Abstract
Background
There are few data on the prevalence and clinical consequences of coronary artery
aneurysms (CAAs) in adult patients with Marfan syndrome (MFS).
Methods
We performed a retrospective cohort study of 109 patients with pathogenic variants
in the FBN1 gene. Diameters of the left main coronary artery (LMCA) and right- coronary artery
(RCA) were measured by computed tomography angiography.
Results
The overall prevalence of CAA was 46%. The prevalence rates of CAA were 18% and 68%
in patients with a native aortic root (group 1) and patients with previous aortic-root
replacement (group 2), respectively. Previous aortic dissection or aortic intervention,
longer time from aortic-root replacement, higher systemic score, significant mitral
valve involvement, and diffuse aortic disease were correlated with CAA. During a mean
follow-up of 8.5 ± 7.6 years, 4 patients developed pseudoaneurysms of the coronary
anastomoses, requiring surgery.
Conclusions
CAAs are common in adult patients with MFS and are associated with a more severe aortic
phenotype and a longer follow-up after aortic-root replacement. Our study demonstrates
that coronary artery size should be regularly followed, mostly after aortic-root replacement
and in patients with severe aortic phenotypes. Large multicentre studies are warranted
to elucidate the most appropriate surveillance plan.
Résumé
Contexte
Rares sont les données sur la prévalence et les conséquences cliniques de l’anévrysme
de l’artère coronaire (AAC) chez les patients adultes atteints du syndrome de Marfan.
Méthodologie
Nous avons réalisé une étude de cohorte rétrospective sur 109 patients porteurs de
variants pathogènes du gène FBN1. Les diamètres de l’artère coronaire principale gauche (ACPG) et de l’artère coronaire
droite (ACD) ont été mesurés par angiographie par tomodensitométrie.
Résultats
La prévalence globale de l’AAC était de 46 %. Les taux de prévalence de l’AAC étaient
de 18 % et de 68 % chez les patients présentant une racine aortique native (groupe
1) et chez ceux ayant subi un remplacement de la racine aortique (groupe 2), respectivement.
Une corrélation a été observée entre l’AAC et une dissection ou une intervention aortique
antérieure, un temps écoulé plus long depuis le remplacement de la racine aortique,
un score systémique plus élevé, une atteinte importante de la valve mitrale et une
maladie aortique. Pendant le suivi d’une durée moyenne de 8,5 ± 7,6 ans, de faux anévrysmes
anastomotiques des coronaires nécessitant une intervention chirurgicale sont apparus
chez quatre patients.
Conclusions
Les AAC sont courants chez les patients adultes atteints du syndrome de Marfan et
sont associés à un phénotype aortique plus sévère et à une période de suivi plus longue
après le remplacement de la racine aortique. Notre étude démontre que le diamètre
de l’artère coronaire devrait faire l’objet d’un suivi régulier, en particulier après
le remplacement de la racine aortique et chez les patients présentant un phénotype
aortique sévère. Des études multicentriques de grande envergure permettraient de déterminer
les modalités de surveillance les plus appropriées.
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References
- Marfan syndrome: improved clinical history results in expanded natural history.Genet Med. 2019; 21: 1683-1690
- Redo aortic root operations in patients with Marfan syndrome.Int J Angiol. 2018; 27: 92-97
- Frequency of coronary ostial aneurysms after aortic root surgery in patients with the Marfan syndrome.Am J Cardiol. 2002; 89: 1135-1138
- Prevalence and natural history of coronary ostial aneurysms in Marfan patients.J Comput Assist Tomogr. 2019; 43: 115-118
- Multimodality imaging of diseases of the thoracic aorta in adults: from the American Society of Echocardiography and the European Association of Cardiovascular Imaging: endorsed by the Society of Cardiovascular Computed Tomography and Society for Cardiovascular Magnetic Resonance.J Am Soc Echocardiogr. 2015; 28: 119-182
- Lumen diameter of normal human coronary arteries. Influence of age, sex, anatomic variation, and left ventricular hypertrophy or dilation.Circulation. 1992; 86: 232-246
- Management of coronary artery aneurysms.JACC Cardiovasc Interv. 2018; 11: 1211-1223
- Gender differences in coronary artery diameter are not related to body habitus or left ventricular mass.Clin Cardiol. 2014; 37: 605-609
- Normal proximal coronary artery diameters in adults from India as assessed by computed tomography angiography.Clin Diagn Res. 2016; 10: TC10-TC13
- Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology.Genet Med. 2015; 17: 405-424
- Increased frequency of FBN1 truncating and splicing variants in Marfan syndrome patients with aortic events.Genet Med. 2015; 17: 177-187
- The physiological significance of coronary aneurysms in Kawasaki disease.EuroIntervention. 2011; 7: 944-947
- Evaluation of coronary flow velocity dynamics and flow reserve in patients with Kawasaki disease by means of a Doppler guide wire.J Am Coll Cardiol. 1998; 31: 833-840
- Comparison of flow patterns in ascending aortic aneurysms and volunteers using four-dimensional magnetic resonance velocity mapping.J Magn Reson Imaging. 2007; 26: 1471-1479
- Spontaneous coronary artery dissection and its association with heritable connective tissue disorders.Heart. 2016; 102: 876-881
Article info
Publication history
Published online: March 08, 2021
Accepted:
March 2,
2021
Received:
December 3,
2020
Footnotes
See page 1230 for disclosure information.
Identification
Copyright
© 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
