Outcomes In Tetralogy Of Fallot Research Articles

Abstract 18520: Impaired Ubiquitin-Proteasome Function in Right Ventricular Myocardium Correlates to Postoperative Outcomes in Tetralogy of Fallot

Introduction: At the time of initial repair, patients with Tetralogy of Fallot (TOF) undergo a sudden shift from a pressure to volume loaded right ventricle (RV). It is difficult to predict how TOF RVs will respond to this hemodynamic transition. Identifying mechanisms and targets may enable interventions to improve outcomes. Hypothesis: RV myocardial gene expression profiles at the time of TOF repair correlate to pre- and early postoperative clinical outcomes. Methods: RNAseq was performed from RV myocardium resected at the time of initial TOF repair (n=44). Gene expression profiling was performed using a weighted gene correlation network analysis (WGCNA) against pre- and post-op clinical variables. Mouse pulmonary artery band (PAB, n=9) stratified by degree of RV dilation was used to correlate pathways. Results: WGCNA identified a gene module that was associated with younger age at repair and worse TOF postop outcomes (prolonged milrinone, decreased right atrial and right ventricular strain) without a concomitant association with preop oxygen saturation. Biological process gene-set enrichment analysis using the 2023 Gene Ontology library revealed enrichment of regulators for ubiquitination, cell cycle/mitosis, DNA repair, microtubule polymerization, and Rho/GTPase. PAB mouse RV demonstrated a RV-dilation severity-dependent increase in bound ubiquitin (R 2 =0.61, p=0.01) and loss of free ubiquitin (R 2 =0.72, p=0.004; Figure). Conclusions: WGCNA reveals a RV myocardial gene expression profile that is associated with preoperative characteristics and early postoperative outcomes, and which is enriched for ubiquitination regulators. The PAB mouse recapitulates a severity-dependent increase in bound ubiquitin and loss of free ubiquitin which is consistent with literature suggesting proteostatic dysfunction and accumulation of misfolded proteins in RVF. Targeting proteostatic disruption prior to TOF repair may improve postoperative outcomes.

Aberrant White Matter Organization Correlated With Neurodevelopment Outcomes in Tetralogy of Fallot: An Atlas-Based Diffusion Tensor Imaging Study

BackgroundWhite matter injury (WMI) and impaired neurodevelopment are common in children with congenital heart disease. However, the effect of WMI on neurodevelopmental outcomes is still rarely reported. In this study, we aimed to investigate microstructural changes in white matter (WM) and its relationship with neurodevelopmental outcomes and further explore the underlying neurophysiological mechanisms of neurocognitive impairments in the tetralogy of Fallot (ToF). MethodDiffusion tensor imaging (DTI) data were acquired in preschool-aged children with ToF (n = 29) and normal controls (NC, n = 19), and neurodevelopmental assessments were performed with the Wechsler Preschool and Primary Scale of Intelligence in ToF. The differences in DTI metrics including fractional anisotropy, mean diffusivity, axial diffusivity, and radial diffusivity were evaluated between ToF and NC. Correlations between WM microstructural changes and neurodevelopmental outcomes were further analyzed. ResultsSignificant WM differences were found in the uncinate fasciculus, cingulum hippocampus, superior longitudinal fasciculus, and corticospinal tract between children with ToF and NC. Impaired WM integrity was correlated with the verbal comprehension index and working memory index in ToF. ConclusionsThis study demonstrated WM microstructure injury, and this injury is related to worse language and working memory performance in preschool-aged children with ToF. These findings suggested that DTI metrics may be a potential biomarker of neurocognitive impairments in ToF and can be used to predict future neurodevelopmental outcomes, which also provide new insights into the underlying neurophysiological mechanisms of neurocognitive impairments in ToF.

Neurodevelopmental Outcomes in Tetralogy of Fallot: A Systematic Review.

