Is there any need for a shunt in the treatment of tetralogy of Fallot with one source of pulmonary blood flow?

OBJECTIVES: In symptomatic patients, performing a primary repair of tetralogy of Fallot (TOF), irrespective of age or placing a shunt, remains controversial. The aim of the study was to analyse the policy of primary correction. METHODS: Between May 2005 and May 2012, a total of 87 consecutive patients with TOF, younger than 6 months of age, underwent primary correction. All patients had one source of pulmonary blood flow, with or without a patent ductus arteriosus. The median age at surgery was 106 ± 52.3 days (8-180 days). Twelve patients (13.8%) were newborns. Two groups were analysed: group I, patients <1 month of age; group II, patients between 2-6 months of age. RESULTS: There was no early or late death at 7 years of follow-up. There was no difference in bypass time or hospital stay between the two groups, but the Aristotle comprehensive score (P < 0.0001), ICU stay (P = 0.030) and the length of ventilation (P = 0.014) were significantly different. Freedom from reoperation was 87.3 ± 4.3% and freedom from reintervention was 85.9 ± 4.2% at 7 years, with no difference between the two groups. Neurological development was normal in all patients, but 1 patient in Group II had cerebral seizures and showed developmental delay. Growth was adequate in all patients, except those with additional severe non-cardiac malformations that caused developmental delay. Eighty-five per cent of the patients were without cardiac medication. CONCLUSIONS: Even in symptomatic neonates and infants <6 months of age, primary repair of TOF can be performed safely and effectively. One hundred per cent survival at 7 years suggests that early primary repair causes no increase in mortality in the modern era. Shunting is not necessary, even in symptomatic newborns, thus avoiding the risk of shunt-related complications and repeated hospital stays associated with a staged approach.

Interventions after Norwood procedure: comparison of Sano and modified Blalock-Taussig shunt.

Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock-Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. Forty-six (43.0 %) patients underwent interventions, mostly for dilatation of the aortic arch ([DAA] n = 26 [24.3 %]; Sano n = 10, BT n = 16, p = 0.6), dilatation of the shunt ([DS] n = 15 [14.0 %]; Sano n = 11, BT n = 4; p = 0.002), or closure of aortopulmonary collaterals ([APC] n = 15 [14.0 %]; Sano n = 3, BT n = 12; p = 0.08). Mean interval after NP and body weight at DAA, DS, and APC were 72.4 ± 18.9, 108.5 ± 15.8, and 110.7 ± 17.8 days and 4.5 ± 1.3, 4.9 ± 1.9, 5.3 ± 1.2 kg, respectively. The interventions were not associated with mortality but with a greater rate of complications (9 of 46 [21.4 %]) compared with the rate after diagnostic catheterization (0 of 45, p = 0.03). Complications included closure of the femoral or subclavian artery (n = 5), cerebral embolic or bleeding events (n = 4), cardiopulmonary resuscitation (n = 3), and temporary heart block (n = 2). Actuarial survival was similar from the postoperative month 8 onward at 78.6 ± 4.9 % (95 % confidence interval [CI] 67.0-86.5 %) for Sano and 78.4 ± 6.8 % (95 % CI 61.4-88.6 %) for BT (p = 0.95). Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.

Quantification of morbidity associated with congenital heart surgery.

