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

Abstract

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

Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases

“…( 290,294 ) Commonly reported causes include arteriosclerosis and fibromuscular dysplasia. ( 280,290 ) Recently, attention has been drawn to HHT ( 296 ) and the combination of alpha‐1 antitrypsin deficiency and chronic liver disease requiring LT as risk factors. ( 297 ) Pseudoaneurysms of the SA are mostly related to pancreatitis, infection, and trauma.…”

A Case of an Splenic Artery Aneurysm and Focal Nodular Hyperplasia Associated with an Abdominal Vascular Abnormality of Hereditary Hemorrhagic Telangiectasia

“…In contrast, thoracoabdominal artery aneurysms accounted for 13.2%, of which splenic artery aneurysms have been reported to account for 4.3%, and are considered to be relatively rare (13). A subsequent large study revealed that 24.7% of patients with HHT had splenic artery aneurysms, which was significantly higher than that in a sex-and age-matched control group (14). The risk factors for the development of splenic artery aneurysms include female sex, older age, multiple pregnancies, and pregnancy.…”

Hereditary Hemorrhagic Telangiectasia—A Case Series Experience from a Liver Transplant Center in Romania

“…Regarding arterial malformations, there are other brief reports which suggest the increased risk of developing arterial aneurysms, mainly splenic artery aneurysms (SAA) in patients with HHT [ 20 ]. Sellier et al found a 4.57 higher rate of SAA in their prospective study of 186 HHT patients who were screened with a multidetector CT [ 21 ]. In our case series we also encountered one patient with a splenic artery aneurysm that was successfully treated with embolization ( Figure 1 ).…”