Reverse Takotsubo cardiomyopathy associated with tonic clonic seizure: a rare form of Takotsubo cardiomyopathy
- 1 Cardiology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
- 2 Critical Care, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
- 3 Cardiology, University Hospitals Birmingham, Birmingham, West Midlands, UK
- 4 Cardiology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, West Midlands, UK
- Correspondence to Dr Usman Ali; usman.ali8@nhs.net
Abstract
A woman in her 30s presented with generalised tonic clonic seizure secondary to known pilocytic astrocytoma. This seizure activity resolved spontaneously after 5 min. On clinical assessment, she was neurologically stable and further neurological investigations did not reveal progression of previously recognised pilocytic astrocytoma. Incidentally, she was found to have an elevated troponin, which significantly increased on serial assessment. ECG was unremarkable and echocardiography revealed regional wall motion abnormalities involving basal segments of the left ventricle with apical sparing. She underwent cardiac MRI, which confirmed the presence of regional wall motion abnormalities seen on echocardiography; however, there was no evidence of myocardial oedema or late gadolinium enhancement. Subsequently, she had an invasive coronary angiogram with intravascular ultrasound which ruled out acute coronary plaque event and coronary dissection. In view of the above, a diagnosis of reverse Takotsubo cardiomyopathy was made. This is a relatively rare entity characterised by the presence of akinesia/hypokinesia in the basal segments with preserved apical contractility; often seen in younger patients.
Background
Elevated troponin associated with multiple conditions include both cardiac and non-cardiac causes.1 It is imperative to identify underlying aetiology to guide the most appropriate management. We describe a case of an elevated troponin-associated cardiac regional wall motion abnormalities following a generalised tonic clonic seizure. The specific pattern of regional wall motion abnormalities identified is a rare clinical entity known as reverse Takotsubo cardiomyopathy (RTCM). Often precipitated by stress, RTCM has contrasting regional wall motion abnormalities compared with the well-known Takotsubo cardiomyopathy (TCM) and occurs more commonly in younger patients.2
Case presentation
A woman in her 30s, with a previous history of right thalamic subependymal pilocytic astrocytoma, presented with a generalised tonic clonic seizure while watching television. Seizure activity was short lived but lead to urinary incontinence and terminated spontaneously. This was followed by a period of postictal confusion that lasted around 30 min after which the patient made a complete recovery. She did not have a fever, headache, chest pain, shortness of breath or any focal neurological symptoms. She was a non-smoker, consumed 20 units of alcohol per week and lived with her husband and two children.
The patient had her first seizure 7 years ago, following which she had further investigations and a biopsy in 2005 revealing grade 1 right thalamic subependymal pilocytic astrocytoma. In 2009, she was found to have aqueductal stenosis and underwent endoscopic third ventriculostomy. Surveillance scans since then have shown no progression. She also suffered from bulimia nervosa in the past and had polycystic ovarian syndrome. Two months prior to her presentation, she gave birth via caesarean section; she was not breast feeding at the time of presentation. Following her recent caesarean section, the patient was started on penicillin for a wound infection from which she recovered well. This was in addition to levetiracetam which she had been taking on long-term basis. One month prior to hospital admission, she had the COVID-19 vaccination with AZD 1222. This was well tolerated with no reported side effects. Additionally, she had a progesterone only hormonal implantation for contraception. There was no history of any other new medications or use of recreational drugs. There were no known risk factors for ischaemic heart disease.
On arrival, her blood pressure was 136/89 mm Hg, heart rate 95 bpm, respiratory rate 17/min, oxygen saturations of 95% on room air and she was apyrexial. Her Glasgow Coma Scale(GCS) score was 15/15 with bilaterally equal and responsive pupils and she did not have any focal neurological signs on her central nervous system examination. Cardiovascular, respiratory and abdominal examinations were unremarkable.
Initial investigations and neurosurgical plan
Laboratory investigations were essentially unremarkable with normal blood sugars, electrolytes and metabolic profile. High sensitivity troponin I, done in the emergency department, was elevated at 35 ng/L (reference range <16 ng/L). Serial monitoring of high sensitivity troponin I revealed an interval rise to 209 ng/L and 225 ng/L. ECG revealed normal sinus rhythm with no acute ischaemic changes or arrhythmias (figure 1).
ECG.
Initial imaging investigations included a chest X-ray, which did not show any focal consolidation or cardiomegaly. She underwent a CT head with contrast, which revealed stable appearance of the ventricles and right thalamic region. Patient was reviewed by the neurosurgical team who suggested an outpatient EEG, MRI scan, levetiracetam dose up-titration and follow-up with an epilepsy nurse. Given the significant interval rise in the troponins, there was concern regarding the possibility of myocardial ischaemia and injury. Due to this, she was admitted under the care of the cardiology team.
