Takotsubo cardiomyopathy with Guillain-Barré syndrome Dalvir Gill, MD, Vanessa Goyes Ruiz, MD, Ryan Dean, MD, and Kan Liu, MD
Figure 1. Electrocardiogram on admission.
A 70-year-old woman presented with progressive lower extremity weakness and heaviness accompanied with chest pain. Troponin T was elevated, and an echocardiogram showed a left ventricular ejection fraction of 30% and a hypokinetic left ventricular apex. Neurophysiologic testing was consistent with Guillain-Barré syndrome, which was treated with intravenous immunoglobulin therapy. Repeat echocardiogram showed an improved left ventricular ejection fraction and no left ventricular wall motion abnormalities. Takotsubo cardiomyopathy is a rare complication of Guillain-Barré syndrome; less than 10 cases have been reported.
G
uillain-Barré syndrome (GBS) is a relatively uncommon condition in which the body’s immune system attacks part of the peripheral nervous system (1). It can affect the nerves that control muscle movement, pain, and temperature sensation and can be fatal if it affects the respiratory muscles (2). Rarely has it been associated with takotsubo cardiomyopathy (TC), a transient cardiac syndrome that mimics acute coronary syndrome. We present an older woman who developed TC in association with GBS. Proc (Bayl Univ Med Cent) 2017;30(3):307–308
CASE REPORT A 70-year-old woman with a history of post-polio syndrome presented with progressive, worsening lower extremity weakness and heaviness accompanied with chest pain. On presentation she was hemodynamically stable; the electrocardiogram (Figure 1) did not show any ischemic changes, and her troponin T level was elevated to 0.42 ng/mL. On admission, two-dimensional echocardiogram showed a left ventricular ejection fraction of 30% with a severely hypokinetic anterior septum and left ventricular apex. She underwent cardiac catheterization, which showed normal coronary arteries and confirmed the hypokinetic left ventricular walls (Figure 2). She was started on carvedilol and enalapril. Acute findings were not seen on computed tomography of the head, and her lumbar puncture was normal. From the Department of Internal Medicine (Gill, Ruiz, Dean) and Division of Cardiology (Liu), State University of New York Upstate Medical University, Syracuse, New York. Corresponding author: Dalvir Gill, MD, Department of Internal Medicine, State University of New York Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210 (e-mail: [email protected]). 307
lead to arrhythmias, acute coronary syndrome, and myocarditis. TC can be difficult to distinguish. The criteria to diagnose TC include transient hypokinesis, akinesis, or dyskinesis of the left ventricular wall with or without apical involvement in the absence of obstructive coronary artery disease (4, 5). The pathogenesis in relation to GBS is not well understood. Dysregulation of autonomic tone with excessive sympathetic activation in GBS with elevated catecholamine levels is one hypothesis (5). The pathophysiology of TC seems to be from sympathetic excitation of the brain triggering catecholamine release, causing hyperdynamic basal contraction and apical systolic dysfunction (4, 5). In some instances, intravenous immunoglobulin has been associated with the development of TC (5). The treatment of TC is supportive, with beta-blockers and angiotensin-converting enzyme inhibitors until left ventricular ejection fraction improvement. TC is a rare complication during the acute phase of GBS and must be distinguished from autonomic dysfunction, as the diagnoses involve different treatments.
Figure 2. Left ventriculogram in ventricular systole.
On the second day of admission, she underwent neurophysiology testing with findings consistent with GBS. She was treated with intravenous immunoglobulin therapy, which provided significant improvement in strength in her lower extremities. An echocardiogram was repeated 4 months later showing an improved left ventricular ejection fraction of 50% and no left ventricular wall motion abnormalities. DISCUSSION TC is a rare complication of GBS, with fewer than 10 cases reported. GBS is an autoimmune process that affects the peripheral nervous system, causing autonomic dysfunction that may involve the heart (3). Autonomic dysfunction in GBS can
308
1. 2.
3.
4. 5.
Flachenecker P. Autonomic dysfunction in Guillain-Barré syndrome and multiple sclerosis. J Neurol 2007;254(Suppl 2):II96–II101. Iga K, Himura Y, Izumi C, Miyamoto T, Kijima K, Gen H, Konishi T. Reversible left ventricular dysfunction associated with Guillain–Barré syndrome—an expression of catecholamine cardiotoxicity? Jpn Circ J 1995;59(4):236–240. Palferman TG, Wright I, Doyle DV. Electrocardiographic abnormalities and autonomic dysfunction in Guillain-Barré syndrome. Br Med J 1982;284(6324):1231–1232. Bybee KA, Prasad A. Stress-related cardiomyopathy syndromes. Circulation 2008;118(4):397–409. Gianni M, Dentali F, Grandi AM, Sumner G, Hiralal R, Lonn E. Apical ballooning syndrome or takotsubo cardiomyopathy: a systematic review. Eur Heart J 2006;27(13):1523–1529.