BACKGROUND: Tetralogy of Fallot (TOF) represents between 7 and 10% of the total cases of congenital heart defects (CHD) and is estimated to be the most common cyanotic CHD, requiring medical or surgical intervention within the first year of life. Current advances in prenatal screening and fetal echocardiography led to increased rates of prenatal diagnosis of TOF. Furthermore, improvements in initial medical care, surgical repair, and long-term care are associated with excellent long-term survival until adulthood. Consequently, issues of morbidity have come under the spotlight, specifically neurodevelopmental and psychiatric adverse outcomes, which affect the quality of life of TOF survivors. METHOD: This study is a systematic review of English articles, using PUBMED and applying the following search terms, Tetralogy of Fallot, neurodevelopment, autism, cerebral palsy, attention-deficit hyperactivity disorder. Data were extracted by two authors. RESULTS: Most researchers suggest that TOF survivors score lower in neurodevelopmental tests than healthy populations of the same age and are in danger of neurodevelopmental impairments. Furthermore, it is suggested that TOF adolescents show higher rates of psychiatric disorders. CONCLUSIONS: The neurodevelopment of TOF survivors is not intensively studied. Existing studies in TOF survivors focus on different developmental aspects, using different evaluation methods and thus making conclusions for either one of the four aspects of neurodevelopment (executive function, cognition, and adaptive function, speech-language and motor function, or neuropsychiatric domain). The poor outcomes of these isolated studies indicate the need for future research as well as for continuous neuropsychological assessment and close monitoring of children and adolescents with TOF.

Abstract 10599: The Effects of Gestational Age and Size on Management Strategies and Outcomes in Neonatal Tetralogy of Fallot

Introduction: Data suggests there are benefits to both staged (SR) and complete repair (CR) for neonatal Tetralogy of Fallot (nTOF), though data is lacking on whether high-risk groups would benefit from one strategy over another. In a large state with variable practice patterns, we studied the impact of gestational age (GA) and weight by GA (birth weight z-score: BWz) at birth on surgical strategy and outcomes in nTOF, hypothesizing that GA and BWz would influence these outcomes. Methods: Population-based cohort study (2011-2017) of infants with nTOF (defined as catheter or surgical intervention prior to 44 weeks corrected GA) was performed. BWz was categorized as appropriate, small or large for GA. Surgical strategy and timing were compared by GA ( < 36 weeks preterm (n= 96), 37-38 weeks early term (n= 97), > 38 weeks full term (n= 152)) and BWz. Multivariable (MV) models were constructed to evaluate composite outcome of mortality or significant morbidity (BPD, IVH, NEC, PVL etc.) at 1 year and total number of hospital days in first year of life. Results: There were 345 patients included (SR= 194; CR= 151). Surgical strategy did not differ by GA or BWz. Within CR group, full term and early term neonates underwent surgery 20 (95%CI: -27.1, -14.1; p<0.001) and 15 days (95%CI: -22.1, -8.2; p<0.001) sooner than preterm neonates. BWz was not associated with surgical timing. A stratified analysis trended toward lower survival probability among preterm neonates undergoing CR vs. SR (p= 0.1). In a MV model including GA, BWz, surgical approach and DOL surgery, only GA was associated with the composite outcome. The odds of a poor outcome was 0.45 (95%CI:0.24,0.86) times lower in full term vs. preterm neonates, p= 0.02. The adjusted total burden of hospital days in the first year was 32 (95%CI: -47.7,-16.9; p<0.001) and 22 (95%CI: -36.5,-7.7; p= 0.003) days less in early term and full term compared to preterm neonates. Conclusions: In nTOF, GA and BWz were not associated with surgical strategy though premature neonates were more likely to wait longer for their initial intervention. One-year outcomes appear to be strongly influenced by GA and not surgical strategy; however, there was a trend to suggest that SR may result in better survival probability among premature neonates.

Density of aortopulmonary collaterals predicts in-hospital outcome in tetralogy of Fallot with pulmonary stenosis.

