Spontaneous triple-vessel coronary artery dissection in a patient with Ehlers-Danlos Type IV

Background: Ehlers-Danlos syndrome type IV -vascular typeis an inherited connective tissue disorder characterized by several clinical features: easy bruisability, translucent skin, acrogeria and uterine, intestinal or arterial rupture. The arteries most frequently involved are the iliac, splenic, renal arteries and the aorta. Diagnosis is made utilizing clinical criteria (Beighton) and collagen gene mutation testing via skin biopsy. Case: We present a case of a 53 year old, Caucasian female with Ehlers-Danlos Type IV and history of myocardial infarction secondary to aortic dissection and coronary artery dissection (left anterior descending, left circumflex and right coronary artery) with subsequent coronary artery bypass grafting. The patient arrived to the emergency room with left-sided chest pain radiating to her left arm. Electrocardiography showed ST-segment depressions in leads I and aVL with T-wave inversions in leads V4-V6. On laboratory she had troponin elevation peaking at 3.35. Coronary computerized tomographic angiography was performed due to concerns of coronary artery rupture with invasive coronary angiography; revealing a slit-like narrowing of approximately 50% of her proximal left anterior descending artery and multiple ectatic to aneurysmal coronary artery segments involving the three major coronary vessels. Our approach was to pursue conservative management, optimizing her therapeutic regimen to prevent chest pain recurrence. Conclusion: Spontaneous coronary artery dissection is extremely rare and when it occurs case-reports have shown that it affects the left anterior descending artery. In this case we present a patient with triple vessel involvement which to the best of our knowledge has only been reported once. We believe that patients with EhlersDanlos Type IV who present with chest pain, should be evaluated with non-invasive imaging (i.e. CCTA or Cardiac MRI), as invasive procedures could potentially be more harmful than beneficial.