The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions.
Can J Cardiol
; 37(6): 877-886, 2021 06.
Article
en En
| MEDLINE
| ID: mdl-33059007
ABSTRACT
BACKGROUND:
The reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions.METHODS:
The TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Québec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions.RESULTS:
We included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival (< 85% for cTOF and < 60% for TOF-PA) but similar numbers of interventions and hospitalisations.CONCLUSIONS:
The anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Tetralogía de Fallot
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Síndrome de Down
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Atresia Pulmonar
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Síndrome de Deleción 22q11
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Procedimientos Quirúrgicos Cardíacos
Tipo de estudio:
Etiology_studies
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Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Adolescent
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Adult
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Child, preschool
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Female
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Humans
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Male
País/Región como asunto:
America do norte
Idioma:
En
Revista:
Can J Cardiol
Asunto de la revista:
CARDIOLOGIA
Año:
2021
Tipo del documento:
Article
País de afiliación:
Canadá