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Canadian Journal of Cardiology

Short-term Prognostic Value of Heart Failure Diagnosis in a Contemporary Cohort of Patients With Adult Congenital Heart Disease

Published:December 26, 2022DOI:https://doi.org/10.1016/j.cjca.2022.12.018

      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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