The Journal for Nurse Practitioners
Volume 5, Issue 10 , Pages 767-772, November 2009

Apical Ballooning Syndrome: The Broken Heart Syndrome

  • Joanne L. Thanavaro

      Affiliations

    • Joanne L. Thanavaro, DNP, APRN-BC, ACRN-BC, DCC, is an associate professor of nursing at the St. Louis University School of Nursing in St. Louis, MO.
    • ,
  • Kristin L. Thanavaro

      Affiliations

    • Kristin L. Thanavaro, MD, is a resident in internal medicine at the University of Maryland Medical Center in Baltimore.

Article Outline

Abstract 

Four case studies of apical ballooning syndrome are reported to demonstrate the typical presentation, specific cardiac imaging, and clinical outcomes. Most patients are women and present with symptoms of acute coronary syndrome precipitated by emotional distress or physical stressors. Electrocardiograms (ECGs) may vary from nonspecific ST-T changes to full-blown manifestations of ST segment elevation infarct. Cardiac markers demonstrate minimal or moderate elevations. Basal hypercontractility and apical ballooning are classical cardiac imaging and coronary arteries are usually normal. Patients are expected to have an uneventful hospital course and complete left ventricular function recovery, and most patients do not have any recurrences.

Keywords:  apical ballooning syndrome , broken heart syndrome , emotional distress , left ventriculogram , physical stressor

 

Apical ballooning syndrome (ABS) is a reversible, transient cardiomyopathy, present in about 2% of patients presenting with an acute coronary syndrome (ACS).1, 2 ABS was first described in the Japanese population in the 1990s; this disease entity was named Tako-Tsubo cardiomyopathy because of the way the left ventricle appeared on ventriculogram. The hypokinetic mid-ventricle and apex with a rounded bottom and narrow neck in systole resemble the traditional Japanese octopus trap for which it was named.1 ABS is also referred to as “stress” or “ampulla” cardiomyopathy, and as “the broken heart syndrome.”

This cardiomyopathy is unique in that it is often precipitated by emotional or physical stressors including natural disasters, the sudden death of a loved one, serious medical illness, and noncardiac surgery.1, 3 There have also been reports of professional sporting events triggering ABS.4 Because most patients in early publications were Japanese, ABS was initially thought to be a geographically isolated phenomenon with Asian descents.5 This cardiomyopathy has recently been documented in European, Australian, and North American patients, and the lack of prior reports in the western world may have simply been due to the lack of awareness of the disease entity.5, 6 ABS is an emerging and under-recognized cardiomyopathy mimicking ST elevation infarct, and a high index of clinical suspicion is needed to correctly identify this transient cardiomyopathy.

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Epidemiology 

Most patients with ABS are postmenopausal women with a preceding acute emotional or physical stressor. In some cases, however, no trigger event is identified and the cause is idiopathic. In a patient with suspected ABS, a thorough medical and social history is necessary to identify possible triggering events. Prasad et al1 found that about 90% of cases of ABS were in women, with the median age being 58 to 75 years. The reason for this female predominance is unknown. Leading theories have suggested that withdrawal from estrogens after menopause and its effects on microcirculation could play a role in the development of ABS.1, 5

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Etiology 

The pathophysiology of ABS remains controversial. Originally, epicardial vasospasm was thought to be the cause of the cardiomyopathy, but studies and angiography failed to confirm this process.1, 2 More recently, catecholamines and microvascular ischemia have been proposed mechanisms of ABS because these neurogenic particles are often elevated with severe emotional and physical stress. It is believed that increased catecholamines stun the myocardium and lead to the cardiomyopathy.7 Other conditions with elevated catecholamines, such as pheochromocytoma, have been associated with a decreased ejection fraction.8, 9 There have also been other descriptions of myocardial stunning in response to systemic conditions; in particular, subarachnoid hemorrhage and status asthmaticus have been implicated in cases of left ventricular dysfunction in response to a neurally-mediated trigger.10, 11

Wittstein et al2 evaluated 19 patients with ABS after severe emotional stress and found that 13 of these patients had supraphysiologic levels of catecholamines and stress-related neuropeptides. When compared to patients with myocardial infarction, the ABS patients had initial levels of plasma catecholamines that were multiple times higher than the other group.2 This study hypothesizes that microvascular ischemia resulting from sympathetically-mediated spasm could lead to the myocardial stunning. Cardiac microvasculature has been shown to be abnormal during angiography; Bybee et al12 found that TIMI (thombolysis in myocardial infarction) frame counts were prolonged in the 3 main epicardial arteries, indicating slow coronary blood flow during acute presentation of the apical ballooning syndrome. While the exact etiology of ABS remains unknown, theories of myocardial stunning and microvascular ischemia are the latest leading hypotheses. If these mechanisms do in fact play a role in ABS, this could explain patients' clinical improvement with beta-adrenergic blocking agents. By decreasing the amount of catecholamine stimulation on the myocardium, the deleterious effects can be blocked.

