great arteries associated with ventricular septal defect: surgical results and long-term outcome.

OBJECTIVES: To identify potential risk factors influencing early and late outcome following the arterial switch operation (ASO) for transposition of the great arteries associated with ventricular septal defect including double-outlet right or left ventricle. METHODS: All patients who underwent ASO in our department until August 2000 (n=105) were included in this study. There were 77 transpositions of the great arteries with ventricular septal defect, 22 Taussig-Bing hearts and six patients with double-outlet morphology. The median age at operation was 24 days. Aortic arch obstruction was present in 25 patients; in 13 of these patients, a repair with aortic arch reconstruction was done before ASO. The usual coronary artery pattern was present in 59% of the patients. In six patients, we found an intramural course of at least one coronary artery. The ventricular septal defect was closed with a patch through the right atrium (n=35), the aorta (n=25), the pulmonary artery (n=25) or the right ventricle (n=3); in 17 patients a combined approach was necessary. RESULTS: There were five hospital deaths (4.7%, 95% confidence limit 2-11%). The median duration of follow-up was 72 months. Fourteen patients underwent 15 reoperations 33 months after repair (median), eight for right ventricular outflow tract obstruction or neopulmonary stenosis. Four late deaths occurred, two due to complications related to coronary artery anomalies. Statistical analysis revealed no significant risk factor whatsoever correlating with death or need for reoperation. Survival after 12 years was 91.6%, and freedom from reoperation was 82.6%. Latest follow-up data showed that 13% of patients were in NYHA class II and/or required medical treatment; 87% were in NYHA class I. CONCLUSIONS: ASO associated with patch closure of ventricular septal defect can be performed early in life with a low risk of mortality (<5%), low incidence of reintervention (<15%) and promising long-term outcome.     

Does left ventricular outflow tract obstruction influence outcome of interrupted aortic arch repair?

BACKGROUND: In previous studies left ventricular outflow tract obstruction (LVOTO) has been recognized as an important factor affecting survival and reoperation probability in patients having repair of an interrupted aortic arch (IAA). METHODS: All 72 patients who underwent operation for IAA from January 1, 1985 to June 30, 1997 were reviewed. The presence or absence of LVOTO was noted and the immediate and long-term results were analyzed. RESULTS: Type A IAA was found in 23 patients and type B IAA in 49 patients. Anomalous right subclavian artery was noted in 15 patients, all of whom had type B IAA. LVOTO was identified in 36 patients before arch repair and was associated with the presence of type B IAA and anomalous right subclavian artery (p = 0.02 and 0.007, respectively). There were 2 hospital deaths (within 30 days) for a mortality of 2.8% (confidence limit [CL] = 0% to 6.6%). There were 7 late deaths over 3,737 patient-months of follow-up (9.7%, CL = 2.9% to 16.6%). Actuarial survival for the whole cohort was 84.8% (CL = 73.2% to 94.4%) at 12 years. There was 87% 10-year survival (CL = 60% to 93%) for patients with LVOTO at presentation compared with 83% (CL = 62% to 92%) for patients without LVOTO (p = 0.85, hazard function 0.87). Twenty-eight patients have required at least one reoperation. The use of conduits to restore right ventricular to pulmonary artery continuity predicted the need for reoperation (p = 0.0001). Patients with presence of a nonseptatable heart were also more likely to need reoperation (p = 0.027) when compared to the rest of the cohort. Freedom from reoperation was 47.3% (CL = 30% to 62%) at 12 years. In patients with LVOTO, freedom from reoperation (55% at 10 years, CL = 33% to 72%) was not significantly different from those patients without LVOTO at presentation (29% at 10 years, CL = 7% to 56%; p = 0.97, hazard function 0.7). Actuarial freedom from recurrent arch obstruction requiring reintervention was 82.7% (CL = 66.7% to 98.7%) at 12 years. CONCLUSIONS: Neonatal complete repair of patients with IAA is possible with low mortality. LVOTO was not a significant risk factor for hospital or late death in patients having complete repair of IAA during this period. The use of conduits for right ventricular to pulmonary artery continuity and the presence of a nonseptatable heart are important risk factors for further operation and will continue to provide added morbidity to these patients.     

