Primary Repair Of Tetralogy Of Fallot Research Articles

Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants

ObjectiveWe sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF). MethodsA single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time. ResultsOf 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values < .05). Over time, patients with TOF-variant demonstrated relatively greater annular (P = .020), sinus of Valsalva (P < .001), and sinotubular junction (P < .001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P < .001), moderate or greater aortic regurgitation (P < .001), and aortic valve repair or replacement (P = .045). ConclusionsPatients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up.

End-diastolic forward flow in repaired tetralogy of Fallot: Mid-term outcomes from a single center.

Pulmonary arterial end-diastolic forward flow (EDFF) following repaired tetralogy of Fallot (rTOF) is recognized as right ventricular (RV) restrictive physiology, which is closely related to poor prognosis. This study sought to review mid-term experience and investigate the risk factors of EDFF in the rTOF patients. From September 2016 to January 2019, 100 patients (age < 18 years old) who underwent complete tetralogy of Fallot (TOF) repair were enrolled and were divided into EDFF group (n = 52) and non-EDFF group (n = 48) based on the presence of postoperative EDFF. Elastic net analysis was performed for variable selection. Univariate and multivariate logistic analyses were used to analyze the correlation between risk factors and EDFF. End-diastolic forward flow group had lower systolic blood pressure (P = 0.037), diastolic blood pressure (P = 0.027), and higher vasoactive-inotrope score within 24 h after surgery (P = 0.022) than non-EDFF group. Transannular patch (TAP) was an independent predictor of postoperative EDFF [P = 0.029, OR: 2.585 (1.102∼6.061)]. Patients were followed up for a median of 2.6 years [interquartile range (IQR) 1.6] after the first TOF repair. During follow-up, the prevalence of the EDFF was lower in those with pulmonary valve (PV) reconstructions than that in those undergoing patch enlargement without PV reconstructions in the primary TOF repair (P < 0.001). End-diastolic forward flow was associated with TAP. Patients with EDFF might have a transient hemodynamic instability in the early postoperative period. PV reconstructions in the TOF repair might reduce the incidence of EDFF in the mid-term follow-up.

Abstract 12930: Plasma Proteome Distinguishes Adaptive and Maladaptive Signaling in Repaired Tetralogy of Fallot

Introduction: Patients who have undergone tetralogy of Fallot (TOF) repair are subject to chronic volume and/or pressure overload, leading to a 40% probability of right ventricular (RV) failure by the third decade of life. We sought to identify a plasma proteome profile of the systemic response to right heart stress to better understand the mechanisms of RV failure and identify novel RV remodeling biomarkers. Methods: Patients with repaired TOF were recruited at the time of standard of care cardiac MRI (CMR) (N=14), and blood samples were collected. Plasma proteomics was assessed using data independent acquisition mass spectrometry. Proteins that were significantly affected between groups were identified and used for pathway enrichment analysis. Results: Patients underwent primary TOF repair at median 2.9m (IQR 0.8, 4.1) and CMR at median 16yrs (IQR 15, 18). Patients were grouped based on median indexed RV end diastolic volume (RVEDVi). Group 1: median RVEDVi 146ml/m 2 (IQR 139, 164) (N=9); Group 2: median RVEDVi 114ml/m 2 (IQR 104, 130) (N=5); p&lt;0.001. RV ejection fraction was equal between groups (mean 56% vs. 55%; p=0.68). Forty-one proteins were significantly differentially abundant: 19 enriched in Group 1 and 22 enriched in Group 2 (Fig 1; p&lt;0.05). Adaptive signaling in Group 1 was characterized by upregulation of proteins involved in antioxidant mechanisms (SOD1, TXN, PRDX1&amp;2), calcium handling (S100A6, CALM1), and proteostasis (UBB, HSPA8). Maladaptive signaling was characterized by upregulation of glycolytic pathways (MDH1, PGK1) and heme protein oxidation (ALAD) and downregulation of proteins to counter acute stress (complements, ORM1&amp;2). Conclusion: Patients with repaired TOF and moderate-severe RV dilation have a distinct maladaptive plasma proteomic profile independent of ejection fraction. These data suggest that profiling the circulating plasma proteome may be used to monitor disease progression prior to the onset of clinical heart failure.

