Determinants and Clinical Outcomes of Patients With Tetralogy of Fallot Lost to Cardiology Follow-up.

BACKGROUND: Various rates of loss to follow-up (LTFU) have been reported in patients with congenital heart disease, but return to follow-up is rarely considered in those analyses. Outcomes of LTFU patients are difficult to assess because the patients no longer attend cardiac care. We leveraged data from the TRIVIA cohort, which combines more than 30 years of clinical and administrative data, allowing us to study outcomes even after LTFU. METHODS: This population-based cohort included 904 patients with tetralogy of Fallot (TOF) born from 1982 to 2015 in Québec, Canada. Risk factors for LTFU and outcomes were calculated by Cox models and marginal means/rates models. Outcomes of LTFU patients were compared with propensity score-matched non-LTFU patients. RESULTS: The cumulative risk of experiencing 1 episode of LTFU was 50.3% at 30 years. However, return to follow-up was frequent and the proportion of patients actively followed was 85.9% at 10 years, 76.4% at 20 years, and 70.6% at 30 years. Factors associated with a reduced risk of LTFU were primary repair with conduit (hazard ratio [HR] 0.29, 95% confidence interval [CI] 0.15-0.58) and transannular patch (HR 0.60, 95% CI 0.46-0.79). LTFU patients had lower rates of cardiac hospitalisations (HR 0.49, 95% CI 0.42-0.56) and cardiac interventions (HR 0.32, 95% CI 0.25-0.42), but similar rates of cardiac mortality (HR 0.95, 95% CI 0.24-3.80). CONCLUSIONS: There was a lower proportion of LTFU patients compared with previous studies. Factors associated with lower rates of LTFU were conduits and non-valve-sparing surgery. LTFU patients had lower rates of cardiac procedures and cardiac hospitalisations.

Characterizing the Use of Exercise Testing in Repaired Tetralogy of Fallot Patients: A Multi-Institutional Survey.

Cardiopulmonary exercise testing (CPET) is an important tool used in the management of patients with congenital heart disease. However, there are no clear guidelines for its use in specific populations, such as repaired Tetralogy of Fallot (rTOF). We sought to characterize current practice patterns and attitudes regarding exercise testing in the rTOF population using an online survey distributed to pediatric cardiologists. Analyses were performed using qualitative statistics, Wilcoxon rank-sum, Kruskal-Wallis test, and chi-squared analysis. 103 clinicians completed the survey with 83% routinely sending symptomatic rTOF patients for exercise testing and 59% for asymptomatic patients. Respondents who routinely test asymptomatic patients reported higher levels of perceived helpfulness of exercise testing (p < 0.01) and comfort with CPET interpretation (p < 0.01). Although a large majority of respondents found exercise testing to be helpful (81% either "somewhat" or "very" helpful"), a considerably smaller portion indicated comfort with CPET interpretation (49% either "comfortable" or "very comfortable"). Nearly all respondents (92%) reported changing management primarily based on exercise testing results. However, the frequency of changes varied, with 10% "frequently", 62% "occasionally", 19% "rarely" changing management. There was a statistically significant relationship between the perceived helpfulness of exercise testing and the likelihood of management changes (p < 0.01). While exercise testing is used to make clinical decisions, our findings suggest that in some cases, management changes may be made without a similar degree of confidence in interpreting CPET findings. The variability in attitudes and practices highlights the need for evidence-based guidelines addressing exercise testing in rTOF, particularly for asymptomatic patients.

Genetic Risk Score Constructed from Polymorphisms in the VEGFA, TBX5, and SMAD7 Genes Provides Novel Insights into the Molecular Mechanisms of the Tetralogy of Fallot and Ventricular Septal Defect (Case-Control Study from the Pakistani Population).

