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Case Reports
. 2022 Apr 9;6(4):ytac161.
doi: 10.1093/ehjcr/ytac161. eCollection 2022 Apr.

May-Thurner syndrome in patients with postural orthostatic tachycardia syndrome and Ehlers-Danlos syndrome: a case series

Cameron K Ormiston  1 , Erika Padilla  1 , David T Van  1 , Christine Boone  2 , Sophie You  3 , Anne C Roberts  2 , Albert Hsiao  2 , Pam R Taub  1
Affiliations
Case Reports

May-Thurner syndrome in patients with postural orthostatic tachycardia syndrome and Ehlers-Danlos syndrome: a case series

Cameron K Ormiston et al. Eur Heart J Case Rep. .

Abstract

Background: Postural orthostatic tachycardia syndrome (POTS), Ehlers-Danlos syndrome (EDS), and May-Thurner syndrome (MTS) are three syndromes that are often misdiagnosed or underdiagnosed. The true prevalence of these syndromes may be higher than currently reported. The following case series is the first to report a three-way association between POTS, EDS, and MTS.

Case summary: We describe three patients with concomitant POTS, EDS, and MTS. Although abdominopelvic vasculature evaluation can be difficult via conventional imaging techniques, we present the use of novel dynamic contrast-enhanced magnetic resonance angiography with Differential Subsampling with Cartesian Ordering (DISCO) and four-dimensional flow magnetic resonance imaging to aid vasculature evaluation and the diagnosis of MTS. Two patients underwent left common iliac vein stenting to treat MTS, experiencing significant improvement in their POTS symptoms and quality of life.

Discussion: Ehlers-Danlos syndrome, POTS, and MTS may interact synergistically to exacerbate symptoms. Patients with EDS should be evaluated for possible POTS and pelvic venous complications. Left common iliac vein stenting for MTS can mitigate POTS symptoms by decreasing lower extremity venous pooling and should be considered in this patient population. Further research is needed to understand the exact mechanism and intricacies of this syndrome triad.

Keywords: 4D flow MRI; Case series; Dysautonomia; Ehlers-Danlos syndrome; May-Thurner syndrome; Pelvic congestion syndrome; Postural orthostatic tachycardia syndrome.

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Figures

Figure 1
Figure 1
(A) Left common iliac vein antegrade venogram, (B) abdominopelvic vasculature–coronal view. (A and B) Severe stenosis of the left common iliac vein (arrow pointing into dashed box in A) with lumbar collateral venous filling (three blue arrows pointing leftward along lumbar collaterals in A). The left common iliac vein is compressed by the right common iliac artery (dashed box).
Figure 2
Figure 2
Left common iliac vein antegrade venogram. Moderate–severe compression of the left common iliac vein by the right common iliac artery (dashed box). Extensive pelvic venous collateral filling is present (arrows).
None
See this image and copyright information in PMC

References

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