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FC10-162
Anesthetic management of a patient with Loeys-Dietz syndrome

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Aortic dissection peripartum is uncommon but conveys high morbidity and mortality.1Loeys-Dietz syndrome (LDS) is a rare autosomal dominant aortopathy that is associated with aortic aneurysm or dissection. There is limited data to guide the management of patients with LDS. Peripartum imaging including transthoracic echocardiogram are needed to make case by case decisions.2

 

A 38-year-old G3P1 at 34 weeks with a past medical history of LDS was evaluated at our high-risk obstetric anesthesiology clinic. Her obstetrical history was significant for a previous low transverse cesarean delivery (CD). She was diagnosed with LDS given her family history. Her father developed an aortic dissection at the age of 40, and later succumbed to a cerebral aneurysm. The patient was routinely followed by cardiology ever since her diagnosis. Her echocardiogram revealed a normal biventricular size and functions, and a 33-34 mm aortic root (normal 22-36 mm). This was reported to be stable when compared to a prior echocardiogram. Magnetic Resonance Angiography of the chest and brain demonstrated no aortic aneurysm, mild stable focal dilation of the distal aorta, and no cerebral aneurysm. Previous cervical spine images were unremarkable; no cervical instability.

 

Given her obstetrical history, the plan was to proceed with a scheduled repeat CD. A spinal anesthetic was performed using intrathecal injection of hyperbaric bupivacaine 0.75% 12mg, 

15 mg of fentanyl and preservative morphine 0.1 mg. After a T4 level was achieved, surgery was commenced. Her intraoperative course was uneventful, and she delivered a vigorous 2.28 kg baby boy. On post-operative day 1, the patient showed symptoms of post-dural puncture headache. An epidural blood patch (EBP) was performed on post-operative day 2. A total of 25ml of autologous blood were injected into the epidural space. The EBP was successful and patient was discharged home on post-operative day 4. 

 

When caring for a patient with LDS, it is important to obtain brain and chest imaging to decide the mode of delivery of the patient and morbidity and mortality risk. Cesarean delivery is recommended for parturients with an aortic diameter of more than 4.5 cm. Extrapolating from data of patients with Marfan syndrome, there is a 10% risk of aortic dissection peripartum in patients with a maximal aortic diameter > 40 mm.1Early neuraxial analgesia for labor is preferred to avoid catecholamine surge during labor. Dural ectasia and cervical spine instability should be taken into account to tailor the anesthetic plan.2The use of oxytocin is controversial, as it has been related to aortic dissection. A high level of suspicion for aortic dissection should be maintained when caring for patients with LDS, and a multidisciplinary approach is recommended. 

 

1.     Int J Obstet Anesth 2018;37: 73-85

2.     Int J Cardiol 2017;226:21–5.

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