Long-term Outcomes of Tetralogy of Fallot: A Study from the Pediatric Cardiac Care Consortium

Clayton A. Smith, Courtney McCracken, Amanda S. Thomas, Logan G Spector, James D. St Louis, Matthew E. Oster, James H. Moller, Lazaros Kochilas

Research output: Contribution to journalArticle

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Abstract

Importance: Tetralogy of Fallot (TOF) is a surgically repairable form of cyanotic congenital heart disease. Multicenter data for long-term survival following repair are sparse. Objective: To evaluate the long-term transplant-free survival of TOF by surgical strategy adjusted for era and patient characteristics. Design, Setting, and Participants: Retrospective cohort study enriched with data from the National Death Index and the Organ Procurement and Transplantation Network through 2014. Multicenter cohort from the Pediatric Cardiac Care Consortium (PCCC), a large, US-based clinical registry for interventions for congenital heart disease. The cohort included patients with adequate identifiers for linkage with the National Death Index and the Organ Procurement and Transplantation Network who were enrolled in the PCCC registry between 1982 and 2003 and survived surgical repair of simple TOF. Data were analyzed between September 2015 and April 2018. Exposures: We examined patient-associated and surgery-associated risk factors affecting survival. Main Outcomes and Measures: We analyzed the transplant-free survival during early (<6 years) and late (≥6 years) phase after TOF surgical repair. Results: Of the 3283 patients who survived repair for simple TOF and met the study's inclusion criteria, 56.4% were male and 43.6% were female. Twenty-five-year survival following TOF repair was 94.5%. Multivariable analysis demonstrated increased risk of early mortality with staged repair (HR, 2.68; 95% CI, 1.59-4.49) and non-valve-sparing operation (HR, 3.76; 95% CI, 1.53-9.19). 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) and late postoperative phase (HR, 4.41; 95% CI, 2.62-7.44). Conclusions and Relevance: Long-term survival after simple TOF repair is excellent. Staged repair and non-valve-sparing operations were negatively associated with survival in the early postrepair phase but not the late postrepair phase. These data are important for patients with repaired TOF and their caretakers and may guide surgical strategies for optimizing the long-term outcomes of this population.

Original languageEnglish (US)
Pages (from-to)34-41
Number of pages8
JournalJAMA Cardiology
Volume4
Issue number1
DOIs
StatePublished - Jan 1 2019

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Tetralogy of Fallot
Pediatrics
Survival
Tissue and Organ Procurement
Organ Transplantation
Registries
Heart Diseases
Transplants
Cohort Studies
Retrospective Studies
Outcome Assessment (Health Care)
Mortality

PubMed: MeSH publication types

  • Journal Article

Cite this

Smith, C. A., McCracken, C., Thomas, A. S., Spector, L. G., St Louis, J. D., Oster, M. E., ... Kochilas, L. (2019). Long-term Outcomes of Tetralogy of Fallot: A Study from the Pediatric Cardiac Care Consortium. JAMA Cardiology, 4(1), 34-41. https://doi.org/10.1001/jamacardio.2018.4255

Long-term Outcomes of Tetralogy of Fallot : A Study from the Pediatric Cardiac Care Consortium. / Smith, Clayton A.; McCracken, Courtney; Thomas, Amanda S.; Spector, Logan G; St Louis, James D.; Oster, Matthew E.; Moller, James H.; Kochilas, Lazaros.

In: JAMA Cardiology, Vol. 4, No. 1, 01.01.2019, p. 34-41.