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Presentation 

Patients with ABS typically present similarly to those with an acute myocardial infarction, with chest pain or dyspnea, usually following an emotional or physical stressor. In most cases, patients are hemodynamically stable, although hypotension and pulmonary edema can be present. Most patients have an elevation in cardiac biomarkers that follow the rise and fall pattern typically associated with acute myocardial infarction.5 However, these elevations are only modest, and not typically as high as those seen in an acute MI. The most common electrocardiographic presentation is ST elevation, most often in precordial leads.5 This ST elevation is transient, so it is not always observed and documented.1 The ECG can also reveal new bundle branch blocks, Q waves, or nonspecific ST-T changes.5

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Diagnosis 

Bybee et al5 recently proposed 4 main diagnostic criteria for ABS including: transient apical and mid-left ventricular akinesis/dyskinesis beyond a single epicardial vascular distribution; absence of obstructive coronary disease or acute plaque rupture; new ST elevation or T wave inversion on ECG; and absence of recent head trauma, intracranial bleeding, pheochromocytoma, myocarditis, or hypertrophic cardiomyopathy. Precise diagnostic criteria for ABS has not been established and the diagnosis is often made in the setting of a clinical presentation of ACS with the characteristic apical ballooning of the left ventricle and lack of coronary artery disease.3, 5, 13 In most cases, a physical or emotional stressor acts as a trigger, but this is not always present.1, 3

Cardiac Imaging and Coronary Anatomy 

Because patients with the apical ballooning syndrome typically present with ST elevation and elevated cardiac biomarkers, they are generally taken to the cardiac catheterization laboratory. Upon visualization of the coronary arteries, there is either no detectable obstruction or mild, non-obstructing coronary vessel disease.5 On left ventriculogram, the apex and mid-ventricle are hypokinetic, while the pumping function of the base is spared. This produces the typical “apical ballooning” after which the disease is named. Patients have a depressed left ventricular ejection fraction, which typically resolves over a few days to weeks.

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Treatment 

The cardiomyopathy associated with ABS is transient and usually resolves within a few weeks. During the acute phase, the patient should be treated with the same therapies used to treat myocardial ischemia and cardiomyopathy, including aspirin, beta-blockers, angiotensin converting enzyme inhibitors (ACEI), anticoagulation, and continuous telemetry monitoring.1 Although ACEI and beta-blockers are used to treat ABS, there has not been any clinical data showing that either of these agents improves survival or prevents recurrence.2 However, studies have anecdotally shown that patients treated with these agents tend to improve, and the use of this pharmacotherapy has continued in clinical practice.

Congestive heart failure can be a complication of ABS and should be treated accordingly with diuresis, inotropic agents, and an intra-aortic balloon pump, if needed. Other complications that have been described include ventricular free wall rupture, mitral regurgitation, and ventricular and atrial arrhythmias.1, 14 In addition, the hypokinesis of the left ventricle could lead to formation of a mural thrombus; anticoagulation with warfarin should be considered on a case-by-case basis to prevent thromboembolism.

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Prognosis 

Death from ABS is rare; a recent study by Gianni showed that in-hospital mortality was 1.1%, with a recurrence rate of 3.5%.2, 13 Notably, prognosis is worse in patients in which a specific emotional trigger is identified and there is a possibility for a recurrence of cardiomyopathy during a similar emotional stress.1 Improvement in ejection fraction is usually seen with a week or two of the diagnosis and should be confirmed by a repeat echocardiogram 4 to 6 weeks later.1 Because the natural progression of the ABS is unknown, patients should have yearly cardiovascular follow-ups to determine the necessity and efficacy of their treatment regimen.

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Case Studies 

ABS normally has a typical presentation, variation of ECGs and cardiac markers, specific cardiac imaging, and good clinical outcome, as illustrated in the following case studies.

Case Study 1 

MM is a 51-year-old woman who presented to an emergency department with an episode of chest pain after severe emotional stress from losing a pet dog that she had for 13 years. Her chest pain was located at the mid sternal area and radiated to her neck and jaw with associated diaphoresis. Her symptoms were relieved with sublingual nitroglycerine and oxygen supplement in the emergency department.

Coronary risk factors included a smoking history, hyperlipidemia, and a family history of coronary heart disease. She also had a history of benign palpitations treated with verapamil SR and hypothyroidism with thyroid hormone supplement. Cardiac examination revealed normal heart sounds without murmurs or gallops.

