Symptomatic Tetralogy Of Fallot Research Articles

Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?

ObjectivesThe optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy. MethodsWe performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years). ResultsAfter the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group. ConclusionsIn symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up.

Abstract 14744: Driving Distance to a Primary Cardiac Center is Associated With Mortality in Patients With Tetralogy of Fallot Undergoing Neonatal Interventions

Introduction: Advances in tetralogy of Fallot care have improved survival, however, associations of sociodemographic disparities and mortality are less well described, particularly among patients with symptomatic tetralogy of Fallot (sTOF) requiring neonatal intervention. Our multicenter Congenital Cardiac Research Collaborative (CCRC) study investigated sociodemographic factors associated with mortality in sTOF patients. Hypothesis: Sociodemographic factors related to a lower socioeconomic status are associated with increased mortality in patients with sTOF. Methods: Retrospective cohort study of neonates with sTOF receiving surgical or catheter-based palliation or primary repair at <30 days old from 1/2005 - 11/2017 at one of nine U.S. centers in the CCRC. Primary outcome variable was mortality at last follow up. Primary predictor variable was childhood opportunity index (COI), a national composite score based on 29 neighborhood-level indicators of poor vs excellent health. Secondary predictor variables included distance to primary cardiac center, race/ethnicity, and maternal insurance type. Sociodemographic factors were evaluated using multivariable Cox proportional hazard models, adjusting for center and patient anatomic and genetic characteristics resulting in hazard ratios (HR) with 95% CI. Results: There were 417 neonates with sTOF, with an overall follow-up mortality of 10% at median follow-up of 4.8 years (25th - 75th %ile: 2.1 - 8.2). Most patients were male (53%), non-Hispanic White (52%), prenatally diagnosed (58%), and had public insurance (54%). On adjusted multivariable analysis, infants >100 miles from a primary heart center had a 3x higher hazard of mortality (HR 2.94; 95% CI, 1.13-7.61; p=0.026) compared to those living <50 miles from a heart center. All other sociodemographic factors (COI, race/ethnicity, and insurance type) were not significantly associated with mortality on multivariable analysis. Conclusions: Proximity to a specialized pediatric cardiac center remains a significant determinant of survival in neonates with sTOF, emphasizing the importance of access to cardiac care. Sociodemographic impact on patient morbidity and lifelong mortality in TOF requires further investigation.

Abstract 13226: Young Infants With Symptomatic Tetralogy of Fallot: Superior Survival With Staged Repair

Background: The optimal strategy to treat symptomatic young infants with tetralogy of Fallot (TOF) is unclear. Objective: We performed a balanced comparison of staged repair (SR) vs. primary repair (PR) at two institutions that have exclusively adopted each strategy. Methods: Between 1993 and 2021, 143 consecutive infants under 4 months of age underwent systemic-to-pulmonary shunt at the Royal Children’s Hospital, Melbourne, Australia. These were compared with 122 consecutive infants in the same age group who underwent PR between 2004 and 2018 at the Children’s National Hospital, Washington, D.C. The primary outcome was mortality. Secondary outcomes were component and cumulative hospital stay, durations of perioperative support, hospital complications, and early and late reinterventions. Propensity-score matching was performed for risk adjustment. Mean follow-up was 9.3 years. Results: In the unmatched comparison, the SR cohort was younger, more commonly on prostaglandin infusion and mechanical ventilation, more likely to require urgent procedures, and had lower weight and smaller pulmonary annulus size. Hospital mortality after initial procedure (SR: 2.8% [4/143] vs. PR: 2.5% [3/122]; p = 0.86) and 10-year survival (SR: 95% [95% confidence interval (CI): 90 - 98%] vs. PR: 90% [95% CI: 81 - 95%]; p = 0.65) were similar. Hazard of reinterventions favored the PR group during early (<6 months) follow-up (hazard ratio: 0.22; 95% CI: 0.11 - 0.48; p < 0.001) but was similar at late follow-up. Most secondary outcomes cumulatively favored the PR group. Among 57 propensity-score matched pairs, the SR group had superior survival at 10 years (98% [95% CI: 88 - 99.8%] vs. 85% [95% CI: 69 - 93%]; p = 0.02). Conclusions: In this two-institutional, propensity-score matched comparison of SR vs. PR in symptomatic young infants with TOF, the SR strategy was associated with superior long-term survival and equivalent late reinterventions, at a cost of increased early reinterventions.