OBJECTIVESThe aim of this study was to characterize the anatomy of aortopulmonary collateral (APC) arteries in tetralogy of Fallot and pulmonary stenosis and to determine whether APC density identified on preoperative multidetector cardiac computed tomography predicts in-hospital outcome.METHODSThe retrospective single-centre study includes consecutive 135 (2015–2019) patients who underwent one-stage repair. Preoperative multidetector cardiac computed tomography, echocardiography and clinical outcomes were reviewed. The cut-off value of indexed total distal APC cross-sectional area (APC-CSA) was identified by receiver operating characteristic curve. Logistic regression was used for predictors analysis.RESULTSThe median age and body weight were 19.7 (10.1–89.7) months and 10 (8.3–18) kg. A total of 337 APCs were detected with only one demonstrating severe stenosis. There was a strong and significant correlation between mean APC diameter per patient and age (r = 0.70, P < 0.001). APCs were imaged but mainly received no interventions. In-hospital mortality was similar between patients with high (indexed APC-CSA ≥3.0 mm2/m2) and low (<3.0 mm2/m2) APC density (P = 0.642). Significantly greater patients with high indexed APC-CSA experienced the in-hospital composite outcome of death, arrest, renal/hepatic injury, lactic acidosis or extracorporeal membrane oxygenation (P = 0.007). High APC density was associated with greater dosing (P = 0.008) and longer (P = 0.01) use of inotropic support, prolonged pleural drainage (P = 0.013), longer ventilation (P = 0.042), intensive care unit (P = 0.014) and hospital (P = 0.027) duration. No reintervention and death occurred in the follow-up with the median duration of 24.4 (11–36.6) months. Multivariable analysis showed the Nakata index (P = 0.05) and high APC density (P = 0.02) independently predicted the composite outcome.CONCLUSIONSIn tetralogy of Fallot and pulmonary stenosis, APCs are likely to be dilated bronchial arteries. Preoperative multidetector cardiac computed tomography-derived APC density was as important as Nakata index in predicting the occurrence of in-hospital composite outcome. The APC-CSA of 3.0 mm2/m2 maybe considered as a threshold for risk stratification.

Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

BackgroundTetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort.Methods and ResultsFetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes.ConclusionsPerinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

Right ventricular diastolic function in post-surgical Tetralogy of Fallot patients: A pilot study to make a comparison between echocardiography and cardiac MRI

Background: right ventricular (RV) systolic dysfunction is associated with poor outcomes in Tetralogy of Fallot (ToF) patients. Conversely, the influence of diastolic dysfunction in this setting is poorly known. In addition, evaluation of RV diastolic function by cardiac MRI is rarely performed.Materials and methods: twenty-four surgically treated ToF patients were enrolled in the study (54% males; median age at study: 28 (18–50) years; median age at surgery: 1.9 (0.4–8.2) years. They were studied by echocardiography to assess RV diastolic function in terms of traditional and TDI-derived parameters. At cardiac MRI, RV diastolic function was assessed by using phase-contrast analysis of flow through the tricuspid valve in short axis view. Diastolic dysfunction was graded as impaired relaxation, pseudo-normal, or restrictive physiology.Results: fifteen (62.5%) ToF subjects had echocardiographic evidence of diastolic dysfunction and eleven (45.8%) at cardiac MRI. Statistically significant correlation between echocardiographic and cardiac MRI parameters of diastolic dysfunction was weak (p < 0.05). The degree of RV diastolic dysfunction (expressed as E/E’ at TDI) was associated with right atrial volume indexed for body surface area at cardiac MRI (p < 0.0001). Greater number of interim palliative procedures was associated with higher E/E' (RV diastolic dysfunction) at echocardiography (p < 0.0003).Conclusions: diastolic dysfunction, as determined by echocardiography and cardiac MRI-derived measures, is prevalent in ToF. These measures are weakly associated with each other. Notwithstanding the limitations of this pilot study, the development of cardiac MRI parameters capable of correlating with RV relaxation is needed in ToF setting.

