Stress Cardiomyopathy in a Patient with Advanced Stage Amyotrophic Lateral Sclerosis

    Authors

    Abstract

    Stress cardiomyopathy is an entity of unknown etiology characterized by transient systolic dysfunction of the left ventricle and regional wall motion abnormality which suggest myocardial infarction, but with an absence of angiographic evidence of obstructive coronary artery disease. Patients present with chest pain or/and dyspnea, while ECG changes are similar to acute myocardial infarction with ST-elevation. An important factor in the development of stress cardiomyopathy are high catecholamine levels in the blood as a result of the hyperactivity of the sympathetic nervous system caused by a stressful event. Amyotrophic lateral sclerosis (ALS) is an incurable progressive neurodegenerative disease that causes muscle weakness and ultimately ends in death due to respiratory muscle paralysis and respiratory failure. High catecholamine levels and increased sympathetic activity have been described in patients with ALS, which suggests that ALS is a risk factor for developing stress cardiomyopathy. In this article, we present a patient at an advanced stage of ALS who developed stress cardiomyopathy.

    Keywords

    stress cardiomyopathy, amyotrophic lateral sclerosis, acute ST-segment elevation myocardial infarction

    DOI

    https://doi.org/10.15836/ccar2020.16

    Full Text

    ## Case report A 63-year-old female patient with a medical history of ALS that was diagnosed fifteen years ago and with chronic global respiratory insufficiency with the application of continuous home oxygen therapy and consequent paraplegia, multiple spinal discus hernias in the neck area and dyslipidemia, was admitted to the emergency room with a clinical picture of acute coronary syndrome with chest pain starting approximately an hour before arrival and ST-elevation in the V2-V5 to 0.2 mV leads ( Figure 1 ). Based on the clinical picture and ECG, a tentative diagnosis of STEMI with anterolateral localization was established and the patient was referred to urgent coronarography. Coronarography excluded significant obstructive CAD ( Figure 2 ) and treatment was continued at the Intensive Care Unit. Echocardiography showed hypokinesis and discreet ballooning of the septoapical segment of the LV with a borderline global systolic function of 50%. Serial monitoring of myocardial necrosis markers did not find dynamics typical of ACS (peak high-sensitivity troponin concentrations were only 53.3 ng/L). After approximately 3 hours since the onset of the first symptoms, the patient no longer had any chest pain. On the second day of hospitalization the patient developed a clinical picture of refractory global respiratory insufficiency (O 2 saturation at 76%; pH 7.09) with consequent hypercapnia (pCO 2 15.3 kPa), due to which the patient was intubated and placed on mechanical ventilation. In further course of treatment, the patient was completely dependent on assisted ventilation and could not be removed from the respirator. ECG showed incomplete resolution of the ST-segment elevation on the front wall with the appearance of biphasic T waves in the V2-V5 leads ( Figure 3 ). Echocardiographic control examinations showed complete recovery of kinetics with LVEF recovery to 60%. There were no subsequent signs of heart failure or significant heart rhythm disorders, and the patient was treated symptomatically. The treatment course was prolonged and complicated by the development of respiratory (Pseudomonas aeruginosa) and urinary infections (E. coli, K. pneumoniae ESBL), for which reserve group antibiotics were administered. Given the respiratory status of the patient and since mechanical ventilation would be the destination therapy, the patient received a tracheotomy and was equipped with a home respirator. Due to further chronic care and conditioning requirements of the patient and education of the family, the patient was transferred to a specialized pulmonary center. ECG in emergency room (elevation of ST-segment up to 0.2 mV which suggests acute ST-segment elevation myocardial infarction of the anterior wall). (A) Coronary angiogram of the patient – left main coronary artery / left anterior descending artery / circumflex artery. (B) Coronary angiogram of the patient – right coronary artery. Patient ECG on day 3 of medical treatment (incomplete resolution of ST elevation with biphasic T waves). ## Discussion The patient with advanced ALS we have described developed stress cardiomyopathy followed by rapid development of respiratory insufficiency requiring permanent mechanical ventilation. Considering the clinical course and the available test results as well as our previous clinical experience ( 14 ), we established the diagnosis of stress cardiomyopathy despite considering the differential diagnosis of prolonged coronary artery spasm or spontaneous resolution of an intracoronary clot during the first hours of the treatment. Catecholamine cardiotoxicity associated with sympathetic nervous system hyperactivity has been emphasized as one of the more important pathogenic mechanisms for the development of Takotsubo cardiomyopathy ( 15 ). Increased levels of catecholamines have been demonstrated in patients with ALS ( 16 ). Autonomic dysfunction in ALS is still not sufficiently elucidated, but there is evidence of reduced activity of the parasympathetic and increased activity of the sympathetic nervous system ( 17 , 18 ). Sympathetic hyperactivity and elevated concentrations of plasma noradrenaline have been found already in the early stage of ALS ( 19 ), although sympathetic hyperactivity is not considered to necessarily be primary in ALS but rather secondary and dependent on the disease progression ( 20 ). Takotsubo cardiomyopathy manifests in different neurological diseases ( 21 , 22 ). The literature describes 20 cases of Takotsubo cardiomyopathy in patients with ALS, and its clinical significance, pathophysiology, and outcomes have not been sufficiently investigated ( 23 - 31 ). Due to the previously described changes in the autonomic nervous system in which the local release of noradrenaline as a consequence of increased sympathetic activity plays a crucial role, ALS represents a risk for the development of Takotsubo cardiomyopathy, which can manifest in the presence of a stress-inducing factor (e.g. infection, respiratory insufficiency, surgical procedure, etc.) ( 31 , 32 ). This implies the existence of etiological differences between Takotsubo cardiomyopathy in ALS and those in acute neurological disorders where the disease itself causes cardiomyopathy ( 31 ). The incidence of Takotsubo cardiomyopathy is higher in ALS in comparison with other forms in the heterogenous group of motor neuron diseases ( 32 ) and in comparison with synucleinopathies (Parkinson’s disease, Lewy body dementia, multiple system atrophy), which can also be ascribed to increased sympathetic activity ( 31 ). Outcomes in such patients are generally poorer in comparison with those who do not have motor neuron disease, most likely due to weakness of primary musculature as a consequence of the primary illness ( 32 ). Latest studies suggested that Takotsubo cardiomyopathy should be suspected in patients with diagnosed ALS who present with chest pain and dyspnea, especially in the advanced phase of the disease ( 30 ). ## Conclusion Our patient with advanced ALS was diagnosed with Takotsubo cardiomyopathy on the basis of the clinical picture and patient processing. This avoided prescription of the copious amounts of medication that guidelines recommend for acute MI. Takotsubo cardiomyopathy should be suspected in patients with motor neuron disease and the clinical picture of acute MI, thus avoiding the application of long-lasting and unnecessary medication treatment for non-existent CAD.

