Long-term Outcomes of Tetralogy of Fallot: A Study From the Pediatric Cardiac Care Consortium
Open accessAbstract
Multicenter retrospective cohort of 3,283 patients who survived repair for simple TOF; 25-year survival after repair was 94.5%, with analysis of patient- and surgery-associated risk factors affecting survival.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
11 of 22 cited sources checked · 4 supported · 1 flagged · accuracy not yet measured
- Study design and conductcitation
There are important limitations in the use of NDI for ascertainment of COD.
“There are also important limitations in the use of NDI for ascertainment of COD as highlighted by some comparison studies of assigned COD between NDI-based vs direct review methods.”
Quote in the paperNot a substantial claim1 of 1 sources checked- cites [27] 27. Olubowale OT, Safford MM, Brown TM, et al. Comparison of expert adjudicated coronary heart disease and cardiovascular disease mortality with the national death index: results from the Reasons for Geographic and Racial Differences in Stroke (REGARDS) study. J Am Heart Assoc. 2017;6(5): e004966. doi:10.1161/JAHA.116.004966 source
- Premisecitation
NDI data tended to overestimate coronary disease as the COD, especially in older patients with multiple comorbidities.
“More specifically, NDI data tended to overestimate coronary disease as the COD, especially in older patients with multiple comorbidities.27”
Quote in the paperNot a substantial claim1 of 1 sources checked- cites [27] 27. Olubowale OT, Safford MM, Brown TM, et al. Comparison of expert adjudicated coronary heart disease and cardiovascular disease mortality with the national death index: results from the Reasons for Geographic and Racial Differences in Stroke (REGARDS) study. J Am Heart Assoc. 2017;6(5): e004966. doi:10.1161/JAHA.116.004966 source
The Discussion reports that older participants were more likely to have a CHD death recorded by NDI when expert adjudicators found another cause. It does not support the added claim that this occurred especially in patients with multiple comorbidities; it instead says disagreement was less likely among participants with a history of CHD or CVD. The Discussion also reports similar overall mortality rates despite classification discrepancies, so the overestimation finding is best confined to older participants. (LLM, conf=0.88)
Experimental — accuracy not yet measured
- Study design and conductcitation
Linkage of the PCCC to the registries of the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) extends the follow-up time of PCCC-enrolled persons for major outcomes with nearly 90% sensitivity and almost 100% specificity for death and transplant events.
“Linkage of the PCCC to the registries of the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) extends the follow-up time of PCCC-enrolled persons for major outcomes with nearly 90% sensitivity and almost 100% specificity for death and transplant events.14,15”
Quote in the paperSubstantial claim2 of 2 sources checked- cites [14] 14. Spector LG, Menk JS, Vinocur JM, et al; In-Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium. In-hospital vital status and heart transplants after intervention for congenital heart disease in the pediatric cardiac care consortium: completeness of ascertainment using the National Death Index and United Network for Organ Sharing Datasets. J Am Heart Assoc. 2016;5(8):e003783. doi:10.1161/JAHA.116.003783 source
Split into 2 atoms — 1 confirmed, 1 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measured - cites [15] 15. Spector LG, Menk JS, Knight JH, et al. Trends in long-term mortality after congenital heart surgery. J Am Coll Cardiol. 2018;71(21):2434-2446. doi:10. 1016/j.jacc.2018.03.491 source
Split into 2 atoms — 0 confirmed, 2 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measured
- Findingown data
The high incidence of deaths from unintentional injury and suicides reported in the Taiwanese study was not confirmed in our cohort.
“with the exception of the high incidence of deaths from unintentional injury and suicides, which were not confirmed in our cohort.”
Quote in the paperNot a substantial claim1 of 1 sources checked- cites [10] 10. Chiu SN, Wang JK, Chen HC, et al. Long-term survival and unnatural deaths of patients with repaired tetralogy of Fallot in an Asian cohort. Circ Cardiovasc Qual Outcomes. 2012;5(1):120-125. doi: 10.1161/CIRCOUTCOMES.111.963603 source
- Findingcitation
The rates of sudden death and heart failure in our cohort are similar to those reported in other studies.