Longitudinal changes in extent of late gadolinium enhancement in repaired Tetralogy of Fallot: a retrospective analysis of serial CMRs

BackgroundRight ventricular (RV) late gadolinium enhancement (LGE) occurs due to surgical scarring and RV remodeling, and has been shown to be associated with clinical outcomes in Tetralogy of Fallot (TOF). However, it is not known if cardiovascular magnetic resonance (CMR) LGE extent progresses over time, and therefore, it is not known if serial reassessment of LGE is necessary. We determined the rate of progression in the extent of RV LGE on serial CMR examinations in repaired TOF.MethodsRetrospective review of 127 patients after TOF repair (49% male, median age at first CMR 18.9 years (Interquartile range (IQR) 13.3,27.0) who had at least two CMRs (median follow-up duration of 4.0 years (IQR 2.1,5.9)) was performed. 84/127 patients had no interventions between serial CMRs (Group 1) while 43/127 patients had transcatheter or surgical intervention between CMRs (Group 2). The extent of RV LGE was assessed using 2 methods: a semiquantitative RV LGE score and a quantitative RV LGE extent expressed as % of RV mass. Mixed effects linear regression modeling to estimate changes in LGE over time.ResultsRV LGE was present in all patients on the first CMR. % RV LGE extent and LGE score did not increase over time in either patient group. The mean 5 year rates of change were small and negative for both % RV LGE extent [− 2.3 (95% CI − 2.9, − 1.8, p < 0.001) in Group 1, and − 1.9 (95% CI − 3.2, − 0.7, p = 0.004) in Group 2], and RV LGE score [− 0.9 (95% CI − 1.1, − 0.6, p < 0.001) in Group 1, and − 0.5 (95% CI − 1.1, − 0.0, p = 0.047) in Group 2].ConclusionsIn this serial CMR evaluation of children and adults with repaired TOF, no significant progression in the extent of RV LGE was seen on intermediate term follow-up. Given recent concerns regarding the safety of gadolinium-based contrast agents, frequent assessment of LGE may not be necessary in follow-up.

Angiographic Anatomy of Major Aortopulmonary Collateral Arteries and Association With Early Surgical Outcomes in Tetralogy of Fallot.

BackgroundDue in part to the heterogeneity of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries (MAPCAs), research on this condition has focused on relatively basic anatomic characteristics. We aimed to detail pulmonary artery (PA) and MAPCA anatomy in a large group of infants, assess relationships between anatomy and early surgical outcomes, and consider systems for classifying MAPCAs.Methods and ResultsAll infants ( <1 year of age) undergoing first cardiac surgery for tetralogy of Fallot/MAPCAs from 2001 to 2019 at Stanford University were identified. Preoperative angiograms delineating supply to all 18 pulmonary segments were reviewed for details of each MAPCA and the arborization and size of central PAs. We studied 276 patients with 1068 MAPCAs and the following PA patterns: 152 (55%) incompletely arborizing PAs, 48 (17%) normally arborizing PAs, 45 (16%) absent PAs, and 31 (11%) unilateral MAPCAs. There was extensive anatomic variability, but no difference in early outcomes according to PA arborization or the predominance of PAs or MAPCAs. Patients with low total MAPCA and/or PA cross‐sectional area were less likely to undergo complete repair.ConclusionsMAPCA anatomy is highly variable and essentially unique for each patient. Though each pulmonary segment can be supplied by a MAPCA, central PA, or both, all anatomic combinations are similarly conducive to a good repair. Total cross‐sectional area of central PA and MAPCA material is an important driver of outcome. We elucidate a number of novel associations between anatomic features, but the extreme variability of the pulmonary circulation makes a granular tetralogy of Fallot/MAPCA classification system unrealistic.

Prognostic implications of inferior vena cava haemodynamics in ambulatory patients with tetralogy of Fallot.