Research output: Contribution to journalArticle

Smith, CA, McCracken, C, Thomas, AS, Spector, LG, St Louis, JD, Oster, ME, Moller, JH & Kochilas, L 2019, 'Long-term Outcomes of Tetralogy of Fallot: A Study from the Pediatric Cardiac Care Consortium' JAMA Cardiology, vol. 4, no. 1, pp. 34-41. https://doi.org/10.1001/jamacardio.2018.4255
Smith, Clayton A. ; McCracken, Courtney ; Thomas, Amanda S. ; Spector, Logan G ; St Louis, James D. ; Oster, Matthew E. ; Moller, James H. ; Kochilas, Lazaros. / Long-term Outcomes of Tetralogy of Fallot : A Study from the Pediatric Cardiac Care Consortium. In: JAMA Cardiology. 2019 ; Vol. 4, No. 1. pp. 34-41.
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abstract = "Importance: Tetralogy of Fallot (TOF) is a surgically repairable form of cyanotic congenital heart disease. Multicenter data for long-term survival following repair are sparse. Objective: To evaluate the long-term transplant-free survival of TOF by surgical strategy adjusted for era and patient characteristics. Design, Setting, and Participants: Retrospective cohort study enriched with data from the National Death Index and the Organ Procurement and Transplantation Network through 2014. Multicenter cohort from the Pediatric Cardiac Care Consortium (PCCC), a large, US-based clinical registry for interventions for congenital heart disease. The cohort included patients with adequate identifiers for linkage with the National Death Index and the Organ Procurement and Transplantation Network who were enrolled in the PCCC registry between 1982 and 2003 and survived surgical repair of simple TOF. Data were analyzed between September 2015 and April 2018. Exposures: We examined patient-associated and surgery-associated risk factors affecting survival. Main Outcomes and Measures: We analyzed the transplant-free survival during early (<6 years) and late (≥6 years) phase after TOF surgical repair. Results: Of the 3283 patients who survived repair for simple TOF and met the study's inclusion criteria, 56.4{\%} were male and 43.6{\%} were female. Twenty-five-year survival following TOF repair was 94.5{\%}. Multivariable analysis demonstrated increased risk of early mortality with staged repair (HR, 2.68; 95{\%} CI, 1.59-4.49) and non-valve-sparing operation (HR, 3.76; 95{\%} CI, 1.53-9.19). 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) and late postoperative phase (HR, 4.41; 95{\%} CI, 2.62-7.44). Conclusions and Relevance: Long-term survival after simple TOF repair is excellent. Staged repair and non-valve-sparing operations were negatively associated with survival in the early postrepair phase but not the late postrepair phase. These data are important for patients with repaired TOF and their caretakers and may guide surgical strategies for optimizing the long-term outcomes of this population.",
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AU - Spector, Logan G

AU - St Louis, James D.

AU - Oster, Matthew E.

AU - Moller, James H.

AU - Kochilas, Lazaros

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N2 - Importance: Tetralogy of Fallot (TOF) is a surgically repairable form of cyanotic congenital heart disease. Multicenter data for long-term survival following repair are sparse. Objective: To evaluate the long-term transplant-free survival of TOF by surgical strategy adjusted for era and patient characteristics. Design, Setting, and Participants: Retrospective cohort study enriched with data from the National Death Index and the Organ Procurement and Transplantation Network through 2014. Multicenter cohort from the Pediatric Cardiac Care Consortium (PCCC), a large, US-based clinical registry for interventions for congenital heart disease. The cohort included patients with adequate identifiers for linkage with the National Death Index and the Organ Procurement and Transplantation Network who were enrolled in the PCCC registry between 1982 and 2003 and survived surgical repair of simple TOF. Data were analyzed between September 2015 and April 2018. Exposures: We examined patient-associated and surgery-associated risk factors affecting survival. Main Outcomes and Measures: We analyzed the transplant-free survival during early (<6 years) and late (≥6 years) phase after TOF surgical repair. Results: Of the 3283 patients who survived repair for simple TOF and met the study's inclusion criteria, 56.4% were male and 43.6% were female. Twenty-five-year survival following TOF repair was 94.5%. Multivariable analysis demonstrated increased risk of early mortality with staged repair (HR, 2.68; 95% CI, 1.59-4.49) and non-valve-sparing operation (HR, 3.76; 95% CI, 1.53-9.19). 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) and late postoperative phase (HR, 4.41; 95% CI, 2.62-7.44). Conclusions and Relevance: Long-term survival after simple TOF repair is excellent. Staged repair and non-valve-sparing operations were negatively associated with survival in the early postrepair phase but not the late postrepair phase. These data are important for patients with repaired TOF and their caretakers and may guide surgical strategies for optimizing the long-term outcomes of this population.

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