Congenital heart defects are common and complex birth-defect malformations in developed and developing countries. It is a multifactorial disease that involves the interaction of either gene-gene or gene-environment. This comparative study was the first report on the genotypic-phenotypic correlation in the Pakistani population. The single nucleotide polymorphisms (SNPs) were further tested for association with maternal diabetes mellitus or hypertension. In addition, the cumulative genetic risk score (GRS) for low to moderately-associated SNPs was calculated for each study subject, which can ultimately guide us for better therapeutic options and prevention strategies. According to the predefined selection criteria, 376 subjects were recruited. The multiplex mini-sequencing genotyping technique opted for the cost-effective genotyping of selected loci. The association of variants with the disease was examined using logistic regression analysis. The statistical and graphical analysis was conducted using SPSS, Haploview, SNPStats, and GraphPad Prism. The results for all SNPs analysis suggested a nonsignificant association with overall congenital heart defect risk except rs3809923. However, interestingly on stratified analysis variants, rs3809923 and rs3809922 showed an association only with tetralogy of Fallot. The remaining risk factor analysis for maternal hypertension and diabetes mellitus association with SNPs were nonsignificant. The GRS was the first time constructed for this low to moderately-associated variants. Interestingly, the cumulative GRS was significantly different from the control group revealing the cumulative effect of these polymorphisms panel in patients. In conclusion, the use of GRS in the clinical setting can predict better risk association and patient outcomes.

Prevalence and determinants of tricuspid regurgitation after repair of tetralogy of Fallot.

BACKGROUND: The prevalence and determinants of tricuspid regurgitation (TR) in patients with repaired Tetralogy of Fallot (rTOF) remain incompletely understood. OBJECTIVES: To explore the prevalence of and factors associated with TR in patients with rTOF, specifically, the relationship of right ventricular (RV) dilatation with TR severity. METHODS: Patients (≥17 yrs) with rTOF referred to our service (2000-2019) were identified. Those with severe pulmonary stenosis, significant shunt, or previous tricuspid valve surgery were excluded. Using standard cardiac MRI protocols, RV, right atrial (RA) and tricuspid valve (TV) parameters were measured and compared. RESULTS: 68 consecutively eligible patients with rTOF were included in the study (27 ± 9 yrs., 35% female). Despite substantial RV volume overload (mean RVEDVi 153 mL/m2), the majority of the cohort (78%) had no or only mild TR. RA volumes, tenting height/area and annular diameter were normal (4.9 ± 2.0 mm, 1.1 ± 1.0 cm2 and 32.4 ± 6.2 mm, respectively). There was no significant correlation of TR fraction with RVEDVi (r = 0.13; p = 0.30), RVEF (r = 0.09; p = 0.44) or tricuspid annular diameter (r = 0.07; p = 0.62). Only RAVi showed a weak but significant correlation with TR fraction (0.29; p = 0.03). In a pooled cohort analysis, including both rTOF patients and adults with a dilated RV from pre-tricuspid shunt lesions, only rTOF was independently associated with higher TR fraction (p = 0.017). CONCLUSION: Despite substantial RV dilatation in a cohort with rTOF, there was surprisingly little TR. We found poor correlation between RVEDVi, RA volumes, tricuspid annular dilatation and the presence of significant TR. These findings question commonly held notions regarding the pathophysiology of functional TR in these patients.

Extra-cardiac diagnoses and postnatal outcomes of fetal tetralogy of fallot.

OBJECTIVE: Past studies of fetal tetralogy of fallot (ToF) have reported extra-cardiac anomalies (ECAs) in 17%-45%, genetic syndromes in as low as 20% and poor postnatal outcomes. This study sought to examine these factors in a contemporary cohort. METHODS: A retrospective review examining 83 fetuses with ToF diagnosed 2012-2019. Referral indication, ToF subtype, additional cardiac, extra-cardiac and genetic diagnoses, pregnancy outcomes and survival were documented. RESULTS: The mean gestational age at diagnosis was 23 ± 4 weeks. Of 94% (78/83) with genetic testing (GT), 30% (23/78, 95%CI 21%-40%) had genetic anomalies (GA), including Trisomy 21 (39%, 9/23), 22q11 deletion (35%, 8/23), Trisomy 13 or 18 (17%, 4/23) and 9% (2/23) others. A further 4% (3/78) had VACTERL association. Forty-one percent (34/83, 95%CI 31%-52%) had ≥1 major ECA of whom 41% (14/34) also had a genetic anomaly. OUTCOMES: 22% (18/83) pregnancy termination, 5% (4/83) intrauterine death and 72% (60/83) live birth. Of live births, 3% (2/60) experienced neonatal death, 7% late death (4/60) and 90% (54/60) were alive at last follow-up (mean age 3.5 ± 2.4 years). CONCLUSION: In a cohort of fetuses with ToF and high rates of GT, compared to previous reports, GA were more common and there were similar rates of ECAs.

