Reversible Stress Cardiomyopathy in Guillain-Barré Syndrome

A Case Report

A. Gravos; A. Destounis; K. Katsifa; P. Tselioti; K. Sakellaridis; V. Grammatikopoulou; C. Tsapas; A. Nodarou; P. Batiani; A. Prekates

Disclosures

J Med Case Reports. 2019;13(150) 

In This Article

Abstract and Introduction

Abstract

Background: Guillain-Barré syndrome is an autoimmune disorder in which autoantibodies mainly affect the peripheral nervous system. Autonomic dysfunction is a common and severe complication of Guillain-Barré syndrome. Cardiomyopathy, though, is a rare complication in Guillain-Barré syndrome, with only a few cases reported in the literature.

Case Presentation: We present a case of a 65-year-old Greek woman with Guillain-Barré syndrome who developed cardiomyopathy shortly after admission to the intensive care unit due to respiratory deterioration. Her estimated left ventricular ejection fraction upon admission was 20%. The result of coronary angiography was negative for coronary artery disease, and cardiac magnetic resonance imaging excluded myocarditis. Her clinical condition improved with supportive therapy, and her estimated left ventricular ejection fraction at discharge was normal.

Conclusions: Clinicians should be aware of this potentially lethal complication of Guillain-Barré syndrome and the therapeutic options, because early diagnosis can improve prognosis. Routine electrocardiographic and echocardiographic assessments should be performed in patients with Guillain-Barré syndrome presenting with hemodynamic instability.

Introduction

Guillain-Barré syndrome (GBS) is an autoimmune disease mainly affecting the peripheral nervous system. It may present with autonomic dysfunction (hypotension, hypertension, sinus tachycardia, paroxysmal tachyarrhythmias or bradyarrhythmias, and electrocardiographic [ECG] changes). Manifestations of GBS vary from monoparesis to life-threatening paralysis of the respiratory muscles.[1–4]

Cardiovascular abnormalities in GBS are attributed to autonomic neuropathy and are seen variably in two-thirds of affected patients.[2] There have been few case reports associating GBS and ECG abnormalities or left ventricular dysfunction.[5–12] Usually explained by temporary alterations in cardiac innervations or catecholamine cardiotoxicity, ECG abnormalities are often regressive.

The pathophysiology remains unclear, but the role of catecholamine-mediated myocardial stunning may be predominant. The association of GBS with stress cardiomyopathy is not well understood. Dysregulation of autonomic tone with excessive sympathetic activation in GBS with elevated catecholamine levels has been reported. The dysregulation of the parasympathetic and sympathetic systems is responsible for alterations in peripheral vascular resistance, most often causing transient or permanent hypotension.[13]

Rare cases of sudden cardiac death or cardiovascular collapse might be attributed to lethal arrhythmias or acute heart failure episodes, which could be prevented by transthoracic echocardiographic (TTE) examination and hemodynamic continuous monitoring.[4,8]

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