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    Stress Cardiomyopathy in a Patient with Advanced Stage Amyotrophic Lateral Sclerosis

    Case Report
    Issue1-2
    Published
    Pages16-21
    PDF via DOIhttps://doi.org/10.15836/ccar2020.16
    stress cardiomyopathy
    amyotrophic lateral sclerosis
    acute ST-segment elevation myocardial infarction

    Authors

    Ivana Sopek Merkaš*ORCIDCroatia
    Krunoslav FučkarORCIDCroatia
    Dora CerovecORCIDCroatia
    Nenad LakušićORCIDCroatia
    Ljubica Vincelj ŠalkovićORCIDCroatia
    Duško CerovecORCIDCroatia
    Igor ŠestoORCIDCroatia

    Abstract

    Stress cardiomyopathy is an entity of unknown etiology characterized by transient systolic dysfunction of the left ventricle and regional wall motion abnormality which suggest myocardial infarction, but with an absence of angiographic evidence of obstructive coronary artery disease. Patients present with chest pain or/and dyspnea, while ECG changes are similar to acute myocardial infarction with ST-elevation. An important factor in the development of stress cardiomyopathy are high catecholamine levels in the blood as a result of the hyperactivity of the sympathetic nervous system caused by a stressful event. Amyotrophic lateral sclerosis (ALS) is an incurable progressive neurodegenerative disease that causes muscle weakness and ultimately ends in death due to respiratory muscle paralysis and respiratory failure. High catecholamine levels and increased sympathetic activity have been described in patients with ALS, which suggests that ALS is a risk factor for developing stress cardiomyopathy. In this article, we present a patient at an advanced stage of ALS who developed stress cardiomyopathy.