Incidence of Infective Endocarditis Among Patients With Tetralogy of Fallot.

Background Patients with tetralogy of Fallot (ToF) are considered at high risk of infective endocarditis (IE) as a result of altered hemodynamics and multiple invasive procedures, including pulmonary valve replacement (PVR). Data on the long-term risk of IE are sparse. Methods and Results In this observational cohort study, all patients with ToF born from 1977 to 2018 were identified using Danish nationwide registries and followed from date of birth until occurrence of first-time IE, emigration, death, or end of study (December 31, 2018). The comparative risk of IE among patients with ToF versus age- and sex-matched controls from the background population was assessed. Because of rules on anonymity, exact numbers cannot be reported if the number of patients is <4. A total of 1164 patients with ToF were identified and matched with 4656 controls. Among patients with ToF, 851 (73.1%) underwent early surgical intracardiac repair and 276 (23.7%) underwent PVR during follow-up. During a median follow-up of 20.3 years, 41 (3.5%) patients with ToF (comprising 24 [8.7%] with PVR and 17 [1.9%] without PVR) and <4 (<0.8%) controls were admitted with IE. The incidence rates of IE per 10 000 person-years were 22.4 (95% CI, 16.5-30.4) and 0.1 (95% CI, 0.01-0.7) among patients and controls, respectively. Moreover, PVR was associated with a further increased incidence of IE among patients with ToF (incidence rates per 10 000 person-years with and without PVR were 46.7 [95% CI, 25.1-86.6] and 2.8 [95% CI 2.0-4.0], respectively). Conclusions Patients with ToF are associated with a substantially higher incidence of IE than the background population. In particular, PVR was associated with an increased incidence of IE. With an increasing life expectancy of these patients, intensified awareness, preventive measures, and surveillance of this patient group are decisive.

Long-term follow-up and sex differences in adults operated for tetralogy of Fallot.

OBJECTIVE: Adults operated for tetralogy of Fallot (TOF) have high risk of ventricular arrhythmias (VA). QRS duration >180 ms is an established risk factor for VA. We aimed to investigate heart function, prevalence of arrhythmias and sex differences in patients with TOF at long-term follow-up. METHODS: We included TOF-operated patients≥18 years from our centre's registry. We reviewed medical records and the most recent echocardiographic exam. VA was recorded on ECGs, 24-hour Holter registrations and from implantable cardioverter defibrillator. RESULTS: We included 148 patients (age 37±10 years). Left ventricular global longitudinal strain (LV GLS, -15.8±3.1% vs -18.8±3.2%, p=0.001) and right ventricular (RV) GLS (-15.8±3.9% vs -19.1±4.1%, p=0.001) were lower in men at all ages compared with women. Higher RV D1 (4.3±0.5 cm vs 4.6±0.6 cm, p=0.01), lower ejection fraction (55%±8% vs 50%±9%, p=0.02), lower RV GLS (-18.1±4.0 ms vs -16.1±4.8 ms, p=0.04) and N-terminal pro-brain natriuretic peptide (NT-proBNP) over reference range (n=27 (23%) vs n=8 (77%), p<0.001) were associated with higher incidence of VA. QRS duration was longer in men (151±30 ms vs 128±25 ms, p<0.001). No patients had QRS duration >180 ms. QRS duration did not differ in those with and without VA (143±32 ms vs 137±28 ms, p=0.06). CONCLUSIONS: Our results confirmed reduced RV function in adults operated for TOF. Male patients had impaired LV and RV function expressed by lower LV and RV GLS values at all ages. Reduced cardiac function and elevated NT-proBNP were associated with higher incidence of VA and may be important in risk assessment.

Risk Factors of Right Ventricular Dysfunction and Adverse Cardiac Events in Patients with Repaired Tetralogy of Fallot.