Baylor University Medical Center Proceedings
Volume 30, Number 3
Figure 1. Electrocardiogram on admission.
A 70-year-old woman presented with progressive lower extremity weakness and heaviness accompanied with chest pain. Troponin T was elevated, and an echocardiogram showed a left ventricular ejection fraction of 30% and a hypokinetic left ventricular apex. Neurophysiologic testing was consistent with Guillain-Barré syndrome, which was treated with intravenous immunoglobulin therapy. Repeat echocardiogram showed an improved left ventricular ejection fraction and no left ventricular wall motion abnormalities. Takotsubo cardiomyopathy is a rare complication of Guillain-Barré syndrome; less than 10 cases have been reported.
G
uillain-Barré syndrome (GBS) is a relatively uncommon condition in which the body’s immune system attacks part of the peripheral nervous system (1). It can affect the nerves that control muscle movement, pain, and temperature sensation and can be fatal if it affects the respiratory muscles (2). Rarely has it been associated with takotsubo cardiomyopathy (TC), a transient cardiac syndrome that mimics acute coronary syndrome. We present an older woman who developed TC in association with GBS. Proc (Bayl Univ Med Cent) 2017;30(3):307–308
CASE REPORT A 70-year-old woman with a history of post-polio syndrome presented with progressive, worsening lower extremity weakness and heaviness accompanied with chest pain. On presentation she was hemodynamically stable; the electrocardiogram (Figure 1) did not show any ischemic changes, and her troponin T level was elevated to 0.42 ng/mL. On admission, two-dimensional echocardiogram showed a left ventricular ejection fraction of 30% with a severely hypokinetic anterior septum and left ventricular apex. She underwent cardiac catheterization, which showed normal coronary arteries and confirmed the hypokinetic left ventricular walls (Figure 2). She was started on carvedilol and enalapril. Acute findings were not seen on computed tomography of the head, and her lumbar puncture was normal. From the Department of Internal Medicine (Gill, Ruiz, Dean) and Division of Cardiology (Liu), State University of New York Upstate Medical University, Syracuse, New York. Corresponding author: Dalvir Gill, MD, Department of Internal Medicine, State University of New York Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210 (e-mail: [email protected]). 307
lead to arrhythmias, acute coronary syndrome, and myocarditis. TC can be difficult to distinguish. The criteria to diagnose TC include transient hypokinesis, akinesis, or dyskinesis of the left ventricular wall with or without apical involvement in the absence of obstructive coronary artery disease (4, 5). The pathogenesis in relation to GBS is not well understood. Dysregulation of autonomic tone with excessive sympathetic activation in GBS with elevated catecholamine levels is one hypothesis (5). The pathophysiology of TC seems to be from sympathetic excitation of the brain triggering catecholamine release, causing hyperdynamic basal contraction and apical systolic dysfunction (4, 5). In some instances, intravenous immunoglobulin has been associated with the development of TC (5). The treatment of TC is supportive, with beta-blockers and angiotensin-converting enzyme inhibitors until left ventricular ejection fraction improvement. TC is a rare complication during the acute phase of GBS and must be distinguished from autonomic dysfunction, as the diagnoses involve different treatments.
Figure 2. Left ventriculogram in ventricular systole.
On the second day of admission, she underwent neurophysiology testing with findings consistent with GBS. She was treated with intravenous immunoglobulin therapy, which provided significant improvement in strength in her lower extremities. An echocardiogram was repeated 4 months later showing an improved left ventricular ejection fraction of 50% and no left ventricular wall motion abnormalities. DISCUSSION TC is a rare complication of GBS, with fewer than 10 cases reported. GBS is an autoimmune process that affects the peripheral nervous system, causing autonomic dysfunction that may involve the heart (3). Autonomic dysfunction in GBS can
308
1. 2.
3.
4. 5.
Flachenecker P. Autonomic dysfunction in Guillain-Barré syndrome and multiple sclerosis. J Neurol 2007;254(Suppl 2):II96–II101. Iga K, Himura Y, Izumi C, Miyamoto T, Kijima K, Gen H, Konishi T. Reversible left ventricular dysfunction associated with Guillain–Barré syndrome—an expression of catecholamine cardiotoxicity? Jpn Circ J 1995;59(4):236–240. Palferman TG, Wright I, Doyle DV. Electrocardiographic abnormalities and autonomic dysfunction in Guillain-Barré syndrome. Br Med J 1982;284(6324):1231–1232. Bybee KA, Prasad A. Stress-related cardiomyopathy syndromes. Circulation 2008;118(4):397–409. Gianni M, Dentali F, Grandi AM, Sumner G, Hiralal R, Lonn E. Apical ballooning syndrome or takotsubo cardiomyopathy: a systematic review. Eur Heart J 2006;27(13):1523–1529.
Baylor University Medical Center Proceedings
Volume 30, Number 3
