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To this day, controversy still exists regarding the optimal method to treat symptomatic neonates and infants with Tetralogy of Fallot (TOF). Symptomatic (severely cyanotic or ductal dependent) infants with TOF can undergo either a staged repair approach (consisting of initial palliation followed by complete repair) or primary repair. Traditionally, initial palliative procedures have been surgical, for example placement of a Blalock-Taussig-Thomas (BTT) shunt. Recent advances in technology have facilitated the introduction of catheter-based procedures as palliative techniques, for example, patent ductus arteriosus (PDA) stenting and right ventricular outflow tract (RVOT) stenting as more durable solutions than balloon pulmonary valvuloplasty (BPV). In this article, we discuss the rationale for these procedures, technical aspects of these procedures and outcomes data compared to traditional surgical procedures. Recent data have suggested that RVOT and PDA stenting procedures offer many advantages over traditional surgical palliative procedures as palliative methods in this patient population. This comes at a cost of increased reintervention burden, which may be considered part of the overall treatment strategy in smaller neonates and can be minimized with a focus on technical aspects and overall treatment strategies. Advanced surgical techniques are required at the eventual complete repair to negotiate removal of stent material and pulmonary artery reconstruction in some instances. Further adoption of catheter based palliative procedures for infants with symptomatic TOF has the potential to tip the outcomes towards favoring a staged approach, particularly in high-risk infants.

Reoperative aortic root replacement, following prior biologic or mechanical valved conduit aortic root prosthesis, presents a technical challenge. The rapid-deployment aortic valve prosthesis is an approved alternative to traditional bioprosthetic aortic valve replacement. We present three clinical cases in which rapid-deployment aortic valve prostheses were utilized in lieu of reoperative full aortic root replacement. All three patients recovered uneventfully. The rapid-deployment valve insertion in a prior surgical aortic root prosthesis is a safe option to avoid reoperative full aortic root replacement.

We present a rare clinical scenario of a patient with tetralogy of Fallot, hypertrophic cardiomyopathy, and concomitant scimitar syndrome. We created a scimitar vein cuff from a recipient heart, performed its translocation, and subsequently performed heart transplantation. This ingenuity reduces the likelihood of scimitar vein obstruction.

Herein, we present a neonatal case of coarctation of the aorta, with aortic arch thrombus confirmed by echocardiography. We performed thrombus removal and aortic arch repair emergently. This critical condition necessitates quick preoperative evaluation with echocardiography. Moreover, postoperative evaluation using computed tomography is reasonable to assess an aortic arch configuration, and exclude the remnant thrombus.

Potential surgical strategies for hypoplastic branch pulmonary arteries (BPAs) during tetralogy of Fallot repair include (1) extensive patch augmentation to the hilum (PATCH), (2) limited extension arterioplasty to the proximal pulmonary artery (EXTENSION), or (3) leaving the native vessels unaugmented (NATIVE). We explored the effect of these strategies on reintervention and BPA growth.From 2000 to 2012, 434 children underwent complete tetralogy of Fallot repair. Risk-adjusted parametric models were used to analyze the risk of BPA reintervention for (1) all children, (2) children with BPAs of 4 mm or smaller, and (3) children with BPAs of 3 mm or smaller. Repeated-measures analysis of more than 2,000 echocardiograms was used to characterize postoperative BPA growth and right ventricular pressure by using nonlinear mixed models.Overall survival (99% [3 deaths]) was excellent. The 10-year freedom from BPA reintervention was 84%. In risk-adjusted models (including baseline BPA z-score), PATCH had a decreased freedom from reintervention (73%; p0.01) vs EXTENSION (87%) or NATIVE (91%). For children with BPAs of 4 mm or smaller (28 PATCH, 60 EXTENSION, 75 NATIVE), baseline characteristics were similar. The risk-adjusted 5-year freedom from reintervention was 68% for PATCH, 76% for EXTENSION, and 85% for NATIVE. PATCH trended toward an increased risk of reintervention (p = 0.07). For children with BPAs of 4 mm or smaller left in their NATIVE state, only ∼15% required reintervention. After adjustment for baseline BPA z-score, the time-related BPA growth was decreased (p0.014) and right ventricular pressure was increased (p = 0.03) for the PATCH group.Aggressive PATCH augmentation of hypoplastic BPAs improves the short-term geometry but may lead to late stenosis and higher rates of reintervention. Hypoplastic BPAs in tetralogy of Fallot tend (∼85%) to grow well without instrumentation.

