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Obstetric anesthetic management in a patient with Loeys Dietz syndrome and prior spontaneous coronary artery dissection
Abstract Number: SUN-08
Abstract Type: Case Report/Case Series
Background: Loeys Dietz syndrome (LDS) is a rare genetic condition involving a mutation in the TGF-β signaling pathway, resulting in vascular and connective tissue abnormalities. Parturients with LDS carry an increased risk of uterine rupture and aortic dissection due to the increased hemodynamic stress of pregnancy, portending a significant increase in maternal morbidity and mortality. Accordingly, obstetric and anesthetic management should focus on early detection and monitoring, aggressive blood pressure control, reduction in sympathetic stimulation via early labor analgesia, and close follow up (1).
Case: A 40 year old G2P1 with LDS presented for induction of labor at 40 3/7 weeks gestation. She had a history of known aortopathy with a mildly dilated aortic root (39 mm) and fibromuscular dysplasia of the vertebral, carotid, renal, and iliac arteries. In addition, she had a history of spontaneous coronary artery dissection five months following her first delivery.
Induction was initiated with vaginal misoprostol and augmented via artificial rupture of membranes. An arterial line was placed and continuous ECG monitoring was employed for close hemodynamic monitoring. An epidural was placed for labor analgesia early in labor. A healthy male infant was delivered via normal spontaneous vaginal delivery. The patient received a prophylactic dose of rectal misoprostol, then a dose of carboprost for continued bleeding. She subsequently reported dyspnea, which resolved spontaneously. Postpartum, she was observed in the intensive care unit for 24 hours. No untoward cardiac events were observed.
The patient experienced atypical chest pain 3 weeks postpartum, which spontaneously resolved. Evaluation revealed stable aortic dilation and cardiac function with no evidence of recurrent dissection.
Discussion: LDS is associated with significant maternal morbidity and mortality. Patients should undergo CT or MR angiography to monitor for vascular abnormalities, serial maternal echocardiography to monitor for aortopathies and cardiomyopathies, and a fetal echocardiogram. In the peripartum period, patients should have close hemodynamic monitoring, aggressive blood pressure control to reduce the risk of aneurysm or dissection, and consideration for assisted second stage of labor to reduce hemodynamic fluctuations. Early epidural placement for labor analgesia may reduce the sympathetic stimulation and aortic wall stress associated with labor. Cesarean delivery should be considered in particularly high risk patients to reduce the stress of labor. Careful selection of medications in the setting of postpartum hemorrhage is essential to avoid hypertension and uterine rupture; methylergonovine is relatively contraindicated, and the use of oxytocin in this population is the subject of ongoing research. Patients should have close follow up, as cardiac events may occur several months postpartum (1).
(1) Frise CJ et al. Int J Cardiol 2017 Jan 1; 226:21-25