Prior Pulmonary Artery Banding Research Articles

Long-term results following atrioventricular septal defect repair

BackgroundAtrioventricular septal defects (AVSD) represent 4–7% of congenital cardiac malformations. Definitive early repair is favored over prior pulmonary artery banding and delayed definitive repair in many centers. The aim of this study was to analyze long-term outcomes following AVSD repair over a 21-year period.MethodsA total of 202 consecutive patients underwent surgical AVSD correction between June 1999 and December 2020. Surgery was performed using the double-patch technique. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were In-hospital mortality and overall long-term freedom from reoperation.ResultsMedian age at operation was 120 days (IQR 94–150), median weight was 5.0 kg (4.2–5.3). None of the patients died within the first 30 postoperative days. In-hospital mortality was 0.5% (1/202 patients). Median follow-up was 57 months (11–121). Overall freedom from reoperation at 5, 10 and 15 years was 91.8%, 86.9% and 86.9%, respectively.ConclusionAVSD repair with the double-patch technique is a safe and effective procedure with good early postoperative outcomes and low long-term reoperation rates.

Predictors of reoperation and mortality after complete atrioventricular septal defect repair.

Previous studies investigating risk factors associated with reoperation or mortality after repair of complete atrioventricular septal defect (CAVSD) often have not included sizeable cohorts undergoing modified single-patch repair. Both double patch and modified single-patch techniques have been widely used in Australia since the 1990s. Using a large multi-institutional cohort, we aimed to identify risk factors associated with reoperation or mortality following CAVSD repair. Between January 1990 and December 2015, a total of 829 patients underwent biventricular surgical repair of CAVSD in Australia at 4 centres. Patients with associated tetralogy of Fallot and other conotruncal abnormalities were excluded. Demographic details, postoperative outcomes including reoperation and survival, and associated risk factors were analysed. Fifty-six patients (6.8%) required early reoperation (≤30 days) for significant left atrioventricular valve regurgitation or residual septal defects. Freedom from reoperation at 10, 15 and 20 years was 82.7%, 81.1% and 77%, respectively. Patients without Down syndrome and moderate left atrioventricular valve regurgitation on postoperative echocardiogram were found to be independent risk factors for reoperation. Operative mortality was 3.3%. Overall survival at 10, 15 and 20 years was 91.7%, 90.7% and 88.7%, respectively. Prior pulmonary artery banding was a predictor for mortality, while later surgical era (2010-2015) was associated with a reduction in mortality risk. Improved survival in the contemporary era is in keeping with improvements in surgical management and higher rates of primary CAVSD repair over time. The presence of residual moderate left atrioventricular valve regurgitation on postoperative echocardiography is an important factor associated with reoperation and close surveillance is essential to allow timely reintervention. Primary CAVSD repair at age <3 months should be preferenced to palliation with pulmonary artery banding due to the association of pulmonary artery banding with mortality in the long-term.

Porcine Model of the Arterial Switch Operation: Implications for Unique Strategies in the Management of Hypoplastic Left Ventricles

There are no reports on the performance of the arterial switch operation (ASO) in a normal heart with normally related great vessels. The objective of this study was to determine whether the ASO could be performed in a healthy animal model. Cardiopulmonary bypass (CPB) and coronary translocation techniques were used to perform ASO in neonatal piglets or a staged ASO with prior main pulmonary artery (PA) banding. Primary ASO was performed in four neonatal piglets. Coronary translocation was effective with angiograms confirming patency. Piglets could not be weaned from CPB due to right ventricle (RV) dysfunction. To improve RV function for the ASO, nine piglets had PA banding. All survived the procedure. Post-banding RV pressure increased from a mean of 20.3 ± 2.2 mmHg to 36.5 ± 7.3 mmHg (p = 0.007). At 58 ± 1 days post-banding, piglets underwent cardiac MRIs revealing RV hypertrophy, and RV pressure overload with mildly reduced RV function. Catheterization confirmed RV systolic pressures of 84.0 ± 6.7 mmHg with LV systolic pressure 83.3 ± 6.7 mmHg (p = 0.43). The remaining five PA banded piglets underwent ASO at 51 ± 0 days post-banding. Three of five were weaned from bypass with patent coronary arteries and adequate RV function. We were able to successfully perform an arterial switch with documented patent coronary arteries on standard anatomy great vessels in a healthy animal model. To our knowledge this is the first time this procedure has been successfully performed. The model may have implications for studying the failing systemic RV, and may support a novel approach for management of borderline, pulsatile left ventricles.