OBJECTIVE: The objective of this study was to estimate the morbidity according to observed complications after congenital heart surgery over 1-year period. METHODS: The previously established list of conditions prone to affect patients' well-being or increase cost of in-hospital stays was used systematically to score the severity of postoperative complications from 1 to 4 points. The morbidity score was calculated by adding the scores of observed complications. When the sum amounted to more than 5 points, a morbidity score of only 5 points was attributed. If no complication was detected, a score of 0.5 points was assigned. The resulting morbidity scores were correlated with the length of stay in the intensive care unit (ICU) and in the hospital, the duration of mechanical ventilation, and Aristotle complexity scores. RESULTS: A total of 542 primary procedures performed in the year 2011 were studied. Aristotle basic and comprehensive scores amounted to 7.78 ± 2.65 and 10.15 ± 3.83, respectively. Mortality was 1.85% (10/542). The standardized ratio of surgical performance reached 103.10%. Total cavopulmonary connection with extracardiac fenestrated conduit constituted the most frequent operation (n = 34). No complication was observed following 183 (33.8%) procedures. More than two complications were observed in 114 cases (21%). The three most frequent unfavorable conditions were "mechanical ventilation 25 to 95 hours" (n = 150), low cardiac output syndrome (n = 56), and cardiac arrhythmia requiring medication (n = 50). The estimated mean morbidity score amounted to 2.26 ± 1.80 points. Scores ranged from 0.68 ± 0.50 for primary closure of atrial septal defect to 4.50 ± 0.79 for the Norwood procedure. They were perfectly related to the length of ICU stay and to the duration of mechanical ventilation (Spearman coefficient r = 1). Correlation was high with the length of hospital stay (r = 0.83), Aristotle basic score (r = 0.89) (r = 0.96), and comprehensive score (r = 0.94) (C-index = 0.97). The observed mean morbidity score was statistically not different from the expected mean morbidity score according to the basic Aristotle complexity: p = 0.73. CONCLUSION: Quantification of morbidity indicates the length of ICU stay and the duration of mechanical ventilation as the best surrogates for morbidity. Such benchmarking and scoring of observed postoperative complications paves the way for an accurate assessment and improvement of quality care in congenital heart surgery.

Ross-Konno procedure in infants: mid-term results.

OBJECTIVES: The aim of study was to analyse the mid-term results of the Ross-Konno procedure in infants. METHODS: Between 2000 and 2011, 16 infants, including five newborns, with complex left ventricular outflow tract (LVOT) obstruction underwent the Ross-Konno procedure. Twelve patients (75%) required multiple concomitant procedures such as: mitral valve (MV) surgery (four patients), resection of endocardial fibroelastosis (EFE) and myectomy (six patients), closure of ventricular septal defect (four patients) and aortic arch reconstruction (three patients). The median age at operation was 4.2 months (from 6 to 333 days). RESULTS: There was one late death with a median follow-up of 6.2 years. Actuarial survival is 93.3% at 5 years follow-up (95% confidence interval: 61.2-99.0). Postoperatively, two patients required extracorporeal membrane oxygenation support and one MV replacement. The median length of stay in hospital was 30 days (from 11 to 77 days). At 5 years of follow-up, seven patients had no aortic regurgitation (AR) and nine patients (56%) had trivial AR with no gradient in LVOT. Freedom from mitral regurgitation (MR) ≥ moderate or MV replacement was 70%. MR was associated with either structural abnormalities of MV or with development of EFE. Freedom from redo was 81 and 53% at 1 and 5 years of follow-up. Sixty percent of patients are without medication. All patients are in sinus rhythm. CONCLUSIONS: With the technical aspects of this procedure well accomplished, the risk of surgery is minimal and functional outcome is encouraging. However, early postoperative morbidity is significant. At the mid-term follow-up, there was no residual or recurrent outflow tract obstruction and excellent function of the neoaortic valve. A high incidence of MR associated with the development of EFE and structural abnormalities of the MV is worrisome; however, concomitant MV surgery is not associated with increased mortality. In the case of the development of EFE, an early indication for operation might protect MV function. The reoperation rate is high due to early conduit failure.

Does the shunt type determine mid-term outcome after Norwood operation?