Objectives This study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a “best practice” protocol for imaging and interpreting images in establishing the diagnosis of AAOCA. Background AAOCA is associated with sudden death in the young. Underlying anatomic risk factors that can cause ischemia-related events include coronary arterial ostial stenosis, intramural course of the proximal coronary within the aortic wall, interarterial course, and potential compression between the great arteries. Consistent protocols for diagnosing and evaluating these features are lacking, potentially precluding the ability to risk stratify patients based on evidence and plan surgical strategy. Methods For a prescribed set of anatomic AAOCA features, percentages of missing data in institutional echocardiographic reports were calculated. For each feature, agreement among institutional echocardiographic reports, ICL review of images, and surgical reports was evaluated using the weighted kappa statistic. An echocardiographic imaging protocol was developed heuristically to reduce differences between institutional reports and ICL review. Results A total of 13%, 33%, and 62% of echocardiograms were missing images enabling diagnosis of intra-arterial course, proximal intramural course, and high ostial takeoff, respectively. There was poor agreement between institutional reports and ICL review for diagnosis of origin of coronary artery, interarterial course, intramural course, and acute angle takeoff (kappa = 0.74, 0.11, –0.03, 0.13, respectively). Surgical findings were also significantly different from those of reports, and to a lesser extent ICL reviews. The resulting protocol contains technical recommendations for imaging each of these features. Conclusions Poor agreement between institutional reports and ICL review for AAOCA suggests need for an imaging protocol to permit evidence-based risk stratification and surgical planning. Even then, delineation of echocardiographic details in AAOCA will remain imperfect.

Background We hypothesized that primary sutureless (SL) repair of total anomalous pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair pulmonary vein obstruction (PVO) and different modes of PVO compared with standard repair (SR). Methods One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital pulmonary vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups. The mode of PVO was expressed as central or peripheral. The Mann-Whitney test, Kaplan-Meier analysis, and Cox regression were used. Results The SL group had more infracardiac or mixed TAPVD ( p = 0.02) and preoperative PVO ( p = 0.07). There were no differences between SR and SL groups in survival (5-year survival, 83.1% versus 82.5%, respectively; p = 0.73) and composite outcome (death, intervention, PVO, 5-year survival, 76.4% versus 80.7%, respectively; p = 0.225). The SL group had a lower incidence of PVO of moderate or greater degree (SR, 11.3% versus SL, 2.9%; p = 0.05) than the SR group, especially in the infracardiac and mixed TAPVD cohort ( p = 0.011), with a lower pulmonary vein score (SR, 8 versus SL, 4; p = 0.01). The SL group had peripheral PVO exclusively (100%), whereas the SR group predominantly had central PVO (76.4%; p = 0.005). There was a trend toward less reoperation in the SL group (SR, 10.4% versus SL, 2.9%; p = 0.08). Survival after reoperation was comparable to primary TAPVD repair types as well as reoperation repair types. Conclusions Primary SL appeared to be associated with a lower incidence and severity of PVO. The primary SL repair eliminated the risk of developing central PVO, although a relatively benign type of peripheral PVO could occur.

Objectives To evaluate the durability of aortic valve (AoV) repair relative to other strategies for children with significant aortic insufficiency (AI). Methods From 2001 to 2012, 90 children with greater than or equal to moderate AI underwent surgery. Resulting procedures were classified according to final operative outcome: AoV repair (repair; n = 46, 51%), Ross procedure (Ross; n = 21, 23%) or replacement with mechanical or tissue prosthesis [aortic valve replacement (AVR); n = 23, 26%]. Repeated measures (n = 1081 echocardiograms) mixed-model analysis and parametric multiphase risk-adjusted hazard analysis were used to evaluate haemodynamic parameters and durability of operations. Results Mean age at operation was similar for repair and Ross groups, but slightly higher for the AVR group (10.6, 11 and 13.2, respectively; P = 0.04). Baseline annular dimensions were similar among groups. Of 46 repairs, 85% involved pericardial leaflet extensions (commonly with leaflet shaving and/or commisuroplasty). The remaining repairs were commissuroplasties. On multivariable analysis, repair was associated with increased early (∼1-2 years) AI and increased outflow tract peak pressure gradients relative to Ross and AVR procedures. On univariate analysis, repairs tended to have a larger annulus size compared with Ross or AVR; however, this was not significant on multivariable analysis. There were 25 reinterventions (surgical reoperation = 16; transcatheter intervention = 9) for 22 children. Freedom from surgical reoperation was 64, 100 and 51% at 6 years for repairs, Ross and AVR, respectively (P = 0.05); however, three of five reoperations after AVR were for failed bioprosthetic devices. The freedom from reintervention was not significantly influenced by the type of AoV operation (P = 0.43). Conclusions Durability of aortic valve repair for children is limited by recurrence of AI and/or stenosis, often within the first few years. After repair, reoperation should be anticipated within ∼7 years.