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot

BackgroundNeonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. ObjectivesThe objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. MethodsNeonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. ResultsWe included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). ConclusionsDespite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.

D-15 | Radiation Exposure from Cardiac Catheterization in Neonates With Symptomatic Tetralogy of Fallot

D-15 | Radiation Exposure from Cardiac Catheterization in Neonates With Symptomatic Tetralogy of Fallot

Abstract 11531: Neurodevelopmental Outcomes After Neonatal Tetralogy of Fallot Repair or Palliation: Scope of The Problem and Does Treatment Strategy Matter

Introduction: Neonates with symptomatic tetralogy of Fallot (sTOF) require early intervention with either staged repair (SR; initial palliation with subsequent complete repair) or primary repair (PR). Implications of these neonatal management strategies on late outcomes are not well understood. Thus, we aimed to assess neurodevelopmental (ND) outcomes and identify risk factors for ND concerns. Methods: From 2005-2017, sTOF patients who underwent neonatal intervention across 9 centers were identified. To assess ND, all living patients were sent age-appropriate parent-rating scales of executive function (Behavior Rating Inventory of Executive Function - BRIEF) and psychosocial symptoms (The Behavior Assessment System for Children, 3rd Edition - BASC-3). The results were compared by primary treatment strategy (SR vs. PR) using multivariable models. Patient and clinical factors were compared between those with normal and clinically significant abnormal results (T-score ≥60). Results: Of 572 patients in the initial cohort, surveys were sent to 511 survivors and returned from 144 (28%, 53% male, age 3.2-15.9 years). Up to 37% of respondents had a clinically concerning score on ≥ 1 scales, including 36% with ≥1 abnormal scale present on the BRIEF and 30% with an abnormal scale on the BASC. Across SR and PR strategies, there was no difference in ND outcomes (BRIEF adjusted odds ratio (AOR) 1.55 (0.57, 4.21, p=0.39); BASC AOR 1.13 (0.43, 2.99, p=0.81)). Factors associated with clinically significant executive dysfunction included genetic syndrome (p=0.001), need for invasive ventilatory support (p=0.003) and inotropic support (p=0.01) prior to the index procedure. Only genetic syndrome (p=0.026) was associated with clinically significant psychosocial problems. Conclusions: Late ND abnormalities, specifically executive dysfunction and psychosocial concerns, are common in sTOF patients. While initial treatment strategy does not appear to have impact, intrinsic patient characteristics and pre-procedure measures of clinical severity may predict late ND outcomes and help target increased surveillance for ND complications. Forthcoming analyses will explore existing ND service utilization and parental concerns in the context of these findings.

Anomalous coronary artery in Tetralogy of Fallot-Feasibility of right ventricular outflow tract stenting as initial palliation.

This study addresses the outcome of right ventricle outflow tract (RVOT) stenting in Tetralogy of Fallot (ToF) with anomalous coronaries crossing the RVOT. RVOT stenting in ToF patients has emerged as an alternative to Blalock Taussig shunting. This is a single center study of patients who underwent RVOT stenting for symptomatic ToF at Birmingham Children's Hospital between 2005 and 2020. A total of 122 patients underwent RVOT stenting as initial palliation over a 15-year period, 10 patients had anomalous coronaries crossing the RVOT (study group) and 112 not (comparative group). Median age of the study group was 72.5 days (interquartile range [IQR]: 28-103) with a weight of 4.7 kg (IQR: 3.5-4.9). No significant differences were found between the two groups regarding the patients' weights and ages, procedure and screening times, or hospital stay. Four had valve sparing stenting. Oxygen saturations increased from a median of 75.5% (IQR: 70-82) to 94.5% (IQR: 90-95), p < 0.002. Postprocedure median hospital stay was 3 days (IQR: 2-6). Six patients underwent interstage catheterization reintervention and one needed early surgical palliation due to stent suboptimal position. Complete repair could be delayed for a median of 11.1 months (IQR: 5.6-19.2). At surgical repair, the patients had a median age of 12.3 months (IQR: 7.5-25.6) and weight of 7.7 kg (IQR: 6.8-10.8). There were no deaths. RVOT stenting in ToF with anomalous coronaries is safe and effective. Dilatable stents should be used when two-stage delayed conduit repair is the default approach.

Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot.

Neonates with symptomatic tetralogy of Fallot (sTOF) may undergo palliations with varying physiology, namely systemic to pulmonary artery connections (SPC) or right ventricular outflow tract interventions (RVOTI). A comparison of palliative strategies based on the physiology created is lacking. Consecutive sTOF neonates undergoing SPC or RVOTI from 2005-2017 were reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was survival with successful complete repair (CR) by 18months. A variety of secondary outcomes were assessed including overall survival, hospitalization-related comorbidities, and interstage reinterventions. Propensity score adjustment was utilized to compare treatment strategies. The cohort included 252 SPC (surgical shunt = 226, ductus arteriosus stent = 26) and 68 RVOTI (balloon pulmonary valvuloplasty = 48, RVOT stent = 11, RVOT patch = 9) patients. Genetic syndrome (29 [42.6%] v 75 [29.8%], p = 0.04), weight < 2.5kg (28 [41.2%] v 68 [27.0%], p = 0.023), bilateral pulmonary artery Z-score < -2 (19 [28.0%] v 36 [14.3%], p = 0.008), and pre-intervention antegrade flow (48 [70.6%] v 104 [41.3%], p < 0.001) were more common in RVOTI. Significant center differences were noted (p < 0.001). Adjusted survival to CR by 18months (HR = 0.87, 95% CI = 0.63-1.21, p = 0.41) and overall survival (HR = 2.08, 95% CI = 0.93-4.65, p = 0.074) were similar. RVOTI had increased interstage reintervention (HR = 2.15, 95% CI = 1.36-3.99, p = 0.001). Total anesthesia (243 [213, 277] v 328 [308, 351] minutes, p < 0.001) and cardiopulmonary bypass times (117 [103, 132] v 151 [143, 160] minutes, p < 0.001) favored RVOTI. In this multicenter comparison of physiologic palliation strategies for sTOF, survival to successful CR and overall survival were similar; however, reintervention burden was significantly higher in RVOTI.

Comparative Costs of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot

Recent data have demonstrated that overall mortality and adverse events are not significantly different for primary repair (PR) and staged repair (SR) approaches to management of neonates with symptomatic tetralogy of Fallot (sTOF). Cost data can be used to compare the relative value (cost for similar outcomes) of these approaches and are a potentially more sensitive measure of morbidity. This study sought to compare the economic costs associated with PR and SR in neonates with sTOF. Data from a multicenter retrospective cohort study of neonates with sTOF were merged with administrative data to compare total costs and cost per day alive over the first 18months of life in a propensity score-adjusted analysis. A secondary analysis evaluated differences in department-level costs. In total, 324 subjects from 6 centers from January 2011 to November 2017 were studied (40% PR). The 18-month cumulative mortality (P = 0.18), procedural complications (P = 0.10), hospital complications (P = 0.94), and reinterventions (P = 0.22) did not differ between PR and SR. Total 18-month costs for PR (median $179,494 [IQR: $121,760-$310,721]) were less than for SR (median: $222,799 [IQR: $167,581-$327,113]) (P< 0.001). Cost per day alive (P = 0.005) and department-level costs were also all lower for PR. In propensity score-adjusted analyses, PR was associated with lower total cost (cost ratio: 0.73; P< 0.001) and lower department-level costs. In this multicenter study of neonates with sTOF, PR was associated with lower costs. Given similar overall mortality between treatment strategies, this finding suggests that PR provides superior value.

Transcatheter Approaches to Palliation for Tetralogy of Fallot.

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.

