Broken Heart Syndrome

What a rare cardiomyopathy can teach us

• Emily Greenwald, BS, EMT-P, , Ben Usatch, MD, FAAEM, NREMT-P
• March 2009 JEMS Vol. 34 No. 3
• 2009 Mar 1

An ALS crew responded to a multi-vehicle collision with injuries. They found their patient to be a 68-year-old female, boarded and collared by a BLS crew on scene. The patient’s three-week-old compact sedan was struck several times, and although it sustained exterior damage, the interior compartment was without intrusion or damage other than airbag deployment. 

The patient’s only complaint was severe chest pain, described as 10/10, sub-sternal and pressure-like without radiation. She denied shortness of breath, palpitations, nausea, dizziness and diaphoresis. She told the EMTs that after the collision, she "jumped out of her car, afraid that it would catch fire" and developed the chest discomfort immediately after exiting the car. 

Her past medical history was significant for diabetes mellitus and coronary artery disease, with six stents placed during prior catheterizations. A rapid assessment revealed no apparent signs of trauma. A detailed examination of her thorax revealed no skin discoloration or redness from the seatbelt, no tenderness with palpation, and clear, equal breath sounds. These findings lowered the ALS crew’s suspicion of chest trauma as an explanation for the patient’s symptoms. 

Initial vitals obtained were pulse 72, respirations 12, BP 170/68, blood glucose 97 mg/dl. During a routine ECG, leads II and III demonstrated marked ST elevations. In light of the patient’s presentation and noted ECG abnormalities, the crew performed a 12-lead ECG, which displayed ST elevation in leads II, III, AVF, V4, V5 and V6. Reciprocal changes were appreciated in V2. The ECG suggested inferior-lateral myocardial infarction (MI) and, per protocol, the local hospital was notified for early catheterization lab activation. 

The patient regularly took a full dose of aspirin (325 mg), so no aspirin was administered during transport. After high-flow O and three sublingual nitroglycerin sprays, her pain persisted at 10/10. Morphine administration was withheld because of rapid arrival at the hospital. 

The emergency department physician reviewed the ECG and moved the patient to the cath lab well within the accepted 90-minute "door-to-balloon" timeframe. The catheterization revealed patent coronary arteries with no signs of high-grade occlusion. Significant akinesis of the inferior ventricles was appreciated, however, consistent with Takotsubo cardiomyopathy (transient left ventricular apical ballooning syndrome).

Discussion
"Takotsubu" means "octopus trap" in Japanese and describes the peculiar shape of the left ventricle (LV) in this unique clinical presentation. Takotsubo cardiomyopathy is characterized by a change in the pumping action of the LV where, rather than a coordinated squeeze efficiently ejecting blood into the aorta, the LV contracts in an exaggerated way—halfway down its length. By doing so, the LV "pinches" itself into two parts: a superior portion of normal pressure and normal wall stress and an inferior portion of high pressure and resulting high wall stress. This area of apical ballooning resembles a takotsubo—a trap with a narrow neck and round bottom that Japanese fishermen use to collect octopi. 

The syndrome accounts for about 2% of STEMIs in Japan and is seen predominantly in post-menopausal women who have some recent history of physical or emotional distress. It’s sometimes called "broken heart syndrome," because some cases seem to be brought on only by despair over an emotionally stressful event. Our patient described above clearly experienced a "stress bolus" when faced with the stress of the incident. 

Takotsubo cardiomyopathy masquerades as a STEMI and is a rule-out diagnosis requiring interventional cardiac imaging. Because of the relative downstream ischemia caused by the unusual motion of the LV, ST-segment elevation, depression, and/or T-wave inversions are often seen on 12-lead ECGs. Cardiac markers (CPK and Troponin I) are often elevated. The exact pathophysiology is still being investigated, but it appears that there’s some contributing combination of coronary vascular spasm and catecholamine cardiotoxicity.

The patient’s cardiomyopathy resolved within two to three weeks as is typical in almost all patients with this condition. Routine hospital management after diagnosis includes monitoring for symptoms of cardiogenic shock, heart failure and arrhythmias. It should be noted that the first case of LV rupture caused by Takotsubo was reported in 2004. Consequently, Takotsubo syndrome can be considered a rare but plausible cause of sudden death as a result of ventricular wall rupture. 

Cardiomyopathies 
Although Takotsubo cardiomyopathy may be one of the rarest cases that an EMS provider may encounter, cardiomyopathies are far more common. Cardiomyopathy literally means "heart muscle disease." As would be expected with any malfunction of the heart’s pumping mechanism, cardiomyopathies present the responding EMS crew with the potential for a critically ill patient. 

Cardiomyopathies are basically divided into two entities—restrictive and dilated. Restrictive, or infiltrative, cardiomyopathies are characterized by a heart of normal size that has lost its elasticity. This cardiac "stiffness" impedes the filling of the ventricles during diastole. This lack of preload markedly reduces filling pressures and cardiac output. The most common cause is myocardial scarring as a result of myocardial infarction. The ischemic tissue loses its stretching capability as healthy muscle is replaced by scar tissue. LV hypertrophy from longstanding hypertension is another common cause. In addition, there are genetic causes of restrictive cardiomyopathy brought about by mutational changes that affect the cellular structure of the myocardial wall. 