AIM: This study evaluates the risk factors associated with right ventricular (RV) dilation and dysfunction leading to pulmonary valve replacement (PVR) or adverse cardiac events in repaired Tetralogy of Fallot (rToF) patients. METHODS: Data from all rToF patients who underwent magnetic resonance imaging (MRI) evaluation at our hospital between February 2007 and September 2020 were collected. RESULTS: Three hundred and forty-two patients (60% males, 42% older than 18 years), with a median age of 16 years (IQR 13-24) at the time of MRI, were included. All patients underwent complete repair at a median age of 8 months (IQR 5-16), while palliation was performed in 56 patients (16%). One hundred and forty-four patients (42%) subsequently received pulmonary valve replacement (PVR). At the multivariate analysis, male gender was an independent predictor for significant RV dilation, RV and left ventricular (LV) dysfunction. Transventricular ventricular septal defect (VSD) closure and previous palliation significantly affected LV function and RV size, respectively. Male gender and the transventricular VSD closure were independent predictors for PVR. CONCLUSIONS: Male gender and surgical history (palliation, VSD closure approach) significantly affected the long-term outcomes in rToF patients and should be taken into consideration in the follow-up management and in PVR timing in this patient population.

Risk Factors for Maternal and Perinatal Complications during Pregnancy among Women with Tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease during pregnancy. Studies of risk factors are of great significance to maternal and fetal outcomes in patients with TOF. AIMS: To identify predictive risk factors for maternal and perinatal complications in women with TOF. SUBJECTS AND METHODS: 78 patients with TOF who delivered at Shanghai Obstetrical Cardiology Intensive Care Center between January 1993 and December 2017 were retrospective reviewed. A logistic regression model was used to identify risk factors for maternal and perinatal complications. RESULTS: There was no maternal death, five patients developed cardiac failure, sustained arrhythmias requiring treatments were recorded among 2 patients. Factors identified for maternal complications included previous cardiac events and oxygen saturation <90%. Oxygen saturation <90% was found to be independent predictor of maternal cardiac complications (RR = 21.455, 95%CI 2.186-210.572, P = 0.009). The perinatal survival rate was 87.18%, there were 52 term deliveries (66.67%). Perinatal poor outcomes include 9 therapeutic abortions (11.54%), 1 neonatal death (1.28%), 16 premature births (20.51%), 18 small for gestational age children (23.08%), 3 neonatal asphyxia (3.85%), and 3 neonatal cardiac malformations (3.85%). Factors identified for perinatal complications included without cardiac surgery, higher hemoglobin values, higher hematocrit values, oxygen saturation <90%, right ventricular hypertrophy, pulmonary stenosis, ventricular septal defect, and pulmonary hypertension. Oxygen saturation <90% was found to be independent predictor of perinatal complications (RR = 8.270, 95%CI 1.374-49.790, P = 0.021). CONCLUSIONS: Oxygen saturation <90% is associated with maternal and perinatal risks. Women with TOF whose oxygen saturation <90% are not recommended for pregnancy because of high maternal and perinatal complications.

Growth and development of children under 5 years of age with tetralogy of Fallot in a Chinese population.

Congenital Heart Defects (CHDs) are associated with different patterns of malnutrition and growth retardation, which may vary worldwide and need to be evaluated according to local conditions. Although tetralogy of Fallot (TOF) is one of the first described CHDs, the etiology outcomes in growth and development of TOF in early age child is still unclear in most cases. This study was designed to investigate the growth retardation status of Chinese pediatric TOF patients under 5 years old. The body height, body weight and body mass index (BMI) of 262 pediatric patients (138 boys and 124 girls) who underwent corrective surgery for TOF between 2014 and 2018 were measured using conventional methods. The average body height, body weight and BMI of the patients were significantly lower than WHO Child Growth Standards, while the most affected was body height. Meanwhile, higher stunting frequency and greater deterioration of both the body height and weight happened in elder age (aged 13-60 months) rather than in infant stage (aged 0-12 months) among these patients. Our results confirmed that intervention should be given at early age to prevent the growth retardation of TOF patients getting severer.

