Sixty Years After Tetralogy of Fallot Correction.
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ABSTRACT:
SUBMITTER: Shenoy C
PROVIDER: S-EPMC6301050 | biostudies-literature | 2019 Jan
REPOSITORIES: biostudies-literature
Publications
Sixty Years After Tetralogy of Fallot Correction.
Shenoy Chetan C Moller James H JH
The Annals of thoracic surgery 20180702 1
This report describes one of the early cases of open surgical correction of tetralogy of Fallot performed by C. Walton Lillehei and colleagues at the University of Minnesota and discusses findings from the patient's follow-up 60 years later. ...[more]
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Project description:A 59-year-old Japanese woman was admitted with heart failure due to severe pulmonary regurgitation and tricuspid regurgitation, in addition to atrial fibrillation 45 years after surgical correction of tetralogy of Fallot (TOF). She had been under treatment with medication and catheter ablation for arrhythmia including ventricular tachycardia for the past 28 years. She underwent pulmonary valve replacement as well as tricuspid and mitral valvuloplasty, which obviously improved her status even though her right ventricular end-diastolic volume index exceeded the recommended threshold. Patients who have undergone surgical correction of TOF need to be managed over the long term. <Learning objective: For a long term after surgical correction of tetralogy of Fallot (TOF), appropriate managements are needed for arrhythmia and heart failure related to heart valve disease. Even though her right ventricular end-diastolic volume index exceeded the recommended threshold by the current published guidelines, re-operation for heart valve diseases improved the present patient. We have to accumulate evidence to make useful guideline of re-operation of TOF in Japan.>.
| S-EPMC7817902 | biostudies-literature
Project description:Thanks to advances in pediatric cardiology, most infants with tetralogy of Fallot (TOF) now survive into adulthood. This relatively new population of adult patients may face long-term complications, including pulmonary regurgitation (PR), right ventricular (RV) tract obstruction, residual shunts, RV dysfunction, and arrythmias. They will often need to undergo pulmonary valve (PV) replacement and other invasive re-interventions. However, the optimal timing for these procedures is challenging, largely due to the complexity of evaluating RV volume and function. The options for the follow-up of these patients have rapidly evolved from an angiography-based approach to the surge of advanced imaging techniques, mainly echocardiography, cardiac magnetic resonance (CMR), and computer tomography (CT). In this review, we outline the indications, strengths and limitations of these modalities in the adult TOF population.
| S-EPMC7475417 | biostudies-literature
Project description:ImportanceThe choice of the right surgical technique for correction of tetralogy of Fallot (TOF) is contentious for patients with a moderate to severe right outflow tract obstruction. The use of a transannular patch (TAP) exposes patients to chronic pulmonary regurgitation, while valve-sparing (VS) procedures may incompletely relieve pulmonary obstruction.ObjectiveTo compare 30-year outcomes of TOF repair after a VS procedure vs TAP.Design, setting, and participantsThis retrospective population-based cohort study was conducted among all patients with TOF born in the province of Quebec, Canada, from 1980 to 2015 who underwent complete surgical repair. Patients who received a TAP or VS procedure were matched using a propensity score based on preoperative factors in a 1:1 ratio. Data were analyzed from March 2020 through April 2021.ExposuresThe study groups were individuals who received TAP and those who received VS. The VS group was further stratified by the presence of residual pulmonary stenosis.Main outcomes and measuresThe primary outcome was all-cause mortality, with 30-year survival evaluated using Cox proportional-hazards models. Secondary outcomes included the cumulative mean number of cardiovascular interventions, pulmonary valve replacements (PVRs), and cardiovascular hospitalizations were evaluated using marginal means/rates regressions.ResultsAmong 683 patients with TOF (401 patients who underwent TAP [58.7%] and 282 patients who underwent a VS procedure [41.3%]), adequate propensity score matching was achieved for 528 patients (264 patients who underwent a VS procedure and 264 patients who underwent TAP). Among this study cohort, 307 individuals (58.1%) were men. The median (interquartile range [IQR]) follow-up was 16.0 (8.1-25.4) years, for a total of 8881 patient-years, including 63 individuals (11.9%) followed up for more than 30 years. Individuals who received a VS procedure had an increased 30-year survival of 99.1% compared with 90.4% for individuals who received TAP (hazard ratio [HR], 0.09 [95% CI, 0.02-0.41]; P = .002). Patients who underwent TAP had an increased 30-year cumulative mean number of cardiovascular interventions compared with patients who underwent a VS procedure without residual pulmonary stenosis (2.0 interventions [95% CI, 1.5-2.7 interventions] vs 0.7 interventions [95% CI, 