Infants with severe tetralogy of Fallot may undergo (1) early primary surgical repair (EARLY) or (2) early transcatheter palliation (CATH) before delayed surgical repair. We compared these strategies with (3) elective single-stage tetralogy of Fallot repair (IDEAL).From 2000 to 2012, 453 children underwent tetralogy of Fallot repair (excluding systemic-pulmonary shunts), including 383 in the IDEAL (75%), 42 in the EARLY (9%), and 28 in the CATH (6%) groups. IDEAL repair at The Hospital for Sick Children occurs after 3 months. Risk-adjusted hazard analysis compared freedom from surgical or catheter reintervention. Somatic size, branch pulmonary artery size, and right ventricle systolic pressure were modeled using 2780 echocardiogram reports via mixed-model regression.CATH involved right ventricular outflow tract stent in 18 patients, right ventricular outflow tract balloon in 9 patients, and ductal-stent in 1 patient. Three patients died (1 per group). Risk-adjusted freedom from surgical reoperation was 89% ± 4%, 88% ± 5%, and 85% ± 6% for the IDEAL, EARLY, and CATH groups, respectively, at 10 years. Patients in the EARLY and CATH groups had similar reoperation rates, except for neonates (1 month), for whom EARLY repair conferred an increased risk of reoperation. Risk-adjusted freedom from catheter reintervention was lower in the EARLY group (76%) and especially for the CATH group (53%) at 10 years versus the IDEAL group (83%). Somatic growth and progression of right ventricle systolic pressure were similar among groups at 8 years. Although those undergoing EARLY (P = .02) and CATH (P = .09) tend to have smaller branch pulmonary arteries initially, late pulmonary artery size was not significantly different among groups.Early primary repair for neonates may increase surgical reoperation, whereas transcatheter palliation comes at a cost of increased catheter reintervention. However, overall outcomes between groups, in terms of survival, growth, and hemodynamic parameters, were comparable, suggesting that both strategies are a reasonable option for children with severe tetralogy of Fallot.

Introduction: Infants with severe tetralogy of Fallot (TOF) may undergo: 1) early primary surgical repair (EARLY) or 2) early catheter palliation (CATH) prior to delayed surgical repair. We compared these two strategies to 3) usual elective single stage TOF repair (USUAL). Methods: We studied 453 TOF repairs (2000-2012, excluding BT shunts). USUAL strategy at our institution is repair ≥3 months. Risk adjusted hazard analysis compared freedom from surgical or catheter reintervention. Somatic size, branch PA size and RV systolic pressure were modeled using 2543 echo reports via mixed model regression. Results: Table: group characteristics for USUAL (383), EARLY (42) and CATH (28). CATH involved: RVOT stent=18, RVOT balloon=9, ductal stent=1. Risk adjusted freedom from surgical reoperation was 88%, 87% and 85% for USUAL, EARLY and CATH respectively, at 10 years. EARLY and CATH had similar reoperation rates, except for very young children ( Risk adjusted freedom from catheter reintervention was higher for EARLY (76%) and especially so for CATH (53%) at 10 years, versus USUAL (83%; Figure). Somatic growth and progression of RVSP (37-40mmHg) was similar among groups at 8 years. EARLY (P=.02) and CATH (P=.09) tend to have smaller bPAs initially. The CATH group tend to remain smaller in the long-term, whereas growth in EARLY matches USUAL. Conclusions: Early primary repair at very young ages comes with a cost of increased late surgical reoperation. Early transcatheter palliation tends to come with a cost of increased late transcatheter intervention - possibly related to slower branch PA growth.