AimsRight atrial pressure (RAP) provides a composite measure of right ventricular diastolic dysfunction, right atrial compliance, and volume status, and these three variables are typically abnormal in adults with repaired tetralogy of Fallot (TOF). RAP is a well‐established prognostic metric in patients with pulmonary hypertension, and recent data suggest that RAP is associated with clinical outcomes in TOF. The purpose of this study was to determine the role of inferior vena cava (IVC) haemodynamics (size and collapsibility) for the assessment of RAP and its potential application for risk stratification and prognostication in the TOF population.Methods and resultsAdult TOF patients with echocardiographic assessment of IVC haemodynamics were divided into patients with (derivation cohort, n = 256) and without (validation cohort, n = 492) cardiac catheterization data. We assessed the correlation between IVC haemodynamics, RAP, and disease severity indices [arrhythmias, peak oxygen consumption (VO2), and heart failure hospitalization] in derivation cohort and compared it with the correlations in the validation cohort. IVC haemodynamics correlated with RAP (r = 0.52, P < 0.001), with disease severity indices {atrial arrhythmias [area under the curve (AUC) 0.81], ventricular arrhythmias [AUC 0.67], heart failure hospitalizations [AUC 0.78], and peak VO2 [r = 0.53]}, and with transplant‐free survival in the derivation cohort. Similar correlations between IVC haemodynamics, disease severity indices, and transplant‐free survival were also observed in the validation cohort.ConclusionsThese findings suggest that IVC haemodynamics can potentially be used for risk stratification and prognostication in TOF patients and can complement the current risk models that are based predominately on right ventricular volumes and systolic function.

Ventricular response to dobutamine stress cardiac magnetic resonance imaging is associated with adverse outcome during 8-year follow-up in patients with repaired Tetralogy of Fallot.

AimsThe aim of this study was to evaluate the possible value of dobutamine stress cardiac magnetic resonance imaging (CMR) to predict adverse outcome in Tetralogy of Fallot (TOF) patients.Methods and resultsIn previous prospective multicentre studies, TOF patients underwent low-dose dobutamine stress CMR (7.5 µg/kg/min). Subsequently, during regular-care patient follow-up, patients were assessed for reaching the composite endpoint (cardiac death, arrhythmia-related hospitalization, or cardioversion/ablation, VO2 max ≤65% of predicted). A normal stress response was defined as a decrease in end-systolic volume (ESV) and increase in ejection fraction. The relative parameter change during stress was calculated as relative parameter change = [(parameterstress − parameterrest)/parameterrest] * 100. The predictive value of dobutamine stress CMR for the composite endpoint was determined using time-to-event analyses (Kaplan–Meier) and Cox proportional hazard analysis. We studied 100 patients [67 (67%) male, median age at baseline CMR 17.8 years (interquartile range 13.5–34.0), age at TOF repair 0.9 years (0.6–2.1)]. After a median follow-up of 8.6 years (6.7–14.1), 10 patients reached the composite endpoint. An abnormal stress response (30% vs. 4.4%, P = 0.021) was more frequently observed in composite endpoint patients. Also in endpoint patients, the relative decrease in right ventricular ESV decreased less during stress compared with the patients without an endpoint (−17 ± 15 vs. −26 ± 13 %, P = 0.045). Multivariable analyses identified an abnormal stress response (hazard ratio 10.4; 95% confidence interval 2.5–43.7; P = 0.001) as predictor for the composite endpoint.ConclusionAn abnormal ventricular response to dobutamine stress is associated with adverse outcome in patients with repaired TOF.

Exercise testing and spirometry as predictors of mortality in congenital heart disease: Contrasting Fontan physiology with repaired tetralogy of Fallot.

Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality. All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test. Of the 42 cases, 27 had a Fontan circulation and 15 with TOF. All Fontan patients had a low peak VO2 but there was no significant difference between cases and controls (52.5±14.7 v. 57.4%±13.5% predicted, P=.11). Spirometry values were significantly lower in Fontan cases than controls (eg, FVC 67.4±19.1 v 77.6%±14.9% predicted, P=.007). Spirometry values were also lower in TOF cases than controls (% predicted FVC 62.8±16.7 v 75±14, P=.006). In contrast to the Fontan analysis, both %peak predicted VO2 and VE/VCO2 slope were worse in TOF cases than controls (50.1±13.5 v. 68.5%±15.0% predicted VO2 , P=.0004; 33.9±12.9 v 26.6±4.4, P=.002). Multivariable analysis also identified different predictors of mortality among the anatomic subgroups. Spirometric data (FVC) correlated most strongly with mortality in Fontan patients while the VE/VCO2 slope was most associated with outcome in TOF patients. Variables most predictive of mortality in Fontan and TOF patients diverge but spirometry was abnormal and associated with mortality in both groups. When compared with age-matched controls, reduced FEV1 and FVC correlated most strongly with mortality in Fontan patients while VE/VCO2 slope correlated with mortality for TOF patients. These findings further support the importance of lung health in patients with complex congenital heart disease.