The challenges of an aging tetralogy of Fallot population.

Introduction: Advancements in surgery and management have resulted in a growing population of aging adults with tetralogy of Fallot (TOF). As a result, there has been a parallel growth in late complications associated with the sequelae from the underlying cardiac anomalies as well as the surgical and other interventional treatments.Areas covered: Here, we review challenges related to an aging population of patients with TOF, particularly late complications, and highlight advances in management and key areas for future research. Pulmonary regurgitation, heart failure, arrhythmias, and aortic complications are some of these late complications. There is also a growing incidence of acquired cardiovascular disease, obesity, and diabetes associated with aging. Management of these late complications and acquired comorbidities continues to evolve as research provides insights into long-term outcomes from medical therapies and surgical interventions.Expert opinion: The management of an aging TOF population will continue to transform with advances in imaging technologies to identify subclinical disease and valve replacement technologies that will prevent and mitigate disease progression. In the coming years, we speculate that there will be more data to support the use of novel heart failure therapies in TOF and consensus guidelines on the management of refractory arrhythmias and aortic complications.

Prognostic Value of Preoperative Absolute Lymphocyte Count in Children With Tetralogy of Fallot.

Background Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Absolute lymphocyte count (ALC) is a low-cost and easy-to-obtain inflammatory indicator; however, its association with the prognosis of patients with TOF remains unknown. This study aimed to determine the prognostic value of preoperative ALC in children with TOF. Methods and Results This retrospective study included 707 patients aged <6 years who underwent corrective operations for TOF between January 2016 and December 2018 in Fuwai Hospital, China. The end points were mortality, extracorporeal membrane oxygenation placement, postoperative hospital stay >30 days, and severe postoperative complications; patients were grouped on the basis of prognosis: poor prognosis (n=76) and good prognosis (n=631). Univariable and multivariable logistic regression analyses were performed to identify the independent risk factors for poor prognosis, on which a risk scoring system was based. The receiver operating characteristic curve was used to assess model performance. Using another model without ALC, the effect of the addition of ALC was assessed. Results suggested that ALC was an independent factor with a cutoff point of 4.36×109/L. The addition of ALC improved the area under the curve from 0.771 to 0.781 (P<0.001). To avoid reverse causality and further control for confounding factors, the patients were further divided on the basis of ALC level, and a propensity score matching was performed; 117 paired patients were identified for further analysis. Low ALC levels had an odds ratio of 3.500 (95% CI, 1.413-8.672). Conclusions Low preoperative ALC represents an independent predictor of poor prognosis in children with TOF.

[Epidemiological surveillance of congenital heart defects: the Tetralogy of Fallot in the Valencian Region, 2007-2017.] / Vigilancia epidemiológica de las anomalías congénitas cardíacas: La tetralogía de Fallot en la Comunitat Valenciana, 2007-2017.

OBJECTIVE: Tetralogy of Fallot is characterized by the presence of four congenital heart defects. Objective: to describe the temporal trend and distribution of Tetralogy of Fallot, in children under one year in the Valencian Region. METHODS: Cases with Tetralogy of Fallot (code Q21.3 from the ICD10-British Paediatric Association) were selected from the Congenital Anomalies Population-based Registry between 2007-2017. Prevalence per 10,000 births with 95%CI was calculated, and a descriptive analysis of sociodemographic and clinical variables was made. RESULTS: 165 cases were identified (43.6% male, 30.9% female and 25.5% unknown). The overall prevalence was 3.1/10,000 births (95%CI:2.6-3.6), being 2015 and 2017 the years with the highest (4.3/10,000 births and 4.7/10,000 births respectively) and 2011 with the lowest (1.8/10,000 births). 72.1% were live births, 24.8% Termination of Pregnancy for Fetal Anomaly (TOPFA) and 3.0% stillbirths. The prevalence in live births was 2.2/10,000 births (95%CI:1.8-2.7) and in TOPFA it was 0.8/10,000 births (95%CI:0.5-1.0), identifying an increasing trend along the period in the last one. 10.1% of live births died during the first year of life and 55.8% were diagnosed prenatally. Mothers younger than 20 years had the highest prevalence (4.8/10,000 births). CONCLUSIONS: The prevalence obtained in the Valencian Region was slightly lower than EUROCAT's but coincides with that of the registries that are closer geographically, and in all of them it is noted that their increasing trend specifically affects cases ending in TOPFA.