Abstract 12574: Syndromic Neonates With Symptomatic Tetralogy of Fallot Have Lower Early Mortality With Staged Repair Compared to Primary Repair

Introduction: Neonates with symptomatic tetralogy of Fallot (sTOF) require early intervention with staged repair (SR) or primary repair (PR). As sTOF is commonly associated with genetic syndromes, we examined outcomes of SR vs. PR for syndromic sTOF neonates. Hypothesis: We hypothesized that syndromic neonates with sTOF who underwent SR will have lower early mortality compared to PR. Methods: Syndromic neonates with sTOF who underwent initial intervention (initial palliation followed by later SR, or PR) from 2005-2017 were reviewed. The primary outcome was death. Secondary outcomes included complications, and hospital and intensive care unit (ICU) lengths of stay (LOS). Unadjusted and propensity score adjusted outcomes were compared. Results: The 176 syndromic sTOF neonates consisted mostly of DiGeorge (35%), trisomy 21 (16%), and VACTERL (10%). There were 110 and 66 neonates in the SR and PR groups, respectively. DiGeorge syndrome, pulmonary atresia, prematurity, and pre-procedural ventilation were more common in the SR group. The adjusted overall hazard of death was similar between groups, but early mortality favored SR (Figure). Procedural complications were lower in the SR group at the index procedure, but SR is associated with longer cumulative hospital and ICU LOS (Table). Conclusions: In syndromic neonates with sTOF, there was an early survival benefit to SR compared to PR, although there was no overall survival difference. The cumulative cost of the SR strategy was longer hospital and ICU LOS. These findings do not suggest a single superior management strategy in the syndromic sTOF population.

Abstract 11587: Absent Ductus Arteriosus is Relatively Common in Neonates with Symptomatic Tetralogy of Fallot, and Rarely Associated with Critically Deficient Pulmonary Blood Flow at Birth

Introduction: Absent ductus arteriosus (ADA) is a poorly described feature associated with tetralogy of Fallot (TOF). We compare characteristics and outcomes of neonates with symptomatic TOF (sTOF) with ADA to those with a ductus arteriosus (DA). Hypothesis: We hypothesized: neonates with sTOF and ADA would have similar outcomes to those born with a DA, and a small portion of the ADA cohort would have critically-deficient pulmonary blood flow (CDPBF). Methods: Consecutive neonates with sTOF who underwent intervention during the neonatal period from 2005—2017 were identified from the Congenital Cardiac Research Collaborative. Those with DA on echocardiogram performed on the first day of life or on fetal echocardiogram were identified and compared to those with ADA. ADA neonates who required ECMO or intervention to augment PBF on the first day of life were defined as having CDPBF. Patient characteristics and outcomes were compared between DA and ADA groups. Results: Among 519 neonates, 460 (89%) had a DA and 59 (11%) had ADA. ADA neonates were more likely to carry a genetic or syndromic diagnosis, had smaller branch PA sizes, and were more likely to receive a transannular patch compared to those with a DA. Despite this, reintervention rates were similar (data not shown), and there was only a small difference in mortality that did not reach statistical significance. Of ADA neonates, 7/59 (12%) had CDPBF. Compared to ADA neonates without CDPBF, those with CDPBF had lower baseline oxygen saturation (58 vs 87%, p=0.031) and smaller pulmonary valve (PV) annular z-score (-2.9 vs -2.5, p=0.015) with similar reintervention rates and mortality. Conclusion: ADA is relatively common in sTOF. Despite higher rates of comorbidities, mid-term reintervention rates and mortality are similar to those with DA. CDPBF is a rare, high-risk feature of infants with sTOF and ADA. Smaller PV size may be a marker of CDPBF. Future work should focus on prenatal factors to identify fetuses with TOF, ADA and CDPBF.

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot

BackgroundNeonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention. ObjectivesThis study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies. MethodsConsecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment. ResultsThe cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002). ConclusionsIn this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.

Comparison of management strategies for neonates with symptomatic tetralogy of Fallot and weight

