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Lupine Publishers | Variant of Takotsubo Cardiomyopathy with Co-Existent Severe Coronary Artery Disease – A Case Report
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Lupine Publishers | Variant of Takotsubo Cardiomyopathy with Co-Existent Severe Coronary Artery Disease – A Case Report
AbstractTakotSubo Cardio Myopathy (TSCM) is a reversible form of cardiomyopathy characterized by transient left ventricular (LV) systolic dysfunction. Clinically often mimics acute myocardial infarction (AMI) with chest pain or breathlessness with electrocardiographic, cardiac marker(s) and LV wall motion abnormalities. In a third of patients, significant emotional or physical stress precedes the presentation. Often, these patients have normal epicardial coronaries or non-flow obstructive coronary disease. In most patients, LV function shows complete recovery within weeks with favorable outcomes. We report a unique case of TSCM following sudden emotional stress with typical transient apical ballooning; nevertheless, with severe co-existent triple vessel coronary artery disease and complete recovery of cardiac function on medical management.
Keywords: Tako-Tsubo Cardiomyopathy; Apical Ballooning; Coronary Artery Disease
Case Presentation73 year old hypertensive female admitted with sudden onset intermittent chest pains and shortness of breath (< 24 hours after the death of her granddaughters in a road traffic accident). There were no other significant past medical history of note and she never smoked. On arrival, she was tachycardic (heart rate 102) in sinus rhythm, normotensive and clinical examination was unremarkable. Her initial ECG (Figure 1) demonstrated sinus tachycardia with deep T wave inversions in both precordial and limb leads. Her bloods revealed high sensitivity Troponin-T of 395ng/L [normal reference range <15ng/L]. Her bedside echocardiogram revealed typical pattern of “apical ballooning” with severe hypokinesis in the anterior, lateral, septal and inferior walls of the left ventricle, sparing the basal walls. Her left ventricular systolic function was moderately impaired with an ejection fraction (EF) of 40% (Figure 2). Both her clinical presentation and echocardiographic appearances were highly suggestive of “Takotsubo Cardiomyopathy”. Nevertheless, she was conventionally treated for presumed “Non-ST elevation myocardial infarction (NSTEMI)” at the initial stages .
Figure 1: ECG on admission showing sinus rhythm with deep T-wave inversions in both precordial and limb leads.
Figure 2: 2-D Transthoracic echocardiogram (apical view) revealing typical apical ballooning with akinetic apical wall and preserved basal contraction of left ventricle.
Interestingly, her inpatient coronary angiogram revealed severe triple vessel coronary artery disease (CAD). This was evident by the presence of mild disease in the left main stem, severe mid vessel calcific LAD lesion, severe obtuse marginal (OM1) disease (Figure 3) and chronic total occlusion of her mid right coronary artery (Figures 4a & 4b). However, her distal RCA appears well collateralized from contralateral LC system. The left ventriculogram revealed moderately impaired systolic function with moderate mid to apical anterior and inferior wall hypokinesis, sparing the basal segments consistent with “apical ballooning”. Figure 5, LV in systole] Given her clinical presentation (albeit coronary artery disease and LV dysfunction), multidisciplinary consensus favored medical management as NSTEMI. Expectedly patient agreed and preferred this option too. She was discharged on appropriate treatment and secondary prevention. Interestingly, her Cardiac MRI (CMR) scan at 1-month post discharge showed near normalization of LV function with calculated EF 50% with no evidence of infarction or myocardial oedema or fibrosis. Thence, improvement in her LV function with no evidence of infarction on CMR confirms the diagnosis of Takotsubo Cardiomyopathy, despite her significant CAD. At 12-month follow up, patient still remains stable and symptom free; therefore, with plans to consider surgical coronary revascularization, if worsening symptoms on top of medical therapy [2,3].
Figure 3: Coronary angiogram of left system revealing severe mid calcific left anterior descending artery disease with contralateral collaterals to distal right coronary artery.
Figure 4a: Coronary angiogram of left system showing severe branch vessel disease in obtuse marginal branch of left circumflex artery.
Figure 4b: Angiogram of right coronary artery showing chronic total occlusion at the proximal segment.
Figure 5: Left ventriculogram at systole confirming the typical pattern of “apical ballooning” sparring the basal anterior and inferior segments.
DiscussionWith her established macrovascular risk factors for CAD (hypertension and age) and clinical presentation together with abnormalities in ECG, cardiac enzymes and echocardiogram, the working differential diagnoses were:
a. Acute Coronary Syndrome (ACS) i.e., Non-ST elevation myocardial infarction
b. TakotSubo Cardiomyopathy or transient apical ballooning syndrome or stress induced cardiomyopathy or broken heart syndrome.