Abstract 16765: Sustained Total All-Region Perfusion During the Norwood Operation is Associated With Improved Post-Operative Recovery

Introduction: We developed a technique for Stage 1 “Norwood” reconstruction with continuous perfusion of the head, heart, and lower body at mild hypothermia, deemed Sustained Total All-Region (STAR) perfusion. Hypothesis: We hypothesized that STAR perfusion would shorten cardiopulmonary bypass and procedural times and expedite post-operative recovery, due to lack of cooling and re-warming and decreased total body ischemia. Methods: Between 2012-2019, 65 infants underwent Norwood reconstruction at our institution. Those who underwent prior pulmonary artery banding were excluded, yielding a cohort of 51 infants who underwent primary Norwood reconstruction. Outcomes for patients who underwent Norwood reconstruction using STAR perfusion (n=21) were compared to those who underwent Norwood reconstruction with conventional techniques using regional cerebral perfusion only (n=30). STAR perfusion was performed with innominate artery cannulation as well as olive tip cannulas inserted into the opened descending aorta and native aortic root to provide continuous perfusion of the head, heart, and lower body throughout the procedure (Figure), under mild hypothermia (32-34°C). Results: Norwood reconstruction with STAR perfusion was associated with shorter median CPB times vs. conventional perfusion techniques (161 vs. 226 mins,p&lt;0.0001) and elimination of myocardial ischemia (0 vs. 90 minutes cross-clamp time,p&lt;0.0001). Total operative time was reduced by over 3 hours (319 vs 514 mins,p&lt;0.0001), likely in part due to improved post-bypass coagulopathy. Improvements in post-operative recovery were noted with STAR perfusion, including more rapid normalization of serum lactate (19.3 vs. 26.5 hours, p=0.0009), lower peak serum lactate (8.6 vs.10 mmol/dL, p=0.04) and fewer days to chest closure (3 vs. 5 days, p=0.04). Survival to hospital discharge (95% vs 83%, p=0.38) was similar between groups. Conclusions: Norwood reconstruction with STAR perfusion is a safe technique that eliminates ischemic time to the heart and lower body and allows for the procedure to be performed at warmer temperatures. We found evidence of reduced CPB and operative times, as well as improved hemostasis, end-organ function, and post-operative recovery with this technique.

Case Report: Favoring biventricular repair beyond the conventional boundaries with primary arterial switch operation in a young adult

Transposition of great arteries (TGA) presents in neonates or in infancy. We report a case of TGA with ventricular septal defect (VSD) and pulmonary stenosis (PS) in an adult male patient of 23 years age. Arterial switch operation with VSD closure and neo-aortic valve replacement was done. The patient recovered well in the post-operative period. In adult patients, conversion from atrial to arterial switch has been widely reported, both directly and after prior pulmonary artery banding in two stages, but primary arterial switch for TGA has not been reported previously. In this patient there was a benefit of having a large VSD and severe PS.

The Outcomes of Superior Cavopulmonary Connection Operation: a Single Center Experience.

