Recurrent Broken Heart Syndrome Case Report and Review

Abstract

Tako-Tsubo cardiomyopathy (TTC), also known as apical ballooning syndrome, Broken heart syndrome or stress cardiomyopathy, is a reversible form of cardiomyopathy that is usually precipitated by a stressful event. The recurrence of this syndrome is not a very common phenomenon. We report a case of recurrent Tako-Tsubo cardiomyopathy (TTC) precipitated by emotional stress twice with good prognosis.

Introduction

Tako-Tsubo cardiomyopathy (TTC), also known as apical ballooning syndrome, Broken heart syndrome or stress cardiomyopathy, is a reversible form of cardiomyopathy that is usually precipitated by a stressful event. TTC is more prevalent in women, but has also been reported in men. Although apical ballooning is a typical feature of TTC, other variants have been described in literature. The recurrence of this syndrome is uncommon. We present a case of recurrent TTC and literature review.

Case Report

A 68-year-old woman with prior history of TTC in 2004 with complete resolution, presented to our hospital with chest pain that started while driving. She described her pain as chest pressure or heaviness, 7/10 in severity with radiation to left arm. The pain, although similar in character to her episode of TTC in 2004, was more severe in intensity with partial resolution with nitroglycerin. There were no recent medication changes and she mentioned being in perfect shape day prior to presentation.

The patient’s past medical history included hypertension, hyperlipidemia, TTC in 2004, and non-obstructive coronary artery disease by coronary angiogram in2004. It was thought at that time that destruction of her house by Hurricane was the probable trigger of TTC.

Patient’s mother and brother had recently been placed in a hospice and she also was under significant financial stress. She was a former smoker, but denied ever using recreational drugs.

Her home regimen included Alendronate Sodium 70 mg/week, Aspirin 81 mg/day, Diltiazem 240 mg/day, Lisinopril/Hydrochlorothiazide 20 /25 mg/day and Rosuvastatin 20 mg at night.

The patient noted allergies to Iodine and was pre-medicated during last cardiac catheterization with intravenous steroids.

In the emergency room her blood pressure was 128/76, heart rate 97 bpm, respiratory rate 12 to14, saturation 95% on room air. Physical exam was unremarkable and did not show signs of heart failure.

ECG showed dynamic ECG changes with ST depression in leads V4-V6 (see Image 1). She had mild elevated cardiac marker initially that increased subsequently to peak Troponin –I 1.29 NG/ML and peak CK-MB was 15.3 NG/ML. Her basic metabolic panel and complete blood count were within normal limits.

The patient had an echocardiogram prior to admission which showed a normal ejection fraction. Her ECG was normal before the current presentation, but had similar ECG changes during the previous TTC episode in 2004. (Image 2).

The patient was taken to cardiac catheterization lab for ongoing chest pain and found to have non-obstructive coronary artery disease. Her Echocardiogram showed mid-distal left ventricular severe hypokinesis with apical ballooning; left ventricular ejection fraction was severely depressed.

The patient was admitted to telemetry unit with gradual improvement in chest pain. The patient was started on low dose Beta blockers and ACE-inhibitors.

On Day 4 of hospitalization, the repeat echocardiogram showed complete recovery of the left ventricular ejection fraction, and the patient was discharged home.

Our case is an example of recurrent TTC with emotional stress trigger and rapid recovery. The patient has similar clinical presentation as well as ECG and echocardiographic changes, as her prior episode with the same outcome.

Discussion

Tako-Tsubo cardiomyopathy (TTC), also known as apical ballooning syndrome, Broken heart syndrome or stress cardiomyopathy, is a reversible form of cardiomyopathy that is usually precipitated by a stressful event. ^{1 3} In 2006 American Heart Association Classification of Cardiomyopathies, TTC is classified under acquired cardiomyopathy. ² The recurrence rate is estimated to be between 5-11%. ³  Currently, no factors have been found to be significantly associated with an increase in future recurrence, however, Angiotensin Converting Enzyme Inhibitor Use after the first episode has been associated with a lower incidence of future recurrence. ⁴

The syndrome was first described in the Japanese population in early 1990s and was given the name ‘Ta¬ko-Tsubo’ after the round bottomed and narrow necked Japanese fishing pot that resembles the characteristic shape of the left ventricle during systole in this condition. ^{2 3}  Although apical ballooning is a typical feature of TTC, other variants have been described in literature including the transient ballooning involving the mid-ventricle with hypercontractility of the apical and basal segments, called “apical sparing variant” or “mid-ventricular ballooning syndrome”. ⁵  The other reported variant is called “reverse Tako-Tsubo” cardiomyopathy with hyperdynamic apex and akinetic mid and basal segments. ⁶ The atypical TTC patterns seems to be possibly effecting younger age group as compared to the typical TTC and may have more benign course. ^{6 7} Involvement of right ventricle is also been reported. ⁸ The recurrent TTC can present in different pattern in same patient with variable severity. ⁹

Although, TTC has mostly been described in post-menopausal woman, mean age in the 60s, it is encountered in men as well. ^{3 10}  A wide variety of emotional and physical stress triggers have been reported including but not limited to death of a loved one, earthquakes, a heated argument, financial loss, a post-operative state, severe pain, acute exacerbation of asthma or chronic obstructive pulmonary disease, severe sepsis, thyroid disorder, Dobutamine stress test, subarachnoid hemorrhage, seizures and anorexia nervosa. ^{3 11 12 13 14 15}  In men physical trigger seems more common. ⁹ However, despite careful evaluation, a triggering event may not be identified in as many as one-third of patients. ^{3 11}

The pathophysiology of TTC still remains a matter of debate. The two main hypotheses are higher sympathetic tone and catecholamine toxicity.  A hypothesis of transient microcirculatory dysfunction is also being entertained. There has been a recent work by Suzuki H et al that showed alteration in brain blood flow during acute and recovery phase of TTC. ¹⁶

Clinically, TTC mimics acute coronary syndrome. It is estimated to be present in 1-2% of the cases with suspected acute coronary syndrome. ³ The most common symptom on presentation is retrosternal angina pain. Usually most of the patients undergo coronary angiography for the same reason. But recently, other imaging modalities including CT-coronary angiography (especially in recurrent cases) and cardiac MRI have also been used. ¹⁶ Cardiac magnetic resonance imaging (MRI) may be helpful in differentiating TTC, which is characterized by the absence of delayed gadolinium enhancement, from MI in which delayed subendocardial hyperenhancement is seen. ¹⁸ Cardiac MRI is also useful in differentiating TTC from myocarditis, which is characterized by delayed patchy hyper-enhancement. Other proposed modalities for TTC include speckle tracking with echo, I23BMIPP nuclear scan, BNP/Troponin ratio etc. ^{19 20}

TTC usually carries good prognosis and with complete recovery of myocardial function. Rarely, cases of complicated course have been reported including severe pump failure (Killip grade ≥ II), death, arrhythmia, conduction disorders and thromboembolic phenomena. ^{21 22} In small study of 107 patients with TTC in Japan showed a relationship between increased mortality and morbidity in patients with high levels of BNP and elevated leucocyte count. ⁶