VISCERAL ARTERY FIBROMUSCULAR DYSPLASIA – CLINICAL PRESENTATION AND VASCULAR BED INVOLVEMENT IN THE ARCADIA-POL STUDY

Abstract

Objective: 

Visceral artery fibromuscular dysplasia (VA FMD) is being rarely reported, with clinical picture ranging from asymptomatic to life-threatening. The aim of the study is to evaluate the prevalence and clinical characteristics of VA FMD.

Design and method: 

Out of 330 patients enrolled into ongoing ARCADIA-POL study since January 2015 (instituted as Polish-French collaboration) we present 225 patients (185females, mean age:46.1 ± 14.7ys) with confirmed FMD in at least one vascular bed. All patients underwent detailed clinical evaluation including ABPM, biochemical evaluation, biobanking, duplex Doppler of carotid and abdominal arteries and whole body angio-CT. In the current analysis, we focused on the prevalence and clinical characteristics of VA FMD (defined as the presence of typical FMD lesions in superior or inferior mesenteric arteries, as well as celiac trunk, splenic and hepatic arteries).

Results: 

Visceral FMD was present in 32 patients (19.1%). Among them, 75% (n = 24) were females, the mean age was 48.6 ± 17.2 years and 96.9% of patients have multivessel FMD. 28 patients reported FMD lesions in mesenteric arteries or celiac trunk, 6 patients in splenic and 8 patients in other visceral arteries. In 28 (12.4%) patients, aneurysms were found in visceral arteries, most commonly in splenic arteries (23 pts[10.2%]). In 2 patients, aneurysms were present in more than one visceral artery. In one patient, a dissection of the celiac trunk was identified. Patients with VA FMD most commonly reported abdominal pain (10 patients, 31.2%). 5 (15.6%) patients suffered from weight loss and 6 (18.6%) from postprandial abdominal pain. 3 patients with VA FMD experienced emergencies. Two patients underwent intestine resection due to acute mesenteric ischemia caused by occlusion of the upper mesenteric artery and of the celiac trunk respectively. In a third patient, the primary manifestation of VA FMD was rupture of hepatic aneurysm complicated by hypovolemic shock. The aneurysm was eventually embolized, with favorable clinical outcome.

Conclusions: 

In ARCADIA-POL study, VAFMD was present in every fifth patient. The clinical presentations of VA FMD varied from asymptomatic lesions to emergencies, including critical ischemia and life-threatening aneurysm bleeding. Our results support screening for VA FMD in patients with FMD in other vascular beds.