Aortic Branch Aneurysms and Vascular Risk in Patients With Marfan Syndrome
J Am Coll Cardiol . 2021 Jun 22;77(24):3005-3012. doi: 10.1016/j.jacc.2021.04.054.
Fecha de la publicación: 22/06/2021
Autor: Angela Lopez-Sainz (1), Laia Mila (2), Jose Rodriguez-Palomares (2), Javier Limeres (2), Chiara Granato (3), Lucia La Mura (4), Ana Sabaté (5), Andrea Guala (6), Laura Gutiérrez( 2), Laura Galian-Gay (2), Augusto Sao-Aviles (3), Sergi Bellmunt (7), Rafael Rodriguez (8), Hug Cuellar-Calabria (9), Albert Roque (9), Ignacio Ferreira-González (10), Artur Evangelista (11), Gisela Teixido-Tura (12)
Palabras clave: Aneurysm Aorta, Aortic branches, Marfan syndrome
PMID
- PMID: 34140103
- PMCID: PMC8091372
- DOI: 10.1016/j.jacc.2021.04.054
Affiliations
1Department of Cardiology, Vall d’Hebron Hospital Universitari, Barcelona, Spain; Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain.
2Department of Cardiology, Vall d’Hebron Hospital Universitari, Barcelona, Spain; Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain; CIBERCV Enfermedades Cardiovasculares, Instituto de Salud Carlos III, Madrid, Spain.
3Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain.
4Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain; Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy.
5Department of Pediatric Cardiology, Vall d’Hebron Hospital Universitari, Barcelona, Spain.
6Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain; CIBERCV Enfermedades Cardiovasculares, Instituto de Salud Carlos III, Madrid, Spain.
7Department of Angiology, Vascular and Endovascular Surgery, Vall d’Hebron Hospital Universitari, Barcelona, Spain.
8Department of Cardiac Surgery, Vall d’Hebron Hospital Universitari, Barcelona, Spain.
9Department of Radiology, IDI (Institut de Diagnòstic per la Imatge), Vall d’Hebron Hospital Universitari, Barcelona, Spain.
10Department of Cardiology, Vall d’Hebron Hospital Universitari, Barcelona, Spain; Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain; CIBERESP Epidemiología y Salud Pública, Instituto de Salud Carlos III, Madrid, Spain.
11Department of Cardiology, Vall d’Hebron Hospital Universitari, Barcelona, Spain; Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain; CIBERCV Enfermedades Cardiovasculares, Instituto de Salud Carlos III, Madrid, Spain. Electronic address: arturevangelistamasip@gmail.com.
12Department of Cardiology, Vall d’Hebron Hospital Universitari, Barcelona, Spain; Grupo de Enfermedades Cardiovasculares, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain; CIBERCV Enfermedades Cardiovasculares, Instituto de Salud Carlos III, Madrid, Spain. Electronic address: gteixido@vhebron.net.
Abstract
Background: Aortic branch aneurysms are not included in the diagnostic criteria for Marfan syndrome (MFS); however, their prevalence and eventual prognostic significance are unknown.
Objectives: The goal of this study was to assess the prevalence of aortic branch aneurysms in MFS and their relationship with aortic prognosis.
Methods: MFS patients with a pathogenic FBN1 genetic variant and at least one magnetic resonance or computed tomography angiography study assessing aortic branches were included. Aortic events and those related to aneurysm complications were recorded during follow-up.
Results: A total of 104 aneurysms were detected in 50 (26.7%) of the 187 patients with MFS (mean age 37.9 ± 14.4 years; 54% male) included in this study, with the iliac artery being the most common location (45 aneurysms). Thirty-one patients (62%) had >1 peripheral aneurysm, and surgery was performed in 5 (4.8%). Patients with aneurysms were older (41.9 ± 12.7 years vs. 36.7 ± 14.8 years; p = 0.040) and had more dilated aortic root (42.2 ± 6.4 mm vs. 38.8 ± 8.0 mm; p = 0.044) and dyslipidemia (31.0% vs. 9.7%; p = 0.001). In a subgroup of 95 patients with no previous aortic surgery or dissection followed up for 3.3 ± 2.6 years, the presence of arterial aneurysms was associated with a greater need for aortic surgery (hazard ratio: 3.4; 95% confidence interval: 1.1 to 10.3; p = 0.028) in a multivariable Cox analysis adjusted for age and aortic diameter.
Conclusions: Aortic branch aneurysms are present in one-quarter of patients with MFS and are related to age and aortic dilation, and they independently predict the need for aortic surgery. The systematic use of whole-body vascular assessment is recommended to identify other sites of vascular involvement at risk for complications and to define the subgroup of patients with more aggressive aortic disease.
Copyright © 2021. Published by Elsevier Inc.