Conventional electrocardiogram (ECG) still remains a costeffective important tool in the assessment of CAD. Nevertheless, its low sensitivity (<50%) and specificity (<80%) in predicting coronary disease still remains a major limitation. Clinicians mostly rely on surface ECGs to localize ischaemic myocardial territory (highly specific) prior to coronary revascularization of the culprit vessel(s) routinely in ACS patients. Clinically TSCM (typical LV appearance of Japanese “Octopus trap” with apical ballooning) often mimics AMI (chest pains, elevated cardiac enzymes and ECG changes) and is characterized by a transient left ventricular dysfunction. Though its incidence and prevalence doesn’t appear to have vastly changed over the years; however clinicians are more comfortable in diagnosing TSCM with better access to imaging (echocardiogram, Cardiac MRI) and invasive investigations (coronary angiogram) nowadays. The Revised Mayo Clinic Criteria suggested the presence of the following to confirm the diagnosis of TSCM [4,5].
a. Transient hypokinesis, akinesis or dyskinesis of the left ventricular mid segments with or without apical involvement– distinctively the extent of regional wall motion abnormalities observed will be beyond the distribution of single epicardial coronary vessel and with often a stressful trigger (but not always present). b. Absence of obstructive coronary artery disease or angiographic evidence of plaque rupture c. New electrocardiographic abnormalities or modest elevation in cardiac Troponin d. Absence of pheochromocytoma and myocarditis.
However, concomitant coronary artery disease (CAD) has been reported in 10 – 29% of patients who have been diagnosed with TSCM. In the International Takotsubo Registry (n=1750) 15.3% had some degree of coronary artery disease, although the extent of coronary artery involvement was not discussed in TSCM patients. The new Inter TAK diagnostic criteria highlight the clinical entity that TSCM can exist with concomitant CAD too (Table 1). Our patient meets with these diagnostic criteria for “Takotsubo Cardiomyopathy” together confirming that there was no evidence of infarction in her Cardiac MRI (no sub-endocardial late gadolinium enhancement in her LV or microvascular oedema) as well as complete recovery of her LV function rather than the initially presumed diagnosis of ACS .
Table 1: International Takotsubo Diagnostic Criteria (8).
The pathophysiology for the condition is not well completely understood yet; nonetheless, stress related acute catecholaminergic surge, multi-vessel epicardial coronary spasm, micro-vascular dysfunction, abnormal fatty acid metabolism, myocardial stunning, micro-infarction, direct catecholamine toxicity to myocytes and possible reperfusion injury were proposed as possible mechanisms. On top of the typical transient apical ballooning, other atypical variants including inverted or reversed, mid ventricular or localized have been reported in the literature. In the majority of cases of suspected TSCM, it is appropriate to perform coronary angiography to exclude an acute coronary syndrome given the overlap of symptoms. Also, there is little evidence to support the clinicians in the management of TSCM with co-existent coronary artery disease as highlighted in this case and the clinical dilemma encountered. Therefore, we believe long term follow-up would be really helpful in understanding the disease as well as their management and prognosis for these TSCM patients [7,8].
ConclusionWe report an unusual case of Takotsubo Cardiomyopathy with co-existent severe triple vessel coronary artery disease. This case illustrates the importance of good history taking and the judicious use of diagnostic imaging to arrive at a prompt diagnosis and appropriate management. With limited evidence in literature to support clinicians as how best to manage these patients with severe coronary artery disease with Takotsubo Cardiomyopathy, we highlight the need for International registries with long term follow-ups.
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Fired? Are you sure? OK.
Note 2: This happens in Sweden, where there's no at-will employment. Once an employee is past the initial 6 month probation period, you can't fire them without a cause, which also requires an established paper trail.
Note 3: I am not a native English speaker, and professional terms may be wrong. I'm happy to take any corrections.
So, my friend's father (since retired) was a mechanical engineer. He was around 55 when this happened and VERY experienced in his field. In fact, he had some skill sets that were close to unique to the extent that you might be able to replicate them, but at extreme costs - we're talking multiple people from multiple companies from multiple countries taking weeks if not months to get up to speed with specific projects to do the same things.
He was also a no bullshit kind of guy who did his job, did it well but also pointed out problems and expected others to point out problems to him. He was extremely solution-oriented and had no time for office politics or "keeping a positive attitude" at work. Basically, your every day grumpy older engineer who really knew his thing and always ready to help if you asked, but not very forthcoming in team building exercises and so on.
He also ran his own business on the side, doing minor projects and so on. As was required by his employer, he had reported this and was sure to not cause any conflicts of interests, so his employer knew and accepted this.
He was considered a valuable employee and got several awards (that he cared little for, but anyway) during his many years with this employer. By all accounts, they paid him well, respected his knowledge and accomodated his style and he returned the favour by working very hard and making sure to mentor younger and newly employed engineers to make them effective co-workers.