Perioperative Factors Influence the Long-Term Outcomes of Children and Adolescents with Repaired Tetralogy of Fallot.

Tetralogy of Fallot (TOF) often carries long-term seqüelae following surgical intervention. We hypothesized that early perioperative factors are associated with long-term adverse right ventricular (RV) remodeling, diminished exercise capacity, and increased morbidity. We conducted a retrospective cohort study of patients operated for TOF that underwent cardiac magnetic resonance imaging study (CMR), exercise stress test (EST), and detailed review of past medical history. Outcome variables included measures of RV size, and function, maximal work rate, and oxygen consumption, and interim hospitalizations, surgeries, and catheterizations. Thirty-nine subjects were included. Age at surgical repair was 0.3 ± 0.3years and age at testing was 9.7 ± 1.4years. On CMR, there was borderline RV dilation with moderate pulmonary insufficiency (PI) [RF 32% (8; 43)] and normal RV ejection fraction [60% (55; 67)]. On EST, there was low percent-predicted maximal oxygen consumption (77 ± 20%), and percent-predicted maximal work rate (84 ± 23%). On multivariable analysis, mechanical ventilation and Blalock-Taussig (BT) shunt prior to complete surgical repair were associated with the number of future hospitalizations. Duration of cardiopulmonary bypass and prior BT shunt were associated with future catheterizations. Prior BT shunt was a predictor of worse RVEF, while duration of mechanical ventilation and use of transannular patch were predictors of worse PI. Longer duration of mechanical ventilation (or LOS) was associated with worse maximal work rate. Surgical and perioperative factors may portend long-term RV remodeling and outcome in TOF. Further studies are warranted to explore these associations and potential underlying mechanisms.

Late gadolinium enhancement and adverse outcomes in a contemporary cohort of adult survivors of tetralogy of Fallot.

Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes. Single centre cohort study SETTING: National tertiary referral center Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29.5 years (range 17.5-64.2). Prospective follow-up for mean 2.4 years (SD 1.29). Cardiovascular magnetic resonance was performed, and late gadolinium enhancement mass was estimated for the LV using the 5-SD remote myocardium method, and for the RV using a segmental scoring system. Cohort characterization was determined through the use of a computerized database. Survival analysis from time of scan to first adverse event, defined as an episode of atrial arrhythmia, sustained ventricular arrhythmia, hospitalization with heart failure, or implantable cardioverter-defibrillator insertion. Eleven patients experienced an adverse outcome in the follow-up period, although there were no deaths. LV late gadolinium enhancement was associated with adverse outcomes in a univariate model (P = .027). However, when adjusted for age at scan the significant variables included NYHA class (P = .006), peak oxygen uptake (P = .028), number of prior sternotomies (P = .044), and higher indexed RV and LV end diastolic volumes (P = .002 and P < .001), but not RV or LV late gadolinium enhancement. Formal quantification of late gadolinium enhancement is not currently as helpful in ascertaining prognosis compared to other, more easily assessed parameters in a contemporary cohort of tetralogy of Fallot survivors, however assessment particularly of the LV holds promise for the future.