The admitting ECG showed anteroseptal infarct and T-wave abnormalities without definite ST segment elevation (Figure 1A) and the troponin was elevated to 10 ng/mL. The left ventriculogram demonstrated apical ballooning with an appearance of Tako-Tsubo cardiomyopathy and an ejection fraction of 40% (Figure 1C). Coronary angiogram revealed no obstructive coronary disease (Figure 1D and 1E).

Conservative medical treatment was recommended with beta blocker, ACEI, and ASA, and the patient had an uneventful recovery. Her 2D echocardiogram 6 months later showed complete recovery of left ventricular function without residual apical ballooning. She has since discontinued all her cardiac medications and has been free of cardiac events for 72 months.

Case Study 2 

NW is a 71-year-old woman who presented to the emergency department with an episode of epigastric abdominal discomfort associated with nausea and vomiting. She was found to have markedly elevated pancreatic enzymes, compatible with acute pancreatitis. She also had vague chest discomfort with electrocardiographic changes of acute anterior myocardial infarction with ST-T abnormalities (Figure 2A) and peak troponin elevation of 5.5 ng/mL.

Coronary risk factors included hyperlipidemia and diabetes mellitus. The patient had a history of hypothyroidism with thyroid supplement and cholelithiasis with cholecystectomy 12 years prior. Cardiac examination revealed normal heart sounds with systolic murmur and without gallops.

The left ventriculogram demonstrated apical ballooning with an appearance of Tako-Tsubo cardiomyopathy and an ejection fraction of 30% (Figure 2C). Coronary angiogram revealed no obstructive coronary disease (Figure 2D and 2E).

Conservative medical treatment was recommended with beta blocker, ACEI, and ASA, and the patient had an uneventful recovery. She was subsequently found to have pancreatic carcinoma and eventually expired without having any additional cardiac evaluation.

Case Study 3 

JB is a 67-year-old woman who presented to the emergency department with an episode of severe chest pain associated with nausea and without shortness of breath or diaphoresis. Her symptoms were not relieved with routine antianginal therapy and she was subsequently placed on intravenous 2b3a agent (Integrelin) without recurrent chest pain.

Her past medical history was significant for a history of transient ischemic attack, requiring a hospital admission 1 week prior. She had a normal left ventricular function on her 2D echocardiogram during that hospital admission. Her coronary risk factors included hypertension, diabetes mellitus, and hyperlipidemia. Cardiac examination revealed normal heart sounds without murmurs or gallops.

The admitting ECG showed anterior infarct without ST segment elevation (Figure 3A) and an elevated troponin of 3.6 ng/mL. The left ventriculogram demonstrated apical ballooning with an appearance of Tako-Tsubo cardiomyopathy and an ejection fraction of 35% (Figure 3C). Coronary angiogram revealed no obstructive coronary disease (Figure 3D and 3E).

Conservative medical treatment was recommended with beta blocker, ACEI, and ASA, and the patient had an uneventful recovery. Her 2D echocardiogram 2 months later showed complete recovery of left ventricular function without residual apical ballooning. She has been doing well and has been free of cardiac events for 12 months.

Case Study 4 

LC is a 74-year-old woman who presented to the emergency department with an episode of chest pain, which persisted for several hours in spite of appropriate antianginal treatment.

The patient had no history of prior illness or recent emotional distress. Her coronary risk factors included hypertension and hyperlipidemia. Cardiac examination revealed normal heart sounds without murmurs or gallops.

The admitting ECG showed acute anteroseptal infarct with minimal ST segment elevation (Figure 4A) and troponin peak of 0.63 ng/mL. The left ventriculogram demonstrated apical ballooning with an appearance of Tako-Tsubo cardiomyopathy and an ejection fraction of 35% (Figure 4C). Coronary angiogram revealed no obstructive coronary disease (Figure 4D and 4E).

Conservative medical treatment was recommended with beta blocker, ACEI, and ASA, and the patient had an uneventful recovery. Her 2D echocardiogram 7 months later showed persistent left ventricular dysfunction without significant recovery in spite of cardiomyopathy treatment with beta blocker, unloading therapy, and aldosterone antagonist. She has also been doing well without further cardiac events for 11 months.