To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia.Infants aged3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%). Multiphase parametric-hazard models were used to analyze competing outcomes.Risk-adjusted 6-year survival was lower after SPS (85%; P = .04) versus PAB (93%) or SCPC (93%). Survival after SPS when the main pulmonary artery (MPA) was closed (n = 21) or banded (n = 4) was 60%, versus 93% without MPA intervention (P = .02). After SPS, survival before SCPC was lower with an open ductus arteriosus (n = 7; 76% vs 97%; P = .02). Similarly, after SPS, risk-adjusted survival was similar to that for patients who had an initial PAB or SCPC when MPA intervention was avoided and the ductus arteriosus either closed spontaneously before SPS, or was closed during SPS. For all patients reaching SCPC (n = 277), survival to Fontan was not significantly influenced by whether PBF persisted through the MPA.Tricuspid atresia patients with SPS represent a high-risk subgroup. Avoiding an open ductus arteriosus and concomitant MPA intervention during SPS may help mitigate the risk associated with SPS. The presence of antegrade PBF through the MPA, at initial and staged operations, did not significantly influence survival to Fontan operation.

This study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a "best practice" protocol for imaging and interpreting images in establishing the diagnosis of AAOCA.AAOCA is associated with sudden death in the young. Underlying anatomic risk factors that can cause ischemia-related events include coronary arterial ostial stenosis, intramural course of the proximal coronary within the aortic wall, interarterial course, and potential compression between the great arteries. Consistent protocols for diagnosing and evaluating these features are lacking, potentially precluding the ability to risk stratify patients based on evidence and plan surgical strategy.For a prescribed set of anatomic AAOCA features, percentages of missing data in institutional echocardiographic reports were calculated. For each feature, agreement among institutional echocardiographic reports, ICL review of images, and surgical reports was evaluated using the weighted kappa statistic. An echocardiographic imaging protocol was developed heuristically to reduce differences between institutional reports and ICL review.A total of 13%, 33%, and 62% of echocardiograms were missing images enabling diagnosis of intra-arterial course, proximal intramural course, and high ostial takeoff, respectively. There was poor agreement between institutional reports and ICL review for diagnosis of origin of coronary artery, interarterial course, intramural course, and acute angle takeoff (kappa = 0.74, 0.11, -0.03, 0.13, respectively). Surgical findings were also significantly different from those of reports, and to a lesser extent ICL reviews. The resulting protocol contains technical recommendations for imaging each of these features.Poor agreement between institutional reports and ICL review for AAOCA suggests need for an imaging protocol to permit evidence-based risk stratification and surgical planning. Even then, delineation of echocardiographic details in AAOCA will remain imperfect.

Introduction: We introduced the NASA “threat and error model” to our surgical unit; all admissions are considered “flights”, which should pass through stepwise de-escalations in risk. Hypothesis: Errors significantly influence risk de-escalation and contribute to poor outcomes. Methods: Patient flights (524) were tracked real-time for threats, errors and unintended states (figure). Expected risk de-escalation was: wean from mechanical support, sternal closure, extubation, ICU discharge and discharge home. Data were accrued via performance personnel, bedside data, reporting mechanisms and staff interviews. Infographics of flights were openly discussed weekly. Results: In 12% (64/524) of flights, the child failed to de-escalate sequentially through expected risk levels; unintended increments instead occurred. Failed de-escalations were highly associated with errors (426; 257 flights), however seemingly benign (P The most dangerous errors were “apical” errors typically (84%) occurring in the OR which led to cycles of propagating unintended states (n=110): these had 43% (47/110) rate of failed de-escalation (vs 4%, P Conclusions: Deaths and brain injury almost always occur from propagating error cycles that originate in the OR and are often amplified by additional ICU errors. Improvements in threat management, error detection/rescue and vigilance at times of failed de-escalation will translate into improved outcomes.

