Abstract
As life expectancy in patients with congenital heart disease (CHD) has improved, the
risk for developing noncardiac morbidities is increasing in adult patients with CHD
(ACHD). Among these noncardiac complications, malignancies significantly contribute
to the disease burden of ACHD patients. Epidemiologic studies of cancer risk in CHD
patients are challenging because they require large numbers of patients, extended
follow-up, detailed and validated clinical data, and appropriate reference populations.
However, several observational studies suggest that cancer risks are significantly
elevated in patients with CHD compared with the general population. CHD and cancer
share genetic and environmental risk factors. An association with exposure to low-dose
ionizing radiation secondary to medical therapeutic or diagnostic procedures has been
reported. Patients with Down syndrome, as well as, to a lesser extent, deletion of
22q11.2 and renin-angiotensin system pathologies, may manifest both CHD and a predisposition
to cancer. Such observations suggest that carcinogenesis and CHD may share a common
basis in some cases. Finally, specific conditions, such as Fontan circulation and
cyanotic CHD, may lead to multisystem consequences and subsequently to cancer. Nonetheless,
there is currently no clear consensus regarding appropriate screening for cancer and
surveillance modalities in CHD patients. Physicians caring for patients with CHD should
be aware of this potential predisposition and meet screening recommendations for the
general population fastidiously. An interdisciplinary and global approach is required
to bridge the knowledge gap in this field.
Résumé
Comme l’espérance de vie des patients atteints d’une cardiopathie congénitale augmente,
le risque d’apparition de maladies non cardiaques est aussi en hausse chez les adultes
qui sont atteints de ce type de cardiopathie. Parmi les complications non cardiaques,
les affections malignes contribuent fortement au fardeau de la maladie dans cette
population. La réalisation d’études épidémiologies sur le risque de cancer chez les
patients atteints d’une cardiopathie congénitale présente des défis, car de telles
études exigent un grand nombre de sujets qu’il faut suivre sur une longue période,
des données cliniques détaillées et validées et des populations de référence appropriées.
Malgré tout, plusieurs études observationnelles semblent indiquer que le risque de
cancer est considérablement élevé chez les patients atteints d’une cardiopathie congénitale
comparativement à la population générale. De fait, les facteurs de risque génétiques
et environnementaux sont les mêmes pour la cardiopathie congénitale et pour le cancer.
Une association avec l’exposition à de faibles doses de rayonnements ionisants secondaire
aux démarches thérapeutiques ou diagnostiques a été rapportée. Les patients atteints
du syndrome de Down ainsi que, dans une moindre mesure, ceux qui présentent une délétion
22q11.2 ou dont le système rénine-angiotensine est atteint, sont susceptibles d’avoir
à la fois une cardiopathie congénitale et une prédisposition au cancer. De telles
observations semblent indiquer que la carcinogenèse et la cardiopathie congénitale
pourraient dans certains cas avoir des causes communes. Enfin, certaines conditions,
comme la présence d’un circuit de Fontan et une cardiopathie congénitale cyanogène,
pourraient avoir des effets sur plusieurs organes et finir par provoquer l’apparition
d’un cancer. Il n’en demeure pas moins qu’il n’existe à l’heure actuelle aucun consensus
clair quant aux stratégies appropriées de dépistage du cancer et aux modalités de
surveillance des patients atteints de cardiopathie congénitale. Les médecins qui traitent
des patients atteints de cardiopathie congénitale doivent être au fait de cette prédisposition
potentielle et suivre à la lettre les recommandations en matière de dépistage s’appliquant
à la population générale. Une approche interdisciplinaire et globale s’impose afin
de combler les lacunes des connaissances dans ce domaine.
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Article info
Publication history
Published online: October 09, 2019
Accepted:
September 17,
2019
Received:
May 21,
2019
Footnotes
See page 1758 for disclosure information.
Identification
Copyright
© 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