Predictors of short versus prolonged PCICU stay after primary repair of tetralogy of Fallot at a tertiary care unit, Karachi: A single-center study

BackgroundPediatric Cardiac Intensive Care Units (PCICU) stays have been associated with adverse outcomes as documented in the literature. However, data remain scarce on the factors which influence the length of such stays in the developing world. ObjectiveTo find out predictors of short versus prolonged PCICU stay after primary repair of tetralogy of Fallot (ToF). MethodsA prospective study was conducted at the Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases (NICVD), Karachi, Pakistan, from March 2021 to August 2021. A total of 93 patients of any age undergoing primary ToF repair were included. Patients having a PCICU stay <48 h were labeled as short PCICU stay, while those with a PCICU stay ≥48 h were termed as prolonged PCICU stay. Multivariate binary logistic regression analysis was performed to identify factors predicting prolonged PCICU stay. ResultsOverall, the mean age was noted to be 10.75 ± 4.54 years. The mean duration of PCICU was calculated to be 74.1 ± 50.2 h (Range: 24–340 h). There were 57 (61.3%) patients with prolonged PCICU, while 36 (38.7%) had a short duration of PCICU stay. In multivariate binary logistic regression analysis, significantly low SpO2 at the time of presentation, intraoperative tricuspid regurgitation, high postoperative inotropic score, postoperative pulmonary embolism, and postoperative bleeding from a wound were found to be significant predictors of prolonged PCICU stay after primary repair of ToF. ConclusionsThe findings of this study are paramount given the identification of factors associated with the prolonged stay, which will help in the future prediction of the prognosis of treated patients.

Comparison of median sternotomy and left anterior mini-incision for pulmonary valve replacement following primary tetralogy of Fallot repair.

Pulmonary insufficiency requiring reintervention frequently occurs after primary tetralogy of Fallot repair. Repeat interventions present a challenge for both the surgeon and patient. We compare a minimally invasive, 5 cm left anterior mini-incision to redo median sternotomy for pulmonary valve replacement in tetralogy of Fallot patients. Following Internal Review Board approval, we conducted a single institution retrospective review of patients with tetralogy of Fallot who underwent pulmonary valve replacement via redo median sternotomy or left anterior mini-incision between 13 July, 2016 and 6 March, 2020. Twenty-three patients underwent pulmonary valve replacement following primary tetralogy of Fallot repair between March 2016 and March 2020. Twelve patients received a redo-median sternotomy from March 2016 to August 2018. Left anterior mini-incision was first offered in August of 2018 and was chosen by all eleven patients thereafter. The two groups had similar baseline characteristics including preoperative pulmonary valve dysfunction. Early trends suggest a longer cardiopulmonary bypass time for patients who received left anterior mini-incisions. Other outcomes were comparable, including operative times, blood product requirements, residual pulmonary valve dysfunction, postoperative pain, narcotic requirements, ICU length of stay, total length of stay, and postoperative complications. In patients who have previously undergone primary repairs of tetralogy of Fallot, outcomes for pulmonary valve replacement via left anterior mini-incision are comparable to those via redo median sternotomy.

Long-term follow-up after transatrial-transpulmonary repair of tetralogy of Fallot: influence of timing on outcome.

