Case Presentation:
Exercise-induced chest pain is a hallmark of coronary artery ischemia. When unusual ischemic symptoms predominate however, they point to rare extrathoracic vascular compression syndromes.
A 28-year-old Caucasian man reported worsened daily left-sided chest and abdominal pain. The pain was chronic, of pressure-like quality, constant but worsened by exertion and mostly by eating resulting in significant unintentional weight loss. A work-up for myocardial infarction, pulmonary embolism and peptic ulcer disease was negative. CTA of the chest showed proximal celiac artery narrowing at the diaphragmatic crus with post-stenotic dilation. A duplex scan of the celiac artery showed dynamic compression at the level of the median arcuate ligament with peak systolic velocity increasing from 171 cm/sec to 500 cm/sec in a respiratory cycle, confirming the diagnosis. The patient received laparoscopic median arcuate ligament release with neurolysis of the celiac ganglion. His symptoms resolved and a repeat duplex scan performed 3 months later showed widely patent celiac trunk and superior mesenteric artery.
Discussion:
The median arcuate ligament connects the left and right diaphragmatic crura across the aortic hiatus at the T12/L1 vertebral level. Low insertion can cause symptomatic stenosis of the proximal celiac artery. The pathophysiology is is known to be related to ischemic and neuropathic mechanisms. In the absence of common causes of abdominal pain such as peptic ulcer disease, gallbladder disease, and pancreatitis, median arcuate ligament syndrome should be considered. The presence of chronic post-prandial abdominal pain, unintentional weight loss, and epigastric bruit in the absence of a history of vomiting should trigger a work up for median arcuate ligament syndrome particularly if there is no obvious cause of abdominal pain. Typically vomiting is absent which differentiates median arcuate ligament syndrome from gastric outlet obstruction or superior mesenteric artery syndrome.
Conclusions:
Patients presenting with such clinical findings should be evaluated with a duplex scan of the celiac artery in both phases of respiration to look for extrinsic compression of the celiac artery by the median arcuate ligament. This case also illustrates the potential for exertional chest pain in a young person to be due to extrathoracic vascular compression.