Differential diagnosis
Differentials for this presentation and elevated troponin include:
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Troponin elevation secondary a complicated seizure—this is often seen following seizures, however, is more common in patients with vascular risk factors.3 This was initially thought to be the most likely cause.
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Spontaneous coronary artery dissection (SCAD)—a non-atherosclerotic cause of coronary artery obstruction. This can be seen in women without any risk factors for ischaemic heart disease. It can be present in patients across a wide range of age groups but is commonly seen in middle-aged women.
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Acute coronary syndromes—this needs to be considered in any patient with clinically significant troponin rise.
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TCM—given she had a recent stress in the form of an epileptic seizure associated with troponin rise, this was also considered as a possible cause of her troponin rise.
Cardiac investigations
Echocardiogram revealed moderate left ventricular systolic dysfunction with akinetic basal to mid segments (video 1).
Cardiac MRI confirmed the regional wall motion abnormalities, as evident on echocardiography, with minimal oedema but no convincing enhancement to confirm a myocardial infarction (figure 2).
Cardiac MRI.
Coronary angiogram with intravascular ultrasound revealed normal coronary arteries with no evidence of SCAD (figure 3). Left ventriculogram further confirmed basal segment contractile impairment in keeping with RTCM (video 2).
Coronary angiogram with intravascular ultrasound (IVUS).
Treatment
Patient was started on a small dose of ACE inhibitor (ACEi) and beta-blocker. Levetiracetam was titrated as per neurosurgical advice.
Outcome and follow-up
Follow-up was arranged with repeat echocardiography performed 3 months after the initial presentation. This revealed complete recovery of left ventricular systolic function; as a result, the ACEi and beta-blocker were subsequently stopped.
Discussion
TCM also referred to as ‘broken heart syndrome’ is a type of stress cardiomyopathy. It is characterised by the presence of transient akinesia/hypokinesia in left ventricular mid segments, with or without apical involvement, in patients with either troponin rise or ECG changes who have unobstructed coronary arteries.4 However, atypical patterns of left ventricular impairment also exist.2 It is more common in postmenopausal women and usually presents with chest pain and dyspnoea.5 6
Pathophysiology appears to be multifactorial and usually involves a preceding physical or emotional stress. On occasions, the underlying stressor may not be obvious or easily identifiable. Excessive sympathetic stimulation and catecholamine excess have been implicated as the probable underlying mechanisms due to which myocardial stunning and cardiac dysfunction ensue in these patients.7
ECG changes in the form of ST elevation are present in 56%–100% of the patients according to different studies.5 6 Elevation in cardiac enzymes is seen in 56% of the patients.5 Due to its similarity with acute myocardial infarction, virtually all of these patients undergo coronary angiography and the hallmark of this disease is absence of clinically significant or obstructive coronary artery disease on coronary angiography.5
Various patterns of contractile dysfunction exist in patients with stress cardiomyopathies. In typical TCM, apical ballooning of the left ventricle is seen on cardiac imaging due to the akinesia/dyskinesia of mid to apical segments with preserved contractility of basal segments.4 8 In RTCM, there is hypokinesia mainly of the basal segments with apical sparing.9 Ramaraj and Movahed identified that among the patients presenting with TCM, 23.3% of the patients have reverse-type TCM.2
As opposed to the classical TCM, RTCM is commonly seen in young individuals and is almost always associated with preceding stress.2 Individual cases have been reported in the past after consumption of an energy drink as well.9 In our case report, we demonstrate that seizure activity can also act as an inciting factor for the development of RTCM. Moreover, it is not necessary for these patients to have symptoms of chest pain or shortness of breath which are commonly seen with TCM. Furthermore, classical ECG changes seen in TCM might be absent in the patients with RTCM.
There is no specific treatment for patients presenting with TCM. Management is predominantly supportive and aims at treating complications, for example, insertion of intra-aortic balloon pump in the patients presenting with cardiogenic shock.10 Potential complications of TCM include cardiogenic shock, mitral regurgitation, ventricular rupture and arrhythmias.11
Learning points
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Recognition of reverse Takotsubo cardiomyopathy (RTCM) as a potential clinical diagnosis in the patients with typical patterns of regional wall motion abnormalities for RTCM.
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Seizure can precipitate RTCM and this should be kept in mind as a possible cause of troponin rise before attributing it to seizure alone.
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RTCM can involve younger patients as opposed to elderly individuals seen in TCM.
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Presence of chest pain and shortness of breath is not an integral feature of RTCM.
Ethics statements
Patient consent for publication
Footnotes
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Twitter @drusmanali1
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Contributors UA—joint first author—conceptualisation, data collection, article writing and proof-reading. AK—joint first author—conceptualisation, data collection, article writing and proof-reading. SND—conceptualisation, supervision, proof-reading. AN—conceptualisation, supervision, proof-reading.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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