“The rates of sudden death and heart failure in our cohort are similar to those reported in other studies.9,18”
Quote in the paperNot a substantial claim2 of 2 sources checked- cites [9] 9. Nollert G, Fischlein T, Bouterwek S, Böhmer C, Klinner W, Reichart B. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol. 1997;30(5):1374- 1383. doi:10.1016/S0735-1097(97)00318-5 source
The Discussion (page unavailable) reports that sudden cardiac death remained the major cause of late death after tetralogy of Fallot repair. The follow-up passage (page unavailable) reports symptoms of congestive heart failure, but it does not establish a comparable heart-failure rate; the source also cannot assess similarity to the citing authors’ cohort. (LLM, conf=0.78)
Experimental — accuracy not yet measured - cites [18] 18. Nørgaard MA, Lauridsen P, Helvind M, Pettersson G. Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot. Eur J Cardiothorac Surg. 1999;16(2):125-130. doi:10.1016/ S1010-7940(99)00137-2 source
The Discussion reports seven late sudden deaths and says that incidence was identical to that in another study. Table 1 lists deaths involving heart failure, but our copy does not compare heart-failure rates with other studies. Nor can this source establish whether either rate is similar to the citing authors’ own cohort. (LLM, conf=0.82)
Experimental — accuracy not yet measured
- Findingcitation
The CODs in our cohort were similar to those reported in a study from Taiwan.
“The CODs in our cohort were similar to those reported in a study from Taiwan, 10”
Quote in the paperNot a substantial claim1 of 1 sources checked- cites [10] 10. Chiu SN, Wang JK, Chen HC, et al. Long-term survival and unnatural deaths of patients with repaired tetralogy of Fallot in an Asian cohort. Circ Cardiovasc Qual Outcomes. 2012;5(1):120-125. doi: 10.1161/CIRCOUTCOMES.111.963603 source
- Findingown data
Overall long-term transplant-free survival following discharge alive after TOF repair was 98.6%, 97.8%, 97.1%, 95.5%, and 94.5% at 1, 5, 10, 20, and 25 years after repair.
“Twenty-five-year survival following TOF repair was 94.5%.”
- Also stated in: § Long-term Survival After Complete TOF Repair · plain
“Overall long-term transplant-free survival following discharge alive after TOF repair was 98.6%, 97.8%, 97.1%, 95.5%, and 94.5% at 1, 5, 10, 20, and 25 years after repair (Figure 1A).”
Quote in the paperSubstantial claim - Also stated in: § Long-term Survival After Complete TOF Repair · plain
- Findingown data
The survival of those with a genetic abnormality was significantly disadvantaged compared with those without during the whole postoperative study period.
“In addition, the survival of those with a genetic abnormality was significantly disadvantaged compared with those without during the whole postoperative study period (Figure 2E).”
Quote in the paperSubstantial claim - Findingown data
In-hospital mortality following TOF repair was 4.1%.
“In-hospital mortality following TOF repair was 4.1% and occurred more commonly in infants, patients weighing less than 2.5 kg (proxy for prematurity) at repair, patients with prior shunt palliation, in earlier surgical eras (1982-1989 and 1999-2000), and concurrent genetic syndrome (Table 1).”
Quote in the paperNot a substantial claim - Findingown data
The late period had a low but steadily increasing hazard beyond 6 years following repair and with acceleration around 20 years postoperatively.
“(2) a late period with low but steadily increasing hazard beyond 6 years following repair and with acceleration around 20 years postoperatively.”
Quote in the paperSubstantial claim - Findingown data
The median age at death was 1.0 years (IQR, 0.6-2.1 years), with range 3 days to 19.7 years.
“The median age at death was 1.0 years (IQR, 0.6-2.1 years), with range 3 days to 19.7 years.”
Quote in the paperNot a substantial claim - Findingown data
Review of cause of death by surgical era did not reveal any significant differences.
“Review of COD by surgical era did not reveal any significant differences (data not shown).”
Quote in the paperNot a substantial claim - Findingown data
Presence of a genetic abnormality was associated with increased risk of death in the late postoperative phase (HR, 4.41; 95% CI, 2.62-7.44).
“and late postoperative phase (HR, 4.41; 95% CI, 2.62-7.44).”
- Also stated in: § Factors Associated With Long-term Mortality · plain
“In the later phase, presence of a genetic abnormality was the only association with increased risk of death (HR, 4.41; 95% CI, 2.62-7.44).”
Quote in the paperSubstantial claim - Also stated in: § Factors Associated With Long-term Mortality · plain
- Findingown data
After covariate adjustment, staged surgical strategy was associated with increased early mortality (HR, 2.68; 95% CI, 1.59-4.49).
“Multivariable analysis demonstrated increased risk of early mortality with staged repair (HR, 2.68; 95% CI, 1.59-4.49)”
- Also stated in: § Factors Associated With Long-term Mortality · plain
“After covariate adjustment, staged surgical strategy (HR, 2.68; 95% CI, 1.59-4.49)”
Quote in the paperSubstantial claim - Also stated in: § Factors Associated With Long-term Mortality · plain
- Findingown data
Presence of a genetic abnormality was associated with increased risk of death in the early postoperative phase (HR, 3.64; 95% CI, 2.05-6.47).
“Presence of a genetic abnormality was associated with increased risk of death both in the early (HR, 3.64; 95% CI, 2.05-6.47)”
Quote in the paperSubstantial claim - Findingown data
After covariate adjustment, non-valve-sparing approach was associated with increased early mortality (HR, 3.76; 95% CI, 1.53-9.91).