IntroductionThe superior cavopulmonary connection operation is one of the stages of the palliative surgical management for patients with functionally single ventricle. After surviving this stage, the patients are potential candidates for the final palliative procedure: the Fontan operation.ObjectivesThis study aimed to analyze the outcomes of superior cavopulmonary connection operations in our center and to identify factors affecting the survival and the progression to Fontan stage.MethodsThe outcomes of 161 patients were retrospectively analyzed after undergoing superior cavopulmonary connection operation in our center between 2005 and 2015.ResultsThe early mortality rate was 2.5%. Five (3.1%) patients underwent takedown of the superior cavopulmonary connection. The rate of exclusion from the Fontan stage was 8.3%. Statistical analysis revealed that elevated mean pulmonary artery pressure preoperatively and the prior palliation with pulmonary artery banding were risk factors for both early mortality and takedown; however, the age, the morphology of the single ventricle and the type of operation were not considered risk factors.ConclusionThe superior cavopulmonary connection operation can be performed with low rate mortality and morbidity; however, the elevated mean pulmonary artery pressure preoperatively and the prior pulmonary artery banding are associated with poor outcomes.

P2 Outcomes in adults following anatomic repair of congenitally corrected transposition of the great arteries

<h3>Introduction</h3> Anatomic repair (double switch operation) for congenitally corrected transposition of the great arteries (ccTGA) is associated with good short to mid-term outcomes. An increasing number of these patients are surviving to adulthood. There is a paucity of data in adults regarding late sequelae. We sought to determine the long-term outcomes in older patients who have previously undergone a double switch operation for ccTGA. <h3>Methods</h3> A retrospective analysis of 16 patient records from 2001 to 2015 from a single institution was conducted. Median age was 23 years (range 18–56 years, 14 male). Six patients had undergone a Senning-arterial switch and 10 patients underwent a Rastelli-Senning procedure. Associated defects included VSD (n = 13), pulmonary stenosis (n = 2) and pulmonary atresia (n = 13). Three patients had undergone prior pulmonary artery banding and 7 patients systemic-pulmonary artery shunting. We determined freedom from re-operation/intervention, development of systemic left ventricular (LV) dysfunction, systemic mitral regurgitation, conduction disorders and arrhythmias. <h3>Results</h3> Median follow-up was 19 years (18–21 years). Eleven patients required further surgery with 4 patients having undergone two subsequent operations. Following anatomic repair, median time to 1^st operation was 11 years (7–13 years). Indications for re-operation included re-do RV-PA conduit in the Rastelli-Senning patients (n = 8) and aortic valve replacement in the Senning-arterial group (n = 3). 5 patients developed atrio-ventricular conduction block requiring a pacemaker. Three patients developed atrial arrhythmias, with 2 requiring ablation procedures. Six patients developed LV dysfunction. There were no deaths or need for cardiac transplantation in this series. <h3>Conclusion</h3> The majority of patients required a second operation, largely in the Rastelli-Senning group. In the Senning-arterial group, late aortic valve insufficiency occurred in 50% of the patients. Conduction disease and atrial arrhythmias contribute to late morbidity in these patients. Nevertheless, the majority of patients are free of heart failure. Despite good short-term outcomes and survival following anatomic repair, careful long-term evaluation for structural and electrophysiological abnormalities is required.

Implications of Early Aortic Stiffening in Patients With Transposition of the Great Arteries After Arterial Switch Operation