Discussion of Case Studies 

All 4 patients are postmenopausal women and 3 of them experienced a preceding acute emotional or physical stressor. Case study 1 lost her pet dog; case study 2 was suffering from acute medical illness with severe acute pancreatitis; and case study 3 had a preceding neurogenic event with possible transient ischemic attack 1 week prior to her presentation. Case study 4 had no apparent acute emotional or physical stressor. All patients had symptoms suggestive of acute coronary syndrome, various electrocardiographic presentations, mild to moderate cardiac marker release, and classical cardiac imaging, including apical ballooning and normal coronary arteries. None of these 4 patients suffered any significant cardiac rhythm or hemodynamic consequences from the acute event and made an uneventful recovery from their myocardial injury. Two patients had complete left ventricular function recovery without residual apical ballooning (case study 1 and 3) and 1 patient did not show any left ventricular function improvement (case study 4). The other patient did not survive her other subsequent medical illness long enough to have additional cardiac follow-up (case study 2). The 3 survivors demonstrated no residual cardiac symptoms with (case study 4) or without medications (case study 1 and 3) and had no recurrences for 11 (case study 4), 12 (case study 3), and 72 months (case study 2).

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Conclusion 

Nurse practitioners (NPs) may encounter patients with apical ballooning syndrome during acute care in the emergency department or in the intensive care unit, as well as in the office setting, during their early recovery phase. An understanding of this transient cardiomyopathy will help NPs make the correct diagnosis and provide proper care for patients in the acute care setting, as well as explain the possible causes to patients and their families. Reassurance of a potentially good outcome, the need for only short-term medication therapy, and a low incidence of recurrence may enhance patients' recovery. Early assessment of left ventricular function recovery during outpatient follow-up is important in adjusting the medical treatment for patients with apical ballooning syndrome.

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References 

  1. Prasad A , Lerman A , Rihal CS . Apical ballooning syndrome (Tako-Tsubo or stress cardiomyopathy): a mimic of acute myocardial infarction . Am Heart J . 2008;155:408–417
  2. Wittstein IS , Thiemann DR , Lima JAC , et al.   Neurohumoral features of myocardial stunning due to sudden emotional stress . NEJM . 2005;352:539–548
  3. Sharkey SW , Lesser JR , Zenovich AG , et al.   Acute and reversible cardiomyopathy provoked by stress in women in the United States . Circulation . 2005;111:472–479
  4. Witte DR , Bots ML , Hoes AW , Grobbee DE . Cardiovascular mortality in Dutch men during 1996 Europen Football Championship: longitudinal study . BMJ . 2000;7276:1552–1554
  5. Bybee KA , Kara T , Prasad A , et al.   Systematic review: transient left ventricular apical ballooning: a syndrome that mimics ST elevation myocardial infarction . Ann Intern Med . 2004;141:858–865
  6. Cangella F , Medolla A , De Fazio G , et al.   Stress induced cardiomyopathy presenting as acute coronary syndrome: Tako-Tsubo in Mercogliano, Southern Italy . Cardiovasc Ultrasound . 2007;5:1–6
  7. Akashi YJ , Nakazawa K , Sakakibara M , Miyake F , Musha H , Sasaka K . 123I-MIBG myocardial scintigraphy in patients with “TakoTsubo” cardiomyopathy . J Nucl Med . 2004;45:1121–1127
  8. Otsuka M , Kohno K , Itoh A . Periodic fluctuation of blood pressure and transient left ventricular apical ballooning in pheochromocytoma . Heart . 2006;92:1837
  9. Sanchez-Recalde A , Costero O , Oliver JM , Iborra C , Ruiz E , Sorbino JA . Images in cardiovascular medicine: pheochromocytoma-related cardiomyopathy: inverted Takotsubo contractile pattern . Circulation . 2006;113:e738–e739
  10. Kono T , Morita H , Kuroiwa T , Onaka H , Takatsuka H , Fujiwara A . Left ventricular wall motion abnormalities in patients with subarachnoid hemorrhage: neurogenic stunned myocardium . J Am Coll Cardiol . 1994;24:636–640
  11. Yamanaka O , Yasumasa F , Nakamura T , Ohno A , Endo Y , Yoshimi K , et al.   “Myocardial Stunning”-like phenomenon during a crisis of pheochromocytoma . Jpn Circ J . 1994;58:737–742
  12. Bybee KA , Prasad A , Barsness G , Wright RS , Rihal CS . Clinical characteristics, outcomes and impaired myocardial microcirculation in patients with transient left ventricular apical ballooning syndrome: a case-series from a U.S. Medical Center . Am J Cardiol . 2004;94:343–346
  13. 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;27:1523–1529
  14. Porodi G , Del Pace S , Salvadori C , Carrabba N , Olivotto I , Gensini GF . Tuscany Registry of Tako-Tsubo Cardiomyopathy. Left ventricular apical ballooning syndrome as a novel cause of acute mitral regurgitation . J Am Coll Cardiol . 2007;50:647–649

 In compliance with national ethical guidelines, the authors report no relationships with business or industry that would pose a conflict of interest.

PII: S1555-4155(08)00604-1

doi:10.1016/j.nurpra.2008.10.016

The Journal for Nurse Practitioners
Volume 5, Issue 10 , Pages 767-772, November 2009

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