Tricuspid Repair at Pulmonary Valve Replacement Does Not Alter Outcomes in Tetralogy of Fallot

Chronic pulmonary regurgitation after tetralogy of Fallot repair often leads to progressive right ventricle dilation, dysfunction, and frequently, pulmonary valve replacement. For those with significant tricuspid regurgitation at the time of pulmonary valve replacement, it is unknown whether concomitant tricuspid valve repair improves postoperative outcomes. This is a retrospective review of patients after tetralogy of Fallot repair who underwent pulmonary valve replacement between 1999 and 2012. Preoperative and postoperative echocardiograms were assessed for tricuspid regurgitation (vena contracta) and right ventricular size and function (Tomtec software). Sixty-two patients underwent pulmonary valve replacement. Thirty-six (58%) had greater than or equal to moderate tricuspid regurgitation on preoperative echocardiogram. Significant predictors were not identified. Of the 36, 18 (50%) underwent concomitant tricuspid valve repair at the time of pulmonary valve replacement. After surgery, there was a significant reduction in the degree of tricuspid regurgitation (p < 0.001) and measures of right ventricular size (p < 0.05) in both cohorts. Between surgical groups, there was no statistical difference in the grade of tricuspid regurgitation (p= 0.47) or measures of right ventricular size (p > 0.4) at 6-month follow-up. Tricuspid regurgitation is a common finding in repaired tetralogy of Fallot, although risk factors for its development remain unclear. After pulmonary valve replacement with or without tricuspid valve repair there is significant improvement in the degree of tricuspid regurgitation and right ventricular size. Finally, 6 months after pulmonary valve replacement there were no statistical differences between those patients undergoing concomitant tricuspid valve repair and those undergoing pulmonary valve replacements alone.

Clinical study on standardized scoring system after repair of tetralogy of Fallot

Objective To establish a standardized scoring system after tetralogy of Fallot(TOF) correction and explore its clinical application values. Methods During the period of Jan.2006 and Apr.2014 at the Third Affiliated Hospital, Zhengzhou University and Henan Provincial People's Hospital, a total of 624 infants undergoing stage I anatomic correction were selected and their common clinical parameters such as liver size, systolic blood pressure, urinary volume, oxygenation index and occurrences of complications were recorded and scored.The cases were divided into low-risk, medium-risk and high-risk groups according to scores.The relevant endpoint parameters, including mortality or survival, on-pump time, intensive care unit(ICU) duration, hospitalization and medical expenses were recorded to analyze their relationship with postoperative scores. Results The data indicated that the short-term mortality after repair of tetralogy of Fallot was 5%.Moreover, there was no statistical difference(P=0.309) in the mortality between the low-risk group (total score 7 and mortality of >80%). The postoperative standby time, ICU stay, total hospital stays and cost increased with the rise of the score(P<0.001), and there were no statistical differences in hospital stays(P=0.469) and cost(P=0.365) between the medium-risk group and the high-risk group. Conclusions Based upon common clinical parameters, a standardized scoring system has been successfully established for patients after TOF correction, which plays an important role in gauging the disease progression and predicating the immediate postoperative outcomes of TOF. Key words: Tetralogy of Fallot; Prognosis; Scoring

Assessing Ventricular Scar in Tetralogy of Fallot

The prevalence of repaired congenital heart disease is increasing, and late mortality is an important clinical concern. Patients with tetralogy of Fallot (TOF) contribute the largest fraction of subjects in many articles that address late mortality.1–3 Multicenter studies and single-center groups have mined their databases for retrospective analyses in patients with TOF. These studies have tried to determine which interventions can alter the long-term course of the disease and when these interventions should be performed.4–6 In 2013, we know more about the long-term outcomes in TOF than in any other form of severe congenital heart disease. We have both surgical and catheter-based valve approaches to alter the major long-term hemodynamic problems. It would seem that if we can assist anyone with severe adult congenital heart disease, in TOF we stand on the tallest shoulders and might see the farthest. Article see p 905 For these reasons, we would hope to have the best success at treating ventricular tachycardia (VT) in this population. Antiarrhythmic medications and defibrillator therapies are offered to those patients with the highest risk of life-threatening VT. Ablation serves as a useful adjunct therapy, either to prevent future arrhythmia or to reduce implantable cardioverter-defibrillator shocks. In this issue of Circulation: Arrhythmia and Electrophysiology , Moore et al7 report the results of gross and microscopic evaluation from 27 postmortem specimens with TOF. The authors’ goal was to describe the dimensions of the right ventricular muscular isthmuses and quantify the degree of fibrosis. These isthmuses are narrow bands of conducting muscle; they are anatomically defined by features of the ventricle and by postsurgical scar. These isthmuses help …