ObjectiveTo compare management strategies for neonates <2.5 kg with tetralogy of Fallot and symptomatic cyanosis who either undergo staged repair (SR) (initial palliation followed by later complete repair) or primary repair (PR). MethodsConsecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing <2.5 kg at initial intervention and between 2005 and 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Primary outcome was mortality and secondary outcomes included component (eg, initial palliation, complete repair, or primary repair) and cumulative (SR: initial palliation followed by later complete repair) hospital and intensive care unit lengths of stay, durations of ventilation, inotrope use, cardiopulmonary bypass time, procedural complications, and reintervention. Outcomes were compared with propensity score adjustments with PR as the reference group. ResultsThe cohort included 76 SR (initial palliation: 53 surgical and 23 transcatheter) and 44 PR patients. The observed risk of overall mortality was similar between SR and PR groups (15.8% vs 18.2%: P = .735). The adjusted hazard of mortality remained similar between groups overall (hazard ratio, 0.59; 95% confidence interval, 0.26-1.36; P = .214), as well as during short-term (<4 months: hazard ratio, 0.37; 95% confidence interval, 0.13-1.09; P = .071) and midterm (>4 months: hazard ratio, 1.32; 95% confidence interval, 0.30-5.79; P = .717) follow-up. Reintervention in the first 18 months was common in both groups (53.2% vs 48.4%; hazard ratio, 1.69; 95% confidence interval, 0.96-2.28; P = .072). Adjusted procedural complications and neonatal morbidity burden were overall lower in the SR group. Cumulative secondary outcome burdens largely favored the PR group. ConclusionsIn this study comparing SR and PR treatment strategies for neonates with tetralogy of Fallot and symptomatic cyanosis and weight <2.5 kg, mortality and reintervention burden was high and independent of treatment strategy. Other potential advantages were observed with each approach.

Abstract 16503: Comparison of Management Strategies for the Neonate With Symptomatic Tetralogy of Fallot and Hypoplastic Branch Pulmonary Arteries: Results From the Congenital Catheterization Research Collaborative

Introduction: Symptomatic neonates with tetralogy of Fallot (sTOF) and hypoplastic branch pulmonary arteries (hPA) are at high risk. Management strategies include staged repair (SR) - initial palliation (IP) followed by later complete repair (CR) - or primary repair (PR). A balanced comparison of these approaches is needed in the sTOF neonate with hPA. Methods: Consecutive neonates with sTOF and hPA - defined as one hPA z-score &lt;-2.0 with contralateral z-score &lt;0 - who underwent SR or PR from 2005-17 were retrospectively reviewed by the Congenital Catheterization Research Collaborative. Primary outcome was mortality and secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital, ICU and surgical morbidities, and reintervention. Outcomes were compared between treatment strategies using propensity score adjustment (PSA) to account for baseline differences between groups (PR=reference group). Results: The cohort comprised 188 neonates including 121 SR (IP: 29 transcatheter; 54 surgery) and 67 PR patients. Presence of pulmonary atresia, lower gestational age and birthweight were more common in the SR cohort. Median right and left hPA z-scores were -2.19 (IQR -2.65, -1.92) and -2.33 (-2.84, -1.99), respectively. There were significant differences in treatment strategy across centers (p&lt;0.01). Overall survival was similar between groups, before (p=0.33) and after PSA (HR 1.91, 0.48-7.7, p=0.36). Reintervention was more common in the SR group overall (HR 1.7, 1.03-2.7, p=0.04), but there was no difference after definitive repair (HR 1.21, 0.71-2.0, p=0.49). Secondary outcomes are shown in Table 1. Conclusions: In this multicenter comparison of SR or PR for management of neonates with sTOF and hPA, after PSA, no difference in mortality was found. Neonatal morbidities largely favored the SR group, but cumulative morbidities and reintervention favored the PR group. Further study is needed to determine late impact of these early advantages to SR.

Symptomatic Tetralogy of Fallot in Young Infants: Primary Repair or Shunt-Pediatric Health Information System Database Analysis.

Compare the early outcomes and hospital charges of early primary repair and systemic-to-pulmonary artery shunt for neonates and young infants (≤90 days of age) with tetralogy of Fallot using data from the Pediatric Health Information System database. The Pediatric Health Information System database was queried for patients <90 days of age with primary diagnosis of tetralogy of Fallot who underwent nonelective surgical repair or palliation between January 2008 and December 2014. The initial cohort of 821 patients (group 1 early primary repair, N = 554; group 2 systemic-to-pulmonary artery shunt, N = 267) was propensity score matched (248 patients in each group) to account for baseline imbalances in age and prostaglandin use. Comparison of unmatched groups revealed younger age and higher incidence of extracardiac anomalies ( P = .02) and prematurity ( P = .04) in group 2. Mortality was comparable between the groups (group 1: 20 [4%] of 554 vs group 2: 11 [4%] of 267, P = .74). Irrespective of the type of procedure, prematurity (odds ratio [OR] = 3.3, 95% confidence interval [CI]: 1.5-7.4) and extracardiac anomalies (OR = 2.5, 95% CI: 1.2-5.3) were independent risk factors for mortality. Propensity score-matched analysis revealed no significant differences in patient mortality ( P = 1), duration of ventilation ( P = .64), hospital length of stay ( P = .69), or hospital charges ( P = .08) between the two groups. Outcomes and hospital charges associated with nonelective early primary repair are comparable to systemic-to-pulmonary artery shunt in symptomatic patients <90 days old with tetralogy of Fallot.