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.     

Incidence and results of reoperations following the Senning operation: 27 years of follow-up in 314 patients at a single center

OBJECTIVES: Atrial switch procedures for transposition of the great arteries are associated with reoperations mainly for systemic ventricular dysfunction and baffle complications. This study aims at identifying the results of reoperations following the Senning operation. METHODS: Records of 314 hospital survivors who had undergone the Senning operation were reviewed for details concerning cardiac reoperations. RESULTS: Reoperations were required in 32 patients (systemic ventricular failure n=12, baffle complications n=11, left ventricular outflow tract obstruction n=7, aortic coarctation n=2) during a mean follow-up time of 18.2+/-5.7 years. Freedom from reoperation and survival at 25 years was 88.0+/-2.1% and 90.9+/-2.3%, respectively. Among patients who underwent reoperation for systemic ventricular failure (arterial switch and Senning take-down without prior pulmonary artery banding n=2, with prior banding n=3, banding without conversion n=4, tricuspid valve repair n=3), two patients died at the time of arterial switch and Senning take-down, and two patients died 4 and 16 months after tricuspid valve repair, respectively. Mean follow-up time after the first reoperation was 7.7+/-5.9 years. Survival after reoperation for systemic ventricular failure at 30 days, 1 year, and 10 years, was 91.7+/-8.0%, 83.3+/-10.8%, and 64.8+/-14.3%, respectively. Survival after reoperation for baffle complications and left ventricular outflow tract obstruction at 10 years was 85.7+/-13.2% and 83.3+/-15.2%, respectively. CONCLUSIONS: Reoperations following the Senning operation are rare. Reoperations for baffle complications or left ventricular outflow tract obstruction can be performed with good results in the mid-term. However, reoperations for systemic ventricular failure are demanding, and are associated with a high operative and mid-term mortality.     

Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly

BACKGROUND: This study evaluates the results of the arterial switch operation for early total repair of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing heart). METHODS: From 1986 through April 2003, 27 patients with Taussig-Bing anomaly underwent arterial switch operation. Twenty patients were neonates (n = 11) or infants younger than 3 months (n = 9). Obstruction of aortic arch (n = 19) or subaortic right ventricular outflow tract obstruction (n = 20) and unusual coronary artery patterns (n = 19) were common. Total correction as a single procedure was performed in 21 patients. Events are depicted by Kaplan-Meier curves. RESULTS: There was 1 patient hospital death at 2 months after repair. One patient died late that was not cardiac related. Survival was 92% +/- 6% at 8 months and remained constant thereafter. Four patients underwent reoperation (1 for residual aortic arch obstruction and 3 for subvalvular and valvular pulmonary stenosis). Freedom from reoperation decreased to stabilize at 83% +/- 8% after 2 years. The risk to have right ventricular outflow tract obstruction develop was 33% +/- 10% at 1 year, increasing slowly and leveling out at 57% +/- 12% at year 5 and thereafter. Statistical analysis revealed no significant risk factor for death or need for reoperation. CONCLUSIONS: The Taussig-Bing anomaly should be corrected in the neonatal period or in early infancy by arterial switch operation, closure of the ventricular septal defect, and simultaneous correction of associated cardiovascular anomalies as a one-stage procedure. Right ventricular outflow tract obstruction often complicates the postoperative course and is the main cause for reintervention.     