    Full Text

    ## Case report A 63-year-old female patient with a medical history of ALS that was diagnosed fifteen years ago and with chronic global respiratory insufficiency with the application of continuous home oxygen therapy and consequent paraplegia, multiple spinal discus hernias in the neck area and dyslipidemia, was admitted to the emergency room with a clinical picture of acute coronary syndrome with chest pain starting approximately an hour before arrival and ST-elevation in the V2-V5 to 0.2 mV leads ( Figure 1 ). Based on the clinical picture and ECG, a tentative diagnosis of STEMI with anterolateral localization was established and the patient was referred to urgent coronarography. Coronarography excluded significant obstructive CAD ( Figure 2 ) and treatment was continued at the Intensive Care Unit. Echocardiography showed hypokinesis and discreet ballooning of the septoapical segment of the LV with a borderline global systolic function of 50%. Serial monitoring of myocardial necrosis markers did not find dynamics typical of ACS (peak high-sensitivity troponin concentrations were only 53.3 ng/L). After approximately 3 hours since the onset of the first symptoms, the patient no longer had any chest pain. On the second day of hospitalization the patient developed a clinical picture of refractory global respiratory insufficiency (O 2 saturation at 76%; pH 7.09) with consequent hypercapnia (pCO 2 15.3 kPa), due to which the patient was intubated and placed on mechanical ventilation. In further course of treatment, the patient was completely dependent on assisted ventilation and could not be removed from the respirator. ECG showed incomplete resolution of the ST-segment elevation on the front wall with the appearance of biphasic T waves in the V2-V5 leads ( Figure 3 ). Echocardiographic control examinations showed complete recovery of kinetics with LVEF recovery to 60%. There were no subsequent signs of heart failure or significant heart rhythm disorders, and the patient was treated symptomatically. The treatment course was prolonged and complicated by the development of respiratory (Pseudomonas aeruginosa) and urinary infections (E. coli, K. pneumoniae ESBL), for which reserve group antibiotics were administered. Given the respiratory status of the patient and since mechanical ventilation would be the destination therapy, the patient received a tracheotomy and was equipped with a home respirator. Due to further chronic care and conditioning requirements of the patient and education of the family, the patient was transferred to a specialized pulmonary center. ECG in emergency room (elevation of ST-segment up to 0.2 mV which suggests acute ST-segment elevation myocardial infarction of the anterior wall). (A) Coronary angiogram of the patient – left main coronary artery / left anterior descending artery / circumflex artery. (B) Coronary angiogram of the patient – right coronary artery. Patient ECG on day 3 of medical treatment (incomplete resolution of ST elevation with biphasic T waves). ## Discussion The patient with advanced ALS we have described developed stress cardiomyopathy followed by rapid development of respiratory insufficiency requiring permanent mechanical ventilation. Considering the clinical course and the available test results as well as our previous clinical experience ( 14 ), we established the diagnosis of stress cardiomyopathy despite considering the differential diagnosis of prolonged coronary artery spasm or spontaneous resolution of an intracoronary clot during the first hours of the treatment. Catecholamine cardiotoxicity associated with sympathetic nervous system hyperactivity has been emphasized as one of the more important pathogenic mechanisms for the development of Takotsubo cardiomyopathy ( 15 ). Increased levels of catecholamines have been demonstrated in patients with ALS ( 16 ). Autonomic dysfunction in ALS is still not sufficiently elucidated, but there is evidence of reduced activity of the parasympathetic and increased activity of the sympathetic nervous system ( 17 , 18 ). Sympathetic hyperactivity and elevated concentrations of plasma noradrenaline have been found already in the early stage of ALS ( 19 ), although sympathetic hyperactivity is not considered to necessarily be primary in ALS but rather secondary and dependent on the disease progression ( 20 ). Takotsubo cardiomyopathy manifests in different neurological diseases ( 21 , 22 ). The literature describes 20 cases of Takotsubo cardiomyopathy in patients with ALS, and its clinical significance, pathophysiology, and outcomes have not been sufficiently investigated ( 23 - 31 ). Due to the previously described changes in the autonomic nervous system in which the local release of noradrenaline as a consequence of increased sympathetic activity plays a crucial role, ALS represents a risk for the development of Takotsubo cardiomyopathy, which can manifest in the presence of a stress-inducing factor (e.g. infection, respiratory insufficiency, surgical procedure, etc.) ( 31 , 32 ). This implies the existence of etiological differences between Takotsubo cardiomyopathy in ALS and those in acute neurological disorders where the disease itself causes cardiomyopathy ( 31 ). The incidence of Takotsubo cardiomyopathy is higher in ALS in comparison with other forms in the heterogenous group of motor neuron diseases ( 32 ) and in comparison with synucleinopathies (Parkinson’s disease, Lewy body dementia, multiple system atrophy), which can also be ascribed to increased sympathetic activity ( 31 ). Outcomes in such patients are generally poorer in comparison with those who do not have motor neuron disease, most likely due to weakness of primary musculature as a consequence of the primary illness ( 32 ). Latest studies suggested that Takotsubo cardiomyopathy should be suspected in patients with diagnosed ALS who present with chest pain and dyspnea, especially in the advanced phase of the disease ( 30 ). ## Conclusion Our patient with advanced ALS was diagnosed with Takotsubo cardiomyopathy on the basis of the clinical picture and patient processing. This avoided prescription of the copious amounts of medication that guidelines recommend for acute MI. Takotsubo cardiomyopathy should be suspected in patients with motor neuron disease and the clinical picture of acute MI, thus avoiding the application of long-lasting and unnecessary medication treatment for non-existent CAD.