OBJECTIVES: With improved short-term outcomes the right ventricular to pulmonary artery shunt (Sano) has become the preferred pulmonary blood source in the Norwood procedure in many centres. However, most studies analysed consecutive cohorts, with a first modified Blalock-Taussig shunt (BT) followed by the Sano cohort. Besides, neither comprehensive preoperative risk analysis nor outcome beyond 1 year of age was investigated. METHODS: This study reviews 109 neonates undergoing the Norwood procedure in the same interval between October 2002 and December 2009. The Sano (38) or BT shunt (71) was assigned according to the surgeon's preference. Two neonates subsequently underwent successful biventricular repair and were excluded. The Aristotle comprehensive score (ACS) was used to evaluate preoperative risk, with high-risk patients (n = 39) classified as having an ACS ≥ 20, and low-risk patients (n = 68) given an ACS <20. RESULTS: Mean Aristotle score at the Norwood operation was 18.8 ± 0.4 and 18.9 ± 0.3 (P = 0.9) in Sano and BT, respectively. Mean follow-up interval was 4.1 ± 2.1 years (range: 1.7-8.9 years). Actuarial survival was similar, stabilizing from the 8th postoperative month onwards at 78.6 ± 4.9% (95% CI: 67.0-86.5%) for Sano and 78.4 ± 6.8% (95% CI: 61.4-88.6%) for BT; P = 0.95. Midterm actuarial survival was higher in low-risk patients, 88.2 ± 3.9% (95% CI: 77.8-93.9%) than in high-risk patients: 61.5 ± 7.8% (95% CI: 44.5-74.7%, P = 0.0003). No survival benefit was detected in low- or high-risk cases for either shunt type. Risk factors for midterm mortality were cardiorespiratory failure requiring ventilation (13/34, P = 0.004), and ACS ≥ 20 (15/39, P = 0.001), but not shunt type (8/37, P = 0.95). Increased number of shunt-related interventions before the Glenn procedure were noted with Sano (32.4 versus 6.5%, P = 0.002). CONCLUSIONS: Preoperative risk factors, regardless of shunt type, influence midterm survival after the Norwood procedure with an excellent outcome in low-risk patients, while high-risk cases still incur a significant mortality. Sano shunt interventions occurred with increased numbers. Although, Sano shunt may be the only feasible option in some instances, given the possible negative effects of ventriculotomy on right ventricle function, the widespread use of Sano shunt should be reconsidered.

Aortopulmonary window associated with interrupted aortic arch: report of surgical repair of eight cases and review of literature.

BACKGROUND: Patients with combined aortopulmonary window (APW) and interrupted aortic arch (IAA) malformations are rarely seen. We reviewed cases with such association with emphasis on surgical management and long-term outcome. METHODS: 109 patients with IAA and 25 patients with APW were operated upon between 1981 and 2011. The clinical records, operation and follow-up data were analyzed. Long-term outcome was completed with the help of either outpatient data or inquiring. Related literature was investigated. RESULTS: Combined APW/IAA was found in 8 cases: 7.3% (8/109) of those with IAA diagnosis and 32% (8/25) with APW. All APWs were proximal. 7 patients had interruption type A. A ventricular septal defect (VSD) was associated in one case only. Median age at surgery was 10 days. The two first patients were operated upon without cardio-pulmonary bypass (CPB) and one died during operation. The other six underwent single stage approach under CPB with no death: overall early mortality of 12.5% (⅛). APW was closed with one or two patches; aortic arch was reconstructed either directly (extended end-to-side anastomosis) or by patch augmentation. There was no late death and no reoperation during mean follow-up of 118 months (range 1-360 months). Six patients were in functional NYHA class I, the remainder in class II. The cumulative APW/IAA incidence from literature varies between 4.9% (56/1105) and 22.2% (42/189). Early mortality reaches 15.1% (8/53) (95% CI: 6.8% - 27.6%). CONCLUSION: Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation.

A morbidity score for congenital heart surgery based on observed complications.