Midgestation Fetal Pulmonary Annulus Size Is Predictive of Outcome in Tetralogy of Fallot

Surgical management of tetralogy of Fallot (TOF) is increasingly moving toward valve-sparing approaches rather than transannular patch (TAP). We evaluate whether fetal pulmonary valve (PV) size is predictive of postnatal course and surgical approach in TOF. In this retrospective study, fetal and postnatal demographic, clinical, and echocardiographic data on 66 patients diagnosed prenatally with TOF were collected. We compared those with midgestation PV z-score > -3.5 to those with z-score ≤-3.5. We analyzed fetal and postnatal PV size and growth and outcomes between groups Gestational age at first fetal echo was 23 weeks (range 18-28). PV diameter and z-score on midgestation echo were 3.5 mm (1.3-6.0) and -2.8 (-0.5 to -6.0) respectively. Patients with PV z-score ≤ -3.5 on first fetal echo had smaller PV diameter (4.5 vs. 5.0 mm, P = .047) and PV z-score (-3.8 vs. -2.8, P < .001) in late gestation and at time of surgery (6.0 mm vs. 7.0 mm, P = .01; z-score = -2.9 vs. -1.7, P = .007). Similarly, those with smaller fetal PV z-score had smaller main and branch pulmonary arteries at time of surgery. PV growth rate over gestation was similar between groups, while after-birth PV growth rate was lower in those with smaller PV (0 mm/month vs. 0.6 mm/month, P = .002). Those with smaller pulmonary valve were more likely to be cyanotic (P = .05), to undergo surgery at <1 month (P < .01), and to have a TAP repair (P = .01). Among patients undergoing valve-sparing repair, those with smaller PV underwent more reinterventions for residual valvar PS (P < .01). Midgestation fetal PV size is predictive of postnatal PV and PA size in TOF. Midgestation PV size has implications for timing and type of surgical management as well as for need for reintervention in valve-sparing repair patients and is therefore important to consider in prenatal counseling for TOF fetuses.

Circulation: Clinical Summaries

<i>Circulation:</i> Clinical Summaries

22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot

We sought to investigate the impact of 22q11.2 deletion on perioperative outcome in tetralogy of Fallot. We conducted a retrospective review of patients with tetralogy of Fallot who underwent complete surgical reconstruction at The Children's Hospital of Philadelphia between 1995 and 2006. Inclusion criteria included diagnosis of tetralogy of Fallot and known genotype. Fisher exact and Mann-Whitney tests were used for categoric and continuous variables, respectively. Regression analysis was used to determine whether deletion status predicts outcome. We studied 208 subjects with tetralogy of Fallot, 164 (79%) without and 44 (20%) with 22q11.2 deletion syndrome. There were no differences in sex, race, gestational age, age at diagnosis, admission weight, and duration of mechanical ventilation. Presenting anatomy, survival, complications and reoperations were also comparable between patients with and without 22q11.2 deletion syndrome. Those with 22q11.2 deletion syndrome had more aortopulmonary shunts preceding complete surgical repair (21% vs 7%, P=.02). This association was present after adjustment for presenting anatomy (stenosis, atresia, or absence of pulmonary valve and common atrioventricular canal) and surgical era. In addition, those with 22q11.2 deletion syndrome had longer cardiopulmonary bypass time (84 vs 72 minutes, P=.02) and duration of intensive care (6 vs 4 days, P=.007). Genotype affects early operative outcomes in tetralogy of Fallot resulting, in particular, in longer duration of intensive care. Future studies are required to determine factors contributing to such differences in this susceptible population.