Myocardial Infarction and Coronary Artery Dissection During Pregnancy Associated with Type IV Ehlers-Danlos Syndrome

Abstract

Ehlers-Danlos syndrome (EDS) is a rare, genetically transmitted connective tissue disorder which occurs with an incidence of 1 in 5000. Nine subtypes of EDS have been identified, with type IV being associated with sudden death due to rupture of the bowel, uterus, or major blood vessels. We describe a case of a patient with type IV EDS who presented at 30 weeks' gestation with preterm labor which was treated with subcutaneous terbutaline followed by oral maintenance therapy. Approximately 72 hours later she suffered a myocardial infarction, which led to coronary artery dissection and death. This is the first case of type IV EDS in pregnancy that resulted in maternal death due to coronary artery dissection. We suggest that betamimetic tocolytics should be avoided in patients with EDS and preterm labor.

Pregnancy-related deaths and complications in women with vascular Ehlers–Danlos syndrome

“…1,6,14 The data from Rudd et al, 1 in which there is probably ascertainment bias, are incorporated into the data provided by Pepin et al 6 and into the data we have presented in this article. Most of the other publications present single-case reports of complications and then progressively summarize the data from these reports, [3][4][5]7,8,11,[15][16][17][18][19] although some small series are appearing. 9,20 Inevitably, this leads to a view of vEDS and pregnancy that is neither objective nor encouraging.…”

Acute Myocardial Infarction Associated With Pregnancy

“…Thrombolytic therapy is considered to be relatively contraindicated in pregnancy, and because pregnant patients have been traditionally excluded from clinical trials, the information available is anecdotal (14,21,38,42,43,49,55). Recent clinical experience with the use of TT in pregnancy has been mostly with tPA and primarily in patients with stroke, prosthetic heart valve thrombosis, pulmonary embolism, or deep vein thrombosis (124,125).…”

Pregnancy-Related Maternal Deaths

“…Virtually any artery may be involved, including the coronary arteries, the aorta, the cerebral arteries, the iliac arteries, the splenic artery, the renal arteries, or any other medium to large artery. 18,19,[23][24][25][26][27] In addition, vena caval rupture has been reported in pregnancy. 18 In the present study, arterial dissections/ruptures accounted for 11% of maternal deaths investigated and autopsied.…”

“…Specifically, type IV Ehlers-Danlos syndrome (a disorder of collagen) can be implicated in many vascular dissections. 18,19,23,[25][26][27][28] Postmortem fibroblast cultures with subsequent testing at referral research centers allows for a definitive diagnosis. 18,19,28 Pregnancy likely acts as a contributing factor in these cases, based on the physiologic stresses and estrogenic effects associated with pregnancy.…”