OBJECTIVES: The aim of this study was to develop a morbidity score based on observed postoperative complications after congenital heart surgery. METHODS: Conditions or diseases that impair patients after congenital heart surgery or increase costs of hospital stay were called complications and attributed to scores ranging from 1 (mild) to 4 (severe) points, according to estimated severity or costliness. 'No complication' was assigned 0.5 points. From January to March 2011, scores for each observed 'complication' for every main (primary) surgical procedure were recorded and a morbidity score was calculated. In conformity with the Aristotle score methodology, if the sum of observed complication scores amounted to more than 5 points, a morbidity score of only 5 points was attributed. The estimated morbidity score was compared with the morbidity score attributed by the Aristotle basic complexity (ABC) score. RESULTS: One hundred and thirty-nine primary procedures were carried out. The mean ABC and Aristotle comprehensive complexity scores reached 8.31 ± 2.52 and 9.62 ± 3.47 points, respectively. Two patients died. No complication was detected after 46 procedures. Overall, there were 185 listed 'morbidity' conditions in connection with the other 93 surgical procedures, rendering a total score of 385 points. The most frequent event was 'mechanical ventilation 25-95 h': n = 39. The mean morbidity score was 2.14 ± 1.63. The morbidity scores ranged from 0.5 points (n = 46) to 5 points (n = 23) with a median of 2.0 points. The scores for 11 different procedures that were performed at least five times positively correlated with the corresponding Aristotle morbidity scores: Pearson's coefficient r = 0.75. But the morbidity score for bidirectional cavopulmonary anastomosis (3.14 ± 1.77) was higher than the corresponding Aristotle morbidity score (2.0). It was lower for 'conduit placement, right ventricle to pulmonary artery': 1.08 ± 0.97, versus 2.0, and for arterial switch operation: 2.08 ± 1.11, versus 3.0. CONCLUSIONS: The reported morbidity scores need to be tested on larger series and in different institutions. The introduced morbidity score has the potential to quantify postoperative complications accurately. Its estimation over time can facilitate the assessment of quality of congenital heart surgery. It will allow comparison of morbidity outcomes across institutions with different case-mixes.

Surgical management of congenital heart disease: contribution of the Aristotle complexity score to planning and budgeting in the German diagnosis-related groups system.

Planning and budgeting for congenital heart surgery depend primarily on how closely reimbursement matches costs and on the number and complexity of the surgical procedures. Aristotle complexity scores for the year 2010 were correlated with hospital costs and with reimbursement according to the German diagnosis-related groups (DRG) system. Unit surgical performance was estimated as surgical performance (complexity score × hospital survival) times the number of primary procedures. This study investigated how this performance evolved during years 2006 to 2010. Hospital costs and reimbursements correlated highly with Aristotle comprehensive complexity levels (Spearman r = 1). Mean costs and reimbursement reached 35,050 ± 32,665 and 31,283 ± 34,732, respectively, for an underfunding of 10.7%. Basic and comprehensive unit surgical performances were respectively 3036 ± 1009 and 3891 ± 1591 points in 2006. Both performances increased in sigmoid fashion to reach 3883 ± 1344 and 5335 ± 1314 points, respectively, in 2010. Top performances would be achieved in year 2011, and extrapolated costs would comprise about 19,434,094.92 (95% confidence interval, 11,961,491.22-22,495,764.42). The current underfunding of congenital heart surgery needs correction. The Aristotle score can help to adjust reimbursement according to complexity of procedures. Unit surgical performance allows accurate budgeting in the current German DRG system.

Functional outcome of anatomic correction of corrected transposition of the great arteries.

OBJECTIVE: Anatomic correction of corrected transposition of the great arteries, utilizing the morphologic left ventricle as a systemic pumping chamber, is considered the preferred method. The purpose of the study was to analyze the intermediate functional outcome following anatomical correction. METHODS: Between 1997 and 6/2010, 23 patients with corrected transposition of the great arteries and associated lesions underwent anatomical correction. Seventeen (74%) and six patients (26%) had situs solitus {S,L,L} and situs invs {I,D,D}, respectively. Fifteen patients (65%) had undergone 18 palliations before the corrective operation. The median age at palliation was 0.23 years, with a range of 0.016-8.4 years. A corrective, modified Senning-arterial switch procedure was performed in nine patients, 13 patients underwent a modified Senning-Rastelli procedure, and in one patient a combination of modified Senning and aortic translocation (Bex/Nikaidoh) was used. The median age at the corrective operation was 2 years (from 0.3 to 15.7 years). RESULTS: There was no mortality or heart transplant within the mean follow-up of 3.4 years. Freedom from reintervention was 77% at 5 years. There were no signs of obstruction of the systemic and pulmonary venous tunnels. The function of both ventricles was normal in all patients, even in the four patients who required retraining of the left ventricle. Mild aortic regurgitation was noticed in three patients. Preoperatively detected significant tricuspid regurgitation either disappeared or became trivial after the operation in all the six patients. All patients except two are in sinus rhythm; one patient is pacemaker-dependent preoperatively and one is pacemaker-dependent postoperatively. There were no clinically apparent neurological problems. All patients, but one, are in the New York Heart Association (NYHA) class I. CONCLUSIONS: Anatomic correction of corrected transposition of the great arteries can be performed in selected patients without mortality and with acceptable morbidity. The mid-term functional outcome is excellent, resulting in normal ventricular function, even in retrained left ventricles, and minimal incidence of complete heart block. The long-term function of the aortic valve, intraventricular tunnels, conduits, and ventricles requires close surveillance.