0.5-1.1 interventions]; mean ratio [MR], 0.36 [95% CI, 0.25-0.50]; P < .001) and patients who underwent a VS procedure with at least moderate residual stenosis (1.3 interventions [95% CI, 0.9-1.9 interventions]; MR, 0.65 [0.45-0.93]; P = .02). Results were similar for PVR, with a 30-year cumulative mean 0.3 PVRs [95% CI, 0.1-0.7 PVRs] for patients who underwent a VS procedure without residual pulmonary stenosis (MR, 0.22 [95% CI, 0.12-0.43]; P < .001) and 0.6 PVRs (95% CI, 0.2-1.5 PVRs) for patients with at least moderate residual stenosis (MR, 0.44 [95% CI, 0.21-0.93]; P = .03), compared with 1.4 PVRs (95% CI, 0.8-2.5 PVRs) for the TAP group. No statistically significant difference was found for cardiovascular hospitalizations.Conclusions and relevanceThis study found that patients who underwent a VS procedure had increased 30-year survival, fewer cardiovascular reinterventions, and fewer PVRs compared with individuals who underwent TAP, even in the presence of significant residual pulmonary stenosis. These findings suggest that it is beneficial to perform a VS procedure when possible, even in the presence of moderate residual stenosis, compared with the insertion of a TAP.
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Project description:Background Despite excellent operative survival, correction of tetralogy of Fallot frequently is accompanied by residual lesions that may affect health beyond the incident hospitalization. Measuring resource utilization, specifically cost and length of stay, provides an integrated measure of morbidity not appreciable in traditional outcomes. Methods and Results We conducted a retrospective cohort study, using de-identified commercial insurance claims data, of 269 children who underwent operative correction of tetralogy of Fallot from January 2004 to September 2015 with ≥2 years of continuous follow-up (1) to describe resource utilization for the incident hospitalization and subsequent 2 years, (2) to determine whether prolonged length of stay (>7 days) in the incident hospitalization was associated with increased subsequent resource utilization, and (3) to explore whether there was regional variation in resource utilization with both direct comparisons and multivariable models adjusting for known covariates. Subjects with prolonged incident hospitalization length of stay demonstrated greater resource utilization (total cost as well as counts of outpatient visits, hospitalizations, and catheterizations) after hospital discharge (P<0.0001 for each), though the number of subsequent operative and transcatheter interventions were not significantly different. Regional differences were observed in the cost of incident hospitalization as well as subsequent hospitalizations, outpatient visits, and the costs associated with each. Conclusions This study is the first to report short- and medium-term resource utilization following tetralogy of Fallot operative correction. It also demonstrates that prolonged length of stay in the initial hospitalization is associated with increased subsequent resource utilization. This should motivate research to determine whether these differences are because of modifiable factors.
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Project description: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.
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Project description:Ventricular arrhythmia is an important cause of late death in patients with repaired tetralogy of Fallot (rTOF). By using an rTOF canine model, we investigated the role of repolarization alternans and its electrophysiological mechanisms.Six dogs received right ventricular outflow tract (RVOT) transannular patch, pulmonary valve destruction, and right bundle branch ablation to simulate rTOF. After 1 year, we performed high-resolution dual-voltage and calcium optical mapping to record action potentials and calcium transients on the excised right ventricular outflow tract wedges. Another 6 dogs without operation served as control. The rTOF group was more susceptible to action potential duration alternans (APD-ALT) and spatially discordant APD-ALT than control (threshold for APD-ALT: 516±36 vs 343±36 ms; P=0.017; threshold for discordant APD-ALT: 387±30 vs 310±14 ms; P=0.046). We detected 2 episodes of ventricular tachycardia in the rTOF group, but none in the control. Expressions of Kv4.3 and KChIP2 decreased in the rTOF group. Expression of connexin 43 also decreased in the rTOF group with a corresponding decrease of conduction velocity and might contribute to spatially discordant APD-ALT. We also found distinct electrophysiological features of the RVOT, including biphasic relationship between magnitude of APD-ALT and pacing cycle length, uncoupling of APD-ALT, and calcium transients alternans, and shortened APD, but unchanged, APD restitution in rTOF.We demonstrated novel electrophysiological properties of the RVOT. In an rTOF model, the RVOT exhibits increased susceptibility to temporal and spatially discordant APD-ALT, which was not totally dependent on calcium transient alternans.
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Project description: Not available
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