Prevalence, predictors, and mortality of Super-Fontan patients in a single-centre nationwide cohort
Jazyk angličtina Země Anglie, Velká Británie Médium print-electronic
Typ dokumentu časopisecké články
PubMed
39871467
DOI
10.1017/s1047951125000228
PII: S1047951125000228
Knihovny.cz E-zdroje
- Klíčová slova
- Exercise tolerance, Fontan circulation, Super-Fontan, peak oxygen consumption, total cavopulmonary connection,
- MeSH
- dítě MeSH
- Fontanova operace * MeSH
- lidé MeSH
- míra přežití trendy MeSH
- mladiství MeSH
- prevalence MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- trikuspidální atrézie chirurgie mortalita MeSH
- vrozené srdeční vady * chirurgie mortalita MeSH
- zátěžový test * MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
AIMS: To evaluate the prevalence, long-term mortality, and clinical characteristics in total cavopulmonary connection patients with excellent functional outcomes. METHODS AND RESULTS: A retrospective study of cardiopulmonary exercise test results in 288 patients after total cavopulmonary connection from a single-centre nationwide database. A subgroup of 88 (30.6%) patients (45 women; 51.1%), at a median age 13.0 [interquartile range 11.0; 18.0] years achieved ≥80% of predicted VO2peak value (Super-Fontan phenotype). Survival free from death or heart transplantation 20 years after surgery was 100.0% in the Super-Fontan group versus 94.0% in the rest of the cohort (p = 0.04). Super-Fontan patients were younger, had lower body mass index, lower regurgitant fraction on atrioventricular valve, and larger preoperative McGoon ratio than the rest of the cohort (p = 0.002, p < 0.0001, p = 0.004, and p = 0.04, respectively). Females and tricuspid atresia patients were significantly more prevalent in the Super-Fontan group than in the rest of the cohort (p = 0.02 for both). There was no difference regarding systemic ventricle morphology, fenestration presence, or ejection fraction of systemic ventricle between the Super-Fontan group and the rest of the total cavopulmonary connection cohort (p = 0.06, p = 0.09, and p = 0.64, respectively). CONCLUSIONS: The subgroup of Super-Fontan patients has unique clinical characteristics when compared to the rest of the total cavopulmonary connection nationwide cohort. Besides superior VO2peak results, Super-Fontan patients tend to have lower long-term mortality, body mass index, and atrioventricular valve, greater preoperative pulmonary dimensions, and a higher prevalence of females and tricuspid atresia patients. There was no variance in morphology of the systemic ventricle, or presence of fenestration.
Children's Heart Centre Motol University Hospital Prague Czech Republic
Department of Rehabilitation and Sports Medicine Motol University Hospital Prague Czech Republic
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