Reparative operations for interrupted aortic arch with ventricular septal defect

From January, 1975, through September, 1982, 24 infants underwent primary or staged repair of interrupted aortic arch (IAA) with ventricular septal defect (VSD). Seven patients had IAA type A and 17 patients had type B. Eleven of the patients, median age 5 days, underwent staged operations and 13 infants, median age 6 days, underwent primary repair. Palliation was by tube graft interposition (six), subclavian-aortic anastomosis (three), left carotid-aortic anastomosis (one), or end-to-side aortic anastomosis (one) combined with pulmonary artery banding (eight) or early VSD closure. With palliation, there were three (27%) early deaths among the eleven patients and one (13%) late death among the eight remaining. Delayed repair at 5 days to 14 months (median 7 months) in seven patients incurred three (43%) early and no late deaths. Primary repair in 13 patients consisted of VSD closure combined with graft interposition (12) or end-to-side aortic anastomosis (one), with three (23%) early and no late deaths. Nine of 14 survivors had hemodynamic evaluation by catheterization 1 to 3 years following repair. None had a significant residual VSD or pressure gradients between the ascending and thoracic aorta. Six had subaortic stenosis, two mild (gradient less than 20 mm Hg) and four severe (gradient greater than 50 mm Hg), necessitating operation. Results of operations in neonates with IAA continue to improve. Essential in management is an awareness that subaortic stenosis and hypocalcemia may be accompaniments of this anomaly. Based on these data, we prefer primary repair for IAA with VSD.     

Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival,patterns of ventricular recovery and late outcome

BACKGROUND: To determine the early and late outcomes of patients presenting with anomalous left coronary artery from the pulmonary artery who had repair by aortic reimplantation. METHODS: From January 1952 to July 2000, 67 patients presented with anomalous coronary artery from the pulmonary artery. Forty-seven patients who had repairs performed by aortic reimplantation are the subject of this study. The median age at repair was 7.7 months. Before repair, 10 infants (21%) presented in extremis requiring ventilatory and inotropic support, and 38 infants (80%) presented in heart failure. Autologous pericardial hood coronary arterioplasty was used in 4 patients, and concomitant mitral valve repair was used in 1 patient. RESULTS: Hospital survival was 92%. Five children required postoperative extracorporeal membrane oxygenation for a median of 4 days (range, 2 to 8 days). Patients who had extracorporeal membrane oxygenation were significantly more likely to have presented in critical condition (40% vs 3% if no extracorporeal membrane oxygenation; p = 0.006) or with ventricular arrhythmias (67% vs 7%; p = 0.027), to have presented with significantly lower preoperative repair median ejection fraction (10%, n = 5 vs 40%, n = 38; p = 0.01) or to have presented with more severe left ventricular dilatation (p = 0.03). Within a 15-year or less follow-up (mean, 4.7 years) there were no late deaths. Kaplan-Meier survival was 91% at 5 years, and freedom from reoperation was 93% at 10 years. At late follow-up, echocardiography demonstrated significant improvements in mean ejection fraction (64% +/- 9% vs 33% +/- 21% preoperatively, p < 0.0001); moderate mitral regurgitation (9% vs 38% preoperatively, p < 0.02); and wall motion abnormalities (15% vs 81% preoperatively, p < 0.002). The ratio of measured left ventricular end-diastolic dimension to the 95th percentile of normal declined from 1.4 +/- 0.3 to 1.0 +/- 0.1 (p < 0.0006). Children who had extracorporeal membrane oxygenation had normal ejection fractions and ventricular dimensions at follow-up (n = 3). Repeated measures of mixed linear regression analysis demonstrated that normalization of ejection fraction and left ventricular function occurred within 1 year of repair. Improvements in mitral regurgitation lagged behind normalization of ejection fraction and left ventricular dilatation. CONCLUSIONS: Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation yields excellent early survival and late functional outcomes even in critically ill infants.     

Primary repair for aortic arch obstruction associated with ventricular septal defect

Background

The aim of the present study was to evaluate the current outcome and reoperation rate after applying a one-stage correction strategy for interrupted aortic arch (IAA) with ventricular septal defect (VSD) and also for aortic coarctation and hypoplastic aortic arch (CoA-HyAA) with VSD beginning September 1999.

Methods

Twenty-four consecutive patients with IAA (n = 12) or CoA-HyAA (n = 12) with VSD underwent early one-stage correction. Patients' mean age was 12 days (range, 2 to 188); mean weight was 3.6 kg (range, 2.1 to 7.3), 6 patients were less than 2.5 kg. Three IAA were type A, 5 type B1, 3 type B2, and 1 type C. Associated anomalies included a large VSD in all, left ventricular outlet tract obstruction in 5, transposition of the great arteries, aortopulmonary window, persistent truncus arteriosus, and double-outlet right ventricle in 1 patient. Selective brain perfusion through innominate artery and selective coronary perfusion through aortic root during aortic arch reconstruction was used in all patients. Mean follow-up was 2.2 ± 0.9 years.