“and non-valve-sparing operation (HR, 3.76; 95% CI, 1.53-9.19).”
- Also stated in: § Factors Associated With Long-term Mortality · plain
“non-valve-sparing approach (HR, 3.76; 95% CI, 1.53-9.91)”
Quote in the paperSubstantial claim - Also stated in: § Factors Associated With Long-term Mortality · plain
- Findingown data
There were 3894 total patients with simple TOF who underwent repair and met inclusion criteria.
“There were 3894 total patients with simple TOF who underwent repair and met inclusion criteria.”
Quote in the paperSubstantial claim - Findingown data
There were 161 in-hospital deaths following surgery: 47 in the staged group and 114 in the primary repair group.
“There were 161 in-hospital deaths following surgery: 47 in the staged group and 114 in the primary repair group.”
Quote in the paperNot a substantial claim - Findingown data
Coexistence of a genetic anomaly remains a risk factor for mortality in both early and late postoperative phases.
“Not surprisingly, coexistence of a genetic anomaly remains a risk factor for mortality in both early and late postoperative phases.”
Quote in the paperSubstantial claim - Findingown data
After adjustment for covariates, these factors remained significantly associated with increased risk of perioperative death.
“After adjustment covariates, these factors remained significantly associated with increased risk of perioperative death (eTable 2 in the Supplement).”
Quote in the paperSubstantial claim - Findingown data
The overall hazard of mortality or transplant in patients with simple TOF following repair showed an early steep hazard followed by a late, increasing hazard.
“The overall hazard of mortality or transplant in patients with simple TOF following repair showed an early steep hazard followed by a late, increasing hazard.”
Quote in the paperSubstantial claim - Findingown data
Most deaths among those surviving TOF repair were directly (n = 63 [43.5%]) or indirectly (n = 76 [52.4%]) attributed to TOF.
“Most deaths among those surviving TOF repair were directly (n = 63 [43.5%]) or indirectly (n = 76 [52.4%]) attributed to TOF (Table 3)”
Quote in the paperNot a substantial claim - Findingown data
Staged repair and non-valve-sparing repair were associated with a rapid decrease in survival in the first 2 years following repair.
“Stratified transplant-free survival patterns (Figure 2A-F) demonstrated a rapid decrease in survival in the first 2 years following repair for those undergoing staged repair and non-valve-sparing repair”
Quote in the paperSubstantial claim - Findingreasoning
The early postdischarge period had a high hazard that decreases rapidly before reaching a minimum at 6 years following discharge.
“(1) an early postdischarge period with high hazard that decreases rapidly before reaching a minimum at 6 years following discharge”
Quote in the paperSubstantial claim - Findingown data
Following repair, 3733 were discharged alive.
“Following repair, 3733 were discharged alive.”
Quote in the paperNot a substantial claim - Findingown data
Long-term transplant-free survival beyond the first 6 years following repair is excellent.
“Long-term transplant-free survival beyond the first 6 years following repair is excellent and influenced primarily by the coexistence of an associated genetic condition.”
Quote in the paperSubstantial claim - Findingown data
Staged strategy is associated with increased risk of early death/transplant (HR 2.68, 95% CI 1.59-4.49, P<.001).
“Staged strategy | 2.68 (1.59-4.49) | <.001”
Quote in the paperSubstantial claim - Findingown data
Genetic condition is associated with increased risk of early death/transplant (HR 3.64, 95% CI 2.05-6.47, P<.001).
“Genetic condition | 3.64 (2.05-6.47) | <.001”
Quote in the paperSubstantial claim - Findingown data
Genetic condition is associated with increased risk of late death/transplant (HR 4.41, 95% CI 2.62-7.44, P<.001).
“Genetic condition | 4.41 (2.62-7.44) | <.001”
Quote in the paperSubstantial claim - Findingown data
Non-valve-sparing operation is associated with increased risk of early death/transplant (HR 3.76, 95% CI 1.53-9.19, P=.004).
“Non-valve-sparing operation | 3.76 (1.53-9.19) | .004”
Quote in the paperSubstantial claim - Findingown data
Male sex is associated with increased risk of late death/transplant (HR 1.56, 95% CI 0.96-2.56, P=.08).
“Male | 1.56 (0.96-2.56) | .08”
Quote in the paperNot a substantial claim - Findingown data
Era 1982-1989 is associated with increased risk of early death/transplant compared to 2000 and beyond (HR 2.31, 95% CI 1.06-5.03, P=.04).
“1982-1989 | 2.31 (1.06-5.03) | .04”
Quote in the paperSubstantial claim - Findingown data
Congenital heart disease was the underlying cause of death in 63 (43.45%) of late deaths.