Congenital heart surgery: surgical performance according to the Aristotle complexity score.

OBJECTIVES: Aristotle score methodology defines surgical performance as 'complexity score times hospital survival'. We analysed how this performance evolved over time and in correlation with case volume. METHODS: Aristotle basic and comprehensive complexity scores and corresponding basic and comprehensive surgical performances were determined for primary (main) procedures carried out from 2006 to 2009. Surgical case volume performance described as unit performance was estimated as 'surgical performance times the number of primary procedures'. RESULTS: Basic and comprehensive complexity scores for the whole cohort of procedures (n=1828) were 7.74±2.66 and 9.89±3.91, respectively. With an early survival of 97.5% (1783/1828), mean basic and comprehensive surgical performances reached 7.54±2.54 and 9.64±3.81, respectively. Basic surgical performance varied little over the years: 7.46±2.48 in 2006, 7.43±2.58 in 2007, 7.50±2.76 in 2008 and 7.79±2.54 in 2009. Comprehensive surgical performance decreased from 9.56±3.91 (2006) to 9.22±3.94 (2007), and then to 9.13±3.77 (2008), thereafter increasing up to 10.62±3.67 (2009). No significant change of performance was observed for low comprehensive complexity levels 1-3. Variation concerned level 4 (p=0.048) which involved the majority of procedures (746, or 41% of cases) and level 6 (p<0.0001) which included a few cases (20, or 1%), whereas for level 5, statistical significance was almost attained: p=0.079. With a mean annual number of procedures of 457, mean basic and comprehensive unit performance was estimated at 3447±362 and 4405±577, respectively. Basic unit performance increased year to year from 3036 (2006, 100%) to 3254 (2007, 107.2%), then 3720 (2008, 122.5%), up to 3793 (2009, 124.9%). Comprehensive unit performance also increased: from 3891 (2006, 100%) to 4038 (2007, 103.8%), 4528 (2008, 116.4%) and 5172 (2009, 132.9%). CONCLUSIONS: Aristotle scoring of surgical performance allows quality assessment of surgical management of congenital heart disease over time. The newly defined unit performance appears to well reflect the trend of activity and efficiency of a congenital heart surgery department.

Surgical treatment of transposition of great arteries with ventricular septal defect and left ventricular outflow tract obstruction: midterm results.

Our purpose was to evaluate our single-center experience with the treatment of transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO). Between 1992 and 2009, 42 patients were operated on. Twenty-three patients underwent the Rastelli operation, 8 patients underwent arterial switch operation (ASO) with associated LVOTO procedures, 4 patients underwent the réparation à l'étage ventriculaire (REV) procedure, 3 patients underwent the Bex/Nikaidoh (BN) procedure, and the Fontan operation was performed in 4 patients. The median age at final operation was 20.7 months (range, 0.3-234). The overall survival rate was 97% (1 early death), with a median follow-up of 8.2 years. There were no differences in survival among the surgical groups. Event-free survival was 100%, 84%, 59%, and 24% at 1, 5, 10, and 15 years of follow-up, respectively, with it being worse in the Rastelli group (P < .0348). The last echocardiography showed good function of the systemic ventricle in all patients; LVOTO pressure gradient greater than 30 mm Hg was observed in 2 patients (5%), and right ventricular outflow tract obstruction (RVOTO) pressure gradient >30 mm Hg was observed in 12 patients (31%). All patients are in sinus rhythm, and 74% of them are without medication. All surgical approaches are safe and show excellent midterm functional outcome. ASO is the best option if the LVOTO is resectable. Intraventricular rerouting (Rastelli or REV) is the method of choice in the majority of patients, but Rastelli has a significant reintervention rate. The BN operation has the potential to minimize utilization of the Fontan operation, which was used in the past if the intracardiac anatomy was unfavorable.