Results

There was no early, no late mortality, and no postoperative neurologic complications. Mean crossclamp duration was 72 ± 32 minutes, lowest temperature 22.8 ± 4°C and selective brain and coronary perfusion duration was 34 ± 13 minutes. Eighteen patients required delayed sternal closure at 1.7 days postoperatively. New perioperative management reduced the overall morbidity. Four patients after IAA plus VSD repair developed aortic arch restenosis and were successfully treated by balloon dilatation. One patient with d-TGA underwent right ventricular outflow tract reconstruction of right ventricular outlet tract obstruction 7 months after the initial repair. Pressure gradients across the anastomosis at most recent follow up were less than 10 mm Hg. All patients are asymptomatic and are developing normally.

Conclusions

One-stage complete correction is feasible in newborns with aortic arch obstruction with VSD. Complex cardiac anatomy presents no additional risk for the procedure. The early one-stage correction yields excellent surgical results and good functional outcome.     

One-stage total repair of aortic arch anomaly using regional perfusion.

OBJECTIVE: Primary repair of aortic arch obstructions and associated cardiac anomalies is a surgical challenge in neonates and infants. Deep hypothermic circulatory arrest prolongs myocardial ischemia and might induce cerebral and myocardial dysfunction. METHODS: From March 2000 to December 2005, 69 neonates or infants with aortic arch anomaly underwent one-stage biventricular repair with continuous cerebral perfusion in the presence of a nonworking beating heart using the dual perfusion technique on the innominate artery and aortic root. Preoperative diagnoses of arch anomaly comprised aortic coarctation (n=54) or an interrupted aortic arch (n=15). Combined anomalies were ventricular septal defect (n=52), anomalous origin of the right pulmonary artery from ascending aorta (n=3), hypoplastic left heart syndrome (n=2), truncus arteriosus (n=2), atrioventricular septal defect (n=2), double outlet right ventricle (n=1), total anomalous pulmonary venous return (n=1), partial anomalous pulmonary venous return (n=1), and aortic stenosis (n=1). RESULTS: The mean regional perfusion time was 27.8+/-9.8 min. There was no operative mortality. Postoperative low cardiac output was present in four patients (5.8%). A neurologic complication was noted in one patient (1.5%) who developed transient chorea, but recovered completely. During 32.8+/-17.5 months of follow-up, one late death (1.5%) occurred. There was neither reoperation associated with arch anomaly nor recoarctation except in one patient. One patient developed left main bronchial compression necessitating aortopexy. CONCLUSIONS: One-stage total arch repair using our regional perfusion technique is an excellent method that may minimize neurologic and myocardial complications without mortality. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in neonates and infants.     

Intermediate-term survival and functional results after arterial repair for transposition of the great arteries.

An assessment of late morbidity and mortality is essential before arterial repair can be considered truly corrective for patients with transposition of the great arteries. We describe the early and intermediate-term results in 126 patients who underwent arterial repair. Operation was performed at a median age of 6 days, with 76 patients operated on within the first 7 days of life. Coronary artery anatomy differed from the usual arrangement in 37 patients. Simultaneous procedures included ventricular septal defect closure (35) and repair of interrupted aortic arch (2) or coarctation (5). Hospital mortality was seven of 126 (5.5%), with three deaths among the most recent 100 patients (3%). There were one late, noncardiac death and one late death after reoperation. Reoperation for pulmonary artery stenosis was required in 10 of the first 63 patients (16%), all of whom underwent pulmonary artery reconstruction with separate patches for closure of the coronary excision sites. Of the last 63 patients, all of whom underwent pulmonary artery reconstruction with a single pantaloon-shaped pericardial patch, one (2%) required reoperation for pulmonary artery stenosis. Doppler flow studies and echocardiography performed in 115 of 119 surviving patients at a mean of 12 months after repair demonstrated normal left ventricular function, minimal left ventricular outflow gradients, and no more than trivial aortic regurgitation. Peak gradient across the right ventricular outflow tract was 19 +/- 3 mm Hg in patients with separate pulmonary artery patches and 5 +/- 2 mm Hg in those with a single pantaloon patch (p = 0.0001). Follow-up is 96% complete from 1 month to 8 years after operation (mean 2.5 years). The actuarial survival rate at 5 years, including operative mortality, was 92%. All patients are in sinus rhythm, and none requires antiarrhythmic medications. These data suggest that pulmonary artery reconstruction with a single pantaloon patch may be associated with a decreased requirement for reoperation. Intermediate-term survival and functional results are excellent after arterial repair for transposition of the great arteries.     

Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.     

Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery

BACKGROUND: This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery. METHODS: Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months). RESULTS: Fifteen-year actuarial survival was 92.9% +/- 4.9% for coronary transfer, 40.0% +/- 21.9% for subclavian interposition, and 89.9% +/- 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% +/- 7.4%). Left ventricular shortening fraction increased from 17.3% +/- 6.3% before operation to 34.1% +/- 4.6% at last follow-up (p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r(2) = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% +/- 5.2%) and best longitudinal shortening fraction recovery (36.4% +/- 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors. CONCLUSIONS: Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.     

Truncus arteriosus repair: outcomes, risk factors, reoperation and management.

OBJECTIVES: Truncus arteriosus (TA) continues to be associated with significant morbidity and mortality, but there have been clinically significant improvements with early repair. METHODS: Sixty patients underwent physiological correction of TA between November 1978 and January 2000. The average age was 76 days (range, 3 days--20 months). Associated cardiac anomalies were frequently encountered, the most common being severe truncal valve regurgitation (n=7), interrupted aortic arch (n=6), coronary artery anomalies (n=6), non-confluent pulmonary arteries (n=4), and total anomalous pulmonary venous return (n=1). Truncal valve replacement was performed initially or subsequently in seven patients with severe regurgitation (mechanical prostheses in six patients and a cryopreserved aortic homograft in one patient). Right ventricle--pulmonary artery continuity was established with an aortic (n=16) or pulmonary homograft (n=32) in 48 patients, a Dacron polyester porcine valved conduit in five, a non-valved polytetrafluoroethylene (PTFE) tube in three, direct anastomosis to the right ventricle with anterior patch arterioplasty in three, and a bovine jugular venous valve conduit in one patient. RESULTS: There were ten hospital deaths (17%; 70% confidence limit, 7--25%). Multivariate and univariate analyses demonstrated a relationship between hospital mortality and associated cardiac anomalies. In the 43 patients without these associated cardiac anomalies, the early survival was 91% (group I). In the 17 patients with one or more of these risk factors, the survival was 71% (group II, P=0.002). There was one late death. Twenty-three patients (46%) required reoperation for right ventricular outflow tract (RVOT) obstruction at a mean follow-up time of 59.1 months. In 23 patients, the RVOT reconstruction was performed with a PTFE monocusp, and six patients had of a variety of replacement conduits inserted. Postoperatively, there were 34 (68%) patients in New York Heart Association functional class I and 16 (32%) in class II. Twenty-eight surviving patients are reported as doing well without any medication. The freedom of reoperation in the 39 hospital survivors (group I) without risk factors was 64% at 7 years; and 36% at 10 years in the 11 patients (group II) surviving with risk factors. CONCLUSIONS: Associated cardiac anomalies were risk factors for death after the repair of TA. In the absence of these associated lesions, TA can be repaired with an excellent surgical outcome in the neonatal and early infancy period.     

Surgical treatment of interrupted aortic arch in infants under three months of age

From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.     