OBJECTIVESOur goal was to report the long-term serial follow-up after transatrial–transpulmonary repair of tetralogy of Fallot (TOF) and to describe the influence of the timing of the repair on outcome.METHODSWe included all patients with TOF who had undergone transatrial–transpulmonary repair between 1970 and 2012. Records were reviewed for patient demographics, operative details and events during the follow-up period (death, pulmonary valve replacement, cardiac reinterventions and hospitalization/intervention for arrhythmias). In patients with elective early primary repair of TOF after 1990, a subanalysis of the optimal timing of TOF repair was performed.RESULTSA total of 453 patients were included (63% male patients; 65% had transannular patch); 261 patients underwent primary elective repair after 1990. The median age at TOF repair was 0.7 years (25th–75th percentile 0.3–1.3) and decreased from 1.7 to 0.4 years from before 1990 to after 2000, respectively (P < 0.001). The median follow-up duration after TOF repair was 16.8 years (9.6–24.7). Events developed in 182 (40%) patients. In multivariable analysis, early repair of TOF (<6 months) [hazard ratio (HR) 3.06; P < 0.001] and complications after TOF repair (HR 2.18; P = 0.006) were found to be predictive for an event. In a subanalysis of the primary repair of TOF after 1990, the patients (n = 125) with elective early repair (<6 months) experienced significantly worse event-free survival compared to patients who had elective repair later (n = 136). In multivariable analysis, early repair (HR 3.00; P = 0.001) and postoperative complications (HR 2.12; P = 0.010) were associated with events in electively repaired patients with TOF.CONCLUSIONSTransatrial–transpulmonary repair of TOF before the age of 6 months may be associated with more events during the long-term follow-up period.

Valve-sparing repair with intraoperative balloon dilation in tetralogy of Fallot: Midterm results and therapeutic implications

ObjectivesThe significant morbidity of long-term pulmonary regurgitation (PR) has driven the development of pulmonary valve (PV) sparing repair strategies in patients with tetralogy of Fallot (ToF). We assessed mid-term PV function in patients who underwent primary ToF repair with valve-sparing intraoperative balloon dilation (IBD) technique. MethodsWe evaluated 162 consecutive patients with ToF and pulmonary stenosis (ToF-PS) who underwent valve-sparing repair with IBD under 1 year of age. ResultsMedian age at surgery was 98 days (interquartile range [IQR], 72-126) and median follow-up was 2.5 years (IQR, 0.6-4.9). Median preoperative PV annulus z score was −2.2 (IQR, −2.5 to −1.8). Twenty-five patients (15.4%) required reintervention for residual valvular stenosis. Multivariable analysis demonstrated preoperative annulus z score less than −2.45 (P = .036) and younger age at surgery (P = .001) were independent risk factors for early reintervention for stenosis. Freedom from at least moderate PR was 77%, 61%, and 43% at 1, 3, and 5 years postrepair. Right ventricular dimensions were not significantly different compared with a matched cohort of patients undergoing transannular patch repair at midterm follow-up. ConclusionsPatients with ToF-PS who undergo valve-sparing repair with IBD develop progressive PR. Compared with transannular patch repair, the extent of RV dilation at midterm follow-up is not significantly different. Patients younger than 3 months of age and those with an annulus z score less than −2.45 experience higher rates of early reintervention for PV stenosis. In these patient subgroups, alternative strategies should be considered. This study suggests valve-sparing repair with IBD does not preserve long-term PV function in patients with ToF-PS.

Right ventricular outflow tract reintervention after primary tetralogy of Fallot repair in neonates and young infants

ObjectiveTo assess the outcomes following primary tetralogy of Fallot (TOF) repair in neonates and young infants with pulmonary stenosis (PS) and pulmonary atresia and compare differences in reintervention on the right ventricular outflow tract (RVOT) among those undergoing valve sparing repair (VSR), transannular RVOT patch (TAP), and right ventricle–to–pulmonary artery (RV-PA) conduit surgeries. MethodsData were collected retrospectively in 101 patients who underwent TOF repair over a 10-year period between January 2005 and September 2015. The primary endpoint was reintervention on the RVOT, defined as a surgical procedure or cardiac catheterization–based RVOT reintervention. ResultsForty-three patients had TOF/PS, of whom 24 (56%) underwent TAP and 19 (44%) underwent VSR. Fifty-eight patients had TOF/PA, 14 (24%) underwent TAP and 44 (76%) underwent RV-PA conduit repair. Overall patient mortality was 2.9% (3 of 101). Thirty-three patients underwent surgical reintervention, and 52 underwent catheterization-based reintervention. Patients with TOF/PA who underwent RV-PA conduit repair had a higher surgical reintervention rate than those who underwent TAP (45% vs 21%). Patients with TOF/PSs undergoing VSR with a lower median birth weight (2.5 kg vs 3.7 kg) required more surgical reintervention. ConclusionsNeonatal TOF repair can be performed with low mortality but frequent RVOT reinterventions. Surgical reintervention is earlier and the rate is higher among patients with TOF/PA undergoing RV-PA conduit repair compared with those undergoing TAP. Although there were no overall differences in RVOT reintervention rate between patients with TOF/PS undergoing VSR and those undergoing TAP, a lower birth weight in the patients undergoing VSR is associated with a higher surgical reintervention rate.