Pulmonary artery sling with tracheal stenosis.

Pulmonary artery sling (PAS) is a rare congenital heart disease in which the left pulmonary artery (LPA) originates from the right pulmonary artery (RPA) and encircles the distal trachea and right mainstem bronchus as it courses between the trachea and esophagus. Typically, patients with PAS have some respiratory symptoms, either due to external tracheal compression that can be corrected by relief of the sling mechanism, or due to severe diffuse tracheal stenosis with complete rings (ring-sling complex). The diagnosis of PAS is optimally made by echocardiography, while bronchoscopy is the key to the assessment of tracheal stenosis. Diagnosis is indication for surgery. Repair using a strategy of median sternotomy, cardiopulmonary bypass, division of the LPA and reimplantation into the main pulmonary artery (MPA), and simultaneous tracheal repair takes preference. Tracheal repair should be considered only in clinically symptomatic patients. The techniques of free tracheal autograft plasty or slide tracheoplasty offer promising results, and the choice of tracheal reconstruction should be guided by the clinical experience of the surgeon. Coexisting intracardiac pathologies are repaired at the same time. Postoperative care requires close multidisciplinary effort to achieve the best long-term result.

Survival and reintervention after neonatal repair of truncus arteriosus with valved conduit.

OBJECTIVE: Neonatal primary repair has progressively become the treatment of choice for truncus arteriosus with encouraging survival. However, use of valved conduits to reconstruct the right ventricular outflow tract (RVOT) inevitably induces reintervention. This study estimates survival and rate of catheter-interventional and surgical reinterventions. METHODS: Thirty-five consecutive neonates who underwent truncus repair with 27 homografts and 8 Contegras from 1987 to 2007 were studied. Interrupted aortic arch (IAA) was associated in nine patients. Actuarial survival and freedom from reintervention were evaluated according to Kaplan-Meier method. RESULTS: Five patients died early after repair. Two died late and one death was related to reintervention. Survival was 91.9%+/-5.4% from postoperative month 2 onwards when IAA was not associated and 41.7%+/-17.3% from month 4 in IAA presence. During a median follow-up of 68 months (range 1-180 months), 42 reinterventions (of which 17 reoperations) were performed in 21 patients. Rate of reintervention was 2.6 per early survivor per 10 years. RVOT obstruction constituted the main indication: branch pulmonary arteries often being involved (n=25). Uncommon indication was subaortic stenosis (n=3), aortic arch obstruction (n=2) and truncal valve regurgitation (n=2). At year 10, freedom from first, second and third reintervention was 17.9 %+/-8.1%, 46.1%+/-10.6% and 81.9%+/-9.5%, respectively. Sixteen first conduits were explanted. Freedom from first conduit replacement was 87.5%+/-6.8%, 64.1%+/-10.2% and 39.5%+/-10.7% at year 1, 3 and 5, respectively. Homografts enjoyed higher durability than Contegras. CONCLUSION: Neonatal repair of truncus arteriosus results in high survival, the only risk being IAA association. The rate of reintervention is heavily influenced by stenosis of branch pulmonary arteries.

Primary early correction of tetralogy of Fallot irrespective of age.

