Median Arcuate Ligament Syndrome Presenting as POTS

Abstract

Introduction: Patients with POTS and persistent gastrointestinal symptoms warrant careful evaluation to identify vascular disorders such as Median Arcuate Ligament Syndrome (MALS) that occurs when the median arcuate ligament (MAL) impinges and compress the celiac artery (CA) & celiac plexus, resulting in varying degrees of CA stenosis and celiac plexus excitation. Methods: We searched our patient database from 02/2013- 03/2015 using the terms: POTS, Tilt, and MALS. MALS was diagnosed when Celiac artery Peak systolic velocity (PSV) was >2.5 m/s in expiration or ≥ 3 times the abdominal aorta PSV and 50-70%, CT-angio celiac artery stenosis. Results: 41 patients with MALS and POTS; (37 females and 4 males), median age 16.8 years, with abdominal pain (100%), fatigue (100%), nausea (100%), dizziness (100%), delayed gastric emptying (58%), weight loss(77%), vomiting (26%), epigastric bruit (38%), clammy extremities (72%), palpitations (94%), headaches (58%), syncope (12%). 27(65%) patients had hyperadrenergic POTS as within 10 minutes of Tilt HR increased (from 88 ± 4 to 136 ± 5 bpm), systolic BP (SBP) increased from 112 ± 4 to 132 ± 5 mm Hg, and SBP in late phase II of the Valsalva maneuver was 152 ± 5 versus 110 ± 7 in controls; P < 0.05, SBP overshoot in phase IV was 42 ± 4 versus 16 ± 3 mm Hg in controls; P < 0.05. CA PSV in expiration was 380 ± 45 m/s versus 142 ± 21 m/s in controls; P < 0.01. Median follow up-11.8 months- Post Laparoscopic division of MAL, resulted in resolution of orthostatic and gastrointestinal symptoms and Quality of life (QOL) score improvement from 43.2 preoperative to 84.6 postoperative; P < 0.01. Conclusions: MALS a potentially curable syndrome should be suspected in patients with POTS and gastrointestinal symptoms.