“Congenital heart disease | 63 (43.45)”
Quote in the paperNot a substantial claim - Findingown data
Most deaths after TOF repair were directly or indirectly associated with TOF and were reported as sudden death, arrhythmia, and congestive heart failure.
“Most deaths after TOF repair were directly or indirectly associated with TOF and were reported as sudden death, arrhythmia, and congestive heart failure.”
- Also stated in: § Conclusions · plain
“Within the 25-year follow-up period, most deaths were associated with the underlying diagnosis of TOF and mediated by arrhythmias and congestive heart failure.”
Quote in the paperSubstantial claim - Also stated in: § Conclusions · plain
- Findingown data
Long-term transplant-free survival beyond the first 6 years following repair is influenced primarily by the coexistence of an associated genetic condition.
“Long-term transplant-free survival beyond the first 6 years following repair is excellent and influenced primarily by the coexistence of an associated genetic condition.”
Quote in the paperSubstantial claim - Study design and conductown data
There were no significant differences in the clinical characteristics of patients with TOF with and without identifiers.
“There were no significant differences in the clinical characteristics of patients with TOF with and without identifiers (data not shown).”
Quote in the paperSubstantial claim - Premisereasoning
Because most of these studies are single-center, they are not suitable to address the issue of center effect.
“Because most of these studies are single-center, they are not suitable to address the issue of center effect.”
Quote in the paperSubstantial claim - Premisecitation
Mortality differences between staged and primary repair strategies have been inconsistent among multiple studies supporting each strategy.
“Mortality differences between staged and primary repair strategies have been inconsistent among multiple studies supporting each strategy.18,[20][21][22]”
Quote in the paperSubstantial claim0 of 1 sources checked- cites 18,[20][21][22]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
- Interpretationreasoning
These findings suggest that residual morbidity remains a major factor affecting survival of these individuals despite improvements in acute and midterm treatment.
“These findings suggest that residual morbidity remains a major factor affecting survival of these individuals despite improvements in acute and midterm treatment.”
Quote in the paperSubstantial claim - Interpretationreasoning
At least some of the residual morbidity can be explained by coexisting genetic abnormalities.
“This indicates that at least some of the residual morbidity can be explained by coexisting genetic abnormalities.”
Quote in the paperSubstantial claim - Premisecitation
Reported midterm survival (97% at 7 years of postoperative follow-up) with infundibulum-preserving mini-incision strategy is similar to the outcomes in our study.
“Reported midterm survival (97% at 7 years of postoperative follow-up) with this strategy is similar to the outcomes in our study”
Quote in the paperNot a substantial claim0 of 2 sources checked- cites [25] 25. McKenzie ED, Maskatia SA, Mery C. Surgical management of tetralogy of fallot: in defense of the infundibulum. Semin Thorac Cardiovasc Surg. 2013; 25(3):206-212. doi:10.1053/j.semtcvs.2013.10.003 source
- cites [26] 26. Morales DL, Zafar F, Heinle JS, et al. Right ventricular infundibulum sparing (RVIS) tetralogy of fallot repair: a review of over 300 patients. Ann Surg. 2009;250(4):611-617. source
- Premisecitation
Attributed COD based on NDI data have been shown to correlate well to other methods of death ascertainment including case-by-case review by nosologists or review with state-level death records with discordance rates less than 10%.
“Nonetheless, attributed COD based on NDI data have been shown to correlate well to other methods of death ascertainment including case-by-case review by nosologists or review with state-level death records with discordance rates less than 10%.28,29”
Quote in the paperSubstantial claim0 of 2 sources checked- cites [28] 28. Doody MM, Hayes HM, Bilgrad R. Comparability of national death index plus and standard procedures for determining causes of death in epidemiologic studies. Ann Epidemiol. 2001;11(1):46-50. doi:10.1016/S1047-2797(00) 00177-0 source
- cites [29] 29. Sathiakumar N, Delzell E, Abdalla O. Using the National Death Index to obtain underlying cause of death codes. J Occup Environ Med. 1998;40(9): 808-813. doi:10.1097/00043764-199809000- 00010 source
- Study design and conductnone given
The PCCC registry lacks information regarding potentially important contributors to long-term mortality such as presurgical variables affecting choice of surgical strategy, frequency and type of subsequent procedures, medications used, and socioeconomic variables.
“The PCCC registry lacks information regarding potentially important contributors to long-term mortality such as presurgical variables affecting choice of surgical strategy, frequency and type of subsequent procedures, medications used, and socioeconomic variables.”
Quote in the paperNot a substantial claim
- May combine two claims. 24
- Classified as probably not a substantial claim. 5
May combine two claims.
Interpretationown dataEarlier surgical eras had an inverse association with the chances of survival in the early postoperative phase, indicating higher residual postoperative morbidity, which is consistent with previous reports.
