Higher prevalence of splenic artery aneurysms in hereditary hemorrhagic telangiectasia: Vascular implications and risk factors.


Journal

PloS one
ISSN: 1932-6203
Titre abrégé: PLoS One
Pays: United States
ID NLM: 101285081

Informations de publication

Date de publication:
2020
Historique:
received: 19 02 2019
accepted: 02 12 2019
entrez: 24 1 2020
pubmed: 24 1 2020
medline: 9 4 2020
Statut: epublish

Résumé

Splenic artery aneurysm (SAA) is a rare but potentially fatal condition. Rupture results in 25% mortality up to 75% in pregnant women with 95% fetal mortality. Brief reports suggest an increased risk of developing SAA in patients with HHT. We analyzed enhanced multidetector CT data in 186 HHT patients matched (gender and ± 5 year old) with 186 controls. We screened for SAA and recorded diameter of splenic and hepatic arteries and hepatic, pancreatic and splenic parenchymal involvements. We determined by univariate and multivariate analysis, the relationship with age, sex, genetic status, cardiovascular risk factors (CVRF) and visceral involvement. SAA concerned 24.7% of HHT patients and 5.4% of controls, p<0.001. Factors associated with increased risk of SAA in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03-4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02-1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01-4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or CVRF. We found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF. These results suggest the presence of a vascular intrinsic involvement. It should lead to screening all HHT patients for SAA. The vasculopathy hypothesis could require a change in management as screening of all systemic arteries and even the aorta and to further research in the field.

Sections du résumé

BACKGROUND
Splenic artery aneurysm (SAA) is a rare but potentially fatal condition. Rupture results in 25% mortality up to 75% in pregnant women with 95% fetal mortality. Brief reports suggest an increased risk of developing SAA in patients with HHT.
METHODS
We analyzed enhanced multidetector CT data in 186 HHT patients matched (gender and ± 5 year old) with 186 controls. We screened for SAA and recorded diameter of splenic and hepatic arteries and hepatic, pancreatic and splenic parenchymal involvements. We determined by univariate and multivariate analysis, the relationship with age, sex, genetic status, cardiovascular risk factors (CVRF) and visceral involvement.
RESULTS
SAA concerned 24.7% of HHT patients and 5.4% of controls, p<0.001. Factors associated with increased risk of SAA in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03-4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02-1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01-4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or CVRF.
CONCLUSIONS
We found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF. These results suggest the presence of a vascular intrinsic involvement. It should lead to screening all HHT patients for SAA. The vasculopathy hypothesis could require a change in management as screening of all systemic arteries and even the aorta and to further research in the field.

Identifiants

pubmed: 31971937
doi: 10.1371/journal.pone.0226681
pii: PONE-D-19-04950
pmc: PMC6977744
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

e0226681

Déclaration de conflit d'intérêts

The authors have declared that no competing interests exist.

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Auteurs

Jacques Sellier (J)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Carma Karam (C)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Alain Beauchet (A)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Axel Dallongeville (A)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Stephen Binsse (S)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Sandra Blivet (S)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Isabelle Bourgault-Villada (I)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Philippe Charron (P)

Pitié-Salpêtrière Hospital, Department of Genetics, AP-HP, Paris, France.

Thierry Chinet (T)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Mélanie Eyries (M)

Pitié-Salpêtrière Hospital, Department of Genetics, AP-HP, Paris, France.

Carole Fagnou (C)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Jérome Lesniak (J)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Gilles Lesur (G)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Jérome Lucas (J)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Agnès Nicod-Tran (A)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Augustin Ozanne (A)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Aurélien Palmyre (A)

Pitié-Salpêtrière Hospital, Department of Genetics, AP-HP, Paris, France.

Florent Soubrier (F)

Pitié-Salpêtrière Hospital, Department of Genetics, AP-HP, Paris, France.

Mostafa El Hajjam (M)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

Pascal Lacombe (P)

Ambroise Paré Hospital, AP-HP, Boulogne-Billancourt, University of Versailles-Saint Quentin en Yvelines, France.

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