Abstract
Background
Heart failure (HF) is the primary cause of premature death in adult congenital heart
disease (ACHD). This study aimed to describe the impact of a HF diagnosis on short-term
prognosis and to investigate the added prognostic value of an HF diagnosis to the
ACHD Anatomic and Physiologic classification (ACHD-AP).
Methods
This study included 3995 patients followed in a tertiary care centre (last follow-up
after January 1, 2010). Survival curves were plotted, and predictors of the primary
end point (death, heart transplantation, or ventricular assist device [VAD]) were
identified with the use of Cox proportional hazard models and compared with the use
of Harrell’s C-statistic.
Results
Mean age at baseline was 35.7 ± 13.3 years. The prevalence of ACHD-HF was 6.4%. During
a median follow-up of 3.1 years (IQR 2.1-3.6 years), 27.3% of ACHD-HF patients reached
the primary end point, compared with 1.4% of ACHD patients without HF. Event-free
survivals were 78.3%, 61.9%, and 57.5% at 1, 3, and 5 years in ACHD-HF patients, compared
with 99.3%, 98.3%, and 98.0% in ACHD patients without HF (P < 0.001). An HF diagnosis (HR 6.9, 95% CI 4.3-11.2) and the physiologic classification
(HR 2.6, 95% CI 1.9-3.7) were independently associated with the primary end point.
The addition of HF to the ACHD-AP classification yielded a Harrell’s C-index of 0.8631,
providing a significant improvement over the ACHD-AP classification alone (P = 0.0003).
Conclusions
The risk of mortality, transplantation, or VAD is increased in ACHD-HF patients. An
HF diagnosis appears to be a valuable prognostic marker in addition to the ACHD-AP
classification.
Résumé
Contexte
Chez les patients adultes atteints d’une cardiopathie congénitale (CPC), l’insuffisance
cardiaque (IC) est la première cause de décès prématuré. Notre étude visait à décrire
les répercussions d’un diagnostic d’IC sur le pronostic à court terme et à examiner
la valeur pronostique qu’un tel diagnostic peut ajouter à celle de la classification
anatomique et physiologique de la CPC (CAP-CPC).
Méthodologie
L’étude comprenait 3995 patients ayant fait l’objet d’un suivi dans un centre de soins
tertiaires (dernier suivi effectué après le 1er janvier 2010). Les courbes de survie ont été tracées et des facteurs de prédiction
de la survenue d’un des événements du critère principal (décès, transplantation cardiaque
ou pose d’un dispositif d’assistance ventriculaire) ont été établis à l’aide de modèles
de Cox à risques proportionnels et comparés au moyen de la statistique C de Harrell.
Résultats
L’âge initial moyen était de 35,7 ± 13,3 ans. La prévalence d’une IC chez les patients
adultes atteints d’une CPC (CPC-IC) était de 6,4 %. Sur une période de suivi d’une
durée moyenne de 3,1 ans (intervalle interquartile : 2,1 à 3,6 ans), 27,3 % des patients
adultes présentant une CPC-IC avaient atteint le critère principal, comparativement
à 1,4 % des patients adultes atteints d’une CPC sans IC. Le taux de survie sans événement
s’établissait à 78,3 %, 61,9 % et 57,5 % à 1, 3 et 5 ans, respectivement, chez les
patients adultes atteints d’une CPC-IC, comparativement à 99,3 %, 98,3 % et 98,0 %
chez les patients adultes atteints d’une CPC sans IC (p < 0,001). Un diagnostic d’IC (rapport des risques instantanés [RRI] : 6,9; intervalle
de confiance [IC] à 95 % : 4,3 à 11,2) et la classification physiologique (RRI : 2,6;
IC à 95 % : 1,9 à 3,7) étaient associés, de façon indépendante, au critère principal.
L’ajout de l’IC à la CAP-CPC a donné un indice C de Harrell de 0,8631, ce qui constitue
une amélioration significative par rapport à la CAP-CPC seule (p = 0,0003).
Conclusion
Le risque de mortalité et la nécessité d’une transplantation ou d’un dispositif d’assistance
ventriculaire sont accrus chez les patients adultes atteints d’une CPC-IC. Il semble
donc qu’un diagnostic d’IC soit un marqueur pronostique important, en plus de la CAP-CPC.
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Article info
Publication history
Published online: December 26, 2022
Accepted:
December 19,
2022
Received:
August 18,
2022
Publication stage
In Press Journal Pre-ProofFootnotes
See page 9 for disclosure information.
Identification
Copyright
© 2022 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
