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Peripartum seizure and cardiac arrest in the setting of tuberous sclerosis complex
Abstract Number: T5C-1
Abstract Type: Case Report/Case Series
Tuberous sclerosis is an autosomal dominant genetic disorder with an incidence of approximately one in 5000-10,000. While highly variable in expression, tuberous sclerosis complex (TSC) is characterized by a constellation of findings including seizures, intellectual disability, renal impairment, and a variety of tumors in multiple organs including cardiac rhabdomyomas.(1) We present a case of peripartum seizure followed by cardiac arrest in a woman with TSC.
Our patient is a 25 year-old gravida 3 para 1 at 39 weeks gestation with past medical history significant for TSC, complex partial seizures, cardiac rhabdomyoma without obstruction, and left clear cell renal cell carcinoma who presented for scheduled repeat Cesarean delivery. A spinal anesthetic with 0.75% bupivacaine, fentanyl, and morphine was administered. Immediately after delivery, the patient displayed tonic clonic seizure activity which progressed to apnea, hypotension, and pulseless electrical activity (PEA) arrest. Advanced cardiac life support (ACLS) was immediately initiated and the patient was intubated. The patient was subsequently defibrillated for pulseless ventricular tachycardia. Return of spontaneous circulation (ROSC) was obtained approximately four minutes after initial arrest. Shortly after transfer to the ICU, the patient had another seizure, became unresponsive, was reintubated, and again developed cardiac arrest. After a brief period of CPR, ROSC was achieved. Transthoracic echocardiogram revealed only her known rhabdomyomas and chest computerized tomography angiography was negative. An implantable cardioverter-defibrillator was placed for secondary prevention. Four months later she suffered recurrent cardiac arrest following partial nephrectomy and expired.
Despite the relatively high incidence of TSC, there is a paucity of literature published regarding obstetric anesthetic outcomes and considerations. The recurrent cardiac arrests suffered in this patient were ultimately without definitive diagnosis. Possible contributors were a catecholamine surge associated with seizure activity as well as her cardiac rhabdomyomas. Although the behavior of a cardiac rhabdomyoma is benign, the positioning within critical areas in the heart can lead to lethal arrhythmias. Typically, women with TSC tolerate both regional and general anesthesia; however, a pregnancy complicated by maternal TSC deserves close observation for potential neurologic and cardiovascular sequelae.(2)
References: 1. Hines RL. Stoelting’s Anesthesia and Co-Existing Disease. 7th Ed. 2018; p.265-303. 2. Sharma, et al. J Reprod Infertil 2017;18(2):257-260.