Addisonian Crisis Presenting As Acute Ventricular Failure in the Postpartum

Presentation Number: SAT-0773
Date of Presentation: June 21st, 2014

YunYing Shi* and Dorothy Santos Martinez
UCLA, Los Angeles, CA

Abstract

Introduction: Addison’s disease is often difficult to diagnose due to non-specific presenting symptoms. Hypovolemic hypotension is a commonly associated cardiovascular complication. Rarely, cardiogenic shock can also occur during an Addisonian crisis. We report here a postpartum young woman with undiagnosed Addison’s disease who presented with acute biventricular systolic failure as the primary marker for a fulminate adrenal crisis.

Case: A 32-year-old Caucasian female was admitted from an outside hospital (OSH) for heart transplant evaluation with presumed postpartum cardiomyopathy. Patient’s pregnancy was complicated by hyperemesis gravidarum started 2 months into her pregnancy. She did not have any history of hypotension while pregnant and had an uneventful vaginal delivery of a healthy, full-term boy. Two weeks postpartum, her emesis worsened, accompanied by 30lb weight loss, dizziness, and low blood pressure (BP) requiring IV hydration. Six months postpartum, she presented at OSH due to hemodynamic collapse with left ventricular ejection fraction (LVEF) <10%, requiring intubation and pressor support. Hospital course was complicated by acute renal failure, sepsis, anemia, right lower extremity arterial thrombus, stroke and seizure. On admission, her TSH was 106 mcIU/mL (n: 0.3-4.7 mcIU/mL). Levothyroxine IV 50mcg daily and hydrocortisone 100mg IV q8h were started by OSH.  Repeat TFT after 5 days of therapy at our hospital showed elevated TSH (16 mcIU/mL, n: 0.3-4.7 mcIU/mL), borderline-low Free T4 (0.6 ng/dL, n: 0.6-1.7 ng/dL), low Free T3 (122 pg/dL, n: 222-383 pg/dL), and positive thyroid peroxidase antibody and thyroglobulin antibody. Liothyronine 5mcg twice daily was added. Despite rapid full recovery of ventricular function (LVEF 60-65%) in two weeks, she continues to have intermittent low BP on hydrocortisone IV 25mg daily. Further evaluation showed elevated ACTH (2150 pg/mL, n: 4-48 pg/mL), elevated prolactin (152 ng/mL, n: 3.0-23.1 ng/mL), elevated rennin (6.3 ng/mL/hr, n: 0.6-1.6 ng/mL/hr), and normal aldosterone (n<1.0 ng/dL). A diagnosis of Addison’s disease was made. Her 21-hydroxylase antibody was later confirmed to be positive. Patient’s BP improved with the addition of mineralocorticoid. She was eventually discharged in stable condition on oral levothyroxine, hydrocortisone, and fludrocortisone.

Conclusion: New onset Addison’s disease is rare in pregnancy. It has been suggested that during the pregnancy, free cortisol produced by the fetal adrenal cortex can offer some maternal protection. However, the withdrawal of the cortisol source after delivery can result in acute adrenal insufficiency, in our case, leading to the rare presentation of acute ventricular dysfunction. Therefore, a high index of suspicion for Addison’s disease is required in postpartum women presenting with hemodynamic instability with a history of hyperemesis gravidarum.  

 

Nothing to Disclose: YS, DSM