Dilated cardiomyopathy presents when the heart’s pumping action is impaired by intrinsic ventricular wall weakness. The LV becomes larger, allowing itself to fill with more blood, and the ventricle walls thicken in an attempt to increase strength of contraction. However, the heart can never quite reach the same stroke volume as a healthy heart, and the increase in the amount of myocardium inevitably becomes a liability.

Magnifying this effect, the peripheral arteries constrict in an attempt to maintain BP despite a weakened heart, thus paradoxically increasing afterload and the work of the LV. As a result, the increased inefficiency of the LV causes blood to back up. This high pulmonary blood pressure stretches the LV cavity further, resulting in a thick-walled, oversized heart that’s even more inadequate and prone to lethal complications. Coronary artery disease, hypertension and valve dysfunction are all known causes of dilated cardiomyopathy. It’s important to note that the majority of dilated cardiomyopathies are idiopathic. 

Although some cardiomyopathies are of unknown etiology, alcohol has been shown to be the most frequent identifiable cause of heart muscle disease. It’s been suggested that minimal amounts of alcohol can have a cardio-protective effect, but large sustained amounts of alcohol present an unknown mechanism of damage to myocardium. Men aged 35–55 who, for at least 10 years have derived approximately 30–50% of their caloric intake from alcohol, are at most risk for developing alcoholic cardiomyopathy.

As Americans age, EMS practitioners will encounter patients with cardiomyopathies with increasing frequency. The first clue to this diagnosis may lie in the pile of pill bottles on a patient’s dresser. A medication list that includes calcium channel blockers, beta-blockers, digoxin, antiarrythmics or ace inhibitors should suggest a patient who’s harboring a cardiomyopathy. 

Complications encountered in patients with sick heart muscles are predictable. With the stretching of tissue responsible for electrical conductivity, both atrial and ventricular arrhythmias are common, often requiring rapid prehospital interventions (both pharmacological and electrical). Surviving patients will usually leave the hospital with an internal pacemaker/defibrillator placed to address recurring arrhythmia. A decrease in cardiac output can cause worsening venous congestion and resulting signs of fluid overload (presenting as pulmonary edema, peripheral edema and jugular-venous distension). This often requires emergent use of diuretics, nitrates, afterload reduction and respiratory support (hopefully CPAP) by responding medics. This presentation can be severe, sudden and described as "flash pulmonary edema."

In its most extreme form, cardiomyopathies may manifest as cardiogenic shock, requiring prehospital pressor and ventilatory support and emergent cardiac interventional procedures in the hospital. Because of this, disposition decisions with these patients are a critical part of the prehospital provider’s management, as these patients are often best served in facilities with full cardiac services available.

Conclusion
Takotsubo is a rare but fascinating cardiomyopathy that EMS practitioners may encounter. General cardiomyopathies are far more common and are likely to be seen on a regular basis in the field. Aggressive management of these often-critical patients can be life saving. Early in the patient encounter, an IV should be placed to allow fluid resuscitation and medication administration. High-flow oxygen should be administered at a minimum with early consideration of CPAP or intubation in the face of severe dyspnea. Early and continual cardiac monitoring and pulse oximetry, augmented with frequent BP checks, will assist in trending patient status and arrhythmia recognition. Aggressive management and close monitoring are the keys in caring for this challenging group of patients.  JEMS

Emily Greenwald , BS, EMT-P, is a full-time paramedic in the suburbs surrounding Philadelphia. She begins medical school this summer. Contact her at  emilysmile@aol.com.  

Ben Usatch , MD, FAAEM, NREMT-P, has been involved in EMS for more than 25 years. He serves as the medical director for community- and hospital-based units in the Philadelphia area. He works as an attending physician and the EMS director at the Lankenau Hospital in Wynnewood. Contact him at  docrags@drexel.edu.   

References

1. Merli, E, Sutcliffe S, Gori M: "Tako-tsubo cardiomyopathy: New insights into the possible underlying pathophysiology." European Journal of Echocardiography. 7(1):53–61, 2006. 
2. Gianni M, Dentali F, Grandi A, et al: "Apical ballooning syndrome or takotsubo cardiomyopathy: A systematic review." European Heart Journal. 27(13):1523–1529, 2006. 
3. Akashi YJ, Tejima T, Sakurada H, et al: "Left ventricular rupture associated with Takotsubo cardiomyopathy." Mayo Clinic Proceedings. 79(6):821–824, 2004. 
4. Metzl MD, Altman EJ, Spevack DM, et al:. "A case of takotsubo cardiomyopathy mimicking an acute coronary syndrome." Nature Clinical Practice Cardiovascular Medicine. 3(1):53–56, 2006.
5. Davidson DM: "Cardiovascular effects of alcohol." Western Journal of Medicine. 151(4)430–439, 1989.  

Check out a video of Takotsubo cardiomyopathy in action.

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