The elastic function of the aorta in patients with transposition of the great arteries after arterial switch operation (ASO) is suspected to be important for long-term prognosis. Fifty-one patients and 34 controls were studied at 3.0 Tesla with MRI. Forty-three patients (12.8±6.9 years) underwent 1-stage ASO, 8 patients (23.8±6.9 years) had prior pulmonary artery banding (2-stage ASO). Aortic dimensions, distensibility, pulse wave velocity, aortic arch angle, left ventricular (LV) mass, LV systolic function and left atrial (LA) volumes, and LA passive emptying function as marker of LV diastolic function were assessed. Compared with controls, patients had increased aortic root areas (602.6±240.5 versus 356.8±113.4 mm(2)/m; P<0.01) and reduced distensibility of the thoracic aorta most pronounced at the aortic root (3.2±2.0 versus 9.1±4.7×10(-3) mm Hg(-1); P<0.01). Aortic distensibility correlated negatively with the aortic areas (P<0.01). Pulse wave velocity was higher in adults after ASO (5.0±1.0 versus 3.8±1.3 m/s; P<0.01). In contrast to controls pulse wave velocity and distensibility correlated with age in patients (P=0.04 to <0.01), LV mass was higher in patients (P=0.02). LA volumes correlated negatively with aortic root and ascending aortic distensibility and positively with pulse wave velocity (P<0.05). In patients, LA passive emptying function was lower (27.3±8.9 versus 41.1±6.0; P<0.01) and correlated with aortic root distensibility (P=0.004). Reduced aortic bioelasticity and aortic root dilatation are present in transposition of the great artery patients post ASO and are likely to contribute to LV diastolic dysfunction. Impaired aortic bioelasticity was strongly associated with age, suggesting the usefulness of follow-up studies for early onset of degenerative cardiovascular disease.

The Arterial Switch Operation: 25-Year Experience With 258 Patients

At our institution, the arterial switch operation for transposition of the great arteries has transitioned from the Gore-Tex patch (W.L. Gore & Associates, Flagstaff, AZ) for pulmonary artery reconstruction to redundant pantaloon pericardial patch (RPPP). The (U-shaped) coronary artery button was used for coronary reimplantation. This study investigates overall mortality and factors for neopulmonary artery, neoaortic, and coronary artery surgical reintervention. We performed a retrospective chart review of all patients who underwent arterial switch between 1983 and 2007. Our surgical database, operative reports, and cardiology clinic charts were reviewed. Time to event was plotted as Kaplan-Meier curves. Predictors of time-to-event were examined using Cox proportional hazard modeling. A total of 258 patients underwent arterial switch during the study. Mortality declined from 15% (era I: 1983 to 1990) to 11% (era II: 1991 to 1998) to 7% (era III: 1999 to 2007). Era III had a significantly later time to death compared with era I (hazard ratio [HR] 0.62, p = 0.04). The RPPP had a lower neopulmonary artery reintervention rate compared with Gore-Tex; 9 of 225 (4%) versus 3 of 21 (14%), p = 0.008. Complex anatomy increased risk for neopulmonary reintervention (HR 3.3, p = 0.03). Surgical reintervention rate for coronary arteries was 2%. Complex coronary anatomy (HR 17.9, p = 0.01) predicted coronary reintervention. Predictors of neoaortic reintervention were prior pulmonary artery band (HR 4.3, p = 0.03), complex anatomy (HR 3.5, p = 0.01), and coronary artery anatomy (HR 3.5, p = 0.04). Arterial switch operation mortality has decreased. Conversion to RPPP reduced neopulmonary artery reintervention. The (U-shaped) coronary artery button technique is associated with low coronary reintervention rates. Complex coronary anatomy increases coronary and aortic reintervention. Prior pulmonary artery banding and complex anatomy increase aortic reintervention.

Fate of the Aortic Valve Following the Arterial Switch Operation

We evaluated the long-term outcome of aortic valve after arterial switch operation (ASO). A retrospective review of 324 hospital survivors after ASO was performed. Echocardiographic findings and incidence and progression of aortic regurgitation (AR) were investigated. Endpoints of the study were the first documented occurrence of moderate or severe AR or need for aortic valve replacement (AVR). Upon discharge from hospital 5.2% of the patients showed mild AR, progressing to 11.4% after 1 year. At a mean follow-up time of 14.4 ± 0.54 years, AR was absent in 307 (94.7%), trivial in six (1.8%), mild in nine (2.7%), and moderate-to-severe in two (0.6%) patients. There is a progression of AR with time after ASO (p < 0.001). A total of two patients reached the combined endpoint, both of whom underwent AVR at a mean time of 10.82 years after ASO. Freedom from aortic valve reoperation was 98.7 ± 0.9% at 5 and 10 years and 94.7 ± 4% at 15 years. Univariate analysis identified the following risk factors: ventricular septal defect (VSD) (p = 0.005), prior pulmonary artery banding (p < 0.003), and postoperative incidence of mild AR (p < 0.0001). AR is not rare after ASO, but it is stable without progressive intensity; new AR developed in 5.2% after 10 years, and is present only in 2.7% after a median follow-up 14.4 ± 0.54 years. If trivial AR is excluded, it is present only in 0.9%. New AR can develop even up to 15 years. However, severe AR and need for AVR are rare.