Pulmonary vein characteristics that influence survival after repair of stenosis have not been defined. We sought to develop a predictive model relating postrepair survival to preoperative pulmonary vein characteristics on computed tomography and magnetic resonance imaging.Patients who underwent pulmonary vein stenosis repair (1990-2012) with preoperative computed tomography and magnetic resonance imaging were reviewed. We measured pulmonary vein short and long cross-sectional diameters at the left atrial junction (downstream), vein bifurcation (upstream), and narrowest point, and calculated the total cross-sectional area indexed for body surface area. The relationship between pulmonary vein dimensions and survival was related via risk-adjusted parametric hazard analyses.Of 145 patients who underwent surgical repair, 31 had preoperative computed tomography and magnetic resonance imaging and were analyzed. Surgical repairs were sutureless (n = 30) or pericardial patch reconstruction (n = 1). Mean follow-up was 4.28 ± 4.2 years. In-hospital mortality was 9.7%; unadjusted survival was 75% ± 7%, 69% ± 8%, and 64% ± 7% at 1, 3, and 5 years, respectively. Median downstream total cross-sectional area indexed for body surface area was 163 mm(2)/m(2), upstream total cross-sectional area indexed for body surface area was 263 mm(2)/m(2), and total cross-sectional area indexed for body surface area at maximal stenosis, localized at the left atrial junction in approximately two thirds of patients, was 163 mm(2)/m(2). Smaller upstream total cross-sectional area indexed for body surface area (P = .030) and greater number of stenotic pulmonary veins (P = .0069) were associated with increased early (1 year) risk of death. Smaller downstream total cross-sectional area indexed for body surface area tended to be associated with a late risk of death (P = .059).Smaller upstream or downstream total cross-sectional area indexed for body surface area negatively influenced survival. Early survival seemed especially poor for patients with a greater number of stenotic veins and upstream pulmonary vein involvement. The total cross-sectional area indexed for body surface area measurements can help to inform prognosis and stratify patients for enrollment in clinical trials of agents directed at pulmonary vein pathology.

Early survival advantages after Norwood with right-ventricle-(RV)-to-pulmonary-artery conduit (NW-RVPA) over Norwood-operation with a Blalock-Taussig shunt (NW-BT) are offset by concerns regarding delayed RV dysfunction. We compared trends in survival, RV dysfunction, and tricuspid valve regurgitation (TR) between NW-RVPA and NW-BT for propensity-matched neonates with critical left ventricular outflow tract obstruction (LVOTO).In an inception cohort (2005-2014; 21 institutions), 454 neonates with critical LVOTO underwent Norwood stage 1. Propensity-score matching paired 169 NW-RVPA patients with 169 NW-BT patients. End-states were compared between NW-RVPA and NW-BT using competing-risks, multiphase, parametric, hazard analysis. Post-Norwood echocardiogram reports (n = 2993) were used to grade RV dysfunction and TR. Time-related prevalence of ≥moderate RV dysfunction and TR were characterized using nonlinear mixed-model regression, and compared between groups via multiphase, parametric models.Overall 6-year survival was better after NW-RVPA (70%) versus NW-BT (55%; P.001). Additionally, transplant-free survival during this time was better after NW-RVPA (64%) versus NW-BT (53%; P = .004). Overall prevalence of ≥moderate RV dysfunction reached 11% within 3 months post-Norwood. During this time, RV dysfunction after NW-BT was 16% versus 6% after NW-RVPA (P = .02), and coincided temporally with an increased early hazard for death. For survivors, late RV dysfunction was5% and was not different between groups (P = .36). Overall prevalence of ≥moderate TR reached 13% at 2 years post-Norwood and was increased after NW-BT (16%) versus NW-RVPA (11%; P = .003). Late TR was similar between groups.Among propensity-score-matched neonates with critical LVOTO, NW-RVPA offers superior 6-year survival with no greater prevalence of RV dysfunction or TR than conventional NW-BT operations.

BACKGROUND: In older children with severe aortic insufficiency (AI), a strategy of aortic valve REPAIR is often pursued in order to delay ROSS procedure or aortic valve replacement with a mechanical/bioprosthetic device (AVR). We aimed to test this strategy of valve REPAIR by evaluating its durability. METHODS: From 2001-2012, 90 children with severe AI underwent: REPAIR (N1⁄446, 51%), ROSS (N1⁄421, 23%) or AVR (N1⁄423, 26%). Repeated measures (N1⁄41081 echos) mixed model analysis were used to evaluate haemodynamic outcomes. RESULTS: Mean age at operation was identical for REPAIR and ROSS (11 years), but slightly older for AVR (13 years). Annular dimensions were largest for REPAIR versus either AVR or ROSS (P1⁄4.01, figure). Cardiopulmonary bypass times were significantly shorter for REPAIRS versus either ROSS or AVR (figure, P