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.

Tetralogy of Fallot with Cor Triatriatum Dexter in an Adult Patient: A Case Report

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect with only approximately 3% of uncorrected patients surviving past age 40. In this case report, we present a 48-year-old mentally retarded man suffering from congenital spastic quadriplegia who was diagnosed with a unique combination of symptomatic TOF and cor triatriatum dexter (CTD). Reduced preload because of CTD with spastic quadriplegia that prevented physical exertion is believed to have facilitated this patient's unusually long survival.

Determinants of short-term mortality and morbidity after the complete repair of tetralogy of Fallot in infant groups under 12 months and one-four years of age

In patients with tetralogy of Fallot, infants less than 12 months old and children between one and four years old were compared after total repair surgery for determination of outcome of surgery, risk factors influencing mortality and morbidity. Fifty- two patients with tetralogy of Fallot between five months and four years of ages were included into the study. The patients were divided into two groups depending on their ages; Group 1; one year old and younger (n=21) whereas, group 2; one and four years old (n=31). Statistical analysis was performed using Mann-Whitney U, Chi-square or where appropriate Fisher's exact tests and logistic regression analysis was applied for determination of predictors of mortality. The parameters that were different between two groups include; age (Group 1; 10.00 ± 1.67 months and Group 2; 2.39 ± 0.77 years, p<0.001), weight (Group 1; 9.74 ± 2.23 kg, Group 2; 11.97 ± 1.78 kg, p<0.001), McGoon ratio (Group 1; 1.94 ± 0.29, Group 2; 2.19 ± 0.27, p=0.001). Mortality is found in 3 patients in group 1 (14.2%) whereas, in five patients in group 2 (16%) and the difference was not statistically significant. In group 2 in only one patient (0.03%) had complete atrioventricular block and required permanent pacemaker implantation. When patients were compared according to groups with and without mortality, the significant differences were found in following variables: peritoneal dialysis (p=0.001), pleural effusion (p=0.02), right ventricular pressure (p=0.001) and right ventricle/aorta pressures ratio (p=0.001). However, none of these risk factors had significant value in prediction of mortality. Depending on these results, in patients under one year of age with symptomatic tetralogy of Fallot, if there are no other pathologies that have potential to increase risk of mortality, the complete repair surgery can be performed with same amount of risks and similar morbidity and mortality ratios.

Symptomatic Neonatal Tetralogy of Fallot: Repair or Shunt?

The management of neonates with symptomatic tetralogy of Fallot (TOF) requiring surgical intervention in the first month of life remains controversial. We reviewed our experience with neonates 30 days or greater with TOF from 2002 to 2008 requiring surgical intervention. Thirty-seven consecutive symptomatic nonrandomized neonates with TOF or its variants (including TOF with complete atrioventricular septal defect or absent pulmonary valve but excluding pulmonary atresia) had either a shunt (n = 17) or primary repair (n = 20). The shunted patients more commonly required emergency operation (24% vs 0%; p = 0.036); otherwise, the two groups were similar. One infant with preoperative bowel ischemia died one day postoperatively after emergency shunting. There were two late deaths 11 and 12 months postoperatively, both in primary repair patients. The 16 surviving shunted patients had TOF repair 216 +/- 99 days after the original shunt. The shunted patients had shorter intensive care unit and hospital stays for the first operation, which became equivalent when the second hospitalization (repair) values were added. The primary repair patients more frequently had a transannular patch and a tendency to more frequent delayed sternal closure. Four primary repair (20%) and two shunted (12.5%) patients required subsequent cardiac operations after complete repair (p = not significant). Shunting or primary repair of neonates with symptomatic TOF provides equivalent mortality and results. Shunted patients had fewer transannular patch repairs despite having more emergent initial operations. Compared with the primary repair group, shunted patients had decreased intensive care unit and hospital stays for the first hospitalization, which were neutralized when the second operation (repair) values were added.