Cardiac tamponade as the initial presentation of autoimmune polyglandular syndrome Type 2: a case report

Abstract

Background: Cardiac tamponade is a rare but serious manifestation of autoimmune polyglandular syndrome Type 2 (APS 2). Patients often present with symptoms of thyroid dysfunction and adrenal insufficiency, but the insidious onset of the disease may lead to delayed diagnosis, which can progress rapidly to haemodynamic instability requiring urgent intervention.

Case summary: A 39-year-old previously healthy male was admitted with cardiac tamponade complicated by cardiac arrest requiring emergent pericardiocentesis. An extensive work up revealed primary adrenal insufficiency and Hashimoto's thyroiditis. His positive autoantibodies to thyroid peroxidase and 21-hydroxylase combined with rapid improvement with initiation of corticosteroids and levothyroxine confirmed a diagnosis of APS 2.

Discussion: Although this disease is often difficult to diagnose given its vague symptoms, it should be considered in the differential diagnosis for young patients presenting with pericardial effusion or cardiac tamponade of unknown origin. Early diagnosis and management are critical and often result in rapid improvement after appropriate treatment.

Keywords: Adrenal insufficiency; Autoimmune polyglandular syndrome Type 2; Cardiac tamponade; Case report.

Figure 1

Electrocardiograms. The patient initially presented with tachycardia and low voltage (A), the latter of which did not resolve immediately after pericardiocentesis (B).

Figure 2

Transthoracic echocardiograms. Initial echo at the outside hospital did not reveal right ventricular diastolic collapse (A), but did show increased variability of mitral inflow velocities (B). Repeat echo at our tertiary hospital showed right ventricular diastolic collapse (C and D), while variations in inflow velocities partially reflected the patient’s intubated status (E and F). In the cath laboratory (G), ∼200 mL was removed, which essentially resolved the effusion (H).