Impairment of aortic elastic properties in patients with transposition of the great arteries post arterial switch operation

Methods Fourty-two d-TGA patients (median 15, range 0.5-31.0 years) were examined with 3.0 T MRI. 26 patients had simple transposition and 16 patients had additional risk factors (ventricular septal defect (VSD) n = 7, aortic coarctation n = 3, prior pulmonary artery banding n = 5, and ASO without Lecompte maneuver n = 1). 34 heart healthy subjects were enrolled as controls (median 11.4, range 2.3-31.3 years).

Risk factors for aortic insufficiency and aortic valve replacement after the arterial switch operation☆

Long-term results after the arterial switch operation have shown that patients may develop aortic insufficiency, and that some even require aortic valve replacement. A retrospective review of 479 hospital survivors after the arterial switch operation (ASO) was performed. Echocardiographic findings were reviewed and the incidence, as well as the progression, of aortic insufficiency (AI) was investigated. The combined end point of the study was defined as the first documented occurrence of moderate or more aortic insufficiency or the need for aortic valve replacement (AVR). Upon discharge from the hospital 15% of the patients showed an AI of at least grade I, progressing to 20.7% after 1 year. At a mean follow-up time of 9.3+/-6 years, 249 patients (53%) were free from AI, trivial AI was present 179 patients (38%), mild AI in 34 patients (7.2%) and moderate AI in 7 patients (1.5%). There is a progression of AI with time after ASO (r=0.26, p<0.001). A total of 18 patients reached the combined end point, out of which 11 underwent an AVR at a mean time of 11.2 years after ASO. Freedom from the end point was 99.7+/-0.3%, 97.5+/-1%, 91.9+/-2%, 84.6+/-6% at 5, 10, 15 and 20 years, respectively. The following risk factors were identified by univariate analysis: Taussig-Bing anomaly (p=0.01), ventricular septal defect (VSD) (p=0.006), prior pulmonary artery banding (p=0.004), age over 12 months at time of ASO (p=0.001) and a postoperative incidence of trivial AI (p<0.0001). Independent risk factors by multivariate analysis were the presence of a left ventricular outflow tract obstruction (p<0.0001) and at least a trivial AI at 1 year after the ASO (p<0.0001). The incidence of trivial or mild AI after the ASO is considerable and a progression over time is evident. However, severe AI and the need for AVR are rare. Patients with VSD or Taussig-Bing anomaly, and those with left ventricular outflow tract obstruction exhibit a higher risk of developing significant aortic insufficiency. Particularly patients who have developed an AI at 1 year after the ASO need to be under close observation.

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

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. Records of 314 hospital survivors who had undergone the Senning operation were reviewed for details concerning cardiac reoperations. 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. 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.

Risk Factors for Neo-Aortic Root Enlargement and Aortic Regurgitation Following Arterial Switch Operation

