The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions

Blais, Samuel; Marelli, Ariane J.; Vanasse, Alain; Dahdah, Nagib; Dancea, Adrian; Drolet, Christian; Colavincenzo, J.; Vaugon, Esther; Dallaire, Frédéric

doi:10.1016/j.cjca.2020.10.002

Cited by 31 publications

(25 citation statements)

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“…The algorithms used to identify causes of death, cardiovascular interventions, and hospitalizations have been described previously. 22…”

Section: Discussionmentioning

confidence: 99%

“…The detailed rationale and design of the TRIVIA study have been described previously. 21,22 We included all patients with TOF who had at least mild pulmonary RVOT obstruction and underwent surgical correction with a VS procedure or TAP. We excluded individuals with pulmonary atresia, TOF with absent pulmonary valve syndrome, and concomitant severe heart defects.…”

Section: Study Design and Populationmentioning

confidence: 99%

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Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot

et al. 2021

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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.OBJECTIVE To 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.EXPOSURES The 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, 0.1-0.7PVRs] 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, (continued) Key Points Question Do valve-sparing (VS) operations have better long-term outcom...

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“…The algorithms used to identify causes of death, cardiovascular interventions, and hospitalizations have been described previously. 22…”

Section: Discussionmentioning

confidence: 99%

Section: Study Design and Populationmentioning

confidence: 99%

Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot

et al. 2021

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“…The most frequent ones are del22q11 deletion (Di George) syndrome and Down syndrome (DS) in 15% and 7% of cases, respectively, followed by Noonan, Holt-Oram, and VACTERL syndromes [1][2][3][4][5][6][7]. Previously, an association has been reported between the presence of a genetic syndrome in patients with ToF and additional risks to the primary repair [8][9][10][11][12] due to the presence of associated anatomical abnormalities, immunodeficiency, or anomalies of pulmonary vascular resistance [10][11][12]. In fact, it is well established in the literature that DS patients, even those with a structurally normal heart, have a greater risk of developing pulmonary arterial hypertension and are more susceptible to common cardiovascular risk factors such as sedentary lifestyle, obesity, and hypertension [13,14].…”

Section: Introductionmentioning

confidence: 99%

“…Therefore, in addition to the different perioperative outcomes, syndromic patients with ToF could have a worse long-term outcome when compared to non-syndromic ones [8][9][10][11]. It is possible that the consequences of ToF repair due to significant chronic pulmonary regurgitation (PR) are worse in syndromic patients.…”

Section: Introductionmentioning

confidence: 99%

Syndromic and Non-Syndromic Patients with Repaired Tetralogy of Fallot: Does It Affect the Long-Term Outcome?

Calcagni

Calvieri

Baban

et al. 2022

JCM

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Background: The impact of genetic syndromes on cardiac magnetic resonance imaging (cMRI) parameters, particularly on right and/or left ventricular dysfunction, associated with clinical parameters following the repair of Tetralogy of Fallot (rToF) is not well known. Therefore, this study aimed to assess the differences in clinical, surgical, and cMRI data in syndromic and non-syndromic rToF patients. Methods: All syndromic rToF patients undergoing a cMRI without general anesthesia between 2010 and 2020 who were able to match with non-syndromic ones for birth date, sex, type of surgery, timing of cMRI, and BSA were selected. Demographic, clinical, surgical, MRI, ECG, and Holter ECG data were collected. Results: A total of one hundred and eight rToF patients equally subdivided into syndromic and non-syndromic, aged 18.7 ± 7.3 years, were studied. Del22q11.2 and Down syndrome (DS) were the most frequent syndromes (42.6% and 31.5%, respectively). Regarding the cMRI parameters considered, left ventricular (LV) dysfunction (LVEF < 50%) was more frequently found in syndromic patients (p = 0.040). In addition, they were older at repair (p = 0.002) but underwent earlier pulmonary valve replacement (PVR) (15.9 ± 5.6 vs. 19.5 ± 6.0 years, p = 0.049). On multivariate Cox regression analysis, adjusted for age at first repair, LV dysfunction remained significantly more associated with DS than del22q11.2 and non-syndromic patients (HR of 5.245; 95% CI 1.709–16.100, p = 0.004). There were only four episodes of non-sustained ventricular tachycardia in our cohort. Conclusions: Among the cMRI parameters commonly taken into consideration in rToF patients, LV dysfunction seemed to be the only one affected by the presence of a genetic syndrome. The percentage of patients performing PVR appears to be similar in both populations, although syndromic patients were older at repair and younger at PVR. Finally, the number of arrhythmic events in rToF patients seems to be low and unaffected by chromosomal abnormalities.

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“…To the Editor: We thank Abdi et al for their comment on our recent study. 1,2 They rightfully pointed out that there is more to tetralogy of Fallot (TOF) outcomes than perinatal factors and we would not dare suggest that the trajectory of care of patients with TOF is influenced only by native anatomy and genetic conditions, although they are very important factors to consider.…”

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confidence: 99%

Reply to Abdi et al.—The Big Picture of the Long-term Outcomes of Tetralogy of Fallot

Blais

Dallaire

2021

Canadian Journal of Cardiology

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The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions

Abstract: See editorial by Chetan and Mertens, pages xxx-xxx of this issue.

Cited by 31 publications

References 30 publications

Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot

Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot

Syndromic and Non-Syndromic Patients with Repaired Tetralogy of Fallot: Does It Affect the Long-Term Outcome?

Reply to Abdi et al.—The Big Picture of the Long-term Outcomes of Tetralogy of Fallot

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