Peripartum cardiomyopathy in a patient with Addison's disease
Abstract Number: S-65
Abstract Type: Case Report/Case Series
Introduction: Pregnancy in the setting of Addison’s disease (adrenal insufficiency) is a rare and hazardous event, fraught with potential fetal and maternal complications requiring careful anesthetic management.(1) We present a case wherein Addison’s disease may have precipitated peripartum cardiomyopathy (PPCM).
Case Report: A 47 year old G5P1 female at 37 weeks presented with a PMH significant for COPD, recent HAP, IDDM, hypothyroidism, primary Addison’s disease, morbid obesity, and depression for a TOLAC. On admission, she was also found to have severe pre-eclampsia. Given the possibility of life-threatening adrenal crisis associated with Addison’s in the context of labor and this patient’s complicated history, a thorough assessment was performed. The potential for repeat cesarean section mandated the formulation of a well-delineated pre-, intra-, and post-operative plan. Initially, the patient was optimized with hydrocortisone, used in conjunction with nitroglycerin and insulin drips. An epidural was placed to reduce physiologic stress. The patient failed her trial of labor, requiring a repeat cesarean section with regional anesthesia. Intraoperatively, the patient received a stress dose of hydrocortisone. Still, she exhibited hypotension immediately following delivery. As cardiac instability is common in Addison’s patients during surgery, supportive care was immediately initiated with the administration of ephedrine and hetastarch. Post-operatively, the patient was closely monitored, glucocorticoids were continued, and aggressive post-operative pain control was instituted to minimize further stress. The next day, the patient complained of chest pain and SOB, consistent with PPCM and accompanying cardiogenic pulmonary edema. This led to acute respiratory failure requiring intubation for two days. She underwent catheterization, demonstrating depressed EF 40% without CAD. Subsequently, she was transferred to the MICU for diuresis and a steroid taper. The patient was discharged within seven days, exhibiting full recovery.
Discussion: Addison’s disease constitutes a management challenge, particularly in the context of complicated parturients. Despite adherence to treatment guidelines of Addison’s disease, this patient endured a protracted hospitalization due to PPCM. While the underlying etiology of PPCM remains unclear, autoimmune factors likely contribute.(2) Though no association between Addison’s and PPCM has previously been reported, this patient’s primary Addison’s disease, an autoimmune disorder, may have acted in concert with her pre-eclampsia to predispose her to PPCM. Awareness of such a link could help improve early recognition and ensure prompt intervention.
1. Erichsen, MM et al. Sexuality and fertility in women with Addison’s disease. J Clin Endocrinol Metab. 2010 Sep;95(9):4354-60.
2. Ansari, AA et al. Autoimmune mechanisms as the basis for human peripartum cardiomyopathy. Clin Rev Allergy Immunol 2002 Dec;23(3): 301-24.