Takotsubo Cardiomyopathy: My Broken Heart Syndrome Experience

Ever hear of someone dying from a broken heart? The “broken heart syndrome,” also known as “Takotsubo cardiomyopathy,” or “stress induced cardiomyopathy,” or “apical ballooning syndrome,” has been increasingly recognized over the past 2 decades as a real entity. While not usually life-threatening (as far as we know), the incidence of sudden cardiac death is difficult to measure.

Sudden emotional upset (or other physical stress) can suddenly trigger a cascade of events, resulting in acute systolic heart failure with a characteristic regional wall motion pattern causing severe hypokinesis of the mid and apical myocardial segments with preservation of the basal segments. This results in “apical ballooning” of the left ventricle. The Japanese word “Takotsubo” refers to an octopus trap with a similar shape. The octopus can get in, but is not able to maneuver itself to turn around and get out.

The actual pathophysiology behind this unusual phenomenon remains unclear, but it has been hypothesized that a sudden catecholamine surge is the cause. Interestingly, a similar acute left ventricular dysfunction pattern can occur with cocaine intoxication. It is well known that cocaine causes a significant increase in the sympathetic nervous system. The difficult part about making the clinical diagnosis of a stress-induced cardiomyopathy is the major overlap in symptoms between this disorder and an acute coronary syndrome. The typical presentation is chest pains, acute shortness of breath and generalized weakness. Rhythm disturbances and hypotension can cause syncope, as well. Diaphoresis, palpitations and nausea are common. Sounds just like an acute MI, doesn’t it?

I can’t tell you how many times I have explained to a patient that they are having a heart attack from a thrombus in a coronary artery and we need to do an angiogram to open the vessel and restore blood flow, just to be eating my words later asking them to forget everything I previously said because their coronary arteries were normal on the angiogram ... they have Takotsubo cardiomyopathy!

Diagnosis of “Broken Heart Syndrome” 

Echocardiography is the best means to diagnosis the severe left ventricular systolic dysfunction and wall motion pattern present in a stress-induced cardiomyopathy. However, a coronary angiogram is usually needed to ensure that an acute coronary syndrome is not present, as there is significant clinical overlap between the presentations of these two markedly different conditions as mentioned above.

Here is a clip from an echocardiogram of a patient with a stress-induced cardiomyopathy:

 

Here is left ventricular angiography (LV gram) from a patient with a stress-induced cardiomyopathy:

The ECG pattern during a Takotsubo cardiomyopathy episode can vary from normal to ST depression, ST elevation or even a left bundle branch block. Cardiac biomarkers, including troponin and creatine kinase (CK) can be minimally or markedly elevated. The B-type natriuretic peptide is universally elevated, as well.

Some atypical variants have been described as well, which include a “reverse Takotsubo” where the basal segments of the heart become akinetic, while the cardiac apex is hypercontractile.

Treatment of “Broken Heart Syndrome”

The great news is that, universally, the left ventricular function returns to normal relatively quickly, from 1 to 4 weeks. The mainstay of treatment is beta-blockers and, at least temporarily, ACE inhibitors. There is no good clinical evidence to verify the reduction of cardiac endpoints, including cardiac death or recurrence of the cardiomyopathy, with these therapies. Diuresis may be needed if pulmonary congestion is present. Most clinicians will keep patients on beta-blockers for their lifetime, although again there is no definite evidence to support this approach.

When cardiogenic shock occurs from a stress-induced cardiomyopathy, there are two potential causes which are very important to distinguish: severe left ventricular systolic dysfunction and left ventricular outflow tract obstruction.

When the left ventricular systolic function is so severely reduced that hypotension and shock occur, inotropes such as dobutamine or milrinone are very helpful. These same drugs can actually worsen hypotension if the basal segments of the myocardium are so hypercontractile that they actually cause dynamic left ventricular outflow tract obstruction similar to that seen in hypertrophic obstructive cardiomyopathy (HOCM).

The stress is not always emotional upset

In my short cardiology career, I have seen a couple dozen cases of Takotsubo cardiomyopathy occur without any known deaths, although reports have shown ranges from 0% to 8% mortality. I had one patient who had a recurrent episode, but in most cases it occurred only once per patient. The trigger in one case was the death of a spouse and another the death of a pet. I recall one case that occurred after a urologic procedure and another in an elderly woman during a urinary tract infection. A handful had no identifiable trigger, which is interesting. Even though it is called “broken heart syndrome,” a majority of cases that I have seen have not occurred from emotional upset. Thus, the term stress-induced cardiomyopathy is more appropriate.

What triggers have you seen induce Takotsubo cardiomyopathy?

- by Steven Lome, DO, RVT