Anatomical correction of complex forms of transposition of the great arteries in neonates

OBJECTIVE: The arterial switch operation has become the procedure of choice for the simple transposition of the great arteries (TGA) while in the complex forms of the defect the staged approach is frequently implemented. The aim of this study is to compare groups of patients with simple transposition and children with the complex form of the defect and identify factors affecting early and late outcome. DESIGN: From 1997 to 2003, 135 consecutive neonates with TGA underwent arterial switch operation and simultaneous reparation of all associated defects. Univariate and multivariate analysis of perioperative variables and follow-up data was performed. Patients were divided into two groups. Group I (n=84, 62.2%) included neonates with simple transposition (TGA/IVS), Group II (n=51, 37.8%) included children with complex transposition (TGA/VSD). RESULTS: Overall early mortality was 8.1% and there was one late death (0.7%). One-month, 1-year and 5-year actuarial survival rates were 91.8, 91.1 and 91.1%, respectively. There were no differences in the early and late survival rate between groups. Reintervention rate for right ventricular outflow tract obstruction (RVOTO) was 13.3% (balloon plasty or reoperation). The freedom from reintervention at 1, 3 and 5 years was 98.4, 87.9 and 85.4%, respectively. There were no differences in the need for reintervention between groups. The significant differences between groups concerned: age at operation (p<0.001), associated anomalies (p=0.002) including aortic arch anomalies (p=0.002) and coronary artery anomalies (p=0.02), application of delayed chest closure (p=0.015), and occurrence of sepsis (p=0.032). Risk factors for early death were: left ventricule dysfunction related to age at operation (p=0.016) and resternotomy in intensive care unit (p<0.001). There were no differences between groups concerning these risk factors as far as circulatory arrest time, aorta clamping time, and early and late morbidity. CONCLUSIONS: The arterial switch operation can be the treatment of choice for various forms of TGA with low early and late mortality and morbidity rates. The main cause of early death is still left ventricular dysfunction. Such well-known predictors of poor outcome as presence of ventricular septal defect, coronary artery anomalies and aortic arch anomalies did not affect early and late findings. The presented approach of early simultaneous anatomical correction of TGA and all associated anomalies ensures good condition of children with low necessity for reintervention.     

Stroke in surgery of the arteriosclerotic descending thoracic aortic aneurysms: influence of cross-clamping technique of the aorta.

OBJECTIVE: The risk of stroke caused by dislodgment of loose atheromatous plaque or mural emboli is increased by cross-clamping of the aorta. Some patients undergo descending thoracic aortic aneurysm repair with proximal aortic cross-clamping between the left common carotid artery and the left subclavian artery. The objective of this study was to determine the influence of proximal aortic cross-clamping in arteriosclerotic aneurysm or dissecting aneurysm repair. METHODS: Between May 1984 and May 2003, 81 patients underwent elective surgery for distal arch or descending aortic aneurysm repair with proximal aortic cross-clamping between the left common carotid artery and the left subclavian artery. To evaluate the influence of the proximal aortic cross-clamping, patients were divided into two groups: patients who had undergone arteriosclerotic aneurysm repair (group I, n=25) and patients who had undergone dissecting aneurysm repair (group II, n=56). RESULTS: Eight (9.9%) of the 81 patients had a stroke. Six strokes occurred in operations for arteriosclerotic aneurysm repair group I and two strokes occurred in operations for dissecting aneurysm repair group II (24 vs 3.6%; p=0.009). In-hospital mortality rates were 12% in group I and 8.9% in group II (p=0.70). Major postoperative complications included renal failure requiring hemodialysis (in 4.2% of the patients in group I and in 8.3% of the patients in group II, p=0.99) and pulmonary complication (in 20% of the patients in group I and in 16% of the patients in group II, p=0.67). CONCLUSION: Cross-clamping between head vessels should be avoided if at all possible when operating on patients who have arteriosclerotic descending thoracic aneurysms.     