Preliminary Experience With the Use of an Extracellular Matrix to Augment the Native Pulmonary Valve During Repair of Tetralogy of Fallot.

During repair of tetralogy of Fallot (TOF), when a transannular patch is needed in case of a small annulus and dysplastic pulmonary valve, we chose to reconstruct the right ventricular outflow tract by augmenting the divided anterior leaflet with an extracellular matrix (ECM) patch to produce a competent valve. In this study, we present our preliminary experience and early outcomes. From March 2013 to December 2015, of the 206 patients who underwent primary repair of TOF, 52 required a transannular incision. The median age was 18 months and the median weight was 8.2 kg. The native hinge mechanism of the valve was preserved by dividing only the anterior leaflet at the time of the transannular incision and augmenting it with an ECM patch that was sutured to the endocardium and to the divided leaflet. Two patients died due to reasons not related to the use of the patch. Intraoperative evaluation showed a competent pulmonary valve without significant outflow gradient. During early follow-up (median 20 months), all patients were doing well without any decongestive therapy. Valve regurgitation was assessed as severe in 2, moderate in 26, and mild in 22 patients. In the majority of patients, the valve appeared thin and pliable in spite of mild to moderate regurgitation. Early experience with the use of ECM in repair of TOF shows satisfactory outcomes. It does not show obvious growth of the material. Long-term follow-up will be required in order to assess whether the valve function is durable without acquisition of significant regurgitation.

Outcomes of Pulmonary Valve Replacement for Correction Pulmonary Insufficiency after Primary Repair of Tetralogy of Fallot (TOF)

Background Total correction of Tetralogy of Fallot (TOF) anomaly in early childhood has been practiced in many centers with good results, but in some of patients after few years sever Pulmonary valve insufficiency occurred. Materials and Methods At a cross- sectional study from January 2015 to January 2016, 10 patients who had history of primary repair of TOF with free pulmonary insufficiency (PI) that underwent of pulmonary valve replacement (PVR) with bioprosthetic valves were evaluated. Results Themean age of patients was 6.5 + 0.753 years old (ranged 8-12 years old) and male to female ratio was 6/4. The mean of Intensive care unit (ICU) stay and Hospital stay was 4.5+ 0.712 days (ranged 3-8) and 11.5+ 0.357 days (ranged 9- 16). Mean of cardiopulmonary bypass time and operation time was 45 + 0.684 min (ranged 32-60) and 83 + 0.317 min (ranged 65-112). In this study we did not find any mortality and ventricular arrhythmia and Heart block. There was only one case (10%) with superficial wound infection that was controlled. At 6 months follow up, all of patients were alive, but Echocardiography sign of Right Ventricular (RV) failure was present in 2 patients (20%) recently. Conclusion Although for Pulmonary insufficiency after primary TOF repair there is controversial in studies, but we had good results of PVR with Bioprosthesis in TOF patients.

Predictors of Intensive Care Unit Morbidity and Midterm Follow-up after Primary Repair of Tetralogy of Fallot.