OBJECTIVE: The policy of early repair of patients with tetralogy of Fallot, irrespective of age, as opposed to initial palliation with a shunt, remains controversial. The aim of our study was to analyze the midterm outcome of primary early correction of tetralogy of Fallot. METHODS: Between 1996 and 2005, a total of 61 consecutive patients less than 6 months of age underwent primary correction of tetralogy of Fallot in two institutions. The median age at surgery was 3.3 months, and 27 patients (44%) were younger than 3 months of age, including 12 (20%) newborns. We analyzed the patients in 2 groups: those younger than 3 months of age, and those aged between 3 and 6 months. RESULTS: There was one early (1.6%), and one late death. Actuarial survival was 98.4%, 96.7%, 96.7% at 1, 5, and 10 years respectively, with a median follow up of 4.5 years. There was no difference in survival, bypass time, lengths of ventilation, and hospital stay between the groups. A transjunctional patch was placed significantly more often in the patients younger than 3 months (p = 0.039), with no adverse effect on survival and morbidity during the follow-up. Freedom from reoperation was 98.2%, 92.2%, and 83% at 1, 5, and 10 years respectively, with no difference between the groups. CONCLUSION: Elective primary repair of tetralogy of Fallot in asymptomatic patients is delayed beyond 3 months of age. In symptomatic patients, primary repair of tetralogy of Fallot is performed irrespective of age, weight and preoperative state. This approach is safe, and provides an excellent midterm outcome with acceptable morbidity and rates of reintervention. The long-term benefits of this approach must be established by careful follow-up, with particular emphasis on arrhythmias, right ventricular function, and exercise performance.

Early cardiac perforation after atrial septal defect closure with the Amplatzer septal occluder.

We report a case of aortic perforation after transcatheter closure of an atrial septal defect by an Amplatzer septal occluder. During emergency surgery, perforations of the dome of the right atrium and the noncoronary sinus of Valsalva of the aorta were repaired. Atrial septal defect was primarily closed. A short anterosuperior rim should be considered a risk factor for aortic perforation in transcatheter atrial septal defect closure.

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification.

BACKGROUND: The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO). We reviewed our experience with DORV patients and two ventricles that underwent repair, applying this nomenclature. METHODS: Between January 2000 and January 2005, 50 patients with DORV and two viable ventricles underwent surgical intervention: 44 patients had biventricular repair, 3 had 1.5 ventricular repair, 2 underwent a Fontan, and 1 died prior to corrective surgery. Median age at repair was 9.1 months (range: 4D-4Y). Eighteen patients (36%) were DORV-Fallot (including 5 with AVSD and heterotaxy), 9 (18%) were DORV-TGA (Taussig-Bing), 12 (24%) were DORV-VSD, and 11 (22%) were DORV non-committed VSD. Corrective surgery included 35 repairs with a VSD-aorta baffle+/-RVOTO procedure and 12 arterial switches with a VSD-PA baffle (9 Taussig-Bing and 3 DORV-ncVSD). Associated procedures included 13 VSD enlargements, 8 subaortic resections, 9 arch repairs, 5 AVSD repairs, and 7 others. RESULTS: There were three deaths in the 50 patients studied (overall mortality of 6%). Excluding one patient that died prior to corrective surgery and the two patients palliated with a Fontan procedure, the actual surgical mortality for a corrective repair was 4.3% (2/47 patients). Two surgical deaths occurred following, respectively, one repair of a Taussig-Bing with an interrupted arch and a Swiss cheese VSD and one repair of ncVSD-type with pulmonary atresia that had undergone a previous cavo-pulmonary anastamosis. No late deaths occurred. Two late reoperations included a heart transplant in a DORV-Fallot patient with Swiss cheese VSD and subaortic resection in a DORV-ncVSD patient. Angioplasties were needed for PA stenosis (n=2) and aortic arch obstruction (n=2). Four patients had LV to aorta baffle gradients between 10 and 20 mmHg. All patients were of NYHA class I/II. CONCLUSIONS: The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality. Biventricular repair can be safely achieved on selected DORV, including DORV-ncVSD and DORV with AVSD and heterotaxy lesions traditionally indicated for a single ventricle palliative approach.

Ross-Konno procedure in neonates: report of three patients.

The Ross-Konno procedure, applied to neonates with severe left ventricular outflow tract (LVOT) obstruction, offers a satisfactory solution in fully releasing the LVOT gradient, and in replacing the aortic valve with a pulmonary autograft with an excellent growth potential. We reported on three recent neonatal cases.