“Earlier surgical eras had an inverse association with the chances of survival in the early postoperative phase, indicating higher residual postoperative morbidity, which is consistent with previous reports.8,9,17,19”
Quote in the paperSubstantial claim1 of 4 sources checked- cites [8] 8. Hickey EJ, Veldtman G, Bradley TJ, et al. Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades. Eur J Cardiothorac Surg. 2009;35(1):156- 164. doi:10.1016/j.ejcts.2008.06.050 source
- cites [9] 9. Nollert G, Fischlein T, Bouterwek S, Böhmer C, Klinner W, Reichart B. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol. 1997;30(5):1374- 1383. doi:10.1016/S0735-1097(97)00318-5 source
In “Survival according to date of operation” (page unavailable), the paper says operative and 1-year mortality were exactly the same before and after 1970. It reports improved long-term survival in the later era, not an early postoperative survival disadvantage in the earlier era; the passage does not establish higher residual postoperative morbidity. (LLM, conf=0.90)
Checked by a reasoning model — may be wrong.
Experimental — accuracy not yet measured - cites [17] 17. Cuypers JA, Menting ME, Konings EE, et al. Unnatural history of tetralogy of Fallot: prospective follow-up of 40 years after surgical correction. Circulation. 2014;130(22):1944-1953. doi:10.1161/ CIRCULATIONAHA.114.009454 source
- cites [19] 19. Kim H, Sung SC, Kim SH, et al. Early and late outcomes of total repair of tetralogy of Fallot: risk factors for late right ventricular dilatation. Interact Cardiovasc Thorac Surg. 2013;17(6):956-962. doi: 10.1093/icvts/ivt361 source
May combine two claims.
PremisecitationPrior studies detected no significant difference in long-term survival between patients treated with transannular patch (TAP) vs those with valve-sparing operation despite less valve replacements in the valve-sparing group.
“In regards to the use of valve vs nonvalve-sparing approach, prior studies detected no significant difference in long-term survival between patients treated with transannular patch (TAP) vs those with valve-sparing operation despite less valve replacements in the valve-sparing group.23,24”
Quote in the paperSubstantial claim1 of 2 sources checked- cites [23] 23. Kim GS, Han S, Yun TJ. Pulmonary annulus preservation lowers the risk of late postoperative pulmonary valve implantation after the repair of tetralogy of Fallot. Pediatr Cardiol. 2015;36(2):402- 408. doi:10.1007/s00246-014-1021-4 source
- cites [24] 24. Luijten LW, van den Bosch E, Duppen N, et al. Long-term outcomes of transatrial-transpulmonary repair of tetralogy of Fallot. Eur J Cardiothorac Surg. 2015;47(3):527-534. doi:10.1093/ejcts/ezu182 source
May combine two claims.
InterpretationcitationThe difference in COD between our cohort and the Taiwanese study may be associated with local factors and the relatively small number of patients and death events in the Taiwanese study, limiting generalization for reported COD in categories with relatively rare events.
“This difference may be associated with local factors and the relatively small number of patients and death events in the Taiwanese study, limiting generalization for reported COD in categories with relatively rare events.10”
Quote in the paperNot a substantial claim1 of 1 sources checked- cites [10] 10. Chiu SN, Wang JK, Chen HC, et al. Long-term survival and unnatural deaths of patients with repaired tetralogy of Fallot in an Asian cohort. Circ Cardiovasc Qual Outcomes. 2012;5(1):120-125. doi: 10.1161/CIRCOUTCOMES.111.963603 source
Split into 2 atoms — 0 confirmed, 2 partially supported, 0 disputed, 0 misattributed, 0 unverifiable. Expand the claim to see per-atom verdicts.
Experimental — accuracy not yet measured
May combine two claims.
Findingown dataThe most frequent causes of death outside CHD/CVD were external causes (n = 16 [11%]), other medical causes (n = 19 [13.1%]), other congenital anomalies (n = 10 [6.9%]), and neoplasms (n = 5 [3.5%]).
“The most frequent CODs outside CHD/CVD were external causes (n = 16 [11%]), other medical causes (n = 19 [13.1%]), other congenital anomalies (n = 10 [6.9%]), and neoplasms (n = 5 [3.5%]).”
Quote in the paperNot a substantial claimMay combine two claims.
Findingown dataCardiovascular disease, without reference to TOF, accounted for 15.2% of deaths (n = 22), while 41.4% (n = 60) were assigned to a non-CHD/non-CVD condition.
“Cardiovascular disease, without reference to TOF, accounted for 15.2% of deaths (n = 22), while 41.4% (n = 60) were assigned to a non-CHD/non-CVD condition.”
Quote in the paperNot a substantial claimMay combine two claims.
Findingown dataThree thousand one hundred sixty-eight underwent primary repair, while 726 had a staged approach with initial shunt followed by repair.