BackgroundOur objectives were to review our institutional early and midterm experience with primary tetralogy of Fallot (TOF) repair, and identify predictors of intensive care unit (ICU) morbidity.MethodsWe analyzed perioperative and midterm follow-up data for all cases of primary TOF repair from 2001 to 2012. The primary endpoint was early mortality and morbidity, and the secondary endpoint was survival and functional status at follow-up.ResultsNinety-seven patients underwent primary repair. The median age was 4.9 months (range, 1 to 9 months), and the median weight was 5.3 kg (range, 3.1 to 9.8 kg). There was no early surgical mortality. The incidence of junctional ectopic tachycardia and persistent complete heart block was 2% and 1%, respectively. The median length of ICU stay was 6 days (range, 2 to 21 days), and the median duration of mechanical ventilation was 19 hours (range, 0 to 136 hours). By multiple regression analysis, age and weight were independent predictors of the length of ICU stay, while the surgical era was an independent predictor of the duration of mechanical ventilation. At the 8-year follow-up, freedom from death and re-intervention was 97% and 90%, respectively.ConclusionPrimary TOF repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery remain significant predictors of morbidity.

Risk factors for morbidity in infants undergoing tetralogy of fallot repair

Background:Primary repair of tetralogy of Fallot (TOF) has low surgical mortality, but some patients still experience significant postoperative morbidity.Aim:To review our institutional experience with primary TOF repair, and identify predictors of intensive care unit (ICU) morbidity.Settings and Design:Medium-sized pediatric cardiology program. Retrospective study.Subjects and Methods:We retrospectively reviewed all the patients with TOF and pulmonic stenosis who underwent primary repair in infancy at our institution from January 2001 to December 2012. Preoperative, operative, and postoperative demographic and morphologic data were analyzed. ICU morbidity was defined as prolonged ICU stay (≥7 days), and/or prolonged duration of mechanical ventilation (≥48 h).Statistical Analysis Used:Multiple logistic regression analysis.Results:Ninety-seven patients underwent primary surgical repair during the study period. The median age was 4.9 months (1-9 months) and the median weight was 5.3 kg (3.1-9.8 kg). There was no early surgical mortality. Incidence of junctional ectopic tachycardia (JET) and persistent complete heart block was 2 and 1%, respectively. The median length of ICU stay was 6 days (2-21 days) and median duration of mechanical ventilation was 19 h (0-136 h). By multiple regression analysis, age and weight were independent predictors of length of ICU stay, while surgical era was an independent predictor of duration of mechanical ventilation.Conclusion:Primary TOF repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery remain significant predictors of morbidity.

Primary repair of symptomatic neonates with tetralogy of Fallot with or without pulmonary atresia

Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.

Primary tetralogy of Fallot repair: predictors of intensive care unit morbidity.

Primary repair of tetralogy of Fallot has low surgical mortality, but some patients still experience significant postoperative morbidity. Our objectives were to review our institutional experience with primary tetralogy of Fallot repair, and identify predictors of intensive care unit morbidity. We reviewed all patients with tetralogy of Fallot who underwent primary repair in infancy from 2001 to 2012. Preoperative, operative, and postoperative demographic and morphologic data were analyzed. Intensive care unit morbidity was defined as prolonged intensive care unit stay (≥ 7 days) and/or prolonged duration of mechanical ventilation (≥ 48 h). 97 patients who underwent primary surgical repair during the study period were included in the study. The median age was 4.9 months (range 1-9 months) and the median weight was 5.3 kg (range 3.1-9.8 kg). There was no early surgical mortality. The incidence of junctional ectopic tachycardia and persistent complete heart block was 2% and 1%, respectively. The median intensive care unit stay was 6 days (range 2-21 days) and the median duration of mechanical ventilation was 19 h (range 0-136 h). Age and weight were independent predictors of intensive care unit stay, while surgical era predicted the duration of mechanical ventilation. Primary tetralogy of Fallot repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery were significant predictors of morbidity.

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

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. 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. 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. 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.