OBJETIVO: La Tetralogía de Fallot está caracterizada por la presencia de cuatro anomalías congénitas cardíacas. El objetivo de este trabajo fue describir la tendencia temporal y distribución de la Tetralogía de Fallot en menores de un año en la Comunitat Valenciana. METODOS: Se seleccionaron los casos con Tetralogía de Fallot (código Q21.3 de la CIE10 de la Asociación Pediátrica Británica) nacidos entre 2007-2017 del Registro Poblacional de anomalías congénitas de la Comunitat Valenciana. Se calculó la prevalencia por 10.000 nacimientos con IC95% y se realizó un análisis descriptivo de las variables sociodemográficas y clínicas. RESULTADOS: Se identificaron 165 casos (43,6% niños, 30,9% niñas y 25,5% de sexo desconocido). La prevalencia global fue 3,1/10.000 nacimientos (IC95%:2,6­3,6), siendo los años de mayor prevalencia 2015 y 2017 (4,3/10.000 nacimientos y 4,7/10.000 nacimientos respectivamente) y 2011 el de menor (1,8/10.000 nacimientos). El 72,1% fueron nacidos vivos, el 24,8% Interrupciones Voluntarias del Embarazo y el 3,0% nacidos muertos. La prevalencia en nacidos vivos fue 2,2/10.000 nacimientos (IC95%:1,8-2,7) y en Interrupciones Voluntarias del Embarazo fue 0,8/10.000 nacimientos (IC95%:0,5-1,0), identificándose en la segunda una tendencia en aumento a lo largo del periodo. El 10,1% de nacidos vivos fallecieron durante el primer año de vida y el 55,8% se diagnosticaron prenatalmente. El grupo con mayor prevalencia fueron las embarazadas menores de 20 años (4,8/10.000 nacimientos. CONCLUSIONES: La prevalencia obtenida en la Comunitat Valenciana fue ligeramente inferior a la de EUROCAT pero coincide con la de registros próximos geográficamente, y en todos ellos destaca que su tendencia creciente afecta específicamente a casos que finalizan en Interrupciones Voluntarias del Embarazo.

Pattern of congenital heart disease among children presenting to the Uganda Heart Institute, Mulago Hospital: a 7-year review.

BACKGROUND: Congenital heart disease (CHD) is the most common congenital anomaly in children. Over half of the deaths due to CHD occur in the neonatal period. Most children with unrepaired complex heart lesions do not live to celebrate their first birthday. We describe the spectrum of congenital heart disease in Uganda. METHODS: We retrospectively reviewed the data of children with CHD who presented to the Uganda Heart Institute (UHI), Mulago Hospital Complex from 2007 to 2014. RESULTS: A total of 4621 children were seen at the UHI during the study period. Of these, 3526 (76.3%) had CHD; 1941(55%) were females. Isolated ventricular septal defect (VSD) was the most common CHD seen in 923 (27.2%) children followed by Patent ductus arteriosus (PDA) 760 (22%) and atrial septal defects (ASD) 332 (9.4%). Tetralogy of Fallot (TOF) and Truncus arteriosus were the most common cyanotic heart defects (7% and 5% respectively). Dysmorphic features were diagnosed in 185 children, of which 61 underwent genetic testing (Down syndrome=24, 22q11.2 deletion syndrome n=10). Children with confirmed 22q11.2 deletion had conotruncal abnormalities. CONCLUSION: Isolated VSD and Tetralogy of Fallot are the most common acyanotic and cyanotic congenital heart defects. We report an unusually high occurrence of Truncus arteriosus.

Risk factors for severe pulmonary regurgitation after repair of tetralogy of Fallot with transannular patch.