The objectives of this study were to evaluate changes in dimension of the neo-aortic annulus, aortic root, and aortic anastomosis following arterial switch operation (ASO) and to identify risk factors for developing abnormal neo-aortic root enlargement and aortic regurgitation (AR). Prior studies report development of neo-aortic root dilatation and AR in a small subset of patients after ASO. Predisposing factors for neo-aortic root dilatation and development of moderate/severe AR are poorly understood. We performed a retrospective review of all patients with d-transposition of the great arteries (d-TGA) or double-outlet right ventricle with subpulmonary ventricular septal defect (VSD) who underwent ASO from May 1986 to January 2001. Serial echocardiograms were reviewed to measure neo-aortic annulus, root, and anastomosis diameter (z scores) and to determine progression of AR. Potential risk factors were assessed for developing neo-aortic root enlargement and AR. There were 119 patients (44 female and 75 male): 73 patients had simple d-TGA, 36 had d-TGA with ventricular septal defect, and 10 had a Taussig-Bing heart. The median duration of follow-up was 65 months (range, 12-180). The median neo-aortic root (z = 0.55+/-2.2; p < 0.01) and aortic annulus dimensions (z = 1.57+/-1.75; p < 0.01) were significantly increased over the study period. Aortic anastomosis diameter correlated with growth of the ascending aorta (z = 0.55+/-1.24). Development of severe neo-aortic root enlargement was associated with prior pulmonary artery (PA) banding (p < 0.01), the presence of a VSD (p = 0.03), and Taussig-Bing anatomy (p < 0.01) but was independent of coronary arterial anatomy, coronary arterial transfer technique, or associated lesions (p > 0.05). At latest follow-up, there was no or trivial AR in 88 patients, mild AR in 29 patients, and moderate to severe AR in 3 patients. Risk factors for developing mild or worse AR included severe or rapid neo-aortic root dilatation (p < 0.01). Only 3 patients required surgical intervention for AR. Despite the significant prevalence of neo-aortic root enlargement at intermediate follow-up after ASO, there is a low incidence of significant AR. Prior PA banding, the presence of VSD, and Taussig-Bing anatomy are risk factors for severe root enlargement. Surgical intervention for AR was rare (2%), however, serial surveillance of such patients is vital to monitor for neo-aortic root enlargement and potential aortic valve dysfunction.

Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors

Background This study was conducted to evaluate surgical outcome and to identify risk factors for hospital mortality and reoperation after repair of complete atrioventricular septal defect. Methods A total of 147 consecutive children underwent repair between January 1986 and December 1998. Of those, 106 had Down syndrome, 37% had normal chromosomes, and 4 had other syndromes; 108 underwent primary repair, 19 had prior pulmonary artery banding, and 20 had additional tetralogy of Fallot. The median weight at primary repair was 4.5 kg. A two-patch technique was used in 88%. Results The 30-day mortality was 15% (70% confidence interval [CI] 12% to 19%). A double orifice atrioventricular valve was found to be a significant risk factor ( p = 0.002), with 6 of 11 patients dying. If double orifice atrioventricular valve patients are excluded, the mortality rate falls to 12% (70% CI 9% to 15%). No difference in mortality was found between Down syndrome and chromosomally normal children but the latter more commonly required reoperation. Chromosomally normal children frequently have a dysplastic common atrioventricular valve (24% versus 3% in Down children, p < 0.001). In a multivariate Cox model including both variables, the presence of a dysplastic atrioventricular valve was a significant risk factor for reoperation. After controlling for the presence of a dysplastic atrioventricular valve, Down syndrome retained a significant protective effect but the upper limit of the confidence interval was close to 1. Conclusions The presence of a double orifice atrioventricular valve emerged as an unforeseen risk factor for death.

Midterm results of surgical treatment of systemic ventricular outflow obstruction in Fontan patients

