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Epidural Anesthesia for Cesarean Section in a Parturient with Moyamoya Disease and Congenital Heart Disease
Abstract Number: T-44
Abstract Type: Case Report/Case Series
Introduction: Moyamoya (MM) is a rare progressive cerebrovascular disorder including B/L stenosis of the distal internal carotid arteries and formation of collateral circulation at the base of the brain. We report a case of a parturient with MM and congenital heart disease (CHD) who presented in labor, and underwent a Cesarean section (C/S) with epidural anesthesia.
Case report: A 31 y/o G4P0121, presented in labor at 38 3/7 weeks gestation. Eight years prior she suffered an ischemic stroke and was diagnosed with MM. She underwent a left EC-IC bypass and recovered fully. She was advised to continue anticoagulation and undergo a right EC-IC bypass, but declined, and later suffered a TIA. PMH also included repair of an ostium primum VSD and mitral valve (age 2). She then required a St. Jude mitral valve (age 9), with reoperation at age 31 for valve thrombosis. A bovine valve was placed, tricuspid annuloplasty completed, and a Maze procedure performed for atrial fibrillation. During pregnancy, anticoagulation was continued, (warfarin to enoxaparin) and she remained well-compensated on metoprolol (NYHA Class 2). A TTE at 27 weeks showed mild MR, TR, and pulmonary HTN; moderate RA and severe LA enlargement; and normal RV and LV systolic function. With multi-disciplinary cooperation (neurology, cardiology, MFM), the delivery plan included replacing enoxaparin with heparin at 37 weeks, and an assisted delivery under regional anesthesia (RA). When she presented, coags were normal and a lumbar epidural was placed. Due to arrest of labor (5-6cm) and a non-reassuring FHR, the epidural was augmented and an uncomplicated C/S was performed. A healthy female was delivered (Apgars 8,9) and our patient was discharged on POD #3.
Discussion: MM occurs in two groups: juveniles present with cerebral ischemia, adults with hemorrhagic symptoms. Revascularization is recommended for ischemic attacks; medical therapy includes antiplatelet, anticoagulant and cerebral vasodilator drugs. MM patients face an increased risk of cerebrovascular events (hemorrhage, ischemia) during pregnancy. Maintaining normotension and normocapnia during parturition is essential in MM, especially in combination with CHD. Hypertension (labor, increased catecholamines) may increase afterload and worsen regurgitant flow leading to heart failure, as well as causing intracranial hemorrhage. Hypotension (rapid sympathectomy) and hypocapnia compromise cerebral blood flow. Hypercapnia (IV opioids, sedatives) may “steal” blood from affected areas of the brain and also worsen pulmonary HTN. Both GA and RA have been used for C/S. RA allows continuous neurological assessment, and avoids hypertension (intubation and extubation), but may be problematic with anticoagulation. An epidural or sequential CSE permits gradual hemodynamic changes and postoperative analgesia.(1) A planned vaginal delivery requires a good working epidural for an assisted second stage.
References: 1. IJOA 2006;15:152-158