“Three thousand one hundred sixty-eight underwent primary repair, while 726 had a staged approach with initial shunt followed by repair.”
Quote in the paperNot a substantial claimMay combine two claims.
Findingown dataIn-hospital mortality following TOF repair occurred more commonly in infants, patients weighing less than 2.5 kg at repair, patients with prior shunt palliation, in earlier surgical eras (1982-1989 and 1999-2000), and concurrent genetic syndrome.
“In-hospital mortality following TOF repair was 4.1% and occurred more commonly in infants, patients weighing less than 2.5 kg (proxy for prematurity) at repair, patients with prior shunt palliation, in earlier surgical eras (1982-1989 and 1999-2000), and concurrent genetic syndrome (Table 1).”
Quote in the paperSubstantial claimMay combine two claims.
Findingown dataOver a median follow-up period of 18.5 years (maximum, 33 years; IQR, 14.6-22.4 years), 145 deaths (3 following transplant) and 5 transplants occurred.
“Over a median follow-up period of 18.5 years (maximum, 33 years; IQR, 14.6-22.4years), 145 deaths (3 following transplant) and 5 transplants occurred.”
Quote in the paperSubstantial claimMay combine two claims.
Findingown dataA more rapid early mortality was noted for those who underwent repair in the earliest era, but long-term survival was similar across all eras.
“A more rapid early mortality was noted for those who underwent repair in the earliest era, but long-term survival was similar across all eras (Figure 2F).”
Quote in the paperSubstantial claimMay combine two claims.
Findingown dataThere is a trend toward increased mortality in the subgroup undergoing right ventricle-pulmonary artery conduit placement, at a time when procedures for an outgrown conduit are expected to take place, but the relatively small number of this group limits conclusions.
“There is also a trend toward increased mortality in the subgroup undergoing right ventricle-pulmonary artery conduit placement, at a time when procedures for an outgrown conduit are expected to take place, but the relatively small number of this group limits conclusions.”
Quote in the paperNot a substantial claimMay combine two claims.
Findingown dataDuring the study period, there was a trend toward earlier primary repair (aged <1 year), but otherwise, there was no significant difference in the use of staged vs primary repair or between valve-sparing vs non-valve-sparing strategies.
“During the study period, there was a trend toward earlier primary repair (aged <1 year), but otherwise, there was no significant difference in the use of staged vs primary repair or between valve-sparing vs non-valve-sparing strategies (eTable 1 in the Supplement).”
Quote in the paperNot a substantial claimMay combine two claims.
Findingown dataOf the 3733 discharged alive, 3283 patients had direct identifiers available and compose the cohort submitted to the NDI/OPTN, while 450 had insufficient identifiers and were excluded.
“Of these, 3283 patients had direct identifiers available and compose the cohort submitted to the NDI/OPTN, while 450 had insufficient identifiers and were excluded.”
Quote in the paperSubstantial claimMay combine two claims.
Findingown dataThe subgroup repaired with a right ventricle-pulmonary artery conduit (n = 88) experienced a higher number of deaths 2 to 4 years after TOF repair compared with the other non-valve-sparing patients, but the number of events is too small to detect a significant difference in long-term survival.
“This group experienced a higher number of deaths 2 to 4 years after TOF repair compared with the other non-valve-sparing patients, but the number of events is too small to detect a significant difference in long-term survival (eFigure 2 in the Supplement).”
Quote in the paperNot a substantial claimMay combine two claims.
Findingown dataIn the PCCC cohort, the late-phase survival and cause of death have not substantially changed over time.
“In the PCCC cohort, the late-phase survival and COD have not substantially changed over time.”
Quote in the paperSubstantial claimMay combine two claims.
Findingown dataThe trough of the hazard function is around 6 years followed by a slow steady increase throughout the follow-up period.
“Insert: zoomed view of the hazard function that shows the trough of the hazard around 6 years followed by a slow steady increase throughout the follow-up period.”
Quote in the paperNot a substantial claimMay combine two claims.
Findingown dataThe hazard of mortality/transplant following discharge from definitive TOF repair shows an early peaking hazard that rapidly decreases during the first year and reaches a minimum at 4 years following discharge followed by an increasing late hazard period through 30 years following TOF repair.
“Hazard function shows an early peaking hazard that rapidly decreases during the first year and reaches a minimum at 4 years following discharge followed by an increasing late hazard period through 30 years following TOF repair.”
Quote in the paperSubstantial claimMay combine two claims.
Interpretationown dataPatients with repaired TOF have a biphasic hazard for mortality, an early steep hazard during the first 4 years after TOF repair, and a late hazard with a slow increase in mortality after 6 years and acceleration 20 years later.
