Stress Cardiomyopathy Diagnosis and Treatment

Medina de Chazal H, Del Buono MG, Keyser-Marcus L, et al.
Stress Cardiomyopathy Diagnosis and Treatment: JACC State-of-the-Art Review. J Am Coll Cardiol 2018;72:1955-1971.

The following are summary points to remember from this state-of-the-art review of stress cardiomyopathy:

  1. Stress cardiomyopathy is a clinical syndrome characterized by an acute and transient (<21 days) left ventricular (LV) systolic (and diastolic) dysfunction often related to an emotional or physical stressful event, most often identified in the preceding days (1-5 days).
  2. The exact pathophysiology of stress cardiomyopathy remains unclear, but the link between the brain and heart has long been known. An increase in cerebral blood flow in the hippocampus, brainstem, and basal ganglia has been shown in the acute phases. There is a complex neocortical and limbic integration in response to stress through an activation of brainstem noradrenergic neurons and stress-related neuropeptides (i.e., neuropeptide Y [NPY] produced by the arcuate nucleus in the hypothalamus). In a predisposed individual, who may have enrichment in NPY/norepinephrine granules and risk factors for endothelial dysfunction, an intense stimulation for an adrenergic stimulation may be sufficient to trigger stress cardiomyopathy in response to emotional or physical stress.
  3. The incidence is approximately 15-30 cases per 100,000 per year in the United States, but the true incidence is likely higher, if one considers that milder forms may not receive medical attention. Stress cardiomyopathy occurs more frequently in postmenopausal women and registry data suggest that about 90% of cases occurred in this demographic. Diabetes mellitus has been described as a risk factor for stress cardiomyopathy; it is present in 10-25% of patients, and is associated with increased mortality. Asthma exacerbation is another possible trigger of stress cardiomyopathy, mainly following medical interventions (short-acting ß2 adrenergic receptor agonist, epinephrine, and intubation). Cannabis use disorder has been identified as a risk factor for stress cardiomyopathy and is associated with a threefold higher risk of cardiac arrest.
  4. Emotional and/or physical stress are triggers for stress cardiomyopathy. Of note, emotional stress seems to be more common in women, whereas physical stressful triggering events are more common among men. The most common emotional stressors reported include the death of a loved one, assault and violence, natural disasters, and great financial loss, with most involving a sense of doom, danger, and/or desperation. Episodes of stress cardiomyopathy may, however, also follow unexpected pleasant events, “happy heart syndrome.” Physical stressors reported include acute critical illness, surgery, severe pain, sepsis, and exacerbations of chronic obstructive pulmonary disease or asthma.
  5. Stress cardiomyopathy can be classified according to either primary or secondary form, depending on whether it is the primary reason of care-seeking (primary form) or the patient is already in the health care setting during evaluation or treatment of another critical illness (secondary form). Differentiating these two situations is relevant due to their different characteristics and clinical outcomes.
  6. Anatomical variants include: a) with wall motion abnormalities resembling Takotsubo or “octopus pot,” with a narrow neck and globular lower portion in the apical ballooning form, which remains the typical pattern, and is present in 75-80% of patients; b) the midventricular ballooning pattern, in which the mid-LV is hypo- or akinetic, with normal apical and basal contraction, is present in 10-20% of patients and is associated with more severe reduction in cardiac output and cardiogenic shock; c) the basal or inverted Takotsubo is found in <5% of patients and is associated with less severe hemodynamic compromise; d) biventricular dysfunction (0.5% of cases); and e) focal dysfunction (rare), including isolated right ventricular dysfunction.
  7. The typical patient with stress cardiomyopathy is a postmenopausal woman who presents with acute or subacute onset of chest pain (>75%) and/or shortness of breath (approximately 50%), often with dizziness (>25%) and occasional syncope (5-10%). In the majority of the cases, the patient has experienced an emotional or physical stress that he/she may not share with the health care provider unless asked. Physical examination reveals features of acute decompensated left-sided heart failure and a systolic murmur when there is accompanying LV outflow tract obstruction.
  8. A definite diagnosis cannot be established at presentation because of the need to demonstrate the reversible nature of the condition. The InterTAK International Registry Group has developed a scoring system that takes into account five clinical variables from history and two variables from the electrocardiogram to create a score that translates into a probability of stress cardiomyopathy (InterTAK diagnostic score). A score ≥50 has a specificity of 95% for Takotsubo, whereas a score ≤31 suggests acute coronary syndrome with a specificity of 95%.
  9. In stress cardiomyopathy, LV function returns to normal within a few weeks; however, several complications may occur before the systolic function recovers, and the in-hospital mortality is as high as 5%. Complications include acute heart failure and cardiogenic shock, LV outflow tract obstruction, arrhythmias, systemic thromboembolism, and intramyocardial hemorrhage and rupture. The goal of therapy remains supportive, including management and prevention of complications and prevention of recurrence.
  10. Recurrences are common, 2-4% per year and up to 20% at 10 years; even after recovery of LV ejection fraction, in contrast to previous perception, patients who have experienced stress cardiomyopathy may experience symptoms such as fatigue (74%), shortness of breath (43%), chest pain (8%), palpitations (8%), and exercise intolerance in comparison with control subjects with no previous stress cardiomyopathy.

Clinical Topics: Acute Coronary Syndromes, Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Prevention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Stress

Keywords: Acute Coronary Syndrome, Adrenergic Agonists, Arrhythmias, Cardiac, Asthma, Cannabis, Cardiomyopathies, Chest Pain, Diabetes Mellitus, Dyspnea, Electrocardiography, Epinephrine, Heart Arrest, Heart Failure, Postmenopause, Pulmonary Disease, Chronic Obstructive, Risk Factors, Secondary Prevention, Sepsis, Shock, Cardiogenic, Stress Disorders, Traumatic, Stress, Psychological, Stroke Volume, Syncope, Systolic Murmurs, Takotsubo Cardiomyopathy, Thromboembolism, Ventricular Dysfunction, Right

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