The Puig-Massana-Shiley annuloplasty ring for mitral valve repair: experience in 126 patients

Between October, 1982, and December, 1984, 126 patients at the Texas Heart Institute underwent mitral valve repair for mitral insufficiency utilizing the Puig-Massana-Shiley annuloplasty ring. Resection of a triangular-shaped wedge of the mural leaflet and direct suture repair was done in 42 patients, and anterior leaflet repair was used in 2 patients. There were 79 male (63%) and 47 female (37%) patients with a mean age of 58 years. Preoperatively, 95% were in New York Heart Association (NYHA) Functional Class III or IV. Concomitant cardiac operations were performed in 82 patients and included coronary artery bypass grafting (49%), aortic valve replacement (16%), repair of ventricular septal defect (2%), resection of left ventricular aneurysm (2%), and repair of atrial septal defect (1%). There were 8 early deaths (6.3%) and 11 late deaths (8.7%). In 44 patients undergoing mitral valve repair as an isolated primary procedure, operative mortality was 2.3%. Murmurs of mitral insufficiency were present in 5 patients postoperatively, but only 1 required early reoperation for mitral valve replacement. Follow-up data have been obtained on 80% of the patients. Postoperative Functional Class was obtained for 63 of the 82 surviving patients and showed 92% of these patients to be in NYHA Functional Class I or II. Mitral valve repair incorporating the Puig-Massana-Shiley annuloplasty ring and valve leaflet revision is a reliable technique that is not technically demanding. We believe these methods should be attempted for correction of pure mitral insufficiency, particularly in circumstances where other cardiac repairs are required.     

Resection of Kommerell's diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division.

OBJECTIVES: A Kommerell's diverticulum in patients with a right aortic arch may become aneurysmal and be an independent cause of tracheoesophageal compression, even after ligation and division of a left ligamentum. We review the indications for and results of Kommerell's diverticulum resection and left subclavian artery transfer in children with a right aortic arch who previously underwent vascular ring (ligamentum) division. METHODS: From 1998 through 2001, eight children have been referred with recurrent respiratory symptoms (n=8) and/or recurrent dysphagia (n=4) after vascular ring division. Each child had a right aortic arch with a left ligamentum and had undergone division of the ligamentum elsewhere. All had a Kommerell's diverticulum that was not addressed at the initial operation. All patients had a repeat left thoracotomy with resection of the diverticulum. Five patients had division and reimplantation of the left subclavian artery into the left carotid artery to relieve the sling-like effect of the retroesophageal left subclavian artery on the right aortic arch. One other patient had primary Kommerell's diverticulum resection and transfer of the left subclavian artery to the left carotid artery. RESULTS: The mean age at the initial operation was 1.7+/-0.9 years, and the mean age at reoperation was 8.0+/-3.7 years. In all patients postoperative bronchoscopy confirmed relief of the tracheal compression. There were no complications related to the subclavian artery transfer. Two patients developed postoperative chylothorax, one requiring thoracic duct ligation. The median hospital stay was 5 days. All patients had dramatic resolution of their preoperative symptoms. CONCLUSIONS: Kommerell's diverticulum is an important anatomic structure that can cause recurrent symptoms in patients with a right aortic arch after ligamentum division. In selected patients, reoperation with resection of the Kommerell's diverticulum and transfer of a retroesophageal left subclavian artery results in relief of symptoms. This technique has become our procedure of choice as a primary operation for children with a right aortic arch and a significant Kommerell's diverticulum.     

带膜支架腔内修复联合解剖外旁路术治疗Stanford A型主动脉夹层

目的探讨带膜支架腔内修复联合解剖外旁路术对Stanford A型主动脉夹层的临床效果。方法通过建立各种解剖外旁路来重建无名动脉、左颈总动脉或左锁骨下动脉以延长锚定区,然后一期或分期行带膜支架主动脉腔内修复术;可采取颈动脉入路或股动脉入路来完成主动脉腔内修复术。结果全组34例中升主动脉夹层8例,主动脉弓部夹层26例,除1例术中死亡外,其余33例均成功接受带膜支架主动脉腔内修复术。30d病死率为8．8％（3／34）,内漏发生率为11．8％（4／34）,脑梗死发生率为5．9％（2／34）。随访6—70个月,平均24．5个月,2例失访。结果显示假腔内完全血栓形成16例,部分血栓形成13例。结论带膜支架腔内修复联合解剖外旁路术为Stanford A型主动脉夹层提供了一种新的治疗方法,具有微创、安全性较高,并发症较少的特点,但应注意适应证的选择。     

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.