“Patients with repaired TOF have a biphasic hazard for mortality, an early steep hazard during the first 4 years after TOF repair, and a late hazard with a slow increase in mortality after 6 years and acceleration 20 years later.”
Quote in the paperSubstantial claimMay combine two claims.
Interpretationown dataStaged repair and non-valve-sparing operation were both associated with increased early-phase hazard for death following repair, ultimately translating to worsened long-term survival.
“Staged repair and non-valve-sparing operation were both associated with increased early-phase hazard for death following repair, ultimately translating to worsened long-term survival.”
Quote in the paperSubstantial claim- Rests on an assumption: Non-valve-sparing operation exposes patients to a higher likelihood of subsequent reoperation and perioperative complications.
May combine two claims.
Interpretationown dataSurvival in the PCCC cohort exceeds 94% at 25 years after TOF repair, which is comparable with single-center US and international registry studies reporting 30-year survival between 80% and 98%.
“Survival in the PCCC cohort exceeds 94% at 25 years after TOF repair, which is comparable with single-center US and international registry studies reporting 30-year survival between 80% and 98%.[7][8][9]17”
Quote in the paperSubstantial claim0 of 1 sources checked- cites [7][8][9]17
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
May combine two claims.
Study design and conductreasoningThe large number of participating centers in the PCCC allows us to leverage center variation in the TOF surgical strategy and compare the effectiveness of each strategy in a quasiexperimental design based on center and regional variation.
“The large number of participating centers in the PCCC allows us to leverage center variation in the TOF surgical strategy and compare the effectiveness of each strategy in a quasiexperimental design based on center and regional variation.”
Quote in the paperSubstantial claimProbably not a substantial claim: may not pass S3 (load-bearing).
InterpretationcitationIt is possible that some of the deaths in the TOF population may have been misclassified.
“Extrapolating to our study, it is possible that some of the deaths in the TOF population may have been misclassified.”
Quote in the paperNot a substantial claimClassified as probably not a substantial claim. May combine two claims.
Study design and conductnone givenThe primary strengths of our study are the large number of patients and participating centers as well as the length of follow-up.
“The primary strengths of our study are the large number of patients and participating centers as well as the length of follow-up.”
Quote in the paperSubstantial claimMay combine two claims.
Interpretationown dataThe data highlight the shortcomings of early repair, staged, and non-valve-sparing approaches in the early period following repair.
“The data highlight the shortcomings of early repair, staged, and non-valve-sparing approaches in the early period following repair.”
Quote in the paperSubstantial claimProbably not a substantial claim: may not pass S3 (load-bearing).
Study design and conductnone givenThere is limited information regarding the status of the RVOT following surgery.
“In addition, there is limited information regarding the status of the RVOT following surgery.”
Quote in the paperNot a substantial claimClassified as probably not a substantial claim. May combine two claims.
Study design and conductnone givenType of surgical RVOT reconstruction and valve approach were assigned by the respective PCCC surgical codes; manual review of operative notes was performed when ambiguity existed.
“Type of surgical RVOT reconstruction and valve approach were assigned by the respective PCCC surgical codes; manual review of operative notes was performed when ambiguity existed.”
Quote in the paperSubstantial claimClassified as probably not a substantial claim. May combine two claims.
PremisecitationThe Pediatric Cardiac Care Consortium (PCCC) is the oldest and one of the largest clinical registries for interventions for congenital heart disease in the United States.
“The Pediatric Cardiac Care Consortium (PCCC) is the oldest and one of the largest clinical registries for interventions for congenital heart disease in the United States.[11][12][13]”
Quote in the paperSubstantial claim0 of 1 sources checked- cites [11][12][13]
Couldn’t be matched to a paper in Crossref, so it can’t be checked.
The paper relies on this without support.
Non-valve-sparing operation exposes patients to a higher likelihood of subsequent reoperation and perioperative complications.
“Patients with non-valve-sparing approach are exposed to higher likelihood for subsequent reoperation and perioperative complications.”
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Studies based on multicenter surgical registries found increased survival and decreased reintervention rates among patients who underwent a VS procedure compared with those who underwent TAP.
“The risk-benefit balance of VS procedures compared with TAP remains unclear in this setting even if studies3,16,17,18,19,20 based on multicenter surgical registries found increased survival and decreased reintervention rates among patients who underwent a VS procedure compared with those who underwent TAP.”
From Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot (2021) · cites this paper as [3]
The Factors Associated With Long-term Mortality section (page not provided) reports higher early mortality with non-valve-sparing than valve-sparing repair, supporting a survival advantage for valve-sparing procedures in this multicenter registry. The Patterns of Long-term Survival section says non-valve-sparing patients have a higher likelihood of subsequent reoperation, but the Strengths and Limitations section says the registry lacks data on the frequency of subsequent procedures. Thus, this paper does not establish decreased reintervention rates, and its measured survival comparison is not specifically valve-sparing versus TAP. (LLM, conf=0.85)
Experimental — accuracy not yet measuredSmith et al found that patients with TAP had a 3.5-fold increased risk of undergoing a PVR compared with patients who did not undergo TAP.