Outcome and Incidence of Re-Intervention After Surgical Repair of Tetralogy of Fallot

Timing of primary repair of tetralogy of Fallot (TOF) remains controversial. We evaluated the long-term outcome of early primary treatment strategy in a patient cohort with TOF less than eight months of age. A group of 120 patients with TOF less than eight months of age (5 ± 2.4 months) underwent early primary repair of TOF between October 1998 and August 2009. Sixty-one patients received a transanular (TAN) repair, and 59 patients received a right ventricular outflow tract (RVOT) + main pulmonary artery (MPA) double patch repair with concomitant pulmonary valve reconstruction. There was no early or late mortality. The follow-up was 100% completed. There were eight reoperations and eight patients underwent catheter intervention for severe pulmonary valve insufficiency or stenosis, obstruction of right ventricular outflow tract, and stenosis of pulmonary arteries. Actuarial survival was 100% at ten years. At latest follow-up 80 patients were in NYHA Class I without any antiarrhythmic medications. On latest echocardiography, 90 (75%) patients had mild to moderate pulmonary regurgitation, and 10 had a right ventricular outflow tract gradient more than 40 mmHg. These data strongly support the concept of early primary repair of TOF in patients with well developed pulmonary arteries. Early primary repair is associated with an excellent early and late outcomes, an acceptable risk of reoperation and re-intervention, and a low incidence of significant right ventricular dysfunction.

Valve-Sparing Tetralogy of Fallot Repair With Intraoperative Dilation of the Pulmonary Valve

Deleterious long-term effects of pulmonary regurgitation after tetralogy of Fallot (TOF) repair have become evident during the last two decades. Subsequently, different groups have developed strategies aimed at preserving the pulmonary valve function. However, the results of these approaches are not well known. From July 2009 through March 2012, 38 patients underwent primary repair of TOF at the authors' institution. Of these, 12 children (7 boys) underwent attempted pulmonary valve-sparing surgery with intraoperative dilation of the pulmonary valve. The technical details as well as the echocardiographic preoperative and follow-up data for this repair were recorded, with a special focus on the feasibility of the technique and the effects on pulmonary valve function. No patient in the series died. At repair, the median age was 6 months (range 3.4-126 months), and the median weight was 7.6 kg (range 4.7-47 kg). Intraoperative dilation of the pulmonary valve was technically feasible for all the patients. Two patients had unsuccessful dilation and underwent a transannular patch procedure. During a median follow-up period of 22 months (range 6-30 months), the pulmonary valve diameter and z-score improved significantly. Moreover, the annular size normalized, whereas the mean right ventricular outflow tract (RVOT) gradient remained at the mild level (median, 24 mmHg; range 12-36 mmHg). At the most recent follow-up evaluation, three patients showed moderate pulmonary regurgitation. Intraoperative dilation of the pulmonary valve in patients undergoing TOF repair is feasible and provides good relief of obstruction. Moreover, the pulmonary valve annulus grows through the follow-up period. Longer follow-up studies are needed to evaluate the exact role of this strategy in this population.

Tetralogy of Fallot with Aortic Stenosis: Repair Without Aortic Valve Intervention

A 14-day-old boy with tetralogy of Fallot (ToF), ductus-dependent pulmonary circulation, and significant aortic valve stenosis (AVS) underwent primary repair of ToF without aortic valve intervention. The pressure gradient through the aortic valve decreased spontaneously after the operation. This may have been due to the reduction of blood flow through the aortic valve postoperatively. For patients with ToF, AVS, and ductus-dependent circulation, ToF repair alone may alleviate the severity of the AVS.

Invited Commentary

Invited Commentary

Is early primary repair for correction of tetralogy of Fallot comparable to surgery after 6 months of age?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether early primary repair for correction of tetralogy of Fallot (TOF) resulted in better outcomes than surgery after 6 months of age. Altogether 650 relevant papers were identified using the below mentioned search, eight papers represented the best evidence to answer the specific question. The author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses were tabulated. We conclude that early primary repair of TOF has been shown to be comparable to later repair, with several retrospective series concluding that there is no increase in mortality with children under 6 months of age. Freedom from reintervention has also been shown to be similar irrespective of the age primary repair is undertaken. However, it has been observed that length of intensive care unit stay, period of mechanical ventilation and the need for inotropes is increased in patients undergoing primary repair at <3 months of age.