Then his firm was acquired by a larger firm, and a new management team installed. Initally, everyone was promised that things would remain the same, but with the new management came a new office culture. The new management pressured for unpaid overtime, for a more "American" corporate culture with cheering and clapping and so on. He considered it extremely cringe and refused to participate. His status as a long-standing and knowledgeable employee kept him safe for some time, before the new management realised that resistance to the new "culture" centered around him and started pressuring him to play along. When he did not, they turned increasingly hostile, realising that he held a lot of "soft power" in the company, having mentored a large percentage of the engineers and resistance to their leadership centering around him. They started ordering him to work overtime, he answered that he was on time with his projects and that if they had identified an emergency requiring overtime, they would have to bring it up with the union to negotiate the over-time and make sure it was an actual emergency - the contract with the union said no over-time unless in an emergency. They tried to force him to participate in the cheering and clapping by making it mandatory for him to attend and yelling at him to participate and he did but so unenthusiastically that the event turned even more cringe and people started laughing.
The workday turned more and more hostile, and he knew that things would come to head sooner or later. Being an experienced engineer and knowing how to document things, he already had his ducks in a row.
Then it finally happened - they caught him answering an e-mail for his side business on his work laptop, brought him in and fired him on the spot for theft of company resources. He sat at the conference table and looked the three managers in their eyes, one after the other and asked.
"Are you sure you want to do this?"
They all said yes.
"Are you REALLY sure you want to do this?"
He was escorted to his desk by security to leave his phone, his badge and his computer at the desk and then escorted out.
Once out of the building, he phoned his union representative, who immediately cancelled the firing, claiming there was no just cause, which meant that it would go to the labour board for arbitration. You see, the company had an IT policy that it was ok to use the company laptop for personal business, including a side business, as long as you were on a break and compliant with IT security protocols, and the company was aware of and had approved his side business. And he was on a break. Of course, he had his declaration of a side business (signed by his former manager) and the IT policy available and sent both to the union representative.
Then he called his lawyer and asked him to send the pre-prepared cease and desist on two patents he held - patents that were not that significant and nothing he could make any serious money out of since they were mostly for very specific things used by the solutions he designed and used at his employer's, but still his that he had brought with him into the employment and allowed the employer to use in exchange for a slightly higher pay (all duly documented in his contract, of course).
Then he went home for some vacation and tending his side business. He was always a man to prepare and had enough money saved up to last him for a good time, to the extent that he considered retiring entirely. My friend said he had two job offers from competitors that had looked to sniping him for some time within the week - basically as soons as they learned he was available. He was gracious, but declined, but offered them to consult with his side business, now that he had the time, which they eagerly accepted - at twice the hourly rate he had made at his earlier employer's.
His colleagues started ringing the day after for advice, since the projects he had managed could not go on without him, he was perfectly polite, but denied any information and help, saying he had left everything he had with management and to contact them, as he was no longer employed there. Several clients that phoned his private number were told the same thing. Since his private number was not on a public registry, he suspected that both colleagues and clients spent some time and/or money to find it.
It took two weeks before a manager phoned him and asked things. He politely declined to answer, got yelled at and replied with something like "I am sorry, you must have mistaken me for someone who works for you." and hung up.
This happened a few times, and the next week HR phoned him and stated the firing had been a mistake and he was welcome back to his job. He again politely declined, saying that he awaited the labour board's decision, but until then he was happy to consult for them. At six times his hourly pay (after taxes and adminstrative costs, of course). After a few days of wrangling and trying to negotiate, they had to accept. And then he sprung the patent issue on them, forcing them to pay for those too. Less than two and a half week after being fired he was back at his desk.
After roughly three months, the firing came to the labour board. The employer stated that they believed they had handled the issue correctly, but were still willing to offer my friend's father his position back, in the interest of "good will" and "reconciliation". My friend's father and the union simply stated that he was now employed elsewhere (his own company) and no longer available. The labour board ruled in my friend's father's and the unions favour, and he got the normal damages - 3 months pay damage and 24 months pay severance package, including pension and of course the lawyer costs of the union paid by the employer.
According to my friend, her father continued to work there until he retired, working 20 hours or so per week and 10-15 hours for other companies, making a pretty penny, continuing to charge them three times what he charged their competitors as an "arsehole tax".
The managers were not fired, but they were moved into their own group apart from the rest of the department when it came to bonus calculations and the costs of her father's consultancy fees and the costs of the labour board arbitration were budgeted there, meaning they were constantly over budget and thus ineligible for bonuses for several years, which was a decent percentage of the incentives at that company, making at least one of them quit.
My friend also said her father usually met any management complaints with a big shit-eating grin and "What are you going to do? Fire me?" after that.
Edit: Spelling corrections.