Background. Achieving unobstructed blood flow from the systemic ventricle to the aorta is important during the Fontan procedure for complex cyanotic congenital heart disease when there is systemic ventricular outflow obstruction (SVOO). Because SVOO can progress after the Fontan procedure if there is morphologic obstruction, we have adopted a policy of relieving obstructions to systemic blood flow. Methods. Twenty-five patients were treated by the Fontan procedure with SVOO. Twenty-one patients had undergone prior pulmonary artery banding and 10 patients had undergone prior arch repair. Systemic ventricular outflow obstruction progressed in 5 patients after the Fontan procedure. Main diagnosis was single ventricle in 12, tricuspid atresia in 5, transposition of the great arteries in 4, double-outlet right ventricle in 3, and common atrioventricular canal in 1. Mean age at operation was 6.5 years (range 1 to 15 years) and the average preoperative pressure gradient across the ascending aorta and systemic ventricle was 29 mm Hg (range 0 to 100 mm Hg). The Damus-Kaye-Stansel procedure was performed in 18 patients (double-barrel anastomosis in 13, end to side anastomosis in 5), and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in 7. Double-barrel anastomosis has been our first choice since 1994, if the pulmonary valve is intact. Follow-up has ranged from 4 months to 14 years (average 5.0 years). Twenty-three of the 25 patients have undergone recatheterization (average 21.4 months later). Results. No early deaths were found; one late death was reported of a patient with single right ventricle (4.0%). The postoperative average pressure gradient was 1.1 mm Hg (0 to 10 mm Hg), and the average right atrial pressure was 14 mm Hg (9 to 20 mm Hg). In all patients who underwent ventricular septal defect or bulboventricular foramen enlargement, regular sinus rhythm was maintained postoperatively. Regarding the Damus-Kaye-Stansel procedure, there was minimal progression of semilunar valve insufficiency except in 1 patient who underwent end-to-side anastomosis with moderate pulmonary regurgitation postoperatively. Conclusions. The midterm results of the Fontan procedure with SVOO have been satisfactory. Because SVOO might progress after the Fontan procedure if there is morphologic obstruction, an appropriate strategy to relieve obstruction to systemic blood flow should therefore be performed concomitantly with the Fontan procedure.

Does banding the pulmonary artery affect pulmonary valve function after the Damus-Kaye-Stansel operation?

Background. The Damus-Kaye-Stansel (DKS) operation can be an effective palliation in patients who have single-ventricle physiology and systemic outflow obstruction. Pulmonary artery banding (PAB) may be used as a preliminary procedure in these patients to limit overperfusion of the pulmonary circulation. In some series, the DKS operation has been associated with pulmonary insufficiency (PI). We retrospectively analyzed medical records of our patients who had PAB and later DKS to determine the incidence of PI in these patients. Methods. Between 1982 and 1996, 15 patients underwent PAB before DKS. Median age at PAB placement was 7 days and median duration of PAB was 7 months. Echocardiograms obtained before PAB, before DKS, and at the most recent post-DKS follow-up were reviewed. Results. Follow-up ranged from 1 to 15 years (mean follow-up, 7.5 years). One patient had trivial PI before PAB, which progressed to moderate PI at the last follow-up. Only 1 other patient had mild PI, but only at the last follow-up after DKS. Conclusions. These findings suggest that prior PAB does not appear to cause significant PI in patients slated for DKS, and the incidence of significant PI after the DKS operation is relatively low.

Institutional experience with a protocol of early primary repair of double-outlet right ventricle

Our institution has adopted a protocol of primary repair for all patients with double-outlet right ventricle. Since May 1989, 24 consecutive neonates and infants with double-outlet right ventricle and atrioventricular concordance (median age, 4 months) underwent anatomic biventricular repair. One patient (4%) received prior pulmonary artery banding but was still repaired as a neonate at 22 days of age. Twelve patients had a subaortic ventricular septal defect (VSD), 5 patients a subpulmonary VSD, 3 patients doubly committed VSD, and 4 patients a noncommitted VSD. Sixty-nine of 72 associated lesions were repaired simultaneously. Four types of repairs were used: intraventricular rerouting in 16 patients, arterial switch operation with VSD closure into the pulmonary artery in 4 patients, Rastelli-type repair with extracardiac conduit in 3 patients, and the Damus-Kaye-Stansel repair with concomitant repair of aortic arch obstruction in 1 patient. Ventricular septal defect enlargement was necessary in 15 patients. Repair of subpulmonary stenosis and of subaortic stenosis was carried out in 13 and 4 patients, respectively. Three patients underwent simultaneous repair of aortic arch obstruction with no mortality. Two of the patients with noncommitted VSD had simultaneous repair of complete atrioventricular canal and repair of severe pulmonary venous obstruction. The perioperative mortality was 8% (2 patients, and there was one late death (4%). Two patients (9%) underwent early successful reoperations (5 and 8 weeks postoperatively). The two reoperations were for residual VSD (1 patient) and severe mitral regurgitation (1 patient). All 21 survivors are alive at a mean follow-up of 40 months (range, 7 months to 6 years). The estimated 5-year actuarial survival is 88%, with no deaths after 2 months postoperatively. Ninety-five percent of long-term survivors have no restriction of physical activities because of cardiac status and are receiving no cardiac medications. An institutional protocol of early anatomic biventricular repair of double-outlet right ventricle in infants and neonates achieves excellent survival, making palliative operations unnecessary. Associated lesions should be repaired simultaneously. The complexity of these malformations requires a highly individualized and flexible surgical approach.