“Smith et al3 found that patients with TAP had a 3.5-fold increased risk of undergoing a PVR compared with patients who did not undergo TAP.”
From Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot (2021) · cites this paper as [3]
In “Patterns of Long-term Survival After TOF Repair” (page unavailable), Smith et al. report that prior studies found fewer valve replacements after valve-sparing repair than after transannular patch repair. Our copy does not report a 3.5-fold risk of pulmonary valve replacement or present that comparison as a finding of Smith et al.’s own study. (LLM, conf=0.85)
Experimental — accuracy not yet measuredThe 2019 Pediatric Cardiac Care Consortium study reported an overall survival of 94.5% and a 3.8-fold increased risk of mortality for the non-VS group.
“The 2019 Pediatric Cardiac Care Consortium study3 reported an overall survival of 94.5% and a 3.8-fold increased risk of mortality for the non-VS group.”
From Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot (2021) · cites this paper as [3]
The Long-term Survival After Complete TOF Repair section reports 94.5% transplant-free survival at 25 years among patients discharged alive after repair—not an unqualified overall survival rate. Factors Associated With Long-term Mortality reports an adjusted hazard ratio of 3.76 for non-valve-sparing versus valve-sparing repair, but only in the early period, less than six years after repair. (LLM, conf=0.88)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
Each contradiction between two passages listed here was confirmed by an independent check.
Time of minimum hazard differs across descriptions
MinorThe Figure 1 caption places the minimum hazard at 4 years after discharge, while the results text places it at 6 years.
“Hazard function shows an early peaking hazard that rapidly decreases during the first year and reaches a minimum at 4 years following discharge”
“an early postdischarge period with high hazard that decreases rapidly before reaching a minimum at 6 years following discharge”
Survival advantage is assigned to the higher-risk approaches
MinorThe results sentence says staged and non-valve-sparing repair produce a long-term survival advantage over primary and valve-sparing repair. Elsewhere, the paper says those same approaches increase early mortality and worsen long-term survival.
“Stratified transplant-free survival patterns (Figure 2A-F) demonstrated a rapid decrease in survival in the first 2 years following repair for those undergoing staged repair and non-valve-sparing repair, resulting in a long-term survival advantage over single-stage and valve-sparing approaches throughout the subsequent 20 years (Figure 2C and D, respectively).”
“Staged repair and non-valve-sparing operation were both associated with increased early-phase hazard for death following repair, ultimately translating to worsened long-term survival.”
Adjusted hazard-ratio confidence limit differs between text and table
MinorThe results text gives an upper 95% confidence limit of 9.91 for the adjusted non-valve-sparing hazard ratio, whereas both Table 2 and the abstract give 9.19.
“non-valve-sparing approach (HR, 3.76; 95% CI, 1.53-9.91)”
“| Non-valve-sparing operation | 3.76 (1.53-9.19) | .004 |”
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- Authors
- Smith, C. A., McCracken, C., Thomas, A. S., Spector, L. G., St Louis, J. D., Oster, M. E., Moller, J. H., & Kochilas, L.
- Affiliations
- Emory University (US)
- University of Minnesota (US)
- Department of Pediatric Surgery, University of Missouri, Kansas City School of Medicine
- Corresponding author
- Kochilas, Lazaros
- Venue
- JAMA Cardiology
- Publisher
- American Medical Association (AMA)
- Published
- 2019-01-01
- Volume
- 4
- Issue
- 1
- Pages
- 34
- ISSN
- 2380-6583 (ISSN-L)
- PMC
- PMC6439686
- OpenAlex
- W2904473257
- Cited by
- 176
- References
- 28
- Citations by year
- 2026: 22 · 2025: 28 · 2024: 29 · 2023: 26 · 2022: 26 · 2021: 24 · 2020: 13 · 2019: 6 · 2018: 1 · 2012: 1
- FWCI
- 8.57
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- JAMA Cardiology (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Heart, Lung, and Blood Institute (R01HL122392)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiovascular Conditions and Treatments
- Keywords
- tetralogy of Fallot, long-term survival, transplant-free survival, cyanotic congenital heart disease, surgical repair, staged repair, genetic abnormalities, mortality risk factors, pediatric cardiac surgery
- MeSH terms
- Adolescent, Cardiac Care Facilities, Cause of Death, Child, Child, Preschool, Female, Humans, Infant, Male, Pediatrics, Registries, Retrospective Studies, Risk Factors, Tetralogy of Fallot, United States, Cohort Studies, Survival Analysis, Treatment Outcome, Hospital Mortality, Young Adult
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