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Cesarean Delivery in a Patient with Myotonic Dystrophy, Severe Cardiopulmonary Decompensation and Pulmonary Hypertension
Abstract Number: FCA-439
Abstract Type: Case Report Case Series
BACKGROUND AND AIMS:
Myotonic dystrophy (MD) is a rare disorder characterized by progressive myopathy and myotonia but systemic complications may include cardiomyopathy and difficulty weaning from mechanical ventilation (MV) . Discussion of obstetrical cases in the context of these comorbidities are sparse, and to our knowledge, this is the first case of regional anesthesia for full-term delivery in a parturient with MD and concomitant cardiopulmonary compromise.
A 27 year-old G3P0020 at 35w2d with a history of MD and prolonged tracheostomy in her childhood was admitted to the hospital for delivery planning and progressive hypoxia requiring supplemental oxygen since 25 weeks gestation. Hospital monitoring revealed periodic episodes of non-sustained ventricular tachycardiac, and echocardiogram showed severe right ventricular enlargement with septal wall flattening during both systole and diastole. Cesarean section after betamethasone and optimization was scheduled. Prior to surgery, arterial line and defibrillation pads were placed. A combined spinal-epidural (CSE) was placed at L3-4 (loss of resistance 4 cm; catheter 8 cm at skin) without intrathecal drug administration. Epidural testing was negative for intrathecal or vascular placement. Intravenous epinephrine and vasopressin infusions were initiated to maintain hemodynamic stability. Due to incomplete sensory deficit, local infiltration using lidocaine was used on skin and uterine incision points. Uterine incision was made 33 minutes after skin incision, and a viable male with APGAR scores of 3 at 1 minute and 7 at 5 minutes was delivered at 36w2d. The patient continued BiPAP in the intensive care unit.
The choice of vasoactive agents in parturients with MD and cardiopulmonary compromise should reflect pulmonary vascular selectivity  and fluid balance after auto-transfusion. Given the risk of prolonged MV, neuraxial anesthesia was critical to maintaining spontaneous respiration. Epidural or intrathecal opioid was avoided due to risk of apnea , and intrathecal anesthetic avoided due to sympathectomy. A CSE without an intrathecal dosing may provide adequate anesthesia with aid of local infiltration of lidocaine.
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