Systemic ventricular outflow obstruction progresses after the Fontan operation

To evaluate the course of systemic ventricular outflow obstruction after the Fontan operation, the records of 57 hospital survivors of that procedure were reviewed. Ventricular outflow obstruction was identified in 7 patients (group 1) and was absent in 50 patients (group 2). Overall, the ventricular outflow gradient in group 1 was 6.3 ± 2.9 mm Hg (mean ± standard error) before the Fontan operation and 7.6 ± 3.9 mm Hg at hospital discharge. Ventricular outflow obstruction subsequently progressed to 80.1 ± 17.3 mm Hg (range, 33 to 165 mm Hg; p < 0.02) a mean of 28 months postoperatively. One patient died of severe progressive ventricular outflow obstruction. Group 1 did not differ from group 2 in age, ventricular morphology, presence of a subaortic outflow chamber, prior shunt, or length of follow-up. Compared with group 2, however, patients in group 1 more commonly had an aorta arising from a hypoplastic ventricle ( p < 0.001) and had undergone prior pulmonary artery banding ( p = 0.005). We conclude that systemic ventricular outflow obstruction occurs commonly after a Fontan procedure (incidence, 12%; 70% confidence interval, 9% to 18%) and is a progressive lesion.

Systemic ventricular size and performance before and after bidirectional cavopulmonary anastomosis

In patients eventually requiring Fontan operation, preoperative ventricular dilation is a risk factor for poor postoperative outcome. Bidirectional cavopulmonary anastomosis (BCPA) has been advocated to reduce ventricular volume load and decrease ventricular dilation; however, this effect has not been documented. To determine the effectiveness of this operation at reducing ventricular size while maintaining ventricular function, we reviewed preoperative and early postoperative echocardiograms in 20 patients undergoing BCPA. Ten of these patients also had later follow-up studies. Ventricular size was assessed by measurement of end-diastolic and end-systolic area, and heart rate was recorded at each study time. Ventricular function was assessed by area shortening. Indexed end-diastolic area was significantly smaller after BCPA than before (26.2 +/- 10.5 vs 21.4 +/- 6.5 cm2/m2). Area shortening and heart rate did not change significantly. Patients who had prior systemic-to-pulmonary shunts had a significant decrease in end-diastolic area after BCPA (30.2 +/- 12.2 vs 22.4 +/- 7.3 cm2/m2); those with prior pulmonary artery banding did not have a significant decrease (22.5 +/- 7.2 vs 19.9 +/- 6.5 cm2/m2). Patients with ventricles having left ventricular morphologic features had a significant decrease in end-diastolic area after BCPA (29.2 +/- 10.8 vs 22.8 +/- 6.5 cm2/m2); those with right ventricular morphologic features did not have a significant decrease (19.2 +/- 5.9 vs 18.0 +/- 5.9 cm2/m2). We conclude that BCPA results in a significantly reduced systemic ventricular preload and size while normal ventricular performance is maintained. Other factors such as prior palliation and ventricular morphologic features may affect the magnitude of the decrease in ventricular size seen after BCPA.