Seok-Jin Choi1, Yoon-Ho Hong2, Je-Young Shin1, Byung-Nam Yoon3, Sung-Yeon Sohn4, Chan Soon Park5, Jung-Joon Sung6. 1. Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea. 2. Department of Neurology, Seoul National University-Seoul Metropolitan Government Boramae Medical Center, Seoul, Republic of Korea. 3. Department of Neurology, Inha University Hospital, Incheon, Republic of Korea. 4. Department of Neurology, Eulji University Hospital, Daejeon, Republic of Korea. 5. Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea. 6. Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea. Electronic address: jjsaint@snu.ac.kr.
Abstract
OBJECTIVE: To investigate the frequency, features, and prognosis of takotsubo cardiomyopathy (TTC) in patients with amyotrophic lateral sclerosis (ALS). METHODS: We reviewed detailed clinical, laboratory, and cardiovascular data from 64 ALS patients (38 men and 26 women) who underwent echocardiographic evaluation for various reasons at a single referral center between January 2011 and December 2015. RESULTS: TTC was diagnosed in 9 ALS patients (4 men and 5 women). Mean age was 61.3years (range 55-71years), and median disease duration was 51.5months (range 18-134months). All patients were bulbar or cervical onset, and were at advanced stages of ALS when TTC was diagnosed. Acute exacerbation of dyspnea was an invariable presentation, and chest discomfort mimicking acute coronary syndrome was present in 2 patients. Six patients had significant hypotension requiring intravenous fluid challenge and inotropic support. Three patients showed altered mentality, and 2 of them suffered cardiopulmonary arrest. CONCLUSIONS: TTC should be suspected in ALS patients presenting with acute exacerbation of dyspnea and chest discomfort, particularly at advanced stages of the disease. This study highlights the need for proper evaluation and management of cardiac dysfunction in ALS.
OBJECTIVE: To investigate the frequency, features, and prognosis of takotsubo cardiomyopathy (TTC) in patients with amyotrophic lateral sclerosis (ALS). METHODS: We reviewed detailed clinical, laboratory, and cardiovascular data from 64 ALSpatients (38 men and 26 women) who underwent echocardiographic evaluation for various reasons at a single referral center between January 2011 and December 2015. RESULTS: TTC was diagnosed in 9 ALSpatients (4 men and 5 women). Mean age was 61.3years (range 55-71years), and median disease duration was 51.5months (range 18-134months). All patients were bulbar or cervical onset, and were at advanced stages of ALS when TTC was diagnosed. Acute exacerbation of dyspnea was an invariable presentation, and chest discomfort mimicking acute coronary syndrome was present in 2 patients. Six patients had significant hypotension requiring intravenous fluid challenge and inotropic support. Three patients showed altered mentality, and 2 of them suffered cardiopulmonary arrest. CONCLUSIONS: TTC should be suspected in ALSpatients presenting with acute exacerbation of dyspnea and chest discomfort, particularly at advanced stages of the disease. This study highlights the need for proper evaluation and management of cardiac dysfunction in ALS.
Authors: L Brylev; V Fominykh; V Chernenkaia; I Chernenkiy; K Gorbachev; A Ataulina; A Izvekov; M Monakhov; A Olenichev; S Orlov; I Turin; M Loginov; S Rautbart; A Baymukanov; V Parshikov; V Demeshonok; A Yakovlev; T Druzhkova; A Guekht; N Gulyaeva Journal: Metab Brain Dis Date: 2021-09-24 Impact factor: 3.584