BACKGROUND: Severe pulmonary regurgitation may result in right ventricular volume overload and decreased right ventricular function. Severe pulmonary regurgitation can be predicted prior to repair of tetralogy of Fallot. The aim of this study was to determine the risk factors for severe pulmonary regurgitation in repaired tetralogy of Fallot with transannular patch. METHODS: This was a cross-sectional study in 43 patients with repaired tetralogy of Fallot using transannular patch. This study was carried out in Dr. Cipto Mangunkusumo hospital during 2015 to 2018. Participants were followed up for routine examination using echocardiography. We used bivariate and multivariate logistic regression using STATA 12.1 to identify risk factors for severe pulmonary regurgitation in this population. RESULTS: A total of 43 patients composed of 22 boys and 21 girls with repaired tetralogy of Fallot using transannular patch were enrolled in the study. Median age of participants was 6 years at admission (2.1-18.5 years) and 3.4 years (1-17 years) at repair. Median length of follow-up was 2.1(1-4.3) years. Risk factors associated with severe pulmonary regurgitation after tetralogy of Fallot repair were McGoon ratio > 1.8 (odds ratio = 6.9; 95% confidence interval = 1.6-30) and follow-up duration >1.9 years (odds ratio = 3.6; 95% confidence interval = 0.9-15.2). CONCLUSION: McGoon ratio > 1.8 and follow-up duration > 1.9 years are associated with severe pulmonary regurgitation after tetralogy of Fallot repair.

Long-Term Follow-Up of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator: The DAI-T4F Nationwide Registry.

BACKGROUND: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce. METHODS: A Nationwide French Registry including all patients with tetralogy of Fallot with an ICD was initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event end point was the time from ICD implantation to first appropriate ICD therapy. Secondary outcomes included ICD-related complications, heart transplantation, and death. Clinical events were centrally adjudicated by a blinded committee. RESULTS: A total of 165 patients (mean age, 42.2±13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (interquartile range) follow-up of 6.8 (2.5-11.4) years, 78 (47.3%) patients received at least 1 appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively; P=0.03). Overall, 71 (43.0%) patients presented with at least 1 ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) patients in primary prevention, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with, respectively, 0, 1, 2, or ≥3 guidelines-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (hazard ratio, 3.47 [95% CI, 1.19-10.11]), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 (P=0.006). Patients with congestive heart failure or reduced left ventricular ejection fraction had a higher risk of nonarrhythmic death or heart transplantation (hazard ratio, 11.01 [95% CI, 2.96-40.95]). CONCLUSIONS: Patients with tetralogy of Fallot and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03837574.

Incidence of systemic venous anomalies in tetralogy of Fallot on multidetector CT angiography: A retrospective single-center study of 973 patients.

OBJECTIVES: The present study sought to estimate the incidence and evaluate the patterns and associations of systemic venous anomalies in patients with tetralogy of Fallot on multidetector computed tomography (CT) angiography. METHODS: We retrospectively reviewed CT angiographies of 973 pediatric age group patients diagnosed with tetralogy of Fallot, performed at our institution between 1st January 2015 and 31st December 2019, for systemic venous drainage patterns. We also proposed a structured classification and reporting system on CT angiography which would simplify the characterization, classification, and communication of these anomalies. RESULTS: Variations in systemic venous drainage patterns were observed in 146 (15.01%) patients. The most commonly observed anomaly was persistence of left superior caval vein, which was seen in 112 (11.51%) patients, with the most prevalent pattern being the presence of bilateral superior caval veins with no intercommunicating vein (n = 85). The second most common anomaly observed was presence of anomalous brachiocephalic vein (39/973 [4.01%]). Patients with an anomalous course of brachiocephalic vein had significantly higher prevalence of a right aortic arch than those without (23/39 [58.97%] vs 188/934 [20.13%]; P < .0001). Similarly, a higher prevalence of pulmonary atresia was noted in patients with an anomalous course of brachiocephalic vein compared to those without (8/39 [20.51%] vs 80/934 [8.57%]; P = .0109). CONCLUSION: There is a high incidence of anomalies of superior caval veins and brachiocephalic vein in the presence of tetralogy of Fallot. With advancements in corrective cardiac surgery and use of intraoperative cardiopulmonary bypass, the presence of systemic venous anomalies assumes greater significance making preoperative identification of these anomalies imperative.