BACKGROUND: 51 years ago, the first Mustard operation was performed at our institution and was successful; the patient is still alive today. The procedure’s success was such that many thought they were “cured”. They were not; “failing Mustards” with severe RV failure, TR and often pulmonary hypertension present to us with increasing frequency. We reviewed our experience with an emphasis on this “Mustard dilemma”. METHODS: In 2010-2013, we undertook multiple iterations of cross-sectional follow-up of all 546. Mustard procedures as part of a “double-switch” operation were not included. All points of last known contact were pursued via as many avenues (primary cardiologists, surgeons, institutions, family physicians). RESULTS: Institutional data protection policies were extremely difficult obstacles, as were breaks in follow-up continuity caused by physician retirement or death. The 546 Mustards (1963 2007) were performed at mean age 2.1 years for diagnoses including predominantly variants of arterial transposition or DORV. Most had undergone prior palliation via atrial septectomy (249), systemic-pulmonary shunt (19), PA band (44) or cavopulmonary connection (3). DHCA was used in w60% (mean 55 minutes). In-hospital mortality was 9%. Following discharge, late survival was excellent, with very slow rising late hazard for death over the subsequent decades (instantaneous risk w1%/ year after 40 years). Estimated survival 50 years after surgery is w46% overall (but 58% for isolated TGA). There have been 184 known deaths (33%). Of the 372 presumed survivors, the median age today would be 36 years (IQ range 30 40). More than 20% of adult Mustards we follow have decompensated heart failure by age 36, and this proportion rises disproportionately thereafter (figure). At last follow-up, prevalence of moderate TR was 20% and RV dysfunction 34%. Nevertheless, follow-up with a cardiologist is known for only 50% (182/372) of presumed survivors since 2010, and 60% (224/372) since 2000. The remaining 148 (40% of survivors) only had last known points of cardiology contact in the 1980s or 1990s. CONCLUSION: The bulk of adult Mustard survivors are approaching high-risk periods for onset of heart failure. The insidious onset of RV dysfunction, TR and pulmonary hypertension emphasizes the need for national tracking registry for this and other complex CHD patients, similar to those for cancer patients, to prevent patients re-presenting with excessive risk for any intervention, including transplantation. Canadian Cardiovascular Society (CCS) Poster HEART FAILURE: IMAGING, TREATMENT AND OUTCOMES Sunday, October 26, 2014

BACKGROUND: In tetralogy of Fallot (TOF), the risk of cyanotic spells can lead to emergency surgery like palliative systemicpulmonary shunt before the ideal time to do complete repair. During corrective surgery in case of insufficient development of the pulmonary valve, the use of a trans-annular patch is required which, in the long-term, causes excessive dilatation of the right ventricle, arrhythmia and sudden death. The development of the pulmonary branch and the onset of cyanotic spells are conditioned by the volume of the trans-valvular flow. METHODS: We conducted a retrospective study to determine whether elective primary pulmonary valvuloplasty (EPPV) in the neonatal period 1) allows a better development of the pulmonary valve and artery; 2) reduces the risk of cyanotic spells and the use of emergency surgery; 3) increase the possibilities of conservative surgery of the pulmonary valve. Patients with simple TOF who received an EPPV within the first month of live between 2000 and 2010were compared to an observational group (OBS).Main parameters included progress of O2-saturation, pulmonary valve and artery growth, need for urgent aorto-pulmonary shunt, need for trans-annular patch reconstruction. RESULTS: Of a total of 203 patients identified, 42 were analyzed (23 EPPV and 19 OBS). At birth O2-saturation was 91.52 7.12 respectively vs 94.16% 3.5%; p1⁄4 0.043; The Z score of the pulmonary valve was -3.01 2.12 vs 0.508 1.71 (p1⁄40.66) and Nakata index 115.12 39.67 vs 156.3 43.06 mm2/m2 (p1⁄40.001). At the time surgery the growth of pulmonary annulus was z-score: -3.47 2.09 (p1⁄40.73) vs -3.11 2.43 (p1⁄40.214). Nakata index increased to 174.51 93.74 (p1⁄4 0.57) in EPPV and to 151.19 74.14 (p1⁄40.14) in OBS, this increase was not significant between the 2 groups however (39.59 91.39 vs 4.85 78.34, p1⁄40.214). The need for pulmonary re-intervention after corrective surgery was lower in EPPV 4/22(18%) vs. OBS 6/20(30 %); p1⁄40.04. CONCLUSION: Neonatal EPPV in simple TOF allows appropriate growth of the pulmonary arteries and reduces the rate of re-intervention on the pulmonary arteries after corrective surgery. In this small series, there was no significant difference in the use of a transannular patch or on cyanotic spells.